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Pediatric Surgery Update Review Articles
BILIARY ATRESIA: An Overview
IMPACT OF MINIMALLY INVASIVE SURGERY IN CHILDREN
NEURONAL INTESTINAL DYSPLASIA: A Role for Surgery?
ROLE OF INTERNET IN PEDIATRIC SURGERY
THE PEDIATRIC INGUINAL HERNIA: Is Contralateral Exploration Justified?
TRICHOBEZOAR IN A 11-YEAR OLD GIRL: A Case Report
DELAYED DIAGNOSIS AND MANAGEMENT OF BRONCHIAL RUPTURE FOLLOWING BLUNT THORACIC TRAUMA - Carlos E. Prieto-Velhote, Manoel C. Prieto-Velhote, Tais F.de Oliveira-Velhote
VENTILATORY STRATEGIES TO PREVENT LUNG INJURY IN THE NEWBORN - Ramzi A. Kilani, MD, FAAP
THE INCIDENCE OF INGUINAL HERNIA ASSOCIATED WITH UNDESCENDED TESTIS. - Naguib N.N., Samuel D.G., Izzidien A.Y.
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NEURONAL INTESTINAL DYSPLASIA:
A Role for Surgery?
by: Humberto L. Lugo-Vicente, MD. FACS, FAAP*
During the past two decades motilities disorders of bowel function in infants and children have been distinguished with the help of histochemical, radiographics and manometric studies performed to patients. The surgeon is occasionally asked to render care to many of this unfortunate children in the hope that sphincterotomy, enterostomies, resections, and pull-through surgery can yield a cure or improvement of symptoms.
Motilities disorders plague the clinical picture of the child with recurrent bouts of constipation, diarrhea, enterocolitis, bloody stools, abdominal distension, colicky abdominal pain, and encopresis. Symptoms can be present since birth, or develop later in the child's life. There is progressive constipation and megacolon formation in many of this infants.
Normal propulsive motility of the gastrointestinal tract is dependent on normal anatomy, musculature and innervation of the bowel wall. Control is exerted by extrinsic and intrinsic nerves, chemoreceptors and mechanoreceptors located in the bowel wall that unfortunately are practically inaccessible for investigation and experimentation (1).
Gastrointestinal motility disorders are viewed as those about the esophagus (i.e. achalasia), gastro-pyloric area (i.e. hypertrophic pyloric stenosis), small bowel, colonic (Hirschsprung's disease, neuronal intestinal dysplasia), and pseudo-obstructive problems such as the adynamic bowel of prematures and chronic intestinal pseudo-obstruction (CIPO) (1).
Using colorectal biopsy specimens patients with inborn errors of colonic innervation have been classified as: aganglionosis (52%), hypoganglionosis (5%), and neuronal intestinal dysplasia (43%). An additional half of the biopsies will not fit this classification due to moderate malformation such as dysganglionosis, hypogenetic and heterotopic nerve cell characteristics (2).
Neuronal intestinal dysplasia (NID), first described by Meier-Ruge in 1971, is a poorly understood colonic motility disorder of neuronal structure in the bowel wall (3). NID is characterized by several histochemical and pathological findings such as:
1- hyperplasia of submucous and myenteric plexus with formation of giant ganglia,
2- isolated ganglion cells in the mucosal lamina propia and between muscle layers of muscularis mucosa,
3- moderate elevation of acetylcholinesterase in the parasympathetic fibers of mucosal lamina propia and circular muscle, and
4- hypoplastic or aplastic myenteric plexus sympathetic innervation (4,5).
The most characteristic alteration identified is acetylcholinesterase elevation of parasympathetic fibers, and the less reliable diagnostic feature, the giant ganglion cells. Not all patients demonstrate the whole spectrum of histological traits depicted above.
The etiology of NID has eluded us. The development of NID in previously normal bowel, the association with other intestinal malformations, and the clinical heterogeneity of this patients suggest that NID is a reaction of the neural intestinal system caused by congenital obstructive factors or inflammatory disease (6). Major histocompatibility complex II expression has been found markedly elevated in Hirschsprung's Disease (HD) and NID cases (normally nerve tissue is deficient of the antigen). This has lent support that the bowel may be highly susceptible to an abnormal response of immune origin (7).
Initially described as a localized and disseminated form of disease, Fadda in 1983 re-classify it into two types ( A and B), with a common clinical feature in both: chronic constipation and megacolon (8). In type A the disease is confined to the colon causing a functional bowel obstruction with acute onset. Symptoms are present since birth and comprise: constipation, ulcerative colitis, painful straining, and bloody stools. Contrast studies of the colon display rigid, spastic segmental contraction of bowel, ulcer, erosions, and no peristalsis. Manometric studies will show absent recto-inhibitory reflex (4,5,8,9,10). Histologically there is aplasia or hypoplasia of myenteric sympathetic innervation and increase acetylcholinesterase activity in lamina propia, circular muscle and muscularis mucosa (5,9). Ganglion cells are present, excluding the diagnosis of Hirschsprung's disease (HD).
Type B NID is more common, symptoms commence around six month of life, there is constipation and adynamic distal bowel with megacolon undistinguishable from Hirschsprung's disease. Histology is characterized by dysplastic parasympathetic submucous plexus with giant ganglion cells and hyperplasia, elevated acetylcholinesterase levels, and isolated ganglia in lamina propia (5,8,9,10). This type is more commonly found associated to HD, anorectal malformations, MEN IIB syndrome and CIPO (8). Manometry shows that these patients have non-proportional relaxation of the internal anal sphincter, anorectal hyperexcitability, and increase amplitude of anorectal fluctuations (11).
NID and HD
It is estimated that isolated NID is eight time rarer, and affects longer segments of bowel than Hirschsprung's Disease (HD) (5,12). Both disease process have been reported in 20-75% of patients (10). This has shifted the attention to patients with HD who persists with clinical problems after adequate pull-through resection.
HD is characterized by lack of enteric ganglion cells, hyperplasia of abnormal nerve fibers and a non-propulsive, non-relaxing segment of bowel. Classically the etiology is attributed to a failure in cranio-caudal migration of parasympathetic neural crest cells to the distal bowel. Factors leading to failure of differentiation after migration of neural crest cells could be responsible for HD complex etiology. A plausible explanation for the failure of relaxation of the bowel involved is a deficiency of enteric inhibitory nerves that mediates the relaxation phase of peristalsis. This nerves are intrinsic to the gut and are classified as non-adrenergic and non-cholinergic. Nitric oxide (NO) has recently been implicated as the neurotransmitter that mediates the relaxation of smooth muscle of the GI tract in HD. It's absence in aganglionic bowel might account for the failure of relaxation during peristalsis. Besides, adhesions molecules (absent in aganglionic bowel) during early embryogenesis might restrict the neuro-ectodermal origin involved in the initial contact between nerves and muscle cell (synaptogenesis) suggesting that developmental anomaly of innervated muscle and absent NO causes the spasticity characteristic of HD (1,2,7,13,14.,15). Initial management consist of leveling colostomy in ganglionic bowel with later pull-through surgery.
Patients with symptoms of obstruction (constipation, enterocolitis) persisting after surgery for HD could be hastened by: mechanical (anastomotic stricture) reasons, functional (NID, residual aganglionosis) problems, and infectious etiology (C. Difficile) (16). Sonographic follow-up analysis of colonic motility in patient with HD and NID after corrective surgery for HD shows that with time the dysmotility changes of the ganglionic NID colon improves (17). Retrospectives studies evaluating the influence of retained NID colon in patients with repaired HD have identified that the NID segment of bowel can be preserved without increasing the risk of morbidity or mortality to them (18). The actual incidence of NID associated with HD could be explored by monitoring the histological findings of the proximal bowel during initial colostomy construction. Biopsy of this colostomy segment should warned us of the presence of NID changes.
NID and CIPO
Chronic Intestinal Pseudo-Obstruction (CIPO) is a rare disorder of intestinal motility in infants and children characterized by recurrent attacks of abdominal pain, distension, vomiting, constipation and weight loss lacking obvious mechanical lesions. The disease can be familial or sporadic. Suggested etiology is degeneration of enteric nervous or smooth muscle cells. The diagnosis is based an history, physical exam, radiographies and motility studies. X-Ray hallmarks are: absent strictures, absent, decreased or disorganized intestinal motility, and dilated small/large bowel loops. Associated conditions identified in 10-30% of patients are bladder dysfunction (megacystis) and neurological problems. Histologic pattern portrayed: myenteric plexus hyperplasia, glial cell hyperplasia, and small ganglion cells (hypoganglionosis) (19,20,21). Management is primary supportive: intestinal decompression (NG), long-term TPN and antibiotic prophylaxis (22,23). Motility agents are unsuccessful. Venting gastrostomy with home parenteral nutrition has shortened the high hospitalization rate associated to this disease process24. A similar condition can be seen in early fed prematures due to immaturity of intestinal motility.
NID accounts for 30% of patients with CIPO symptoms as attested by histologic sections (25). It is illogical to relate symptoms of dysmotility to submucous plexus changes seen on biopsy, and almost entirely ignore the myenteric plexus that is ultimately concerned with colonic motor activity.
Management of NID
Management has switched from a more aggressive attitude to a more conservative approach. Repeated bouts of obstructive episodes have been an indication for colostomy, and more serious symptoms have ended with resection (5). The indications for surgery in NID cannot be sustained alone on the histochemical picture of the biopsy specimen, but on the clinical situation of the patient, since NID is best a histopathologic condition rather than an unique clinico-pathological entity (26,27,28).
Type A NID with its acute fulminant course during the neonatal period has an unfavorable progression with early indications for surgery. Colostomy is reserved for neonatal obstruction with associated severe enterocolitis. It is probable that many sick infants will show a clinical picture similar to severe necrotizing enterocolitis before being diagnosed the condition. A word of caution should be exerted against extensive colonic resections for this disease process. This could impair colonic water absorption, stool consistency and may overwhelm fecal incontinence problems (28).
Type B NID runs a more chronic path and management is more conservative. There is clinical evidence gathered that the colonic motility disturbance associated matures and improves by the fourth year of life of the child (5,10). If problems persists beyond the fourth year of life more aggressive management is warranted. In general patients can be managed with saline colonic irrigations, TPN, high dose lactulose, and paraffin oil until clinical improvement and normalization of biopsy results are obtained (28,29). Prokinetic agents (cisapride) can be of help in some groups of patients, with the addition of neostigmine in clinically resistant cases16. Surgery is rarely deemed necessary in this subgroup of patients.
Neuronal intestinal dysplasia is a poorly understood colonic motility disorder with characteristic histopathological findings and clinical presentation. It is often associated with Hirschsprung's disease and can constitute a cause of failure of clinical improvement after adequate resectional pull-through surgery. Other conditions associated with NID are: CIPO, anorectal malformations and MEN II syndrome patients.
To increase the diagnostic yield of NID the pathologist should be aware and use histochemistry evaluation of the rectal biopsy specimen in patients with history of constipation or unexplained bouts of diarrhea. Adequate sampling of the temporary proximal colostomy done to HD patients should be examined for NID pathological changes.
Treatment has centered around the clinical picture with most cases managed medically with prokinetic agents, colonic irrigations, and bowel cathartics until improvement and normalization of histology occur. There is evidence of progressive maturation of the enteric nervous system with time. Surgery is indicated for patients with severe clinical deterioration after failed medical management.
1- Molenar JC, Tibboel D, van der Kamp AWM, et al: Diagnosis of Innervation-Related Motility Disorders of the Gut and Basic Aspects of Enteric Nervous System Development. Prog Pediatr Surg 24:173-185, 1989
2- Meier-Ruge W: Classification of malformations of colorectal innervation. Verh Dtsch Ges Pathol 75:384-385, 1991
3- Meier-Ruge W: Ueber ein Erkrankungsbild des Kolos mit Hirschsprung-Symptomatik. Verh Dtsch Ges Pathol 55:506, 1971
4- Scharli AF, Meier-Ruge W: Localized and Disseminated Forms of Neuronal Intestinal Dysplasia Mimicking Hirschsprung's Disease. J Pediatr Surg 16(2):164-170, 1981
5- Munakata K, Okabe MI, Sueoka H: Clinical and Histologic Studies of Neuronal Intestinal Dysplasia. J Pediatr Surg 20(3):231-235, 1985
6- Sacher P, Briner J, Hanimann B: Is neuronal intestinal dysplasia (NID) a primary disease or a secondary phenomenon? Eur J Pediatr Surg 3(4):228-230, 1993
7- Hirobe S, Doody DP, Ryan DP, et al: Ectopic Class II Major Histocompatibility Antigens in Hirschsprung's Disease and Neuronal Intestinal Dysplasia. J Pediatr Surg 27(3):357-363, 1992
8- Rintala R, Rapola J, Louhimo I: Neuronal Intestinal Dysplasia. Prog Pediatr Surg 24:186-192, 1989
9- Fadda B, Maier WA, Meier-Ruge W, et al: Neuronal Intestinal Dysplasia: Critical 10-years' analysis of Clinical and Biopsy Results. Z Kinderchir 38(5):305-311, 1983
10- Pistor G: Functional Colonic Ultrasonography: Normal Findings of Colonic Motility and Follow-Up in Neuronal Intestinal Dysplasia. Prog Pediatr Surg 24:155-164, 1989
11- Krebs C, Silva MC, Parra MA: Anorectal Electromanometry in the Diagnosis of Neuronal Intestinal Dysplasia in Childhood. Eur J Pediatr Surg 1(1):40-44, 1991
12- Briner J, Oswald HW, Hirsig J, et al: Neuronal Intestinal Dysplasia- Clinical and Histochemical Findings and its association with Hirschsprung's Disease. Z Kinderchir 41(5):282-286, 1986
13- Robey SS, Kuhajda FP, Yardley JH: Immunoperoxidase Stains of Ganglion Cells and Abnormal Mucosal Nerve Proliferations in Hirschsprung's Disease. Human Pathol 19(4):432-437, 1988
14- Gittes GK, Kim J, Yu G, et al: Severe Constipation with Diffuse Intestinal Myenteric Hyperganglionosis. J Pediatr Surg 28(12):1630-1632, 1993
15- Cuffari C, Rubin SZ, Krantis A: Routine Use of the Nitric Oxide Stain in the Differential Diagnosis of Hirschsprung's Disease. J Pediatr Surg 28(9):1202-1204, 1993
16- Moore SW, Millar AJW, Cywes S: Long-Term Clinical, Manometric, and Histological Evaluation of Obstructive Symptoms in the Postoperative Hirschsprung's Patient. J Pediatr Surg 29(1):106-111, 1994
17- Pistor G, Hofman- von Kap-herr S: Functional Colon Sonography in Neuronal Intestinal Dysplasia. Fortschr Med 102(14):397-400, 1984
18- Hanimann B, Inderbitzin D, Briner J, et al: Clinical Relevance of Hirschsprung-associated Neuronal Intestinal Dysplasia. Eur J Pediatr Surg 2(3):147-149, 1992
19- Bindl L, Emons D, Haverkamp F, et al: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: A Neuropathy? Z Kinderchir 44(4):249-252, 1989
20- Gil-Vernet JM, Casasa JM, Boix-Ochoa J, et al: Intestinal dysmotility pseudo obstruction. Cirugia Pediatrica 5(2):87-95, 1992
21- Peck SN, Altschuler SM: Pseudo-obstruction in Children. Gastroenterol Nursing 14(4):184-188, 1992
22- Fonkalsrud EW, Pitt HA, Berquist WE, et al: Surgical Management of Chronic Intestinal Pseudo-obstruction in Infancy and Childhood. Prog Pediatr Surg 24:221-225, 1989
23- Ament ME, Vargas J: Diagnosis and Management of Chronic Intestinal Pseudo-obstruction Syndromes in Infancy and Childhood. Arquivos de Gatroenterologia 25(3):157-165, 1988
24- Pitt HA, Mann LL, Berquist WE, et al: Chronic Intestinal Pseudo-obstruction. Management with Total parenteral nutrition and a venting enterostomy. Arch Surg 120(5):614-618, 1985
25- Milla PJ, Smith VV: Intestinal Neuronal Dysplasia. J Pediatr Gastroenterol Nutr 17:356-357, 1993
26- Schofield DE, Yunis EJ: Intestinal Neuronal Dysplasia. J Pediatr Gastroenterol Nutr 12:182-189, 1991
27- Scharli AF: Neuronal Intestinal Dysplasia. Pediatr Surg Int 7(1):2-7, 1992
28- Koletzko S, Ballauff A, Hadziselimovic F, et al: Is Histological Diagnosis of Neuronal Intestinal Dysplasia Related to Clinical and Manometric Findings in Constipated Children? Results of a Pilot Study. J Pediatr Gastroenterol Nutr 17:59-65, 1993
29- Simpser E, Kahn E, Kenigsberg K, et al: Neuronal Intestinal Dysplasia: Quantitative Diagnostic Criteria and Clinical Management. J Pediatr Gastroenterol Nutr 12(1):61-64, 1991
*Associate Professor in Pediatric Surgery, Department of Surgery, University of Puerto Rico, School of Medicine, and Universidad Central del Caribe, School of Medicine.
