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: 2.51). 06/2008; 28(7):592-603. DOI: 10.1002/pd.2007
Source: PubMed

ABSTRACT 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.

  • [Show abstract] [Hide abstract]
    ABSTRACT: This study aimed to characterize the surgical complications, especially gastroesophageal reflux disease (GERD), intestinal adhesion obstruction (IAO), and diaphragmatic hernia recurrence, in patients with congenital diaphragmatic hernia (CDH). Between January 1995 and December 2013, we determined the incidence of surgical complications and their predictors in CDH patients. We also examined whether the CDH repair and patch closure were associated with the incidence of IAO and the severity of adhesion. Seventy-four CDH survivors were evaluated. GERD occurred in 28 patients (37.8 %) and recurred in 8 patients (10.8 %). Stomach herniation was a risk factor for GERD, and occurred in 25 patients. IAO occurred in 13 patients (17.6 %). In 240 neonatal laparotomies in the same period, the incidence of IAO was significantly higher in patients who underwent CDH repair than in patients who underwent other neonatal laparotomy (p = 0.023). Surgical time and intraoperative bleeding were significantly greater following CDH repair with an artificial patch compared with CDH repair with direct closure. Surgical complications are major sequelae in survivors of CDH repair. CDH repair and artificial patch closure were significantly associated with the incidence of IAO and the severity of adhesion.
    Pediatric Surgery International 08/2014; 30(9). DOI:10.1007/s00383-014-3575-x · 1.06 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Abstract Infants with forms of pulmonary hypertension (PH) that persist or develop beyond the first week of life are an understudied group of patients with up to 40%-60% mortality. The clinical management of the progressive PH that develops in these infants is challenging because of the nonspecific signs and symptoms of clinical presentation, the limited diagnostic sensitivity of standard echocardiographic techniques, and the lack of proven therapies. The signaling mechanisms that underlie the structural and functional abnormalities in the pulmonary circulation of these infants are not yet clear. The ability to improve outcomes for these patients awaits technological advances to improve diagnostic capabilities and therapeutic discoveries made in basic science laboratories that can be tested in randomized clinical trials.
    09/2013; 3(3):454-66. DOI:10.1086/674438
  • [Show abstract] [Hide abstract]
    ABSTRACT: 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.
    08/2014; 3(3):30-6. DOI:10.5409/wjcp.v3.i3.30