Spine Unit, Department of Orthopaedic Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Spine (Phila Pa 1976). 2013 Jan 15;38(2):E94-100. doi: 10.1097/BRS.0b013e31827a7566.
Prospective patient series.
To evaluate outcome of children experiencing severe osteogenesis imperfecta (OI) with thoracic insufficiency syndrome (TIS), who were treated with a novel expandable spinothoracic fixation device.
Current treatment options for TIS in patients with OI are limited. As in other patients with TIS, thoracic elongation is the treatment of choice; however, the use of vertical expandable prosthetic titanium rib device is contraindicated for patients with poor bone quality.
From 2007 to 2009, 4 patients with type III OI with TIS, aged 8 to 12 years, were treated with a novel expandable spinothoracic fixation device. Pre- and postoperative mobility, Cobb angle, PO2, PCO2, forced vital capacity, forced expiratory flow, use of accessory muscles when breathing, body weight, complication rates, and patient satisfaction were assessed.
Prior to surgery, 2 patients were wheelchair bound and 2 walked with assistive devices. Average follow-up was 24 months (range, 14-36 mo). Mean Cobb angle improved by up to 32% in the coronal plane. Pulmonary function improved in all patients, with mean increases of 45% in forced vital capacity, 93% in forced expiratory flow, and 43% in PO2. PCO2 declined 30% on average, returning to normal values. Mean patient weight at 6-month follow-up increased by 10.9%. Patients moved from less than 3rd weight percentile per age group to the 3rd to 7th percentiles at the 6-month follow-up, with continuing weight gain. There were no major complications. All patients and their caregivers were satisfied that the procedure led to improved self-image and functional level.
Thoracic elongation improved pulmonary function, facilitated weight gain, and made an indirect correction of spinal deformities (Cobb angle) by more than 30%, with no significant complications. Because of the rarity of this condition, a multicenter study to gain experience in a larger number of patients is recommended.
前瞻性患者系列研究。
评估采用新型可扩张胸脊柱固定装置治疗严重成骨不全症（OI）伴胸壁发育不全综合征（TIS）患儿的结果。
目前，OI 伴 TIS 患者的治疗选择有限。与其他 TIS 患者一样，胸壁延长是首选治疗方法；然而，对于骨质量差的患者，使用垂直可扩张假体钛肋骨装置是禁忌的。
2007 年至 2009 年，4 例 3 型 OI 伴 TIS 的患儿，年龄 8 至 12 岁，采用新型可扩张胸脊柱固定装置治疗。评估术前和术后的活动能力、Cobb 角、PO2、PCO2、用力肺活量、用力呼气流量、呼吸时辅助肌肉的使用、体重、并发症发生率和患者满意度。
术前，2 例患儿坐轮椅，2 例患儿使用辅助器械行走。平均随访时间为 24 个月（范围，14-36 个月）。Cobb 角在冠状面最多改善了 32%。所有患者的肺功能均改善，用力肺活量平均增加 45%，用力呼气流量增加 93%，PO2 增加 43%。PCO2 平均下降 30%，恢复正常水平。6 个月随访时，患者平均体重增加 10.9%。患者从年龄组不到第 3 个体重百分位增加到 6 个月随访时的第 3 到第 7 个百分位，并持续增加体重。无重大并发症。所有患者及其家属均满意该手术可改善自我形象和功能水平。
胸壁延长可改善肺功能，促进体重增加，并使脊柱畸形（Cobb 角）间接矫正超过 30%，且无明显并发症。由于这种情况罕见，建议开展多中心研究，以在更多患者中积累经验。
