Astur Nelson, Flynn John M, Flynn John M, Ramirez Norman, Glotzbecker Michael, van Bosse Harold J, Hoashi Jane S, d'Amato Charles R, Kelly Derek M, Warner William C, Sawyer Jeffrey R
*Campbell Clinic, Le Bonheur Children's Hospital, Memphis, TN †Children's Hospital ∥Shriners Hospital, Philadelphia, PA ‡Hospital de la Concepcion, San German, PR §Boston Children's Hospital, Boston, MA ¶Shriners Hospital, Portland, OR.
J Pediatr Orthop. 2014 Jan;34(1):8-13. doi: 10.1097/BPO.0b013e3182a00667.
Although arthrogryposis multiplex congenital (AMC) is a rare condition, rapidly progressive scoliosis is common in children with AMC. Only a limited number of studies characterize the nature of these curves, and even fewer describe surgical outcomes. To determine efficacy or rib-based distraction in these patients, we reviewed the outcomes of the use of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) device in what we believe is the first study to report this.
Search of the Chest Wall Spinal Deformity Study Group database identified 10 children with AMC and early-onset scoliosis who were treated with the VEPTR device at 6 different pediatric health centers. The 7 female and 3 male patients had their initial surgery at an average age of 5 years. Mean follow-up was 4.2 years.
The most common curve was from T5 to L2. After initial VEPTR insertion, the scoliosis decreased from a mean of 67 to 43 degrees (37% correction) and kyphosis from 65 to 48 degrees (29% correction). The mean proximal junctional kyphosis after initial insertion was 33 degrees. At final follow-up, scoliosis and kyphosis were 55 degrees (17% correction) and 62 degrees (8% correction), respectively. Spinal growth during the treatment period showed a mean T1-S1 increase of 4.2 cm or approximately 1 cm/y. In the 62 procedures performed over the course of the study period, 6 complications occurred in 4 patients: 3 infections, 2 rib failures, and 1 implant failure. Six patients had proximal junctional kyphosis of ≥45 degrees at the last follow-up.
In children with AMC, rib-based distraction using the VEPTR is an effective treatment method for controlling scoliosis and kyphosis and maintaining thoracic growth, but proximal junctional kyphosis remains a problem.
尽管先天性多发性关节挛缩症（AMC）是一种罕见疾病，但快速进展性脊柱侧凸在AMC患儿中很常见。仅有少数研究描述了这些脊柱侧凸曲线的特点，而描述手术结果的研究更少。为了确定在这些患者中基于肋骨撑开的疗效，我们回顾了垂直可扩展人工钛肋骨（VEPTR）装置的使用结果，我们认为这是第一项报告该结果的研究。
检索胸壁脊柱畸形研究组数据库，确定了10例患有AMC和早发性脊柱侧凸的儿童，他们在6个不同的儿科医疗中心接受了VEPTR装置治疗。7名女性和3名男性患者首次手术的平均年龄为5岁。平均随访时间为4.2年。
最常见的脊柱侧凸曲线是从T5到L2。初次植入VEPTR后，脊柱侧凸从平均67度降至43度（矫正37%），后凸从65度降至48度（矫正29%）。初次植入后近端交界性后凸的平均度数为33度。在最后一次随访时，脊柱侧凸和后凸分别为55度（矫正17%）和62度（矫正8%）。治疗期间脊柱生长显示T1-S1平均增加4.2 cm或约1 cm/年。在研究期间进行的62例手术中，4例患者出现了6例并发症：3例感染、2例肋骨断裂和1例植入物失败。6例患者在最后一次随访时近端交界性后凸≥45度。
在患有AMC的儿童中，使用VEPTR进行基于肋骨的撑开是控制脊柱侧凸和后凸以及维持胸廓生长的有效治疗方法，但近端交界性后凸仍然是一个问题。
