Frontonasal dysplasia: A case report

Frontonasal dysplasia is an uncommon congenital anomaly with diverse clinical phenotypes and highly variable clinical characteristics, including hypertelorism, a broad nasal root, median facial cleft, a missing or underdeveloped nasal tip, and a widow's peak hairline. Frontonasal dysplasia is mostly inherited and caused by the ALX genes (ALX1, ALX3, and ALX4). We report a rare case of a frontonasal dysplasia patient with mild hypertelorism, a broad nasal root, an underdeveloped nasal tip, an accessory nasal tag, and a widow's peak. We used soft tissue re-draping to achieve aesthetic improvements.

Clinical Features and Management of a Median Cleft Lip

BACKGROUND: Median cleft lip is a rare anomaly consisting of a midline vertical cleft through the upper lip. It can also involve the premaxillary bone, the nasal septum, and the central nervous system. In our current report, we present the clinical features of 6 patients with a median cleft lip and their surgical management according to the accompanying anomalies. METHODS: From December 2010 to January 2014, 6 patients with a median cleft lip were reviewed. Five of these cases underwent surgical correction; alveolar bone grafting was performed in a patient with a median alveolar cleft. The surgical technique included inverted-U excision of the upper lip and repair of the orbicularis oris muscle. The mean follow-up period was 20.4 months (range, 7.4-44.0 months). RESULTS: The study patients presented various anomalous features. Five patients received surgical correction, 4 with repair of the median cleft lip, and one with iliac bone grafting for median alveolar cleft. A patient with basal sphenoethmoidal meningocele was managed with transoral endoscopic surgery for repair of the meningocele. Successful surgical repair was achieved in all cases with no postoperative complications. CONCLUSIONS: Relatively mild forms of median cleft lip can be corrected with inverted-U excision with good aesthetic outcomes. In addition, there is a broad spectrum of clinical features and various anomalies, such as nasal deformity, alveolar cleft, and short upper frenulum, which require close evaluation. The timing of the operation should be decided considering the presence of other anomalies that can threaten patient survival.

Case Report of Rib Bone Graft for Correction of Bifid Nose of Median Facial Cleft

Craniofacial cleft is a rare congenital anomaly with a wide range of clinical manifestation and severity of deformity. In 1976, Tessier announced classification system on the basis of anatomical observation derived from clinical finding or operative dissection. Nowadays, this system is in common use because it is in accordance with terminology and observational finding and clinical manifestation is accordant with operative finding. Median facial cleft(No. 0-14 facial cleft) has a wide range of congenital malformation from a midline cleft upper lip to orbital hypertelorism, among which the bifid nose is frequently associated with hypertelorism. The manifestation of a bifid nose is variable from a simple central groove at the nasal tip to a complete clefting of the osteocartilaginous framework. In consequence, the planning of correction of the bifid nose must be individualized. We contrived correction of bifid nose using rib bone graft containing small amount of costal cartilage with maneuver of 2mm incision on nasal root skin together with fixation with 9mm miniscrew through an open approach in two No. 0-14 facial cleft patients with mild hypertelorism and bifid nose. With this method we could obtain satisfactory results in the standpoint of function as well as aesthetics. We think that this method is appropriate for correction of bifid nose of mild median facial cleft.