AAP Article
Growth morbidity in patients with cloacal exstrophy: a 42-year experience

https://doi.org/10.1016/j.jpedsurg.2016.02.075Get rights and content

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 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.

Level of evidence: 2b

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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    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

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