Spontaneous perforation of the biliary tract and portal vein thrombosis in infancy

Spontaneous biliary perforation (SBP) is an uncommon cause of surgical jaundice in the first few weeks of life and is characterised by the occurrence of a punched-out defect in the bile duct, typically where the cystic duct joins the common hepatic duct. In most cases the site occurs anteriorly and bile leaks into the general peritoneal cavity. We now describe two cases of SBP where the perforation occurred posteriorly, limiting the leak and resulting in delayed recognition. Surgical management in both cases consisted of hepaticojejunostomy-en-Roux. Both cases were complicated, although not immediately, by portal vein thrombosis and one by chylous ascites. Posterior SBP, presumably due to their intimate anatomical relation with the portal vein, seem predisposed to such complications.     

Spontaneous biliary perforation in infancy and childhood: Clues to diagnosis

Spontaneous perforation of extrahepatic bile duct is rare. The cause is idiopathic once trauma and choledochal cyst are ruled out. The condition presents a diagnostic dilemma. Preoperative recognition is necessary as early surgical intervention gives excellent prognosis. We report clinical observations made in three cases with acute presentations. Diagnosis is to be suspected by the presence of jaundice after an initial anicteric period of good health with biliary ascites. This is confirmed by bilious abdominal paracentesis, signs of peritonitis and absent free gas on x ray. The constellation of these three findings was constant in three patients. The presented paper highlights the same as reliable clues to diagnosis.     

Spontaneous biliary perforation : a rare entity in late infancy and childhood

Spontaneous perforation of the biliary tract is rare in infants & children with less than 100 cases reported in English Literature till date. We report two cases of this rare clinical entity in a 9-month-old boy and other of a 2-year old boy. Both patients presented with clinical features of acute peritonitis. Laparotomy revealed sterile biliary peritonitis with a normal cholangiogram in the infant and biliary perforation in other child. Simple drainage in the former and cholecystostomy with external biliary drainage in later resulted in a satisfactory outcome.     

Spontaneous infantile choledochal cyst perforation

Spontaneous perforation is a rare complication of infantile choledochal cysts. A 23-weeks female with acute biliary peritonitis is described and differentiation from a spontaneous perforation of the extrahepatic bile duct highlighted.     

Spontaneous perforation of the extrahepatic bile duct in an infant

 A 1.2-year-old male presented with gradual-onset biliary ascites, mild icterus, and failure to thrive due to spontaneous bile-duct perforation (SPBD) confirmed by technetium 99Tc HIDA scan and abdominal paracentesis. Peritoneal tube drainage for 2 weeks helped the perforation to seal spontaneously, as there was no distal obstruction. No surgery was needed. Only less than 100 cases of SPBD have been reported in the English literature. The pathogenesis and treatment options are reviewed and discussed. Accepted: 10 May 2000     

Spontaneous biliary perforation presenting as gastric outlet obstruction

Spontaneous biliary perforation (SBP) is a rare, surgically correctable cause of jaundice in neonates. The presenting feature is usually biliary ascites, and in rare cases, biliary peritonitis. This article reports a case of SBP, which presented with features of gastric outlet obstruction, leading to an erroneous preoperative diagnosis. Most probably this is the first report of such an unusual presentation of SBP. The child underwent exploratory laparotomy and a bilio-enteric bypass with drainage of the right subhepatic space, which led to a prompt resolution of the symptoms     

Spontaneous biliary perforation in a child with features of Ivemark syndrome

A case of spontaneous biliary perforation is reported for the following noteworthy aspects: its rare mode of presentation as an acute intestinal obstruction; its presentation in late infancy; and its association with Ivemark syndrome. Accepted: 24 November 1997     

Spontaneous perforation of the extrahepatic biliary tree in infancy. A case report

Spontaneous perforation of the bile duct in infancy is rare, with less than 55 cases described in the literature to date. The authors report the case of a 30-day-old neonate who presented with a 2-week history of progressive abdominal distension and intermittent jaundice. Disofenin technicium 99m sequential scintiscanning provided a preoperative noninvasive confirmation of the diagnosis of biliary ascites secondary to spontaneous perforation of the extrahepatic biliary tract. Distal common bile duct atresia was identified intraoperatively, and end-to-side Roux-en-Y hepaticojejunostomy was performed, with an uneventful postoperative recovery. The etiology, diagnosis, and treatment of spontaneous neonatal biliary perforation is discussed.     

Spontaneous biliary perforation and necrotizing enterocolitis

A case of spontaneous bile-duct perforation in a 5-month-old boy with a history of necrotizing enterocolitis in his 1st week of life is reported. To our knowledge, this is the second case reported with such an antecedent, supporting a vascular etiology for some cases of spontaneous biliary perforation. Accepted: 7 July 1998     

Idiopathic biliary perforation in a 10-year-old boy

A 10-year-old boy of African origin with AIDS and Stage IV Hodgkin’s lymphoma presented with a short history of abdominal distension and bile ascites shown to be due to a perforation of his common bile duct. This was treated initially by laparotomy, external peritoneal drainage and endoscopic biliary stenting although without success. He then underwent a laparotomy and biliary diversion (Roux-en-Y hepaticojejunostomy) with satisfactory resolution of his bile duct pathology. Although the co-morbid conditions are likely to be contributory factors, biliary perforation in either disease has not been reported before.     

