Lluna González J, Barrios Fontoba J E, Cavalle Garrido T, Gutiérrez San Roman C, Malo Concepción P, Carrasco Moreno J I, Mínguez Esteban J R, Tomás Collado E, Aparici Izquierdo R
Departamento de Cirugía Pediátrica, Hospital Infantil La Fe, Valencia.
Cir Pediatr. 1995 Jan;8(1):2-6.
Our experience in 12 cases of pediatric patients with scimitar syndrome is reported. Except for one, all of them presented cardiac or respiratory manifestations. The symptomatology was related to associated defects (3 atrial septal defects and 1 multiple peripheric pulmonary stenosis), degree of pulmonary hypoplasia, size of the right to left shunt and pulmonary hypertension. 3 patients underwent surgical treatment. One of them died during operation and the other two have had a good evolution. Nine patients with later respiratory manifestations have improved their condition progressively without surgical intervention. Therapeutic approach for patients with scimitar syndrome, respiratory manifestations and onset beyond the neonatal period, should be conservative.
本文报告了我们对12例小儿弯刀综合征患者的治疗经验。除1例外,所有患者均有心脏或呼吸系统表现。症状与相关缺陷(3例房间隔缺损和1例多发性外周肺动脉狭窄)、肺发育不全程度、右向左分流大小及肺动脉高压有关。3例患者接受了手术治疗。其中1例在手术中死亡,另外2例术后恢复良好。9例后期出现呼吸系统表现的患者未经手术干预病情逐渐好转。对于弯刀综合征、有呼吸系统表现且发病于新生儿期后的患者,治疗方法应保守。