Kyphectomy for severe kyphosis with pyogenic spondylitis associated with myelomeningocele: a case report

Myelomeningocele is a defect of the neural tube that occurs during the first 3 to 4 weeks of human embryogenesis [1]. The pathologic features of myelomeningocele include a defect of the posterior bony elements of the spine and poor viable muscular covering, resulting in the exposure of the dural sac containing neural tissues. Eight to 28% of patients with myelomeningocele develop kyphosis [2-8], since the deficiency of posterior bony elements of the spine as well as lateral displacement of spinal extensor muscles resulted in the functional loss of spinal erection, forcing the spine into flexion [1,2,9]. The progressive kyphosis causes various disorders including respiratory insufficiency, trunk imbalance, bladder and bowel dysfunction, and refractory skin ulcers at the bony prominence of the kyphosis [1,2,5,7,8,10]. Since kyphosis progresses rapidly with skeletal growth and conservative treatments, bracing are rarely effective for preventing the progression of kyphosis, therefore, surgical treatments were performed during an infantile, juvenile or adolescent periods in the majority of cases. To our knowledge, only one adult patient (20 years old) who was surgically treated for hyperkyphosis was reported in 142 patients from ten previous articles [1-11]. We report a rare adult case of severe kyphosis associated with myelomeningocele that developed pyogenic spondylitis at the apex of kyphosis.A 32-year-old female suffering from a refractory skin ulcer on her back was referred to our hospital. She was surgically treated for myelomeningocele soon after birth and had also received a ventricle-atrial shunt at the age of 17 years and a ventricle-peritoneal shunt at the age of 19 years for hydrocephalus. She was unable to walk because of complete paraplegia below the level of T8. The ulcer had been treated twice with a musculocutaneus flap, at the ages of 11 and 31 years which failed.The ulcer was located at a bony prominence of the apex of the kyphosis (Figure 1). The cult