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

  • Yong Han
  • Hangzhou WangEmail author
Case Report


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.


Jaffe-Campanacci syndrome Intracranial arterial dolichoectasia Skull damage 



The study was supported by Science and education program of Suzhou (KJXW2017023).

Compliance with ethical standards

Conflict of interest

The authors received no financial and/or material support for the research reported in this paper. The authors state that there are no conflicts of interest arising from the research reported in this paper.

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of NeurosurgeryChildren’s Hospital of Soochow UniversitySuzhouPeople’s Republic of China

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