Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia

Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Prenatal Diagnosis (Impact Factor: 3.27). 07/2008; 28(7):592-603. DOI: 10.1002/pd.2007
Source: PubMed


Significant advances in the postnatal management of patients with congenital diaphragmatic hernia (CDH) have resulted in a remarkable improvement in survival rates over the past two decades. The success of current postnatal management of CDH patients has rendered fetal intervention to be limited to the most severe cases, and the role for prenatal treatment of CDH patients remains unclear. The adoption of lung-preserving strategies including high-frequency oscillatory ventilation (HFOV) and extracorporeal membrane oxygenation (ECMO) have improved CDH outcomes especially in those patients with significant ventilatory or circulatory compromise. Survival rates of up to 90% are being reported in some high-volume centers. However, the increased survival in CDH patients has been accompanied by an increase in neurological, nutritional and musculoskeletal morbidity among the long-term survivors. This has resulted in the need to provide resources for the long-term follow-up and support of this patient population. In this article, the postnatal management strategies and primary and secondary outcomes of high-volume international pediatric surgical centers will be reviewed. Finally, the role of a multidisciplinary management team for the follow-up of long-term CDH survivors will be discussed.

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    • "In the current era, the management of CDH has focused on the early and aggressive treatment of pulmonary pathology rather than the surgical repair of the anatomic defect. These techniques have included " gentle ventilation, " extracorporeal membrane oxygenation (ECMO), and the advanced medical management of pulmonary hypertension [2] [3]. As a result, mortality has decreased in tertiary care centers capable of delivering such management. "
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    ABSTRACT: Although intuitive, the benefit of prenatal evaluation and multidisciplinary perinatal management for fetuses with congenital diaphragmatic hernia (CDH) is unproven. We compared the outcome of prenatally diagnosed patients with CDH whose perinatal management was by a predefined protocol with those who were diagnosed postnatally and managed by the same team. We hypothesized that patients with CDH undergoing prenatal evaluation with perinatal planning would demonstrate improved outcome. Retrospective chart review of all patients with Bochdalek-type CDH at a single institution between 2004 and 2009 was performed. Patients were stratified by history of perinatal management, and data were analyzed by Fisher's Exact test and Student's t test. Of 116 patients, 71 fetuses presented in the prenatal period and delivered at our facility (PRE), whereas 45 infants were either outborn or postnatally diagnosed (POST). There were more high-risk patients in the PRE group compared with the POST group as indicated by higher rates of liver herniation (63% vs 36%, P = .03), need for patch repair (57% vs 27%, P = .004), and extracorporeal membrane oxygenation use (35% vs 18%, P = .05). Despite differences in risk, there was no difference in 6-month survival between groups (73% vs 73%). Patients with CDH diagnosed prenatally are a higher risk group. Prenatal evaluation and multidisciplinary perinatal management allows for improved outcome in these patients.
    Full-text · Article · May 2011 · Journal of Pediatric Surgery

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    ABSTRACT: Much of current knowledge and momentum in the field of pulmonary hypertension (PH) has not penetrated the increasingly heterogeneous pediatric population managed by pediatric subspecialists worldwide. It is critical to prospectively test specific agents in children. Yet, the broad spectrum of pediatric pulmonary vascular diseases and the lack of clinically meaningful end points appropriate for the pediatric population pose challenges for the conduct of sufficiently powered pediatric studies. It is therefore no surprise that in the midst of significant advances in the field, there are no agents approved for use in children afflicted with PH with the exception of inhaled nitric oxide (iNO) for infants with persistent PH of the newborn (PPHN). The current and widely accepted WHO framework by which pediatric subspecialists are guided is not easily translated into the expanding range of situations encountered in pediatric practice. Like PPHN, it may be necessary to approach these pediatric pulmonary hypertensive conditions from outside the frameworks proposed for typical adult-onset diseases. Exploration of the potential intrinsic differences between children and adults may yield important discoveries regarding the genesis of disease in children and adults alike Fortunately, most children with PH are seen within specialized pediatric centers where there are untapped opportunities for multicenter collaboration. Our aim in this chapter is to approach pediatric PH over the age and development continuum from the vantage point of the pediatric subspecialists who encounter these patients in practice. We will review the abnormalities of pulmonary vascular tone and structure that impact the health and survival of children currently cared for in pediatric PH centers. Many of these disease processes occur commonly from our vantage point, yet are underrepresented in the broader field of PH research or current classification schemes. We will also address the challenges that pediatric subspecialists face in defining PH and assessing its severity in infants or children across the developmental and disease spectrums.
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