Can we improve outcome of congenital diaphragmatic hernia?

Frontiers in Pediatrics

Objective: To report treatment strategies' evolution and its impact on congenital diaphragmatic hernia (CDH) outcome. Design: Registry-based cohort study using the CDH Study Group database, 1995-2013. setting: International multicenter database. Patients: CDH patients entered into the registry. Late presenters or patients with very incomplete data were excluded.

2012

The treatment of congenital diaphragmatic hernia (CDH) still represents a challenge, even for the specialised multidisciplinary teams in centres that provide treatment for CDH. Despite significant progress in the fields of pathophysiology, prenatal diagnosis, surgical techniques and intensive care, CDH is a disease still burdened with a high mortality. Due to the paucity of randomised studies, there are no standard guidelines for treatment. The present review looks at existing diagnostic and therapeutic principles based on the available literature.

PubMed, 2013

The treatment of congenital diaphragmatic hernia (CDH) still represents a challenge, even for the specialised multidisciplinary teams in centres that provide treatment for CDH. Despite significant progress in the fields of pathophysiology, prenatal diagnosis, surgical techniques and intensive care, CDH is a disease still burdened with a high mortality. Due to the paucity of randomised studies, there are no standard guidelines for treatment. The present review looks at existing diagnostic and therapeutic principles based on the available literature.

Seminars in Perinatology, 2004

Congenital diaphragmatic hernia (CDH) occurs in 1 of every 2,000 to 4,000 births and accounts for 8% of all major congenital anomalies. Recurrence risk for a subsequent pregnancy is estimated at 2%. The mortality rate for CDH when diagnosed antenatally, varies with fetal age and with the presence or absence of hydramnios and degree of pulmonary hypoplasia. The prognosis has improved dramatically in recent years, primarily due to advances in neonatal and surgical interventions. Neonatal survival rates with an antenatal diagnosis now exceed 80% in some centers. Treatment for infants with CDH reflects other pediatric surgical problems in that a majority of the clinical research that shapes treatment is retrospective in nature. Because CDH is a relatively rare disease, using a compilation of cases, such as the CDH database provides, greatly aids our understanding of this disease process. Moreover, the application of a quality assessment scale provides the practitioner with a knowledge base to critically evaluate the published retrospective data.

European Journal of Pediatric Surgery, 2015

Aim This study aims to define patterns in the management of congenital diaphragmatic hernia (CDH). Methods A total of 180 delegates (77% senior surgeons) from 44 (26 European) countries completed a survey at the 2014 European Pediatric Surgeons' Association meeting. Results Overall, 34% of the surgeons work in centers that treat < 5 cases of CDH/y, 38% work in centers that treat 5 to 10 cases/y, and 28% work in centers that treat > 10 cases/y. Overall, 62% of the surgeons work in extra corporeal membrane oxygenation (ECMO) centers and 23% in fetal surgery centers. Prenatal work up and delivery: 47% surgeons request prenatal magnetic resonance imaging, 53% offer karyotyping, 22% perform a fetal intervention, 74% monitor head-to-lung ratio, and 55% administer maternal steroids. Delivery is via cesarean section for 47% surgeons, at 36 to 38 weeks for 71% surgeons, and in a tertiary care center for 94% of the surgeons.

NeoReviews, 2011

Management of congenital diaphragmatic hernia (CDH) remains challenging. Despite advances in technologic and therapeutic strategies such as high-frequency mechanical ventilation, inhaled nitric oxide (iNO), and delayed surgical repair, the condition continues to carry a substantial mortality risk. In recent years, with increasing prenatal identification of fetuses affected by CDH, more effort has been directed toward strategies allowing in utero intervention, with the goal of improving survival. Unfortunately, a universally successful fetal treatment for CDH remains elusive. Over the past several decades, collective knowledge has markedly increased regarding the range of short- and long-term morbidities experienced by CDH survivors, and medical care has evolved as understanding of the disease has progressed. Affected patients demand and deserve a multidisciplinary approach, both in the inpatient arena and follow-up setting, with experts in neonatology, surgery, pulmonology, cardiolo...

Romanian Journal of Pediatrics

Congenital diaphragmatic hernia (CDH) is a structural birth defect that with varying degrees of severity that results in significant neonatal morbidity and mortality. CDH occurs in 1:2,500 live births, and despite the advances in prenatal diagnosis and neonatal intensive care, the mortality and morbidity rate in infants with CDH is significant. In this article, we tried to summarize the main modalities of diagnosis, prognosis and medical treatment in CDH cases, found in specialty literature.

Journal of Pediatric Surgery, 2006

Background: The impact of bgentle ventilationQ (GV) strategies on morbidity and mortality of patients with congenital diaphragmatic hernia (CDH) in our institution has not been determined. This study reviews the primary and secondary outcomes of our patients with CDH treated with the GV approach. Method: We performed a retrospective chart review of respiratory, neurologic, nutritional, and musculoskeletal morbidities in patients with CDH treated at a single institution between 1985 and 1989 with conventional ventilation (CV) compared with those treated from 1996 to 2000 with GV.

Background: Despite the advances in management, congenital diaphragmatic hernia (CDH) has continued to pose a significant challenge to paediatric surgeons. This is amplified in a setting like ours where there is a dearth of facilities to cope with the problem of CDH. This study was undertaken to highlight the peculiarities of the management of CDH in a poor resource setting. Methods: All confirmed cases of CDH were prospectively documented from 2003 till date. Results: Seven children were treated from 2003 till date. The diaphragmatic defect was on the left side in six (83.8%) and on the right side in one (17.7%). All the patients had primary closure of the defect without patch via an abdominal approach. The three patients presenting at birth died while the remaining four patients survived. Conclusion: With inadequate neonatal intensive care facilities, the severe early presenting CDH has a dismal prognosis. In contrast, the late presenting CDH poses more diagnostic challenges; but once identified and appropriate treatment instituted, it has an excellent prognosis. We recommend that physicians should include CDH in the differential diagnosis of patients with birth asphyxia and in patients with chronic respiratory symptoms with failure to thrive.

Journal of Pediatric Surgery, 2004

Background: There is considerable debate regarding the optimal management of congenital diaphragmatic hernia (CDH) in high-risk infants (those cases presenting with respiratory distress within 2 hours of birth or those diagnosed prenatally). The aim of this study was to analyze patient outcomes using a new treatment protocol for CDH in a tertiary care non-extracorporeal membrane oxygenation (ECMO) neonatal unit. Methods: The records of 78 consecutive neonates with CDH presenting to Bambino Gesù Children's Hospital from 1996 to 2001 were analyzed retrospectively. Of these infants, 70 highrisk patients were identified (prenatal diagnosis or respiratory distress requiring intubation and assisted ventilation within 2 hours after birth), regardless of associated anomalies, medical condition on presentation, or degree of pulmonary hypoplasia. A prenatal diagnosis was made in 46 of 70 (66%) patients. Associated lethal malformations were present in 6 of the children (8.5%). The patients were placed in 3 historical groups: