Concurrent right diaphragmatic hernia and esophageal atresia

Abstract

The occurrence of a coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula is extremely rare and is considered highly lethal. Only 19 cases of CDH with EA have been reported in the world literature to date. This is a very challenging clinical problem, and the neonate is likely to deteriorate rapidly. Such a case is reported with a successful outcome, probably the first survivor with a right CDH and EA. Management guidelines for such a case are discussed.

The co-occurrence of congenital diaphragmatic hernia, esophageal atresia/tracheoesophageal fistula, and lung hypoplasia

“…There are only about 20 individual case reports of EA/TEF with CDH in the literature (Ahmed, 1970;Gibon et al, 1978;Bowen, 1983;Rawlings et al, 1984;Udassin and Peleg, 1987;Takehara and Okada, 1993;Sapin et al, 1996;al-Salem et al, 1997;Thakral and Sajwani, 1998). The need for a very large population of monitored births to ascertain such rare cases is the probable reason for the lack of epidemiological studies of the combinations of these defects.…”

“…Although there are no epidemiological studies of the co-occurrence of CDH, EA/TEF, and LH, there are 11 case reports in the literature, as reviewed by Thakral and Sajwani (1998). A few cases are reported in epidemiological studies of CDH (Torfs et al, 1992) and of EA/TEF (Torfs et al, 1995), studies in which LH is assumed to be part of the Bochdalek hernia.…”

Congenital diaphragmatic hernia associated with esophageal atresia, tracheoesophageal fistula, and truncus arteriosus in a premature newborn

“…This protocol had to be changed after EA with TEF findings because the positive pressure ventilation can lead to uncontrolled bowel distension that worsens ventilation parameters and causes irreversible hypoxic damage [10]. The surgical approaches depend both on the type of the diaphragmatic hernia and on the presence or absence of TEF.…”

“…Diagnostic and therapeutic tools remain challenging medical problems [8,10]. In general, accurate prenatal diagnostic and new postnatal therapeutic approaches can lead to successful outcomes in these life-threatening cases of congenital anomalies.…”

“…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 fistula (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.…”