Elsevier

Journal of Pediatric Surgery

Volume 42, Issue 9, September 2007, Pages 1526-1532
Journal of Pediatric Surgery

Retrospective study of 111 cases of congenital diaphragmatic hernia treated with early high-frequency oscillatory ventilation and presurgical stabilization

https://doi.org/10.1016/j.jpedsurg.2007.04.015Get rights and content

Abstract

Background

The prognosis of babies with congenital diaphragmatic hernia (CDH) remains unsatisfactory despite recent advances in medical and surgical treatment. Most authors agree that the best way to improve outcomes for this disease is to focus on pulmonary hypoplasia and persistent pulmonary hypertension (PPH), the 2 most unfavorable prognostic factors for patient survival. However, controversy remains regarding the best treatment of CDH. In the past decade, several institutions have developed treatment protocols that include high-frequency oscillatory ventilation (HFOV), preoperative stabilization, and no thoracic drain. This strategy is 1 of several “gentle ventilation” strategies. We describe our 10-year experience in treating a cohort of 111 infants with CDH managed with this “gentle ventilation” strategy.

Methods

From October 1994 to June 2005, 111 babies with CDH were treated at our institution with HFOV. Babies progressed to inhaled nitric oxide and extracorporeal membrane oxygenation if severe PPH persisted. After a period of preoperative stabilization, surgery was performed via an abdominal approach. In case of large defects or diaphragmatic agenesis, a prosthetic patch was used. No thoracic drain was left in place at the end of surgery. The charts of all babies were reviewed. General characteristics, respiratory management, as well as perioperative and postoperative data were analyzed and correlated with survival. Predicted and actual survival rates in high-, intermediate-, and low-risk groups were analyzed on the basis of the equation described by the Congenital Diaphragmatic Hernia Study Group in 2001.

Results

The overall survival rate in our group of patients with CDH was 69.4% regardless of side of the defect. Incidence of a prenatal diagnosis before the 25th gestational week, coexistence of severe congenital heart disease (overall incidence, 5.4%), or other major associated anomalies, as well as the presence of a diaphragmatic agenesis were significantly higher in nonsurvivors. Thirty-six had severe PPH, of which 26 (76.5% of nonsurviving patients) died. Survivors and nonsurvivors had significant differences in blood gas analysis and respiratory management data recorded before and after the diaphragmatic correction. Ninety-nine (89%) patients underwent correction of the diaphragmatic defect. A patch was used in 44 (44%) patients and 15 of them died (survivors, 37.7%; nonsurvivors, 68.2%; P = .0111). Six (43%) of 14 patients with a preoperative pneumothorax (survivors, 10.3%; nonsurvivors, 27.3%; P > .05) and 7 (58%) of 12 patients with a postoperative pneumothorax needing a thoracic drain (survivors, 6.5%; nonsurvivors, 31.8%; P = .0013) died. In all cases, pneumothorax was ipsilateral. Two patients required oxygen therapy at discharge. The predicted survival rate was 69%; there was no difference between predicted and actual overall survival as well as between predicted and actual survival in low-risk (predicted survival rate, >66%), intermediate-risk (predicted survival rate, 34%-66%), and high-risk (predicted survival rate, <33%) groups.

Conclusions

The CDH treatment strategy that includes HFOV, preoperative stabilization and no thoracic drain ensures survival with minimal pulmonary morbidity (low rate of pulmonary infections and low rate of patients requiring oxygen at home) in most affected babies. Persistent pulmonary hypertension has been the most challenging factor that ultimately determined the final outcome, and availability of new vasoactive drugs is mandatory to ameliorate the prognosis especially in high-risk patients. Meanwhile, survival comparisons of low-, intermediate-, and high-risk groups between institutions using different protocols will allow the identification of the best strategy for CDH management.

Section snippets

Patients and methods

From October 1994 to June 2005, 111 neonates (69 male and 42 female infants) with CDH were admitted to the NICU of Bergamo's Ospedali Riuniti. Their clinical charts were retrospectively evaluated. All CDH newborns were included, except those transferred postoperatively.

All inborn and outborn infants with CDH at arrival were treated with the same protocol. If a prenatal diagnosis was made, a cesarean delivery was scheduled at term of the gestation. In case of preterm delivery, that is, before

General characteristics and findings

Seventy-five infants with CDH (67.6%) were born at our hospital. Among the inborn patients the survival was 62.6% (47/75) compared to 83.3% (30/36) among outborns (P = .027). Outborn patients were admitted after a mean of 48 hours (range, 1-840 hours) of life. Prenatal diagnosis was made in 68 (61.3%) patients: 43 (63.2%) of 68 infants were diagnosed before the 25th week of gestation and 23 (53.5%) of these 43 patients died (Table 1). Mean gestation age at birth of the whole series was 37.6 ±

Discussion

The present study reports an overall 69.4% survival rate in a large cohort of patients with CDH, with only 2 infants diagnosed late after birth. These results compare favorably with other reported series (53%-78%) [1], [4]. Mortality in inborn patients was significantly higher compared to outborns (37% vs 16%). This may reflect a “hidden mortality” before transfer in outborns: survivors had less severe pulmonary hypoplasia and absence of lethal associated anomalies, as suggested by Boloker et

Acknowledgments

We are grateful to Professor Max R. Langham Jr, Professor of Surgery and Pediatrics, Chief, Division of Pediatric Surgery, University of Tennessee, Memphis (USA), for his suggestions and advice while reviewing the manuscript.

References (24)

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  • Operative repair in congenital diaphragmatic hernia: How long do we really need to wait?

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    In a cohort of 51 neonates with CDH managed over a 12-year period, Desfrere et al. reported high post-operative survival rates associated with an OI < 10 [24]. Similar to our approach, this study, as well as those from Migliazza et al. early respiratory support with high frequency ventilation, as have others [16,17,25]. All reported similar success utilizing an OI < 10 to determine physiologic stability, with operative repair at a mean age of 48–96 h and survival.

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