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[The so-called megaureter-megacystis syndrome: a case report].

Y Kurokawa¹, L K Soo, K Yokota

¹Department of Urology, School of Medicine, Tokushima University.

Nihon Hinyokika Gakkai Zasshi. the Japanese Journal of Urology

|October 12, 1999

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Megaureter-megacystis syndrome is a rare condition characterized by a giant bladder and dilated ureters. This case highlights a rare presentation in a 4-year-old boy, emphasizing the need for timely diagnosis and intervention.

Area of Science:

Pediatric Urology
Nephrology
Medical Imaging

Background:

Megaureter-megacystis syndrome is a rare congenital anomaly characterized by bladder enlargement and ureteral dilation.
The condition can lead to significant urinary tract complications, including infections and kidney damage.

Observation:

A 4-year-old boy presented with symptoms of urinary tract infection, kidney dysfunction, and abdominal pain.
Diagnostic imaging revealed bilateral hydronephrosis, hydroureters, and a significantly enlarged bladder (megacystis).
Voiding cystourethrography confirmed bilateral grade V vesicoureteral reflux, indicating severe backward flow of urine.

Findings:

The patient was diagnosed with megaureter-megacystis syndrome, a rare condition with only two previous reports in Japan.

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Functional studies showed a large bladder volume with a notable post-void residual volume.

The severity of the vesicoureteral reflux necessitated surgical intervention.

Implications:

This case underscores the importance of early recognition and comprehensive evaluation of megaureter-megacystis syndrome in pediatric patients.
Surgical management, such as the Cohen's antireflux procedure, can effectively address severe vesicoureteral reflux.
Further research into the long-term outcomes and management strategies for this rare syndrome is warranted.