Congenital Diaphragmatic Hernia Survivors Research Articles

Sensorineural hearing loss in congenital diaphragmatic hernia survivors is associated with postnatal management and not defect size

Background/PurposeWe determined the incidence of sensorineural hearing loss (SNHL; >20dB at any frequency) in a contemporary cohort of congenital diaphragmatic hernia (CDH) survivors at a single tertiary care center and identified potential risk factors for SNHL. MethodsFrom 2000 through 2011, clinical and audiologic data were collected on 122 surgically-repaired Bochdalek CDH patients. CDH defect size, duration of ventilation, and cumulative aminoglycoside treatment were used for multivariate logistic regression. ResultsIncidence of SNHL was 7.4% (9/122). We identified one significant independent predictor of SNHL: cumulative length of aminoglycoside treatment (P<.001; OR 1.44, 95% CI: 1.13–1.83). ConclusionsOver the last decade, the incidence of SNHL in our CDH patients is low compared to previous reports in the literature (7.4%) and is not associated with CDH defect size or the need for extracorporeal membrane oxygenation. Prolonged duration of aminoglycoside treatment increases the risk of SNHL independent of defect size and duration of ventilation.

Incidence and factors associated with sensorineural and conductive hearing loss among survivors of congenital diaphragmatic hernia

PurposeThe reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution. MethodsWe retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher’s exact test and continuous variables by Mann–Whitney t-test (p≤0.05). ResultsA total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p=0.0130), prolonged course of hospitalization (p=0.0011), duration of mechanical ventilation (p=0.0046), requirement for tracheostomy (p=0.0013), and duration of loop diuretic (p=0.0005) and aminoglycoside therapy (p=0.0003). ConclusionsWe have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language.

Neurodevelopmental outcome in congenital diaphragmatic hernia: Evaluation, predictors and outcome.

To review the reported neurodevelopmental outcome of congenital diaphragmatic hernia (CDH) survivors, identify important predictors of developmental disabilities, and describe the pathophysiological mechanisms contributing to adverse outcome. A Medline search was performed for English-language articles cross-referencing CDH with pertinent search terms. Retrospective, prospective, and longitudinal follow-up studies were examined. The reference lists of identified articles were also searched. Neurodevelopmental dysfunction has been recognized as one of most common and potentially most disabling outcome of CDH. Intelligence appears to be in the low normal to mildly delayed range. Neuromotor dysfunction is common during early childhood. Behavioral problems, hearing impairment, and quality of life related issues are frequently encountered in older children and adolescence. Disease severity correlates with the degree of neurological dysfunction. Neurodevelopmental follow-up in CDH children should become standard of care to identify those who would benefit from early intervention services and improve neurological outcomes.

Antenatal maternally-administered phosphodiesterase type 5 inhibitors normalize eNOS expression in the fetal lamb model of congenital diaphragmatic hernia

PurposePulmonary hypertension (pHTN), a main determinant of survival in congenital diaphragmatic hernia (CDH), results from in utero vascular remodeling. Phosphodiesterase type 5 (PDE5) inhibitors have never been used antenatally to treat pHTN. The purpose of this study is to determine if antenatal PDE5 inhibitors can prevent pHTN in the fetal lamb model of CDH. MethodsCDH was created in pregnant ewes. Postoperatively, pregnant ewes received oral placebo or tadalafil, a PDE5 inhibitor, until delivery. Near term gestation, lambs underwent resuscitations, and lung tissue was snap frozen for protein analysis. ResultsMean cGMP levels were 0.53±0.11 in placebo-treated fetal lambs and 1.73±0.21 in tadalafil-treated fetal lambs (p=0.002). Normalized expression of eNOS was 82%±12% in Normal-Placebo, 61%±5% in CDH-Placebo, 116%±6% in Normal-Tadalafil, and 86%±8% in CDH-Tadalafil lambs. Normalized expression of β-sGC was 105%±15% in Normal-Placebo, 82%±3% in CDH-Placebo, 158%±16% in Normal-Tadalafil, and 86%±8% in CDH-Tadalafil lambs. Endothelial NOS and β-sGC were significantly decreased in CDH (p=0.0007 and 0.01 for eNOS and β-sGC, respectively), and tadalafil significantly increased eNOS expression (p=0.0002). ConclusionsPDE5 inhibitors can cross the placental barrier. β-sGC and eNOS are downregulated in fetal lambs with CDH. Antenatal PDE5 inhibitors normalize eNOS and may prevent in utero vascular remodeling in CDH.

