Working Zachary M, Hsiao Mark, Sanders John C, Bratton Susan L, D'Astous Jacques L
Departments of *Orthopaedic Surgery ‡Anesthesiology §Pediatrics, University of Utah, Salt Lake City, UT †Department of Orthopaedic Surgery, University of Texas Tech-El Paso, El Paso, TX.
J Pediatr Orthop. 2017 Jan;37(1):e4-e9. doi: 10.1097/BPO.0000000000000656.
Ehlers-Danlos syndrome (EDS) is a family of inherited connective tissue diseases. Kyphoscoliotic EDS (kEDS) is associated with severe and early spinal deformity. Very little has been reported regarding the orthopaedic surgical care of kEDS likely due to its rare incidence. A more common subtype is the vascular-type EDS (vEDS, previously labeled type IV), which is associated with reports of vascular complications in the literature.
The case report of a single, fatal complication of spontaneous rupture of the superior vena cava, after extubation, subsequent to revision minimal growing rod lengthening in a child with kEDS. We additionally review prior reported cases of kEDS for pediatric spine surgery and the sentinel event of spontaneous vascular rupture in all EDS patients.
The anterior thoracoabdominal approach in children with kEDS has been associated with severe intravascular complications, with no deaths reported to date. Posterior spinal procedures were associated with frequent overall complications, but no events of perioperative or spontaneous vascular injury were identified before our case.Reports of spontaneous vessel rupture (n=39) appear more frequently in vEDS, but have been documented in patients with EDS type I (classic EDS or cEDS) and kEDS disease. The 30-day mortality in these patients was 59%. The majority of the deaths occurred on the day of the vascular event. Surviving patients overwhelmingly received intraoperative consultation and treatment from either general or vascular surgeons at their respective institutions for assistance.
Providers should consider their proximity to available emergent consultation before operating on patients with EDS of any subtype.
Level V-expert opinion.
埃勒斯-当洛综合征（EDS）是一类遗传性结缔组织疾病。脊柱后侧凸型EDS（kEDS）与严重且早期的脊柱畸形相关。由于其发病率罕见，关于kEDS的骨科手术治疗的报道极少。一种更常见的亚型是血管型EDS（vEDS，先前标记为IV型），文献中有关于其血管并发症的报道。
报告1例kEDS患儿在改良式最小生长棒延长术后拔管时发生上腔静脉自发性破裂这一致命并发症的病例。我们还回顾了先前报道的kEDS患儿脊柱手术病例以及所有EDS患者自发性血管破裂的哨兵事件。
kEDS患儿采用胸腹前路手术已出现严重血管内并发症，迄今为止尚无死亡报告。后路脊柱手术总体并发症频发，但在我们的病例之前未发现围手术期或自发性血管损伤事件。自发性血管破裂的报告（n = 39）在vEDS中似乎更常见，但在I型EDS（经典型EDS或cEDS）和kEDS患者中也有记录。这些患者的30天死亡率为59%。大多数死亡发生在血管事件当天。存活患者绝大多数在各自机构接受了普通外科或血管外科医生的术中会诊和治疗以获得帮助。
在对任何亚型的EDS患者进行手术之前，医疗人员应考虑到距离可获得紧急会诊的地点的远近。
V级——专家意见。
