Journal de la grossesse et de la santé infantile

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Otocephaly:Agnathia Microstomia Synotia Syndrome-A Case Report

Vishnu Sivapatham, Chamara Janaka Prasad Jayamanne, Saman Kumara, Buddima Jayasinghe and Ruwini Fonseka

Otocephaly is characterized by aplasia or hypoplasia of mandible, a small oral fissure and low lying ears at the level of neck usually meeting in the midline. This is due to the failure of migration of the ventral part of the first brachial arch. This rare lethal non familial condition occurs in 1 in 70,000 births. This is the first reported case of otocephaly in Sri Lanka as per our best knowledge, a baby girl born to non-consanguineous parents, who was unable to survive a long time after birth. This case is being reported because of its rarity.