Hernie de coupole diaphragmatique : devenir à long terme

The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.

Enteric Nervous System and Esophageal-Gastrointestinal Motility in Experimental Congenital Diaphragmatic Hernia

Gastroesophageal reflux and intestinal distension have been described in survivors of congenital diaphragmatic hernia (CDH). Deficient enteric innervation demonstrated in experimental models is a likely explanation for these symptoms. This study aimed at further characterizing these anomalies and examining esophageal and intestinal motility in this condition. Pregnant rats received either nitrofen or vehicle on E9.5. Sections of E15, E18, and E21 esophagus and small bowel were stained for protein gene product 9.5, nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase (NADPHd), and acetylcholinesterase (AChE). The proportion of neural tissue/muscle surface was measured and the NADPHd- and AChE-positive motor endplates (MEPs) were counted. E18 and E21 stomachs were stained for AChE, the ganglia were counted and measured. The peristalsis of the esophagus and small bowel was video recorded. The relative neural/muscle surface and the number of NADPHd- and AChE-positive MEPs were decreased on E15 and E18 in the esophagus and small bowel of embryos with CDH, but they tended to improve on E21. The number and the mean surface of stomach ganglia were smaller in E18 and E21 fetuses with CDH. Peristaltic movements were decreased in the esophagus and small bowel of animals with CDH. Deficient enteric innervation impaired gastrointestinal motility in experimental CDH. This could explain some long-term morbidity in the human condition.

Longitudinal neurodevelopmental and neuromotor outcome in congenital diaphragmatic hernia patients in the first 3 years of life

The objective of this study was to longitudinally evaluate the neurodevelopmental (ND) outcome in congenital diaphragmatic hernia (CDH) survivors during the first 3 years of life. The study cohort consists of 47 CDH survivors that were enrolled in our prospective, follow-up program between July 2004 and September 2010, and underwent serial ND evaluations during the first 3 years of life. ND outcomes were evaluated using the Bayley Scales of Infant Development (BSID)-II or BSID-III. Persistent ND impairment was defined as a score that remained 79 for the cognitive, language and psychomotor domains at the most recent follow-up visit compared with the first assessment. The median age at first and last evaluation was 8 (range, 5 to 15) and 29 (range, 23 to 36) months, respectively. During the follow-up, ND scores improved to average in 17%, remained average in 60%, remained delayed in 10%, improved from severely delayed to mildly delayed in 2% and deteriorated from average to delayed in 15%. Motor scores improved to average in 26%, remained average in 55%, remained delayed in 8% and improved from severely delayed to mildly delayed in 11%. Intrathoracic liver position (P=0.004), preterm delivery (P=0.03), supplemental O2 requirement at day of life 30 (P=0.007), age at discharge (P=0.03), periventricular leukomalacia (PVL; P=0.004) and initial neuromuscular hypotonicity (P=0.01) were associated with persistent motor delays. No relationship was found between patient's characteristics and the risk of persistent cognitive and language delays. (1) The majority of children with CDH are functioning in the average range by early preschool age, (2) most children who had early delays showed improvement in their ND outcome, (3) children showing delays in all the three domains were the least likely to show improvement and (4) CDH severity appears to be predictive of persistent psychomotor delays.

Hearing loss in congenital diaphragmatic hernia (CDH) survivors: Is it as prevalent as we think?

