Crenshaw Molly M, Goerlich Cara G, Ivey Lauren E, Sapp Julie C, Keppler-Noreuil Kim M, Scott Allison C, Biesecker Leslie G, Tosi Laura L
National Institutes of Health/National Human Genome Research Institute, Bethesda, MD.
Children's National Health System, NW, Washington, DC.
J Pediatr Orthop. 2018 Mar;38(3):e138-e144. doi: 10.1097/BPO.0000000000001121.
Proteus syndrome (PS) is a rare mosaic disorder comprising asymmetric bony and soft tissue overgrowth leading to significant morbidity. Placement of growth inhibition hardware with subsequent epiphyseal arrest improves leg-length and angular deformities in pediatric patients without PS. The purpose of this study was to review the surgical approach and present outcomes, complications, and recommendations in 8 patients with PS and leg-length discrepancy (LLD).
We conducted a retrospective chart review of 8 patients with PS whose primary reason for surgery was LLD. Patients were eligible if they met clinical diagnostic criteria for PS and if the National Institutes of Health team performed at least 1 of their surgical interventions between 2005 and 2015. Surgical techniques included growth inhibition, with tension band plates, applied ≥1 times, and epiphyseal arrest.
Eight patients, followed for an average of 4.6 years (range, 1.0 to 7.1 y) after the index procedure, were included in this analysis. Average age at first LLD surgery was 9.4 years (range, 6.1 to 13.6 y); the average LLD was 3.4 cm (range, 0.4 to 7.0 cm) at presentation, and 5.0 cm (range, 1.8 to 10.0 cm) at the time of the first LLD surgery. Participants underwent 23 total surgeries (range, 1 to 5 per patient) and 7 patients have completed surgical intervention. For the 7 patients who did not require overcorrection the average LLD at the last clinical encounter was 2.6 cm (range, 0.6 to 7.2 cm). We encountered 2 complications: 2 patients developed mild knee valgus, which responded to standard guided growth techniques.
This case series suggests that growth inhibition and epiphyseal arrest in children with PS can reduce LLD with few complications. Careful monitoring, rapid mobilization, deep venous thrombosis prophylaxis, and sequential compression devices were also integral elements of our surgical protocol.
Level IV.
变形综合征（PS）是一种罕见的嵌合性疾病，表现为不对称的骨骼和软组织过度生长，导致严重的发病情况。对于没有PS的儿科患者，放置生长抑制器械并随后进行骨骺阻滞可改善腿长和角畸形。本研究的目的是回顾手术方法，并介绍8例患有PS和腿长差异（LLD）患者的手术结果、并发症及建议。
我们对8例因LLD而接受手术的PS患者进行了回顾性病历审查。如果患者符合PS的临床诊断标准，且美国国立卫生研究院团队在2005年至2015年期间至少进行了1次手术干预，则该患者符合入选标准。手术技术包括使用张力带钢板进行≥1次的生长抑制以及骨骺阻滞。
本分析纳入了8例患者，在索引手术后平均随访4.6年（范围为1.0至7.1年）。首次LLD手术时的平均年龄为9.4岁（范围为6.1至13.6岁）；初次就诊时的平均LLD为3.4厘米（范围为0.4至7.0厘米），首次LLD手术时为5.0厘米（范围为1.8至10.0厘米）。参与者共接受了23次手术（范围为每位患者1至5次），7例患者已完成手术干预。对于7例不需要过度矫正的患者，最后一次临床随访时的平均LLD为2.6厘米（范围为0.6至7.2厘米）。我们遇到了2例并发症：2例患者出现轻度膝外翻，采用标准的引导生长技术后得到缓解。
该病例系列表明，PS患儿的生长抑制和骨骺阻滞可减少LLD，且并发症较少。仔细监测、快速活动、预防深静脉血栓形成以及使用序贯加压装置也是我们手术方案的重要组成部分。
四级。
