Disappearing cyst of the hepatic hilum in uncorrectable biliary atresia

A 2-month-old boy with biliary atresia (BA) with an extremely rare prenatal history is reported. A cystic lesion in the hepatic hilum was diagnosed during prenatal ultrasonography at 20 weeks gestation. However, it disappeared during later prenatal observation. He was diagnosed with an uncorrectable type of BA after a massive subdural hemorrhage at the age of 66 days. A fetus suspected of having a cyst of the biliary tree should be followed even after the cyst has disappeared spontaneously.     

Liver transplantation for a hilar inflammatory myofibroblastic tumor

A 7-yr-old boy presented with obstructive jaundice secondary to an inflammatory myofibroblastic tumor centered on the hepatic hilum and extending into the liver. The tumor was further complicated by portal vein phlebitis and occlusion. Attempted resection of the tumor with portal vein reconstruction and bilioenteric drainage was unsuccessful and he required urgent orthotopic liver transplantation. In contrast to more peripheral inflammatory myofibroblastic tumors in the liver, hilar lesions are locally aggressive, causing occlusive portal phlebitis and biliary obstruction. Successful management may include the need for liver transplantation.     

Hepatic inflammatory pseudotumor: case report, review of the literature, and a proposal for morphologic classification

Inflammatory pseudotumors (IPT) are uncommon mass lesions arising most typically in the lungs of young adults. These tumors are so named because of the difficulty in distinguishing them preoperatively from malignant lesions. IPT are characterized histologically by localized fibrous proliferations with infiltration by mononuclear leukocytes, particularly plasma cells. Seventeen previous cases of IPT involving the liver and biliary tree have been reported in children. In this location, IPT may lead to biliary obstruction, portal hypertension, cirrhosis and eventually hepatic failure. We describe the youngest patient ever reported with hepatic IPT (HIPT) and biliary obstruction, who was successfully managed with a left hepatic lobectomy and Kasai portoenterostomy. Based on all previous cases of HIPT in both adults and children (74 cases), we propose a morphologic classification of these lesions based on the presence of single versus multiple lesions, with individualized management. Type 1 lesions are large, solitary lesions, often with central necrosis, giving a characteristic radiographic appearance. Type 2 lesions are multiple smaller, solid nodules indistinguishable from metastatic malignancy.     

Rapid enlargement of a choledochal cyst: antenatal diagnosis and delayed primary excision

A case of choledochal cyst (CC) antenatally diagnosed at 29 weeks' gestation is reported. Rapid enlargement of the cyst soon after delivery resulted in complete gastric outlet obstruction (GOO). The lesion was treated by external drainage as a temporary maneuver, with delayed cyst excision and hepaticoduodenostomy at the hepatic hilum performed at 81 days of age. Surgical treatment of CC in early infancy has been reported to be safe and effective. However, delayed primary excision would be an alternative procedure, especially in rare cases showing rapid enlargement resulting in GOO, since this choice has the potential advantage of allowing weight gain and improved nutritional status without risking interim complications due to the drainage procedure. Accepted: 24 March 1997     

Rabdomyosarcoma of the biliary tree

Rabdomyosarcoma of the biliary tree is one of the rare causes of biliary tract obstruction in childhood. Nevertheless it is the most common cause of obstructive jaundice due to neoplastic biliary obstruction. We present a two-year-old child with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the biliary tree. She underwent surgery and, after total excision of the mass, a hepaticojejunostomy and Roux-en-Y anastomosis were performed. She was referred to the Pediatric Oncology Group for follow-up. Rhabdomyosarcoma of the bilary tree, although rare, must be considered in the etiology of obstructive jaundice in children.     

Antenatal diagnosis of biliary atresia (noncorrectable cyst type): a case report.

