Congenital Anomalies of the Upper Urinary Tract
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Questions and Answers

What is the incidence of Unilateral Renal Agenesis (URA)?

  • 1:2000 births
  • 1:1800 births
  • 1:1400 births (correct)
  • 1:1000 births

    • Which of the following is a characteristic of polycystic kidney disease (PCK) in adults?

  • Only cysts present in one kidney
  • Complete absence of cysts in both kidneys
  • Presence of fewer than one cyst in each kidney
  • Presence of two or more cysts in each kidney after age 30-60 (correct)

    • What anomaly is characterized by the complete absence of a ureteric bud?

  • Hypoplasia
  • Horseshoe kidney
  • Supernumerary kidney
  • Unilateral renal agenesis (correct)

    • Which of the following anomalies is classified under anomalies of form and fusion?

    <p>Crossed ectopia</p> Signup and view all the answers

    What condition does a 'thoracic kidney' refer to?

    <p>A kidney located in the thoracic cavity</p> Signup and view all the answers

    What is the diagnosis method for ipsilateral adrenal agenesis?

    <p>U/S or IVU, CT scan</p> Signup and view all the answers

    What is a common clinical feature of crossed renal ectopia?

    <p>Asymptomatic</p> Signup and view all the answers

    What is the incidence of simple renal ectopia?

    <p>1 in 1000</p> Signup and view all the answers

    Which of the following statements about horseshoe kidney is correct?

    <p>It is the most common of all renal fusion anomalies.</p> Signup and view all the answers

    Which complication is more likely associated with simple renal ectopia?

    <p>Urinary calculus formation</p> Signup and view all the answers

    What is the most common location for cysts in the body?

    <p>Kidneys</p> Signup and view all the answers

    Which of the following types of polycystic kidney disease is associated with a higher percentage of offspring being affected?

    <p>Autosomal dominant</p> Signup and view all the answers

    Which symptom is linked to horseshoe kidneys?

    <p>Pelviureteric obstruction</p> Signup and view all the answers

    What is a common long-term prognosis for individuals with horseshoe kidneys?

    <p>Normal life expectancy</p> Signup and view all the answers

    How is infantile polycystic kidney disease characterized?

    <p>Autosomal recessive and incompatible with life</p> Signup and view all the answers

    Which diagnostic method is NOT typically used for diagnosing polycystic kidney disease?

    <p>Blood test</p> Signup and view all the answers

    What treatment method is recommended to manage renal impairment in polycystic kidney disease?

    <p>Dialysis and low protein diet</p> Signup and view all the answers

    What could be a potential complication from large cysts in polycystic kidney disease?

    <p>Cyst rupture and hematuria</p> Signup and view all the answers

    Which condition is characterized by the failure of the ureteric bud to fuse with metanephrose?

    <p>Simple renal cyst</p> Signup and view all the answers

    What is the most common site for ectopic ureters in males?

    <p>Posterior urethra</p> Signup and view all the answers

    How are symptomatic simple renal cysts most commonly treated?

    <p>Aspiration and injection of sclerosing agent</p> Signup and view all the answers

    Which statement about duplication of the ureter is true?

    <p>The ureters can independently open in the bladder.</p> Signup and view all the answers

    What is a potential complication of symptomatic simple renal cysts?

    <p>Hydronephrosis</p> Signup and view all the answers

    In partial duplication of the ureter, what is the likely configuration?

    <p>Two ureters draining a single kidney and uniting before the bladder</p> Signup and view all the answers

    What is the primary treatment option for ectopic ureters with minimal function?

    <p>Upper pole partial nephrectomy</p> Signup and view all the answers

    Which condition is rarely treated even when symptomatic?

    <p>Simple renal cyst</p> Signup and view all the answers

    What is a common clinical feature of ureteropelvic junction obstruction?

    <p>Episodic flank or upper abdominal pain</p> Signup and view all the answers

    What diagnosis method is typically used to visualize hydronephrosis associated with ureteropelvic junction obstruction?

    <p>U/S</p> Signup and view all the answers

    Which treatment option is appropriate for a non-functioning kidney due to ureteroceles?

    <p>Nephrectomy</p> Signup and view all the answers

    Which population is more commonly affected by ureteroceles?

    <p>Women</p> Signup and view all the answers

    What is the most common cause of significant dilation of the collecting system in fetal kidneys?

    <p>Ureteropelvic junction obstruction</p> Signup and view all the answers

    What imaging method provides a diagnostic sparing view for UPJ obstruction?