Address reprints to: Humberto L. Lugo-Vicente, MD- P.O. Box 10426, Caparra Heights Station, San Juan PR 00922-0426. Tel (787) 786-3495 Fax (787) 720-6103 E-mail: titolugo@coqui.net
Published in: Boletin Asociación Medica de Puerto Rico 87(3-4): 60-63, 1995
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by: Humberto L. Lugo-Vicente, MD, FACS, FAAP
Pediatric Surgeon*
Biliary atresia (BA) continues to originate controversy and despair among physicians and patients alike. With the development of liver transplant and new techniques in small size orthotopic replacement, a new insight in BA surgical care has come forth. Where do we stand today in therapy?, What causes this terrible disease?, and What should be the guidelines in management of the cholestatic infants? are some issues reviewed in this monograph.
This condition is the most common cause of persistently direct (conjugated) hyperbilirubinemia in the first three months of life. Kasai portenterostomy and liver transplantation battle hand in hand to become today's leading therapy. One thing is for sure, results after portoenterostomy are decided by the promptness of the initial work-up and referral to surgery. More than 80% of BA babies have satisfactory bile flow after hepatic-portoenterostomy if the procedure is done before their 8th week of life. It's certain, we must view newborns with persistent cholestasis as urgent cases that need immediate assessment and management of their condition.
Biliary atresia is characterized by progressive inflammatory obliteration of the extrahepatic bile ducts, an estimated incidence of one in 15,000 live births, and predominance of female patients (1). The first comprehensive paper was written by J. Thompson of Edimburg in 1882. J.B. Holmes in 1916 classified cases as correctable (10-15%), and non-correctable (85-90%) depending on the pathologic structures identified at the porta hepatis area, and Dr. William Ladd in 1928 ventures into the first successful bile-enteric anastomosis (2). In 1959 Kasai and Suzuki described a new procedure for biliary atresia that transformed management during the following 30 years (3). By 1980 most infants with biliary atresia were managed with the Kasai procedure.
Etio-pathological Considerations
Although much has been written of BA, its pathogenesis remains speculative. The original theory of an embryogenic accident that settled in occlusion of the extrahepatic biliary tree, was challenged by the absence of jaundice at birth, and histologic evidence of patent biliary ducts that progressively disappeared during the first months of life (4). Findings in the obstetric history of older parents, high use of drugs, associated illness, and fetal loss suggested the possibility of exposure to a noxious agent during the reproductive processn (5).
The disease is the result of an acquired inflammatory process with gradual degeneration of the epithelium of the extrahepatic biliary ducts causing luminal obliteration, cholestasis, and biliary cirrhosis (6). The timing of the insult after birth suggests a viral etiology obtained transplacentally; Reovirus type 3 has been implicated (7). Up to 68% of infants with BA show antibodies to Reovirus type 3 in serum, although no viral particle has been isolated (8). Oral and intraperitoneal administration of reovirus to experimental animals causes hepatitis and biliary tract inflammation with fibrosis similar to BA (4,7,9,10).
Almost 20% of patients have associated anomalies such as: polysplenia, malrotation, situs inversus, preduodenal portal vein and absent inferior vena cava. This raises the possibility of a genetic mutation and the hypothesis of laterality with defective development of one side of the body when compared with the contralateral image side (11,12).
Histopathology is distinguished by an inflammatory process in several dynamic stages with progressive destruction, scar formation, and chronic granulation tissue of bile ducts (13). The pathologist cannot be categoric in the diagnosis of BA, since liver changes are compatible with an extrahepatic mechanical obstruction. Two changes merits mention: portal tract interlobular ducts proliferation, and cholestatic histology. Other findings are: giant cell transformation, focal hepatic cell necrosis, interlobular space and portal tract fibrosis. Conditions displaying a similar histology are: alfa-1-antitrypsin deficiency, Alagille's syndrome (hypoplasia of bile ducts), and TPN induced cholestasis. Immunohistochemistry of the portal ducts can show the presence of the epithelial membrane antigen in large ducts, changes specific for BA (13,14).
Three types of microscopic biliary structures have been identified in the most proximal aspect of the extrahepatic remnant removed surgically near the area of the porta hepatis. These are: bile ducts with a mean diameter of 500 µ and bile in the lumen, collecting ductules of biliary glands with a mean diameter of 250 µ, and biliary glands without bile and a mean diameter of 100 µ (15). Postoperative biliary flow after Kasai correlates with the presence and size of bile ducts and collecting ductules exclusively (15,16,17,18). Electron microscopy can exhibit canalicular biliary membrane filaments whose volume and appearance correlates with adequate bile flow (19). The degree of hepatic fibrosis associated also relates with post-op biliary flow. Kasai portoenterostomy relies on the realization that the microscopic structures in the porta hepatis will act as micro-conduits of bile as an internal biliary fistula is created with a segment of bowel. All will eventually merged into one or two ducts.
BA classification is based on findings upon operative cholangiography and the macroscopically specimen morphology as shown inFigure 1 (percent's are obtained from the National Biliary Atresia Registry (20)).
Clinical Manifestations and Diagnosis
Physiologic jaundice of the newborn is a common, benign, and self-limiting condition. Persistent conjugated hyperbilirubinemia (greater than 20% of total or 1.5 mg%) should be urgently appraised. Initial evaluation should include a well-taken history and physical exam, partial and total bilirubin determination, type and blood group, Coomb's test, reticulocyte cell count and a peripheral smear (21).
Cholestasis means a reduction in bile flow in the liver, which depends on the biliary excretion of the conjugated portion. Reduce flow causes retention of biliary lipoproteins that stimulates hypercholesterolemia causing progressive damage to the hepatic cell, fibrosis, cirrhosis and altered liver function tests (22). The etiology of the cholestatic infant is classified in several groups as depicted inTable 1 (23). Those structurally related etiologies are surgical causes of jaundice.
In BA the patient develops insidious jaundice by the second week of life. The baby looks active, not acutely ill and progressively develops acholic stools, choluria and hepatomegaly (24). Non-surgical source of cholestasis shows a sick, low weight infant who is jaundiced since birth. The diagnostic evaluation of the cholestatic infant should include a series of lab tests that can exclude perinatal infectious (TORCH titers, hepatitis profile), metabolic (alpha-1-antitrypsin levels), systemic and hereditary causes (25,26).
Total bilirubin in BA babies is around 6-10 mg%, with 50-80% conjugated. Liver function tests are nonspecific. Lipoprotein-X levels greater than 300 mg% and Gamma Glutamyl Transpeptidase (GGT) above 200 units% suggest the diagnosis (13). The presence of the yellow bilirubin pigment in the aspirate of duodenal content excludes the diagnosis of BA.
Ultrasound study of the abdomen should be the first diagnostic imaging study done to cholestatic infants to evaluate the presence of a gallbladder, identify intra or extrahepatic bile ducts dilatation, and liver parenchyma echogenicity. BA sonographic characteristics are: absent, or small gallbladder that does not contract upon hormonal stimuli, and increased liver echogenicity (27). The postprandial contraction of the gallbladder eliminates the possibility of BA even when nuclear studies are positive. This changes are consistent with neonatal hepatitis (27,28,29).
Nuclear studies of bilio-enteric excretion (DISIDA) after pre-stimulation of the microsomal hepatic system with phenobarbital for 3-5 days is the diagnostic imaging test of choice (30). BA patients will show an increase hepatic uptake during early injection without significant bilio-enteric excretion in delayed films (24 hrs.) The presence of the radio-isotope in the GI tract excludes the diagnosis of BA. Hepatocellular causes of jaundice will show poor concentration of isotope in the liver associated to delayed or absent excretion.
Percutaneous liver biopsy should be the next diagnostic step if previous studies suggest BA and the infant has no associated coagulopathy (31). Findings of BA are: bile duct proliferation and fibrosis. Unfortunately this changes are nonspecific of BA and can be found in neonatal hepatitis (2). The mini-laparotomy is the final diagnostic alternative. Through a small right subcostal incision a gallbladder cholangiogram and liver biopsy is done. Those infant with radiographic evidence of patent extrahepatic biliary tract has no BA. Small, hypoplastic ducts are associated to Alagille's syndrome (32). In BA the gallbladder can be a fibrous remnant, present but filled with white bile (hydrops), with no communication with the biliary tree, or with distal extrahepatic communication. Once the diagnosis of BA is established intraoperative, a Kasai enterostomy is constructed.
The prenatal diagnosis of BA was first reported in 1986. Antenatal sonography showed a cystic structure in the fetal abdomen confirmed as BA 76 hours later (33). Recently, diagnostic laparoscopy has been found useful in the evaluation of the cholestatic infant (34).
Medical management of BA is uniformly fatal.
Kasai portoenterostomy has decreased the mortality of BA during the last 30 years. The procedure done before the first 6-8 weeks of life will yield biliary flow in 75-80% of infants.
Kasai procedure consists of removing the obliterated extrahepatic biliary system, and anastomosing the most proximal part to a bowel segment. Adequate illumination and magnifying loupes are essential. The initial mini-laparotomy incision is extended once the diagnosis is confirmed. The gallbladder, cystic duct and extrahepatic remnant is mobilized, the distal common bile duct is ligated proximally and the dissection goes proximally toward the porta hepatis. At the porta hepatis the remnant looks like a fibrous cord with the shape of a cone. At this point the cord is transected perpendicularly to the liver level and the specimen send to the pathologist. The raw surface left over is anastomosed to a defuntionalize limb of jejunum in a roux-en-Y fashion using small suture bites. It is estimated that 10-15% of cases have distal patency of the extrahepatic bile ducts and the gallbladder can be used as conduit, instead of bowel. This variation in the procedure eliminates the possibility of developing cholangitis, but increases the incidence of anastomotic leak from ischemia during dissection (2,13,24,30,35,36).
Table 2 shows complications associated to Kasai procedure. Cholangitis is probably the most common, serious, and occur in 90% of patient who drain bile during the first year of life. Clinically they manifest fever, leukocytosis, elevated bilirubin in serum, and deterioration of liver function tests. Management consists of antibiotics. Main cause is ascending infection through the interposed bowel segment associated to destruction of lymphatic drainage. Recurrent attacks of cholangitis cause progressive hepatic damage (37,38). Sonography can show cystic intrahepatic bile duct dilatations in children with repetitive attacks. This cysts are either non-communicating or communicating. Management may consist of percutaneous transhepatic drainage, and sclerosis of non-communicating cysts (39). To reduce the incidence of cholangitis the prophylactic uses of antibiotics during the first 18-24 months of life is recommended. Constant attacks of cholangitis are associated to sudden cease of bile flow needing steroid therapy or re-operation.
In pursuit of reducing the episodes of cholangitis Kasai procedure was modified providing an external conduit to diminish the intraluminal pressure and secretory liver gradient. This enabled the measurement of bile volume and concentration. This external conduits did not reduce the incidence of cholangitis. Furthermore they were plagued with several complications such as: enterostomal varices, bile salts deficiency, electrolytes disturbances, and a later procedures to close the enterostomy (40,41). Not exteriorized interposed segments of bowel with intussuscepted valves and jejunal interposition between porta hepatis and duodenum has proved to reduce the incidence of cholangitis increasing survival (42).
Almost three-fourth of patients will develop portal hypertension in spite of adequate postoperative bile flow. They will manifest esophageal varices, hypersplenism, and ascites. Factors associated to this complication are: history of cholangitis, older infant during surgery, re-operations, and elevated portal pressure during initial surgery (43). Esophageal varices usually develop 2-8 years after portoenterostomy and are managed with endoscopic sclerotherapy effectively. Secondary hypersplenism can be managed with partial embolization of the splenic artery. Ascites will need salt restriction and diuretics. Later in life a reduction in this complications is linked to the spontaneous development of portosystemic shunts.
Essential fatty acids malabsorption leading to caloric and nutritional deficiencies should be managed with high concentration medium chain triglyceride formulas. This malabsorption could lead to A, D, K, and E fat soluble vitamin deficiency. Maternal milk is insufficient to provide caloric needs, and formula supplementation should be provided, even with forced tube feedings. The objective is to provide 150 calories and 3-4 grams of protein daily per kilogram of weight, along with vitamin supplementation.
Pruritus is difficult to manage. Antihistamines are first line of treatment due to their tranquilizing effect. Other drugs used are cholestyramine to reduce enterohepatic circulation, phenobarbital to increase biliary flow and rifampin.
The new era of liver transplantation, better surgical techniques, smaller donor accessibility and new inmunosuppresor agents (cyclosporine) has brought second thoughts to the use of Kasai enterostomy. Major liver transplant centers see Kasai as a complementary procedure of historic interest. From 75-80% of patient with BA will be candidates for orthotopic liver transplant (44,45). Some recommendations for those patient initially undergoing a Kasai procedure who could become future candidates for liver transplantation are: Use oblique incisions to reduce adhesions to the liver capsule, the roux-en-Y of jejunum should not be long (40 cm) to be reused later avoiding problems with cyclosporine absorption, and avoid enterostomies (22).
Without surgical management survival of infants with BA is 8-12 months, most dying of irreversible liver failure.
Results of portoenterostomy are associated to a group of prognostic factors studied (seeTable 3). The two most important factors are: age at surgery and histologic liver changes (46,47). Other factors are: caucasian race, morphologic type of BA, size of ductal structures at porta hepatis, postop bile flow, degree of hepatic fibrosis, surgical technique used, type of surgical reconstruction, incidence of cholangitis, and development of symptomatic portal hypertension (20,26).
Age at surgery is probably the most determinant factor of survival after Kasai procedure. Ideally it should be done before 60 days of life. The presence of bile ducts in the extrahepatic remnant and the degree of liver fibrosis correlates with the age of the patient. The older the patient the lesser the possibility of findings adequate size ducts and the worst the hepatic fibrosis. The degree of hepatic fibrosis and degeneration of intrahepatic ducts correlates directly with prognosis irrespective of the size of the ductal micro-structures identified in the porta hepatis area (47,48).
General results of Japanese series fare better when compared to Occident. This is attributed to a racial influence associated. Caucasian race has a worst prognosis than oriental children (20). Morphologically those patient with patent distal extrahepatic ducts and a gallbladder Kasai constructed do better. This is the result of a more physiologic conduit, a reduced number of cholangitis episodes, and a low level inflammatory process (49).
The type of surgical reconstruction has no relationship to survival. Continual attacks of cholangitis will progress to hepatic fibrosis and irreversible damage related with early development of portal hypertension (20). Postoperative bile flow correlates with improved immediate survival, with no guarantee of long term survival.
Results reported by several series can be appreciated inTable 4. Up to 80% of children undergoing portoenterostomy before 60 days of age will drain bile and jaundice will improve. At five years survival will be 29-60%, 25-35% at ten years and 8% at twenty years (13,20,30,49-52).
The greatest mortality in BA occurs during the first two years after portoenterostomy. Long-term follow-up of children living more than ten years after Kasai shows moderate hepatic dysfunction, controlled portal hypertension, a normal intellectual coefficient, and a good quality of life (49,53).
Persistent jaundice in the newborn must be managed urgently. A diagnosis should be established early in the life of the child and Kasai portoenterostomy offered to those infants before their eighth week of life. This will allow that more than one-third of BA children survive this terrible disease.
Hepatic transplantation is reserved for those patients with failed portoenterostomy, progressive liver failure or late-referral to surgery. Liver transplant indications should include patients with bilirubin levels above 10 mg%, low albumin levels, weight loss and uncontrolled ascites.
