Pulmonary morbidity in 100 survivors of congenital diaphragmatic hernia monitored in a multidisciplinary clinic

Background/Purpose: In 1990, the authors began a multidisciplinary follow-u p clinic for congenital diaphragmatic hernia (CDH) patients. Although the n onpulmonary complications associated with CDH have been reported previously from this clinic, the purpose of this report is to detail the pulmonary ou tcome in survivors of CDH with severe pulmonary hypoplasia. Methods: Between 1990 and 1999, one hundred patients were seen in the clini c. Before hospital discharge, all patients had baseline tests performed, wh ich were repeated per protocol at clinic during follow-up. The data were an alyzed by regression analysis to identify and determine the impact of facto rs on outcomes associated with the long-term pulmonary morbidity, Results: The average birth weight was 3.16 kg (+/- 0.7) with a mean Apgar s core of 7 (+/- 2) at 5 minutes. Forty-one patients had an antenatal diagnos is performed. Extracorporeal membrane oxygenation (ECMO) was utilized in 29 patients, and a patch repair was required in 32, whereas 15 patients recei ved both. Average time to extubation was 20.7 (+/- 20) days and mean time t o discharge was 59.7 (+/- 61) days. Regression analysis showed that both th e need for ECMO and a patch repair were independent predictors of delay in extubation (P <.001, R-2 = 36%), and delay in discharge from the hospital ( P =.001, R-2 = 29%). ECMO also was significantly correlated with the need f or diuretics at discharge (P <.001, R-2 = 18%), and with the presence of le ft-right mismatch (P =.009, R-2 = 9%) and V/Q mismatch (P =.005, R-2 = 11%) on subsequent pulmonary ventilation-perfusion examinations. Sixteen patien ts required O-2 at discharge, and diuretics were necessary in 43 patients. Seventeen patients at discharge required bronchodilators, and during the fi rst year an additional 36 required at least transient therapy. Similarly, 6 patients at discharge required steroids, and an additional 35 patients req uired at least transient therapy during the first year. Chest x-rays, altho ugh frequently abnormal, had little correlation with clinical outcome, but did influence medical therapy. V/Q scans had limited utility in patient man agement, and the presence of V/Q mismatch was not highly specific for futur e obstructive airway disease. Nevertheless, V/Q mismatch was sensitive for obstructive airway disease assessed by spirometry. Twenty-five patients ove r 5 years of age performed pulmonary function tests (PFTs), which showed 72 % normal PFT results and 28% with evidence of obstructive airway disease. B efore January 1997, 2 of 8 patients who required urgent treatment in the em ergency department (ED) were admitted to the intensive care unit (ICU) seco ndary to acute respiratory distress. After the implementation of respirator y syncytial viral prophylaxis in January 1997, 8 patients were treated in t he ED for acute respiratory distress, but none required admission to the IC U. Conclusions: Pulmonary problems continue to be a source of morbidity for su rvivors of CDH long after discharge. The need for ECMO and the presence of a patch repair are both predictive of more significant morbidity, but the d ata clearly show that non-ECMO CDH survivors also require frequent attentio n to pulmonary issues beyond the neonatal period. These data show the need for long-term follow-up of CDH patients preferably with a multidisciplinary team approach. J Pediatr Surg 36:133-140. Copyright (C) 2001 by W.B. Saund ers Company.