PurposeThe incidence of sensorineural hearing loss (SNHL;>20dB loss) in CDH survivors is debated. We evaluated long-term audiological outcomes at a single tertiary care center with ECMO capability and an established neonatal follow-up program. MethodsWith REB approval, records of CDH survivors from 2000 to 2010 were retrospectively analyzed. Demographic, postnatal, and audiometric information was gathered. All underwent auditory brainstem response (ABR) or otoacoustic emissions screening before discharge and complete audiological surveillance. Thirty-three patients were evaluated to age 4+ years with others continuing follow-up. ResultsForty-three patient records were reviewed with 1 excluded (transferred to another institution). Median GA and BW were 39weeks (35–41) and 3.1kg (2–4), respectively. Median ventilation days were 10 (2–189) with 34 infants ventilated 5+ days. Sixteen (36%) received HFOV, 21 (49%) iNO, and 5 (12%) ECMO. The median time to CDH repair was 3days (1–23), and 11 (26%) required patch repair. Nine infants (21%) received diuretics and oxygen after discharge. Audiological surveillance identified only one patient with SNHL (received HFO, iNO, and patch repair). ConclusionNeonatal screening identifies CDH survivors at risk for hearing difficulties but must be followed with comprehensive testing until school age. The incidence of SNHL may be less than previously reported in this population.

Late surgical outcomes among congenital diaphragmatic hernia (CDH) patients: Why long-term follow-up with surgeons is recommended

BackgroundCDH patients experience multi-system morbidity. Despite apparent health, late childhood complications do occur. We reviewed the long-term surgical morbidity of our CDH patients to determine whether protracted clinical surveillance is warranted. MethodsA single-institution retrospective chart review of all CDH survivors treated from 1999 to 2011 who are followed at our CDH multidisciplinary clinic was performed. Descriptive and statistical analyses were performed to show risk of surgical complications over time. ResultsA total of 187 CDH patients were treated with 160 surviving to discharge (86%). Primary repair was performed in 115 (73%), and 42 (27%) underwent patch repair. CDH recurrence occurred in 23 (15%) at a median time of 0.7 (range 0–8.5) years (65% asymptomatic). Seventy percent of recurrences occurred before 2years and 17% after 4years. Bowel obstruction occurred in 12 (8%) at a median time of 0.7 (range 0.2–7.2) years post-repair, and chest deformity occurred in 13 (8%) at a median of 5 (range 1.1–6.8) years. For patch repairs, scoliosis occurred in 4 (10%) patients at a median age of 3 (range 0.6–5) years. ConclusionSurgical complications in CDH survivors are common, can occur many years later, and are frequently asymptomatic. Long-term surveillance of CDH patients is recommended for early identification and treatment of complications.

Perinatal factors associated with poor neurocognitive outcome in early school age congenital diaphragmatic hernia survivors

ObjectiveDetermine predictors of neurocognitive outcome in early school age congenital diaphragmatic hernia (CDH) survivors. Study DesignProspective study of infants with CDH at Duke University Medical Center. Neurocognitive delay (NCD) at school age (4 to 7years) was defined as a score<80 in any of the following areas: Verbal Scale IQ, Performance Scale IQ, Expressive Language, or Receptive Language. Logistic regression, Fisher's exact, and the Wilcoxon rank sum test were used to examine the relationship between NCD at early school age and 6 demographic and 18 medical variables. ResultsOf 43 infants with CDH, twenty seven (63%) survived to hospital discharge, and 16 (59%) returned for school age testing at a median age of 4.9years. Seven (44%) of the children evaluated had NCD. Patch repair (p=0.01), extracorporeal membrane oxygenation (ECMO; p=0.02), days on ECMO (p=0.01), days of mechanical ventilation (p=0.049), and post-operative use of inhaled nitric oxide (p=0.02) were found to be associated with NCD at early school age. ConclusionsCDH survivors are at risk for neurocognitive delay persisting into school age. Perinatal factors such as patch repair and ECMO treatment may aid in identifying CDH survivors at high risk for continued learning difficulties throughout childhood.