1- Shim KT, Kasai M, Spence MA: Race and biliary atresia. In Kasai M, Shiraki K (eds): Cholestasis in Infancy. Tokyo Press, 1980, pp 5-10
2- Hays DM, Kimura K: Biliary Atresia: New Concepts of Management. Curr Probl Surg 18(9):541-608, 1981
3- Kasai M, Suzuki S: A new operation for noncorrectable biliary atresia- hepatic portoenterostomy. Shujutsu 13:733-739, 1959
4- Schmeling DJ, Strauch ED, Hoffman MA: Epidemiological and Experimental Observations of Biliary Atresia. In Hoffman MA (ed): Current Controversies in Biliary Atresia. R.G. Landes Co, 1992, pp 15-27
5- Silveira TR, Salzano FM, Howard ER: The relative importance of familial, reproductive and environmental factors in biliary atresia: etiological implications and effect on patient survival. Braz J Med Biol Res 25(7):673-681, 1992
6- Landing BH: Considerations of the pathogenesis of neonatal hepatitis, biliary atresia, choledochal cyst- the concept of infantile obstructive cholangiography. Prog Pediatr Surg 6:113-139, 1974
7- Morecki R, Glaser JH, Horwitz MS. Etiology of biliary atresia: the role of Reo 3 virus. In Daum F, Extrahepatic Biliary Atresia. New York: Marcel Decker, 1983, pp 1-9
8- Morecki R, Glaser JH, Cho S, et el. Biliary Atresia and reovirus type 3 infection. N Engl J Med 307(8):481-484, 1982
9- Riepenhoff-Talty M, Schaekel K, Clark, et al: Group A rotaviruses produce extrahepatic biliary obstruction in orally inoculated mice. Pediatr Res 33(4):394-399, 1993
10- Hart MH, Kaufman SS, Vanderhoof JA, et al: Neonatal hepatitis and extrahepatic biliary atresia associated with cytomegalovirus infection in twins. Am J Dis Child 145(3):302-305, 1991
11- Davenport M, Savage M, Mowat AP, et al: Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup. Surgery 113(6):662-668, 1993
12- Carmi R, Magee CA, Neill CA, Karrer FM: Extrahepatic biliary atresia and associated anomalies: etiologic heterogeneity suggested by distinctive patterns of associations. Am J Med Genet 45(6):683-693, 1993
13- Ohi R, Ibrahim M: Biliary Atresia. Seminars in Ped Surg 1(2):115-124, 1992
14- Witzleben CL: Pathology of Biliary Atresia. In Hoffman MA (ed): Current Controversies in Biliary Atresia. R.G. Landes Co. 1992, pp 28-44
15- Ohi R, Shikes RH, Stellin GP, Lilly JR: In Biliary Atresia Histology Correlates with Bile Flow. J Ped Surg 19(4):467-470, 1984
16- López-Gutierrez JC, Vázquez J, Ros Z, et al: Histopatología de la atresia biliar: correlación con el flujo biliar. Cir Pediatr 4(1):16-18, 1991
17- Schweizer P: "Extrahepatische Gallengangsatresie"-Eine analytische Bewertung prognosticher Faktoren. Ein Beitrag zu einem rationalen Therapieansatz. Z Kinderchir 45(6):365-370, 1990
18- Ohya T, Miyano T, Kimura K: Indications for portoenterostomy based on 103 patients with Suruga II modification. J Pediatr Surg 25(7):801-804, 1990
19- Segawa O, Miyano T, Fujimoto T, et al: Actin and myosin deposition around bile canaliculi: a predictor of clinical outcome in biliary atresia. J Pediatr Surg 28(6):851-856, 1993
20- Karrer FM, Lilly JR, Stewart BA. Hall RJ: Biliary Atresia Registry, 1976 to 1989. J Ped Surg 25(10):1076-1081, 1990
21- Hicks BA, Altman RP: The Jaundiced Newborn. Ped Clinic North Amer 40(6):1161-1175, 1993
22- Martinez-Ibañez V, Boix-Ochoa J, Lloret J, et al: Paediatric liver transplantation: life after portoenterostomy in biliary atresia. J Pediatr Surg 27(1):830-832, 1992
23- Maller ES, Piccoli DA: Diagnostic Evaluation and Care of the Child with Biliary Atresia. In Hoffman MA (ed): Current Controversies in Biliary Atresia. R.G. Landes Co.,1992, pp 45-61
24- Karrer FM, Lilly JR: Biliary Atresia. In Grosfeld JL (ed): Common Problems in Pediatric Surgery. Mosby Year Book, 1991, pp 197-205
25- Rosenthal P, Sinatra F: Jaundice in Infancy. Pediatrics in Review 11(3):79-86, 1989
26- Tagge DU, Tagge EP, Drongowski RA, et al: A long-term experience with biliary atresia. Reassessment of prognostic factors. Ann Surg 214(5):590-598, 1991
27- Okasora T, Toyosaka A, Muraji T, et el: The use of ultrasonography in the diagnosis of biliary atresia. Ped Surg Int 2(4):231-234, 1987
28- Green D, Carroll B: Ultrasonography in the Jaundice Infant: a new approach. J Ultrasound Med 5:323-329, 1986
29- Ikeda S, Sera Y, Akagi M: Serial ultrasonic examination to differentiate biliary atresia from neonatal hepatitis- special reference to changes in size of the gallbladder. Eur J Pediatr 148(5):396-400, 1989
30- Altman RP: Infantile Obstructive Jaundice. In Schiller M (ed): Pediatric Surgery of the Liver, Pancreas and Spleen. W.B. Saunder Co., 1991, pp 59-75
31- Ridaura Sanz C, Navarro-Castilla E: Role of liver biopsy in the diagnosis of prolonged cholestasis in infants. Rev Invest Clin 44(2):193-202, 1992
32- Markowitz J, Daum F, Kahn E, et al: Arteriohepatic dysplasia. I Pitfalls in diagnosis and management. Hepatology 3:74-76, 1983
33- Greenholz SK, Lilly JR, Shikes RH, et al: Biliary Atresia in the Newborn. J Pediatr Surg 21(12):1147-1148, 1986
34- Schier F, Waldschmidt J: Experience with laparoscopy for the evaluation of cholestasis in newborns. Surg Endosc 4(1):13-14, 1990
35- Karrer FM, Lilly JR, Hall RJ: Biliary Tract Disorders and Portal Hypertension. In Ashcraft KW , Holder TM (eds): Pediatric Surgery. W.B. Saunders Co., 1993, pp 478-485
36- Miyano T, Fujimoto T, Ohya T, et al: Current concepts of the treatment of biliary atresia. World J Surg 17(3):332-336, 1993
37- Kobayashi A, Utsonomiya T, Obbe Y, et al: Ascending Cholangitis after successful repair of biliary atresia. Arch Dis Child 48:697-703, 1973
38- Gottrand F, Bernard O, Hadchouel M, et al: Late cholangitis after successful surgical repair of biliary atresia. Am J Dis Child 145(2):213-215, 1991
39- Nakama T, Kitamura, T, Matsui A, et al: Ultrasonographic findings and management of intrahepatic biliary tract abnormalities after portoenterostomy. J Pediatr Surg 26(1):32-36, 1991
40- Battaglini G, Previtera C, Messineo A, et al: Long term prognosis of patients with extrahepatic biliary atresia successfully treated with surgery. Our experience. Minerva Pediatr 43(7-8):493-498, 1991
41- Burnweit CA, Coln D: Influence of diversion on the development of cholangitis after hepatoportoenterostomy for biliary atresia. J Pediatr Surg 21(12):1143-1146, 1986
42- Tanaka K, Shirahase I, Utsonomiya H, et al: A valved hepatic portoduodenal intestinal conduit for biliary atresia. Ann Surg 213(3):230-235, 1991
43- Chiba T, Ohi R, Nio M, et al: Late complications in long-term survivors of biliary atresia. Eur J Pediatr Surg 2(1):22-25, 1992
44- Beath S, Pearmain G, Kelly D, et al: Liver transplantation in babies and children with extrahepatic biliary atresia. J Pediatr Surg 28(8):1044-1047, 1993
45- Kalayoglu M, D'Alessandro AM, Knechtle SJ, et al: Long-term results of liver transplantation for biliary atresia. Surgery 114(4):711-717, 1993
46- Adelman S: Prognosis of uncorrected biliary atresia. J Pediatr Surg 13:389-391, 1978
47- Suruga K, Tsunoda S, Deguchi, et al: The future role of hepatic portoenterostomy as treatment of biliary atresia. J Pediatr Surg 27(6):707-709, 1992
48- Karrer FM: Results of Portoenterostomy. In Hoffman's (ed): Current Controversies in Biliary Atresia. R.G. Landes Co., 1992, pp 73-80
49- Toyosaka A, Okamoto E, Okasora T, et al: Outcome of 21 patients with biliary atresia living more than 10 years. J Pediatr Surg 28(11):1498-1501, 1993
50- Carcassone M, Bensoussan A: Long-term Results in Treatment of Biliary Atresia. Progress Pediatr Surg 10:151-160, 1977
51- Akiyama H, Saeki M, Ogata T: Congenital biliary atresia: Our operative method and the operative results. Jpn J Pediatr Surg 10:673, 1978
52- Kobayashi A, Itabashi, Ohbe Y: Long-term prognosis in biliary atresia after hepatic portoenterostomy: Analysis of 35 patients who survived beyond 5 years of age. J Pediatr 105:243-246, 1984
53- Raffensperger JG: A long term follow-up of three patients with biliary atresia. J Pediatr Surg 26(2):176-177, 1991
* Associate Professor in Pediatric Surgery, Department of Surgery, University of Puerto Rico School of Medicine. Assistant Professor Surgery, Universidad Central del Caribe School of Medicine. Chief- Section of Pediatric Surgery, Department of Surgery, HURRA and Hospital San Pablo.
Address reprints to: Humberto L. Lugo-Vicente, MD, P.O. Box 10426, Caparra Heights Station, San Juan PR 00922-0426. Tel. (809)-786-3495 Fax (809)-720-6103 e-mail: titolugo@coqui.net
Published in Boletín Asociación Médica de Puerto Rico. Vol 87 (7-8-9): 147-153, 1995
THE PEDIATRIC INGUINAL HERNIA:
Is contralateral exploration justified?
From the 1950's until the present, the issue of contralateral exploration in the pediatric inguinal hernia patient has been fairly hotly debated. Proponents of routine contralateral exploration cite the high percentage of contralateral hernia a/o potential hernia (patent processus vaginalis) found at routine exploration, the avoidance of the cost of a second hospitalization, psychological trauma and anxiety to the child and parents over a second operation, the added risk of anesthesia of a second procedure, and the possibility of development of an incarcerated hernia at a later date (1).
Opponents of routine contralateral exploration states that many needless procedures are performed to avoid the development of only a few clinical hernias, that operation lengthened by the contralateral exploration may augment the risks and cite the increase risk of damage to the cord or gonad by the procedure itself (2).
A survey by Rowe and Marchildson in 1981, showed that 80% of pediatrics surgeons habitually explore the contralateral side in male patients, and 90% do so in females patients (3). Surgeons disagree in opinions about exploration depending upon the primary site of hernia, age, sex and the utilization of herniography or some intra-operative technique to check the contralateral side.
With such a broad variance of opinions regarding routine contralateral exploration we decided to study our experience and examined 161 successive patients who underwent bilateral hernia repair by the author during a 30 months period.
The medical charts of all infants and children who underwent consecutive repair of inguinal hernias by the same surgeon (HLV) from July 1985 to December 1987 at the Ramon Ruiz Arnau University Hospital (HURRA) and Hospital San Pablo (HSP), were retrospectively reviewed.
During this period 248 patients were identified, 87 who underwent a unilateral procedure and 161 patients with bilateral inguinal procedures. This last groups of patients comprise the study group. The charts were reviewed for sex, age at operation, gestational age, diagnostic characteristic, associated conditions, pre- and postoperative complications, findings during surgery, and outcome.
The findings during surgery were then compared in the sex, gestational age and age at operation subgroups to decide the effect that this variable had on the results using chi-square analysis. A p < 0.01 was considered significant.
The surgical procedure was performed under general endotracheal anesthesia using 3.5x magnifying loupes. A bilateral transverse inguinal crease incision was done and scarpa's fascia opened. The external spermatic ring was identified and without opening the external oblique fascia the cord was brought forth to the wound area. The hernia sac or processus vaginalis was carefully dissected free from the cord structures and ligated high with silk 000. No further dissection attempts were done if an obliterated processus vaginalis was identified. All specimens were submitted for pathological exam. Scarpa's reaproximated with polyglycolic acid 0000 suture and skin approximated with subcuticular chromic catgut 0000 suture.
There were 161 patients who underwent bilateral inguinal exploration and repair, 81 patients came from HURRA and 80 from HSP. Males were 89 and females 72 for a 1.2:1 ratio.
Age at operation is shown inTable 1, showing that almost two-thirds (61%) were infants younger than two years of age, generally the population of children referred to a pediatric surgeon. In only 110 pts. of the study group we could retrieve the data on gestational age; 89 pts (81%) were at term and 21 (19%) were premature babies.
Table 2 displays the initial clinical mode of presentation of the patients, 69 pts presented with a right inguinal hernia (RIH), 47 with a left inguinal hernia (LIH), and 45 pts with bilateral inguinal hernias (BIH). Males and females were fairly distributed between the group.
Table 3 shows the associated conditions: 25% of our patients had past history of some kind of airway disease process, most commonly bronchial asthma. All cases with undescended testis were pexed concomitantly. None of the umbilical hernias underwent simultaneous repair. A group of 25 patients (16%), suffered an episode of incarceration preop, all were successfully reduced manually and repaired promptly, their mean age was 4.2 mo. No patient suffered from strangulation or testicular edema. At that time 102 (63%) procedures were done as one day surgery, and 59 (37%) as outpatient and the mean operating time was 20+/-8 minutes.
Operative findings during surgery were recorded as a hernia sac (HS), a patent processus vaginalis (PPV), or an obliterated processus vaginalis (OPV). In that group of patients with an initial diagnosis of BIH we found 85 (95%) hernias, 4 (4%) PPV and 1 (1%) OPV, they will not be considered further. Those patients with a unilateral (RIH or LIH) hernia are shown inTable 4. A positive finding (either a hernial sac or a patent processus vaginalis) was identified in 74% RIH and 72% LIH patients when the contralateral side was explored. All hernias were of the indirect type.
The postop complications are listed inTable 5, the most common were two patients with residual scrotal hydrocele that resolved spontaneously six months after surgery. A premature infant with a postconceptual age of 46 weeks had an episode of apnea in the immediate postop period, requiring mechanical ventilation for one day. Another patient needed inhalation therapy for a postintubation croup condition. We did not find testicular damage or hernia recurrence after a mean follow-up of six years, neither a wound infection was recorded. There was no mortality reported in the present study.
When the contralateral findings during surgery were compared and analyzed within the three subgroups of patients (sex, gestational age and age at operation), we found that females had a higher probability of having positive findings than males, as seen inTable 6. No difference was obtained whether the patients had history of prematurity or not. Those infants younger than two months also had the highest probability of having positive findings. We also obtained statistic significance in patients above the two years old, probably the result of the higher frequency of females over males in these subgroups of patients.
If routine contralateral exploration of the unilateral pediatric hernia is to prevail is because it has a high yield of positive findings (HS and PPV), a low complications rate and can be expeditiously accomplished (4).
From this study, there is a high percentage of positive contralateral operative findings (72% in our series), and a very low incidence of significant morbidity following contralateral repair. Our data favors a strong reasoning to justify routine contralateral exploration of infants and children with unilateral hernia by pediatric surgeons. We agree with McGregor et al (5), that we live in a litigious society and gonadal morbidity whether related to the hernia operation or not can eventually result in litigation. There are still a large proportion of infant and child hernia operations that are not performed by pediatric surgeon and perhaps we should highlight that education, confidence, and informed consent is the hallmark of our recommendations. It should be noted that the bilateral procedures took a mean time of 20 minutes. Time should not be spent in tedious dissections of the cord structures so as to increase the yield of PPV identified, this could certainly be a factor in the past experience of other surgeon with regards to gonadal or vas deferens trauma. Nowadays most cases are done as outpatients procedures.
Previous reports have shown a higher incidence of positive contralateral findings in young infants, females, prematures, and when the presenting hernia is on the left side (6,7,8). Our data confirms that young infants and females do have a higher yield of positive findings (92% and 94% respectively). We could not demonstrate that prematurity or left-sided hernias were associated with a higher positive rate (64% and 68% respectively) as substantiated by our statistical analysis. The older children group (2-5 y/o and >6 y/o) with a higher positive yield could be biased since females were represented most commonly. As other authors have stated, the major benefit of contralateral exploration of the pediatric hernia is that it allows discovery and elimination of a patent processus vaginalis so that an indirect inguinal hernia cannot develop (2).
