Pediatric liver transplantation for fibropolycystic liver disease

Fibropolycystic liver disease includes CHF, Caroli's syndrome, and Caroli's disease. Patients with Caroli's disease and Caroli's syndrome have an increased risk of recurrent cholangitis, intrahepatic calculi, biliary cirrhosis, and cholangiocarcinoma. The aim of this study was to examine the post-transplantation outcomes of children with fibropolycystic liver disease. Of the 158 children transplanted at Seoul National University Hospital, there were four patients with Caroli's syndrome, two patients with CHF, and one patient with Caroli's disease. One patient underwent combined liver/kidney transplantation. Associated renal manifestations included ARPKD in three children and nephronophthisis in one child. The indications for LT were recurrent cholangitis, decompensated cirrhosis, and refractory complications of portal hypertension. Both graft and patient survival rates were 100% at a median follow-up period of two yr after LT. Three children with growth failure achieved catch-up growth after LT. In three patients with ARPKD, mean serum creatinine levels increased from 0.53 mg/dL at the time of LT to 0.91 mg/dL at the last follow-up (p = 0.01). LT is an excellent option for children with complications from fibropolycystic liver disease. Renal function should be monitored cautiously after LT in the patients with ARPKD.     

Caroli's disease in children: Is it commonly misdiagnosed?

Aim: Caroli's disease is a simple form of intrahepatic bile duct ectasia. It can be complicated with the involvement of liver parenchyma and portal hypertension. Herein, the difficult management of delayed presentation of Caroli's disease is reported. Methods and results: We report on four different forms of clinical presentation of Caroli's disease: an infant with fulminant liver failure, a teenager with persistent biliary fistula, a boy with hypersplenism in the face of portal hypertension and a girl with variceal bleeding.

Conclusions: Caroli's disease must be included in the differential diagnosis of cystic lesions in the liver. Delayed diagnosis of Caroli's disease is difficult to manage and appropriate investigations are warranted before planning a surgical approach.     

Caroli's Disease: Scintigraphic and Radiographic Findings

Caroli's disease is a rare congenital anomaly of the liver. Since new diagnostic techniques for the evaluation of hepatic disease have been introduced, the number of reported cases of Caroli's disease has been gradually increasing. This report describes the case of a 13 year old girl with nausea, vomiting, fever and hepatomegaly. The diagnosis of Caroli's disease was established by ultrasonography and CT scan. The complications and management are discussed. The important points in management are to relieve symptoms and to conduct regular examinations by ultrasonography for the early detection of hepatobiliary carcinoma.     

Isolated Caroli's disease presenting as an exophytic mass in the liver

A case of isolated Caroli's disease in a 2-year-old female presented as an exophytic cystic mass of the liver. A palpable mass below the right costal margin and displacement of the right colon were the main points of clinical presentation. Preoperatively, sonographic signs of intraluminal vascular tracts were identified. At laparotomy, the mass was found at the inferior border of the right lobe of the liver next to the gallbladder and was easily removed by wedge excision. Intraoperative liver biopsies and histologic findings of the excised lesion confirmed the diagnosis. Accepted: 1 March 1996     

MR cholangiography in children with autosomal recessive polycystic kidney disease

Background. Magnetic resonance cholangiography (MRC) is a relatively new, non-invasive imaging technique of the biliary tree that has shown good correlation with endoscopic retrograde cholangiopancreatography. The liver manifestation of autosomal recessive polycystic kidney disease (ARPKD) is congenital hepatic fibrosis (CHF). CHF may be accompanied by Caroli's disease, which is characterised by a non-obstructive dilation of the intrahepatic bile ducts. Objective. A prospective study was conducted to determine the presence and extent of Caroli's disease in children with ARPKD. Materials and methods. Seven children with ARPKD aged from 3.0 to 10.1 years were examined. CHF was confirmed in all biopsied cases (5 of 7). All children had been followed by repeated abdominal US examinations for many years. The MR examination included a morphological imaging study using a T2-weighted turbo spin-echo sequence and a heavily T2-weighted inversion-recovery turbo spin-echo sequence with three-dimensional maximum intensity projection (MIP) reconstructions for MRC. Results. The diagnosis of Caroli's disease could be made in one case by US; in two other children Caroli's disease was suspected, but the differentiation from hepatic cysts was not possible. By MRC, Caroli's disease could be diagnosed in three of seven children. Furthermore, MRC with MIP reconstructions demonstrated the extent of the disease by showing the entire biliary tree from different angles. Conclusions. MRC is a valuable method to establish the diagnosis and demonstrate the extent of Caroli's disease. Received: 5 January 1998 Accepted: 9 December 1998     

