Congenital diaphragmatic hernia: onset of respiratory distress and size of the defect

Abstract

The purpose of this study was to evaluate the outcome in neonates with congenital diaphragmatic hernia (CDH) either presenting within the first 24 h of life or diagnosed prenatally. The study was particularly focused on the time of onset of respiratory distress and on the use of the Gore-Tex (GT) patch for diaphragmatic reconstruction. Records of 104 neonates with CDH were retrospectively reviewed. The data were analyzed by ANOVA, Kruskal-Wallis test or chi (2) test as appropriate. The result showed that the overall survival rate was 73.1% (76/104). Survival of operated neonates was 91.6% (76/83). Postnatally diagnosed neonates with the onset of respiratory distress within the first minute of life survived in 67%, with the onset between 2 and 10 min survived in 89%, whilst neonates with the onset of respiratory distress after l0 min survived in 100% (P = 0.007). Birth weight, gestational age, time of onset of respiratory distress and Apgar score significantly differed between survivors and nonsurvivors. Primary closure of the diaphragmatic defect was performed in 62 patients while the GT patch was used in 21 patients. The survival of patients with a large defect treated with a GT patch was lower (76.2 vs. 96.8%, P = 0.003). There was only one case of recurrence in our series with the GT patch. Survival depends on the time of onset of respiratory distress and size of the defect, both of which correlate with the degree of pulmonary hypoplasia. The term high-risk CDH is appropriate only for children with respiratory distress within the first 10 min of life and those diagnosed prenatally. The GT patch is a suitable material for the diaphragmatic reconstruction; we suppose that the recurrence is caused by incorrect attachment of the patch to the thoracic wall.

Congenital Diaphragmatic Hernia Presenting in a 7-Day-Old Infant

“…In one review of 104 patients diagnosed either antenatally or within the first 24 hours of life, 18 infants were found to have additional defects. The most common defect was congenital heart disease, followed by renal anomalies, spina bifida, Pierre-Robin syndrome, Fryns syndrome, esophageal atresia, omphalocele, and choledochal cyst [3]. In another case series of late-presenting CDH, 40% were diagnosed with mid-gut malrotation, 7% with multicystic kidney disease, and 7% with a spinal cord deformity [4].…”

“…Up to 80% to 95% of cases are diagnosed by antenatal ultrasound with many of the remaining cases diagnosed within the first 24 hours of life [2, 3]. Rarely, patients develop herniation through a diaphragmatic defect beyond the immediate newborn period.…”

Abnormalities in pulmonary function in infants with high-risk congenital diaphragmatic hernia

“…The management protocol has been described previously 10 and was identical for all patients. Extracorporeal membrane oxygenation (ECMO) was not used in any of the patients.…”

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

“…Despite advances in antenatal diagnosis and postnatal management, mortality rate remains stable around 40-50% [1][2][3][4]. It has been shown that overall survival rate is mainly determined by the rate of antenatal termination, the incidence of CDH associated anomalies, degree of pulmonary hypoplasia and pulmonary hypertension in the neonatal period [1,2,[5][6][7]. Reports of improved survival of CDH should be interpreted with caution, as variations in outcomes are more likely to be explained by case selection bias [2].…”