Spontaneous gastrointestinal perforation in very-low-birth-weight infants – a rare complication in a neonatal intensive care unit

Over a 6-year period (1989–1995), gastrointestinal (GI) perforation was diagnosed in nine preterm infants (mean gestional age 27 weeks, mean birth weight 872 g). Three presented with necrotizing enterocolitis (NEC), two with indwelling-tube-induced perforation of the stomach, one with small-left- colon syndrome, and another with meconium ileus. Spontaneous intestinal perforation occurred in two similar very-low-birth-weight (VLBW) infants, in the distal ileum, on days 8 and 9 of life, respectively. The only clinical sign was extensive abdominal distension, and abdominal X-ray studies revealed free peritoneal air. All findings were distinct from those associated with NEC. Their further clinical course was complicated by reperforation on day 32 and 39, respectively. They subsequently recovered and presented without GI problems at the corrected ages of 4 and 2 months, respectively. In contrast to high mortality of 57% in the group with non-spontaneous intestinal perforations, spontaneous perforation seems to have a good prognosis even in VLBW infants if diagnosed and treated promptly. Accepted: 16 December 1996     

磁共振胆胰管成像在小儿胆道疾病诊断中的价值

目的 探讨磁共振胆胰管成像（MRCP）在小儿胆道疾病诊断中的实用价值。方法 用TOSIBA VISART，1.5T（磁场强度）磁共振机及相关软件和技术，检查新生儿肝炎1例，肝外胆道闭锁1例，胆总管囊性扩张8例，胆道术后腹痛2例。结果 新生儿肝炎者因麻醉欠佳而检查失败，肝外胆道闭锁者呈盲管状的胆总管及2级肝管可辨；8例胆总管囊性扩张者均可见肝外胆道及3级肝管、胰管及其立体构型；2例胆道术后腹痛者均查出病因。结论 MRCP同样适合小儿胆胰疾病的诊断，具有易施行、无放射、无创伤、无并发症，且图像清晰，可多方位显示自然状态下的胆管树三维构型，尤为适合不宜行胆道造影术的病例。     

Spontaneous intestinal perforation after oral ibuprofen treatment of patent ductus arteriosus in two very-low-birthweight infants

AIM: To discuss intestinal side effects of ibuprofen in the treatment of patent ductus arteriosus, after having observed two cases of spontaneous intestinal perforation following ibuprofen treatment. METHODS: Clinical and laboratory records of two preterm infants, who developed intestinal perforation after ibuprofen administration, were evaluated. RESULTS: Gestational ages of infants were 29 wk (male) and 30 wk (female). Both infants developed intestinal perforations without signs of necrotizing enterocolitis. The perforations cured with Penrose drainage alone. CONCLUSION: Although ibuprofen is a reasonable treatment alternative to indomethacin, randomized controlled trials, which address potential adverse effects including spontaneous intestinal perforation, are needed.     

先天性胆管扩张症胆囊粘膜增殖性变化

目的 观察先天性胆管扩张症胆囊粘膜的增殖性变化。方法 先天性胆管扩张症患者４２例,其中儿童３５例,成人７例;慢性胆囊炎胆石症成人２０例,对照组５例。取石蜡切片行ＨＥ和增殖细胞核抗原（ＰＣＮＡ）染色,观测,增生形态指标（即胆囊粘膜皱襞高度、皱襞密度、皱襞细胞密度）以及ＰＣＮＡ阳性率。结果 胆管扩张症、慢性胆囊炎胆石症患者的增生形态指标及ＰＣＮＡ阳性率均高于对照组（Ｐ〈０．０５）。成人胆管扩张症ＰＣＮ     

Spontaneous perforation of the extrahepatic bile duct in infancy: report of two cases and literature review.

BACKGROUND: Spontaneous perforation of the bile duct (SPBD) is a rare disease in infancy. The pathogenesis, diagnostic modalities and treatment options for this condition are reviewed and discussed. METHODS: The authors report 2 new observations of SPBD in 2 male newborns aged respectively 27 and 21 days. RESULTS: The 2 newborns presented with cholestatic jaundice. Abdominal sonography showed an extrahepatic mass. Exploratory laparotomy revealed that the perforation was located in the cystic duct in the first case and in the common bile duct (CBD) in the second. The site of perforation was repaired and both patients underwent simple external biliary drainage. The postoperative course was uneventful for both patients with follow-up ranging from 2 to 4 years. CONCLUSIONS: SPBD is a rare, but important cause of surgical jaundice in infants. The pathogenesis of SPBD is unknown and multifactorial and diagnosis is often problematic. Surgical management is always required and a conservative approach is usually recommended. The prognosis is good with early surgical management.     

Spontaneous gallbladder perforation in a neonate

We describe the case of a 700-g neonate who presented with abdominal discolouration, distension, and rapid clinical deterioration. At laparotomy, a perforation in the neck of the gallbladder was found, which was simply oversewn. Perforation of the gallbladder should be considered in the differential diagnosis in such neonates. The surgical management should be direct repair, without resorting to a cholecystectomy.     

Spontaneous fecal fistula in a neonate

A 19-day-old male neonate was brought to us with a left upper quadrant abdominal wall defect through which bowel was prolapsing, with a double intussusception appearance typical of a patent omphalomesenteric duct. However, the history showed that the defect was not congenital, the child had a normal umbilicus, and at surgery the lesion was shown to be a mid jejunal perforation with prolapse of bowel along both the ascending and descending limbs. Histology revealed presence of inflammation and no heterotopic tissue. We believe this is the first ever report of such a fecal fistula and we speculate on the cause of this entity.     

Recurrent spontaneous perforation of intestinal lymphoma

The possibility of intestinal lymphoma should be kept in mind when operating upon an immunodeficient child with an intestinal perforation without apparent reason; the phenomenon can recur during the post-operative period without being recognized early.