Caroli病的影像学诊断及治疗

目的 探讨Caroli病的影像学特征及其治疗.方法 回顾性分析9例Caroli病临床及影像学表现.9例均有CT扫描,其中6例行MRI及MRCP检查.4例行肝叶切除治疗,1例行肝移植治疗,另外4例保守治疗.结果 ①Caroli病Ⅰ型4例,Ⅱ型5例;②典型的影像学特征为肝内胆管囊状扩张,并与胆管相连通,可见"中心点征"及"蝌蚪"征;③肝叶切除术及肝移植治疗效果较好.结论 MRI结合MRCP可对Caroli病准确诊断及分型.并对病变范围及程度进行准确评估.Caroli病的治疗措施是尽可能的手术切除病灶,无法切除者应考虑肝移植治疗.     

小儿临床活体肝部分移植术

目的探讨小儿临床活体部分移植术的技术。方法 １９７年６月３０日我科成功地进行了１例小儿活体肝部分移植术。供体男性,４０岁,系患儿父亲,行肝左外侧切除术。术中用Ｂ超确认肝血管走向,确定切肝界线,以超声刀和双极电凝器断肝,切肝过程不断阻肝脏血流,肝脏热缺血时间为零。受体为１０岁女童。体重２４ｋｇ,身高１２１ｃｍ,为先天性弥漫性肝内胆管囊性扩张症、复发性胆管炎、肝硬变。结果 供体无任何术后并发症,受体早     

Caroli's syndrome. Report of a case beginning in childhood with favorable course

Caroli's disease is a rare entity that is included in the fibropolycystic abnormalities of the bile ducts. Ultrasonographic patterns consist of evident dilatation of the bile ducts. Although it is thought to be a congenital disease, it usually presents in young adults and few cases have been reported in children. We present the case of a 10-year-old boy with Caroli's syndrome (Caroli's disease, congenital hepatic fibrosis and polycystic renal disease). Evolution was favorable.     

In-flight esophageal variceal bleeding en route for liver transplantation: a case report and review of the literature

Esophageal variceal bleeding owing to portal hypertension is a potential threat in pediatric patients awaiting liver transplantation. We report a case of a three-year-old boy with severe congenital hepatic fibrosis, Caroli's disease, and portal hypertension who developed a life-threatening variceal hemorrhage for the first time in his life during commercial air transport to a liver transplantation center. Factors precipitating variceal bleeding are discussed, particularly those resulting from changes in altitude. It is recommended that prophylactic treatment for variceal bleeding be at least considered in children awaiting liver transplantation prior to prolonged air transportation. Because of differences in capabilities of treatment providers in various locales, and because there are as yet no conclusive data, the treatment of choice must be decided on an individual basis. Equipment for treating in-flight hemorrhage should be available.     

磁共振成像在儿童肝豆状核变性预后判断中的意义

目的探讨磁共振成像（MRI）在儿童肝豆状核变性（hepatolenticular degeneration,HLD）预后判断中的意义。方法对临床确诊为HLD的70例患儿应用MRI进行脑及肝常规序列扫描。其中25例予MRI动态观察,结合临床资料进行综合分析。结果70例患儿中48例显示脑或／和肝异常MRI。25例动态观察患者中12例MRI显示脑和肝异常信号,10例脑受累,3例肝受累。青霉胺剂量不足、初治年龄〉15岁或治疗前病程〉5年者,MRI病灶严重且改善缓慢或无变化。停药2个月以上MRI病灶即可重现,并伴有异常的神经系统症状和体征,但肝型患者可无临床症状和肝功能异常。动态MRI显示,治疗合理组临床及MRI病灶缓解率明显高于治疗不合理组;25例患儿经合理治疗1年以上临床及MRI逐渐好转直至消失,并都能正常学习与工作,13例缓解迁延及11例复发患者均存在治疗不合理因素,且MRI病灶严重,其中12／14例患者不能学习或工作。结论肝豆状核变性经合理治疗后,肝及脑部MRI异常信号可随临床改善而逐渐消失。治疗不合理者MRI病灶严重、改善缓慢、无变化或重现。MRI检查有助于肝豆状核变性预后的判断。     

Radionuclide scintigraphy in Caroli's disease

Congenital cystic dilatation of the intrahepatic bile ducts involving the major intrahepatic radicles was first described by Caroli and hence named as Caroli's disease. We present here a case in which the only symptom was intermittent pain in the abdomen for last one-and-a-half-year and a radionuclide scan done effectively, not only diagnosed the disease but also the associated cholangitis.     

