Redo surgery for biliary atresia

The Kasai redo surgery is important for treating biliary atresia. In the era of liver transplantation (LTx), pediatric surgeons must accurately select patients for redo surgery and ensure that potential LTx can be performed later. Although optimal timing for redo varies among cases, appropriate timing is essential. We reviewed the significance, optimal timing, operative procedures, and indications of Kasai redo surgery. Between 1989 and 2011, 2,630 patients were registered in the Japanese Biliary Atresia Registry (JBAR), and the data collected from JBAR regarding Kasai redo surgery were analyzed. Patients were divided into two groups, Group 1 (1989–1999, n = 1,423) and Group 2 (2000–2011, n = 1,207). The redo incidence significantly reduced in Group 2. Although no significant difference was found in the native liver jaundice-free survival rates between the two groups, the overall survival rate at initial registry was significantly higher in Group 2. This may be because of the limited number of patients selected for redo and increased availability of early LTx. Patients who achieved sufficient bile drainage following the initial Kasai surgery but developed sudden bile flow cessation were the best candidates for Kasai redo surgery; it should be performed only once for this subset.     

Laparoscopic surgery for biliary atresia and choledochal cyst

Minimally invasive surgery in children has evolved to the extent that complex procedures can be performed with safety and outcome comparable with open surgery, with the advantage of minimal scarring. Here we describe the latest laparoscopic techniques used by us at the Juntendo University Hospital, Japan, for treating biliary atresia and choledochal malformation, with presentation of our postoperative management and discussion of preliminary outcomes.     

胆道闭锁Kasai术后自体肝生存良好患儿的营养状况调查

目的回顾性调查胆道闭锁行肝门空肠吻合术(Kasai术)后自体肝生存良好患儿的营养状况,为制定个体化营养方案提供依据。方法收集46例胆道闭锁Kasai手术后1年且自体肝生存良好患儿的临床资料,包括年龄、身高/身长、体重、血清脂溶性维生素(A、D、E、K)水平。以WHO生长曲线软件分别计算患儿年龄别身高Z评分(height-for-age,HAZ)、年龄别体重Z评分(weight-for-age,WAZ)、身高别体重Z评分(weight-for-height,WHZ);以正常参考值为标准,计算营养不良和维生素缺乏的比例。结果46例患儿中男童26例,女童20例,平均年龄(14.5±0.8)个月。患儿HAZ评分为(-0.14±0.86),WAZ评分为(0.21±0.81),WHZ评分为(0.62±1.80),其中生长迟缓2例(4.3%),无低体重及消瘦患儿。25例行脂溶性维生素检查的患儿,其临床和实验室指标与46例自体肝生存良好的患儿均无明显差异(P>0.05)。25例患儿维生素A、D、E、K的水平分别为(290.61±80.26)ng/mL、(30.88±16.15)ng/mL、(7.69±2.77)μg/mL和(1.34±0.98)ng/mL。其中维生素A缺乏4例(16%),维生素D缺乏5例(20%),维生素E缺乏1例(4%),无一例维生素K缺乏者;至少有一种维生素缺乏者有7例(28%)。结论胆道闭锁行Kasai术后1年,自体肝生存良好的患儿营养状况良好;脂溶性维生素A、D缺乏较为常见,应在术后注意监测并补充。     

Screening for biliary atresia

Early diagnosis followed by proper KP is essential for the improvement of long-term prognosis for patients with BA. It is increasingly accepted that KP at ≤?30 days of age significantly improves native liver survival rate. Published analyses in English and Japanese indicate that screening by SCC and DB/CB is potentially feasible. Screening with SCC has been implemented in Tochigi Prefecture, Japan, since 1994. The concept of SCC was introduced from Japan to Taiwan and resulted in nationwide screening with SCC for the first time in Taiwan in 2004, followed by Japan in 2012. Home-based screening using SCC is easy and cost-effective; however, it may cause some difficulties for families in case of stools with intermediate colors. Laboratory-based screening using DB/CB may detect the suspected cases earlier, resulting in an increase in the number of patients with BA who undergo KP at ≤?30 days of age; however, the recall rate is 1% and may be beyond an acceptable range. Further studies are needed to assess the feasibility and cost-effectiveness of both home-based (SCC) and laboratory-based (DB/CB) screening for BA.     

Familial extrahepatic biliary atresia

Only a small number of cases with familial extrahepatic biliary atresia, suggesting a recessive autosomal inheritance, have been reported. However, the cases reported in twins are consistent with an acquired disease. Here the case of two brothers with familial atresia of the extrahepatic biliary system is studied. Diagnosis was confirmed by laparotomy and histologic study of biliary fibrous remnants. In the present cases parental consanguinity might suggest a recessive autosomal inheritance. However, clustering of cases in a family might also suggest a common exposure to responsible factors not so far identified.     

