Spontaneous rupture of choledochal cyst

Choledochal cyst in an uncommon congenital anomaly with classic presentation triad of abdominal pain, jaundice and right upper abdominal mass. Presentation due to biliary peritonitis following cyst rupture is extremely rare. One such case which was successfully treated is being reported.     

Spontaneous rupture of a choledochal cyst in a 11-month-old girl]

Choledochal cysts are rare congenital malformations of the biliary tract. Though most cysts are diagnosed incidentally, some present directly with complications. We report on the case of an 11-month-old girl admitted for abdominal pain, fever and vomiting. Ultrasonography revealed intraabdominal fluid and the absence of a choledochal cyst diagnosed 2 months earlier. Laparotomy for suspected rupture of a choledochal cyst was planned and a choledochojejunostomy with Roux-en-Y was performed. Spontaneous rupture of a choledochal cyst is rare and occurs most frequently in children under the age of 4. The exact cause is yet unknown and several factors have been implicated. The most probable cause is the combination of pancreatic reflux and epithelial irritation of a weakened cyst wall. Choledochal cysts should preferably be treated as soon as the child is 6 months old. Complete excision of the cyst is mandatory because of the risk of malignant transformation.     

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 a choledochal cyst

Two children with spontaneous perforation of a choledochal cyst were treated successfully. In one of the patients an operative cholangiogram demonstrated not only fusiform dilatation of the common bile duct but also an unusual long common channel between the junction of the common bile duct and pancreatic duct, and the papilla. The long common channel, namely an anomalous choledocho-pancreatic ductal junction, under which there were concentrated bile acids and refluxed pancreatic juice, was thought to lead to chronic inflammation of the bile duct, followed by choledochal cyst and/or perforation of the cyst.     

Spontaneous rupture of a choledochal cyst: clues to diagnosis and etiology.

A healthy 3-year-old black girl had acute onset of abdominal pain, vomiting, and elevated aminotransferase, amylase, and lipase levels. Sonographic evaluation suggested a choledochal cyst, but hepatobiliary scintigraphy clearly showed an extrahepatic biliary leak. Exploratory laparotomy confirmed bile peritonitis and a ruptured choledochal cyst. Other cases with this unusual presentation is reviewed in the literature. An anomalous insertion of the pancreatic duct into the common duct appears to facilitate reflux of pancreatic secretions into the biliary tree and may contribute to formation and even perforation of a choledochal cyst.     

Spontaneous rupture of extrahepatic choledochal cyst: two pediatric cases and literature review.

Spontaneous rupture of extrahepatic choledochal cysts is very rare. Neonatal cases generally present with biliary ascites, and older children with acute abdomen. Although the cause is unclear, accumulation of protein plugs in an anomalous pancreaticobiliary junction, irritation of the cyst wall due to reflux of pancreatic juice, and weakness due to a developmental error such as common channel syndrome have all been suggested to play contributing roles. The treatment of choice is complete excision of the cyst and hepatico-jejunostomy. In newborns who are in a poor condition, the primary treatment should be simple peritoneal drainage.     

Neonatal sepsis presenting as a choledochal cyst

We report a case of a neonate who presented with Klebsiella sepsis and a fusiform dilation of the common bile duct on ultrasound suspected to be a choledochal cyst type Ia. In addition, a biliary sludge was noted in the gallbladder. All findings resolved with resolution of the sepsis. We discuss the case and review the literature regarding the association of sepsis with cholestasis and biliary sludge. CONCLUSIONS: we suggest that in any case of sepsis presenting as a choledochal cyst, it is essential to repeat the ultrasound after complete recovery before a surgery is planned.     

Traumatic rupture of a choledochal cyst masking as a duodenal hematoma

Choledochal cysts are rare, congenital malformations of the intra and/or extrahepatic biliary tree. We describe a case in which a patient was transferred to our hospital with a reported duodenal hematoma. The patient ultimately required exploration when his condition deteriorated. Laparotomy resulted in the discovery and successful treatment of a ruptured type IV-A choledochal cyst.     

