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.
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