We conclude by establishing some criteria to justify routine contralateral exploration of the pediatric hernia: the surgeon should be experienced in child surgical care, associated conditions should not increase the surgical risks significantly, time-consuming dissections of the cord structures should be discouraged and the operating time should be kept to a minimum.
A special thanks to Professor Iris Parrilla of the Family Medicine Department, Universidad Central del Caribe, School of Medicine for her assistant in the statistical analysis of the data.
1. Sparkman RS: Bilateral exploration of inguinal hernia in juvenile patients, ,Surgery 51; 393, 1962
2. Rowe MI, Lloyd DA: Inguinal Hernia, In Welch KG, Randolph JG, Ravitch MM, O'neill JA, Rowe MI, (eds): Pediatric Surgery. Chicago: Yearbook, l986, pp779-793
3. Rowe MI, Marchildson MB: Inguinal hernia and hydrocele in infants and children. Surg Clinic North Amer 61: 1137, l981
4. Menton JP, Clatworthy HW: Incidence of patency of the processus vaginalis. Ohio State Med J 53:530-532, l961
5. Mc Gregor DB, Halverson K, Mc Vay CB: The unilateral pediatric inguinal hernia: Should the contralateral side be explored? J Ped Surg 15(3):313-317, l980
6. Gilbert M, Clatworthy HW: Bilateral operations for inguinal hernias and hydroceles in infancy and childhood. Am J Surg 97:255, 1959
7. Bock JE, Sobye JW: Frequency of contralateral inguinal hernia in children.Acta Chir Scand 136:707, l970
8. Recorta FJ, Grosfeld JL: Inguinal hernia repair in the perinatal period and early infancy: Clinical considerations J Ped Surg 19(6):832-837, l984
Published in Boletín Asociación Médica de Puerto Rico. Vol 87(1):8-11, 1995
TRICHOBEZOAR IN A 11-YEAR OLD GIRL:
A Case Report
Carlos A. Santiago-Sánchez, MD*
Priscila Garau-Díaz, MD**
Humberto L. Lugo-Vicente, MD, FACS, FAAP***
Bezoars are masses of solidified organic or non-biological material commonly found in the stomach and small bowel. They have been known for centuries and nowadays continues to be a challenging therapeutic dilemma for surgeons and gastroenterologists alike.
The identification, therapy, and long-term management of patients with bezoars depend on accurate classification and knowledge on the pathophysiology of formation. Four types of bezoars have been described based on their composition: phytobezoars, trichobezoars, lactobezoars, and miscellaneous (1).
We describe a case of a gastric trichobezoar in a pediatric patient managed successfully with surgery.
An 11-year old white girl, 6th grade student, was admitted on September 4, 1994 to the University Pediatric Hospital complaining of a sensation of fullness at the epigastrium, vague feeling of epigastric distress, nausea and anorexia. One day before admission a plain abdominal film done at the Local Health Center showed a large radiopaque image filling the stomach and suggesting an intra-abdominal tumor. The patient was transferred to our supra-tertiary institution for further evaluation and management. Computerized Abdominal Tomography using oral and intravenous contrast material showed a large gastric bezoar (seeFigure 1). Further questioning of the child revealed epigastric complains for months and she confirmed "eating hair when nervous". The family and social history uncovered that her mother was a psychiatry patient and the father an alcoholic with frequent domestic fights, claiming the child responsible for the household crisis. Furthermore the mother menaced the child by telling her "she was going to kill her". Psychiatry evaluation revealed a depressed, frightened, neglected child that relieved her anxiety by eating her hair (trichophagia).
Physical examination revealed a skinny girl with pale conjunctiva. A large, firm, oval shaped, non-tender and mobile mass was palpable at the left upper quadrant of the abdomen. The mass extended from the distal margin of the left rib cage to approximately 2 cm above the navel. On the right side the mass was palpable beyond the midline to the right nipple line. There was no guarding, rigidity or tenderness. No alopecia was noted in the child. The rest of the physical examination was essentially negative.
Laboratory work-up upon admission exhibited a mild hypochromic microcytic anemia (hemoglobin 11.9 gm/dl, and hematocrit 35.6%). Normal coagulation profile, urinalysis, electrolytes, amylase, lipase, and liver function tests. A plain chest film was normal.
The upper gastrointestinal series displayed a large intraluminal space occupying mass lesion with a honeycomb appearance that filled the stomach contour with extension into the proximal duodenum (seeFigure 2).
Upper endoscopy showed a normal esophageal mucosa. The stomach contained a very large, black, hairy ball extending through the pylorus. The gastric mucosa appeared normal without evidence of ulceration. A significant foul, nauseating smell was noted. Biopsy confirmed the hair-nature of the bezoar.
Although fragmentation with Extracorporeal Shock Wave Lithotripsy was considered, the huge size of the bezoar along with the proximal extension to the duodenum contraindicated its use and no further attempt was done. The child was taken to the operating room and the bezoar removed without difficulty using an anterior longitudinal gastrotomy incision. The mass had the shape of the stomach and proximal part of the duodenum, a brilliant surface and a putrefactive odor (seeFigure 3). The gastric mucosa was normal and not adhered to the mass.
Oral feedings were resume on the 6th postoperative day. The child discharged home after adequate psychiatry assessment and therapy.
The word 'bezoar', comes either from the Arabic word "bedzehr", or the Persian word "padzhar", meaning protecting against a poison or an antidote (2,3). In ancient times the solid mass occasionally found in the stomach of a goat or an antelope was thought to have magical healing powers and even rejuvenating properties (4). Medicinal qualities and omens of good luck were also attributed to bezoars (2). In modern medicine, however, the concretion found in the stomach and intestine of humans and referred by the term bezoar is known to be associated not with such positive effects, but with significant morbidity and even mortality (5).
In children four types have been described based on their composition:
1- phytobezoars composed mainly of vegetable or fruit fiber,
2- trichobezoars, comprise mainly of hair,
3- lactobezoars made of milk curd, and
4- miscellaneous (medicational or food bolus) bezoars (5,6).
Phytobezoars are the most common type of bezoars. They consist of vegetable material and indigestible cellulose fiber (7). Persimmons seed and other fruit products are frequent reported factors in their formation. Most develop in adults patients with impaired digestion and previous gastric surgery causing dysmotility disorders such as post-gastrectomy cases for peptic ulcer disease. Ailments other than gastric surgery that has been noted to cause impaired gastric emptying includes: diabetic gastroparesis, myotonic dystrophy, and autovagotomy secondary to tumor invasion (8). When associated with gastric surgery the stomach exhibits a diminished ability to digest, produce acid, pepsin activity, and mechanically reduce food (9).
The classically described bezoar, usually involving psychologically disturbed individuals is the trichobezoar or "hair-ball" bezoar. The trichobezoar is a concretion of hair found in the alimentary tract of animals, especially ruminants, and occasionally in man. Over the centuries these bezoars have been associated with children and emotionally disturbed adult females who ingest hair (trichophagia), carpet, rope, string, etc. The classic pediatric case is that of a partially bald child with a mass in the stomach (3). Hair strand become retained and attached in the folds of the gastric mucosa because the friction surface is insufficient for propulsion by peristalsis (10).
Trichobezoar are seen almost exclusively in female children, 6-10 years old, with bizarre appetite (trichophagia) and emotional disturbances (1). They may produce multiple clinical manifestations such as: large firm movable epigastric mass, fullness, bloating, regurgitation, nausea, vomiting, epigastric pain, hematemesis, and tiredness (2). Originally the mass develops in the stomach and can move to the small bowel by fragmentation of a portion, extension or total translocation (3). Many patients complain of early satiety, and weight loss. Other children will reduce intake and develop failure to thrive. If untreated, chronic obstruction may result in death from malnutrition or other complication such as ulceration, hemorrhage or perforation. Symptoms are intermittent and absent for many years. Rapunzel syndrome is ascribed to those gastric bezoars that have a tail-like extension of twisted hair reaching the ileocecal valve (2).
Lactobezoars have been noted during the last two decades, corresponding to the period of improved neonatal salvage. These bezoars are described in low birth weight neonates fed a highly concentrated formula. Milk products like casein congeal forming the lactobezoar (11).
There is a miscellaneous group of bezoars consisting of medications glues, antiacids, and food bolus. Food bolus that are incompletely chewed contain nuts and fiber or are trapped in narrow gastric segments (12).
Bezoars are diagnosed in most cases by conventional radiological examination, i.e. plain abdominal films, upper gastrointestinal series, ultrasonography, or computerized abdominal tomography (13). When an upper gastrointestinal series is performed with the use of barium, an intragastric mass with a honey-comb like surface around which the contrast medium flows may readily be observed, as seen in our experience. Gastric endoscopy is one of the most sensitive means to diagnosed bezoars, will confirm the diagnosis and determine their nature. Also, is utilized to obtain biopsy specimen to confirm their composition (2,14).
Bezoars can be managed by various means, depending on their underlying nature and location. Prior to 1959 the prevailing therapy for gastric or intestinal bezoars was surgical excision. This carried a high morbidity and mortality. Emergency laparotomy may still be necessary if the bezoar is associated with acute intestinal obstruction. Currently, non-surgical techniques of management of gastric bezoars may include: dissolution, suction, lavage, mechanical endoscopic fragmentation using pulsating jet of water, and fragmentation with extracorporeal shock wave lithotripsy (ESWL) (15,16,17). With ESWL the shock wave needed is half than required by urolithiasis cases (17). Intragastric administration of enzymes (papase, pancrelipase, and cellulase) or drugs (metoclopramide, tagamet, bicarbonate, acetylcysteine) has also been reported in the literature (18,19). If those methods fail, gastrotomy and manual removal is the only means of reliving the patient. Large bezoars will generally need surgery for removal (20).
Besides dissolution or removal, treatment should focus on prevention of recurrence, since elimination of the mass will not alter the conditions contributing to bezoar formation. Psychiatry follow-up may be necessary to reduce the frequency of recurrence.
In summary, the accepted therapies for patients with gastric bezoars include:1- observation, 2- medical dissolution, 3- fragmentation, and 4- laparotomy with gastrostomy. The treatment modality will depend on the type of bezoar involved. Treatment should not only focus on resolution of the established mass, but also prevention of recurrence, since the underlying condition contributing to bezoar formation will not be altered by elimination of the mass.
1-Debakey M, Ochsner A: Bezoars and Concretions- A Comprehensive Review of the Literature with an Analysis of 303 Collected Cases and a Presentation of 8 additional cases. Surgery 4:934-963, 1938; 5:132-160, 1939
2-Deslypere JP, Praet M, Verdonk G: An Unusual Case of the Trichobezoar. The Rapunzel Syndrome. Am J Gastroenterol 77(7):467-470, 1982
3-Sharma V, Sharma ID: Intestinal Trichobezoar with Perforation in a Child. J Pediatr Surg 27(4):518-519, 1992
4-Budd DC, Mc Crany ML: Gastric Phytobezoar: Still postgastrectomy syndrome Ann Surg 44(2):104-107, 1978
5-Andrus C, Ponsky J: Bezoars: Classification, Pathophysiology, and Treatment. Am J Gastroenterol 83:476-478, 1988
6-Rao PLNG, Mitra SK, Pathak IC: Trichobezoars in Children. Int Surg 66:63-65, 1981
7-Stanten A, Peters HE: Enzymatic Dissolution of Phytobezoars. Am J Surg 130:259-261, 1975
8-Goldstein SS, Lewis TH, Roth Stein R: Intestinal Obstruction Due to Bezoars Am J Gastroenterol 79:313-318, 1984
9-Kirks DR, Szemes GC: Autovagotomy and Gastric Bezoar. Gastroenterol 61:96-97, 1971
10-Reisfeld R, Dammert W, Simpson JS: Trichobezoar: An Uncommon Pediatric Problem. Can J Surg 21(3):251-252, 1978
11-Usmani SS, Levenbrown J: Lactobezoar in a full-term breast-fed infant Am J Gatroenter 84(6):647-649, 1989
12-Henke TA, Stillworthy RE Bin Stadt DU et al: Medication Bezoar in 2 Neonates. Am J Diseas Child 136:72-73, 1982
13-Newman B, Girdany BR: Gastric trichobezoars-Sonographic and Computed Tomographic Appearance Ped Radiol 20(7):526-527, 1990
14-Dietrich ND, Gau FE: Postgastrectomy Bezoars; endoscopic Diagnosis and Treatment. Ann Surg 170:432-435, 1985
15-McKechnie JC: Gastroscopic Removal of a Phytobezoar. Gatroenterol 62(5):1047-1050, 1972
16-Lange V: Gastric Phytobezoar: An Endoscopic Technique for Removal. Endoscopy 18:195-196, 1986
17-Benes T, Chmel J, Jodl J, et al: Treatment of a Gastric Bezoar by Extracorporeal Shock Wave Lithotripsy. Endoscopy 23:346-348, 1991
18-Pollard HB, Block GE: Rapid Dissolution of Phytobezoar by Cellulase Enzyme. Am J Surgery 116:933-935, 1968
19-Winkler WP, Saleh J: Metoclopramide in the Treatment of Gastric Bezoar. Am J Gatroenterol 78(7):403-405, 1983
20-Robles R, Parrilla P, Escamilla C, et al: Gastrointestinal Bezoars. British J Surgery 81:1000-1001, 1994
* General Surgery Resident, Department of Surgery, University of Puerto Rico, School of Medicine.
** Assistant Professor of Pediatrics, Section of Pediatric Gatroenterology, University Pediatric Hospital, University of Puerto Rico, School of Medicine.
*** Associate Professor of Pediatric Surgery , Section of Pediatric Surgery, University Pediatric Hospital, and Department of Surgery, University of Puerto Rico, School of Medicine.
IMPACT OF MINIMALLY INVASIVE SURGERY
IN CHILDREN
Humberto L. Lugo-Vicente, MD, FACS, FAAP*
An important medical technological progress of this century corresponds to the application of minimal invasive surgical techniques in adults and children. Laparoscopic surgery is causing an impact in the results of many procedures done during the pediatric age.
Within this review we explore the development of laparoscopic abdominal surgery in children along with basic physiology and complications of establishing a potential working space (pneumoperitoneum). Indications, results, and where we are headed in the management of various of the most common surgical conditions of children are issues discussed.
Laparoscopic surgery has proven safe, efficient, technically feasible and well tolerated in most children. Produces early return to activities, reduced hospital stay, less hospital bills, and better cosmetic results when compared to open (conventional) procedures.
For almost 150 year's physician has struggle to develop techniques of minimal invasive surgery. Unfortunately, the medium, optics and instrumentation of earlier times were archaic.
Development of the fiber optic transmission of light in 1928, the rod-shaped lens of Hopkins in the early 60's and video improvement during the late 70's renew interest in accessing the body cavities by minimally invasive technique using the laparoscope. Our fellow physicians, the gynecologists dominated this field for ten years (1).
The revolution occurred in France in 1987, this time Province of Lyon, when the gallbladder of a lady is removed successfully using laparoscopic technique. Since then, the rest has been evolution (2).
PEDIATRIC LAPAROSCOPY
Pediatric laparoscopy grew slowly and lag behind. The reason is that children usually do well and procedures are of short duration. The optics is of paramount importance when the abdominal cavity is small, and instrumentation should be tailored to body size. We wanted to see how general surgeons did before applying this technique in children. Credentialing became very tedious and time consuming if we consider that two cholecystectomies are done in children for every 100 performed by general surgeons in adults (3). Other Pediatric surgeons thought of this as a Nintendo game or making a ship in a bottle.
The concept behind minimally invasive surgery is that the size of the wound has a direct correlation with the metabolic and endocrine response to surgical trauma. The greater the cutting of fascia, muscle and nerve the higher the catecholamine and catabolic response of the body to surgical trauma.
A potential working space during video-laparoscopic abdominal procedures in children is established with the help of a carbon dioxide pneumoperitoneum. The most popular technique used in children for developing a pneumoperitoneum is the open (Hasson) technique, usually in children less than two years of age (4). Closed or percutaneous (Veress needle) technique is mostly practice in older children and adolescents (5, 6). Insufflation by either technique will cause an increase in intrabdominal pressure (IAP). Studies during congenital abdominal wall defects closure such as gastroschisis and omphalocele has shown that the rise in IAP may cause decrease venous return, decrease renal perfusion, low splanchnic flow, and increased airway pressures (7). In addition, abdominal distension causes pulmonary function abnormalities such as decreased functional residual capacity, basilar alveolar collapse, and intrapulmonary shunting of deoxygenated blood. The cardiac afterload will increase, an effect that may be magnified by hypovolemia.
