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Congenital Diaphragmatic Hernia: The Hardest the Hit the Largest the Hole

Saturday, October 20, 2012: 7:44 AM
Versailles Ballroom (Hilton Riverside)
Laura Valfrè1, Francesco Morini1, Irma Capolupo1, Kevin P. Lally, MD, MS2, Pam A. Lally, MD3 and Pietro Bagolan1, (1)Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, Research Institute, Rome, Italy, (2)Pediatric Surgery, University of Texas Medical School at Houston, Houston, TX, (3)Department of Pediatric Surgery, University of Texas Medical School at Houston, Houston, TX

CONGENITAL DIAPHRAGMATIC HERNIA: THE HARDEST THE HIT THE LARGEST THE HOLE.

Purpose:

The prognosis of patients with congenital diaphragmatic hernia (CDH) has been related to the size of the defect.  The aim of our study was to elucidate if the defect size reflects the magnitude of other embryonic defects in patients with CDH.

Methods :

All patients recorded in the CDH Study Group registry between 2007 and 2010 and with defect classification were included in the study.  Patients were divided according to defect size (A to D; Tsao & Lally, Semin Pediatr Surg, 2008, Figure) and the groups were compared for: gestational age (GA), birth weight (BW), prevalence of associated malformations (MLF), congenital heart disease (CHD), chromosomal anomalies (Chr), hernia sac, and number of abnormal organ systems. The groups were compared by ANOVA or Chi-square test as appropriate. Means±SD or prevalence are given.

Results :

1778 patients were recorded in the registry and 1146 had defect classification: 166 defect A, 492 defect B, 334 defect C, and 154 defect D. The table shows main findings.

Conclusion :

In a large cohort of CDH patients, defect size is directly correlated with prevalence of other anomalies (particularly CHD) and number of abnormal systems, and inversely correlated with gestational age, birth weight, and prevalence of hernia sac. These findings suggest that the size of the diaphragmatic defect may be a marker of the magnitude of the developmental abnormality in the embryo with CDH. The correlation between defect size and embyopathogenesis may explain the relationship between defect size and outcome in patients with CDH.

DEFECT

A

(166 pts)

B

(492 pts)

C

(334 pts)

D

(154 pts)

p

p

(trend)

GA (wks.)

38.10±2.05

37.93±1.89

37.55±2.19

37.25±2.47

0.0002

<0.0001

BW (kgs.)

3.15±0.61

3.08±0.54

2.97±0.60

2.84±0.61

<0.0001

<0.0001

MLF (%)

19.9

21.7

31.1

27.3

0.0396

0.0129

CHD (%)

9.0

13.4

23.4

20.8

<0.0001

<0.0001

Abn. Systems (#)

1.15±0.51

1.07±0.37

1.17±0.54

1.32±0.86

<0.0001

0.0005

Chr (%)

        3.0

1.8

2.6

6.2

0.0719

0.0806

Hernia sac (%)

19.9

19.9

15.6

9.2

0.0045

0.0012