Prune belly syndrome: A mini-review and case report

Igor Vladimirovich Goremykin; Yuriy Marksovich Spivakovskiy; Elena Ivanovna Krasnova; Natalya Andreevna Kuznetsova

doi:10.5281/zenodo.8125630

Authors

Igor Vladimirovich Goremykin
Yuriy Marksovich Spivakovskiy
Elena Ivanovna Krasnova
Natalya Andreevna Kuznetsova

DOI:

https://doi.org/10.5281/zenodo.8125630

Keywords:

Children, chronic kidney disease, kidney transplantation, Megacystis, Prune belly syndrome

Abstract

Prune belly syndrome is a multisystem disease that is clinically manifested by a combination of hypoplasia of the abdominal muscles, bilateral cryptorchidism, and congenital anomalies of the urinary system of varying severity. The prognosis for patients with Prune belly syndrome is mainly determined by the extent of renal parenchyma dysplasia. An interdisciplinary approach involving urologists, nephrologists, surgeons, endocrinologists and other specialists allows us to improve urodynamics, to achieve stable remission of chronic obstructive pyelonephritis to perform kidney transplantation in childhood with a good result.

The article provides a mini-review of the current state of the art in etiopathogenesis, classification, diagnosis and criteria for differentiating patients according to the severity of manifestations. A clinical report of a patient with Prune belly syndrome, which was diagnosed at the age of 7 months. Who developed bilateral refluxing ureterohydronephrosis on the background of severe dysplasia of the renal parenchyma. Staged surgical correction of bilateral refluxing ureterohydronephrosis and orchiopexy at the age of 1 to 3 years was carried out. However, the loss of kidney function caused transplantation at the age of 11 years. The follow-up of this patient to the age of 15 years was presented and an evaluation of the results was discussed.

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