Megacystis–microcolon–intestinal hypoperistalsis syndrome: don’t forget the bladder
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In brief
In brief
This retrospective study of 6 MMIHS patients emphasizes the critical but often overlooked urological component of the syndrome. Early bladder management through clean intermittent catheterization significantly improved urinary infections, upper tract dilation, and preserved renal function, highlighting the need for multidisciplinary care including pediatric urology expertise.
- MMIHS patients require early urological evaluation before symptom onset to prevent urinary retention, infections, and renal injury
- Clean intermittent catheterization (urethral or via cystostomy) effectively manages bladder dysfunction and reduces UTIs in MMIHS
- Bladder management in MMIHS improves upper tract dilation and can reduce stoma prolapse frequency in ostomy patients
- Most MMIHS cases involve ACTG2 gene mutations; multidisciplinary care including pediatric urology preserves renal function
- Prenatal megacystis detection should trigger postnatal urological follow-up protocol to prevent long-term renal complications
Written by the GCMD Library team from the article.
Abstract
Purpose
Megacystis–microcolon–intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant.
Aim of the study is to present urological concerns in MMIHS.
Methods
Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years.
Results
Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up.
Conclusion
We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.