A histopathological study of liver and biliary remnants in the long-term survivors (>10 years) of cases of biliary atresia.

CONTEXT: Biliary atresia (BA) is a destructive process affecting both extra- and intra-hepatic bile ducts leading to fibrosis and obliteration of the biliary tree and cirrhosis usually within 2 years. Factors influencing the outcome of portoenterostomy (PE) have not been clearly defined. AIMS: Our aim was to identify children with no evidence of liver disease 10 years or more after PE and to compare the pathology of liver and biliary remnants in this group with those associated with poor outcome. SETTINGS AND DESIGN: Prospective observational study. MATERIALS AND METHODS: Wedge biopsies of liver and portal remnants, taken at the time of PE, where available, were reviewed. The parameters studied were - presence of large bile ducts (>150 µ), degree of fibrosis and bile duct proliferation (BDP), presence of ductal plate malformation (DPM) and age at operation. STATISTICAL ANALYSIS USED: Fisher's exact test with Freeman Halton extension for univariate analysis and Logistic regression analysis as multivariate analysis. RESULTS: Of 68 cases operated between 1995 and 2001, 14 patients survived >10 years and 54 were associated with poor outcome. Large ducts were significantly more in survivors (70% vs. 26%, P = 0.02). DPM was not seen in any of the survivors and was present in 24% of poor outcome group. Fibrosis and BDP were also significantly less among the survivors (P < 0.001, P = 0.03, respectively). The mean ages at operation in the two groups were 66.8 and 89.6 days, respectively. CONCLUSION: From this study, we feel that lower degree of fibrosis and BDP, absence of DPM, presence of large ducts and younger age at operation were associated with better long-term outcome. Of these, degree of fibrosis was the most significant factor.

En plaque meningioma with angioinvasion.

En plaque meningioma is a rare type of meningioma characterized by infiltrative nature, sheet-like growth and at times invading the bone. We report here a case of en plaque meningioma with typical grade I histomorphology along with unusual feature of angioinvasion. The patient was a 55-year-old man presenting with headache and painful proptosis of right eye. Imaging modalities revealed an en -plaque meningioma extending into the right sylvian fissure, with thickening of right temporal calvarium, greater wing of sphenoid and extension into the orbit. Magnetic resonance angiography showed medial displacement of right middle cerebral artery. The tumor was removed from the sylvian fissure and right temporal convexity. However, only subtotal removal of the intraorbital part was possible. Histology showed a meningothelial meningioma with low tumor cell proliferation, but infiltration into the bone, skeletal muscle and angioinvasion. Recognition of meningiomas en plaque is useful, as these tumors are difficult to resect completely, and are more prone to undergo recurrence or malignant change. In addition, angioinvasion seen in this tumor may have additional prognostic significance.