Congenital Anomalies of Kidney Quiz
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Questions and Answers

What is the main purpose of a renogram in the context of hydronephrosis?

  • To assess bladder function
  • To differentiate between obstructive and nonobstructive hydronephrosis (correct)
  • To evaluate renal blood flow
  • To measure kidney size

Which statement about medical therapy for UPJ obstruction is correct?

  • Conservative treatment is only applicable to adults.
  • Medical therapy has shown to be effective in both adults and children.
  • Monitoring is the primary method of treatment in children initially. (correct)
  • It can effectively reverse UPJ obstruction in adults.

What is the gold standard surgical procedure for UPJ obstruction?

  • Laparoscopic pyeloplasty
  • Endopyelotomy
  • Open pyeloplasty (correct)
  • Pyelolithotomy

Which of the following is NOT one of the accepted criteria for surgical intervention in infants and children?

<p>Urinary tract infection history (D)</p> Signup and view all the answers

What is the success rate of endopyelotomy compared to other surgical options?

<p>80-90% (A)</p> Signup and view all the answers

What is the main issue associated with renal agenesis?

<p>The ureteral bud fails to connect with the metanephric blastema (C)</p> Signup and view all the answers

Which condition is characterized by the absence of both kidneys?

<p>Bilateral renal agenesis (D)</p> Signup and view all the answers

At what gestational age does nephrogenesis typically cease?

<p>36 weeks (B)</p> Signup and view all the answers

What is a common outcome of unilateral renal agenesis?

<p>Hypertrophy of the contralateral kidney (D)</p> Signup and view all the answers

What is the condition referred to as Potter’s syndrome associated with?

<p>Bilateral renal agenesis (D)</p> Signup and view all the answers

In cases of renal hypoplasia, which of the following statements is correct?

<p>The kidneys are smaller with a reduced number of calyces and nephrons (C)</p> Signup and view all the answers

What are potential associated anomalies with bilateral renal agenesis indicated by oligohydramnios?

<p>Cardiovascular and gastrointestinal abnormalities (D)</p> Signup and view all the answers

What is the term for a kidney with partially developed or atrophic renal cortex?

<p>Ask-Upmark kidney (D)</p> Signup and view all the answers

What condition is characterized by a rudimentary kidney?

<p>Aplasia (D)</p> Signup and view all the answers

Which type of kidney abnormality is associated with the presence of large non-communicating cysts and absence of renal cortex?

<p>Multicystic dysplastic kidney (MCDK) (B)</p> Signup and view all the answers

What is the primary method for diagnosing Multicystic Dysplastic Kidney (MCDK)?

<p>Ultrasound (C)</p> Signup and view all the answers

Which of the following complications is NOT associated with Multicystic Dysplastic Kidney (MCDK)?

<p>Pulmonary embolism (C)</p> Signup and view all the answers

Which structural abnormality involves the failure of the kidney to ascend during embryogenesis?

<p>Ectopic kidney (A)</p> Signup and view all the answers

What percentage of patients with horseshoe kidney experience vesicoureteral reflux (VUR) as a complication?

<p>50% (C)</p> Signup and view all the answers

What is the implication of a kidney being described as 'larger than normal' in Multicystic Dysplastic Kidney (MCDK)?

<p>It may lead to hypertension (A)</p> Signup and view all the answers

What is the relationship between associated anomalies and ectopic kidney?

<p>Commonly associated with testicular underdevelopment (B)</p> Signup and view all the answers

What is the most common side affected by ureteropelvic junction (UPJ) obstruction?

<p>Left side (B)</p> Signup and view all the answers

Which imaging technique is primarily used to detect vesicoureteral reflux (VUR) and posterior urethral valves (PUV)?

<p>Voiding Cystourethrogram (VCUG) (D)</p> Signup and view all the answers

What is a common clinical presentation of UPJ obstruction in adults?

<p>Intermittent flank pain (A)</p> Signup and view all the answers

Which statement about the epidemiology of UPJ obstruction is correct?

