Late-presenting congenital diaphragmatic hernia.
BACKGROUND
Late-presenting congenital diaphragmatic hernia (CDH) is a rare subset of CDH, most of the information derived from small series or case reports. The aim of this study was to document the clinical manifestations of late-presenting CDH using a large multicenter database.


METHODS
Information about late-presenting CDH (diagnosed at later than 30 days of age) was identified from the database of the CDH Study Group (3098 cases collected during 1995-2004) and reviewed retrospectively.


RESULTS
Seventy-nine cases (2.6%) from 30 centers met the inclusion criteria. Seven cases had a Morgagni hernia. There were 50 males (65%) and 27 females (35%). The mean age at diagnosis was 372 days (32 days to 15 years). Major associated anomalies (10 cardiac and 7 chromosomal abnormalities) were identified in 12 cases (15%). Presenting symptoms were respiratory in 20 (43%), gastrointestinal in 15 (33%), both in 6 (13%), and none (asymptomatic) in 5 (11%). The hernia was left-sided in 53 (69%), right-sided in 21 (27%), and central or bilateral in 3 (4%). Patients with gastrointestinal symptoms invariably had left-sided hernias (n = 19), whereas patients with respiratory symptoms (n = 24) seemed equally likely to have right- or left-sided lesions. A primary repair without patch was done in all cases with 100% survival.


CONCLUSIONS
Presenting symptoms of late-onset CDH can be respiratory or gastrointestinal, but presentation with gastrointestinal problems was more common in left-sided hernias, whereas respiratory symptoms predominated in right-sided lesions. The prognosis is excellent once the correct diagnosis is made.
Kitano
Yoshihiro
Y
National Center for Child Health and Development, Tokyo Japan.
Lally
Kevin P
KP
Lally
Pamela A
PA
D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D007839:Functional Laterality; D005767:Gastrointestinal Diseases / Q000209:etiology; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000175:diagnosis*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D008297:Male; D012140:Respiratory Tract Diseases / Q000209:etiology; D012189:Retrospective Studies