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Simpson-Golabi-Behmel syndrome associated with cleft palate
Yoshihiro Morita1, Natsuko Kimoto, Hisashi Ogawa
1Department of Oral and Maxillofacial Surgery II, Osaka University, Graduate School of Dentistry, Osaka, Japan.
The Journal of Craniofacial Surgery
|October 1, 2011
Summary
This case study details a rare instance of Simpson-Golabi-Behmel syndrome, an X-linked condition causing overgrowth and anomalies, in a patient with a cleft palate. Surgical repair of the cleft palate was successfully performed.
Area of Science:
- Genetics
- Pediatrics
- Maxillofacial Surgery
Background:
- Simpson-Golabi-Behmel syndrome is a rare X-linked disorder.
- It is characterized by overgrowth and multiple congenital anomalies.
Observation:
- A patient presented with a cleft palate and features consistent with Simpson-Golabi-Behmel syndrome.
- Distinctive facial features included a wide nasal bridge, anteverted nostrils, macroglossia, and prominent jaw.
Findings:
- The patient had both a cleft palate and Simpson-Golabi-Behmel syndrome.
- Surgical intervention using the push-back method was employed for cleft palate repair.
Implications:
- Highlights the importance of recognizing rare genetic syndromes in maxillofacial patients.
- Emphasizes the need for multidisciplinary management of complex congenital anomalies.
- Informs surgical approaches for cleft palate repair in syndromic cases.
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