    <p>CT scan</p> Signup and view all the answers

    What clinical finding might be observed in the case of a retrocaval ureter?

    <p>Obstruction</p> Signup and view all the answers

    What is a possible treatment for a patient with UPJ obstruction?

    <p>Ureteric tapering &amp; reimplantation</p> Signup and view all the answers

    Study Notes

    Congenital Anomalies of the Upper Urinary Tract

    • Agenesis: Complete absence of a kidney, unilateral (1 in 1400 births) or bilateral (rare, incompatible with life).
    • Supernumerary Kidney: Presence of an extra kidney.
    • Hypoplasia: Underdeveloped kidney, smaller than normal.
    • Multicystic Kidney: Kidney with multiple cysts, can be classified as Polycystic Kidney Disease (PKD) depending on age and number of cysts.
      • Polycystic Kidney Disease: A group of genetic diseases characterized by the formation of multiple cysts in the kidneys, can be infantile or adult.
        • Infantile PKD: Rare, autosomal recessive, incompatible with life. Both kidneys are replaced by many cysts, often obstructing labor.
        • Adult PKD: Autosomal dominant, affects 50% of offspring. Both kidneys are replaced by large cysts, causing pressure atrophy of the renal parenchyma and potential ureteral compression.
    • Anomalies of Ascent: Kidney fails to reach its normal location in the "renal fossa".
      • Simple Ectopia: Kidney remains in a lower than normal position.
      • Cephalad Ectopia: Kidney remains in a higher than normal position.
      • Thoracic Kidney: Kidney resides in the chest cavity.
    • Anomalies of Form and Fusion: Involve the shape and connection of kidneys.
      • Crossed Ectopia: Kidney is located on the opposite side of the body, can be with or without fusion.
      • Horseshoe Kidney: Two renal masses lie on either side of the midline and are connected at their lower poles by a parenchymatous or fibrous isthmus.
        • Impeded by the inferior mesenteric artery, located in front of the fourth lumbar vertebra.
        • Blood supply varies, prone to pelviureteric obstruction, infection, and stone formation.
    • Anomalies of Rotation: Deviation from the normal kidney rotation.
    • Anomalies of the Collecting System: Abnormalities in the calyces, infundibulum, and pelvis.
      • Calyceal Diverticulum: Outpouching of the calyx.
      • Hydrocalyx: Dilation of the calyx due to obstruction.
      • Megacalycosis: Enlargement of the calyces.
      • Extrarenal Pelvis: Renal pelvis lies outside the kidney.
      • Bifid Pelvis: Renal pelvis is divided into two segments.
    • Anomalies of Renal Vasculature: Abnormalities in the renal blood vessels.
      • Aberrant, Accessory, or Multiple Vessels: Abnormal blood vessel location or number.
      • Renal Artery Aneurysm: Abnormal dilation of the renal artery.
      • Arteriovenous Fistula: Abnormal connection between an artery and vein.

    Unilateral Renal Agenesis (URA)

    • Incidence: 1 in 1400 births.
    • Often found accidentally, more frequent on the left side.
    • Complete absence of a ureteric bud or interrupted ureteral development prevents kidney maturation.
    • Ipsilateral adrenal agenesis is rare, but genital anomalies are more common.
    • Asymptomatic.
    • Diagnosed by ultrasound, IVU, or CT scan: absent kidney, compensatory hypertrophy of the contralateral kidney.
    • No specific treatment required.

    Simple Renal Ectopia

    • Kidney fails to reach its normal location.
    • Incidence: 1 in 1000.
    • Associated anomalies: contralateral agenesis, hydronephrosis (25%).
    • Asymptomatic.
    • Diagnosed by ultrasound, IVU, CT scan.
    • Not prone to disease, but hydronephrosis or stone formation can occur.

    Horseshoe Kidney

    • Common renal fusion anomaly, found in 1 in 1000 necropsies, more frequent in men.
    • Two renal masses, vertically connected by an isthmus, crossing the midline.
    • Low location.
    • Prone to pelviureteric obstruction, infection, and stones.
    • Diagnosed by ultrasound, IVU, CT scan.
    • Treatment: medical (infection control, pain relief), surgical (stone removal, PUJ stenosis correction, isthmus division).
    • Usually good prognosis, allowing for a normal life.

