Frontonasal Dysplasia: A Clinical Dilemma

Frontonasal dysplasia is a congenital malformation, which results from atypical abnormal development of the cranium and face. It affects the frontal portion of the face, the eyes and the nose. It may occur singly or associated with other clinical signs. We report here a rare case of a 3-year-old boy who presented with features of frontonasal dysplasia. There was no history of consanguinity and no positive family history. This child presented with hypertelorism, wide nasal bridge, absent nasal cartilages, laterally positioned nasal alae and bunched up tissue over the nasal dorsum resembling a second nose. Investigations ruled out the presence of an encephalocele. At present, he is planned for excision and Z-plasty to address the excessive soft tissue over the nose. Correction of orbital hypertelorism using facial bipartition through a cranial approach is scheduled between the ages of 6 and 8 years. A secondary rhinoplasty with reconstruction of nasal cartilages would be required after growth completion.