Oromandibular limb hypogenesis syndrome: a singular variation of a rare syndrome: a case report

Background: A positive prenatal history of maternal fever has been found to express as a range defects in infants including limb reduction, central nervous system defects, facial dysmorphogenesis and foetal death. Oromandibular hypogenesis syndrome is rare spectrum congenital disorders characterised by malformations the tongue, mandible, maxilla limbs. They present sporadic cases with extremely low incidence seldom occur associated syngnathia. Syngnathia can manifest fibrous, bony or combination. Observation: Here we represent case type IV E combination syngnathia, cleft palate, retrognathia hypoglossia-hypodactylomelia. An early surgical release syngnathia was undertaken on having features failure thrive, high risk aspiration related complications due enteral feeding. Conclusion: Delaying procedure could result growth restriction progressive ankylosis Temporomandibular joint (TMJ). It essential document singular variant (OLHS) medical literature.