Management of prenatally diagnosed congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a congenital anomaly that presents with a broad spectrum of severity that is dependent upon components of pulmonary hypoplasia and pulmonary hypertension. While advances in neonatal care have improved the overall survival of CDH in experienced centers, mortality and morbidity remain high in a subset of CDH infants with severe CDH. Prenatal predictors have been refined for the past two decades and are the subject of another review in this issue. So far, all randomized trials comparing prenatal intervention to standard postnatal therapy have shown no benefit to prenatal intervention. Although recent non-randomized reports of success with fetoscopic endoluminal tracheal occlusion (FETO) and release are promising, prenatal therapy should not be widely adopted until a well-designed prospective randomized trial demonstrating efficacy is performed. The increased survival and subsequent morbidity of CDH survivors has resulted in the need to provide resources for the long-term follow up and support of the CDH population.

Preschool neurological assessment in congenital diaphragmatic hernia survivors: Outcome and perinatal factors associated with neurodevelopmental impairment

ObjectiveTo evaluate the preschool neurodevelopmental outcome in CDH survivors between 2 and 5years of age and to identify perinatal and postnatal factors that may be predictive of persistent neurological deficits. MethodsThe study cohort consists of 60 CDH survivors that were enrolled in our prospective, follow-up program between January 2006 and September 2012. Developmental assessment of study participants between 2 and 3years of age was performed using the Bayley Scales of Infant Development, 3rd Edition (BSID-III). Cognitive outcomes of CDH children older than 3years of age were evaluated using The Wechsler Preschool and Primary Scale of Intelligence, 3rd Edition (WPPSI-III). Neurodevelopmental delay was defined by a score of ≤85 in any of the evaluated composite scores. Severe impairments were defined as a score of ≤69 in at least one domain tested. ResultsMean follow-up for children assessed by BSID-III (n=42) and WPPSI-III (n=18) was 28±4.5months and 58±4.0months, respectively. As a group, mean composite and subdomain BSID-III and WPPSI-III scores were within the expected normal range. For the BSID-III group, 9 (22%) had mild deficits in at least one domain, and 6 (14%) had severe deficits in at least one. Only 3 (7%) patients demonstrated severe neurological delays for all BSID-III composite scales. For the WPPSI-III group, 4 (22%) patients scored within the borderline-delayed range for at least one subscale and only one (6%) child had a WPPSI-III VIQ score in the severe delay range. Abnormal muscle tonicity was found in 35% (hypotonicity 33%, hypertonicity 2%). Need for ECMO, prolonged ventilation, hypotonicity, and other surrogate markers of disease severity (P<0.05) were associated with borderline or delayed neurological outcome. ConclusionThe majority of CDH children are functioning in the average range at early preschool and preschool age. Neuromuscular hypotonicity is common in CDH survivors. CDH severity appears to be predictive of adverse neurodevelopmental outcome.

Congenital Diaphragmatic Hernia with Lung Hypoplasia – A Cause of Sudden Death in a Neonate

Sudden unexpected deaths in neonates usually result from underlying natural disease. We report a case of death in a full term apparently healthy female neonate delivered normally at the -M.I.M.E.R. Medical College hospital, Talegaon, Pune, India, who cried after birth, developed cyanosis and died in a few minutes. The foetus was received in the Dept. of Anatomy, M.I.M.E.R. Medical College for embalming and dissection. Detailed examination and dissection revealed congenital diaphragmatic hernia (CDH) with bilateral lung hypoplasia in the newborn. With the advent of better antenatal diagnosis and neonatal care, the chances of survival in CDH have improved. However, there still remains a significant risk of death and complications in infants with CDH, primarily due to the associated lung hypoplasia. The insult to the lungs is now thought to be an independent, associated finding, due to a common genetic pathway involved in the development of the diaphragm and the lungs, rather than a cause-effect relationship. Keywords - Sudden death, neonate, congenital diaphragmatic hernia, lung hypoplasia, genetic pathway

Health-related quality of life and its determinants in children with a congenital diaphragmatic hernia