Hypotension during the establishment of the pneumoperitoneum is a very feared complication. It could be the result of vascular injury, arrhythmia, insufflating too much carbon dioxide, impending heart failure, gas embolism or the development of a pneumothorax (8, 9). We generally insufflate a three-kilogram baby with ten millimeters of mercury of intra-abdominal pressure and a 70-kilogram child with a maximum of fifteen mm of Hg as can be appreciated inGraph 1.
Increase awareness of the intrinsic effects carbon dioxide insufflation may cause in the child abdominal cavity is necessary. Carbon dioxide is absorbed by the diaphragmatic surfaces and cause hypercapnia, respiratory acidosis, and pooling of blood in vessels with decrease cardiac output. This effect is usually controlled by the anesthesiologist increasing minute ventilation by 10% to 20% to maintain normocapnia. Increase dead space or decrease functional residual capacity caused by the Tredelenberg position and administration of volatile anesthetic agents can increment this problem. High risk children where this effect can be potentiate further are those with pre-existent cardio-respiratory conditions causing increase dead space, decrease pulmonary compliance and increase pulmonary artery pressure and resistance. It is estimated that carbon dioxide accumulates primarily in blood and alveoli due to the decrease muscular components to buffer the excess absorbed gas present in children (10). After the procedure, the combination of residual carbon dioxide in the diaphragmatic surface and water forms carbonic acid that upon absorbtion by the lymphatics produces referred shoulder pain. There is always a small risk of ventricular dysrhythmia with insufflation of carbon dioxide in children (3, 11, 12).
Some contraindications for performing laparoscopy during the pediatric age are: history of severe cardio-pulmonary conditions, uncorrectable coagulopathy, prematurity, distended abdomen with air or ascites, and multiple abdominal scars from previous operative procedures (12).
We have already gone through Four Congress of Endosurgery in Children, and what has been the impact? The indications from either diagnostic or therapeutic laparoscopy has grown fairly as can be gathered from Table 1.
I have managed to gather the results of some of the most common laparoscopic procedures done in children and will discuss them. These are: cholecystectomy, appendectomy, groin laparoscopy, in pursuit of the non-palpable undescended testis, splenectomy, and fundoplication.
Laparoscopic Cholecystectomy (LC) has become the procedure of choice for the removal of the disease gallbladder of children. The benefit of this procedure is obvious: safe, effective, and well tolerated. It produces a short hospital stay, early return to activity and reduced hospital bills (3). Several technical differences between the pediatric and adult patient are: lower intrabdominal insufflation pressure, smaller trocar size and more lateral position of placement. Complications are related to the initial trocar entrance as vascular and bowel injury, and those related to the procedure itself, i.e., bile duct injury or leak. Three 5 mm ports and one 10-mm umbilical port are used. Pneumoperitoneum is obtained with Veress needle insufflation or using direct insertion of blunt trocar and cannula. Cholangiography before any dissection of the triangle of Calot using a Kumar clamp is advised by some workers to avoid iatrogenic common bile duct (CBD) injuries during dissection due to anomalous anatomy, and the best method to detect CBD stones (13).
Treatment of CBD stones may consist of:
1- endoscopic sphincterotomy followed by LC,
2- open (conventional) or laparoscopic choledochotomy, or
3- transcystic choledochoscopy and stone extraction.
Children with hemolytic disorders, i.e., Sickle cell disease, have a high incidence of cholelithiasis and benefit from LC with a shorter length of postop stay and reduced morbidity (3).
From April 1992 to 1995 Avilés, Mas & Lugo managed to do 40 cholecystectomies at the University Pediatric Hospital. Twenty-four were done laparoscopically with one conversion and 16 open as can be seen in Table 2 (14).
San Pablo Medical Center performed 4439 cholecystectomies from January 1990 to July 1995; 83 (1.8%) of them in children (Table 3).
Both series stress the issue that LC is superior to the open conventional procedure reducing the operating time, length of stay, diet resumption, and use of pain medication. The child is more pleased with his cosmetic results and activities are more promptly established. We also found that CBD stones can be managed safely with simultaneous endoscopic papillotomy and costs of LC are further reduced employing re-usable equipment and selective cholangiographic indications (3).
Laparoscopic Appendectomy
Semm, a gynecologist, is credited with inventing laparoscopic appendectomy in 1982. With the arrival of video-endoscopic procedures the role of laparoscopic appendectomy in the management of acute appendicitis in children has been studied and compared with the conventional open appendectomy. General advantages of laparoscopic appendectomy identified are: ease and rapid localization of the appendix, ability to explore and lavage the entire abdominal cavity, decrease incidence of wound infection, less cutaneous scarring, more pleasing cosmetically, and a rapid return of intestinal function and full activity. There is certainly some advantage in doing laparoscopic appendectomy in the obese child, teenage female with unclear etiology of symptoms, for athletes, children with chronic right lower quadrant abdominal pain, and cases requiring interval appendectomy (15). Disadvantages are: expensive instrumentation, time-consuming and tedious credentialing, and the major benefit is in the postop period.
Analyzing the results of several series that compare laparoscopic vs. conventional appendectomy in the management of acute appendicitis we can conclude that laparoscopy produces no difference with open appendectomy in respect to operating room complications and postoperative morbidity, has a longer operating and anesthesia time, higher hospital costs, a shorter length of stay, less postop pain, less pain medication requirement, and shorter convalescence. One series warned that complicated cases of appendicitis done by laparoscopy could increase the postoperative infectious rate requiring readmission. Otherwise, they all favored laparoscopic appendectomy in the management of appendicitis (15-19).
Still, unresolved issues in my mind are: Does laparoscopic appendectomy reduce postoperative adhesions? , Is it necessary to remove a normal looking appendix during a negative diagnostic laparoscopy performed for acute abdominal pain? , Will the increase intrabdominal pressure alter the diaphragmatic lymphatic translocation of bacteria favoring higher septic rates in complicated cases? Experimental evidence in animal models favors higher rates of systemic sepsis after sequential development of pneumoperitoneum (20).
Groin Laparoscopy
The issue of contralateral exploration in the pediatric inguinal hernia patient has been hotly debated. Proponents of routine contralateral exploration cite the high percentage of contralateral hernia a/o potential hernia found at exploration, the avoidance of the cost of another hospitalization, psychological trauma and anxiety to the child and parents over a second operation, and the added risk of anesthesia of a second procedure. Most pediatrics surgeons habitually explore the contralateral side. They disagree in opinions about exploration depending upon the primary site of inguinal hernia, age, sex and the use of herniography or some intra-operative technique to check the contralateral side (21).
Recently the use of groin laparoscopy permits visualization of the contralateral side. The technique consists of opening the hernial sac, introducing a 5.5-mm reusable port, establishing a pneumoperitoneum, and viewing with an angle laparoscope the contralateral internal inguinal ring to decide the existence of a hernia, which is repaired if present. Requires no additional incision, avoids risk of vas deferens injury in boys, is rapid, safe and reliable for evaluating the opposite groin in the pediatric patient with unilateralinguinal hernia. Children less than two years of age have a higher yield of positive contralateral findings (12,22,23).
Diagnostic Laparoscopy for the Non-palpable Undescended Testis
The undescended testis identified in 0.28% of males can be palpable (80%) or non-palpable (20%). It is difficult to determine either location or absence of the non-palpable undescended testis by clinical examination. Imaging studies (Ultrasound, CT Scan, Magnetic resonance, gonadal venography) are not reliable in proving its absence. Diagnostic laparoscopy is reliable in finding the non-palpable undescended testis or proving its absence. Furthermore it can be combine to provide surgical management. After reviewing several series (12, 24-36), with non-palpable undescended testes managed by laparoscopy the following three findings were identified:
1- The testis is present; in either an intra-abdominal (38%) or inguinal position (12%). Intrabdominal testes can be managed by first stage laparoscopic internal spermatic vessel clipping and cutting (Stephen-Fowler's), followed by second stage vas-based standard orchiopexy six to nine months later. Inguinal testes are managed by standard inguinal orchiopexy.
2- The testis is absent (vanishing testicular syndrome) as proven by blind ending vas and testicular vessels (36%). These children are spare an exploration. If the vas and vessels exit the internal ring, inguinal exploration is indicated to remove any testicular remnant as histologic evidence, although I have found useful removing the testicular remnant by the laparoscopic approach. The presence of a patent processus vaginalis may suggest a distal viable testis.
3- The testis is hypoplastic, atretic, or atrophic (26%), in which case is removed laparoscopically.
Exact anatomical localization of the testis by laparoscopy simplifies accurate planning of operative repair; therefore, is an effective and safe adjunct in the management of the cryptorchid testis.
Laparoscopic Splenectomy
Laparoscopic splenectomy is another safe and technically feasible video-endoscopic procedures in children. Indications are usually hematological disorders such as Idiopathic thrombocytopenic purpura, spherocytosis, and Hodgkin's staging. Technical considerations of the procedure are based on anatomical facts such as the variability in the splenic blood supply, the ligaments anchoring the organ and the size of the diseased spleen. Generally the avascular splenophrenic and colic ligaments are cauterized, the short gastric and hilar vessels are individually ligated with metallic clips or gastrointestinal staplers, and the spleen is placed in a plastic bag, fracture or morzelized until it is removed through the navel.
Comparing the laparoscopic procedure with the conventional splenectomy, the advantages are: improved exposure, decreased pain, improved pulmonary function, shortened hospitalization, more rapid return to normal activities and excellent cosmetic appearance. Disadvantages are longer operating time, higher costs and the need to open 5-20% of cases due to technical uncontrolled hemorrhage, such as bleeding from the splenic artery (37, 38).
Laparoscopic Fundoplication
Fundoplication for the management of symptomatic gastroesophageal reflux (GER) is another procedure that has evolved recently taking advantage of minimally invasive technique. Indications for performing either the open or laparoscopic fundoplication is the same, namely: life threatening GER (asthma, cyanotic spells), chronic aspiration syndromes, chronic vomiting with failure to thrive, and reflux induced esophageal stricture. Studies comparing the open versus the laparoscopic technique in the pediatric age have found a reduced mean hospital and postoperative stay with laparoscopy.
The lap procedure seems similar to the open regarding efficacy and complication rates. Costs are not excessive, they are even lower if we take into consideration the shorter length of stay. Lower rate of adhesions, pulmonary and wound complications are another benefit of the lap technique suggested. Percutaneous laparoscopic gastrostomy can be done concomitantly for those neurologically impeded children refer with feeding problems and GER (39-43).
Whether to do a complete (Nissen) or partial (Toupee, Thal, or Boix-Ochoa) wrap relies on the experience of the surgeon with the open procedure. He should continue to do whatever procedure he used to perform using open surgery. Long-terms results of complications or recurrence of GER after laparoscopic fundoplication are still pending publication.
Video-Laparoscopic procedures are safe and efficient, technically feasible and well tolerated by children. Opening a child is not a complication. The future of pediatric laparoscopy may involve the use of intrauterine therapeutic fetoscopy.
1- Marlow J: History of Laparoscopy, Optics, Fiberoptics, and Instrumentation. Clin Obstet Gynecol 19: 261-275, 1976
2- Ko ST, Airan MC: Review of 300 consecutive laparoscopic cholecystectomies: development, evolution, and results. Surg Endosc 5: 103-108, 1991
3- Lugo-Vicente HL: Trends in Management of Gallbladder Disorders in Children (in-press).
4- Hasson HM: Open Laparoscopy: A report of 150 cases. J Reprod Med 12:234-238, 1974
5- Moir CR: Diagnostic Laparoscopy and Laparoscopic Equipment. Seminars Pediatr Surg 2(3); 148-158, 1993
6- Lobe TE: Basic Laparoscopy. In Lobe and Schropp 'Pediatric Laparoscopy and Thoracoscopy' WB Saunders ed, 1994, pags 81-93
7- Lacey SR, Bruce J, Brooks SP, et al: The Relative Merits of Various Methods of Indirect Measurement of Intraabdominal Pressure as a Guide to Closure of Abdominal Wall Defects. J Pediatr Surg 22(12): 1207-1211, 1987
8- Versichelen l, Serreyn R, Rolly G, et al: Physiopathologic changes during anesthesia administration for gynecologic laparoscopy. J Reprod Med 29: 697-700, 1984
9- Hodgson C, McClelland RMA, Newton JR: Some effects of the peritoneal insufflation of carbon dioxide at laparoscopy. Anaesthesia 25: 382-389, 1970
10- Liem T, Applebaum H, Herzberger B: Hemodynamic and Ventilatory Effects of Abdominal CO2 Insufflation at Various Pressures in the Young Swine. J Pediatr Surg 29(8): 966-969, 1994
11- Tobias JD: Anesthetic Considerations for Endoscopic Procedures in Children. Semm Pediatr Surg 2(3): 190-194, 1993
12- Holcomb III GW: Laparoscopic Procedure in Children: technical aspects. Gaslini 27: 47-60, 1995
13- Holzman MD, Sharp K, Holcomb GW, et al: An alternative technique for laparoscopic cholangiography. Surg Endosc 8: 927-930, 1994
14- Unpublished results.
15- Holcomb III GW: Laparoscopic Appendectomy in Children. Laparoscopic Surgery 1(3): 145-153, 1993
16- Reiertsen O, Trondsen E, Bakka A. et al: Prospective nonrandomized study of conventional versus laparoscopic appendectomy. World J Surg 1994 May-Jun;18(3):411-5; discussion 415-6
17- el Ghoneimi A, Valla JS, Limonne B, et al: Laparoscopic appendectomy in children: report of 1,379 cases. J Pediatr Surg 1994 Jun;29(6):786-9
18- Naffis D: Laparoscopic appendectomy in children. Semin Pediatr Surg 1993 Aug;2(3):174-7
19- Frazee RC, Roberts JW, Symmonds RE, et al: A prospective randomized trial comparing open versus laparoscopic appendectomy. Ann Surg 1994 Jun;219(6):725-8; discussion 728-31
20- Bloechle C. Emmermann A, Treutl, et al: Effect of peritonitis induced by gastric ulcer perforation in the rat. Surg Endosc 9(8): 898-901, 1995
21- Lugo-Vicente HL: The Pediatric Inguinal Hernia: Is Contralateral Exploration Justified? Boletin Asoc Med PR 87(1):8-11, 1995
22- Chu C, Chou C, Hsu T, et al: Intraoperative laparoscopy in unilateral hernia repair to detect a contralateral patent procesus vaginalis. Pediatric Surgery 8: 385-388, 1993
23- Holcomb III GW: Laparoscopic Evaluation for a Contralateral Inguinal Hernia or a Nonpalpable Testis. Pediatric Annals 22: 678-684, 1993
24- Yu TJ:Use of pediatric laparoscopy for nonpalpable testis. J Formos Med Assoc 1994 Sep;93 Suppl 2:S103-8
25- Musi L, D'Agostino S, Cimaglia ML, et al: Nonpalpable testis: current diagnostic and therapeutic trends. Pediatr Med Chir 1994 Nov-Dec;16(6):513-6
26- Milad MF, Haddad MJ, Zein TA, et al: Laparoscopy for the impalpable testes. Initial experience of one center. Int Surg 1994 Apr-Jun;79(2):163-5
27- Elder JS: Laparoscopy for impalpable testes: significance of the patent processus vaginalis. J Urol 1994 Aug;152(2 Pt 2):776-8
28- Poenaru D, Homsy YL, Peloquin F, et al: The value of laparoscopy in the diagnosis and treatment of non-palpable testicular cryptorchism. Prog Urol 1994 Apr;4(2):206-13
29- Perovic S, Janic N, Laparoscopy in the diagnosis of non-palpable testes. Br J Urol 1994 Mar;73(3):310-3
30- Froeling FM, Sorber MJ, de la Rosette JJ, et al: The nonpalpable testis and the changing role of laparoscopy. Urology 1994 Feb;43(2):222-7
31- Moore RG, Peters CA, Bauer SB, et al: Laparoscopic evaluation of the nonpalpable tests: a prospective assessment of accuracy. J Urol 1994 Mar;151(3):728-31
32- Holcomb GW 3rd, Brock JW 3rd, Neblett WW 3rd, et al: Laparoscopy for the nonpalpable testis. Am Surg 1994 Feb;60(2):143-7
33- Jones C, Kern I: Laparoscopy for the non-palpable testis: a review of twenty-eight patients (1988-90). Aust N Z J Surg 1993 Jun;63(6):451-3
34- Rappe BJ, Zandberg AR, De Vries JD, et al: The value of laparoscopy in the management of the impalpable cryptorchid testis. Eur Urol 1992;21(2):164-7
35-Diamond DA, Caldamone AA:The value of laparoscopy for 106 impalpable testes relative to clinical presentation. J Urol 1992 Aug;148(2 Pt 2):632-4
36- Heiss KF, Shandling B: Laparoscopy for the impalpable testes: experience with 53 testes. J Pediatr Surg 1992 Feb;27(2):175-8; discussion 179
37- Janu PG, Rogers DA, Lobe TE: A Comparison of Laparoscopic and Traditional Open Splenectomy in Childhood. J Pediatr Surg 31(1): 109-114, 1996
38- Gigot JF, de Goyet JDV, Van Beers BE, et al: Laparoscopic splenectomy in adults and children: Experience with 31 patients. Surgery 119(4): 384-389, 1996
39- Lobe TE, Schropp KP, Lunsford K: Laparoscopic Nissen Fundoplication in Childhood. J Pediatr Surg 28(3): 358-361, 1993
40- Collins JB, Georgeson KE, Vicente Y, et al: Comparison of Open and Laparoscopic Gastrostomy and Fundoplication in 120 Patients. J Pediatr Surg 30: 1065-1071, 1995
41- Collard JM, de Gheldere CA, Kock MD, et al: Laparoscopic Antireflux Surgery: What is real progress? Ann Surg 220(2): 146-154, 1994
42- Weerts JM, Dallemagne B, Hamoir E, et al: Laparoscopic Nissen Fundoplication: detailed analysis of 132 patients. Surg Laparosc Endosc 3(5): 359-364, 1993
43- Lobe TE: Laparoscopic Nissen fundoplication in paediatric patient. Gaslini 27(1): 73-79, 1995
* Associate Professor of Pediatric Surgery , Section of Pediatric Surgery, University Pediatric Hospital, and Department of Surgery, University of Puerto Rico, School of Medicine.
