Intracranial arterial dolichoectasia and skull damage in a girl with Jaffe-Campanacci syndrome: Jaffe-Campanacci is a rare syndrome characterised by axillary freckles, multiple non-ossifying fibromas of the long bones and jaw, and café-au-lait spots, associated with some features of neurofibromatosis type 1 (NF1).

Intracranial arterial dolichoectasia and skull damage in a girl with Jaffe-Campanacci syndrome: a case report.:

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Intracranial arterial dolichoectasia and skull damage in a girl with Jaffe-Campanacci syndrome: a case report.

Childs Nerv Syst. 2019 06;35(6):1051-1054

Authors: Han Y, Wang H

Abstract

ᅟJaffe-Campanacci is a rare syndrome characterised by axillary freckles, multiple non-ossifying fibromas of the long bones and jaw, and café-au-lait spots, associated with some features of neurofibromatosis type 1 (NF1). Cherix et al. and Colby and Saul suggested that Jaffe-Campanacci syndrome (JCS) might be a distinct form of NF1. Intracranial arterial dolichoectasia (IADE) is defined as an increase in the length and diameter of at least one intracranial artery. Affected intracranial arteries are dilated, elongated and sometimes tortuous. But in this rare disease of JCS, neither skull damage nor IADE has been previously reported. Here, we introduce the case of an 11-year-old Chinese girl with IADE, skull damage and features of JCS.



PMID: 30721350 [PubMed - indexed for MEDLINE]