Roman Pat, Lypka Michael
Children's Mercy Hospital, Kansas City, MO.
J Craniofac Surg. 2017 Mar;28(2):e126-e127. doi: 10.1097/SCS.0000000000003668.
Laurin-Sandrow syndrome (LSS) is a rare autosomal disorder characterized by polysyndactyly of the hands and feet in a mirror fashion, absence of the radius and tibia with duplicated ulna and fibula, and nasal anomalies. Nasal defects are varied, and range from hypoplastic nasal skeleton to redundant nasal tissue, along with abnormalities of nasal subunits. Only 14 patients of LSS have been described in the literature. The authors present a unique case of a newborn with LSS and anterior nasal stenosis, resulting in respiratory failure. Early surgical intervention to relieve the bony and soft tissue overgrowth of the anterior nasal vault was required to allow for successful extubation.
劳林-桑德罗综合征(LSS)是一种罕见的常染色体疾病,其特征为手足呈镜像多并指(趾)畸形、桡骨和胫骨缺如伴尺骨和腓骨重复,以及鼻畸形。鼻缺陷多种多样,从鼻骨发育不全到鼻组织冗余,同时伴有鼻亚单位异常。文献中仅描述了14例LSS患者。作者报告了1例患有LSS和前鼻孔狭窄的新生儿的独特病例,该患儿出现呼吸衰竭。需要早期手术干预以减轻前鼻穹窿的骨和软组织过度生长,从而成功拔管。