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DOI: 10.1055/s-0030-1248246
© Georg Thieme Verlag KG Stuttgart · New York
Massive Hemobilia and Papillomatosis of the Gallbladder in Metachromatic Leukodystrophy: A Life-Threatening Condition
Publication History
received 26.08.2009
accepted 15.01.2010
Publication Date:
05 May 2010 (online)
![](https://www.thieme-connect.de/media/neuropediatrics/200906/lookinside/thumbnails/10.1055-s-0030-1248246-1.jpg)
Abstract
Polyposis of the gallbladder is rare during childhood. This condition can be associated with three other conditions: metachromatic leukodystrophy, Peutz-Jeghers’ syndrome, and pancreaticobiliary maljunction. We report the case of a child with hemobilia in metachromatic leukodystrophy, which rendered cholecystectomy necessary. Macroscopically, the gallbladder measured 4.6 cm in length and showed an opaque serous surface and focal brown petechiae. Moreover, a yellow polypoid lesion of 2 cm in diameter and a diffuse thickening of the fundus wall were observed. Many reports describe the importance of the association of gallbladder papillomatosis with metachromatic leukodystrophy, but only three cases presented with massive intestinal bleeding, such as our young patient had. It is thus imperative that this life-threatening condition should be well known.
Key words
metachromatic leukodystrophy - hemobilia - gallbladder papillomatosis
References
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Correspondence
Dr. Livia Garavelli
Struttura Semplice
Dipartimentale di Genetica
Clinica
Arcispedale S. Maria Nuova
Azienda Ospedaliera
Viale Risorgimento 80
42100 Reggio Emilia
Italy
Phone: +39/0522/296 244/296 241
Fax: +39/0522/296 266
Email: garavelli.livia@asmn.re.it