Brenna S Fullerton1, Eric A Sparks2, Amber M Hall3, Yee-Ming Chan4, Christopher Duggan5, Dennis P Lund6, Biren P Modi2, Tom Jaksic2, W Hardy Hendren3. 1. 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. 2. Department of Surgery, Boston Children's Hospital, Boston, MA; Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA. 3. Department of Surgery, Boston Children's Hospital, Boston, MA. 4. Division of Endocrinology, Boston Children's Hospital, Boston, MA. 5. Center for Advanced Intestinal Rehabilitation, Boston Children's Hospital, Boston, MA; Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA. 6. Department of Surgery, Lucile Packard Children's Hospital Stanford, Palo Alto, CA.
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 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. LEVEL OF EVIDENCE: 2b.
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 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. LEVEL OF EVIDENCE: 2b.
Authors: Timothy F Tirrell; Farokh R Demehri; Craig W Lillehei; Joseph G Borer; Benjamin C Warf; Belinda H Dickie Journal: European J Pediatr Surg Rep Date: 2022-03-10