Abstract
A 2-year-old boy who presented with a cystic gallbladder mass was found histologically to have metachromatic leukodystrophy. Diffuse hyperplasia of the gallbladder mucosa, multiple tendrillar fronds extending into the lumen, extensive papillomatosis, and the presence of large numbers of macrophages bearing metachromatic material confirmed the diagnosis. The child developed progressive degeneration of the central nervous system, refractory bronchopneumonia, and generalized muscular atrophy. A computed tomographic scan of the brain demonstrated hypodense areas of white matter suggestive of demyelination. This report describes the rare association of gallbladder papillomatosis with a storage disorder and reviews the relevant literature.
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References
Burgess JH, Kalfayan B, Slungaard RK, Gilbert E (1985) Papillomatosis of the gallbladder associated with metachromatic leukodystrophy. Arch Pathol Lab Med 109: 79–81
Dische MR (1969) Metachromatic leukodystrophic polyposis of gallbladder. J Pathol Bacteriol 97: 388–390
Heier L, Daneman A, Lowden JA, Cutz E, Craw S, Martin DJ (1983) Biliary disease in metachromatic leukodystrophy. Pediatr Radiol 13: 313–318
Khon R (1963) Papillomatosis of the gallbladder in metachromatic leukodystrophy. Am J Clin Pathol 52: 737–740
Tobias JD (1992) Anesthetic considerations for a child with leukodystrophy. Can J Anaesth 39: 394–397
Warfel KA, Hull MT (1984) Villous papilloma of the gallbladder in association with leukodystrophy. Hum Pathol 15: 1192–1194
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Oak, S., Rao, S., Karmarkar, S. et al. Papillomatosis of the gallbladder in metachromatic leukodystrophy. Pediatr Surg Int 12, 424–425 (1997). https://doi.org/10.1007/BF01076957
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DOI: https://doi.org/10.1007/BF01076957