Growth Morbidity in Patients with Cloacal Exstrophy: A 42 Year Experience

Abstract Purpose Cloacal exstrophy is associated with multiple comorbidities that affect growth. This report describes long-term growth outcomes in a large cohort of patients with cloacal exstrophy and explores associated comorbidities. Methods Records of 71 patients with cloacal exstrophy who were...

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Published in:Journal of pediatric surgery 2016-06, Vol.51 (6), p.1017-1021
Main Authors: Fullerton, Brenna S, Sparks, Eric A, Hall, Amber M, Chan, Yee-Ming, Duggan, Christopher, Lund, Dennis P, Modi, Biren P, Jaksic, Tom, Hendren, W. Hardy
Format: Article
Language:English
Subjects:
Abnormalities, Multiple
Adolescent
Anus, Imperforate
Bladder Exstrophy - complications
Bladder Exstrophy - surgery
Child
Child, Preschool
Cloaca - abnormalities
Cloaca - surgery
Cloacal exstrophy
Enterocystoplasty
Female
Genitalia - abnormalities
Growth Disorders - complications
Growth Disorders - diagnosis
Growth failure
Hernia, Umbilical
Humans
Intestines - abnormalities
Karyotype
Male
Pediatrics
Short Bowel Syndrome - etiology
Surgery
Syndrome
Young Adult
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Summary:Abstract Purpose Cloacal exstrophy is associated with multiple comorbidities that affect growth. This report describes long-term growth outcomes in a large cohort of patients with cloacal exstrophy and explores associated comorbidities. Methods Records of 71 patients with cloacal exstrophy who were treated between 1974 and 2015 were reviewed, and 62 patients with growth data from 2–20 years of age were included. Genetic sex, gender of rearing, and all heights, weights, and comorbidities were noted for each patient. Height-for-age, weight-for-age, and body mass index z-scores (HAZ, WAZ, and BMIZ) were determined using US Centers for Disease Control 2000 growth data, and average patient z-scores were calculated. Results There were 904 height and 1301 weight measurements available for 62 patients. 31 were genetically 46,XY, 21 of whom underwent gonadectomy in infancy and were raised female. 46,XX patients, 46,XY male patients, and 46,XY female patients all had median HAZ and WAZ substantially lower than the general population, with median HAZ less than − 2, while maintaining normal BMIZ. Short bowel syndrome and enterocystoplasty with intestine were associated with lower z-scores for all parameters. Conclusions Patients with cloacal exstrophy have significant multifactorial long-term growth failure. These benchmark data can be used to further optimize management.
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2016.02.075