Children presenting with hydatid cysts in common bile duct and choledochal cyst

Hydatid cysts developing primarily in the common bile duct are extremely rare. Occasionally, hydatid cysts rupture into the extrahepatic biliary ducts and cause obstruction [1–7]. Two children with intact hydatid cysts in the common bile duct and choledochal cysts were seen during a 2-year period in the Paediatric Surgery Division of the Children's Hospital, Banaras Hindu University, Varanasi. The details of their presentation and operative management are reported.     

Postnatal rupture of an antenatally diagnosed choledochal cyst: first case report

A 7-mo-old girl with an asymptomatic antenatally diagnosed choledochal cyst was referred to the regional paediatric surgical centre for advice on management. Following further radiological investigation and discussion with the supra-regional centre, a date for elective surgery was arranged. However, in the interim, she was admitted as an emergency following rupture of the cyst. She made an uneventful recovery following urgent laparotomy. Conclusion: We recommend prompt paediatric surgical referral following antenatal diagnosis of a presumed choledochal cyst. Delay in surgery may permit expansion and rupture of the cyst, even after a period of asymptomatic stability.     

Pathogenesis of choledochal cyst

The well-known observation that females have a much higher incidence of choledochal cysts than males suggests that this may be a sex-linked hereditary disease. However, up to now there have been no familial case reports in the literature. The author describes a sex-linked familial case. Both, the mother and her single daughter suffered from a type I choledochal cyst with a narrow segment and amylase-negative bilious fluid in the cyst. In order to lend support to the plausibility of a hereditary disease, blood types, histocompatibility, and chromosome status were investigated. Although their blood type was the same (A₁ positive) and histocompatibility antigens were only slightly different, we could not detect a chromosomal defect in either mother or child and could not find any association with other malformations. Despite these implications against the possibility of a hereditary disease, it should be noted that in both patients the nature of the choledochal malformation was the same, especially with regard to the spastic inferior choledochal sphincter (= narrow segment) and the amylase-negative bile. Regarding the forms of choledochal dilatation, the author presumes that types I, IV, and V have a different pathogenesis than types II and III.     

Forme fruste choledochal cyst

Two girls presenting with features of pancreatitis were diagnosed to have minimal dilatation of extra hepatic biliary duct (EHBD) associated with pancreatico-biliary maljunction (PBMJ). A high degree of suspicion is required to diagnose this condition that has been termed Forme fruste choledochal cyst (FFCC). Both did well with pancreatico-biliary disconnection procedure and reconstruction of biliary channel using enteric conduit.     

Why not choledochal cyst?

With excision as a standard procedure for treating choledochal cyst, early diagnosis becomes a central issue in reducing morbidity and mortality from that disorder. In a retrospective analysis of 35 patients treated over a 10-year period, we found the diagnosis was delayed for an average of 11.9 months from the beginning of presenting symptoms. Three factors were responsible: (1) a random combination of non specific presenting symptoms such as pain and vomiting or jaundice and hepatomegaly frequently led to a diagnosis such as acute pancreatitis or hepatitis, in which conservative treatment was considered most appropriate; (2) the three parameters commonly used to diagnose acute pancreatitis (serum amylase, lipase, and Cam/Ccr) may all be elevated in patients with a choledochal cyst; and (3) a combination of several noninvasive examinations such as radionuclide cholescintigraphy, ultrasonography, and upper gastrointestinal X-rays may fail to diagnose some difficult cases of choledochal cyst. To obviate such problems, we recommend a routine ultrasound examination of biliary tract in children with recurrent or progressive obstructive jaundice or unexplained acute pancreatitis. When these noninvasive examinations fail to solve the problem, endoscopic retrograde cholangiopancreatography should be considered. Offprint requests to: J.-H. Chuang     

