Management of spine deformity secondary to facioscapulohumeral dystrophy in pediatric patients. A case description and a literature review.

PURPOSE: The aim of this report is to describe the management of a severe spinal deformity in an adolescent with facioscapulohumeral dystrophy (FSHD) and review the available literature on the topic. METHODS: A 14-year-old patient with a genetically confirmed diagnosis of FSHD was evaluated for right thoracolumbar scoliosis (TL) and severe lumbar hyperlordosis. Spinal radiographs showed a right-sided curve of 32° and in the sagittal plane a lordotic curve T10-S1 -143°, TL junction -51.6°, LL -115°, pelvic incidence (PI) 25.5°, pelvic tilt 63.3°, PI-LL mismatch -90°, and a sagittal imbalance of -146 mm. An MRI scan evidenced atrophy of the paraspinal muscles. An instrumental gait analysis revealed significant pelvic anteversion associated with hip flexion and mild equinus. During follow-up, the patient developed a progressive inability to walk and difficulty sitting along with respiratory compromise and pain. RESULTS: At the age of 16 years, a posterior T2-iliac spinal fusion was performed using pedicle screws and four iliac anchors, with a 4-rod system placed at the lumbopelvic level. Significant correction of the hyperlordosis, the PI-LL mismatch, and sagittal imbalance was achieved, and the patient improved her sitting capacity, quality of life (QoL) and self-esteem and reported a decreased perception of disability at 2-year follow-up. CONCLUSIONS: This is the first published case of spinal deformity secondary to FSHD to use gait analysis to supplement the decision of optimal timing for surgery, and the second published case of spine surgery in a pediatric patient. Although spinal fusion surgery is controversial in ambulatory FSHD patients with extensive deformity, when ambulation is impaired, surgery improves function, prevents progression, and restores sagittal balance, increasing patient's QoL.

Case Report: Giant cell-rich osteosarcoma of the cervical spine in the pediatric age. A rare entity to consider.

Background: Although osteosarcoma is the most common primary malignant bone tumor in children, its location in the axial skeleton is rare, particularly at the cervical spine. Early diagnosis, together with multidisciplinary management, improves survival rates. Safe resection and stable reconstruction are complicated by the particular anatomy of the cervical spine, which raises the risks. Case Presentation: A 12-year-old male patient presented with cervical pain for several months and a recent weight loss of 3 kg. The complementary workup revealed a large destructive bone lesion in C7 with vertebral body collapse, subluxation, partial involvement of C6 and T1, large associated anteroposterior soft tissue components, and spinal canal narrowing. A biopsy suggested giant cell-rich osteosarcoma (GCRO). After 10 cycles of neoadjuvant chemotherapy, surgical resection was performed through a double approach: anterior, for tumoral mass resection from C6-7 vertebral bodies and reconstruction placing a mesh cage filled with iliac crest allograft plus anterior plate fixation; and posterior, for C7 complete and C6 partial posterior arch resection, thus completing a total piecemeal spondylectomy preserving the dura intact, added to a C5-T3 posterior fusion with screws and transitional rods. Postoperative chemo and radiotherapy were administered. Clinical and radiological follow-up showed disease-free survival and no neurological involvement at 3 years. Conclusion: An extensive review of the literature did not find any published cases of GCRO of the cervical spine in pediatric patients. This can be explained by the combination of three peculiar conditions: its location at the cervical spine region, the young age, and the GCRO variant.

Surgical Treatment for Advanced Thoracic Spinal Tuberculosis in Infants: Case Series and Literature Review.

BACKGROUND: Spinal tuberculosis (TB), or Pott's disease, is the most common form of osteoarticular TB. Early diagnosis and anti-TB drug therapy are the mainstays of treatment. However, in advanced stages, surgery is essential to correct spinal deformities and avoid neurological damage. Very few cases in young children requiring surgical treatment have been described. We present 2 cases of surgically treated thoracic spinal TB in patients under 2 years of age and review the literature on this entity in young children. CASE DESCRIPTION: Two male toddlers, aged 21 (patient 1) and 23 (patient 2) months, were admitted due to insidious systemic deterioration associated with neck stiffness and nonspecific abdominal pain in patient 1 and limping in patient 2. The findings of laboratory tests were unremarkable despite an increase in acute-phase reactants. After an extensive workup, results of a magnetic resonance imaging were consistent with spinal TB in both patients. Patient 1 underwent costotransversectomy of the left third and fourth ribs, debridement, T3-T4 corpectomy, and T2-T6 posterior fusion, to which an anterior rib autograft was added. Patient 2 had a right-sided thoracotomy performed at the apex in addition to debridement and T8 corpectomy; the resected rib was used as anterior structural autograft. Several samples of purulent, caseous material were sent for study. On follow-up, progression of proximal junctional kyphosis was evidenced in patient 1, requiring revision surgery to restore sagittal alignment; the patient was asymptomatic at 2-year follow-up. Patient 2 evolved satisfactorily without neurological sequelae and was asymptomatic at 4-year follow-up. CONCLUSIONS: Spinal TB in young children requires appropriate surgical treatment when presenting at advanced stages or having severe deformity or neurological compromise. Despite the challenges associated with young age and ongoing growth, surgery can be performed safely provided proper surgical expertise. After extensive debridement and decompression, priority should be given to restoring sagittal alignment and balance with stable and durable spinal reconstructions.

Variations in the position of the aorta and vertebral safe zones in supine, prone, and lateral decubitus for adolescent idiopathic scoliosis.

PURPOSE: Analyze the modifications in the "safe zones" of T4-L4 vertebral bodies relative to aorta according to patient positioning, as well as in the relationship between the aorta and the vertebrae. METHODS: Patients with adolescent idiopathic scoliosis who underwent surgical treatment during 2017 were included. Preoperative whole spine MRI in supine, prone, and LD positions was performed. The safe zone right (SZR) was defined as the angle formed between X-axis (0º) and a line connecting the origin and the edge of the aorta, and the safe zone left (SZL) was the angle between the edge of the aorta to 180º (X-axis). RESULTS: A total of 21 patients were studied, median age was 15.2 years, and 71.4% were female. The mean SZR lied from 0°-86.1º at T4 to 0°-76.9º at L4 in supine, from 0°-84.05º at T4 to 0º-78.5º at L4 in prone, and from 0° to 91.75º at T4 to 0°-80.4º at L4 in LD. While the mean SZL was located from 155.4º-180º at T4 to 107.9º-180º at L4 in supine, from 134°-180° at T4 to 103.9°-180° at L4 in prone, and from 143.8º-180º at T4 to 106º-180º at L4 in LD. Statistically significant differences were found almost at all levels when comparing supine versus prone and LD. CONCLUSIONS: Patient positioning during spinal surgery significantly modifies T4-L4 vertebral safe zones relative to aorta. These variations should be taken into account when analyzing an MRI performed in supine if the patient is undergoing surgery in a different position, to avoid vascular-related injuries.