International Journal of Clinical Pediatric Dentistry

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VOLUME 9 , ISSUE 1 ( January-March, 2016 ) > List of Articles

CASE REPORT

A Rare Clinical Variant of Oromandibular Limb Hypogenesis Syndrome Type I B

Ritesh Rambharos Kalaskar, Alkesh Godhane, Ashita Kalaskar, Swati Demble

Keywords : Aglossia, Environmental factors, Hypodactyli, Microstomia, OLHS, Rudimentary ears

Citation Information : Kalaskar RR, Godhane A, Kalaskar A, Demble S. A Rare Clinical Variant of Oromandibular Limb Hypogenesis Syndrome Type I B. Int J Clin Pediatr Dent 2016; 9 (1):78-81.

DOI: 10.5005/jp-journals-10005-1338

License: CC BY-NC 4.0

Published Online: 00-03-2016

Copyright Statement:  Copyright © 2016; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Aglossia is a rare congenital malformation that often occurs as an isolated disorder or is observed in association with other congenital deformities, particularly limb defects. We present a unique case of a 7-year-old girl with aglossia, hypodactyli, rudimentary ears, retrognathic and V-shaped mandible. Her parental history revealed intrauterine exposure of medicines. The patient had problems in difficulty in eating, speech, taste sensation and hearing. The present case does not fit into Hall\'s classification of oromandibular limb hypogenesis syndrome (OLHS) which best describes hypoglossia and limb deformities. Therefore, the purpose of this article is to document the rare variant of OLHS which can be included in Hall\'s classification. How to cite this article: Kalaskar RR, Godhane A, Kalaskar A, Demble S. A Rare Clinical Variant of Oromandibular Limb Hypogenesis Syndrome Type I B. Int J Clin Pediatr Dent 2016;9(1):78-81.


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  1. Nevin NC, Burrows D, Allen G, Kernoham DC. Aglossia adactyli syndrome. J Med Genet 1975 Mar;12(1):89-93.
  2. Lustmann J, Lurie R, Struthers P, Garwood A. Hypoglossiahypodactylia syndrome: report of 2 cases. Oral Surg Oral Med Oral Pathol 1981 Apr;51(4):403-408.
  3. Meundi MA, Nair GR, Sreenivasan P, Raj AC. Oromandibular limb hypogenesis syndrome type II B: case report of hypoglossia-hypodactyly. Case Rep Dent 2013;2013:370695.
  4. Firth HV, Boyd PA, Chamberlain PF, Mackenzie IZ, Morriss GM, Huson SM. Analysis of limb reduction defect in babies exposed to chorionic villus sampling. Lancet 1994 Apr 30; 343(8905):1069-1071.
  5. Kettner MR. Kongenitaler Zungendefekt. Deutsch Med Wehnschr 1907;33:532.
  6. Rosenthal R. Aglossia congenita. A report of case of the condition combined with other congenital malformations. Am J Dis Child 1932 Aug;44(2):383-389.
  7. Hall BD. Aglossia-adactylia. Birth defects Orig Artic Ser 1971 Jun;7(7):233-236.
  8. Purohit SK, Kunta SM, Rao PP, Thatte RL. An interesting case of aglossia-adactyli syndrome. Br J Plast Surg 1989 Mar; 42(2):228-229.
  9. Preis S, Majewski F, Hantschman R, Schumacher H, Lenard HG. Goldenhar, Mobius and hypoglossal-hypodactyli anomalies in a patient; syndrome or association? Eur J Pediatr 1996 Jun;155(5):385-389.
  10. Kantapura P, Tanpaiboon P. Thyroid dysfunction in a patient with aglossia. Am J Med Genet A 2003 Oct 15;122A(3):274-277.
  11. Higashi K, Edo M. Conductive deafness in aglossia. J Laryngol Otol 1996 Nov;110(11):1057-1059.
  12. Graham JM Jr, Edwards MJ. Teratogen update: gestational effects of maternal hyperthermia due to febrile illness and resultant pattern of defects in humans. Teratology 1998 Nov;58 (5):209-221.
  13. Wadhwani P, Mohammad S, Sahu R. Oromandibular limb hypogenesis syndrome, type IIA, hypoglossia-hypodactylia: a case report. J Oral Pathol Med 2007 Oct;36(9):555-557.
  14. Gupta S. Isolated aglossia congenital: a rare case of oromandibular limb hypogenesis syndrome type I B. J Oral Maxillofac Pathol 2012 Sep-Dec;16(3):414-419.
  15. Weingarten RT, Walner DL, Holinger LD. Tongue hypoplasia in newborn. Int J Pediatr Otorhinolaryngol 1993 Jan;25(1-3): 235-241.
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