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a recent review of the english - language literature revealed no published reports of management of appendicitis complicated by pylephlebitis with minimally invasive surgical techniques .
we describe successful management with intravenous antibiotics and anticoagulation followed by interval laparoscopic appendectomy in a 5-year - old girl .
a previously healthy 5-year - old girl presented with a 7-day history of fever , vomiting , and anorexia and a 24-hour history of diffuse abdominal pain . at the time of presentation , her rectal temperature was 104.1f .
notable laboratory abnormalities included white blood count 14.5 ( x10/l ) with 83% polymorphonuclear leukocytes , aspartate amino transferase ( ast ) 66
u / l , amino alanine transferase ( alt ) 107 u / l , alkaline phosphatase 267 u / l , and total bilirubin 1.1 mg / dl .
computed tomography ( ct ) of the abdomen and pelvis was obtained and revealed a thickened retrocecal appendix with associated inflammatory changes but no focal fluid collection ( figure 1 ) , thrombosis of the superior mesenteric vein ( figure 2 ) with extension of the thrombus into the left branch of the portal vein , and impaired perfusion of the left lobe of the liver ( figure 3 )
impaired perfusion of the left lobe of the liver , secondary to extension of thrombus into the left branch of the main portal vein .
the patient was admitted to the hospital , hydrated , placed on broad - spectrum intravenous ( iv ) antibiotics , and anticoagulated with iv unfractionated heparin .
a diet was offered on hospital day 4 , which she tolerated without difficulty . on hospital day 6 , a follow - up ct scan was obtained and demonstrated dramatic improvement in hepatic perfusion ( figure 4 ) , despite residual superior mesenteric vein thrombus .
discharge medications included low - molecular weight heparin , enoxaparin 40 mg sq qd ( lovenox , aventis pharmaceuticals , inc , france ) .
she was subsequently converted to oral warfarin , dose - adjusted to maintain an international normalized ratio ( inr ) between 2.0 and 3.0 . improved perfusion of the liver after 6 days of intravenous antibiotics and anticoagulation .
pediatric hematology consultation was obtained , and a hypercoagulability workup was initially consistent with heterozygous protein c deficiency : activity 35% ( normal , 64% to 126% ) .
antithrombin iii level was also mildly low at 60% ( normal , 70% to 115% ) .
protein c antigen level while the patient was on heparin was 99% ( normal , 71% to 157% ) .
however , coagulation functional screens obtained at the time of clot presentation are known to be unreliable .
after 3 months , the warfarin was discontinued ; repeat testing at that time revealed no evidence of protein c deficiency ( activity 82% of normal ) nor of antithrombin iii deficiency ( 101% of normal ) .
four months after initial presentation , the patient underwent laparoscopic appendectomy . intraperitoneal examination revealed mild scarring and evidence of previous inflammation , but no other abnormality ( figure 5 ) .
she had an uncomplicated postoperative course and was discharged home on the third postoperative day .
mild scarring and postinflammatory changes in the right lower quadrant at the time of interval laparoscopic appendectomy 4 months following acute appendicitis , pylephlebitis , and mesenteric venous thrombosis .
before the second half of the 20th century , the incidence of mesenteric venous thrombosis and pylephlebitis complicating appendicitis was less than 1% . since the advent of modern antibiotic therapy and aggressive surgical approaches to appendicitis
, pylephlebitis has become an exceedingly rare , yet still potentially fatal , complication of appendicitis .
although the low incidence makes it difficult to know the true mortality rate of pylephlebitis secondary to appendicitis , modern estimates of the mortality of pylephlebitis from all causes are as high as 50% . although pylephlebitis may rarely occur shortly after the onset of symptoms , it most often is associated with cases of appendicitis in which a delay occurs in presentation or diagnosis .
because the presentation of appendicitis can be atypical in young children , they are at increased risk for delayed presentation to a health care provider and diagnosis .
traditional management of appendicitis complicated by pylephlebitis has included intravenous antibiotic therapy and emergent exploratory laparotomy with appendectomy .
the need for long - term anticoagulation has been a topic of debate for the last century and remains disputed . a review of the english - language literature by using pubmed ( national center for biotechnology information , bethesda , md ) yielded 24 articles published since 1959 , describing 34 cases of mesenteric venous thrombosis and pylephlebitis complicating appendicitis .
additionally , a review by klinefelter et al details 62 cases reported between 1926 and 1959 . of the 34 reported cases since 1959 , only 4 occurred in females .
this is consistent with klinefelter 's observation that roughly 85% of cases of pylephlebitis secondary to appendicitis occur in males .
forty - four percent ( 15/34 ) of the cases occurred in children ( table 1 ) . in the children , pylephlebitis was present at the time of diagnosis of appendicitis in 10 cases .
seven of these patients were treated with urgent exploratory laparotomy and appendectomy , and several of these patients went on to require additional procedures .
two of the 10 patients were treated with interval open procedures , and our current patient was treated with an interval laparoscopic appendectomy . for the remaining 2 patients , no description of the surgical management was included in the report .
of the 3 patients in whom onset of pylephlebitis occurred after appendectomy , 1 patient required subsequent second and third laparotomies , while 2 were managed nonoperatively .
details of the 15 reported cases of mesenteric venous thrombosis and pylephlebitis complicating appendicitis in children since 1959 to our knowledge , the case described in this report is the first case of appendicitis complicated by pylephlebitis that was treated using a minimally invasive approach . using anticoagulation and intravenous antibiotics , we were able to successfully manage the acute presentation nonoperatively .
this is the first reported case of minimally invasive management of appendicitis complicated by pylephlebitis and mesenteric venous thrombosis .
based on our experience we feel that , for children with this rare presentation , interval laparoscopic appendectomy , after a course of anticoagulation and intravenous antibiotics , is an acceptable alternative to routine emergent exploratory laparotomy and appendectomy .
background : although rare , portal mesenteric venous thrombosis and pylephlebitis remain potential life - threatening sequelae of ruptured appendicitis in children . 
 treatment recommendations from recent reports have included urgent exploratory laparotomy with appendectomy , prolonged intravenous antibiotic therapy , and anticoagulation for up to a year.methods:this report describes successful management of pylephlebitis and mesenteric venous thrombosis complicating ruptured appendicitis with intravenous antibiotics and anticoagulation followed by interval laparoscopic appendectomy.results:a previously healthy 5-year - old girl was diagnosed with ruptured appendicitis complicated by pylephlebitis and mesenteric venous thrombosis at the time of presentation . 
 she was treated with intravenous antibiotics and anticoagulated for 3 months . 
 she subsequently underwent interval laparoscopic appendectomy . at 3-year follow - up 
 , she is healthy without evidence of adverse sequelae.discussion:this is the first reported case of successful , minimally invasive management of ruptured appendicitis complicated by mesenteric venous thrombosis and pylephlebitis.conclusion:similar treatment of other children with this rare presentation seems reasonable .