{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T16:43:47Z","timestamp":1787071427720,"version":"build-2736575974"},"reference-count":12,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2012,9]]},"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.4103\/0973-029x.102504","type":"journal-article","created":{"date-parts":[[2012,10,16]],"date-time":"2012-10-16T03:43:20Z","timestamp":1350359000000},"page":"414-419","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":10,"title":["Isolated aglossia congenita: A rare case of oromandibular limb hypogenesis syndrome type I B"],"prefix":"10.4103","volume":"16","author":[{"given":"Shalini R","family":"Gupta","sequence":"first","affiliation":[{"name":"Departments of Oral Medicine and Radiology, Maulana Azad Institute of Dental Sciences, New Delhi, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-20250922","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1136\/jmg.12.1.89","article-title":"Aglossia-adactylia syndrome","volume":"12","author":"Nevin","year":"1975","journal-title":"J Med Genet"},{"key":"R2-20250922","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1002\/ajmg.1320310516","article-title":"Aglossia with congenital absence of the mandibular rami and other craniofacial abnormalities","volume":"4","author":"Neidich","year":"1988","journal-title":"Am J Med Genet Suppl"},{"key":"R3-20250922","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1016\/0030-4220(90)90319-N","article-title":"Persistent buccopharyngeal membrane with cleft palate: A case report","volume":"69","author":"Pillai","year":"1990","journal-title":"Oral Surg Oral Med Oral Pathol"},{"key":"R4-20250922","doi-asserted-by":"crossref","first-page":"46","DOI":"10.4103\/0970-4388.31991","article-title":"Aglossia: A case report","volume":"25","author":"Kumar","year":"2007","journal-title":"J Indian Soc Pedod Prev Dent"},{"key":"R5-20250922","doi-asserted-by":"crossref","first-page":"7926","DOI":"10.4076\/1757-1626-2-7926","article-title":"Isolated aglossia in a six year old child presenting with impaired speech: A case report","volume":"2","author":"Rasool","year":"2009","journal-title":"Cases J"},{"key":"R6-20250922","first-page":"233","article-title":"Aglossia-adactylia","volume":"7","author":"Hall","year":"1971","journal-title":"Birth Defects Orig Artic Ser"},{"key":"R7-20250922","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/0165-5876(94)90109-0","article-title":"Oromandibular \u2013 limb hypogenesis syndrome following chorionic villus sampling","volume":"29","author":"Gruber","year":"1994","journal-title":"Int J Pediatr Otorhinolaryngol"},{"key":"R8-20250922","doi-asserted-by":"crossref","first-page":"e41","DOI":"10.1016\/j.tripleo.2007.09.028","article-title":"Complete and isolated congenital aglossia: Case report and treatment of sequelae using rapid prototyping models","volume":"105","author":"Salles","year":"2008","journal-title":"Oral Surg Oral Med Oral Pathol Oral Radiol Endod"},{"key":"R9-20250922","doi-asserted-by":"crossref","first-page":"2249","DOI":"10.1242\/dev.056929","article-title":"Downregulation of Dlx5 and Dlx6 expression by H and 2 is essential for initiation of tongue morphogenesis","volume":"138","author":"Barron","year":"2011","journal-title":"Development"},{"key":"R10-20250922","first-page":"974","article-title":"Palatal groove formation in neonates and infants with orotracheal tubes","volume":"138","author":"Erenberg","year":"1984","journal-title":"Am J Dis Child"},{"key":"R11-20250922","doi-asserted-by":"crossref","first-page":"F29","DOI":"10.1136\/fn.78.1.F29","article-title":"Deformation of palate in preterm infants","volume":"78","author":"Procter","year":"1998","journal-title":"Arch Dis Child Fetal Neonatal Ed"},{"key":"R12-20250922","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/S0266-4356(97)90712-9","article-title":"Mandibular widening by distraction osteogenesis","volume":"35","author":"Guerrero","year":"1997","journal-title":"Br J Oral Maxillofac Surg"}],"container-title":["Journal of Oral and Maxillofacial Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/01233803-201216030-00023","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T15:46:01Z","timestamp":1787067961000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/01233803-201216030-00023"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,9]]},"references-count":12,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2012]]}},"URL":"https:\/\/doi.org\/10.4103\/0973-029x.102504","relation":{},"ISSN":["0973-029X","1998-393X"],"issn-type":[{"value":"0973-029X","type":"print"},{"value":"1998-393X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,9]]}}}