<p>The male-to-female ratio is approximately 3-4:1. (D)</p> Signup and view all the answers

Which condition can lead to urinary obstruction at the ureteropelvic junction?

<p>Iatrogenic obstruction from surgical interventions (D)</p> Signup and view all the answers

What is a potential complication of persistent hydronephrosis due to UPJ obstruction?

<p>Urinary tract infections (UTI) (D)</p> Signup and view all the answers

In neonates with hydronephrosis, which symptom is typically present?

<p>Flank mass (B)</p> Signup and view all the answers

What characteristic finding is often seen in a Doppler ultrasound of an obstructed kidney?

<p>High resistive indices (A)</p> Signup and view all the answers

What describes a duplex kidney?

<p>It may have an ectopic ureter draining into the vagina. (B), It has two ureters draining to a single bladder. (D)</p> Signup and view all the answers

Which condition is characterized by a kidney crossing the midline?

<p>Crossed fused ectopia (A)</p> Signup and view all the answers

What is often a cause of hydronephrosis?

<p>Ureteropelvic junction obstruction (UPJO) (A)</p> Signup and view all the answers

What are the primary features of hydronephrosis detected via antenatal ultrasound?

<p>Obstruction above the bladder (A), Renal parenchyma becoming echogenic (D)</p> Signup and view all the answers

In a complete duplex kidney, where does the upper pole ureter typically drain?

<p>Into the lower bladder (A)</p> Signup and view all the answers

What grading system is used to assess the severity of hydronephrosis?

<p>Assessment of renal pelvic diameter (A)</p> Signup and view all the answers

What typically characterizes incomplete duplex kidneys?

<p>Two ureters joining prior to bladder entry (A)</p> Signup and view all the answers

What is a critical aspect when assessing hydronephrosis in a prenatal ultrasound?

<p>It is more accurately detected between 18-20 weeks. (C)</p> Signup and view all the answers

Flashcards

Unilateral Renal Agenesis

A rare condition where one kidney does not develop in the fetus. The other kidney usually grows larger to compensate but can be prone to problems like VUR (vesicoureteral reflux).

Bilateral Renal Agenesis

A life-threatening condition where both kidneys are absent. This leads to lack of amniotic fluid (oligohydramnios) causing severe lung problems (pulmonary hypoplasia) and a distinctive facial appearance.

Renal Hypoplasia

A kidney smaller than normal but with a normal structure. The number of nephrons (functional units of the kidney) is reduced. It may be discovered incidentally or lead to chronic renal failure in severe cases.

Segmental Hypoplasia (Ask-Upmark Kidney)

A rare condition where one part of a kidney does not develop properly, resulting in an uneven appearance. Sometimes these parts may have a poor blood supply leading to problems with function.

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Renal Agenesis

A congenital abnormality of the urinary system where the ureteral bud (which helps the kidney develop) fails to connect to the metanephric blastema (kidney tissue) resulting in kidney absence.

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Nephrogenesis

The process of kidney development in the fetus, involving the formation of nephrons (functional units of the kidney) from the metanephric blastema, with the help of the ureteral bud.

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Pronephros

Early stage of kidney development in the fetus where the pronephros, a primitive kidney structure, forms but quickly regresses. This is replaced by the mesonephros, which becomes important for urine production in the early stages.

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Metanephros

The final stage of kidney development where the permanent kidney, the metanephros, forms. This stage involves complex interactions between the ureteral bud and the metanephric blastema.

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Renal Aplasia

A rare condition where the kidneys are not fully developed and lack normal tissue, leading to a small and dysfunctional structure. This is also known as a rudimentary kidney.

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Renal Dysplasia

A condition where the kidney's structure is abnormal, characterized by cysts and unusual cartilage formation. It can affect the entire kidney or just parts of it.

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Polycystic Kidney Disease (PKD)

A developmental abnormality where both kidneys have multiple cysts and lack normal function, occurring more often in adults. It's inherited and can be either autosomal dominant (ADPKD) or recessive (ARPKD).