At 32 weeks of gestation a cystic mass was identified in the hepatic hilum of a fetus by maternal sonography. Laparotomy was performed at 39 days of life after a diagnosis of correctable type of biliary atresia (Type I). A cystically dilated extrahepatic duct, in which the proximal and distal sides of the common bile duct were occluded (Type III with cyst, noncorrectable type), was identified by operative cholangiography. A standard Kasai operation was performed, and 1 year after operation the patient was doing well and was jaundice-free. From this experience in routine maternal sonography, a cystic mass in the hepatic hilum may also suggest Type III biliary atresia with a cyst.     

Intrahepatic cholangiocarcinoma as a rare secondary malignancy after allogeneic hematopoietic stem cell transplantation for childhood acute lymphoblastic leukemia: A case report

Secondary malignancies are a significant cause of non‐relapse mortality in patients who undergo allogeneic HCT. However, secondary liver cancer is rare, and ICC following HCT has never been reported in the literature. Secondary solid cancers typically have a long latency period, and cholangiocarcinoma is classically a malignancy occurring in older individuals. Here, we report the first case of secondary ICC, which presented just 3 years after HCT in a young adult with a history of childhood ALL. A 26‐year‐old male with history of precursor B‐cell ALL presented with asymptomatic elevated liver function tests 3 years after HCT. Laboratories were indicative of biliary obstruction. ERCP showed focal biliary stricturing of the common and left hepatic ducts. MRCP revealed left intrahepatic duct dilatation, suggestive of intrahepatic obstructing mass. Additional workup lead to a clinical diagnosis of ICC. The patient underwent left hepatectomy with extrahepatic bile duct resection and portal lymphadenectomy. Surgical pathology was consistent with moderately differentiated cholangiocarcinoma. Our case illustrates a rare SMN following HCT for ALL. It is the first case report of ICC occurring as a secondary cancer in this patient population. Although cholangiocarcinoma is characteristically diagnosed in the older population, it must remain on the differential for biliary obstruction in post‐HCT patients.     

Ciliated hepatic foregut cyst: from antenatal diagnosis to surgery

Ciliated hepatic foregut cyst is the only ciliated cystic lesion known to occur in the liver. It is an extremely rare, benign and solitary cyst that probably arises from remnants of the embryonic foregut in the liver. We report a 16-month-old girl who underwent surgical excision of a hepatic cyst discovered during antenatal ultrasonography. Surgical exploration and excision were performed because of the uncertain aetiology of the cyst and because on postnatal follow-up US the size of the mass had increased causing extrinsic biliary obstruction. Pathology revealed a ciliated hepatic foregut cyst. This is the fourth child affected by this lesion reported in the literature, the second undergoing surgical excision, and the second with antenatal diagnosis.     

HEPATIC HETEROTOPIAS IN THE JEJUNUM: A Case Study over Time Showing Progressive Degenerative Changes

Multiple foci of heterotopic liver in the jejunum were sequentially discovered in an infant boy at the ages of 1 day, 2 months, and 4 months. This is the second reported case of jejunal heterotopic liver, a rare entity in any site. Progressive histological changes indicative of biliary duct obstruction were observed in the hepatic heterotopias, which demonstrated no connections to the main body of the liver or biliary tree.     

Ductoplasty for an aberrant hepatic duct in a choledochal cyst

The confluence of the right and left hepatic ducts at the hepatic hilum frequently shows normal anatomic variations. Choledochal cysts (CC) are also accompanied by similar variations, and devices for free drainage of bile are occasionally required in biliary reconstruction. We present a CC that had an aberrant posterior branch of the right hepatic duct draining into the distal common hepatic duct. A capacious hepaticoduodenostomy at the hilum was performed after joining the hilar and aberrant ducts.     

