Celebiler Ozhan, Sönmez Ahmet, Erdim Melike, Ozek Memet, Numanoğlu Ayhan
Department of Plastic & Reconstructive Surgery, Marmara University, University Hospital.,Istanbul, Turkey.
J Craniomaxillofac Surg. 2007 Mar;35(2):81-3. doi: 10.1016/j.jcms.2006.10.005. Epub 2007 Apr 20.
In contrast to the common clefts of the lip, alveolus and palate, the atypical clefts of the face may come in myriad patterns of clinical expression and are often not easy to define.
In this report, a case of median craniofacial dysraphia is described.
At presentation, the 3-month-old male patient had a bilateral complete cleft of the lip, alveolus and palate. The nose was wide and a horn was present on the nasal dorsum. 3-D CT AND MRI REVEALED: Duplication of the metopic suture ending at the wide anterior fontanel; orbital hypertelorism; midline cranial cleft ending just superior to the nasal dorsum; frontoethmoidal encephalocoele and holoprosencephaly. The presence of two metopic sutures was confirmed during surgery.
The presented case carries the characteristics of the median cleft face syndrome. However, it differs from similar cases in two respects. First, the patient had two metopic sutures, one on either side of the cranial extension of the median cleft. Second, the patient had a bilateral cleft lip in contrast to the expected median cleft lip deformity.
与常见的唇、牙槽突和腭裂不同，面部非典型裂可能呈现出无数种临床表现形式，且往往难以界定。
在本报告中，描述了一例正中颅面裂畸形的病例。
就诊时，这名3个月大的男性患者患有双侧完全性唇、牙槽突和腭裂。鼻子宽大，鼻背有一个角状物。三维CT和MRI显示：额缝重复，止于宽大的前囟门；眶距增宽；中线颅骨裂止于鼻背上方；额筛部脑膨出和全前脑畸形。手术中证实存在两条额缝。
本病例具有正中裂面综合征的特征。然而，它在两个方面与类似病例不同。第一，患者有两条额缝，分别位于正中裂颅骨延伸部分的两侧。第二，与预期的正中唇裂畸形不同，该患者患有双侧唇裂。
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