CONGENITAL ANOMALIES

Congenital Anomalies occur more frequently in the kidney than in any other organ.  Many of these anomalies cause impairment of renal function.

RENAL AGENESIS

Bilateral Renal Agenesis is very rare but unilateral agenesis is common.  Unilateral Agenesis causes no symptoms; it is usually discovered by accident on x-rays or ultrasound examinations.

RENAL HYPOPLASIA

Hypoplastic Kidneys are smaller in size and have poor function.  Many are dysplastic.  Hypoplastic Kidneys have small Renal arteries and are associated with Hypertension.  Some of the acquired reasons to have Hypoplastic Kidneys include vesico-ureteral reflux, pyelonephritis, and renal artery stenosis.

SUPER NUMERARY KIDNEYS

Presence of a third Kidney is a very rare condition.

MULTICYSTIC KIDNEY

This condition is usually unilateral, nonhereditary.  The entire Kidney is made of irregular small cysts.  This condition happens because of faulty union of the nephron and collecting system.

ADULT POLYCYSTIC KIDNEY DISEASE

• Autosomal dominant hereditary condition
• Always bilateral
• Symptoms occur after age 40
• Kidneys are very large and studded with cysts
• There may be cysts in the liver, spleen, and pancreas
• Symptoms include microscopic or gross hematuria, flank pain, hypertension
• Laboratory tests may show anemia, uremia, proteinuria, and hematuria
• Diagnosis is made by renal ultrasound and CT-Scan
• Surgical decompression or excision of cysts do not improve renal function
• There is no higher incidence of renal cell carcinoma in these patients
• 50% of the children of affected adults will have the disease
• When the disease affects children, it has a very poor prognosis

SIMPLE CYST

Simple Cyst of the Kidney is usually single, unilateral, but may be multiple and bilateral.  Clinically and pathologically it is quite different than polycystic kidneys.  If the diagnosis of simple renal cyst is clearly established, there is no need for any surgical intervention.  It is rare for a cyst to harm the Kidney.

HORSESHOE KIDNEY

Although very rare, this is the most common type of fusion anamoly of the kidney.  Both renal units are joined at the lower pole.  Most patients have no symptoms, however some may develop ureteral obstruction.

ECTOPIC KIDNEY

Also called pelvic kidney, here the kidney fails to ascend normally.

CROSSED ECTOPY

In this condition both Kidneys are on the same side of the body, but they are not attached to each other.

ABNORMAL ROTATION

Normally during 9 months of gestation, Kidneys ascend from Pelvis up and as they ascend, they also, rotate.  Rarely, such rotation may fail to occur.

MEDULLARY SPONGE KIDNEY

Medullary Sponge Kidney is a rare congenital autosomal recessive disease.  It is characterized by dilatation of the distal portion of the collecting ducts with numerous associated cysts and diverticuli.  In many patients, these collecting ducts are filled with calcifications.  Majority of patients have no symptoms, but few have renal colic, urinary tract infections or gross hematuria. Many patients with Medullary sponge kidney have hypercalcemia, Thiazides are effective form of treatment for lowering hypercalcemia and limiting stone formation even if hypercalciuria is not present. If Thiazides are ineffective or not tolerated, inorganic phosphates should be used.

CALYCEAL DIVERTICULUM

A calyceal diverticulum is an intrarenal sac that communicates with the pelviocalyceal system. Calyceal diverticula are generally asymptomatic.