AAP ArticleGrowth morbidity in patients with cloacal exstrophy: a 42-year experience☆
Section snippets
Study design
With IRB approval (IRB-P00017268), the Boston Children's Hospital records of 71 patients with cloacal exstrophy treated at this institution and at Massachusetts General Hospital between 1974 and 2015 were reviewed, and patients who had height and weight recorded between 2 and 20 years of age were included in the study. Of the 62 included patients, 44 patients were followed by a single surgeon (WHH) with extensive expertise in the field. Genetic sex, gender of rearing, and all available height
Results
Of the 62 patients, 31 were genetically 46,XX, and 31 patients were genetically 46,XY, 21 of whom underwent gonadectomy in infancy and were raised as females. The other 10 46,XY patients did not undergo gonadectomy and were raised male. Associated conditions are listed in 1. There were 904 height and 1301 weight measurements taken between 2 and 20 years of age (Fig. 1, Fig. 2).
In all three groups, HAZ was significantly lower than the population norm (Table 1). At the time of the most recent
Discussion
In a large cohort of patients with cloacal exstrophy followed more than 42 years, we found a high prevalence of growth failure, with nearly half of patients having short stature with height-for-age z-score < − 2, a finding which was consistent regardless of sex or gender of rearing. The growth failure observed in this cohort is likely multifactorial. We identified short bowel syndrome and type of urinary tract reconstruction as important risk factors for growth outcomes.
Historically, gonadectomy in
Conclusion
Patients with cloacal exstrophy have significant long-term growth failure, which is likely multifactorial. The benchmark data from this study can be used as a basis to optimize the multidisciplinary management of these complex patients. Specific nutritional recommendations include optimizing support (especially at times of physiologic stress) and monitoring of micronutrients and total body sodium with replacement as necessary. Metabolic consequences of enterocystoplasty should be considered at
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Cited by (7)
Developmental Abnormalities of the Genitourinary System
2023, Avery's Diseases of the NewbornThe dual-staged pathway for closure in cloacal exstrophy: Successful evolution in collaborative surgical practice
2019, Journal of Pediatric SurgeryImpaired growth outcomes in children with congenital colorectal diseases
2018, Journal of Surgical ResearchCitation Excerpt :To our knowledge, this is the first study to identify racial and socioeconomic disparities in children with CHARM anomalies. In a retrospective study of 62 patients with cloacal exstrophy, Fullerton et al.11 also reported substantial height and weight deficits compared to the general population. Despite this finding, BMI was not significantly affected.
Developmental Abnormalities of the Genitourinary System
2018, Avery's Diseases of the Newborn: Tenth EditionDevelopmental Abnormalities of the Genitourinary System
2017, Avery's Diseases of the Newborn, Tenth EditionCloacal exstrophy
2023, Pediatric Surgery: Diagnosis and Management
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Role of the Authors:
Fullerton: Conception/Design, data acquisition, analysis and interpretation, participated in drafting, gave final approval
Sparks: Conception/Design, data acquisition, analysis and interpretation, participated in revision, gave final approval
Hall: Analysis and interpretation, participated in revision, gave final approval
Chan: Analysis and interpretation, participated in revision, gave final approval
Duggan: Conception/Design, analysis and interpretation, participated in revision, gave final approval
Lund: Data acquisition, analysis and interpretation, participated in revision, gave final approval
Modi: Conception/Design, analysis and interpretation, participated in revision, gave final approval
Jaksic: Conception/Design, analysis and interpretation, participated in revision, gave final approval
Hendren: Conception/Design, data acquisition, analysis and interpretation, participated in revision, gave final approval
CD was supported in part by K24 DK104676–06