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Management of congenital diaphragmatic hernia
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  1. C F DAVIS,
  2. A J SABHARWAL
  1. Department of Surgical Paediatrics
  2. Royal Hospital for Sick Children
  3. Yorkhill, Glasgow G3 8SJ

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    Congenital diaphragmatic hernia (CDH) has an incidence of between one in 2000–5000 live births. The hidden mortality associated with CDH includes spontaneous abortions and stillbirths as well as therapeutic abortions (often for other identified anomalies). The management of CDH has been intensely debated since Ladd and Gross stated in 1940 that early surgery was the proper treatment for these patients.1 Controversies have surrounded ideal preoperative management, timing of surgery, and indeed whether surgery should be offered to all.2 Despite the advances in neonatal care, inroads into mortality which might have been anticipated have not materialised and it remains relatively unchanged at 40–50%.3 However, we now stand on the threshold of an exciting stage in the development of neonatal cardiorespiratory support. The role of new therapeutic modalities such as extracorporeal membrane oxygenation (ECMO), surfactant, high frequency ventilation (HFV), inhaled nitric oxide (iNO), in utero interventions, and liquid ventilation have intensified the debate.

    Key messages

    • Infants with CDH should be delivered in specialist centres

    • Gentle ventilation techniques, including permissive hypercapnia to protect the delicate lungs, and strategies to reduce pulmonary hypertension should be employed

    • Surgery should be delayed until pulmonary hypertension has been stabilised in utero surgery offers hope for high risk CDH but techniques are not yet perfected and premature labour remains a problem

    • Postnatal lung growth strategy with perfluorocarbon liquid and ECMO support is encouraging for high risk cases

    Antenatal management

    Prenatal diagnostic rates vary from 10–90% depending on local antenatal ultrasound screening protocols. Prenatal diagnosis allows for counselling of parents and planned management but does not predict outcome.4 Prenatal diagnosis of CDH should lead to careful inspection for other major anomalies and chromosomal analysis is justified. Laterality, presence of liver and stomach in chest, left ventricular mass, presence of polyhydramnios, and ultrasound indices of relative lung volume …

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