Surgeon level variation in outcome of repair of congenital diaphragmatic hernia with particular reference to the management of recurrence

IntroductionCongenital diaphragmatic hernia (CDH) remains a cause of neonatal death. Our aims are to describe contemporary rates of survival and the variables associated with this outcome, contrasting these with our study of two decades earlier and recent reports.Materials and methodsA retrospective review of all infants diagnosed in a regional center between January 2000 and December 2020 was performed. The outcome of interest was survival. Possible explanatory variables included side of defect, use of complex ventilatory or hemodynamic strategies (inhaled nitric oxide (iNO), high-frequency oscillatory ventilation (HFOV), extracorporeal membrane oxygenation (ECMO), and Prostin), presence of antenatal diagnosis, associated anomalies, birth weight, and gestation. Temporal changes were studied by measuring outcomes in each of four consecutive 63-month periods.ResultsA total of 225 cases were diagnosed. Survival was 60% (134 of 225). Postnatal survival was 68% (134 of 198 liveborn), and postrepair survival was 84% (134 of 159 who survived to repair). Diagnosis was made antenatally in 66% of cases. Variables associated with mortality were the need for complex ventilatory strategies (iNO, HFOV, Prostin, and ECMO), antenatal diagnosis, right-sided defects, use of patch repair, associated anomalies, birth weight, and gestation. Survival has improved from our report of a prior decade and did not vary during the study period. Postnatal survival has improved despite fewer terminations. On multivariate analysis, the need for complex ventilation was the strongest predictor of death (OR=50, 95% CI 13 to 224, p<0.0001), and associated anomalies ceased to be predictive.ConclusionsSurvival has improved from our earlier report, despite reduced numbers of terminations. This may be related to increased use of complex ventilatory strategies.

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“…This will not be true for centers that repair most defects by patch. We report elsewhere the association between patch use and recurrence 14…”

Section: Discussionmentioning

confidence: 89%

Congenital diaphragmatic hernia survival in an English regional ECMO center

O’Connor

Tamura

Hannon

et al. 2023

World Jnl Ped Surgery

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Surgical approaches to congenital diaphragmatic hernia

King,

Carr,

Mychaliska

et al. 2024

Seminars in Pediatric Surgery

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Surgical management of the diaphragmatic defect in congenital diaphragmatic hernia: a contemporary review

Jank,

Boettcher,

Keijzer

2024

World Jnl Ped Surgery

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Worldwide, 150 children are born each day with congenital diaphragmatic hernia (CDH), a diaphragmatic defect with concomitant abnormal lung development. Patients with CDH with large defects are particularly challenging to treat, have the highest mortality, and are at significant risk of long-term complications. Advances in prenatal and neonatal treatments have improved survival in high-risk patients with CDH, but surgical treatment of large defects lacks standardization. Open repair by an abdominal approach has long been considered the traditional procedure, but the type of defect repair (patch or muscle flap) and patch material (non-absorbable, synthetic or absorbable, biological) remain subjects of debate. Increased experience and improved techniques in minimally invasive surgery (MIS) have expanded selection criteria for thoracoscopic defect repair in cardiopulmonary stable patients with small defects. However, the application of MIS to repair large defects remains controversial due to increased recurrence rates and unknown long-term effects of perioperative hypercapnia and acidosis resulting from capnothorax and reduced ventilation. Current recommendations on the surgical management rely on cohort studies of varying patient numbers and data on the long-term outcomes are sparse. Here, we discuss surgical approaches for diaphragmatic defect repair highlighting advancements, and knowledge gaps in surgical techniques (open surgery and MIS), patch materials and muscle flaps for large defects, as well as procedural adjuncts and management of CDH variants.

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Surgeon level variation in outcome of repair of congenital diaphragmatic hernia with particular reference to the management of recurrence

Cited by 3 publications

References 41 publications

Congenital diaphragmatic hernia survival in an English regional ECMO center

Congenital diaphragmatic hernia survival in an English regional ECMO center

Surgical approaches to congenital diaphragmatic hernia

Surgical management of the diaphragmatic defect in congenital diaphragmatic hernia: a contemporary review

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