Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease): Case report

Wouter I. Schievink, James F. Mellinger, John L D Atkinson

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

INTRACRANIAL ANEURYSMS ARE uncommon in children, and their presence often leads to suspicion of a systemic connective tissue disorder. We describe the case of a young male patient with progressive hemifacial atrophy (Parry- Romberg disease) and multiple intracranial aneurysms, a previously undescribed association, and propose that a neural crest defect may be the underlying abnormality in this patient. At age 5 years, the patient was treated for a giant aneurysm of the left cavernous carotid artery with carotid ligation in the neck and a superficial temporal artery-middle cerebral artery bypass. At age 12 years, the patient was similarly treated for a giant aneurysm of the right cavernous carotid artery, which had progressed from a previously noted minute dilatation at age 5 years, with carotid ligation and a superficial temporal artery-middle cerebral artery bypass. At age 21 years, the patient was endovascularly treated for a de novo saccular aneurysm of the left posterior cerebral artery at the P1-P2 junction and a fusiform aneurysm of the distal left posterior cerebral artery. Various studies have suggested that the facial dermis, the subcutaneous tissues, and the skeleton, as well as the tunica media of the cervicocephalic arteries, all arise from neural crest cells, and a disorder of neural crest migration might explain the constellation of findings in this patient.

Original languageEnglish (US)
Pages (from-to)1237-1241
Number of pages5
JournalNeurosurgery
Volume38
Issue number6
DOIs
StatePublished - Jun 1996

Fingerprint

Facial Hemiatrophy
Aneurysm
Neural Crest
Posterior Cerebral Artery
Temporal Arteries
Middle Cerebral Artery
Carotid Arteries
Ligation
Tunica Media
Subcutaneous Tissue
Intracranial Aneurysm
Dermis
Skeleton
Connective Tissue
Dilatation
Neck
Arteries

Keywords

  • Cerebral aneurysm
  • De novo aneurysm
  • Hemifacial atrophy
  • Neural crest
  • Parry-Romberg disease

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery

Cite this

Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease) : Case report. / Schievink, Wouter I.; Mellinger, James F.; Atkinson, John L D.

In: Neurosurgery, Vol. 38, No. 6, 06.1996, p. 1237-1241.

Research output: Contribution to journalArticle

Schievink, Wouter I. ; Mellinger, James F. ; Atkinson, John L D. / Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease) : Case report. In: Neurosurgery. 1996 ; Vol. 38, No. 6. pp. 1237-1241.
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