Growth morbidity in patients with cloacal exstrophy: a 42-year experience.
OBJECTIVE
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 to 20years 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.


METHODS
2b.
Department of Surgery, Boston Children's Hospital, Boston, MA; Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA. Electronic address: brenna.sullivan@childrens.harvard.edu.
Brenna S
BS
Fullerton
Department of Surgery, Boston Children's Hospital, Boston, MA; Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA.
Eric A
EA
Sparks
Department of Surgery, Boston Children's Hospital, Boston, MA.
Amber M
AM
Hall
Division of Endocrinology, Boston Children's Hospital, Boston, MA.
Yee-Ming
YM
Chan
Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA; Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA.
Christopher
C
Duggan
Department of Surgery, Lucile Packard Children's Hospital Stanford, Palo Alto, CA.
Dennis P
DP
Lund
Department of Surgery, Boston Children's Hospital, Boston, MA; Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA.
Biren P
BP
Modi
Department of Surgery, Boston Children's Hospital, Boston, MA; Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA.
Tom
T
Jaksic
Department of Surgery, Boston Children's Hospital, Boston, MA.
W Hardy
WH
Hendren
D000015:Abnormalities, Multiple; D000293:Adolescent; D001006:Anus, Imperforate; D001746:Bladder Exstrophy / Q000150:complications* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002988:Cloaca / Q000002:abnormalities* / Q000601:surgery; D005260:Female; D005835:Genitalia / Q000002:abnormalities; D006130:Growth Disorders / Q000150:complications / Q000175:diagnosis*; D006554:Hernia, Umbilical; D006801:Humans; D007422:Intestines / Q000002:abnormalities; D059785:Karyotype; D008297:Male; D012778:Short Bowel Syndrome / Q000209:etiology; D013577:Syndrome; D055815:Young Adult
Cloacal exstrophy; Enterocystoplasty; Growth failure