This paper was presented at the 46th Meeting of the American College of Surgeon, Puerto Rico Chapter, Hotel Marriot, Condado, Puerto Rico, February 1996.
To those great masters that taught me minimally invasive surgical techniques:
Thom Lobe, Keith Georgeson, Douglas Olsen, Zoltan Czabo, Manuel Díaz-Vargas, and Manuel Más
by : Humberto L. Lugo-Vicente, MD, FACS, FAAP *
Internet, the largest network of connected computers, is becoming the ultimate frontier to access information for health providers. This review focus on how developments of this communication technology have become a useful educational resource in Pediatric Surgery, and describes modest ideas in computer network use.
Internet basic resources are electronic mailing (E-mail), discussion groups, file transfer, and browsing the World Wide Web (WWW). E-mail brings physicians with common interest together. Surgeons employ it as a communicating tool. Legal and social responsibility is bounded with its use. Discussion groups permits debate including clinical cases, operations, techniques, research, career opportunities, and meetings. File transfer provides the opportunity of retrieving archives from public libraries. The WWW is the most resourceful tool due to its friendly interface and ease of navigation.
The average physician needs to know almost nothing on how computers work or where they came from to navigate through this pandemonium of information. Click and play with today graphical applications encourage the computer illiterate to connect. Establishing the connections envelops the need of hardware, software and a service provider.
Future development consists of online journals with new ideas in peer-review and authentication, telemedicine progression, international chatting, and centralization of pediatric surgery cyber space information into database or keyword search engines.
INDEX WORDS: pediatrics, surgery, internet
Internet is the largest network of connected computers. More than 30 million computers exchanging physical links through a standard protocol of communication. A super avenue of information and transactions (1). The Net is affecting every aspect of life and dissemination of information relevant to medicine for the health community is not immune to this technology.
The busy surgeon who invests little time searching the literature could find himself with a clinical practice that does not keep pace with recent medical advances. Informatics option to stay updated in the discipline of Pediatric Surgery includes access to printed periodical publications, regular meetings, congress assistance, digital database storage, and Internet resources.
Text, journals, and books are usually outdated by the time they reach the regular subscriber. Not to mention cost of subscription, printing and storage capabilities needed. Meeting and congress dynamic regular sessions can be costly, and access to the full written report is almost never achieved until print publication of the paper is obtained usually six months to one year later. Digital databases (i.e., CD-ROM) store large amount of information, but prices of CD are overwhelming. An additional driver is needed as hardware for reading the stored material. Information is becoming an unlimited commodity, we can have as much as we want at no cost, but are limited by our storage capacity (2).
By agreeing to a set of operating protocols, users have developed innovative techniques to seek out information from different databases accessible via the network along with methods for sharing documents. Internet provides immediate downloadable information and dynamic information on every aspect of life. Still the idea that it represents a frustrating educational event in computing persists. The average person needs to know almost nothing on how computers work or where they came from to navigate through this network. Click and play with today graphical applications encourage the computer illiterate to connect.
The purpose of this review is to highlight how newly ways of communication using Internet navigational technology can be useful educational resources in Pediatric Surgery, and clarify concepts of network communication for future use by physicians.
After the postwar years military intelligence was searching for strategic forms of communication in the after-match of a nuclear holocaust, a system that would defeat current centralized tendencies in communication. The notion of creating several nodes of super computers that convey each other through standard telephones line was developed. Sending the information in small packages that would meet at the other end of the line using a uniform protocol of communication and regrouping (TCP/IP). These nodes would be created around different parts of the world divided in either top level geographical or institutional domains like: government (gov), commercial (com), educational, (edu), military (mil), network resources (net), and other organizations (org).
Scientists were the first to use this system in an effort to consolidate research and establish electronic communication in the flow of new projects. This created an atmosphere of social behavior and effective long distance communication as more nodes grew in each country. Curiously, the initial electronic discussion group developed among scientists was called the Science-Fiction list (3, 4).
World Wide Web (WWW), the crowning glory of the Internet, is developed in Geneva, Switzerland in 1989. The WWW provides a user friendly interface with the capacity to send and receive information through Internet using text, graphics, audio and video utilizing a protocol of marked language (5). Seen today as the best resource to post information that can reach and be accessed in almost every corner of the planet.
Uses of Internet for the Pediatric Surgery community
Internet basic resources are:
electronic mailing (E-mailing),
discussion groups (news groups and list servers),
file transfer,
and the WWW.
Through the initial effort of scientists to establish communication using electronic text mailing convenience over the postal service was foreseen. E-mailing is faster than postal mail. It takes an average of two to eight minutes for messages to arrive to another computer node in very distant geographical zones. The message is stored by the internet service provider (ISP) until the electronic box owner retrieves the message. You do not have to pay extra for e-mailing, and is global in scope. Files can be attached to messages up to half a megabyte in size (a megabyte represents one million characters).
News groups and list servers with discussion interest have developed both in pediatrics and surgery. Messages posted by authors to the list or discussion group are automatically mailed to all subscribers. Posting growth to such lists includes United States, Central and South America, Europe, Middle East, Africa, and Australasia to mention a few. List servers for different surgery and pediatric sub-specialties exist: NICU-Net, PICU-Net, cardiology, gastroenterology, neurology, emergency medicine, critical care, Pediatric pain, etc. (6, 7).
A popular list among Pediatric Surgeons worldwide is called the Pediatric Surgery List. Originally developed by Thomas Whalen for topics discussion that includes clinical cases, operations, techniques, research, career opportunities, and meetings. Intended for pediatric surgeons and interested general surgeons and residents (8). Although the list is in embryological phase, growth will inevitably create a medium of international discussion without precedent. A constant forum for exchange of ideas, difficult cases, consensus on management, and development of our specialty.
File transfer provides the unique opportunity of retrieving archives from public file libraries. Free software is also available. Downloading of data into the hard disk of your computer is very straightforward. Anti-viral programs are available to monitor each access file that can become part of your system whenever you download them from Internet.
Recent poll of the Pediatric Surgery Internet list server members regarding what resource of the Net they use most of the time was done. Almost one-fourth (23%) of the list population (58/246) answered the survey. Electronic mailing (personal and list server/discussion groups) occupied 83% of resources, web browsing 16%, and long distance computing 1%. Pediatric surgeons with access to the Net use it mostly as a communication tool. WWW browsing is slowly developing as a second alternative probably due to absent access to a web browser connection.
E-mailing uses and responsibility
Electronic mailing is the most useful resource of Internet. Mailing lists bring people with common interest together (9). Through it physicians have developed news, chat, and list group discussion. This creates the perfect environment to consult colleagues on a clinical problem, send draft of a paper for peer revision, read journals without paying subscription rates, maintain your continuing medical education credits, and retrieve anything the same day that it is published (1). It will become an essential tool in medical research, teaching medical students, clinical practice, postgraduate studies, and continuing medical education. The lack of a traditional peer-review process and author identification might prevent E-mail text from being taken as authoritative (10).
The common user of the Net is a professional. Environmental motivations have created an informal code of conduct known as net-etiquette. By this is meant politeness in replying. Along with accessibility, identification and social responsibility (11).
Netters (defined as common user of the Net), resent several iatrogenic web disorders: not waste the carrying capacity of the Net (bandwidth), posting unsolicited advertising (spamming), and observing inappropriate online behavior (1). Chain E-mail letters can overcrowd your electronic site. Other problems related to the nature of e-mailing that we must be aware are: sign your posting so that we can know who is writing, do not reply publicly to the whole group when answering privately to one person, and avoid including the entire text of the original message in your reply.
A hot debate among frequent E-mail list servers involves being careful when answering or replying, specially when the answer will hit many members of a list server group. The inclusion of your name and address at the end of your E-mail text represents a legal signature for all aspect of the law: the author name type in ASCII characters (10). Simple rules to observe are: avoid using patients' names, address, record numbers or institutional demographics. When personally responding to electronic medical consultation by an unknown online patient ask yourself: Is he your patient behind the monitor? Have you examined him or review his past medical record? Will my answer be used as possible legal evidence in case this is unintended? The potential for abuse while looking at this information will always exist.
Disclaimers notice stating the medico-legal responsibility behind frequent response to complex medical problems are being asked for to list server administrators (12). This if a response from some member commentator triggers a change in diagnosis or therapy in a given discussion case that causes ultimate damage to the patient involved. The commentator cannot be held responsible of his answer in as much as he had no clear physician-patient relationship, was not paid for this service, or had the opportunity to examine the patient or his medical charts. The disclaimer should include that particular consultation or advice was not the idea of the answer, reliance on this comments should not be done, and printed versions of the E-mail should not appear in any patient medical record (12).
WWW and Pediatric Surgery
WWW can be seen as an endless book, where each page has a divergent story. The web page is the basic unit of information and hypertext provides links to other pages in the same directory, or in different domain sites of the network (5). What makes the WWW the most resourceful area of Internet is the ability to watch text, images, video, audio, and real time pictures embedded in web pages.
Each page has a unique address, also known as uniform resource locator (URL). URL essential ingredients are protocol, domain name, and directory. For example the URL of 'Pediatric Surgery Update' is: http://home.coqui.net/titolugo/index.htm. This means that the protocol is http, the domain name /home.coqui.net/, and the file "index.htm" is the web index page under "/titolugo/" directory.
'Pediatric Surgery Update', the periodical electronic newsletter started on July 1993 as a print form. Initially covered short issues and reviews in the discipline of pediatric surgery. December 1995 marked its development as a web site. The WWW introduction of the print version permitted development of further areas such as: review articles with images, graphs and tables, survey section, technical innovation area, a Pediatric Surgery Online Handbook for residents and medical students, and an area for medical students to developed research and writing skills (13).
Departments and Section of Pediatric Surgery have developed their own web page in the WWW. Through them we have access to such content as: faculty members, facilities, research programs, interests, residency and fellowship programs, and other pediatric and medically relevant links. Continuous medical education credits are part of some web site offering.
Page in the Net, HTML and graphic applications
A web page, the basic unit of the WWW consists of all one medium, such as text, or can include multiple media including graphics, sounds, animation, and video (14). The web page is built using a mark language that is plain text tagged with handles <text>. This is known as hypertext mark-up language (HTML). HTML can easily be learned for later production of a web page. The National Center of Supercomputing Application (NCSA) Beginner's Guide to HTML is used by many to start to understand the hypertext markup language used on the WWW. It is an introduction and does not pretend to offer instructions on every aspect of HTML (15). Computer applications developed as web editors provide the functionality needed to construct a web page with little knowledge of HTML. They are called WYSISYG (What You See Is What You Get) Editors.
Movement between web pages is accomplished by links to other universal resource locators or Internet address. Links can be in different color or underlined text where the cursor of your pointer device changes as though sensing an executable movement. By either clicking the device or hitting the return key, you will be moving to that link. Some links are just libraries composed of downloadable files.
Web editors can be downloaded from different suppliers in the Net. Some are free but most can be obtained as shareware to try them for a limited period. For a list of HTML editing tools or programs available the reader is referred to URL: http://sdg.ncsa.uiuc.edu/~mag/work/HTMLEditors/windowslist.html
Establish the Connection
To gain access to Internet you will need hardware, software, and a service provider.
Hardware is your computer. This includes a monitor, central processing until (CPU) and keyboard. Macintosh and Widows operating systems ease of use graphical environments have prevailed during the last years over the more text-based disk operating system (DOS). A modem is another piece of hardware needed that will provide the telephone line communication.
Computer software that help you navigate the web is known as web browser. Web browsers are in essence a navigational aid for moving around and between the various nodes and links of the WWW (14). Some web browsers are non-graphic like Lynx, and graphical like: Mosaic, Netscape, and MS Internet Explorer. Netscape is the most widely used and industry standard full-features web browser. Latest versions of this software can be downloaded free from their respective site (URL) in the web (16,17).
Internet service providers (ISP) are either private or universities based. The service provider will give you access to the Net using a local or toll-free telephone number. Some may include web space with the monthly rate offer. A university-based ISP usually provides service for a nominal or none rate. Electronic addresses of such users usually end in the suffix -edu. Most physicians with Internet access have it through academic affiliation (9)
Once connected, the Net is a pandemonium of information with no central index. The user will rely on automated index or search engines. Search engines collect database, retrieve programs, or harvest them (2). A collection of search engines can be found at URL: http://www.webcom.com/webcom/power/index.html (The WebCom Power Index). Specific search engines in the field of medicine will help create an atmosphere of librarian resource.
Editors like Spooner's Ped-Info and Lehmann's Points of Pediatric Interest, have developed web sites with collection of information specifically oriented toward pediatric content. The web site has been maintained as a set of WWW pages through which you can link to: Departments of Pediatrics, professional organizations, pediatric practices, Children Hospitals, medical and surgical subspecialties, on-line publications, and pediatric software of interest. Criteria for entry into the database are that the resources must have appeal to pediatricians, and specific pediatric content. Both web sites allow easy access to pediatric information on the WWW for health care professionals and parents (18, 19).
Future of the Net in Pediatric Surgery
The future of Internet will be an unbounded multimedia circus. Real time video and audio technology will permit us view recorded in vivo sessions made in another location paid for through a local phone call. In vivo videos of laparoscopic procedures have already taken place between two continents (United States and Argentina). With the use of a personal computer and a modem, they have transmitted the surgical procedure through live broadcast, via the Net, for the nominal price of a local phone call. Resident surgeons watched and interacted during the surgical procedure. This opens an area by which academic medicine can be telecasted to other parts of the world with least bearing on economical resources (20).
Since anyone can publish in the Net online, electronic journals will develop with new peer-review concepts. Editors, reviewers, and authors will need to adjust to the use of this information technology. Online publication in Pediatric Surgery will increase as printed form of actual journal joins the cyberspace domain. Less paper work on publishing companies may mean a reduction in subscription price, with e-mailing guarantees of providing manuscript of written and published articles.
Cyber citations as proposed by the American Psychological Association or Modern Language Association have yet to be standardized by the American Medical Association to be used as bibliographical style (21). Authors that use Online references will need to keep printed or digital file copy of such articles, since there is no way to avoid drastic changes or movement done to this domain address (22).
International chatting is another area of future development for our pediatric surgery community. Using simple downloadable application like mIRC (internet relay chat) you can connect to an undernet organization channel and chat with groups of people at the same time (23). The International Pediatric Chat channel developed by J. Edlavitch use two weekly sessions to maintain the group online (24).
