Vertical Expandable Rib-based Distraction Device for Correction of Congenital Scoliosis in Children of 3 Years of Age or Younger: A Preliminary Report.

BACKGROUND: This study was designed to evaluate the treatment outcome of very young children with congenital scoliosis aged 3 years or under after surgery with a vertical expandable prosthetic titanium rib (VEPTR)-based distraction device. METHODS: A retrospective study of 13 children undergoing implantation of a vertical expandable rib-based distraction device. From September 2007 to June 2018, 13 children (7 male and 6 female patients) with congenital scoliosis were followed after treatment with a VEPTR. The outcome parameters were complications, thoracic height, kyphosis, lordosis, and coronal major scoliosis curve. In addition, the American Society of Anaesthesiologists (ASA) score, assisted ventilation rating, and hemoglobin and body mass index were analyzed. Data were examined separately by 2 investigators. RESULTS: The mean age at initial surgical treatment was 24.4±10.6 months, follow-up was 91.5±23.1 months. The mean number of surgical procedures per patient was 14±3.9 (total 182 operations). Apart from planned operations every 6 months, 5 unplanned operations (2.7%) were performed. The major scoliosis curve improved significantly from 55.2±21.9 degrees to 40.5±18.7 degrees, thoracic spine height T1-T12 significantly from 111±12 mm to 137±23 mm, T1-S1 height significantly from 211±13 mm to 252±36 mm and space available for the lung from 80.9%±11.3% to 84.4%±8.8% (preoperatively and at 5-y follow-up, respectively). Kyphosis increased significantly from 30±21.4 degrees to 42.6±23.5 degrees and lordosis from 32.4±20.9 degrees to 37.6±10.4 degrees. Hemoglobin levels were virtually unchanged. Weight increased from 10.6±2.8 kg to 20.2±5.0 kg at final follow-up, but body mass index decreased from 16.6±3.7 kg/m² to 15.7±2.9 kg/m². CONCLUSIONS: The expandable rib-based distraction device is an effective method to treat severe congenital scoliosis in very young children. It can control the deformity and promotes spinal and thoracic growth. LEVEL OF EVIDENCE: Level IV.

Nerve transfer from the median to musculocutaneous nerve to induce active elbow flexion in selected cases of arthrogryposis multiplex congenita.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a rare disease which affects mainly upper and lower extremities. Affected patients are not able to eat unassisted due to elbow contracture and nonexistent active elbow flexion. In traumatic brachial plexus palsies, a nerve transfer from either median or ulnar nerve to the musculocutaneous nerve has proved to induce active elbow flexion, and we report our results of such a procedure in a nontraumatic condition, that is, arthrogryposis. METHODS: We selected four patients with AMC type 1 (6 extremities, 2 males, 2 females) diagnosed with AMC presenting to our institution shortly after birth from 2014 to 2016 to perform a nerve transfer from the median nerve to the musculocutaneous nerve in order to induce active elbow flexion. The indication of application of this surgical procedure was based on active finger and wrist flexion, limited contracture of elbow joints and evidence of flexing muscle fibers detected by sonography. RESULTS: Five nerve transfers were conducted with a follow up of 2-5 years. Two extremities reached active elbow flexion motorgrade M4, two M3, and one M1 at latest follow up. One patient developed a postoperative suture granuloma. One nerve transfer was abandoned due to neuroanatomic variation. One extremity was treated with botulinum toxin in triceps muscle in addition to the nerve transfer. CONCLUSIONS: In this series of selected cases of AMC Type 1 we were able to induce active elbow flexion using a nerve transfer technique developed for traumatic and obstetric brachial plexus palsies. In four extremities the procedure achieved independent hand-to-mouth active elbow flexion. Level of evidence four.