Expansion thoracoplasty and vertical expandable prosthetic titanium rib (VEPTR; Synthes Spine Co., West Chester, Pennsylvania, USA) implantation is a new method for the treatment of thoracic insufficiency syndrome and congenital spinal deformity in children. The longitudinal rib implant expands the thorax and indirectly corrects spinal deformity, thus allowing spinal, thoracic and probably lung growth. VEPTR has been used since 1989 in San Antonio, USA, and was introduced to Europe in 2002. This paper describes the preliminary experience with the European patients. Fifteen children with progressive scoliosis had a VEPTR implantation at a mean age of 6 years (11 months to 12 years). Nine children had thoracic insufficiency syndrome due to unilateral unsegmented bars (n = 4), absent ribs (n = 1), hemivertebrae (n = 2) or bilateral fused ribs (n = 2). Six children had severe thoracolumbar scoliosis and pelvic obliquity due to neuromuscular scoliosis. After VEPTR implantation, families and patients reported improvement of the thoracic insufficiency syndrome and better sitting abilities in the neuromuscular patients, as well as radical cosmetic improvement. There were three complications (skin breakage, lumbar hook displacement, rib fracture) after performing fifteen primary VEPTR implantations and 13 expansion surgeries in eight patients. Our experience suggests that expansion thoracoplasty and VEPTR implantation is a safe and efficient method for the treatment of thoracic insufficiency syndrome in young children with severe scoliosis.
Background: Progressive Early-Onset Scoliosis (EOS) in children may lead to surgical interventions with growthfriendly implants, which require repeated lengthening procedures in order to allow adequate growth. Quality of life was studied using the validated German version of the EOS-Questionnaire (EOSQ-24-G) in surgically treated EOS children with different lengthening modalities. Methods: EOSQ-24-G and the KINDL R questionnaire were given to families with EOS children who had been treated by either vertical expandable prosthetic titanium rib implants and repetitive lengthening surgeries every 6 months or children who had received a magnetically expansion controlled implant, which was externally lengthened every 3 months. Results were compared according to differences between the two tests, and with possible influencing factors such as surgical method, severity of scoliosis, relative improvement of curvature, etiology, weight, age, travelling distance, complications, ambulatory ability and others. Results: 56 children with an average curve angle of 69°corrected to 33°(52%; average age 5.6 yrs) answered the EOSQ-24-G and the KINDL R after an average follow-up of 3.9 years. Health-related quality of life (HRQoL) was not affected by the initial scoliosis correction, the number of surgeries or the implant type. However, there was a negative correlation with non-ambulatory status, complications during treatment and for children with a neuromuscular scoliosis. Conclusion: Using the validated EOSQ-24-G, no statistically significant differences were found between the group of children receiving repetitive surgeries and children with external lengthening procedures without surgery. However, results were influenced by the etiology, complication rate or ambulatory ability.
Spine-based fixation of magnetically controlled lengthening devices has been successfully performed for children with early-onset scoliosis. However, spinal manipulation may lead to ossifications, stiffness, and autofusion as previously described. To avoid these problems, a surgical technique combining bilateral externally controlled magnetic device implantation with a rib cradle and pelvic hook fixation was introduced by us in 2011. By using a bilateral single-rib or double-rib cradle fixation and a pelvic hook, the magnetic device is percutaneously inserted. The spine corrects indirectly without further manipulation. In small rib diameter or severe osteoporosis, double-rib cradles are used. Our introduced technique enables modification of the rib vertebral angle, which may be beneficial in children with spinal muscular atrophy and rib-cage deformity. This nonrandomized prospective study describes 18 children with neuromuscular scoliosis treated first by this method. All patients followed the same protocol, with expansion procedures being performed 5 months after surgery and every 3 months thereafter. Clinical, radiologic, and complication data were analyzed, showing a significant reduction in scoliosis and pelvic obliquity and an increase in spinal length, which could be maintained over a follow-up period of 1.2 years. The overall complication rate was lower than previously described, with 8%, 2 of them requiring surgery.
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