Nephrology for the General Practitioner - O.I. Bakaliuk 2003

Certain Urological Conditions in Therapeutic Practice
Congenital Renal Anomalies

Renal anomalies account for approximately 40% of all human Congenital Malformations, and their diverse and atypical Clinical presentation makes timely Diagnosis extremely challenging. The generally accepted Classification of these pathological conditions comprises 6 groups: anomalies of number, size, position, shape, Structure OF THE renal vessels, and combined malformations (M.F. Trapeznikov et al., 1978).

Below is a Brief Overview of those most commonly encountered in family practice.

Renal agenesis. The Clinical symptoms of renal agenesis are minimal when the contralateral Kidney Functions adequately—typically presenting as a dull, mild ache in the lower back caused by Hypertrophy of the contralateral kidney. Occasionally, the initial symptoms are related to The Development of RVH.

Renal Hypoplasia is most frequently unilateral. Clinical symptoms are subtle or entirely absent, and urinalysis changes are minimal. In such cases, it is crucial to determine the functional status of the hypoplastic kidney and its contribution to the development of concomitant UTI or RVH, the symptoms of which most often prompt the patient to seek medical attention.

Renal dystopia is classified into thoracic, lumbar, iliac, and pelvic forms, as well as homo- and heterolateral (Fig. 40). Iliac dystopia is the most common, where the kidney is located at the level of the iliac wing. Clinically, it manifests as pain in the iliac regions due to pressure exerted by the dystopic kidney on adjacent Organs, alongside dysuric disorders.

Horseshoe kidney. In this anomaly, the Kidneys are fused at their lower poles in 95% of cases. Clinical symptoms are numerous and non-specific: intermittent lower back pain, dysuric disorders caused by ureteral kinking, impaired urine outflow and reflux, lower extremity edema due to INFERIOR VENA CAVA compression, or ischemic symptoms resulting from aortic compression. Symptoms of RVH, nephrocalcinosis, and UTI are quite common. In some cases, patients experience epigastric and periumbilical pain, as well as dyspeptic disorders (Gutierrez syndrome). Urinalysis findings in uncomplicated cases are typically unremarkable (moderate PU, aseptic leukocyturia), though a notable prevalence of isolated microhematuria should be noted.

Accessory renal artery. In this anomaly, an accessory renal vessel supplies the lower segment of the kidney, resulting in clinical signs of Hydronephrosis due to impeded urine drainage.

In Froule's syndrome, a variant of renal vascular anomalies, the anterior and posterior Branches of the superior renal artery compress the infundibulum of the upper calyx, leading to its dilation and urinary stasis. Clinically, this presents as persistent flank pain, moderate HTN, and a predisposition to urolithiasis.

The key in such situations is first to suspect these anomalies, as their diagnosis is generally straightforward. Instrumental Diagnostic Methods are primarily utilized (chromocystoscopy, ultrasound, renal duplex Doppler Ultrasonography, excretory urography, computed tomography, and renal angiography), complemented by urine culture, Nechiporenko, Zimnitsky, and Reberg-Tareyev tests, among others.

Treatment is symptomatic. Nephrectomy is indicated when an anomaly is definitively linked to refractory RVH or recurrent UTI.