Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature

This paper highlights the etiology, diagnosis, management and outcome in nine unusual cases of extrahepatic biliary obstruction in children. Extrahepatic biliary atresia and choledochal cyst constituted 127 out of 136 (93%) cases of all pediatric surgical biliary disorders managed between March 2000 and February 2007 at the reporting centre. However, nine children (aged 1.5–15 years) presented with uncommon causes like (1) idiopathic benign non-traumatic inflammatory stricture (n = 3), (2) idiopathic fibrosing chronic pancreatitis (n = 2), (3) post-cholecystectomy type 4 benign biliary stricture (n = 1), (4) post-acute pancreatitis pseudo-cyst of pancreas (n = 1), (5) non-Hodgkin's lymphoma (NHL) with extramural common bile duct compression and gall bladder perforation (n = 1), and (6) Langerhan cell histiocytosis (LCH, n = 1). The clinical features and the diagnostic work up of each group are discussed. A preoperative endoscopic/percutaneous biliary drainage was required in four children because of cholangitis at presentation. A biliary-enteric anastomosis was performed for all seven children in groups (1)–(4). The patients with NHL and LCH were referred for chemotherapy after establishing tissue diagnosis at laparotomy. With a follow-up period of 3 months to 7 years, seven children (with the exception of patients with NHL and LCH) are currently anicteric. This paper draws attention to some infrequently discussed causes of extrahepatic biliary obstruction in children. The management entails a carefully planned combination of endoscopic interventions, interventional radiology and surgery. The outcome in benign cases is usually satisfactory.     

Intermittent ureterovesical junction obstruction in children

While intermittent ureteropelvic junction obstruction is well recognized clinically, intermittent obstruction at the ureterovesical junction has not been previously described. We herein present a case report of intermittent ureterovesical junction obstruction, characterized by intermittent hydroureteronephrosis on ultrasonography and symptoms of flank/abdominal pain, nausea and vomiting.     

Congenital mesenteric hernia causing intestinal obstruction in children

A mesenteric hernia is one type of intraperitoneal hernia. There is no hernia sac in this situation but only a mesenteric defect, which is present at birth. Strangulated mesenteric hernia is a rare cause of intestinal obstruction especially in the pediatric population. Between January 1996 and January 2006, four patients with intestinal obstruction were determined to have congenital mesenteric hernia at Chang Gung Children’s Hospital. Patients consisted of three boys and one girl, ranging in age from 2 years 5 months to 5 years 4 months. All patients presented with symptoms and signs indicative of intestinal obstruction. On physical examination, all patients appeared to be severely ill. A shock-like state was associated with necrosis of the strangulated bowel in two patients. A palpable abdominal mass was present in one patient with volvulus of small bowel. The plain abdominal radiography and the ultrasound scan also showed dilatation of the small bowel and excluded intussusception. All the patients underwent laparotomy after initial resuscitation. During laparotomy, three patients had incarceration of small bowel through the small mesenteric defect, and another one had volvulus with gangrenous bowel entrapped within a large mesenteric defect. The defects ranged in size are from 2 to 7 centimeters in diameter. Simple reduction of the incarcerated bowel with repair of the defect was performed in two patients, resection of gangrenous bowel and primary anastomosis was required in one patient, and resection with end ileostomy was needed in another one patient. There was no postoperative mortality in our patients. Conclusion: A mesenteric hernia is a rare cause of intestinal obstruction, especially in children. Open exploration is the only way to establish a clear anatomic diagnosis.     

儿童肠梗阻的诊断和鉴别诊断

肠梗阻是小儿外科常见的急腹症,病因复杂,病情多变.不但可引起肠管本身解剖和功能上的改变,还可导致全身性生理紊乱,处理不及时可能造成短肠综合征等并发症.及时诊断肠梗阻并明确原因,对抢救、逆转危重状态和减少并发症的发生有重要意义.     

The significance of biliary sludge in children with sickle cell disease

The prevalence of cholelithiasis was studied prospectively by abdominal ultrasound (US) examination in 305 children with sickle cell disease aged 1–18 years (mean 10.45). Gallstones were present in 60 children (19.7%); an additional 50 had biliary sludge only (16.4%). On follow-up of 35 of the 50 children with sludge, 23 (65.7%) had developed gallstones and 5 had already had a cholecystectomy. Five continued to have sludge on follow-up while 7 were reported to have no sludge. Children with US evidence of sludge should be followed up regularly by US, and those who develop gallstones should undergo elective cholecystectomy. For those with biliary sludge only, we recommend elective cholecystectomy if there are hepatobiliary symptoms. Accepted: 16 December 1996     

