Case Report of Gastrointestinal Bleeding in an Adult with Chronic Visceral Acid Sphingomyelinase Deficiency
Figure 1
CT of chest (a), abdomen (b), and abdomen/pelvis (c). (a) Chest CT showing centrilobular emphysema and signs of interstitial lung disease. There are bilateral basal inter- and intralobular septal enhancement (blue arrow) consistent with sphingomyelin deposition seen in ASMD patients. Multiple bronchiectasis (black arrow) is also present. (b) Venous phase of an abdominal contrast CT, showing an enlarged portal vein (arrow) and a moderate amount of ascites fluid in the abdominal cavity. The liver is nodular, enlarged, and steatotic. The patient is asplenic. (c) Coronal and sagittal CT images of abdomen and pelvis show the cluster of varicose veins that are contrast-enhanced (outlined by yellow boxes), situated just above the vaginal lumen, and show extravasation of contrast into the small intestinal lumen.