Kapur R, Kapur R, Sheikh S, Jindal S, Kulkarni S
Department of Pedodontia and Preventive Dentistry, MM College of Dental Sciences and Research, Mullana, District Ambala, Haryana 133 203, India.
J Indian Soc Pedod Prev Dent. 2008 Jan;26 Suppl 1:S34-40.
Hemifacial microsomia is a congenital malformation in which there is a deficiency in the amount of hard and soft tissue on one side of the face. It is primarily a syndrome of the first branchial arch, involving underdevelopment of the temporomandibular joint, mandibular ramus, masticatory muscles and the ear. The affected ear may have an external soft-tissue malformation in addition to being lower set than on the contra lateral side. Hearing loss may result from underdevelopment of the osseous components of the auditory system and a diminished or absent external auditory meatus. Occasionally, second branchial arch defects involving the facial nerve and facial muscles coexist with Hemifacial microsomia. Radiographic examination in case of Hemifacial microsomia is of limited value because of superimposition of normal and abnormal bony structures. The skeletal and soft-tissue findings of a patient with Hemifacial microsomia who underwent three-dimensional computerized tomography is presented here to improve our knowledge and diagnostic skill of this uncommon entity.
半侧颜面短小畸形是一种先天性畸形,其面部一侧的软硬组织量存在不足。它主要是第一鳃弓综合征,涉及颞下颌关节、下颌支、咀嚼肌和耳朵发育不全。患侧耳朵除了比健侧耳朵位置低外,还可能有外部软组织畸形。听力损失可能源于听觉系统骨成分发育不全以及外耳道变窄或缺失。偶尔,涉及面神经和面部肌肉的第二鳃弓缺陷与半侧颜面短小畸形共存。由于正常和异常骨结构的重叠,半侧颜面短小畸形患者的影像学检查价值有限。本文展示了一名接受三维计算机断层扫描的半侧颜面短小畸形患者的骨骼和软组织检查结果,以增进我们对这种罕见病症的认识和诊断技能。