Interposition of the gallbladder — or the absent common hepatic duct and cystic duct

Interposition of the gallbladder is a rare anomaly, but its diagnosis is important since it represents a surgically correctable cause of jaundice. The patients present with jaundice, abdominal pain and sometimes an enlarged gallbladder. Radiological diagnosis may be difficult since the condition may be mistaken for a choledochal cyst, hydrops of the gallbladder or Caroli's disease. The ultrasound, cholangiogram and surgical findings of dilated intrahepatic ducts adjacent to a normal or enlarged gallbladder with no dilatation of the common bile duct are presented in two children with this condition.     

Caroli syndrome

We describe a case of Caroli syndrome (Caroli's disease and congenital hepatic fibrosis) in a 10-year-old boy with bilobar involvement and numerous renal cysts. Liver transplantation offers the only hope for such patients. Accepted: 17 May 1999     

小儿肝移植13例经验回顾

目的 总结小儿肝脏移植13例的经验及教训.方法 从2001年9月至2007年8月对13例终末期肝病患儿实施了肝移植术,其中先天性胆道闭锁7例,先大性肝纤维化3例,肝豆状核变性1例,Caroli病1例.肝脏血管内皮肉瘤1例,年龄5个月至12岁.供体及受体在门诊进行367个月随访并收集数据,对受体存活期与手术方式、术者经验、患儿年龄及原发病、家庭经济状况、父母教育背景等因素的关系进行分析.结果 本组13例患儿全部顺利完成肝移植手术.不同手术方式的结果 :亲体肝移植4例,供体随访6～59个月,均无并发症,健康生活;4例受体,1例于手术后15个月死于急性坏死性肠炎.另外3例无并发症健康生活,其中1例手术后25个月免疫耐受.尸体肝移植9例,目前存活4例,已死亡4例;另外1例手术后14个月因慢性排斥反应行二次肝脏移植手术.不同手术时间的结果 :2001年1月至2003年1I)月间手术6例.已死亡5例,2年生存率为33.3%;2004年4月至2007年4月间开展手术7例,已死亡1例.存活6例,2年牛存率为57.1%.结论肝移植是治疗小儿终末期肝病的有效方法 ,随着经验的积累,手术并发症和病死率有大幅度降低的趋势.医生和护理人员的经验,患儿的病情,家庭的经济实力,家庭主要成员的心理承受力、挽救孩子生命的动机、坚定程度和毅力,医护人员和家长的密切配合是影响肝移植预后的因素.     

Retinitis pigmentosa, terminal renal insufficiency and Caroli syndrome: new associations with Opitz trigonocephaly syndrome

We report on a new patient with Opitz trigonocephaly syndrome. In addition to the findings typical of this mental retardation syndrome, the present patient has retinitis pigmentosa, Caroli's syndrome and renal failure, which is undergoing hemodialysis. This association is never observed before in patients with Opitz trigonocephaly syndrome. This case demonstrate, that with increased survival of patients with mental retardation syndromes, the phenotypes possible are modified.     

Resolution of chronic hepatic sequestration in a patient with homozygous sickle cell disease receiving hydroxyurea

Hepatic sequestration is an uncommon complication in patients with homozygous sickle cell disease. Although transfusion therapy has been effective for the acute condition, no definitive treatment of chronic hepatic sequestration has been identified. We describe a 17-year-old male patient with hemoglobin SS and chronic hepatic sequestration who was treated with long-term (60 months) hydroxyurea. After 36 months of HU therapy, the patient had both an excellent hematologic response and a resolution of hepatic sequestration, as evidenced by disappearance of clinical hepatomegaly, normalization of liver volume on serial computed tomography scans, as well as decreased sinusoidal dilatation and congestion and red blood cell sickling on liver biopsy. The findings in this case suggest that hydroxyurea may benefit patients who have unusual complications of sickle cell disease, such as chronic erythrocyte sickling in the liver.     

Primary hepatic Burkitt lymphoma

Primary hepatic lymphomas are exceedingly rare in children, with less than a dozen cases described to date. The authors present an 8.5-year-old boy with Burkitt lymphoma of the liver who had isolated multifocal liver lesions that exhibited a multilayered progressive enhancing pattern on MRI. Diagnosis was achieved after laparotomy and incisional biopsy that failed to detect disease outside the liver. The patient received short, intensive multiagent chemotherapy. He is currently well 22 months after the end of chemotherapy. This case illustrates that clinical judgment in conjunction with unusual imaging findings may contribute to the diagnosis of common tumors in rare locations.     

