No abstract

Purpose Operative approach, including minimally invasive surgery (MIS) in the repair of congenital diaphragmatic hernia (CDH), is variable among institutions. The short-term recurrent hernia rate is not well described. We evaluated the in-hospital recurrence rate of MIS repairs of infants with CDH from the Congenital Diaphragmatic Hernia Registry (CDHR). Methods Prospectively collected data from CDH infants were analyzed from the CDHR from January 1995 to January 2010. Recurrent hernia was defined as reoperations during initial hospitalization. Operative approaches included abdominal, thoracic, laparoscopic, and thoracoscopic techniques. Results 5,480 infants with CDH were identified of which 4,516 (82.4%) were repaired. Operative data was available in 4,390 infants. One hundred fifty-one infants (3.4%) underwent MIS repairs with twelve reported recurrences (7.9%) compared to one hundred fourteen for open techniques (2.7%, p < 0.05). MIS demonstrated a significant increased odds for recurrence (OR 3.59, 95% CI:1.92 – 6.71) after adjusting for gestational age, birth weight, patch repair, and ECMO. Conclusion Minimally invasive techniques appear to have a significant higher recurrent hernia rate with thoracoscopy being the highest. Although adjusted for patch repair, other factors with regards to disease severity may contribute to differences in outcomes among centers. This study is limited to short-term recurrence during initial hospitalization.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. METHODS: Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm. An integer-based clinical prediction rule was created. The performance of the model was validated by using the remaining data in terms of calibration and discrimination. RESULTS: The final model included the following predictors: very low birth weight, absent or low 5-minute Apgar score, presence of chromosomal or major cardiac anomaly, and suprasystemic pulmonary hypertension. This model discriminated between a population at high risk of death (∼50%) intermediate risk (∼20%), or low risk (&lt;10%). The model performed well, with a C statistic of 0.806 in the derivation set and 0.769 in the validation set and good calibration (Hosmer-Lemeshow test, P = .2). CONCLUSIONS: A simple, generalizable scoring system was developed for CDH that can be calculated rapidly at the bedside. Using this model, intermediate- and high-risk infants could be selected for transfer to high-volume centers while infants at highest risk could be considered for advanced medical therapies.

Infants with CDH are commonly discharged with ≥1 major morbidities. The size of the diaphragmatic defect appears to be the most reliable indicator of a patient's hospital course and discharge burden of disease.