Multiple gastrointestinal atresias with cystic dilatation of the biliary duct

A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome. Accepted: 19 April 1999     

儿童Ⅳa型胆管扩张症的外科治疗探讨

目的探讨儿童Ⅳa型胆管扩张症临床特点及手术治疗。方法选取2015~2017年复旦大学附属儿科医院普外科收治的胆管扩张症患儿且临床资料较完整者,共135例,按Todani分型,Ⅰ型96例,Ⅱ型9例,Ⅲ型1例,Ⅳ型29例。按照纳入标准分组:Ⅳa型29例为A组;Ⅰc型肝内外胆管扩张且互相连续患儿36例设为B组,Ⅰa、Ⅰb型仅肝外扩张患儿56例设为C组,合计121例。回顾性分析患儿术前临床症状、影像学诊断、术中发现、手术过程及预后。结果各组中女性均多于男性,组间差异无统计学意义。以磁共振胰胆管成像(MRCP)为标准,B型超声肝内胆管扩张检出率为30.7%(20/65)。所有B型超声报告均提示第一肝门前囊性占位或胆总管扩张。术前伴随胰腺炎共25例(20.7%)。二次入院患儿共10例,3例经消炎治疗后好转,7例再次手术。术后随访中可见肝内胆管扩张持续存在患儿4例,其中2例无不适症状,予保守治疗,2例患儿行二次手术治疗,手术1个月后扩张胆管消失。结论术中应积极探查肝门处狭窄环及近端扩张胆管,并适当做肝门整形。儿童Ⅳa型胆管扩张症大多预后较好,妥善处理肝外胆道后,肝内胆管扩张术后大多能改善。胰腺炎与囊肿分型无关,术前伴发水肿型胰腺炎患儿多可一期手术。     

Biliary atresia recent insight

Biliary atresia (BA) is a rare disease characterized by ascending obstruction of bile ducts that exclusively affects newborn infants. The etiology of the disease is not known. BA is considered to be a phenotype resulting from several pathogenic processes leading to obstruction of the biliary tree. It usually presents shortly after birth, characterized by persistent jaundice, hepatosplenomegaly, clay-colored stool, and dark urine. It affects both the extra-hepatic biliary ducts (EHBDs) and the intra-hepatic biliary system (IHBDs), but the former is more severely affected. Diagnosis of BA is a great challenge and must be achieved as early as possible to delay progression to cirrhosis. Laboratory tests reveal direct hyperbilirubinemia and, variable levels of transaminases, gamma-glutamyl transpeptidase (GGT), and alkaline phosphatase (ALP), which overlap significantly with other causes of neonatal cholestasis. The intraoperative cholangiogram is considered the gold standard for the diagnosis of BA and is performed routinely in many institutions. BA can be divided into correctable and non-correctable types; the former accounts for (10–15%) of cases, in which the proximal common hepatic duct is patent, allowing primary anastomosis of the EHBDs to the bowel. All patients are subjected to identical surgical and medical treatments; consisting of Kasai portoenterostomy (KPE), which entails removal of the atretic extra-hepatic tissue and a Roux-en-Y jejunal loop anastomosed to the hepatic hilum. Kasai portoenterstomy is considered a transition to liver transplantation, as the pathology may be still ongoing. BA is the most frequent indication for liver transplantation in infants, which is the only treatment that can definitively arrest the natural disease course. In conclusion: BA is a serious liver disease that needs to be further studied, and awareness of BA should be increased among the public and health care workers to prevent the complications of this disease.     

Hepatic duct stone associated with chlamydia sepsis: a rare condition in childhood

Stone formation in the biliary system is a rare condition in infants. A few cases of bile stones in the biliary tree have been reported with underlying predisposing factors, such as sepsis and antibiotic usage. This article describes a surgically treated 16-week-old infant with recurrent cholangitis who had a bile stone in the hepatic duct after chlamydia sepsis. Accepted: 4 February 1997     

Cystic fibrosis mistaken for idiopathic biliary atresia

Previous reports of prolonged jaundice in cystic fibrosis have not described operative and histopathological findings in the liver and biliary tree. In the two cases reported here, obstructive jaundice in the neonatal period was associated with anatomical evidence of intra- or extrahepatic biliary obstruction. Hepatoportoenterostomy, a surgical procedure that is not without complications, was performed on one of the patients for biliary atresia before the diagnosis of cystic fibrosis was suspected. Prolonged obstructive jaundice may be an early manifestation of cystic fibrosis and may resolve without operative management. A sweat test should be performed on all patients with prolonged obstructive neonatal jaundice to rule out cystic fibrosis.     

