A rare congenital midline cervical cleft.

A rare case of congenital midline cervical cleft (CMCC) is presented to highlight the importance of correct recognition of the lesion and appropriate surgical management. A 2-week-old female baby was referred to the paediatric outpatient depar tment at Red Cross War Memorial Children's Hospital with an anterior midline neck lesion that had been diagnosed at birth. She had been born by vaginal delivery after an uncomplicated term pregnancy. Her birth weight was 3 280 g, and she was treated for neonatal jaundice. There was no family history of similar lesions. On physical examination she had a vertical linear lesion that began 1.0 cm above the sternal notch and was 3.0 cm in length with a nipple-like protuberance at the cranial end (level of hyoid bone) (Fig. 1). A superficial midline band was palpable subcutaneously. A blind-ending sinus was noted at the caudal end of the cleft. No discharge from the sinus was noted. No bony spurs on the mandible or sternum were detected on clinical or radiological examination. An ultrasound scan of the thyroid gland showed it to be normal. At the time of scheduled surgery, at 8 months of age, the CMCC was limiting the patient's neck extension. Elliptical surgical excision of the entire cleft and repair by Z-plasty was undertaken. A blind-ending sinus that ended on the manubrium sternum was dissected out. The lesion did not appear to extend up to the mandible or involve the hyoid bone. Histological examination of the resected specimen revealed epidermis overlying prominent skeletal muscle in sections of the superior portion. Respiratory-type epithelium and seromucinous glands were present along the invaginated surface more distally. The distal band showed dense collagen. When the patient was seen at follow-up 6 months later there was a good cosmetic result and no residual contractures. Discussion CMCC is a rare condition, less than 100 cases having been reported in the English literature so far. The first recorded case of CMCC was in 1848 by Luschka. In a series of 672 thyroglossal and other branchial cleft sinuses over a 30-year period, Gargan et al. 1 reported 12 CMCCs (1.7% of their series). This lesion is often misdiagnosed at birth by the primary care physician as a thyroglossal duct cyst 2 or, as in one reported case, referred to the dermatology service as an 'unusual birthmark'. 3 Complete surgical excision is important to prevent neck contractures. Patients with CMCC seem …