Mouth Breathing Center, Division of Pediatric Otorhinolaryngology, UNIFESP, São Paulo Hospital, São Paulo, Brazil.
Oral Surg Oral Med Oral Pathol Oral Radiol. 2013 Oct;116(4):e230-6. doi: 10.1016/j.oooo.2011.12.025. Epub 2012 Jul 20.
The oromandibular and limb hypogenesis syndrome is characterized by aglossia or hypoglossia presenting with limb anomalies. In this case report, we describe congenital hypoglossia associated with glossopalatine ankylosis and middle finger hypomelia, a type III-D malformation in the Hall Classification. The orthodontic and surgical treatment consisted of 3 maxillary expansions, mandibular surgery, and dental alignment. This extensive treatment resulted in acceptable function and esthetic appearance; however, because of lingual hypoglossia, it did not effect perfect occlusion.
口颌和肢体发育不全综合征的特征是舌缺失或发育不全，伴有肢体异常。在本病例报告中，我们描述了与腭舌粘连和中指短缩相关的先天性舌缺失，这是 Hall 分类中的 III-D 型畸形。正畸和手术治疗包括 3 次上颌扩张、下颌手术和牙齿排列。这种广泛的治疗方法取得了可接受的功能和美观效果；然而，由于舌缺失，它并没有达到完美的咬合效果。
