Anterior Unilateral Plagiocephaly in Patient with Alagille Syndrome: Case Report
Autor: | José Masegosa-González, Jose A. Escribano-Mesa, José María Narro-Donate, Ascensión Contreras-Jiménez, Antonio Huete-Allut, Francisco Pedrero-García, Paddy Mendez-Román, Mario Gomar-Alba |
---|---|
Rok vydání: | 2018 |
Předmět: |
0301 basic medicine
medicine.medical_specialty Plagiocephaly Craniosynostosis Craniosynostoses 03 medical and health sciences Anterior plagiocephaly Alagille syndrome Humans Medicine Craniofacial business.industry medicine.disease Surgery Alagille Syndrome Skull 030104 developmental biology medicine.anatomical_structure Child Preschool Printing Three-Dimensional Forehead Female Neurology (clinical) Coronal suture business |
Zdroj: | World Neurosurgery. 114:37-42 |
ISSN: | 1878-8750 |
DOI: | 10.1016/j.wneu.2018.03.027 |
Popis: | Background The polymalformative syndromes and craniofacial anomalies association is a well-known phenomenon in patients with Crouzon, Pfeiffer, Apert, or Muenke disease. Recently, other less frequent pathologies, such as Alagille syndrome, have shown an association with alterations in the development of cranial sutures, resulting in serious cosmetic defects and neurologic disorders. Case Description We report an exceptional case of a 30-month-old girl, a nephroblastoma survivor diagnosed with Alagille syndrome, who was referred to our department with progressive anterior plagiocephaly and premature left coronal suture closure associated with a large compensating right bossing. Despite the patient's age, we offered aggressive surgical treatment performing a new forehead harvested from the skull vertex with orbital rim reconstruction. Conclusions Alagille syndrome is a complex multisystem pathology with a poor craniosynostosis association and only 3 cases have been described in the literature. |
Databáze: | OpenAIRE |
Externí odkaz: |