Mathews Chelsea S, Bumpass David B, McCullough Frances Lynn, McCarthy Richard E
University of Arkansas for Medical Sciences, 4301 W. Markham St., Little Rock, AR 72205, USA; Arkansas Children's Hospital, 1 Children's Way, Little Rock, AR 72202, USA.
University of Arkansas for Medical Sciences, 4301 W. Markham St., Little Rock, AR 72205, USA; Arkansas Children's Hospital, 1 Children's Way, Little Rock, AR 72202, USA.
Spine Deform. 2019 Jan;7(1):171-175. doi: 10.1016/j.jspd.2018.06.002.
Case report.
To describe use of expansion thoracoplasty (ET) for severe thoracic insufficiency syndrome (TIS) in an adolescent with severe spinal deformity.
ET is typically performed in young patients with TIS to increase chest cavity volume, improve alveolar expansion, and potentially improve alveolar proliferation. ET has not been well-described in adolescent patients with TIS.
A mature adolescent with previously treated myelokyphosis and sacral agenesis developed severe TIS with dependence on supplemental oxygen and noninvasive ventilation. She was treated with two-stage bilateral ET and vertical expandable prosthetic titanium rib (VEPTR) placement. Yearly pulmonary function testing (PFT) was performed over 7 years of follow-up.
Significant clinical pulmonary improvement was achieved and maintained at final follow-up, as the patient no longer required supplemental oxygen. Percentage predicted forced vital capacity (FVC) improved from 29% to 36%; percentage predicted forced expiratory volume-1 second (FEV1) improved from 30% to 36%.
This case demonstrates that improvement and stabilization of respiratory function can be achieved with instrumented ET in a skeletally mature adolescent with severe TIS and spinal deformity.
病例报告。
描述扩张胸廓成形术（ET）在一名患有严重脊柱畸形的青少年严重胸廓发育不全综合征（TIS）中的应用。
ET通常用于患有TIS的年轻患者，以增加胸腔容积，改善肺泡扩张，并可能促进肺泡增殖。ET在患有TIS的青少年患者中的应用尚未得到充分描述。
一名患有既往治疗过的脊髓后凸和骶骨发育不全的成熟青少年发展为严重TIS，依赖补充氧气和无创通气。她接受了两阶段双侧ET和垂直可扩张人工钛肋（VEPTR）植入术。在7年的随访期间每年进行肺功能测试（PFT）。
在最后一次随访时实现并维持了显著的临床肺部改善，因为患者不再需要补充氧气。预计用力肺活量（FVC）百分比从29%提高到36%；预计1秒用力呼气量（FEV1）百分比从30%提高到36%。
该病例表明，对于患有严重TIS和脊柱畸形的骨骼成熟青少年，通过器械辅助ET可实现呼吸功能的改善和稳定。
