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Health-related quality of life among children, adolescents, and adults with bladder exstrophy–epispadias complex: a systematic review of the literature and recommendations for future research

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Abstract

Purpose

Bladder exstrophy–epispadias complex (BEEC) is a rare spectrum of genitourinary malformations. Children risk long-term urinary and genital dysfunctions. To achieve a comprehensive understanding, this study aimed to review the literature on generic and disease-specific health-related quality of life (HRQOL) in BEEC patients, and methodologies used.

Methods

A literature search was conducted in Pubmed/CINAHL/Embase/PsycINFO/Cochrane, from inception to May 2018. A meta-analysis of HRQOL in BEEC patients compared to healthy references was performed.

Results

Twenty-one articles (published 1994–2018), describing HRQOL of children and adolescents (n = 5) and adults only (n = 5), or integrated age populations (n = 11), were identified (median sample size 24, loss to follow-up 43%, response rate 84%). Overall HRQOL was reduced in BEEC patients compared to healthy references in 4/4 studies. Impaired physical or general health in BEEC patients has been described in 9 articles, diminished mental health in 11, restricted social health in 10, and sexual health/functioning or body perception impairments in 13 articles. Urinary incontinence was the most common factor related to worse HRQOL (12 studies). In six studies, HRQOL was better than healthy norms. In eligible studies (n = 5), the pooled estimate of the effect of BEEC indicated worse HRQOL for children and adults (0 > effect sizes < 0.5). Thirty-six HRQOL assessments were used, none developed and validated for BEEC.

Conclusions

HRQOL in BEEC patients may be negatively impacted, particularly considering mental and social HRQOL. Sexual health/functioning or body perception impairments may be present in adolescents and adults. However, HRQOL is heterogeneously assessed and subsequent findings are differently reported. Additional research is warranted and can be improved.

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Abbreviations

BE:

Bladder exstrophy is a congenital malformation with a closing defect of the lower part of the abdominal wall. When the child is born, the bony pelvis is open in the front; the bladder and urethra are open and exposed on the outside of the abdomen; the belly button low; and genital malformations are present (see epispadias)

BEEC:

Bladder exstrophy–epispadias complex refers to a spectrum of birth defects including epispadias (mildest form), bladder exstrophy (intermediate form), and cloacal exstrophy (severest form), which involves malformations of the urinary tract, intestinal tract, genital tract, musculoskeletal system, and sometimes the spinal cord

BNR:

Bladder neck repair refers to different reconstructive procedures/methods of the bladder neck in order to improve urinary continence by increasing the outlet resistance

CIC:

Clean intermittent catheterization is a method to empty the bladder of urine by passing a thin, plastic tube through the urethra or a catheterizable stoma intermittently under clean but not sterile conditions

CE:

Cloacal exstrophy is an abdominal defect with the urinary bladder and parts of the intestines exposed externally on the abdomen. The bony pelvis is open. Imperforate anus is common. Spinal defects may also be present. In males, the penis is short, split or even missing. In females, the clitoris is split and there may be duplication anomalies of the internal genitalia.

E:

Epispadias, isolated or in combination with bladder exstrophy, is a closing defect of urethra. In boys, the urethra is opened like a plate on the dorsal side of the penis. The penis is most often short and broad, and curved dorsally. In girls, the urethra opens between a bifid, split clitoris. The vaginal opening may be narrow and ventrally displaced

ES:

Effect size

HRQOL:

Health-related quality of life

KS:

Kidney stones

SDQ:

Strength and Difficulties Questionnaire

UD:

Urinary diversion encompasses surgical procedures which reroute and drain urine flow from its normal pathway. It may include detaching the ureters and connecting them to an intestinal segment creating a stoma on the abdominal surface. The patient will wear an ostomy bag into which the urine drains continuously (non-continent urinary diversion). It can also refer to an internal reservoir with a bowel segment, together with the residual bladder or not, to store urine between intermittent catheterizations through a continent stoma (continent urinary diversion)

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  1. Department of Pediatric Surgery, The Queen Silvia Children’s Hospital, Sahlgrenska University Hospital, 416 85, Gothenburg, Sweden

    Michaela Dellenmark-Blom, Sofia Sjöström, Kate Abrahamsson & Gundela Holmdahl

  2. Department of Pediatrics, The Queen Silvia Children’s Hospital, Institute of Clinical Sciences, Sahlgrenska Academy, Gothenburg University, 416 86, Gothenburg, Sweden

    Michaela Dellenmark-Blom, Sofia Sjöström, Kate Abrahamsson & Gundela Holmdahl

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Dellenmark-Blom, M., Sjöström, S., Abrahamsson, K. et al. Health-related quality of life among children, adolescents, and adults with bladder exstrophy–epispadias complex: a systematic review of the literature and recommendations for future research. Qual Life Res 28, 1389–1412 (2019). https://doi.org/10.1007/s11136-019-02119-7

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