Midline cervical cleft: a rare congenital anomaly.

OBJECTIVES: A midline cervical cleft (MCC) is a rare congenital anomaly due to failure of fusion of the first and second branchial arches during embryogenesis. It may present as a midline defect of the anterior neck skin with a skin projection or sinus, or as a subcutaneous fibrous cord. This report evaluates the clinical features and surgical management of an MCC. METHODS: We analyzed a series of 4 patients with an MCC successfully treated at Great Ormond Street Hospital for Children in London. RESULTS: Three male patients and 1 female patient between 4 and 11 months of age were found to have an MCC. Each patient presented with an erythematous, fibrous band of tissue extending between the chin and the suprasternal notch. Treatment comprised surgical excision of the lesion and Z-plasty repair. We present the embryology, common clinical presentation, investigations, differential diagnosis, and histology, along with a literature review, of this uncommon malformation of the anterior neck. CONCLUSIONS: An MCC is a differential diagnosis to consider when assessing a child with a midline cervical lesion. Early surgical excision with Z-plasty repair of the soft tissue defect is the treatment of choice to prevent long-term complications.

Angiosarcoma of skull base in a 1-year-old child--a case report.

Angiosarcoma is a rare tumour of endothelial cell origin whilst malignant skull base tumours are highly unusual in paediatric patients. This case reports an angiosarcoma involving the clivus and basi-sphenoid region of the skull base, in a 1-year-old boy. This tumour is extremely rare in childhood, particularly in this site. The histological features were consistent with a high-grade haemangioendothelioma, categorised as an angiosarcoma. The characteristics of this rare malignancy and the challenges in its management are discussed. This is the youngest reported patient, to our knowledge, with an angiosarcoma of the skull base.