Oro mandibuar limb hypogenesis syndrome type ii c … a rare case report

Hypoglossia –Hypomelia is rare congenital anomaly affecting the tongue and limbs. Hall in 1971 classified it under a complex group of disorders called oro Mandibular limb hypogenesis Syndrome. It is an extremely rare condition with around 40 cases reported in world literature. The etiology of the syndrome is unknown. Some type of intrauterine trauma is the most widely accepted etiology. The characteristic features of the syndrome are hypoglossia, limb anomalies of variable degrees and micrognathia of the mandible. It is characterized by failure of development of intraoral region and distal extremities .It is congenital and there seems to be no sex predilection. We hereby report a case of 15 years old girl patient presenting with retruded mandible, hypoglossia and limb anomaly (hypomelia) Her parents and other siblings were normal. Positive prenatal history of maternal hyperthermia was obtained suspecting it to be cause of Oro Mandibular limb hypogenesis