Kalaskar Ritesh Rambharos, Godhane Alkesh, Kalaskar Ashita, Demble Swati
Associate Professor, Department of Pediatric Dentistry, Government Dental College and Hospital, Nagpur, Maharashtra, India.
Assistant Professor, Department of Pediatric Dentistry, Government Dental College and Hospital, Nagpur, Maharashtra, India.
Int J Clin Pediatr Dent. 2016 Jan-Mar;9(1):78-81. doi: 10.5005/jp-journals-10005-1338. Epub 2016 Apr 22.
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.
无舌畸形是一种罕见的先天性畸形，常作为一种孤立性疾病出现，或与其他先天性畸形，尤其是肢体缺陷同时存在。我们报告了一例独特的病例，一名7岁女童患有无舌畸形、指（趾）发育不全、小耳畸形、下颌后缩及V形下颌。其家族史显示母亲孕期曾接触药物。该患者存在进食困难、言语障碍、味觉及听力问题。本病例不符合霍尔对颌下颌肢体发育不全综合征（OLHS）的分类，该分类最常用于描述舌发育不全及肢体畸形。因此，本文旨在记录这种可纳入霍尔分类的OLHS罕见变异型。如何引用本文：卡拉卡尔RR，戈达内A，卡拉卡尔A，登布尔S。I B型颌下颌肢体发育不全综合征的一种罕见临床变异型。《国际临床儿科牙科学杂志》2016年；9(1):78 - 81。
