Differentiating biliary atresia from other causes of infantile cholestasis: An appraisal of the histomorphological changes on liver biopsy.

Background: Cholestatic disorders are a significant cause of morbidity and mortality in infants. Characterization of these disorders and differentiating biliary atresia (BA) from other causes of intrahepatic cholestasis is an age-old problem. Objectives: To study the spectrum of different infantile cholestatic disorders in our population, to differentiate BA from other causes of neonatal cholestasis (NC) on a liver biopsy, and validation of the available scoring system for the characterization of these disorders. Materials and Methods: This is an observational cross-sectional study performed over a period of 3 years between 2018 and 2021, done on neonates and infants presenting with cholestatic jaundice. The changes on liver biopsy were evaluated by different histological parameters and available scoring systems to differentiate BA from non-BA causes. Correlation with clinical, biochemical, and imaging findings was done in all cases. Results: This study included 87 cases of NC, of which BA comprised 28 cases (32%), whereas idiopathic neonatal hepatitis (INH) comprised only 12 cases (14%). Portal neutrophilic inflammation (P = 0.000053), ductal cholestasis (P < 0.001), neoductular bile plugs (P < 0.001) and bile ductular proliferation (P < 0.0001) were significantly more in BA, whereas lobular lymphocytic inflammation (P = 0.001) and giant cell transformation of hepatocytes (P = 0.0024) were more frequent in the non-BA group. Using the Lee and Looi scoring system, a histologic score ≥7 was helpful in identifying BA with 85.7% sensitivity, 92.6% specificity, and 90.6% accuracy. Conclusion: BA is the commonest cause of NC in neonates, whereas the frequency of INH is declining. Detailed histomorphologic analysis of liver biopsy, aided with IHC, is the cornerstone for the diagnosis of these disorders.

Recurrent Giant Cell Fibroblastoma in an Infant: A Diagnostic Challenge.

Background: Giant cell fibroblastoma (GCF) shows a wide spectrum of morphological patterns which may lead to a misdiagnosis of sarcoma. Case Report: This 14- month- old baby was referred to us for recurrent left scrotal embryonal rhabdomyosarcoma (ERMS), first diagnosed at 8 months, status post chemotherapy. Review of previous histology, cytology (with frequent multinucleated floret type giant cells but without cross striations) and immunohistochemistry resulted in the change of diagnosis to GCF. It was re-excised, recurred at 20 months of age, and was again re-excised. The morphology was the same in both recurrences as the original. Conclusion: Despite chemotherapy, the histology of multiple recurrences for GCF remained the same as the original. Cytologically, identification of the multinucleated floret like giant cells without cross striations was helpful in differentiating this lesion from embryonal rhabdomyosarcoma.

Tunical Gliomatosis: An Uncommon Histological Entity.

Background Gliomatosis peritonei (GP) is characterized by widespread implants of mature glial tissue in the peritoneum. It is most often associated with immature or mature ovarian teratomas. Case Report: A 6-month old infant developed isolated gliomatosis involving the tunica vaginalis following excision of an immature sacrococcygeal teratoma in the neonatal period. Conclusion: Tunica vaginalis gliomatosis can occur in the setting of a retroperitoneal extragonadal congenital immature teratoma.