PMC5749285_01
PMC5749285_02
(a) Weight-for-age plot for the patient. Growth consistently above 0 Z-score (middle line on the image) throughout the under-five period. (b) Central cyanosis (arrow). (c) Finger clubbing (arrows).
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fig1
He had developed conjunctival chemosis and finger clubbing (Figure 1).
(a) CT scan of the abdomen. Note the hepatic hemangioma (crossed lines 1 and 2). (b) Liver histology. Left to right: Liver parenchyma is present which, although subcapsular, shows evidence of marked distortion of the normal architecture, with fibrosis present surrounding regenerative-type nodules. Masson trichome stain shows extensive interstitial fibrosis surrounding regenerative-type nodules, as can be appreciated on the microphotograph. Reticulin stain shows twinning of the cell plates, which can be appreciated on the microphotograph.
CRIPE2017-7940365.002.jpg
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fig2
He was referred to a hepatobiliary specialist in South Africa, where computed tomographic scan of the abdomen and pelvis showed a large mass in segment III of the left lobe of the liver (4.98 x 4.51 cm), that was suggestive of hemangioma, and had not changed in size over the years (Figure 2). A wedge liver biopsy showed grossly distorted architecture with regenerative micronodules of hepatocytes surrounded by dense fibrotic septa showing portoportal and portovenous linkage (Figure 2(b)(b)).