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OBJECTIVE: To report the unusual association between neonatal hepatitis and polysplenia syndrome.METHODS: Clinical, biochemical, histopathological, surgical and image analysis methods were used.RESULTS: The 36 days old patient presented hyperbilirubinemia with increase of direct reacting bilirubin and high alkaline phosphatase. The physical examination evidenced jaundice and hepatomegaly. The chest X-ray showed situs inversus and dextrocardia. Abdominal ultrasonography presented an increase in liver volume, mainly at the right lobe. There were two cystic lesions at the liver with absence of intrahepatic biliary tract dilatation. Absence of splenomegaly. Biliary scintigraphy didn't show any excretion of radioisotope to the duodenum while transoperative cholangiography presented contrast medium flowing to the duodenum lumen. During laparotomy it was possible to observe polysplenia, abdominal aorta to the right of the lower vena cava, gallbladder at the left lobe of the liver, appendix on the left side of the abdomen and sigmoid colon on the right side. The hepatic wedge biopsy was compatible with idiopathic neonatal hepatitis.CONCLUSION: The finding of polysplenia syndrome in patients with neonatal cholestasis doesn't necessarily indicate the recurrent presence of extrahepatic biliary atresia.

The histopathology of the liver is fundamental for the differential diagnosis between intra- and extrahepatic causes of neonatal cholestasis. However, histopathological findings may overlap and there is disagreement among authors concerning those which could discriminate between intra- and extrahepatic cholestasis. Forty-six liver biopsies (35 wedge biopsies and 11 percutaneous biopsies) and one specimen from a postmortem examination, all from patients hospitalized for neonatal cholestasis in the Pediatrics Service of Hospital de Clínicas de Porto Alegre, were prospectively studied using a specially designed histopathological protocol. At least 4 of 5 different stains were used, and 46 hepatic histopathological variables related to the differential diagnosis of neonatal cholestasis were studied. The findings were scored for severity on a scale from 0 to 4. Sections which showed less than 3 portal spaces were excluded from the study. Sections were examined by a pathologist who was unaware of the final diagnosis of each case. Bile tract permeability was defined by scintigraphy of the bile ducts and operative cholangiography. The F test and discriminant analysis were used as statistical methods for the study of the hepatic histopathological variables. The chi-square method with Yates correction was used to relate the age of the patients on the date of the histopathological study to the discriminatory variables between intra- and extrahepatic cholestasis selected by the discriminant function test. The most valuable hepatic histopathological variables for the discrimination between intra- and extrahepatic cholestasis, in decreasing order of importance, were periportal ductal proliferation, portal ductal proliferation, portal expansion, cholestasis in neoductules, foci of myeloid metaplasia, and portal-portal bridges. The only variable which pointed to the diagnosis of intrahepatic cholestasis was myeloid metaplasia. Due to the small number of patients who were younger than 60 days on the date of the histopathological study (N = 6), no variable discriminated between intra- and extrahepatic cholestasis before the age of 2 months and all of them, except for the portal expansion, were discriminatory after this age. In infants with cholestasis, foci of myeloid metaplasia, whenever present in the liver biopsy, suggested intrahepatic cholestasis. Periportal ductal proliferation, portal ductal proliferation, portal expansion, cholestasis in neoductules, portal cholestasis and portal-portal bridges suggested extrahepatic obstructive cholestasis.

Objective An efficient treatment of extrahepatic biliary atresia demands that the diagnostic differentiation between intra- and extrahepatic neonatal cholestasis be performed by the eighth week of life. This study aimed at evaluate the age of the patients admitted to a general hospital for differential diagnosis of cholestatic jaundice. Methods Forty nine children from the Pediatric Service at Hospital de Clinicas, in Porto Alegre, have been studied between 1984 and 1991, according to the protocol for diagnostic elucidation followed by this hospital, which includes biliary tract scintigraphy with Tc-99m DISIDA and, depending on its results, an wedge or percutaneous liver biopsy. The ages of the children have been compared on the occasion of the procedures. Twenty six cases have been studied retrospectively and 23, prospectively. Results Both the patients with intrahepatic and extrahepatic cholestasis underwent scintigraphy, on average at over eight weeks (age 77.94 +/- 42.98 days) and the histopathological study of the liver was performed approximately two weeks after scintigraphy. Only six patients (12.8% of the 47 cases) underwent the liver biopsy before the first eighth week of life. Conclusions A delay was observed in referring patients for differential diagnosis of neonatal cholestasis and the performance of tests. The need of hospitalization in order to conduct these procedures delays even further this diagnosis, which should be concluded by the eighth week of life.

OBJECTIVE To analyze the evolution of pediatric patients chosen for hepatic transplantation. METHODS A review was made of the clinical charts of the first 65 children and adolescents with chronic liver disease, aged 5 months to 19 years (X = 6.8%), chosen for liver transplantation during the period of August 1994 to March 1996. Data refer to the patients' demographic characteristics, etiology of their liver disease, their psychosocial situation and of their parents, and their clinical and laboratorial evaluation. According to the severity of the disease, patients were classified as active (waiting for a donor), in evaluation, inactive (compensated liver disease), and excluded for psychosocial or medical conditions, or because of bad indication. RESULTS Eight patients (12%) received transplantation, and one of them died. Seven (11%) died when in evaluation or waiting for a donor. Ten patients (15%) were excluded from the waiting list: 6 for social problems, and 4 for medical problems. No patient was excluded for bad indication. Six patients are in the active list, waiting for donor. The other 23 patients (35%) are in evaluation, and 11 (17%) are classified as inactive in the waiting list. CONCLUSIONS Eleven patients (17%) were not operated on due to the advanced stage of the liver disease. We emphasize the necessity of organ donation, and the early contact of the patients with a reference center.