Neonatal hepatitis: a follow-up study

Fifty-six patients with moderate to severe neonatal hepatitis were followed for 12 to 78 months. Two died from causes other than hepatitis itself and were free from liver disease at the time of death. Of the remaining 54 patients, seven died of hepatitis, two are living with chronic liver disease and psychomotor retardation, and 45 are living without liver disease. High peak bilirubin levels and liver histologic findings of periportal fibrosis, moderate to severe portal inflammation, and/or diffuse giant cell transformation appear to be major factors predictive for poor outcome. Cytomegalovirus (CMV) infection was a common associated infection. Evidence of CMV infection was found in 22 (49%) of the 45 patients studied. Three of them died, and one is still living with cirrhosis of the liver. Metabolic disorders such as alpha-1-antitrypsin deficiency, galactosemia, and aminoaciduria and/or aminoacidemia were carefully screened but were not found in these cases. A fatal case had a sibling who had died of a similar disease course. Chinese infants may have metabolic and familial cholestasis diseases requiring further investigation.     

Frequency of urinary tract infection in pediatric liver transplantation candidates

An increased frequency of infections has been reported in patients with chronic liver disease. The tendency of patients in this population to acquire UTI is not completely understood. We aimed at investigating the incidence of UTI in children with cirrhosis, before liver transplantation. Twenty-six children (9 girls, 17 boys; mean age, 7.66 +/- 5.73 yr) with chronic liver disease who had undergone liver transplantation between 2002 and 2004 were included. On admission for liver transplantation, patients were examined for presence of UTI. Serum biochemistry, complete blood cell count, urinalysis and culture, glomerular filtration rate, and abdominal ultrasonography were performed prior to liver transplantation. Ten of 26 patients (38.5%) were found to have symptomatic UTI. Urine cultures revealed E. coli in five (50%), Klebsiella pneumoniae in three (30%), Enterococcus faecalis in one (10%), and Enterobacter aeruginosa in one (10%) patient(s), respectively, as etiologic factors. The etiologies of chronic liver disease in our patients with UTI were BA in five, PFIC in three, Wilson's disease in one, and alpha-1 antitrypsin deficiency in one patient. We found a significantly greater number of UTIs in patients with biliary atresia than in those without biliary atresia (p < 0.05). The mean age of the patients with UTI was 2.75 +/- 3.49 yr, which was significantly lower than in those without UTI (9.75 +/- 4.86 yr, p < 0.05). Levels for white blood cells, thrombocytes, ALT, and alkaline phosphatase were significantly higher in patients with UTI than in those without UTI. There were no significant differences between the groups with regard to serum albumin, bilirubin, AST, GGT, BUN, or creatinine levels, glomerular filtration rate, duration of disease, and PELD scores. In patients with bacteriuria, renal USG revealed normal findings in all, but except one patient who had pelvicalyceal dilatation. Scintigraphic findings demonstrated acute pyelonephritis in six (60%) patients with UTI. VCUG demonstrated vesicoureteral reflux in two patients. In conclusion, symptomatic UTI is common in children with cirrhosis. It occurs more frequently in patients with biliary atresia than it does in patients with other types of chronic liver disease. In febrile children with chronic liver disease, UTI should be considered in the differential diagnosis.     

Emotional and behavioral problems after pediatric liver transplantation: a quantitative assessment

Several uncertainties regarding psychological problems in children who underwent liver transplantation and the need to differentiate these disturbances from those related to the underlying previous chronic liver disease itself exist. This background triggered the present pilot study to investigate, using quantitative assessment methods, the incidence and the type of emotional and behavioral disturbances after liver transplantation. Sixteen liver transplant recipients (aged 5.7-14.4 yr) and 12 age-matched controls with stable chronic liver disease were assessed through the parent report form of Child Behavior Checklist/ 4-18. The mean time elapsed since transplantation was 8.1 yr. No patient or family had received psychological support during chronic liver disease or at any phase of the transplantation process. Transplanted children scored within borderline range for Internalizing and Total Behavioral Problems and within pathological range for Competences, except for the Activity Scale. Transplanted children showed more Total Behavioral (p = 0.005) and Externalizing Problems (p = 0.0005) than controls. Both groups scored within the pathological range for Total Competences with no significant differences between the two groups. Our findings suggest that in the absence of support programs a psychological risk does exist for a long period of time, after transplantation. Regarding Total Behavioral Problems and Externalizing Problems, this risk is higher than in children with chronic liver disease.