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Case Reports
. 2016 Jan-Mar;9(1):78-81.
doi: 10.5005/jp-journals-10005-1338. Epub 2016 Apr 22.

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

Ritesh Rambharos Kalaskar  1 , Alkesh Godhane  2 , Ashita Kalaskar  3 , Swati Demble  4
Affiliations
Case Reports

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

Ritesh Rambharos Kalaskar et al. Int J Clin Pediatr Dent. 2016 Jan-Mar.

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.

Keywords: Aglossia; Environmental factors; Hypodactyli; Microstomia; OLHS; Rudimentary ears..

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Conflict of interest statement

Source of support: Nil

Conflict of interest: None

Figures

Fig. 1:
Fig. 1:
Extraoral photograph showing long narrow face, tapering chin, rudimentary ears, microstomia, and retrognathic mandible
Fig. 2:
Fig. 2:
Extraoral photograph showing rudimentary ear and convex profile
Figs 3A to C:
Figs 3A to C:
Intraoral photograph showing: (A) aglossia, (B) carious maxillary teeth, and (C) V-shaped mandible and carious teeth
Fig. 4:
Fig. 4:
Radiograph of the hand showing hypoplastic first metacarpal of left hand with nonformation of first carpometacarpal joint
Fig. 5:
Fig. 5:
Radiograph of the skull (anteroposterior and lateral view) showing hypoplasia of the maxillary sinus, hypoplasia of the mandibular arch and V-shaped mandible
See this image and copyright information in PMC

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