BackgroundThe development of new therapeutics has led to progress in the early management of congenital diaphragmatic hernia (CDH) in pediatric intensive care units (PICU). Little is known about the impact on the quality of life (QoL) of children and their family. The aim of this study was to assess the impact of CDH treated according to the most recent concepts and methods outlined above on child survivors’ QoL and their parents’ QoL.Patients and methodsThis study incorporated a cross-sectional design performed in two PICU (Marseille, France). Families of CDH survivors born between 1999 and 2008 were eligible. The following data were recorded: socio-demographics, antenatal history and delivery, initial hospitalization history. Self-reported data were collected by mail, including current clinical problems of the children (13-symptom list), children’s QoL (Kidscreen-27 questionnaire), and parents’ QoL (Short-Form 36 questionnaire). Children’s QoL score was compared with controls and QoL of survivors of childhood leukemia. Parent’s QoL was compared with controls. Non-parametric statistics were employed.ResultsForty-two families agreed to participate and questionnaires were completed by 32 of them. Twenty-one children had a current clinical problems related to CDH. All the QoL scores of CHD survivors were significantly lower compared with controls. The physical well-being dimension was significantly higher for CHD survivors compared with survivors of childhood leukemia. Gastro-esophageal reflux at discharge, antenatal diagnosis, length of stay in the PICU, and neuropsychological and respiratory issues significantly impacted QoL scores of children. The parents of CHD survivors had significantly poorer score in emotional role dimension compared with controls.ConclusionThe impact of CDH on QoL seems to be important and must be understood by clinicians who treat these children and their parents.

Using serial oxygenation index as an objective predictor of survival for antenatally diagnosed congenital diaphragmatic hernia

IntroductionBest oxygenation index on day 1 (BOId1) had been shown to predict survival in congenital diaphragmatic hernia (CDH). Serial oxygenation index (OI) may enable better assessment of response to cardiorespiratory support than BOId1. MethodsAll antenatally diagnosed CDH from one tertiary neonatal unit were retrospectively reviewed. Oxygenation index at 6, 12, 24, and 48 hours from birth, as well as BOId1, were compared between survivors and nonsurvivors. The area under the curve and receiver operating characteristic (ROC) curves were used to compare serial OI within the first 24 hours and BOId1 between survivors and nonsurvivors. Statistical significance was set at P < .05. ResultsTwenty-four patients with CDH (13 survivors, 11 nonsurvivors) were included. Both groups were comparable in demographics and variables that could affect outcome. In terms of nonsurvival, ROC curve analysis demonstrated a sensitivity of 78% for serial OI greater than 252 and 56% for BOId1 greater than 8.5, both having a specificity of 100%. The area under the ROC curve for serial OI and BOId1 were 0.96 and 0.85, respectively. The positive predictive value of serial OI (>252) and BOId1 (>11) for nonsurvival were both 100%, with an negative predictive value of 87% and 76%, respectively. ConclusionsOur preliminary study showed that serial OI in the first 24 hours of life is a good predictor of survival. It is simple to use and has the added advantage of assessing response to medical support in CDH. The results support the need for a large prospective study exploring the potential of serial OI to guide management and prognosis.

Mid-term differences in right ventricular function in patients with congenital diaphragmatic hernia compared with controls

Patients with congenital diaphragmatic hernia (CDH) may have abnormal lung development, which may cause detrimental effects on right ventricular (RV) function. This study aimed to determine if there are persistent echocardiographic differences in RV function in patients with CDH years after repair versus control patients. Patients who underwent repair for CDH were recruited. RV function was evaluated by strain analysis and tissue Doppler imaging (TDI). Wilcoxon's rank-sum test was used for analysis. Seven CDH patients and 16 control patients were studied. There was no difference in age between the CDH and control groups (6.2±1.7 years vs. 5.7±1.7 years). TDI demonstrated significantly lower values in the RV early diastolic wave (12.8±1.5 cm/s vs. 16.1±3.1 cm/s) and RV systolic wave (10.2± 0.8cm/s vs. 13.4±1.3 cm/s) when comparing the CDH group and the control group. Interventricular apical septal strain was signifi cantly lower in the CDH group than in the control group (-20.1±4.6% vs. -25.4±4.1%). There was a trend towards lower strain values in the RV mid-lateral segment in the CDH group (-30.8±9.9% versus -39.7±6.0%, P=0.06) and a lower global RV strain (-27.8±3.0% vs. -31.1±3.1%, P=0.06). Patients who underwent CDH repair continue to have differences in RV function years after repair. Follow-up is needed to determine how these differences impact cardiac function in adult survivors of CDH.