Antenatal presentation of biliary atresia

OBJECTIVES: To investigate the incidence, presence of associated anomalies, type of malformation, and natural history of children with biliary atresia (BA) who presented antenatally.Study design The database of 194 infants diagnosed with BA at our tertiary referral unit between 1991 and 2002 was reviewed for infants in whom routine antenatal ultrasound had revealed biliary cystic malformations (BCMs). A retrospective analysis of their medical records was undertaken. RESULTS: Nine infants (six girls) with BCM were identified (4.6% of total referred patients with BA); all were born after 37 weeks' gestational age and presented to us at a median age of 4 weeks (range, 1-14 weeks). The presence of the BCM was confirmed on postnatal ultrasonography. Median age at corrective surgery was 5 weeks (range, 2-16 weeks). At surgery, six patients had type III BA, including one with the biliary atresia-splenic malformation syndrome; two had type II BA; and one had type I BA. Postoperatively, the infants were followed for a median of 2 years (range, 6 months to 11 years). All infants successfully cleared their jaundice. CONCLUSIONS: Improved techniques in routine antenatal ultrasonography can allow early recognition of BCM. This study suggests that BCM noted antenatally may represent early presentation of both biliary atresia-splenic malformation and nonsyndromic BA.     

Transitional care of biliary atresia

Worldwide native liver survival (NLS) for young adults (>20 years) with biliary atresia varies between 14% and 44% with the majority of patients developing complications in adulthood. Cholangitis and portal hypertension with variceal bleeding are the most common complications and development of these during adolescence associated with the need for liver transplantation during adulthood. Adult listing criteria, typically developed on the background of adult liver disease might not be applicable to this patient population and leads to longer waiting time and risk of deterioration of their medical condition.Current data on growth and puberty in young people with biliary atresia surviving with native liver are rare. Pregnancy has been associated with serious complications in particular for those patients with advanced liver disease and, close follow up by specialist teams recommended. The long-term effect of having a chronic liver disease such as biliary atresia on neuro-cognitive and pubertal development has not been sufficiently explored to date despite reports of a high prevalence of additional educational needs in this cohort. In addition, patients and parents report inferior health related quality of life compared to healthy peers and similar to that of children post liver transplantation.Moving on from paediatric to adult services is challenging for young people and their parents and adult health professionals might not be familiar with the condition and complications. Young people deserve to be looked after by specialist, multidisciplinary services who provide holistic care and address their psychosocial needs in addition to the medical needs.     

Potential etiologies of biliary atresia

Biliary atresia is the most common cause of neonatal cholestasis and the leading indication for pediatric liver transplantation worldwide. The disease is caused by a progressive inflammatory and fibrosing obliteration of the extrahepatic bile ducts. Although the cause of this obstruction is largely unknown, patient-based studies have identified environmental and genetic factors that may interact and orchestrate disease pathogenesis. Chief among these factors are infectious and immunologic processes. While infectious agents have varied in different patient populations, studies of liver specimens at different phases of disease point to a pro-inflammatory commitment of lymphocytes at the time of diagnosis, and to their potential role in regulating bile duct obstruction. A review of these studies is the focus of this article.     

Current management of biliary atresia

Extra-hepatic biliary atresia occurs in approximately 1:15,000 live births leading to about 50 new cases/year in the UK. Presentation is with prolonged jaundice, usually in a term baby who develops signs of obstructive jaundice. Management has been improved by public and professional education to encourage early referral and diagnosis to facilitate initial surgery before 8 weeks of age. Surgical management is complementary and includes an attempt to restore biliary flow (the Kasai portoenterostomy) and liver transplantation if necessary. Medical management consists of antibiotics, ursodeoxycholic acid to encourage bile flow, fat soluble vitamin supplementation and nutritional support. Centralising surgery to specialised centres has improved survival of this potentially fatal disease to over 90% in the UK. Over half of infants undergoing portoenterostomy will clear the jaundice and have a greater than 80% chance of a good quality of life, reaching adolescence without transplantation. For those children developing intractable complications of cirrhosis and portal hypertension, liver transplantation provides a 90% chance of achieving normal life.     

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.     

胆道闭锁患儿肝组织细胞因子及相关基因表达的研究

目的 研究胆道闭锁(BA)患儿肝脏组织中上皮-间充质转化过程中特异性细胞因子的表达及胚胎期发育相关基因的表达激活.旨在探讨BA肝脏纤维化等系列改变的分子机制.方法 取经手术证实为BA患儿的8例肝活检组织(年龄2～3个月),以无消化道疾病尸检婴儿8例肝脏组织做正常对照(年龄0～2个月),RT-PCR半定量法检测胆管上皮细胞特异性因子CK19,纤维化早期指标Ⅰ型胶原蛋白COL1A1,发育相关基因Notch基因受体HES1,TGF-β及其阳性信号分子Smad3的表达水平变化.结果 BA患儿肝脏组织中CK-19,COL1A1、HES1、TGF-β及Smad3等基因表达水平均明显提高,实验组及对照组差异显著.结论 BA患儿肝脏组织胶原组织增生,同时有胆管上皮细胞增生,胚胎期肝脏组织发育相关基因Notch信号通路系统重新激活,TGF-β及其阳性信号分子Smad3在这一过程中发挥作用.     

Hypertrophic osteoarthropathy in biliary atresia

This case report describes a child with biliary atresia and hypertrophic osteoarthropathy (HO), the second such case report of HO in a child with biliary atresia. Suggested pathogenetic mechanisms are discussed. Surgical intervention in the treatment of biliary atresia has clearly increased survival in affected children. The relationship between surgical intervention, either successful or unsuccessful, and hepatic HO remains speculative.     

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胆道闭锁是一种肝内外胆管出现阻塞并可导致淤胆性肝硬化而最终发生肝功能衰竭的疾患,是小儿外科领域中最重要的消化外科疾病之一,也是小儿肝移植中最常见的适应证.