Khanna A J, Braverman N E, Valle D, Sponseller P D
Department of Orthopaedic Surgery, The Johns Hopkins Hospital, Bayview Medical Center, 4940 Eastern Ave., Baltimore, MD 21224-2780, USA.
Am J Med Genet. 2001 Feb 15;99(1):63-6. doi: 10.1002/1096-8628(20010215)99:1<63::aid-ajmg1117>3.0.co;2-9.
Rhizomelic chondrodysplasia punctata (RCDP) is a rare peroxisomal disorder leading to multiple developmental malformations, including skeletal deformity. Specifically, involvement of the vertebral bodies has been described. Presented here is a case of a two-year-old female child with RCDP leading to advanced cervical stenosis as detected on magnetic resonance imaging (MRI) studies of the cervical spine. The practicing clinician should be aware of the possibility of cervical stenosis secondary to RCDP and its impact on the management of the patient with this rare disease process.
肢根型点状软骨发育不良(RCDP)是一种罕见的过氧化物酶体疾病,可导致多种发育畸形,包括骨骼畸形。具体而言,已有椎体受累的相关描述。本文报告一例两岁女童患有RCDP,颈椎磁共振成像(MRI)检查发现存在严重的颈椎管狭窄。临床医生应意识到RCDP继发颈椎管狭窄的可能性及其对这种罕见疾病患者治疗的影响。