Renita Lorina Castelino
1*, Shishir Ram Shetty
2, Subhas إabu G
3, Kumuda Arvind Rao H T
41 Post-graduate Student, Department of Oral Medicine and Radiology, A.B Shetty Memorial Institute of Dental Sciences, Mangalore, India
2 Assistant Professor, Department of Oral Medicine and Radiology, A.B Shetty, Memorial Institute of Dental Sciences, Mangalore, India
3 Professor, Department of Oral Medicine and Radiology, A.B Shetty Memorial Institute of Dental Sciences, Mangalore, India
4 Post-graduate Student, Department of Oral Medicine and Radiology, A.B Shetty Memorial Institute of Dental Sciences, Mangalore, India
Abstract
The oromandibular-limb hypogenesis syndrome comprises a group of anomalies which simultaneously affect the mandible,
tongue, and maxilla with or without reductive limb anomalies. It is characterized by failure of development of the intraoral
region and distal extremities. Multiple and variable deformities of the mandible, maxilla and tongue may occur in combination
with a variety of limb defects. The wide range of presentation and combination of anomalies make classification difficult.
They usually feature primarily in sporadic case reports because of their low incidence. The genetic origin of this syndrome
is uncertain. It is congenital and there seems to be no sex predilection. The key radiographic features are retruded
mandible, impacted teeth and malformed phalanges. When compared to available literature, frequently reported features like
hypodontia, hypoglossia, microstomia, protruded maxilla and limb anomalies were present in our case. The case presented
here is one of the rarest subtypes of this rare syndrome.