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Multicystic Dysplastic Kidney (MCDK)

A congenital condition where one kidney is replaced by multiple cysts and lacks function. The other kidney typically grows larger to compensate. It's the most common cause of a large abdominal mass in infants and primarily affects males.

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Ectopic Kidney

A condition where the kidney fails to ascend to its normal position in the abdomen during development. It can be located in the pelvis, thorax, or even cross the midline.

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Horseshoe Kidney

A rare fusion anomaly where the lower poles of both kidneys merge together, forming a horseshoe shape. It's more common in males and often comes with other anomalies.

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Contra-lateral Kidney Hypertrophy

A condition where the kidney is larger than normal due to compensatory hypertrophy. This is often seen in cases like MCDK, where one kidney fails to develop and the other takes over its function.

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DMSA Scan

An investigation used to assess kidney function. A radioactive substance is given to the patient and its uptake by the kidneys is monitored. It helps differentiate between MCDK and hydronephrosis, showing no function in the affected kidney.

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Crossed Fused Ectopia

A condition where one kidney crosses the midline and fuses to the lower pole of the other kidney, with both ureters inserting in their normal positions.

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Duplex Kidney

A congenital condition where a kidney has two distinct drainage systems (pelvicalyceal systems), each with its own ureter. This can be complete (two separate ureters) or incomplete (ureters merging before reaching the bladder).

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Complete Duplex Kidney

A complete duplex kidney with two distinct ureters, where the upper pole ureter opens lowermost and medially into the bladder, making it prone to obstruction.

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Hydronephrosis

An abnormal dilation of the renal pelvis and collecting system, which can be identified in the womb using ultrasound.

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Upper Pole Ureteropelvic Junction Obstruction (UPJO)

A narrowing or blockage of the upper part of the ureter (where it connects to the kidney), leading to hydronephrosis.

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Vesicoureteral Reflux (VUR)

A condition where urine flows backward from the bladder into the ureters and kidneys. This can happen due to a faulty valve at the junction of the ureter and bladder.

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Megaureter

An abnormally large ureter, often caused by a blockage or structural abnormality. This can lead to dilation and problems with urine flow.

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Ureterocele

A pouch or sac-like structure in the ureter, usually near the bladder, that causes a block and leads to problems emptying the kidney.

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Posterior Urethral Valve (PUV)

A condition where the urethra is blocked, usually due to a membrane or valve, causing urine to back up into the bladder and kidneys.

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Ureteropelvic Junction (UPJ) Obstruction

A blockage at the junction of the kidney and ureter, preventing urine from flowing out of the kidney. This can be caused by factors like stenosis, hypoplasia, or a constricted vessel.

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Voiding Cystourethrogram (VCUG)

A test that uses a contrast dye to visualize the kidneys and bladder and detect problems like VUR or reflux.

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Diuretic Renogram

This test uses a radioisotope and a diuretic to assess kidney function and detect obstruction. If kidney function is poor or there is a delayed drainage, it signifies an obstruction.

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Ultrasound (US)

A technique that uses sound waves to create images of the kidneys and urinary tract. It helps detect dilation or other abnormalities.

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What is a Renogram?

A type of kidney scan that uses a special dye and X-rays to check for blockages in the urinary tract.

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What is Hydronephrosis?

A condition where the kidneys swell up with extra fluid.

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What's a UPJ Obstruction?

This is a blockage or narrowing of the tube that connects the kidney to the bladder (ureter), specifically at the upper junction.

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What is Pyeloplasty?

A surgical procedure to widen a narrowed area in the tube connecting the kidney to the bladder.

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What is Endopyelotomy?

A less invasive procedure where a tiny incision is made in the tube connecting the kidney to the bladder using a special balloon.

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Study Notes

Congenital Anomalies of Kidney

  • Congenital kidney anomalies are structural abnormalities present at birth.
  • Kidney development occurs in three phases: pronephros, mesonephros, and metanephros.
  • Kidney development begins around the 4th week and finishes around the 36th week of intrauterine life.

