Congenital Diaphragmatic Hernia: Prognosis and Prenatal Detection

Abstract

Fifty consecutive cases of isolated congenital diaphragmatic hernia were reviewed for prenatal utlrasound and neonatal physical findings. These were compared to survival and the need for extracorporeal membrane oxygenation (ECMO). No prenatal findings predicted survival or the need for ECMO. In left-sided hernias, the presence of the liver in the chest predicted (p = 0.02) the requirement for ECMO. In a cohort of 13 prenatally diagnosed inborn cases, the ultrasound triad of polyhydramnios, mediastinal shift and intrathoracic stomach predicted the presence of liver in the chest (p < 0.03).

Outcome of congenital diaphragmatic hernia

“…35,36,41,51,53 The median overall mortality from these was 60% (range, 50-79%). Six studies presented data of outcome for a total of 98 fetuses/infants with associated anomalies of which 75 died.…”

“…Six studies presented data of outcome for a total of 98 fetuses/infants with associated anomalies of which 75 died. 3,35,36,42,50,51 The median percentage mortality was 93% (IQR, 75-100%).…”

“…9,13,22,23,32,[34][35][36]39,43,45,48,55,56 The median percentage mortality for all infants born alive and treated i n t h e s e c e n t e r s w a s 3 4 % ( I Q R , 2 6 -4 7 % ; mean, 39%; 95% CI, 30-48%). Twenty studies r e p o r t e d d a t a o n 5 3 6 b a b i e s t r e a t e d w i t h ECMO.…”

“…Twenty studies r e p o r t e d d a t a o n 5 3 6 b a b i e s t r e a t e d w i t h ECMO. 2,9,13,22,23,32,35,36,38,39,[43][44][45][47][48][49]55,56,58,60 Median percentage mortality for these ECMO-treated infants was 44% (IQR, 35-50%; mean, 42%; 95% CI, 34-50%). ECMO centers had a significantly lower mortality prior to going to the operating room among infants born alive when compared to tertiary centers (ECMO center's median mortality: 11%; IQR, 8-23%; tertiary center's median mortality: 43%; IQR, 35-48%; P ‫ס‬ 0.005).…”

The Use of Interphase Fish for Prenatal Diagnosis of Pallister–killian Syndrome

“…The mortality rate for prenatally diagnosed infants with isolated CDH is approximately 40 per cent and the prognosis is dependent on pulmonary development and function. Approximately 43 per cent of the fetuses have other anomalies and the mortality rate may be greater than 90 per cent in these cases (Pfleghaar et al, 1995). This may be due to the association between diaphragmatic hernias and cytogenetic abnormalities, with estimates ranging from approximately 4 per cent to greater than 30 per cent (Benacerraf and Adzick, 1987;Eydoux et al, 1989;Thorpe-Beeston et al, 1989;Wenstrom et al, 1991;Sharland et al, 1992;Torfs et al, 1992;Halliday et al, 1994;Bollman et al, 1995;Hanna et al, 1996) with the most common being trisomies for 18, 21, 13, and Pallister-Killian syndrome.…”