A case of Maffucci syndrome

Mathys, L; Jenzer, A; Della Santa, D (2010). A case of Maffucci syndrome. Chirurgie de la main, 29(1), pp. 36-9. Paris: Elsevier Masson SAS 10.1016/j.main.2009.11.002

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The Maffucci syndrome consists of a combination of multiple enchondromas and haemangiomas. It appears in the first two decades of life, with no family history. In this case we are reporting about a 26-year-old female who had suffered from multiple enchondromas since the age of two. At the age of nine, the patient presented with additional haemangiomas, which facilitated making proper diagnosis. She now presents with a massive lesion of her left upper extremity. The patient had initially rejected operative treatment when the disease was at early stages. At later stages, a more complex reconstruction of the hand would have been necessary to secure hand function. This procedure that sometimes induces a risk related to potential necessity of blood transfusion was rejected by the patient for religious reasons. Amputation of the extremity was therefore the last resort procedure.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Orthopaedic, Plastic and Hand Surgery (DOPH) > Clinic of Plastic and Hand Surgery > Hand Surgery

UniBE Contributor:

Mathys, Lukas

ISSN:

1297-3203

Publisher:

Elsevier Masson SAS

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:07

Last Modified:

17 Mar 2015 19:07

Publisher DOI:

10.1016/j.main.2009.11.002

PubMed ID:

20036796

Web of Science ID:

000275851600006

URI:

https://boris.unibe.ch/id/eprint/295 (FactScience: 197417)

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