Vanamo K, Peltonen J, Rintala R, Lindahl H, Jääskeläinen J, Louhimo I
Children's Hospital, University of Helsinki, Finland.
J Pediatr Surg. 1996 Jun;31(6):851-4. doi: 10.1016/s0022-3468(96)90152-9.
Between 1948 and 1980, 107 of 164 patients survived after repair of congenital diaphragmatic defects. To study chest wall and spinal deformities among these patients, 60 survivors (mean age, 29.6 +/- 9.0 years) underwent clinical examination, chest and spinal radiography, spirometry, and diffusing capacity measurements. Twenty-seven of these patients also had body pletysmography, xenon 133 radiospirometry, and a test for bronchial hyperreactivity. Chest asymmetry was present in 29 patients (48%). Eleven patients (18%) had pectus excavatum, and one had a pectus carinatum deformity. Anterior asymmetry, pectus deformities, and a flat chest were more common among the patients who initially had a large diaphragmatic defect. Two patients underwent anterior thoracoplasty because of their deformities. Sixteen patients (27%) had significant scoliosis (Cobb angle > or = 10 degrees). Scoliosis was more common in patients with ventilatory impairment than in those with normal lung function. One patient required spinal stabilization, one with severe kyphoscoliosis remains under observation, and a patient with moderate scoliosis was treated with a spinal brace. The results of this study indicate that chest wall deformities and scoliosis are common among adults with repaired congenital diaphragmatic defects. In most patients the deformity is mild, but some will require surgery. Surveillance until adulthood is appropriate.
1948年至1980年间，164例先天性膈缺损修复术后患者中有107例存活。为研究这些患者的胸壁和脊柱畸形情况，对60名幸存者（平均年龄29.6±9.0岁）进行了临床检查、胸部和脊柱X线摄影、肺活量测定以及弥散功能测量。其中27例患者还进行了人体体积描记法、氙133放射性肺通气测定以及支气管高反应性检测。29例患者（48%）存在胸部不对称。11例患者（18%）有漏斗胸，1例有鸡胸畸形。最初患有较大膈缺损的患者中，前侧不对称、胸壁畸形和平胸更为常见。2例患者因畸形接受了前胸廓成形术。16例患者（27%）有明显脊柱侧凸（Cobb角≥10度）。通气功能受损的患者比肺功能正常的患者更易出现脊柱侧凸。1例患者需要进行脊柱固定，1例重度脊柱后凸侧凸患者仍在观察中，1例中度脊柱侧凸患者接受了脊柱支具治疗。本研究结果表明，先天性膈缺损修复术后的成年患者中胸壁畸形和脊柱侧凸很常见。大多数患者畸形较轻，但部分患者需要手术治疗。进行至成年期的监测是合适的。
