Nishio Yumiko, Hiraki Teruyuki, Taniguchi Hiroko, Ushijima Kazuo
Department of Anesthesiology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka, 830-0011, Japan.
JA Clin Rep. 2018;4(1):2. doi: 10.1186/s40981-017-0141-2. Epub 2018 Jan 5.
Cleidocranial dysplasia is a type of skeletal dysplasia, which is primarily characterized by delayed ossification of skeletal structures. It causes facial and oral abnormalities, resulting in difficult airway management and neuraxial anesthesia.
The patient was a 24-year-old primipara (height 138 cm, weight 42 kg) with a hypoplastic right clavicle, patent fontanelles, dental malalignment, and a high palate. She was diagnosed with cleidocranial dysplasia at birth, although gene examination has not been performed. The fetus was confirmed to have short limbs and large fontanelles during an examination performed at 28 weeks gestation, suspected to have cleidocranial dysplasia. The mother was scheduled for a cesarean section at 37 weeks and 1 day due to cephalopelvic disproportion. Preoperative radiography and magnetic resonance imaging revealed no vertebral and spinal abnormalities, which allowed combined spinal-epidural analgesia (CSEA) to be performed. The surgery was safely concluded under CSEA with no intraoperative respiratory or circulatory problems.
Patients with cleidocranial dysplasia exhibit facial, oral abnormalities, and often vertebral abnormalities. Imaging assessments before neuraxial anesthesia and careful preparation for airway management are required.
锁骨颅骨发育不全是一种骨骼发育异常疾病,主要特征为骨骼结构骨化延迟。它会导致面部和口腔异常,进而造成气道管理和椎管内麻醉困难。
该患者为一名24岁初产妇(身高138厘米,体重42千克),右侧锁骨发育不全,囟门未闭,牙齿排列不齐,且腭部较高。她出生时被诊断为锁骨颅骨发育不全,不过尚未进行基因检测。在妊娠28周的检查中,确认胎儿四肢短小且囟门较大,怀疑患有锁骨颅骨发育不全。由于头盆不称,母亲计划在孕37周零1天行剖宫产术。术前X线摄影和磁共振成像显示无椎体及脊柱异常,因此得以实施腰麻-硬膜外联合镇痛(CSEA)。手术在CSEA下安全完成,术中无呼吸或循环问题。
锁骨颅骨发育不全患者存在面部、口腔异常,且常伴有椎体异常。椎管内麻醉前需进行影像学评估,并仔细做好气道管理准备。