VACTERL 연관에서 동반된 결장폐쇄의 1예
Colon atresia, a rare comorbid condition in VACTERL association
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척추장애, 직장항문기형, 심장기형, 기관식도기형, 신장기형, 사지장애 등 여섯 개의 선천기형 중 세 개 이상일 때 정의할 수 있는 vertebral,anal, cardiac, tracheoesophageal, renal and limb (VACTERL) 연관은 환아에 따라 임상양상이 다양하다. 산전 초음파상 태아복부에 결장폐쇄 의심 종괴가 관찰되었으며 환아는 단일제대동맥, 반척추뼈증, 비골형성저하, 심실중격 결손 및 동맥관 개존, 결장폐쇄를 가지는VACTERL연관의 임상 증상을 나타내었다. 출생 후 결장폐쇄의 치료를 위해 총 2차례의 소아외과 수술을 시행하였다. 본 저자는 VACTERL 연관에서 매우 드물게 동반되는 결장폐쇄를 경험하였으며 타과와의 적절한 협진을 통해 빠른 진단 및 치료를 할 수 있었다.
Abstract
Vertebral, anal, cardiac, tracheoesophageal, renal and limb (VACTERL) association is defined by the presence of at least three of the following six congenital malformations: vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies and limb abnormalities. The abdominal cystic mass, suspicious of colon atresia was identified on prenatal ultrasonography. The new born infant exhibited single umbilical artery, hemivertebra, hypoplastic nasal bone, ventricular septal defect, patent ductus arteriosus, colon atresia. For treatment of colon atresia, two staged surgery was provided by a pediatric surgeon. We experienced colon atresia, a very rare comorbid condition in VACTERL association. Prompt diagnosis and surgical correction was possible with the cooperation of pediatric surgeons, obstetricians and pediatricians.
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