Telemedicine refers to the use of telecommunication technology to simplify health care delivery or distribute medical informatics. Some specific projects represented by this technologic are: Multi campuses linking of hospitals and research centers, linkages between rural health clinics and central hospital, physician-to-hospital links for transfer of patient information, diagnostic consultations, patient scheduling, research, literature searches, video program distribution for public education on health care issues, use of video and satellite relay to train health care professionals in widely distributed or remote clinical settings, and transfer of diagnostic information such as electrocardiograms or X-rays. Some benefits are improved access to areas in needs of health service, reduce cost of traveling, reduces professional isolation, and improving the quality of care given. Development of the infrastructure needed along with cost containment issues are two of the problems faced by this technological advance (25).
Most Pediatric Surgery Organizations (Surgical section AAP, APSA, CAPS, BAPS, etc.) will find themselves generating web sites of their own during the next few years. This will add to the pandemonium of information already established. A future trend in development will be the need to gather all this information in a Pediatric Surgery Cyber Web site with database keyword access. This way a centralized path will exist to organize the varied information buried in the Net.
We must be aware of the negative effects of expansion of computerized information. The WWW can be an intoxicating and seductive place. Long hours glued to the small screen, surfing the cyberspace, and reading E-mail can cause social degradation, increasing disparity and isolation of the individual. Fragmentation of knowledge can be the result. Users must continue to maintain an equilibrium to avoid such side-effects (26,27).
The exponential growth of Internet in the disciplines of Pediatric Surgery will cause a change in patient care, teaching and research. Changes in our specialty will be nurtured through the international use of information posted in the Net. Main use by contemporary pediatric surgeons is as a communicating tool using electronic mailing with WWW browsing slowly growing.
Future developments consist of online journals with new concepts in peer-review and authentication, telemedicine, international chatting, and centralization of cyber space information into database or keyword search engines. Marketing is another frontier in the development of medical informatics technology.
1- Pallen M: Guide to the Internet: Introducing the Internet. BMJ 311: 1422-1424, 1995
2- Thomas P. Copley (September 17, 1996) Make the Link Workshop: Tutorial Number Five [Online] Available: http://www.crl.com/~gorgon/links.html [September 20, 1996]
3- Johnson Phillip (May 8, 1996) History of the Internet [Online] Available: http://dragonfire.net/~Flux/ihistory.html [September 15, 1996]
4- Sterling Bruce (July 5, 1994) Short History of the Internet [Online] Available: http://www.forthnet.gr/forthnet/isoc/short.history.of.internet [September 15, 1996]
5- Pallen M: Guide to the Internet: The World Wide Web. BMJ 311: 1552-1556, 1995
6- Christopher U. Lehmann (1996) Point of Pediatric Interest E-mail Discussion Lists [Online] Available: http://www.med.jhu.edu/peds/neonatology/elists.html#elists [September 6, 1996]
7-Tarczy-Hornoch P: NICU-Net: An Electronic Forum for Neonatology. Pediatrics 97(3): 398-399, 1996
8- PEDIATRIC SURGERY Mail List Subscription Address: Majordomo@UMDNJ.edu Subscription Message: Subscribe PEDSURG-L Human administrator: Thomas V. Whalen, Professor, Robert Wood Johnson Medical School, E-mail: whalen@umdnj.edu
9- Spooner SA: On-Line Resources for Pediatricians. Arch Pediatr Adolesc Med 149: 1160-1168, 1995
10- Elliot SJ, Elliot RG: Internet List Servers and Pediatrics: Newly Emerging Legal and Clinical Practice Issues. Pediatrics 97(3); 399-400, 1996
11- Frisse ME, Kell EA, Metcalfe ES: An Internet Primer: Resources and Responsibilities. Academic Medicine 69(1): 20-24, 1994
12- De Ville, K.A. Internet Listservers and Pediatrics: Newly Emerging Legal and Clinical Practice Issues II. Pediatrics 98: 453-454, 1996
13- Lugo-Vicente HL (September 16, 1996) Pediatric Surgery Update [Online] Available: http://home.coqui.net/titolugo/index.htm [September 18, 1996]
14- Thomas P. Copley (September 17, 1996) Make the Link Workshop: Tutorial Number Four [Online] Available: http://www.crl.com/~gorgon/links.html [September 20, 1996]
15- NCSA (September 1996) A Beginner's Guide to HTML [Online] Available: http://www.ncsa.uiuc.edu/General/Internet/WWW/HTMLPrimer.html [September 18, 1996]
16- Microsoft Corp (1996) Internet Explorer Home [Online] Available: http://www.microsoft.com/ie/ [September 21, 1996]
17- Netscape Communication Corp (1996) Download Netscape Navigator Software [Online] Available: http://home.netscape.com/comprod/mirror/client_download.html [September 21, 1996]
18- Spooner SA (August 1994) Ped Info a Pediatric Web Server [Online] Available: http://www.uab.edu/ped-info [September 16, 1996]
19- Lehmann C (1996) Points of Pediatric Interest [Online] Available: http://www.med.jhu.edu/peds/neonatology/poi.html [September 22, 1996]
20- Telbelian A (September 1996) NOMC broadcasts live surgery via the Internet [Online] Available: http://laparoscopy.com/pictures/vdconf.html [September 17, 1996]
21- Li X., Crane N. (May 20, 1996) Bibliographic Formats for Citing Electronic Information [online]. Available: http://www.uvm.edu/~xli/reference/estyles.html [September 15, 1996]
22- Aruzen MA: Cyber Citations: Documenting Internet Sources Presents Some Thorny Problems. Internet World Sept 1996, pag 72-74
23- mIRC Co. Ltd. (1996) Homepage of mIRC [Online] Available: http://www.geocities.com/SiliconValley/Park/6000/ [September 22, 1996]
24- Edlavitch Julius (September 1996) Home Page of International Pediatric Chat [Online] Available: http://www.pedschat.org/ [September 19, 1996]
25- William Frederick, Moore Mary (August 1995)Telemedicine: Its Place on the Information Highway [Online] Available: http://naftalab.bus.utexas.edu/nafta-7/telepap.html [September 7, 1996]
26- Thomas P. Copley (September 17, 1996) Make the Link Workshop: Tutorial Number Two [Online] Available: http://www.crl.com/~gorgon/links.html [September 20, 1996]
27- Gravanis MB: Computers and social isolation [Letter to the editor] The Pharos 59 (3): 50, 1996
* Associate Professor in Pediatric Surgery, University of Puerto Rico School of Medicine. Member of SCOT (Section of Computers and Other Technologies) Section of the American Academy of Pediatrics.
Address reprints request to: Humberto Lugo-Vicente, MD- P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico 00922-0426 Tel. 787 / 786-3495 Fax 787 / 720-6103 E-mail: titolugo@coqui.net
Date posted: March 1, 1997
Delayed Diagnosis and Management
of Bronchial Rupture following Blunt Thoracic Trauma
Carlos E. Prieto-Velhote, Manoel C. Prieto-Velhote, Tais F.de Oliveira-Velhote
From the Division of Pediatric Surgery, Hospital Carlos Chagas, São Paulo, Brazil.
Address reprints request to: Carlos E. Prieto-Velhote, M.D. - Rua Carlos Weber 1389 - Apto.114 - CEP 05303-000 São Paulo – Brazil. E-mail: cevelhote@mandic.com.br Fax: 55-021-11-64402706
The authors discuss delayed diagnosis of bronchial rupture following blunt thoracic trauma. They report two patients suffering lung atelectasis forty days and two years after blunt thoracic trauma respectively. Bronchoscopic examination was crucial in diagnosis. Both cases managed with bronchial anastomosis doing well eighteen months and four years after surgery.
KEY WORDS: thoracic trauma, blunt thoracic trauma, bronchial rupture, lung atelectasis, bronchial reconstruction.
Bronchial rupture following blunt thoracic trauma is becoming a frequent event (1). Although bronchial rupture accounts for almost 3% of all cases of blunt thoracic trauma (2) the true incidence of bronchial disruption is unknown since most patients die before arriving to a trauma center (3). About 3% of people dying from accidents have tracheobronchial disruption (4). Most of the bronchial lesions are the rsult of motor vehicle accidents, though other etiological agents may be incriminated (5,6). Early diagnosis is important to avoid serious complications of delayed treatment as permanent bronchial stenosis, parenchyma destruction by chronic infection and consequent pulmonic resection (7,8,9).
Bronchial rupture has two main forms of clinical presentation. During the early phase symptoms of dyspnea, cyanosis and thoracic pain are common. Upper rib fractures and pneumothorax are frequently found on chest x-ray examination in these cases. The other main form is associated with minor complaints, no signs of acute respiratory distress or pneumothorax, being frequently misdiagnosed at the emergency room. This second group of patients with delayed diagnosis includes 24% to 68% of all patients suffering traumatic rupture (4). The diagnosis of bronchial rupture is done some time later during routine radiological examination of the thorax or after developing symptoms from the associated atelectasis (4).
This paper discusses two patients managed at our hospital for bronchial rupture after delayed diagnosis.
Two patients were referred to the emergency room of the Hospital Carlos Chagas with lobar atelectasis and history of blunt thoracic trauma.
The first patient is a six-year-old boy with history of a motor vehicle accident. In the initial evaluation at the emergency room he had moderate thoracic pain and dyspnea. A left clavicle fracture and left lung contusion were diagnosed. Stayed in the hospital under medical observation for five days and was sent home with no symptoms of respiratory distress. Progressively develop exercise dyspnea and forty days later during chest x-ray examination total opacification of the left hemithorax is observed. Bronchoscopic examination confirmed the diagnosis of complete rupture of the left main bronchus.
The second patient is a five-year-old girl admitted with mild fever, cough and dyspnea. The chest x-ray examination reveals opacity of the right hemithorax (Fig. 1). History reveals she sustained thoracic trauma after a television set fell over her chest two years previously. Since then, two admissions to different hospitals with diagnosis of pneumonia were reported. A thoracic CT-Scan revealed total atelectasis of the left lung (Fig. 2). Total rupture of the left main bronchus was detected during bronchoscopic examination.
Resection of the bronchial scar with bronchial anastomosis using absorbable synthetic 5-0 sutures resulted in progressive recovery of the affected lung of both patients (Fig. 3). They are both now free of symptoms, four years and eighteen months after surgery respectively.
Although prompt diagnosis and management of bronchial rupture secondary to blunt thoracic trauma is desirable, delayed surgical reconstruction of the main bronchus may be achieved without gross compromise of lung function (10). Frequently a "silent" rupture of the main bronchus may be misdiagnosed. Those cases demonstrates mild symptoms of respiratory insufficiency after trauma, and no pneumothorax or rib fractures at the radiological examination of the chest. Days or even years may go by before the diagnosis is made. Partial or total lung atelectasis and extensive infiltrates in lung parenchyma diagnosed during routine radiological examination may be suspicious of bronchial rupture after history of trauma. Thoracic CT-Scan or Magnetic Resonance Imaging may help in diagnosis (11,12). With suspicion of bronchial rupture, bronchoscopy confirms the diagnosis (2,7,13,14). Both patients had their diagnosis confirmed after fiberoptic bronchial examination which is of paramount importance in patients suffering blunt trauma having symptoms of respiratory distress, pleural air leaks, lobar atelectasis or persistent pneumothorax (7,9).
Immediate surgical correction of bronchial rupture could reduce the incidence of late complications such as secondary tissue infection and persistent bronchial stenosis ( 9, 15). In children, atelectatic lungs heal within a few weeks after bronchial reconstruction if no secondary infection is present (15). Bronchial reconstruction must always be attempted. Lung resection is restricted to those patients with tissue necrosis secondary to infection (9). Our two patients with delayed diagnosis of bronchial rupture had no symptoms of infection. They were successfully managed with resection of the bronchial scar and anastomosis of the main bronchus with absorbable synthetic sutures. After uneventful surgical recovery they had follow-up bronchoscopic examination six moths later with total permeability of the main bronchus, a chest x-ray with good ventilation of the affected lung and minimal elevation of the diaphragmatic dome.
1.Hartley C, Morrit GN: Bronchial rupture secondary to blunt chest trauma. Thorax 48:183-184, 1993
2. Goh SH, Tan SM, Chui P, Low BY: Traumatic bronchial rupture - A case report. Ann Acad Med Singapore 24: 883-886, 1995
3. Baumgartner F, Sheppard B, de Virgilio C, Esrig B, Harrier D, Nelson RJ, Robertson, JN: Tracheal and main bronchial disruptions after blunt chest trauma: presentation and management. Ann Thor Surg 50:569-574, 1990
4. Frimpong-Boateng K, Amoah AGB: Delayed diagnosis and repair of total bronchial rupture: A report of two cases. East Afr Med J 74:114-115, 1994
5. Roux P, Fisher RM: Chest injuries in children: na analysis of 100 cases of blunt chest trauma from motor vehicles accidents. J Pediatr Surg 27:551-555, 1992
6. Lin MY, Wu MH, Chan CS, Lai WW, Chou NS, Tseng YL: Bronchial rupture by blunt chest trauma. Ann Emerg Med 25:412-415, 1995
7. Amauchi W, Birolini D, Branco PD, de Oliveira MR: Injuries to the tracheobronchial tree in closed trauma. Thorax 38: 923-928, 1983
8. Sirbu H, Herse B, Schorn B, Hüttermann U, Dlichau H: Successful surgery after complete disruption of the right bronchial system. Thorac Cardiovasc Surgeon 43: 239-141, 1995
9. Jones WS, Mavroudis C, Richardson JD, Gray LA Jr, Howe WR: Management of tracheobronchial disruption resulting from blunt trauma. Surgery 95: 319-323, 1984
10. Taskinen SO, Salo JÁ, Haltunen PE, Sovijarvi AR: Tracheobronchial rupture due to blunt chest trauma: a follow-up study. Ann Thor Surg 48: 846-849, 1989
11. Huson H, Sais CJ, Amendola MA: Diagnosis of bronchial rupture with MR imaging. J Magn Reson Imaging 3: 919-920, 1993
12. Wan YL, Tsai KT, Yeow KM, Tan CF, Wong HF: CT findings of bronchial transection. Am J Emerg Med 15:176-177, 1997
13.Parat S, Bidat E, Chevallier B, Lacombe P, Azoulay R, Legardere B, Bouledroua MS: Late diagnosis of a bronchial fracture after thoracic injury. Ann Pediatr (Paris) 36:205-207, 1989
14. Rossbach MM, Johnson SB, Gomez MA, Sako EY, Miller OL, Calhoonn JN: Management of major thacheobronchial injuries: a 28-year experience. Ann Thorac Surg 65:182-186, 1998
15. Gaebler C, Mueller M, Schramm W, Eckesberger F, Vecsei V: Tracheobronchial ruptures in children. Am J Emerg Med 14: 279-284, 1996
Figure 1 - Radiological examination of the thorax with massive atelectasis on the left lung after thoracic trauma two years before.
Figure 2 - A CT scan of the same patient showing the trachea (arrowhead) and an amputated left main bronchus (arrow).
Figure 3 - Radiological examination done two months after surgery with good ventilation of the left lung.
VENTILATORY STRATEGIES
TO PREVENT LUNG INJURY IN THE NEWBORN
Ramzi A. Kilani, MD, FAAP
Assistant Professor of Pediatrics
Division of Newborn Medicine
Ventilator-induced lung injury remains an important cause of morbidity and mortality in neonates requiring assisted mechanical ventilation. Historically, neonatal ventilation began as pressure-limited. Pressure, but not volume, was controlled. Hence, the term baro (airway pressure) trauma (lung injury). Animal studies showed, however, that changes in lung volume, and not pressure, caused lung injury [1]. This has led to a new term, volutrauma. Changes in pressure produce changes in volume as reflected by the compliance of the respiratory system.
Once the uninjured lung is recruited, it tends to stay open during exhalation. Surfactant and the structure of the lung and chest wall confer this deflation stability and define functional residual capacity (FRC). When FRC is optimized, end expiatory pressure can be decreased without a great loss of lung volume, as long as the pressure is not decreased below the critical closing pressure of the lung [2].
Therefore, ventilator induced lung injury sequence can occur at the beginning and end of each assisted breath. If inadequate numbers of alveoli were recruited in the previous breath, or if the positive end expiratory pressure was insufficient to maintain recruitment, atelectasis may occur, and can by itself propagate a lung injury sequence.
Once the airway epithelium and pulmonary capillary endothelium are injured, a lung injury sequence is initiated leading to alveolar capillary leak, surfactant disruption, inflammatory response and distal organ injury through efflux of inflammatory mediators from the alveolar space into the general circulation [3]. Oxidant injury may be another serious cause of lung injury. Immature and developing lungs are particularly susceptible to acquired injury.
An understanding of the basic pathophysiology of the underlying respiratory disorder is essential to optimize the ventilatory strategy. Aim for an adequate gas exchange without injuring the lungs.
