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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists


 
CASE REPORT Table of Contents   
Year : 2012  |  Volume : 16  |  Issue : 3  |  Page : 414-419
Isolated aglossia congenita: A rare case of oromandibular limb hypogenesis syndrome type I B


Departments of Oral Medicine and Radiology, Maulana Azad Institute of Dental Sciences, New Delhi, India

Correspondence Address:
Shalini R Gupta
S19/1401, The Close South, Nirvana Country, Gurgaon, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-029X.102504

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Aglossia congenita (AC), congenital total absence of the tongue, is a very rare midline developmental anomaly, hypothesized to be associated with vascular disruption between the fourth and eighth week of gestation. It was classified by Hall (1971) as part of oromandibular limb hypogenesis syndrome (OLHS) type I B. Most of the cases reported with OLHS are actually hypoglossia with limb abnormalities whereas isolated aglossia is an extremely rare entity. A case of isolated AC is presented in a 28-year-old Indian male. He had long narrow face, tapering chin, low set ears, and microstomia. Intraorally, he had narrow palatal vault, constricted oropharyngeal isthmus, oligodontia, and maxillo-mandibular hypoplasia. Interestingly, the patient showed a median palatal groove, which has not been reported before. He also had an unusual acquired adaptive mechanism to compensate for aglossia. This report presents the manifestations of this rare syndrome, its complications, differential diagnosis, and rehabilitation strategies.


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