(SYNONYM: CONGENITAL CYSTIC KIDNEY) Introduction: Inherited as autosomal dominant disease
Bilateral
With standard imaging technique, can be detected at 2nd or 3rd decades
Manifested clinically in the third decades
Other associations-
Polycystic diseases of liver (18%), pancreas, lungs
Pathology: Gross-Kidneys become enormously enlarged bilaterally
The cyst giving the appearance of a collection of bubbles below the renal capsule.
Histological-The renal parenchyma is riddled with cysts of varying size containing clear fluid, thick brown material or sometimes hemorrhagic fluid. (Robins)
Clinical features: Incidence more in women than men.
1. Irregular upper quadrant abdominal mass
2. Loin pain
3. Hematuria
4. Infection
5. Hypertension
6. Uraemia
The patients are “4H club members” with (1777, Swartz)
Headache
Hypertension
Hematuria
Heredity
Irregular upper quadrant abdominal mass: Bilateral knobby enlargement, dull renal angle, resonant band in front.
Loin pain: It is due to weight of the organ dragging upon its pedicle or by stretching of the renal capsule by the cyst. Hemorrhage into a cyst may cause more severe pain, as may the passage of a calculus from the diseased kidney.
Hematuria: Rupture of a cyst into the renal pelvis may cause hematuria which is typically moderate, lasts for a few days & recur at intervals.
Infection: Pyelonephritis is common in patients with congenital cystic kidney, presumely because of urinary stasis.
Hypertension: Present in up to 75 % of patients over the age 20 years with polycystic kidney. It is possibly result from a separate genetic factor linked to the gene for congenital cystic kidneys.
Uraemia: Patients pass large volumes of urine of low specific gravity (1.010 or less). Chronic renal failure develops as functioning renal tissue is replaced progressively by cysts.
Patients complain of anorexia, headache and vague abdominal discomfort. Vomiting and drowsiness due to biochemical derangement.
Investigation:
USG
CT scan
FNA
IVU (Excretory urography)
Blood urea
Serum creatinine
Urine shows low sp. Gravity
As kidney failure develops, a low protein diet will help to post-pone the need for renal replacement.
Conservative treatment for infection, anemia and correction of disturbed Ca metabolism.
Surgical treatment: uncap the cyst (Rovsing’s operation) it is rarely indicated, it is thought that this can preserve renal function by relieving pressure on the parenchyma. It reduces the pain. This can also be done just by aspirating the cyst either under USG guidance or laparoscopically.
Once renal failure sets in, then initial hemodialysis followed by bilateral nephrectomy, is done later renal transplantation is planned for.
POLYCYSTIC KIDNEY DISEASE
Distinct age group of presentation (1030, RL Gupta)
Perinatal: The infant is either stillborn or dies within a few weeks of birth.
Neonatal: It presents in the first month and die within one year fron renal failure.
Infantile: It presents at 3-6 months with large kidneys and hepatosplenomegaly. These patients die in childhood with systemic and portal hyper tension and renal failure.
Juvenile: This group presents in childhood and patients die in their teens from hypertension and its complications.
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