Journal of Oral and Maxillofacial Pathology

CASE REPORT
Year
: 2012  |  Volume : 16  |  Issue : 3  |  Page : 414--419

Isolated aglossia congenita: A rare case of oromandibular limb hypogenesis syndrome type I B


Shalini R Gupta 
 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

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.


How to cite this article:
Gupta SR. Isolated aglossia congenita: A rare case of oromandibular limb hypogenesis syndrome type I B.J Oral Maxillofac Pathol 2012;16:414-419


How to cite this URL:
Gupta SR. Isolated aglossia congenita: A rare case of oromandibular limb hypogenesis syndrome type I B. J Oral Maxillofac Pathol [serial online] 2012 [cited 2020 Oct 1 ];16:414-419
Available from: http://www.jomfp.in/article.asp?issn=0973-029X;year=2012;volume=16;issue=3;spage=414;epage=419;aulast=Gupta;type=0