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Pediatr Surg Int (2005) 21: 684686 DOI 10.1007/s00383-005-1443-4
CASE REPORT
V.unat Z. Stranak K. Pycha T. TlaskalJ. Melichar J. Miletn J. Janota J. Kuera P. Velebil
Congenital diaphragmatic hernia associated with esophageal atresia, tracheoesophageal stula, and truncus arteriosus in a premature newborn
Accepted: 22 January 2004 / Published online: 3 June 2005 Springer-Verlag 2005
Abstract The occurrence of coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal stula (TEF) is extremely rare and is considered highly lethal. The combination of CDH with EA/TEF and truncus arteriosus communis (TAC) has not been reported in the literature to date. The authors describe a premature neonate with this association.
Keywords Congenital diaphragmatic hernia Esophageal atresia Tracheoesophageal stula Truncus arteriosus
Introduction The incidence of congenital diaphragmatic hernia (CDH) ranges between 1:2,000 and 1:5,500 of liveborn infants. Although the mortality of newborns with CDH remains high, it is alarmingly high when CDH is diagnosed before the 24th week of gestation. The main factors responsible for the high mortality are pulmonary hypertension, pulmonary hypoplasia, and the severity of
associated congenital anomalies [4, 6]. Esophageal atresia (EA) and tracheoesophageal stula (TEF) together with CDH form an extremely rare combination of congenital malformations [10, 11], presenting a very serious clinical problem that is considered to be highly lethal [8, 10]. The combination of these malformations and truncus arteriosus communis (TAC) has not been described in the literature until now. Summary reports and perioperative ndings of CDH and EA, with and without TEF, are shown in Table 1.
Case reportA newborn (III/II, male, birth weight 1,650 g, gestational age 35 weeks and 6 days) with left-sided CDH diagnosed prenatally at the 26th gestational week was delivered spontaneously. Immediately after delivery, articial ventilation was introduced. Insertion of a nasogastric tube was unsuccessful. Contrast x-ray conrmed CDH and EA with TEF (Fig. 1). At 6 h after delivery, the TEF was ligated via a right thoracotomy, followed by EA repair (end-to-end). In addition, the CDH was repaired through a left subcostal abdominal incision. There were no complications during surgery or the early recovery period. The child was extubated after 10 days. Echocardiography revealed the presence of a hemodynamically compensated TAC. Screening for further possible congenital defects was negative, and his karyotype was...