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situs inversus totalis is a rare anatomic anomaly with an estimated incidence of 1:20,000 in the general population and an autosomal recessive mode of inheritance .
one associated constellation of malformations includes kartagener 's syndrome , first described in 1904 by siewert in the berliner klinische wochenscrift .
he described a patient in 1901 with dextrocardia and situs inversus , who was also found to have bronchietasis and sinusitis .
adams and churchill reported a series of five patients with this same constellation of findings in 1937 .
vazquez , et al noted that seven percent of babies with biliary atresia had situs inversus or cardiac defects . while disease processes in patients with anatomic developmental anomalies such as gastrointestinal malrotation and nonrotation , visceral situs inversus and situs inversus totalis manifest faithfully their intra - abdominal locations , our standard clinical differential diagnoses often do not account for these variances .
when evaluating a female with abdominal pain , ovulation , hemorrhagic ovarian cysts , pelvic inflammatory disease and endometriosis are all possible etiologies of acute pain , in addition to the gastrointestinal processes of appendicitis , cholecystitis and diverticulitis .
given the often overlapping clinical symptomatology of all of the above disease processes , a careful clinical history coupled with physical examination and an accurate localization of the offending organ becomes paramount .
a 20-year - old woman presented twice during the previous five months to the emergency room with complaints of post - prandial , vague left upper quadrant discomfort .
she had no fever or leukocytosis at these evaluations and was discharged home on each occasion with a working diagnosis of gastroenteritis / gastritis , being treated with oral histamine-2 blockers . on the third presentation , she reported worsening pain with associated nausea and non - bloody emesis .
one five years prior to presentation and one only six months previously . physical examination showed the patient to be afebrile but in mild distress secondary to point tenderness on palpation of her left upper quadrant .
laboratory examination revealed a leukocytosis of 12,000 cells / ml ( normal range : 4.8  10.8 ) , serum bilirubin = 2.0 mg / dl ( 0.2  1.2 ) , alkaline phosphatase = 105 u / l ( 38  126 ) , lactate dehydrogenase = 1000 u / l ( 313  618 ) , serum glutamate - oxaloacetate transferanse = 217 u / l ( 8  40 ) , amylase 78 u / l ( 44  128 ) and lipase 146 u / l ( 23  208 ) .
a chest radiograph demonstrated dextrocardia with an elevated ( but normal in this case ) left hemidiaphragm without other abnormalities ( figure 1 ) .
abdominal ultrasonography showed a left - sided liver and gallbladder with cholelithiasis and dilated intra- and extrahepatic biliary ducts .
given these findings , a 12-lead electrocardiogram was performed , which demonstrated abnormal but characteristic wave morphologies for dextrocardia .
a 2-d echocardiogram of the heart with m - mode doppler yielded no intracardiac malformations .
chest radiograph demonstrates dextrocardia with an elevated left hemidiaphragm when compared to the right , given the presence of a left - sided liver .
after 24 hours of intravenous antibiotics , the patient was taken to the operating room for laparoscopic exploration .
general endotracheal anesthesia was administered , and an infraumbilical port was placed using the open technique for access to the abdominal cavity .
the latter showed no evidence of choledocholithiasis and free efflux of contrast into the duodenum with a mirror - image configuration of the biliary tree ( figure 2 ) .
the appendix was visualized in the left lower quadrant , isolated from the mesoappendix , and resected with an endoscopic stapling device .
an intraoperative cholangiogram visualizes the proper hepatic and common bile ducts with free efflux of contrast into the duodenum with no evidence of filling defects or other abnormalities .
final pathology showed a normal vermiform appendix and gallbladder with chronic cholecystitis and cholelithiasis , containing 11 yellow - green calculi , the largest of which was 5 mm in diameter .
as the bile ducts were free of stones on cholangiography and the alkaline phosphatase level was normal , we believe the ductal dilation noted was secondary to hepatic irritation from the inflammatory process in the gallbladder .
the patient did well postoperatively , with repeat laboratory examination on postoperative day two showing a normal white blood cell count ( 7.5 k ) , serum bilirubin = 1.5 mg / dl , alkaline phosphatase = 111
u / l , lactate dehydrogenase = 492 u / l and serum glutamate - oxaloacetate transferanse = 97 u / l .
the value of laparoscopy in evaluation of patients with atypical abdominal pain has been well established . a small , but real
, subset of the general population has situs inversus totalis or some other type of previously unestablished anomaly of bowel rotation . while chance favors the prepared mind , we are frequently unprepared to evaluate usual disorders set in unusual circumstances .
other investigators have reported the utility of laparoscopy , not only in establishing the diagnosis of rotational bowel abnormalities but also in treating diseases such as acute appendicitis and chronic calcific pancreatitis with a pseudocyst .
dextrocardia , or right looping of the embryological truncus arteriosus and bulbus cordis over the ventricle and atrium , is frequently accompanied by visceral situs inversus .
isolated dextrocardia , by contrast , however , is associated with a high incidence of cardiac defects .
patients with indeterminate visceral situs frequently have asplenia or polysplenia as well as multiple complex cardiac anomalies ( eg , atrial and ventricular septal defects , endocardial cushion defects , double - outlet right ventricle ) .
we believe the presence of dextrocardia mandates further investigation of the status of visceral situs for two reasons .
second , it alerts clinicians and , more importantly the patient of a condition whose repercussions can bring to bear surgical consequences .
sporadic reports of successful laparoscopic cholecystectomy in visceral situs inversus have appeared in the worldwide literature since 1992 .
all stress the feasibility of the laparoscopic approach to treatment with few technical modifications . after entering the peritoneal cavity and insufflation with carbon dioxide , exploration of the abdominal cavity
is undertaken , and the diagnosis of visceral situs inversus , if unknown preoperatively , is discovered .
a standard subxiphoid port is placed , while lateral abdominal ports are placed through the left , rather than right , side of the abdominal wall .
the operative surgeon is positioned to the patient 's right rather than left side and the operation proceeds as usual .
these minor modifications readily facilitate operative exposure of the gallbladder and biliary tree for cholecystectomy and intraoperative cholangiography .
having discussed the issue with our patient preoperatively and in order to preclude any future diagnostic confusion , the appendix was sought , visualized in the left lower quadrant , and excised with an endoscopic stapling device . minimal modification to the standard laparoscopic cholecystectomy protocol is required to treat patients with visceral situs inversus totalis or bowel malrotation anomalies . given the low , albeit real , incidence of associated gastrointestinal conditions , we strongly favor intraoperative cholangiography to clearly delineate the course of the biliary tree and ensure the absence of intraluminal strictures or filling defects .
these considerations , when paid appropriate heed , make laparoscopy not only safe but also expedient and ensure the optimal treatment of patients who have these special instances of common disease processes .
situs inversus totalis is an uncommon anatomic anomaly that complicates diagnosis and management of acute abdominal pain . 
 expedient diagnosis of common intraperitoneal disease processes such as biliary colic , acute appendicitis and diverticulitis is often delayed as a result of seemingly incongruous physical findings . 
 we present the case of a young woman with prior emergency room visits for complaints of a vague left upper quadrant abdominal pain . 
 an ultrasound performed on her third presentation revealed visceral situs inversus with cholelithiasis and dilated intra- and extrahepatic biliary ducts . 
 standard laparoscopic cholecystectomy and cholangiography with a mirror - image surgical approach was performed successfully and without complication .