A clinical case of surgical treatment of a large kidney cyst in a 9-year-old girl with autosomal dominant polycystic kidney disease

Anna A. Oganisyan , Artem S. Vrublevskiy , Revaz Y. Valiev , Filipp O. Turov , Mamay M. Khanov , Sergey G. Vrublevskiy , Elena N. Vrublevskaya

Russian Journal of Pediatric Surgery ›› 2023, Vol. 27 ›› Issue (6) : 462 -469.

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Russian Journal of Pediatric Surgery ›› 2023, Vol. 27 ›› Issue (6) :462 -469. DOI: 10.17816/ps783
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A clinical case of surgical treatment of a large kidney cyst in a 9-year-old girl with autosomal dominant polycystic kidney disease

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Abstract

BACKGROUND: Autosomal dominant polycystic kidney disease is currently the most common inherited form of cystic kidney disease, the frequency of which is about 1 per 400–1000 newborns, what makes the problem relevant. Its progressive course in patients with polycystic disease is primarily associated with the ischemic component, the severity of which depends on the number, location and size of cysts. Arterial hypertension, which is an autosomal dominant polycystic kidney disease complication, plays a leading role in renal failure progression. Given the current lack of specific treatment, the main therapeutic goal is to prevent complications, postpone the development of renal failure. Therefore, if there are large and giant cysts, surgical treatment is an effective modality and should be performed in a timely manner.

CLINICAL CASE DESCRIPTION: The authors present a clinical case of a successful endovideosurgical cyst excision in a -year-old patient with a giant cyst in the right kidney, autosomal dominant polycystic disease, and hypertension of renal genesis.

Endovideosurgical cystectomy in the patient with autosomal dominant polycystic kidney disease has become an important pathogenetic link in the treatment of this pathology. Currently, the patient is under dynamic follow-up for more than one year. During the follow-up period, the patient demonstrates relief of urinary syndrome in the form of disappeared haematuria, stabilization of albuminuria indices, improvement of blood flow parameters in the renal parenchyma, as well as stabilization of arterial pressure.

CONCLUSION: The given clinical example demonstrates that reasonable surgical intervention with the choice of a rational method of access, taking into account the anatomical localization and size of the cystic formation, allowed to obtain a good result in the form of stabilization of renal hemodynamics in a patient with autosomal dominant polycystic kidney disease.

Keywords

renal cysts / autosomal dominant polycystic kidney disease / endourology / pediatric urology

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Anna A. Oganisyan, Artem S. Vrublevskiy, Revaz Y. Valiev, Filipp O. Turov, Mamay M. Khanov, Sergey G. Vrublevskiy, Elena N. Vrublevskaya. A clinical case of surgical treatment of a large kidney cyst in a 9-year-old girl with autosomal dominant polycystic kidney disease. Russian Journal of Pediatric Surgery, 2023, 27(6): 462-469 DOI:10.17816/ps783

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Oganisyan A.A., Vrublevskiy A.S., Valiev R.Y., Turov F.O., Khanov M.M., Vrublevskiy S.G., Vrublevskaya E.N.

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