Ohba Tetsuro, Oda Kotaro, Tanaka Nobuki, Masanori Wako, Endo Tomoka, Haro Hirotaka
Department of Orthopaedic Surgery, University of Yamanashi, Yamanashi, Japan.
J Neurosurg Case Lessons. 2021 Jun 7;1(23):CASE2175. doi: 10.3171/CASE2175.
Upper cervical spine instability is one of the most serious orthopedic problems in patients with Down syndrome. Despite the recent advancement of instrumentation techniques, occipitocervical fusion remains technically challenging in the very young pediatric population with small and fragile osseous elements.
A 27-month-old boy with Down syndrome was urgently transported to the authors' hospital because of difficulty in standing and sitting, weakness in the upper limbs, and respiratory distress. Radiographs showed os odontoideum, irreducible atlantoaxial dislocation, and substantial spinal cord compression. Emergency posterior occipitoaxial fixation was performed using O-arm navigation. Improvement in the motor paralysis of the upper left limb was observed from the early postoperative period, but revision surgery was needed 14 days after surgery because of surgical site infection. The patient showed modest but substantial neurological improvement 1 year after the surgery.
There are several clinical implications of the present case. It warns that Down syndrome in the very young pediatric population may lead to rapid progression of spinal cord injury and life crisis. This 27-month-old patient represents the youngest case of atlantoaxial instability in a patient with Down syndrome. O-arm navigation is useful for inserting screws into very thin pedicles.
颈椎上段不稳是唐氏综合征患者最严重的骨科问题之一。尽管近年来器械技术有所进步，但在骨质小且脆弱的低龄儿童人群中，枕颈融合术在技术上仍具有挑战性。
一名27个月大的唐氏综合征男孩因站立和坐立困难、上肢无力及呼吸窘迫被紧急送往作者所在医院。X线片显示齿突骨、不可复位的寰枢椎脱位及严重的脊髓受压。使用O型臂导航进行了急诊后路枕枢固定术。术后早期观察到左上肢体运动麻痹有所改善，但术后14天因手术部位感染需要进行翻修手术。术后1年患者神经功能有适度但显著的改善。
本病例有几点临床意义。它警示低龄儿童人群中的唐氏综合征可能导致脊髓损伤迅速进展及危及生命。这名27个月大的患者是唐氏综合征患者中寰枢椎不稳最年轻的病例。O型臂导航有助于将螺钉置入非常细的椎弓根。
