A review of fetal megacystis: from diagnosis to long-term prognosis
DOI:
https://doi.org/10.15574/PP.2025.3(103).148156Keywords:
megacystis, lower urinary tract obstruction, megacystis-microcolon-intestinal hypoperistalsis syndrome, vesicoamniotic shuntingAbstract
Megacystis is a rare fetal condition characterized by an abnormal enlargement of the bladder, often associated with lower urinary tract obstructions, genetic mutations (e.g., ACTG2 in Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome), or syndromic disorders such as Prune Belly Syndrome.
Aim - to evaluate prenatal diagnostic techniques, assess the effectiveness of prenatal and postnatal interventions, and analyze long-term renal and urological outcomes in neonates diagnosed with megacystis.
This systematic review consolidates available case reports to evaluate prenatal diagnosis, treatment strategies, and long-term outcomes of neonates diagnosed with megacystis. This review was conducted in accordance with the PRISMA 2020 guidelines. Studies were included if they provided prenatal or postnatal diagnoses, genetic findings, interventions, and clinical outcomes. Risk of bias was evaluated by applying the Joanna Briggs Institute Critical Appraisal Checklist. Due to the heterogeneity of case reports, a narrative synthesis was performed, and findings were categorized based on intervention type and prognosis. A total of 20 case reports were included. The mean birth weight of affected neonates was 1152±532 g, with an average gestational age at diagnosis of 28±9.3 weeks. Prenatal interventions (n=5) demonstrated a 60% survival rate but were associated with preterm birth and oligohydramnios. Postnatal surgical management (n=8), including vesicostomy and catheterization, had a 75% survival rate, although some cases required long-term dependency on total parenteral nutrition due to associated comorbidities. Cases where no intervention was performed (n=7) resulted in 100% mortality, with intrauterine demise or termination due to severe structural anomalies. The survival rates were compared across different intervention groups, highlighting that those cases managed postnatally showed significantly better survival outcomes, while fetal intervention success varied based on gestational age and underlying etiology.
This review highlights the need for standardized diagnostic protocols, genetic screening, and prospective cohort studies to improve treatment decision-making and long-term care for affected neonates.
The authors declare they have no conflict of interest.
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