Low tidal volume ventilation strategy
Focus ventilatory strategies for CMV in infants on prevention of overdistention, use of relatively small tidal volumes, and maintenance of adequate FRC. Studies in healthy infants report tidal volumes in the range of 5-8 cc/kg. A large multicenter trial in adults with acute RDS demonstrated that small tidal volumes (6 vs 12 mL/kg) and permissive hypercapnia significantly reduced mortality rates and ventilatory needs [4]. Insufficient data are available to recommend a specific size of tidal volume in infants with RDS who can be ventilated with even lower tidal volumes of 4-6 cc/kg. In infants with severe pulmonary disease, ventilate with small tidal volumes, because lung heterogeneity and unexpanded alveoli lead to overdistention and injury of the most compliant alveoli if a normal tidal volume is used.
Permissive hypercapnia strategy (PHS)
Permissive hypercapnia, or controlled mechanical hypoventilation, is a strategy for the treatment of patients receiving ventilatory assistance. When using this strategy, priority is given to the prevention or limitation of over ventilation rather than maintenance of normal blood gases and the high alveolar ventilation that frequently is used. Respiratory acidosis and alveolar hypoventilation may be an acceptable price for the prevention of pulmonary volutrauma. Thus, ventilatory strategies, which tolerate mild hypercapnia (PaCO2: 45-55 mm Hg) and/or prevent hypocapnia (particularly during the first days of life), may result in a reduced incidence and/or severity of lung injury [5, 6].
The limited number of randomized controlled trials that evaluated permissive hypercapnea in preterm infants have not demonstrated any overall benefit of ventilatory strategies that target high levels of PaCO2 (>55 mmHg). Further trials are needed to establish the safe, or ideal, range for PaCO2 in ventilated newborn infants [7].
Potential advantages of PHS are decreased volutrauma and lung injury, decrease duration of mechanical ventilation, reduced alveolar ventilation, reduced side effects of hypocapnea, and increased oxygen unloading. Potential disadvantages are cerebral vasodilation, hypoxemia, hyperkalemia, decreased oxygen uptake by hemoglobin, increased pulmonary vascular resistance, intraventricular hemorrhage in prematures, and retinopathy of prematurity.
Although, term and near term infants with respiratory failure and PPHN have been managed successfully with PaCO2 levels as high as 65 mm Hg, There are no published randomized controlled trials (RCTs) comparing outcomes in infants with PPHN using a strategy of permissive hypercapnea with one of hyperventilation or even one of achieving eucapnea.
Clearly, more RCTs are needed before permissive hypercapnea can be recommended routinely for preterm, near term, and term infants with respiratory disorders [8].
Strategies based on alternative modes of ventilation
Technologic advances have resulted in better ventilators. Patient-triggered ventilation and synchronized intermittent mandatory ventilation are being used increasingly in newborns. High-frequency ventilation is another mode of ventilation that may reduce lung injury and improve pulmonary outcomes, though available studies fail to demonstrate consistent benefits.
Patient-triggered ventilation
During conventional ventilation neonates are ventilated with continuous flow, pressure limited, time cycled ventilators. The introduction of triggered ventilation has been a marked improvement in neonatal ventilation. Various triggered ventilation modes have been developed for neonates: Synchronous Intermittent Mandatory Ventilation (SIMV), Assist/Control Ventilation (A/C), and Pressure Support Ventilation (PSV).
Consequences of Asynchrony
Asynchrony between spontaneous ventilation and mechanical breaths can be problematic. Asynchrony causing active expiration against ventilator inflation may occur irregularly or continuously, depending on the ventilator setting. Consequences of active expiration may be a decrease in tidal volume.
Synchronized intermittent mandatory ventilation
This mode of ventilation achieves synchrony between the patient and the ventilator breaths. Synchrony may be achieved by adapting ventilator settings to the spontaneous breathing pattern, by using triggered ventilation modes, or by using heavy sedation or paralysis. Most Neonatologists do not adapt the practice of regular paralysis in infants with active expiration, because paralysis has some disadvantages. Sedation is more often used but also shows some disadvantages.
Synchrony can sometimes be achieved by nearly matching the ventilator frequency to the spontaneous respiratory rate or by simply ventilating at relatively high rates (60-120 min). An alternative approach is to detect the infant's inspiratory effort and use it to trigger positive pressure inflation (triggered ventilation).
Patient triggered ventilation (PTV)
The most frequently used ventilators in newborns are time-triggered at a preset frequency, but because of the available bias flow, the patient also can take spontaneous breaths. In contrast, patient-triggered ventilation (PTV), which also is called assist/control, uses spontaneous respiratory effort to trigger the ventilator. During PTV, changes in airway flow or pressure, chest wall or abdominal movements, or esophageal pressure changes are used as an indicator of the onset of the inspiratory effort. Once the ventilator detects inspiratory effort, it delivers a ventilator breath at predetermined settings (PIP, inspiratory duration, flow).
Despite short-term benefit, large randomized controlled trials report that patient-triggered ventilation does not improve long-term outcomes in infants with RDS [9], although' compared to conventional mechanical ventilation (CMV) PTV has been shown to improve tidal volume, oxygenation and reduce blood pressure fluctuations. PTV compared to SIMV was associated with a trend to a shorter duration of weaning [9].
High-frequency ventilation
High-frequency oscillatory ventilation (HFOV) delivers very small tidal volumes (even smaller than dead space) generated by an oscillatory piston or diaphragm, at extremely high frequencies, superimposed on a variable mean airway pressure. Oscillatory ventilation is unique because exhalation is generated actively, as opposed to other forms of HFV (HFJV and HFII), in which exhalation is passive. Although, multiple studies in animals suggested advantage of HFOV for supporting immature or injured lungs, clinical trials of the routine use of HFV in infants have yielded mixed results [10]. When a high volume strategy (HVS) was used for HFOV, it was associated with significantly lower rates of CLD or death in survivors at 28-30 days and oxygen use at 36-37 weeks postmenstrual age or discharge. There were no overall differences in the rates of IVH or PVL [10].
Recently, Johnson et al (UK) [11] and Courtney et al (USA) [12] published their RCTs comparing HFOV and CMV in preterm infants. Both included infants who were at high risk (GA 23-28 weeks, and BW 601-1200 respectively). The results of the study by Johnson et al. showed no difference between the modes of ventilation in the combined primary outcome of death or chronic lung disease. In contrast, Courtney et al. found that HFOV conferred a small but significant benefit in survival without CLD (56 percent vs. 47 percent), and infants assigned to HFOV were successfully extubated one week earlier. The trial by Johnson received some criticism, while the trial by Courtney et al was conducted under rigorously controlled conditions with well-defined protocols for the management of HFOV [13]. It seems that HFOV administered according to strict protocols may be preferable in the most experienced centers. However, for most preterm infants, CMV with low tidal volumes and reasonable ventilation goals remains the appropriate choice.
PATHOPHYSIOLOGY-BASED VENTILATORY STRATEGIES
Respiratory distress syndrome
RDS is characterized by low compliance and low FRC. An optimal conventional ventilation strategy may include conservative indications for conventional ventilation, the lowest PIP and tidal volume required, modest PEEP (3-5 cm H20), permissive hypercapnia (PaCO2 45-55 mm Hg), judicious use of sedation/paralysis, and aggressive weaning.
Chronic lung disease
BPD usually has heterogeneous time constants among lung areas. Resistance may be increased markedly, and frequent exacerbations may occur. A higher PEEP (4-6 cm H20) often is used, and longer inspiratory and expiratory times with low rates are preferred. The infants' additional spontaneous breaths can be supported by pressure support (PS) mode. PS effects an endurance training of respiratory muscles and reduces the work of breathing. Hypercarbia with compensated respiratory acidosis often is tolerated to avoid lung injury secondary to aggressive mechanical ventilation.
Persistent pulmonary hypertension of the newborn
Persistent pulmonary hypertension of the newborn may be primary or associated with aspiration syndrome, prolonged intrauterine hypoxia, congenital diaphragmatic hernia, or other causes. Ventilatory treatment of infants often is controversial and varies markedly among centers. In general, adjust FiO2 to maintain PaO2 at 80-100 mm Hg to minimize hypoxia-mediated pulmonary vasoconstriction; adjust ventilatory rates and pressures to maintain an arterial pH of 7.45-7.55 (sometimes combined with bicarbonate infusion). Take care to prevent extremely low PaCO2 (<20 mm Hg), which can cause cerebral vasoconstriction and subsequent neurologic injury. Permissive hypercapnea is considered investigational. Addition of inhaled nitric oxide to conventional ventilation reduces the need for extracorporeal membrane oxygenation. Furthermore, the use of HFV to further optimize delivery of inhaled nitric oxide, as a result of its ability to improve lung inflation, has been demonstrated [14].
Congenital Diaphragmatic hernia
An approach that challenged conventional wisdom in the context of a care strategy based on permissive hypercapnea/spontaneous respiration/elective repair has been anecdotally reported by different groups, and demonstrated improved survival. In this approach, lower PaO2 or oxygen saturation (preductal saturations of 75-85% in the first 4 hours of life and > 90% subsequently), higher PaCO2 (60-65 torr or less) and lower PH (> 7.20-7.25) were accepted. The PIP was limited to 24-30 and PEEP to 5 cm H2O. Ventilator adjustments were based on maintaining preductal saturation of 90% or greater with no evidence of evolving metabolic acidosis. Such an approach, although promising, need to be tested in prospective trials to determine the most appropriate management of the infant with CDH [15, 16, 17].
Abdominal Distension
Increased intra-abdominal pressure results in upward pressure on the diaphragm, reduces diaphragmatic excursion, and results in decreased compliance of the respiratory system in newborns with acute intra-abdominal disease, such as necrotizing enterocolitis, bowel obstruction, or postoperatively in infants with gastroschisis, omphalocele, or diaphragmatic hernia. Large tidal volume ventilation further exacerbates the hemodynamic compromise normally caused by positive pressure ventilation. Therefore, a strategy of using small tidal volumes at a higher respiratory rate and adequate PEEP to prevent end expiratory atelectasis seems reasonable. The use of both HFOV and HFJV have been documented to improve ventilation and hemodynamic variables [18].
Sound application of the basic concepts of gas exchange, and pulmonary mechanics is necessary to optimize mechanical ventilation. Employment of pathophysiology-based ventilatory strategies to prevent lung injury, and alternative modes of ventilation should result in further improvement in neonatal outcomes.
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10. Henderson-Smart DJ, Bhuta T, Cools F, Offringa M. Elective high frequency oscillatory ventilation versus conventional ventilation for acute pulmonary dysfunction in preterm infants (Cochrane Review). In: The Cochrane Library, Issue 4, 2002. Oxford: Update Software.
11. Johnson AH, Peacock JL, Greenough A, Marlow N, Limb ES, Martson L, and Calvert SA. High-frequency oscillatory ventilation for the prevention of chronic lung disease of prematurity. N Engl J Med; 347-9: 633-642
12. Courtney SE, Durand DJ, Asselin JM, Hudak ML, Aschner JL, and Stoemaker GT. High-frequency oscillatory ventilation versus conventional mechanical ventilation for very-low-birth-weight infants
13. Stark AR: High-frequency oscillatory ventilation to prevent bronchpulmonary dysplasia - Are we there yet? N Engl J Med, 2002; 347-9: 682-683
14. Kinsella JP, Truog WE, Walsh WF, et al: Randomized, multicenter trial of inhaled nitric oxide and high-frequency oscillatory ventilation in severe, persistent pulmonary hypertension of the newborn. J Pediatr 131:55-62, 1997
15. Boloker J. Bateman DA. Wung JT. Stolar CJ. Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair. Journal of Pediatric Surgery. 37(3):357-66, 2002 Mar.
16. Finer NN. Tierney A. Etches PC. Peliowski A. Ainsworth W. Congenital diaphragmatic hernia: Developing a protocolized approach. Journal of Pediatric Surgery. 33(9):1331-1337, 1998
17. Wilson JM. Lund DP. Lillehei CW. Vacanti JP. Congenital diaphragmatic hernia-A tale of two cities: The Boston Experience. Journal of Pediatric Surgery. 32(3):401-405, 1997
18. Fok TF, Ng PC, Wong W, et al: High-frequency oscillatory ventilation in infants with increased intra-abdominal pressure. Arch Dis Child 76: F123-125, 1997][keszler M, Jennings LL: High-frequency jet ventilation in infants with decreased chest wall compliance. Pediatr Res 41:257 A, 1997
THE INCIDENCE OF INGUINAL HERNIA ASSOCIATED WITH UNDESCENDED TESTIS
*Naguib N.N., Samuel D.G., Izzidien A.Y.
Department of Surgery, Prince Charles Hospital.
Merthyr Tydfil, CF47 9DT.
*Corresponding author: nadernaguib71@yahoo.com
In the eighth week of intrauterine life, the testes arise in the abdominal cavity and descend through the inguinal canal to the scrotum in the third trimester.¹ Testicular descent is a dynamic process, and about 5% of full term infants (and about 25% of premature infants) will not have a descended testis at birth.² 1.5% of boys at 3 months of age will still have undescended testis.³ Cryptorchidism and congenital inguinal hernia commonly co-exist, with the principal defect being a patent process vaginalis.?
In this study, we aim to assess the incidence of inguinal hernia / patent process vaginalis in association with undescended testes.
We conducted a retrospective and prospective study, of 150 pediatric patients (<16 years old) over a 5 year period, who had a first stage orchidopexy at a district general hospital in Wales. Patients' operative notes were reviewed to record the procedure performed, location of the undescended testis, whether unilateral or bilateral, and the presence of associated inguinal hernia / patent process vaginalis.
Over a period of 5 years, 150 pediatric patients had 212 first stage orchidopexy procedures for undescended testes. The age range was 1 – 15 years old with mean age 5.7 and median age of 8. Out of the 150 patients, 62 (41.3%) patients had bilateral procedure, 54 (36%) patients had right side orchidopexy and 34 (22.7%) patients had left side orchidopexy.
The majority of the testes were found in the inguinal canal (158/212 = 74.5 %), 32 (15%) testes were present at the superficial inguinal ring and 16 (7.5%) were at the deep inguinal ring. Only 5 testes (2.4%) were intra-abdominal and 1 testis was ectopic in the superficial inguinal pouch.
Out of the 62 bilateral orchidopexies, 57 (92%) patients had mirror image testes, lying in the same site on the contra lateral side, while 5 (8%) patients had the two testes lying at 2 different levels.
All cases had exploration for the presence of inguinal hernia / patent process vaginalis which was repaired, if found, at the same setting. The overall incidence of inguinal hernia / patent process vaginalis associated with undescended testis was 91% (193/212). The incidences were 93.5% (58/62), 91% (49/54) and 85% (29/34) associated with bilateral, right and left undescended testes, respectively.
Only one of the 58 patients with bilateral undescended testes and inguinal hernia / patent process vaginalis had a unilateral hernia on the right side, the remaining 57 patients had bilateral inguinal hernias / patent processes vaginalis.
There were no complications that occurred in our patient cohort as a result of surgery and no patients have reported a recurrence of their inguinal hernia so far.
The occurrence of a congenital inguinal hernia and undescended testis is related to descent of the testis into the scrotum. The testes usually reach their final destination in the scrotum by the third trimester. In approximately 90% of infants, the processus vaginalis seals and becomes a thin band of tissue without a lumen or opening?. Sacs occur in this region if this muscular obliteration is incomplete and allows for herniation as well as abnormal testicular descent and positioning due to defective muscular propulsion?.
Patients may present at any age with an undescended testis although most studies advocate an early intervention within the first 1-2 years of life. This is seen as beneficial in preserving the testicle as well as reducing the risk of subfertility & malignant transformation seen in later life???.
The incidence of inguinal hernia / patent process vaginalis reported in association with cryptorchidism varies in the literature. Several studies suggest that Cryptochidism is almost always associated with an indirect inguinal hernia???. In a study by Al-Abbadi and Smadi conducted on 37 children, the undescended testis associated with indirect inguinal hernia was reported in 75.68% ?. On the other hand, some studies suggest that only 20% of patients with undescended testes will have an inguinal hernia ¹°?¹¹. Tanyel et al. and Davenport, clarified that although the processus vaginalis is patent in boys with undescended testis, clinical inguinal hernia is only encountered in 10-15% ²??.
Our results confirm that inguinal hernias / patent process vaginalis are a common co-occurrence in children who present with cryptorchidism. And this seems the factor in preventing the normal descend of the testis.
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