Congenital diaphragmatic hernia
ABSTRACT Congenital diaphragmatic hernia (CDH) is a congenital anomaly consisting of a posterolateral defect in the diaphragm also known as a Bochdalek hernia. It occurs in 1 in 2000 to 3000 newborns and is associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care and the new postnatal treatment strategies, many newborns with CDH continue to have high rates of mortality and morbidity as the result of severe respiratory failure secondary to PH and PPH. The pathogenesis of CDH and associated PH and PPH is poorly understood. Herein, we aim to review diaphragm and pulmonary development and correlate this to the abnormalities found in CDH.
- SourceAvailable from: Joe N Kornegay
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- "Valentine et al. speculated that death could occur due to early, severe diaphragmatic involvement . In our experience, these severely affected pups often have dramatic respiratory distress, in keeping with respiratory muscle disease, potentially compounded by delayed pulmonary development (pulmonary hypoplasia), akin to that seen with congenital diaphragmatic hernia . (Kornegay, McLean, unpublished observations, 2011) As GRMD dogs mature, they continue to have respiratory difficulty, as evidenced most notably by increased respiratory rate and abdominal breathing . "
ABSTRACT: Golden retriever muscular dystrophy (GRMD) is a well-established model of Duchenne muscular dystrophy. The value of this model would be greatly enhanced with practical tools to monitor progression of respiratory dysfunction during treatment trials. Arterial blood gas analysis, tidal breathing spirometry, and respiratory inductance plethysmography (RIP) were performed to determine if quantifiable abnormalities could be identified in unsedated, untrained, GRMD dogs. Results from 11 dogs with a mild phenotype of GRMD and 11 age-matched carriers were compared. Arterial blood gas analysis was successfully performed in all dogs, spirometry in 21 of 22 (95%) dogs, and RIP in 18 of 20 (90%) dogs. Partial pressure of carbon dioxide and bicarbonate concentration were higher in GRMD dogs. Tidal breathing peak expiratory flows were markedly higher in GRMD dogs. Abnormal abdominal motion was present in 7 of 10 (70%) GRMD dogs. Each technique provided objective, quantifiable measures that will be useful for monitoring respiratory function in GRMD dogs during clinical trials while avoiding the influence of sedation on results. Increased expiratory flows and the pattern of abdominal breathing are novel findings, not reported in people with Duchenne muscular dystrophy, and might be a consequence of hyperinflation.Neuromuscular Disorders 01/2013; 24(1). DOI:10.1016/j.nmd.2013.10.001 · 3.13 Impact Factor
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- "Congenital diaphragmatic hernia (CDH) is a significant congenital cause of morbidity and mortality, with an incidence of 1:2000 to 1:5000 live births [1e3]. Congenital diaphragmatic hernia manifests on the left in 85% of cases and on the right side 13% of the time, and bilateral defects occur in 2% of cases . Reported survival rates range widely from 54% to 93%  . "
ABSTRACT: PURPOSE: This study examined survival in newborn patients after congenital diaphragmatic hernia (CDH) repair. METHODS: We analyzed the Kids' Inpatient Database Years 2000, 2003, and 2006 for patients admitted at fewer than 8 d of age undergoing CDH repair. We analyzed patient demographics, clinical characteristics, socioeconomic measures, hospital type, operative case volume, and survival using Fisher's exact test and a multivariate binary logistic regression model. RESULTS: Of 847 patients identified, most were male (61%) and white (57%), were treated at urban (99.8%) and teaching (96%) hospitals, and had private insurance (57%). Survival to discharge was 95% in non-extracorporeal membrane oxygenation (ECMO) patients versus 51% for those requiring ECMO (P < 0.0001). Univariate analysis revealed significantly lower survival rates in blacks, Medicaid patients, and patients undergoing repair after 7 d of life. Among ECMO patients, we noted higher survival rates at hospitals conducting four or more ECMO cases per year (66% versus 47%; P = 0.03). Multivariate analysis identified ECMO (hazards ratio [HR] 16.23, P < 0.001), CDH repair at >7 d of age (HR 2.70, P = 0.004), and ECMO patients repaired at hospitals performing <4 CDH ECMO cases per year (HR 3.59, P = 0.03) as independent predictors of mortality. CONCLUSIONS: We conclude that ECMO hospital volume is associated with survival in patients requiring ECMO for CDH repair.Journal of Surgical Research 05/2012; 178(2). DOI:10.1016/j.jss.2012.05.046 · 2.12 Impact Factor
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ABSTRACT: Bochdalek hernia is a congenital posterolateral diaphragmatic defect. It is usually diagnosed in newborns and children; the mortality rate is high due to further congenital anomalies such as pulmonary hypoplasia and pulmonary hypertension. In adulthood, the diagnosis is often incidental, while sometimes it is related to gastrointestinal or respiratory symptoms, or to herniated viscera complications. Thus, surgical treatment is advocated. We herein report a case of an 86-year-old woman with dyspnea and dysphagia. After the diagnosis by barium enema and CT scan of the herniated stomach and the greater omentum through a left side foramen of Bochdalek, she underwent a successful laparoscopic dual mesh repair and was discharged on the 6th postoperative day.10/2010; 62(2):125-8. DOI:10.1007/s13304-010-0022-y