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

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

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Correspondence to Hangzhou Wang.

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Han, Y., Wang, H. Intracranial arterial dolichoectasia and skull damage in a girl with Jaffe-Campanacci syndrome: a case report. Childs Nerv Syst 35, 1051–1054 (2019).

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  • Jaffe-Campanacci syndrome
  • Intracranial arterial dolichoectasia
  • Skull damage