Myxoid liposarcoma of the porta hepatis in childhood

Liposarcoma in childhood is rare. A case of a myxoid liposarcoma arising at the porta hepatis is reported in a 3-year-old boy with initial favourable response to surgery combined with radio- and chemotherapy. Fatal recurrence occurred at age 15 years. The radiological and sonographic findings of liposarcomas are reviewed.     

Bilobar dilatation of the intrahepatic biliary ducts: an unusual presentation of Caroli's disease in childhood

Congenital cystic dilatation of the intrahepatic biliary ducts has been infrequently recognized in childhood. The majority of cases present with dilatation of both the intrahepatic and extrahepatic biliary systems. This report describes a 7-year old boy presenting with hepatomegaly and fever. Computed tomography, ultrasonography, ERCP, and hepatic scintigraphy demonstrated bilobar intrahepatic cystic dilatation of the biliary tract with a normal extrahepatic system. The symptoms were immediately alleviated by a short course of antibiotics. Nevertheless, the patient is at risk for recurrent infection, malignancy, and hepatic failure and is awaiting an appropriate operative solution for his extremely rare anomaly.     

Spontaneous perforation of the extrahepatic biliary tract in infancy and childhood

Spontaneous perforation of the extrahepatic biliary tract in infancy and early childhood is a poorly understood and infrequently reported disorder of unknown etiology. The reported experience with 77 operative cases is reviwed. The onset is generally insidious, but may be acute in 25% of cases. The major symptoms are abdominal distention and jaundice. The younger the patient at the time of onset, the more likely is jaundice to be found. Drainage-type operations were most commonly used in the early reported cases while more recently anastomosis/bypass procedures have predominated due to the relatively frequent occurrence of distal biliary tract obstruction. This disorder appears to be a part of the biliary atresia — neonatal hepatitis — choledochal cyst complex which occurs in the neonatal period and early infancy and appears to be acquired rather than congenital. Because of the theoretical potential for late malignancy, lifelong follow-up with periodic evaluation of the intra- and extrahepatic biliary tracts is recommended. The advisability of liver transplantation (and accompanying immunosuppression) as the primary surgical treatment for biliary atresia may be questioned.     

Focal nodular hyperplasia in biliary atresia patient after Kasai hepatic portoenterostomy

We present the case of a 10-year-old girl with biliary atresia in whom focal nodular hyperplasia (FNH) of the liver occurred following successful “Kasai” hepatic portoenterostomy at the age of 2 months. Her jaundice completely disappeared a few weeks after the operation. A 4-cm diameter liver tumor in segment IV was found when she was 5-years old. Needle biopsy could not establish a definitive diagnosis. Because the tumor size was not changing, she was conservatively followed by abdominal echo at an outpatient clinic. She showed progressive liver dysfunction and ongoing cirrhosis, and so at the age of 10 years, she received living donor liver transplantation at our institute. The tumor was pathologically diagnosed as a FNH. Though a FNH in biliary atresia patients is extremely rare, there are only two cases reported in the literature; it should be considered in the differential diagnosis for a hepatic nodule during a long follow-up course in patients with biliary atresia.     

Successful stenting for Budd–Chiari syndrome after pediatric liver transplantation: a case series and review of the literature

Hepatic venous outflow obstruction, known as Budd–Chiari syndrome, is a rare but serious complication of orthotopic liver transplantation and may lead to graft loss. Our aim was to describe a single center experience of percutaneous interventional management of hepatic outflow obstruction post liver transplantation in children. Three children with post-transplant hepatic venous outflow obstruction were treated successfully with percutaneous stent placement. This resulted in long-term resolution of symptoms and was superior to balloon angioplasty both in our series and in cases reported in the literature. Stenting may be the treatment of choice for hepatic venous obstruction after pediatric liver transplantation, particularly if there is a recurrence of stenosis after balloon dilatation.