Morbidity and Long-Term Follow-Up in CDH Patients

Overall survival of congenital diaphragmatic hernia (CDH) patients has increased since the widespread implementation of delayed operative repair with extracorporeal membrane oxygenation/gentle ventilation strategies starting in the mid-1990s. With the improvements in survival, CDH survivorship has been marked by increased morbidity with a greater need for monitoring and supportive interventions in infancy and early childhood. The multisystem morbidity and complexity of care for these patients mandate the dedicated resources and focus of medical specialties to ensure that the neurodevelopmental, cardiopulmonary, nutritional, and surgical sequelae of CDH are properly addressed. In this article, we will discuss the experience of CDH centers that offer long-term CDH follow-up as well as the patients' morbidities that require active monitoring until transition to adult care.

Multi-institutional follow-up of patients with congenital diaphragmatic hernia reveals severe disability and variations in practice

Background/PurposeSurvivors of congenital diaphragmatic hernia (CDH) have a high incidence of morbidity. Variability in follow-up practices between institutions may affect perception of disability and prevent population-based outcome analysis. MethodsA survey of follow-up practices at 16 centers within a population-based CDH network was performed. A descriptive analysis of outcomes (minimum 24 months postdischarge) of CDH survivors from the 2 largest centers was performed. ResultsThe nature of follow-up of CDH survivors was highly variable in 12 of 16 responding centers, ranging from ad hoc, community-based, and pediatrician-sponsored follow-up to a single perinatal center-based multispecialty CDH clinic. Outcomes at 24 to 36 months were reported from the 2 largest centers (n = 44). Among survivors, neurodevelopmental disability was most common (12/44; 27%) followed by gastrointestinal (9; 20.5%), pulmonary (5; 11.4%), musculoskeletal (5; 11.4%), and cardiac (2; 4.5%). Additional surgery was required in 17 patients (38.6%), including recurrent CDH repair in 7 (15.9%). Five patients (11.4%) had hearing loss. Among 41 children with available 24-month data, 32 (78%), 17 (41.5%), and 14 (34.1%) patients had weights below the 50th, 25th, and 3rd percentiles, respectively. ConclusionCongenital diaphragmatic hernia survivorship is associated with significant disability. Standardization of follow-up practices is essential to enable population-based outcomes analysis.

Prediction of pulmonary hypoplasia in mid‐trimester preterm prelabor rupture of membranes: research or clinical practice?

Prediction of pulmonary hypoplasia in mid‐trimester preterm prelabor rupture of membranes: research or clinical practice?

Outcomes of congenital diaphragmatic hernia: a 12‐year experience

To determine outcomes for babies with congenital diaphragmatic hernia (CDH) diagnosed prenatally and postnatally presenting to a tertiary unit. Fetal medicine database and neonatal surgical unit admission books were reviewed to identify cases of CDH over a 12-year period (Jan 1998- Dec 2009). A total of 132 cases of CDH were diagnosed prenatally and 39 cases diagnosed postnatally. Mean gestation at diagnosis was 22 weeks (range 13-37 weeks). Karyotyping was abnormal in 15.9%; abnormal karyotype in 3.6% of fetuses without other structural anomalies compared with 38.9% when other anomalies were identified. In 45 cases (34.1%) pregnancy was terminated. Two stillbirths occurred (2.3% of ongoing pregnancies). One hundred twenty-four babies were live born, of whom 98 babies underwent surgery. Survival to discharge of all live born babies was 72.6%; survival following surgery was 91.8%. Birthweight had a significant effect on survival (odds ratio 0.22, 95% confidence intervals 0.08-0.66). Survival for live born babies prenatally diagnosed was significantly lower (65.9%) than those diagnosed postnatally (92.3%). Abnormal karyotype was more common when CDH was associated with other anomalies. In multivariate analysis, lower birthweight and prenatal diagnosis had a significant impact on survival. Only prenatally diagnosed CDH survival figures should be utilised in prenatal counselling.