Short term and long term health related quality of life after congenital anorectal malformations and congenital diaphragmatic hernia.
OBJECTIVE
To examine short term and long term health related quality of life (HRQoL) of survivors of congenital anorectal malformations (ARM) and congenital diaphragmatic hernia (CDH), and to compare these patients' HRQoL with that of the general population.


METHODS
HRQoL was measured in 286 ARM patients and 111 CDH patients. All patients were administered a symptom checklist and a generic HRQoL measure. For the youngest children (aged 1-4) the TAIQOL (a preliminary version of the TAPQOL) was used, for the other children (aged 5-15) the TACQOL questionnaire, and for adults (aged >16) the SF-36.


RESULTS
As appeared from the symptom checklists, many patients remained symptomatic into adulthood. In the youngest ARM patients (aged 1-4 years), generic HRQoL was severely affected, but the older ARM patients showed better HRQoL. In the CDH patients, the influence of symptoms on HRQoL seemed less profound. The instruments we used revealed little difference between adults treated for ARM or CDH and the general population.


CONCLUSIONS
These results show that for two neonatal surgical procedures, improved survival does not come at the expense of poor HRQoL in adults. Even though there is considerable suffering in terms of both morbidity and mortality in the youngest group, the ultimate prognosis of survivors of the two studied congenital malformations is favourable. This finding can be used to reassure parents of patients in need of neonatal surgery for one of these conditions about the prospects for their child.
Department of Pediatric Surgery, Sophia Children's Hospital, Erasmus MC, Rotterdam, Netherlands. m.poleij@erasmusmc.nl
M J
MJ
Poley
E A
EA
Stolk
D
D
Tibboel
J C
JC
Molenaar
J J V
JJ
Busschbach
D000293:Adolescent; D000328:Adult; D001003:Anal Canal / Q000002:abnormalities / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003071:Cognition; D005260:Female; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000601:surgery; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009043:Motor Activity / Q000502:physiology; D011379:Prognosis; D011788:Quality of Life; D012007:Rectum / Q000002:abnormalities* / Q000601:surgery; D013997:Time Factors