小儿先天性输尿管中段梗阻的诊疗对策

目的：分析小儿先天性输尿管中段梗阻的病因，诊断及治疗措施。方法：1990年1月－2001年10月收治先天性输尿管中段梗阻10例，年龄7－15岁。所有患儿术前均行相应的检查，包括B超、CT、IVP、MRI及逆行性输尿管造影检查。6例术前确诊，手术的治疗方式：管壁原因所致梗阻为切除病变段输尿管，行端端吻合术；管外原因所致梗阻为去除外在压迫原因，如无法去除则行输尿管离断后复位吻合术，其中2例例行Anderson-Hyne手术。结果：所有患儿均痊愈出院，未出现并发症。术中证实梗阻原因为下腔静脉压迫，迷生血管和束带压迫；输尿管内瓣膜及输尿管狭窄。结论：先天性输尿管中段梗阻少见，病因复杂，术前确诊有利于术者的术中应变性。     

Type I biliary atresia without extrahepatic biliary cyst

Currently, magnetic resonance cholangiography (MRC) is used for the differentiation of biliary atresia (BA) from other causes of infantile cholestasis. The authors present a case of type I BA without an extrahepatic biliary cyst in a 2-month-old girl. MRC clearly visualized the patency of the gallbladder, cystic duct, and hepatic ducts with disappearance of the common bile duct. Intraoperative cholangiography demonstrated a cloudy appearance of the intrahepatic bile ducts, confirming the diagnosis of type I BA. We believe that this is the first reported case of type I BA without an extrahepatic biliary cyst diagnosed by MRC.     

Upper clayx reno-vascular obstruction in children: Fraley's syndrome

Intrarenal vascular obstruction of the upper calyceal infundibulum has been distinguished by Fraley as an entity separate from an asymptomatic vascular impression. Clinically, this form of intrarenal vascular obstruction presents as nephralgia or hematuria. This infundibular obstruction is caused by normal renal vessels, usually the upper segmental artery: radiologically, the vessels produce a constant well-defined filling defect in the infundibulum at IVP, and, the upper calyx group is distended and shows delay in emptying. We found this condition in 14 children from 1968–1975. Control studies showed no progression in the radiological findings, even over a long period. In one of our cases angiography was indicated to exclude tumour or cyst; in another case, surgery was necessary. Complications are urinary tract infection or stone formation. Therefore, children with Fraley's syndrome require regular clinical examination.Presented at the 12th Meeting of the European Society of Pediatric Radiology, Stockholm, Sweden, May 19–22, 1976     

Adhesive small-bowel obstruction in children

Seventy-one episodes of adhesive small-bowel obstruction (ASBO) in 57 children were reviewed to define the relationship of treatment methods to clinical conditions and to examine the place of conservative treatment. Thirteen children received urgent operations on the day of admission because of signs of suspected strangulation or complete obstruction. The remaining 58 episodes underwent initial conservative treatment, which was successful in 35 while 23 required subsequent surgical intervention, an overall incidence of operation in 36 of the 71 (50.7%) episodes of ASBO. Seven episodes required bowel resection, strangulation was found in 3 (4.2%), and mortality occurred in 1 (1.7%) due to delayed operation. Patients with more than one previous successfully treated occurrence of ASBO were significantly more likely to respond to conservative treatment (71.4% vs. 54.4%, P <0.05). Those patients who had undergone laparotomies twice or more before the episode were more likely to be successfully treated by conservative treatment than those who underwent laparotomy only once before (80% vs. 52.4%, P <0.01). Whether or not the treatment of the previous occurrences of ASBO was surgical, we found that the initial treatment of any further episode could be conservative except when evidence of a complete or strangulated bowel obstruction developed.     

Kasai术后肝内胆管囊性扩张的诊治与预后

目的 总结胆管闭锁术后肝内胆管囊性扩张的诊治经验,分析其对患儿预后的影响.方法 1998年6月至2008年3月,对胆管闭锁行葛西手术的患儿进行追踪随访.通过超声检查,发现8例患儿存在肝内胆管扩张.其中3例再行MRI检查,6例再行CT平扫加增强检杳以明确诊断.此8例患儿术后均有不同程度胆管炎症状.5例行PTCD显示肝内胆管囊性扩张.3例患儿行囊肿与空肠胆支再吻合术,2例患儿仪行PTCD置管引流,2例患儿暂未予任何处理.结果 3例经手术治疗的患儿,术后黄疸消退或减轻.仅行PTCD的患儿肝内胆管扩张长期存在.结论 对反复发作的胆管炎应定期行超声检查,尽早发现肝内囊肿;对肝内胆管囊性扩张,无论其影像学分型如何,应根据其具体临床表现进行相应的积极治疗;PTCD无法长期放置,仅是暂时性的治疗,但对手术时寻找囊肿有指示作用,使手术时囊肿的定位相对容易;囊肿的大小、位置与门静脉的关系,压迫的时间、治疗是否及时有效都可影响患儿的预后.     

