The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity.
M. G. Peetsold,Hugo A. Heij,C. M. F. Kneepkens,A. F. Nagelkerke,Jaap Huisman,Reinoud J. B. J. Gemke +5 more
TLDR
It has been suggested that new therapeutic modalities such as nitric oxide, high frequency oxygenation and extracorporal membrane oxygenation (ECMO) might decrease mortality associated with pulmonary hypertension and the sequelae of artificial ventilation, and that follow-up studies that systematically assess long-term sequelae are mandatory.Abstract:
Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a mortality rate of approximately 40-50%, depending on case selection. It has been suggested that new therapeutic modalities such as nitric oxide (NO), high frequency oxygenation (HFO) and extracorporal membrane oxygenation (ECMO) might decrease mortality associated with pulmonary hypertension and the sequelae of artificial ventilation. When these new therapies indeed prove to be beneficial, a larger number of children with severe forms of CDH might survive, resulting in an increase of CDH-associated complications and/or consequences. In follow-up studies of infants born with CDH, many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. CDH has also been associated with persistent pulmonary vascular abnormalities, resulting in pulmonary hypertension in the neonatal period. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity, although the underlying mechanism has not been fully understood yet. In adult CDH survivors incidence of esophagitis is high and even Barrett's esophagus may ensue. Yet, in many CDH patients a clinical history compatible with GERD seems to be lacking, which may result in missing patients with pathologic reflux disease. Prolonged unrecognized GERD may eventually result in failure to thrive. This has been found in many young CDH patients, which may also be caused by insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. In patients treated with ECMO, the incidence of neurological deficits is even higher, probably reflecting more severe hypoxemia and the risk of ECMO associated complications. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but most of them concentrate on the first years after repair and only a few studies focus on the health-related quality of life in CDH patients. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are mandatory. Based on such studies a more focused approach for routine follow-up programs may be established.read more
Citations
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Journal ArticleDOI
Congenital diaphragmatic hernia.
TL;DR: Congenital Diaphragmatic Hernia is defined by the presence of an orifice in the diaphragm, more often left and posterolateral that permits the herniation of abdominal contents into the thorax.
Journal ArticleDOI
Congenital diaphragmatic hernia
Sailesh Kotecha,Angelo Barbato,Andrew Bush,F. Claus,Mark Davenport,C. Delacourt,Jan Deprest,Ernst Eber,Björn Frenckner,Anne Greenough,Andrew G. Nicholson,Juan L. Antón-Pacheco,Fabio Midulla +12 more
TL;DR: Experts are convened to evaluate the current literature and make recommendations on both the antenatal and post-natal management of congenital diaphragmatic hernia and multidisciplinary follow-up is recommended.
Journal ArticleDOI
Re‐evaluation of stomach position as a simple prognostic factor in fetal left congenital diaphragmatic hernia: a multicenter survey in Japan
Yoshihiro Kitano,Hiroomi Okuyama,M. Saito,Noriaki Usui,N. Morikawa,Kouji Masumoto,H. Takayasu,T. Nakamura,H. Ishikawa,Motoyoshi Kawataki,Shusaku Hayashi,Noboru Inamura,K. Nose,H. Sago +13 more
TL;DR: To document outcome and to explore prognostic factors in fetal left congenital diaphragmatic hernia, the objective was to establish a registry of newborns diagnosed with CDH in the neonatal period and establish a baseline for prognosis.
Journal ArticleDOI
Risk factors for chronic lung disease and mortality in newborns with congenital diaphragmatic hernia.
L. van den Hout,I Reiss,Janine F. Felix,Wim C. J. Hop,Pamela A. Lally,Kevin P. Lally,Dick Tibboel +6 more
TL;DR: Despite improvements in neonatal care, the rates of BPD and early mortality in newborns with CDH are still considerable, and several important risk factors for a worse outcome are reported in this nonrandomized prospective observational study.
Journal ArticleDOI
Can we improve outcome of congenital diaphragmatic hernia
L. van den Hout,I. Sluiter,Saskia J. Gischler,A. De Klein,Robbert J. Rottier,Hanneke IJsselstijn,I Reiss,Dick Tibboel +7 more
TL;DR: Etiological factors, prenatal predictors of survival, new treatment strategies and long-term morbidity are described and the international CDH Euro-Consortium is an example of such a collaborative network, which enhances exchange of knowledge, future research and development of treatment protocols.
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Nihilism in the 1990s: The True Mortality of Congenital Diaphragmatic Hernia
TL;DR: In this article, the authors report the incidence of CDH in a geographically defined population over 11 years and assesses the effect of new therapies (highfrequency oscillatory ventilation, extracorporeal membrane oxygenation, inhaled nitric oxide, and delayed surgery) on survival when case selection is avoided.