Renal Agenesis (Absent Kidney)

  • Renal agenesis is a condition where one or both kidneys are absent.
  • It's caused by failure of the ureteral bud to communicate with the metanephric blastema.
  • This condition is relatively rare (1 in 500 to 1 in 3200 live births).
  • Unilateral agenesis sometimes goes undiagnosed until later in life.
  • Bilateral agenesis usually leads to fatal outcomes soon after birth due to respiratory issues.

Renal Hypoplasia

  • Renal hypoplasia involves a smaller-than-normal kidney size.
  • It's not dysplastic, meaning the structure is typical.
  • It has a reduced number of calyces, nephrons, and glomeruli.
  • Unilateral hypoplasia is frequently an incidental finding, often associated with other urinary tract issues or hypertension.
  • Bilateral hypoplasia can be compatible with life, with chronic kidney disease as a possible outcome.
  • Segmental hypoplasia presents as a kidney with only partial development or atrophied renal tissue.

Renal Dysplasia

  • Renal dysplasia involves structural abnormalities.
  • It can affect parts or all of the kidney.
  • Manifestations are cysts and cartilage-like formations within the kidney.
  • Polycystic kidney disease (PKD) is a hereditary form most commonly presenting as bilateral disease.
  • Autosomal-dominant PKD (ADPKD): affects mainly adults.
  • Autosomal-recessive PKD (ARPKD): usually found in children.
  • Multicystic dysplastic kidney (MCDK) is among the most common congenital anomalies in children.
  • MCDK usually involves a non-functioning kidney replaced by many cysts.

Abnormalities in Shape and Position

  • Ectopic kidney: A kidney that doesn't ascend to its normal position during development can be in the pelvis, thorax, or other abnormal areas.
  • Horseshoe kidney: The lower poles of the kidneys fuse across the midline, leading to a "horseshoe" shape. The isthmus is at the level of L4-L5.
  • Crossed fused ectopia: One kidney crosses the midline to the opposite side of the body and is fused to the normal kidney's lower pole.

Abnormalities of the Collecting System

  • Duplex kidney: Characterized by two collecting systems within one kidney, and two ureters. Complete or incomplete.
  • Hydronephrosis: Dilated renal pelvis/collecting system. Possible causes include UPJO. It is more common in the left kidney., It can be detected during fetal ultrasounds (antenatal) and can be assessed further by further diagnostics to pinpoint the cause.

Diagnostic Tests

  • Ultrasound (US).
  • Voiding Cystourethrogram (VCUG).
  • Diuretic Renography.
  • Doppler sonography.

Medical and Surgical Treatments

  • Conservative management for cysts under 5cm.
  • Nephrectomy for cysts over 5cm or significant complications, or if no involution is seen by age 2.
  • Pyeloplasty: surgical correction of ureteropelvic junction obstruction.
    • Laparoscopic
    • Open
    • RALP.
  • Endopyelotomy: minimally invasive procedure using a balloon to open the obstructed area.

Etiology

  • Intrinsic obstruction: narrowing of the ureters due to scar tissue.
  • Extrinsic obstruction: compression of the ureters from nearby structures like vessels.

Epidemiology

  • Congenital anomalies of the kidneys are relatively common, occurring in about 1 in 100 pregnancies.
  • Left kidney more commonly affected than right
  • Male to female ratio varies from 3-4:1

Clinical Presentation

  • Neonates: primarily present with hydronephrosis
  • Older children: include urinary tract infections (UTIs), flank pain, flank masses, and hematuria.
  • Adults: include back and flank pain, UTIs, pyelonephritis, and hypertension.

Ureteropelvic Junction Obstruction (UPJ)

  • A common cause of hydronephrosis.
  • Most commonly detected in left kidney during antenatal ultrasound
  • A frequent cause of hydronephrosis.

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Description

This quiz explores congenital anomalies of the kidney, including renal agenesis and renal hypoplasia. You'll learn about the developmental phases of the kidneys and the implications of these structural abnormalities. Test your knowledge on the causes, prevalence, and outcomes of these conditions.

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