ERCP和MRCP在儿童胆胰疾病中的应用与评估

目的：探讨经内窥镜十二指肠孔头插管进行胰胆管造影（ERCP）和磁共振胰胆管造影（MRCP）在我国小儿胰胆疾病中应用的价值。方法：分析41例接受ERCP检查的患儿B超,CT,ERCP和MRCP的检查结果和ERCP的并发症,并和手术结果进行比较。结果：ERCP显示36例胆总管扩张,3例胆总管结石,1例反复发作的胰腺炎,胰管扩张,1例阴性;MRCP检查23例,胆管扩张20例,胆总管结石2例,阴性1例,ERCP的敏感性和特异性分别为95＝1％,97．4％,MRCP的敏感性和特异性为94．6％,94．8％,3例发生ERCP后并发症,主要是胰腺炎,发生率为4．6％,结论：ERCP和MRCP对小儿胰胆系统疾病的影像学诊断有很高的敏感性和特异性,可作为明确诊断的常规方法。     

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.     

Bleeding tendency in children with biliary atresia

Sixty-eight childrenwith biliary atresia (BA) were admitted to the Department of Pediatric Surgery, Kyushu University Faculty of Medicine and fukuoka Municipal Children's Hospital, from 1971 to 1986. In 11 (16.2%) there was a tendency toward bleeding associated with obstructive jaundice and breast feeding. In 4 of the 11 cases, vitamin K deficiency (VKD) was diagnosed by coagulation studies. The bleeding tendency improved after administrationn of vitamin K in all 4 cases; the other 7 were also suspected of having VKD. The usefulness of the Normotest [7] for the diagnosis of VKD and for mass screening of infantile hepatobiliary disorders, particularly BA, is discussed.     

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 resolution of extrahepatic biliary obstruction in a neonate

A patient with spontaneous resolution of complete extrahepatic biliary obstruction shortly after the neonatal period is described. Liver biopsy prior to resolution revealed widened portal tracts and extensive fibrosis; these changes normalized following resolution. The aetiology was not established but possibilities included a choledochal cyst, localized inflammation; for example, pancreatitis, a calculus of the common bile duct or a congenital membrane. Management of this patient would have been advanced if there were a paediatric size side-viewing duodenoscope which would have diagnostic and therapeutic potentials.     

胆道闭锁婴幼儿肝移植围术期液体管理

目的 分析胆道闭锁( biliary atresia,BA)婴幼儿肝移植围术期各阶段内环境的变化特点,并探讨相应的液体管理措施.方法 2006年9月至2011年8月我院行胆道闭锁婴幼儿肝脏移植手术24例,围术期连续监测患儿血流动力学,定时采血检测酸碱平衡、乳酸、电解质、血糖等指标,记录液体治疗措施.结果 患儿术中血pH值及碱剩余(BE值)于无肝期即刻下降至术毕接近正常,分别为(7.38±0.09)、(-1.44±0.64),术后开始升高、至术后12h达峰,此后逐渐下降至正常;乳酸于无肝期开始升高(3.31 -±0.8) mmol/L,新肝期30 min达峰,术后6h明显下降,术后12h完全降至正常;血清钾浓度(K+)于新肝期后出现显著下降(3.21±0.52) re mol/L;血清钙浓度(Ca2+)自无肝期出现明显下降(1.03±0.12) m mol/L;血清钠浓度(Na+)于新肝期后出现逐渐升高趋势(141.2±2.9)mmol/l;血糖(BG)于无肝期后明显高于术前水平(7.4±2.8) mmol/L.术后患儿电解质及血糖水平有所波动,但与术后即刻比较无明显变化.24例患儿均顺利完成手术,围术期未发生脱水及心肺事件.术后ICU死亡4例,其余患儿均顺利回到病房.结论 胆道闭锁婴幼儿肝移植围术期内环境呈现规律性变化.术中液体治疗原则与一般腹部大手术有所不同;补液速度相似,但是液体比例以胶体液为主,占液体总量的50 ％以上.术后即期液体治疗与普通外科手术相近.     

Duodenal duplication with bile duct obstruction and keratinaceous casts

We report an unusual case of duodenal duplication presenting in a newborn with duodenal and bile duct obstruction. The duplication and biliary tract were filled with keratinaceous casts. To our knowledge, such an association has not been previously reported; this case demonstrates the importance of confirming bile duct patency during operations to remove duodenal duplications.     

Anterior abdominal wall defects and biliary obstruction

Three infants with anterior abdominal wall defects (gastroschisis and exomphalos) who presented with obstructive jaundice secondary to biliary obstruction, are described. All three infants had abnormal biliary systems, with mechanical distortion of the biliary tree. Biliary obstruction secondary to structural biliary anomalies should be considered in patients with abdominal wall defects and cholestasis, as prolonged unrelieved biliary obstruction may lead to biliary cirrhosis and portal hypertension.