Journal of Cranio-Maxillofacial Surgery (2007) 35, 81–83 r 2007 European Association for Cranio-Maxillofacial Surgery doi:10.1016/j.jcms.2006.10.005, available online at http://www.sciencedirect.com Atypical midline cleft with duplication of the metopic suture O ¨ zhan C¸ ELEBI ˙ LER 1 , Ahmet SO ¨ NMEZ 1 , Melike ERDI ˙ M 1 , Memet O ¨ ZEK 2 , Ayhan NUMANOG ˘ LU 1 1 Department of Plastic & Reconstructive Surgery (Head: Prof. Ayhan Numanog˘lu, M.D.); 2 Department of Neurosurgery, Division of Paediatric Neurosurgery (Head: Prof. Memet O ¨ zek, M.D.), Marmara University, University Hospital, Istanbul, Turkey SUMMARY. Introduction: 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. Purpose: In this report, a case of median craniofacial dysraphia is described. Patient: 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. Conclusion: 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. r 2007 European Association for Cranio-Maxillofacial Surgery Keywords: metopic suture duplication, median cleft face syndrome, median cleft INTRODUCTION In contrast to the common clefts of the lip, alveolus and palate, the rare or atypical clefts of the face may not be easy to define, and they may come in myriad patterns of clinical expression. The median craniofa- cial dysraphia is one of the most frequently observed atypical facial clefts, constituting about 37% of all atypical clefts (Ortiz-Monasterio et al., 1987). The anomaly is subdivided into those involving a defi- ciency in tissue (hypoplasia, holoprosencephaly), and those in which there is an excess intervening tissue (hyperplasia) (Kawamoto, 1990; Kawamoto and Patel, 1998). Therefore, the clinical presentation extends across a broad range from tissue deficiency to tissue excess. Those cases with tissue excess and hyperplasia are called the median cleft face syndrome according to DeMyer (1967). The cleft corresponds to number 0–14 craniofacial cleft, according to Tessier (1976) and Kawamoto (1976). The clinical presentation may include, orbital hypertelorism, V-shaped frontal hair- line, bifid cranium, bifid nose, thickened or dupli- cated anterior nasal spine and nasal septum, median cleft of the upper lip (premaxilla and palate). In this report, a case of median craniofacial dysraphia of the hyperplasia type is presented. This case differs from similar cases in two aspects: First, the patient had two metopic sutures, one on either side of the cranial extension of the median cleft. Second, the patient had bilateral clefts of lip in contrast to the expected median cleft lip deformity. CASE REPORT The patient was born by spontaneous vaginal delivery at term, as the first child of unrelated parents and weighed 3000 g. He was admitted to this clinic at 3 months of age. At presentation, he had bilateral complete clefts of lip, alveolus and palate. The nose was wide and a horn was present on the nasal dorsum (Fig. 1). Lower limb spasticity was noted, but no other physical anomaly. Craniofacial 3-D CT and MRI scans revealed bilateral cleft lip, alveolus and palate; a duplicated metopic suture which ended at the wide anterior fontanel; orbital hypertelorism; a midline cranial cleft that ended just superior to the nasal dorsum; a frontoethmoidal encephalocoele and holoprosencephaly (Fig. 2). Bilateral cleft lip repairs were performed at seventh months. Together with the paediatric neurosurgeon the encephalocoele was repaired and the nasal horn excised at the age of nine months. In this operation, a bifrontal craniotomy was performed, and the super- ior orbital rim was removed. Surgical exploration confirmed the presence of two metopic sutures, which had already been diagnosed preoperatively by 3-D CT. There were two paramedian suture lines arising on each side of the median cleft from the supero- medial orbital rims extending to the anterior fonta- nel. During resection of the dorsal bony nasal horn, it was noticed that the nasal septum was duplicated and the horn was extending to the cranial cavity through a bony cleft at the midline (Fig. 3). After excision of ARTICLE IN PRESS 81