Johanson-Blizzard syndrome facial anomaly and its correction using a microsurgical bone graft and tripartite osteotomy.

Abstract

The facial anomaly of Johanson-Blizzard syndrome and its correction are described. The facial anomaly was characterized by cleft-like bony defects located in the inferomedial portion of the orbit apart from the hypoplastic maxilla and the absence of nasal alae. Correction of the facial skeleton was performed using a free vascularized iliac bone graft and tripartite osteotomy to correct the shape of the orbit and elongate the severely hypoplastic maxilla. Those procedures were effective to a certain degree in correcting the facial anomaly of Johanson-Blizzard syndrome.

• Authors: S Kobayashi, K Ohmori, J Sekiguchi
• Journal: The Journal of craniofacial surgery (J Craniofac Surg) Vol. 6 Issue 5 Pg. 382-5 (Sep 1995) ISSN: 1049-2275 [Print] United States
• PMID: 9020718 (Publication Type: Case Reports, Journal Article)

Topics

• Bone Lengthening (methods)
• Child
• Craniofacial Abnormalities (etiology, surgery)
• Craniotomy (methods)
• Ectodermal Dysplasia (complications)
• Exocrine Pancreatic Insufficiency (complications)
• Facial Bones (abnormalities, surgery)
• Female
• Humans
• Maxilla (abnormalities, surgery)
• Microsurgery
• Nose (abnormalities, surgery)
• Orbit (abnormalities, surgery)
• Syndrome
• Tomography, X-Ray Computed