腹腔镜胆总管囊肿切除肝管空肠Roux-en-Y吻合手术总结

目的 本研究对腹腔镜胆总管囊肿切除肝管空肠Roux-en-Y吻合手术6年经验进行总结,探讨该技术的近期和远期疗效.方法 从2001年4月至2007年3月,本课题组采用腹腔镜技术治疗先天性胆总管囊肿患儿102例,平均年龄3.2岁.其中13～28d9例,29d～6个月23例,7个月～3岁28例,4～18岁42例.采用四trocars技术完成胆道造影、胆囊和胆总管囊肿壁全层切除;同时利用腹腔镜头对扩张的肝内胆管进行探查;从脐部切口提出空肠,直视下行Roux-en-Y空肠吻合,然后还纳肠管;经结肠后上提空肠的肝支,镜下将肝管与空肠连续吻合.结果 102例全部在腹腔镜下完成手术,无中转开放手术者,平均手术时间224min(155～456min),手术中出血5～10ml,无手术中需要输血者.21例患儿合并肝门部肝管狭窄,行肝管扩大成形术;其中1例患儿手术后并发胆漏,持续腹腔引流26d,自然愈合.2例患儿分别于手术后第二天至第五天和第七天并发消化道出血.1例5岁女孩手术后7.5h突发心跳骤停,高血钾(K+10.85mmol/L),死亡.其他98例患儿手术后恢复顺利,手术后3～6d出院.1例患儿于手术后6个月发生空肠肝支梗阻坏死,行坏死肠管切除,二次肝管空肠Roux-Y吻合手术.患儿手术后经3～72个月的随访肝功能正常,无结石和胰腺炎发生.结论 腹腔镜胆总管囊肿切除肝管空肠Roux-en-Y吻合手术是治疗先天性胆总管囊肿可靠的手段,镜下放大的手术视野有利于精确的手术操作,近期和远期疗效良好.手术后早期有必要密切观察血钾的变化.     

Spontaneous perforation of choledochal cyst: a case with unusual distribution of fluid in the retroperitoneal space

Images of perforated choledochal cysts typically show an intraperitoneal fluid collection. We report a case with, in addition to free intraperitoneal fluid, fluid collection in the right-side anterior pararenal and perirenal spaces. Surgery confirmed the presence of a perforation at the junction of the cystic duct and the common bile duct. This perforation may explain the biliary leakage extending into the free peritoneal space as well as into the anterior pararenal space and the hepatoduodenal ligament. Anterior pararenal and perirenal spaces communicate with the infrarenal space, and this may result in extension of the fluid into the perirenal space from the anterior pararenal space.     

Nonrefluxing biliary appendicoduodenostomy for choledochal cyst

A new technique of biliary appendicoduodenostomy along with an antireflux procedure was used for the treatment of a choledochal cyst, with a satisfactory result.     

Clinical analysis of liver fibrosis in choledochal cyst

Purpose

Although most patients with choledochal cyst (CC) have a favorable prognosis with prompt treatment, some of them are complicated with liver fibrosis, resulting in prolonged liver dysfunction even after definitive surgery. The aim of this study was to distinguish the high-risk group of liver fibrosis in patients with CC.

Methods

Fifteen patients who underwent liver biopsy during surgery for CC from 1981 to 2012 were enrolled in this study. Liver histology with H&E staining was graded according to Ohkuma’s classification, and the correlation with the clinical characteristics was retrospectively assessed.

Results

Their median age at biopsy was 13.4 months (range 1–42 months), and there were 7 in Ia, 2 in Ic, and 6 in IVa of the Todani classification. There were no significant differences in serum liver function test among those cyst types. The histological grades of liver fibrosis were as follows: grade 0 (no fibrosis) in 7 patients, grade 1 (mild) in 2, grade 2 (moderate) in 3, grade 3 (severe) in 2, grade 4 (cirrhosis) in 1. All 3 patients with grade 3 and 4 had CC with IVa and were under 18-month old. A 3-month-old girl with cirrhosis presented with severe jaundice resulting in living-donor liver transplantation despite bile drainage. A 16-month-old girl with grade 3 suffered from prolonged liver dysfunction and intractable ascites after surgery.

Conclusions

The progression of liver fibrosis is likely to be correlated with IVa, and the postoperative course might be unsatisfactory in advanced cases. The prompt surgical intervention is recommended especially for neonatal and infantile cases with type IVa cyst because irreversible liver cirrhosis could occur as early as in the infantile period.