Predicting inadequate long-term lung development in children with congenital diaphragmatic hernia: an analysis of longitudinal changes in ventilation and perfusion.
OBJECTIVE
Infants born with congenital diaphragmatic hernia (CDH) demonstrate a wide variability in postnatal catch-up lung growth. The goals of this study were to assess the pulmonary development of children born with CDH using sequential ventilation-perfusion (V/Q) scintigraphy and to identify the perinatal factors that correspond to a progressive V/Q mismatch.


METHODS
The records of 137 patients seen between 1990 and 2005 in a multidisciplinary CDH clinic were reviewed. Changes in the ipsilateral V/Q quotient were compared in 46 patients who had 2 or more studies with the following variables: sex, patch repair, laterality, gestational age, and use of extracorporeal membrane oxygenation. An abnormal V/Q quotient was defined as greater than 1.2 (reference range, 0.8-1.2).


RESULTS
Abnormal V/Q scans were identified in 28 (61%) of the 46 patients at the time of the last V/Q study. Patients who underwent a patch repair had nearly 7 times the risk (P < .001) of developing an ipsilateral V/Q mismatch. The use of extracorporeal membrane oxygenation had a variable effect on the probability of an abnormal V/Q study finding. No other variable was significant.


CONCLUSIONS
Many children with CDH develop significant and progressive V/Q mismatches. Although some perinatal variables appear to be predictive of this phenomenon, they may simply be surrogates for a greater degree of pulmonary hypoplasia present at birth. This subpopulation of CDH survivors is an identifiable group clearly at risk and thus requires long-term follow-up.
Hayward
Melissa J
MJ
Department of Surgery, Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA.
Kharasch
Virginia
V
Sheils
Catherine
C
Friedman
Sandra
S
Dunleavy
Mary-Jo
MJ
Utter
Sherri
S
Zurakowski
David
D
Jennings
Russell
R
Wilson
Jay M
JM
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006548:Hernia, Diaphragmatic / Q000000981:diagnostic imaging / Q000503:physiopathology*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D008168:Lung / Q000098:blood supply* / Q000254:growth & development* / Q000503:physiopathology; D008297:Male; D009929:Organ Size; D012123:Pulmonary Ventilation; D011877:Radionuclide Imaging; D012189:Retrospective Studies