These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Moyamoya Disease in a Patient with VACTERL Association.
    Author: Hong CS, Wang AC, Bonow RH, Abecassis IJ, Amlie-Lefond C, Ellenbogen RG.
    Journal: World Neurosurg; 2016 May; 89():729.e7-729.e10. PubMed ID: 26844876.
    Abstract:
    BACKGROUND: VACTERL association is characterized by a group of congenital malformations that tend to occur together. Rarely, concurrent cerebrovascular abnormalities have been reported. In this article, we present the first reported case of moyamoya disease in a patient with VACTERL association. CASE DESCRIPTION: The patient presented in the neonatal period with esophageal atresia with distal tracheoesophageal fistula as well as an imperforate anus. He also had a ventricular septal defect and persistent foramen ovale. At age 11 years, he developed seizures and was diagnosed with moyamoya disease, for which he underwent bilateral pial synagiosis. CONCLUSIONS: Our report adds moyamoya disease to the spectrum of rare diseases that may occur in the context of VACTERL association. Further studies may reveal whether a common pathophysiology exists between the 2 conditions. Our patient's congenital heart disease and the association between renovascular and cardiac disease with moyamoya may suggest a systemic vasculopathy. Moyamoya should be considered in children with VACTERL association who present with neurologic deficits or seizures.
    [Abstract] [Full Text] [Related] [New Search]