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PMID: 30721350 Published · ppublish English

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

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.

Keywords
Intracranial arterial dolichoectasia Jaffe-Campanacci syndrome Skull damage
MeSH 主题词
Abnormalities, Multiple/pathology Bone Neoplasms/complications,pathology Cafe-au-Lait Spots/complications,pathology Child Female Fibroma/complications,pathology Humans Melanosis/complications,pathology Skull/pathology Syndrome Vertebrobasilar Insufficiency/complications,pathology
Article Info
Journal
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
Abbr.
Childs Nerv Syst
ISSN
1433-0350
Corresponding email
Published
2019-00-00
Language
English
Country/Region
Germany
NLM ID
8503227
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