    Congenital Cystic Kidney (Polysystic Kidney)

    • Can be caused by autosomal dominant (ADPKD) or autosomal recessive (ARPKD) inheritance.
    • ADPKD: Autosomal dominant inheritance, both kidneys replaced by many cysts of varying sizes, leading to enlarged kidneys. Cysts contain clear fluid, sometimes blood.
      • Clinical Manifestations: Often asymptomatic before 40 years old. May experience pain, hematuria, infection, hypertension, or renal impairment.
      • Diagnosed by family history, ultrasound, IVU, CT scan, MRI.
      • Treatment: Medical (infection control, hypertension management, pain relief, anemia treatment), surgical (deroofing of large cysts causing symptoms or obstruction, stone removal), or renal transplantation (in cases of renal failure).
    • ARPKD: Autosomal recessive inheritance, rare, incompatible with life. Both kidneys are enlarged and replaced by cysts, often obstructing labor. Failure of ureteric bud to fuse contributes to this condition.

    Simple (Solitary) Renal Cyst

    • Common condition, single or multiple, uni- or bilateral.
    • Usually asymptomatic, diagnosed on ultrasound.
    • Rarely require treatment, except in cases where they are symptomatic: pain, heaviness, infection, bleeding, or hydronephrosis.
    • Treatment options: Aspiration and sclerosing agent injection, Rovsing's operation (deroofing), partial or total nephrectomy.
    • Malignant cysts require radical nephrectomy.
    • Hydatid cyst aspiration is contraindicated due to anaphylaxis risk and dissemination.

    Duplication of the Renal Pelvis and Ureter

    • Duplication of the Renal Pelvis: Incidence: 4%, more common on the left side.
    • Duplication of the Ureter: Incidence: 3%, ureters typically fuse with a common orifice in the bladder, but can open independently.
      • Clinical Features: Often asymptomatic, but prone to infections, calculi, and hydronephrosis.
      • Treatment: Expectant management.
    • Partial Duplication: More common, two ureters drain a single kidney for varying lengths before uniting in the bladder through a single orifice.
    • Complete Duplication: Less frequent, both ureters are fully duplicated, each opening into a separate orifice in the bladder.

    Ectopic Ureters

    • 80% associated with a duplicated collecting system.
    • In males, posterior urethra is the most common termination site.
    • In females, urethra and vestibule are the most common termination sites.
    • Clinical Features: Vary based on orifice location, continuous dribbling in females, UTIs in males.
    • Diagnosed by IVU, ultrasound, CT scan, and cystoscopy.
    • Treatment: Ureteric reimplantation or partial nephrectomy (or nephrectomy if single system).

    Congenital Megaureter

    • Dilated ureter greater than 7 mm.
    • Unilateral or bilateral, more common in males.
    • Asymptomatic, pain, recurrent UTIs.
    • Lower ureter may be obstructed.
    • Associated with vesicoureteral reflux.
    • Diagnosed by IVU.
    • Treatment: Infection control, excision of lower stenotic segment (if present), ureteric tapering and reimplantation, nephroureterectomy for nonfunctioning kidney.

    Postcaval (Retrocaval) Ureter

    • Right ureter passes behind the inferior vena cava.
    • Can cause obstruction.

    Ureteroceles

    • Dilated intramural portion of the ureter due to congenital atresia of the ureteric orifice.
    • More common in women.
    • Associated with ectopic ureters.
    • Prone to stones and UTIs.
    • Clinical Features: Asymptomatic; recurrent UTIs and hematuria.
    • Diagnosed by IVU ("cobra head" appearance), cystoscopy, and cystogram.
    • Treatment: No treatment for asymptomatic cases, cystoscopy with diathermy cauterization, nephrectomy for nonfunctioning kidney.
    • Complex cases may require ureteral reimplantation and vesical reconstruction.

    Ureteropelvic Junction (UPJ) Obstruction

    • Most common cause of collecting system dilation in the fetal kidney.
    • More frequent in boys, predominantly left-sided, 15% bilateral.
    • Etiology: Intraluminal mucosal fold, intrinsic interruption of UPJ circular musculature, extrinsic aberrant renal artery.
    • Presentation: Asymptomatic; episodic flank or upper abdominal pain, nausea and vomiting.
    • Diagnosed by ultrasound, IVU, CT scan, MRI, radionuclide renography, pressure-flow studies (Whitaker test).
    • Treatment: Medical (infection and pain control), surgical (pyeloplasty).

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    Congenital Anomalies of the Upper Urinary Tract (Lec 2) 2024 PDF

    Description

    This quiz covers various congenital anomalies affecting the upper urinary tract, including agenesis, supernumerary kidneys, and multicystic kidney disease. Explore the types of polycystic kidney disease, their genetic implications, and the impact on renal function. Test your knowledge on these rare conditions and their characteristics.

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