Anterior unilateral plagiocephaly treatment in patient with alagille syndrome. A case report.

Anterior unilateral plagiocephaly treatment in patient with alagille syndrome. A case report.

World Neurosurg. 2018 Mar 09;:

Authors: Narro-Donate JM, Méndez-Román P, Huete-Allut A, Escribano-Mesa JA, Gomar-Alba M, Contreras-Jiménez A, Pedrero-García F, Masegosa-González J

Abstract
BACKGROUND: The polymalformative syndromes and craniofacial anomalies association is a well-known phenomenon in patients with Crouzon's, Pfeiffer, Apert or Muenke disease. Recently, other less frequent pathologies, such as Alagille syndrome, are showing an association with alterations in the development of cranial sutures resulting in serious cosmetic defects and neurological disorders.
CASE DESCRIPTION: The authors report an exceptional case of a 30-month-old girl, nephroblastoma survivor, diagnosed with Alagille syndrome who is referred to our department with progressive anterior plagiocephaly and premature left coronal suture closure associated with a large compensating right bossing. Despite the age of the patient, we offered aggressive surgical treatment performing a new forehead harvested from skull vertex with orbital rim reconstruction.
CONCLUSIONS: Alagille syndrome is a complex multisystem pathology with a poor craniosynostosis association and only three cases have been described in the literature.

PMID: 29530693 [PubMed - as supplied by publisher]

http://ift.tt/2GroDRb