Binder syndrome.

Abstract

UNLABELLED:

Patients with chondrodysplasia punctata (CDP) usually present with Binder-type features, and often CDP is misdiagnosed as Binder syndrome. This study reviewed the management and outcome of patients with Binder syndrome and CDP in a multidisciplinary setting.

METHODS:

The notes and radiographs of the patients managed at the Australian Craniofacial Unit with a multidisciplinary setting since 1976 were reviewed, and data were collected on patient demographics, associated medical and surgical problems, subsequent management, and complications.

RESULTS:

Seventy-seven patients were treated over the 30-year period (5 patients were lost to follow-up); of the remaining 72 patients, 60 (83%) had Binder syndrome, and 12 (17%) were patients with CDP. Forty were males, and 32 were females, with an age range of 6 months to 47 years. Thirteen patients (18%) had a strong family history, and 65 patients (90%) have so far undergone surgical correction, and of those, 35 (54%) have completed their treatment, the longest follow-up time being 18 years. The mean number of surgical procedures was 2.4, and 18 patients (28%) had postoperative complications, which included partial necrosis of the maxilla, osteomyelitis of the mandible, facial nerve and inferior alveolar nerve neuropraxia, nasal bone graft exposure, and cellulitis.

DISCUSSION:

Because of the phenotypic characteristics shared by both Binder syndrome and CDP, it is most likely that Binder syndrome is not a syndrome, nor is it an entity, but most likely to be an "association." We would advocate that these patients should be managed in a multidisciplinary setting.

• Authors: Shaheel Chummun, N R McLean, M Nugent, P J Anderson, David J David
• Journal: The Journal of craniofacial surgery (J Craniofac Surg) Vol. 23 Issue 4 Pg. 986-90 (Jul 2012) ISSN: 1536-3732 [Electronic] United States
• PMID: 22777437 (Publication Type: Journal Article)

Topics

• Adolescent
• Adult
• Australia (epidemiology)
• Child
• Child, Preschool
• Chondrodysplasia Punctata (diagnosis)
• Diagnosis, Differential
• Female
• Humans
• Infant
• Male
• Maxilla (abnormalities, surgery)
• Maxillofacial Abnormalities (diagnosis, epidemiology, surgery)
• Middle Aged
• Nose (abnormalities, surgery)
• Phenotype
• Postoperative Complications
• Treatment Outcome