Liver cirrhosis in infancy can be secondary to various etiologies such as biliary atresia, familial cholestatic and metabolic disorders. Wolman's disease (WD) is a lysosomal storage disorder caused by the absence of lysosomal acid lipase enzyme activity and a significant association with infantile cholestasis and cirrhosis. We encountered an infant presenting with advanced cirrhosis and decompensation having splenomegaly for which the underlying etiology was found to be WD and the diagnostic clue came from abdominal X-ray showing bilateral adrenal calcifications. The diagnosis was confirmed by genetic analysis. The outcome was poor and died before 6 months of age without enzyme replacement therapy or hematopoietic stem cell transplantation.

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Purpose:We studied the prevalence of celiac trunk and its anatomical variations on diagnostic computed tomography angiography (CTA) studies and have proposed a new classification to define the celiac artery (CA) variations based on embryology. Material and methods:We retrospectively assessed the celiac trunk variations in 1113 patients who came to our depart ment for diagnostic CTA for liver and renal donor workup. The patient data were acquired from the Picture Archiving and Communication System of our institutions. We analysed the celiac trunk's origin and branching pattern, including the superior mesenteric artery (SMA) and inferior phrenic artery (IPA). Results:We evaluated the CTA studies of 1050 patients. A normal trifurcation pattern, the most common type, was observed in 39% of cases. Variation with CA + left IPA was the most common subtype. Other variations noted in the study and their incidences are listed in the table below. We attempted to propose a new classification based on embryo logy, which comprises 6 main types and their subtypes. We also analysed previous studies from the literature, including cadaveric, postmortem, CTA, and digital subtraction angiography studies and compared them with the present study. Conclusions:Because variations of CA classifications reported to date do not encompass all CA branching pattern variants, we have proposed a new classification that incorporates most of the variants. We reiterate the clinical im portance of anatomical variants of CA, IPA, and SMA in surgical and interventional radiology procedures.

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