Right congenital diaphragmatic hernia: an 18-year experience.
ABSTRACT Prenatal diagnosis and outcome of right congenital diaphragmatic hernia (RCDH) are far less well known than the more common left CDH (LCDH). In addition, onset of RCDH beyond the neonatal period with a spectrum of atypical symptoms is not unusual. A retrospective analysis of RCDH treated at a single center over 18 years has been reviewed with regard to outcome after the introduction of a new treatment protocol for CDH.
All charts of patients with CDH between 1987 and 2004 were reviewed. Twenty-nine patients with RCDH were identified. The patients were divided into 2 historical groups: group 1, from 1987 to 1998 (16 patients), and group 2, from 1999 to 2004 (13 patients). From 1999, at the Department of Pediatric Surgery of Padua, the management of a baby born with CDH has been standardized and includes planned delivery at term, high-frequency oscillatory ventilation since birth, inhaled nitric oxide if required, extracorporeal membrane oxygenation as a "last resort," and delayed repair once the infant is hemodynamically stable. Patients with either prenatal diagnosis of RCDH or early onset of symptoms (<6 hours of life) were considered "high risk," and those with late onset (>6 hours) were considered "low risk."
Of 29 patients, prenatal diagnosis was available in 8 (27.5%) and major associated malformations in 8 patients (27.5%). Eight (50%) of 16 cases from group 1 and 9 (69.2%) of 13 cases from group 2 were high-risk patients. There was a trend in favor of a higher survival among high-risk patients from group 2 (25% vs 44%), although this was not statistically significant. As expected, all low-risk patients survived (P = .0001). Plain thorax x-ray was diagnostic in 23 (82.1%) cases, initially normal in 3, not performed in 1, and misinterpreted as right lower lobe pneumonia in 2. At operation, the prosthetic patch was required in 2 (9%) of 22 cases and the peritoneal sac was found in 4 (13.7%).
(1) The rate of prenatal diagnosis of RCDH was low and remained stable throughout the examined period. (2) The introduction of a treatment protocol, using high-frequency oscillatory ventilation since birth, improved the survival of high-risk patients with RCDH, although the data did not reach statistical significance. (3) The majority (75%) of low-risk patients presented beyond the first week of life with a variety of aspecific gastrointestinal or respiratory symptoms that accounted for initial misdiagnosis. (4) Even in these cases, the outcome was excellent.
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ABSTRACT: Despite advances in therapeutic modalities, congenital diaphragmatic hernia (CDH) still accounts for significant neonatal mortality. This study aimed to describe the demographic features, clinical experiences of postnatal care, and differences between non-survivors and survivors with CDH. We retrospectively reviewed medical records of neonates with CDH admitted to Kaohsiung Chang Gung Memorial Hospital over a 21-year period. Neonates with diaphragmatic eventration and those transferred after surgery were excluded. A total of 24 live-born neonates fulfilled the study criteria; 13 (54%) were boys and 11 (46%) were girls. Eight (33%) patients were prenatally diagnosed. The mean gestational age was 38.8 +/- 1.8 weeks (range, 35-41 weeks). Twenty-three (96%) had Bochdalek hernia [19 (83%) left-sided, 4 (17%) right-sided], and one (4%) had right-sided Morgagni hernia. Additional major congenital anomalies were identified in five patients (21%). The overall mortality was 21% (5/24); all deaths occurred before surgery. Statistically significant differences between survivors and non-survivors were found for right-sided CDH, low 1-minute and 5-minute Apgar scores, and low pH of the first arterial blood gas. Deaths were attributed to severe persistent pulmonary hypertension, unresponsiveness to aggressive resuscitation at birth, and major associated malformations. Seventy-nine percent of our CDH patients survived to hospital discharge. Resuscitation by a skilled neonatology team to prevent low Apgar scores and low pH, careful evaluation of other anomalies, and overcoming pulmonary hypertension might improve the survival rate. Recognizing unfavorable factors in CDH may help clinicians manage the critical care of these babies.Pediatrics & Neonatology 04/2010; 51(2):97-102. · 0.93 Impact Factor
- Pediatric Critical Care Medicine 01/2012; 13(1):103-4. · 2.35 Impact Factor
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ABSTRACT: To systematically investigate the impact of the location of the defect in congenital diaphragmatic hernia on neonatal mortality and morbidity with a special focus on survival at discharge, extracorporeal membrane oxygenation requirement, and the development of chronic lung disease. Retrospective tertiary care center study with a matched-pair analysis of all fetuses that were treated for congenital diaphragmatic hernia between 2004 and 2009. A specialized tertiary care center for fetuses with congenital diaphragmatic hernia. Complete sets of data were available for 106 patients with congenital diaphragmatic hernia. For 17 of 18 infants with right-sided congenital diaphragmatic hernia we were able to allocate infants with left-sided congenital diaphragmatic hernia with no relevant difference in previously described prognostic factors, such as pulmonary hypoplasia and liver herniation. None. There was a strong trend toward better survival in infants with right-sided congenital diaphragmatic hernia than with left-sided congenital diaphragmatic hernia (94% vs. 70%; p = .07). More neonates with left-sided congenital diaphragmatic hernia died of severe pulmonary hypertension despite extracorporeal membrane oxygenation. Fewer neonates with right-sided congenital diaphragmatic hernia died, yet higher degrees of pulmonary hypoplasia and oxygen requirement were observed despite extracorporeal membrane oxygenation. In congenital diaphragmatic hernia, the location of the defect has a substantial impact on postnatal survival and the development of chronic lung disease. In left-sided congenital diaphragmatic hernia, pulmonary hypertension resistant to therapeutic management, including extracorporeal membrane oxygenation, is more common and is associated with a higher rate of neonatal demise. Right-sided congenital diaphragmatic hernia infants have an increased benefit from extracorporeal membrane oxygenation but the better survival entails a higher rate of chronic lung disease.Pediatric Critical Care Medicine 04/2011; 13(1):66-71. · 2.35 Impact Factor