A Rare Case of Median Cleft Face Syndrome
Median Cleft Face Syndrome also known as Frontonasal dysplasia is a rare disorder affecting the craniofacial region. It is characterized by atleast 2 of following features: wide spaced eyes; broad nose; Cleft on one or both side if nose ;no nasal tip ;central celft involving nose, upper lip or the palate. Incomplete formation of skull ( Widows peak Hairline)
During a regular screening and treatment camp undertaken at the Poona Blind school, a 14year old boy presented these features of a dysmorohic face. On a detailed history it was found that his parents were not consanguineously married and the mother did not have any form of allopathic or ayurvedic medicine during gestational period.
-Intercanthal distance = 32.1mm (Normal range = 28mm in that age group)
-Gait, height and weight are normal
-Micro opthalmos with partial loss of vision in right eye and complete loss of vision in left eye.
-Unilateral chewing habit (Leftside)
-Labially erupted right maxillary central incisor
-Delayed eruption of teeth and over retained 55.
-Irregularities in cranial vault.
-Left Maxillary sinus is obliterated.
-The exact cause of this rare disorder is not known. Genetic testing of both parents showed normal karyotype, thus genetic origin got ruled out.
Can you think of possible reasons that may have lead to this developmental deformity?