Congenital diaphragmatic hernia
Department of Pediatric Surgery, Erasmusmc-Sophia, Rotterdam, The Netherlands. Seminars in Pediatric Surgery
(Impact Factor: 2.22).
08/2010; 19(3):180-5. DOI: 10.1053/j.sempedsurg.2010.03.001
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.
Available from: Pascal Auquier
- "Congenital diaphragmatic hernia (CDH) is a rare but severe congenital malformation . Advances in prenatal imaging have allowed for better estimating the severity and prognosis before birth according to criteria evaluating lung volume. "
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ABSTRACT: The 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 methods: This 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.
Forty-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.
The impact of CDH on QoL seems to be important and must be understood by clinicians who treat these children and their parents.
Available from: Elena Pompili
- "The neonatal symptoms of CDH are heralded by respiratory distress with insufficient oxygenation, excavated abdomen with sternal protrusion and displacement of the heart sounds to the contralateral side. The symptoms of insufficient gas exchange are associated with those of persistent pulmonary hypertension
[9,10] caused by arteriolar constriction and closure of the pulmonary arterial bed that forces maintenance of a pattern of persistent fetal circulation in which the blood from the right ventricle is shunted to the left heart, preventing effective gas exchange. The manifestations of CDH may occur at any age as mild respiratory distress or may even be an unexpected finding during a medical check-up for other reasons
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Congenital diaphragmatic hernia is a major malformation occasionally found in newborns and babies. Congenital diaphragmatic hernia is defined by the presence of an orifice in the diaphragm, more often to the left and posterolateral, that permits the herniation of abdominal contents into the thorax. The aim of this case series is to provide information on the presentation, diagnosis and outcome of three patients with late-presenting congenital diaphragmatic hernias. The diagnosis of congenital diaphragmatic hernia is based on clinical investigation and is confirmed by plain X-ray films and computed tomography scans.
In the present report three cases of asymptomatic abdominal viscera herniation within the thorax are described. The first case concerns herniation of some loops of the large intestine into the left hemi-thorax in a 75-year-old Caucasian Italian woman. The second case concerns a rare type of herniation in the right side of the thorax of the right kidney with a part of the liver parenchyma in a 57-year-old Caucasian Italian woman. The third case concerns herniation of the stomach and bowel into the left side of the chest with compression of the left lung in a 32-year-old Caucasian Italian man. This type of hernia may appear later in life, because of concomitant respiratory or gastrointestinal disease, or it may be an incidental finding in asymptomatic adults, such as in the three cases featured here.
Patients who present with late diaphragmatic hernias complain of a wide variety of symptoms, and diagnosis may be difficult. Additional investigation and research appear necessary to better explain the development and progression of this type of disease.
Available from: Joe N Kornegay
- "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 . "
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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.
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