CONGENITAL MALFORMATIONS OF THE UPPER URINARY TRACT

PROF. D. A. NZEH DEPARTMENT OF RADIOLOGY UNIVERSITY OF ILORIN

ANOMALIES OF POSITION-1
PELVIC KIDNEY Results from failure of ascent of a kidney to its normal position in the lumbar region. During IVU, rule out pelvic kidney first before making a diagnosis of renal agenesis or non-functioning kidney. Diagnosis can be made on ultrasound or IVU. Usually has a short ureter which helps to distinguish it from a ptotic kidney. Blood supply is from an aberrant artery. Vulnerable to traumatic injury. INTRATHORACIC KIDNEY (SUPERIOR ECTOPIA) Occurs when upward migration of the kidney does not stop in the abdomen but continues into the thoracic cavity. Diagnosed by ultrasound and IVU. Tends to be unilateral. Appears as a posterior mediastinal mass projecting into the thorax from underneath the diaphragm. May be associated with diaphragmatic eventration or Bochdalek foramen hernia.

ANOMALIES OF POSITION-2
MALROTATED KIDNEY Results from failure of full rotation of the kidney during development. Calyces do not point laterally. CROSSED ECTOPIA (NON FUSED / FUSED) Position of the ectopic kidney is lower than that of the normal kidney. A crossed ectopic kidney may or may not be fused. The crossed ectopic kidney is almost always fused with the kidney on the opposite side. NEPHROPTOSIS Not a true congenital anomaly but there is downward kidney displacement. Has normal length of ureter which is usually not obstructed. Shows a higher degree of mobility than normal. May be better demonstrated on IVU by an erect view.

ANOMALIES OF NUMBER
SINGLE KIDNEY (DUE TO CONTRALATERAL AGENESIS) Malrotation and ectopia may be seen in the single kidney. Associated congenital anomalies are: vertebral/vascular; anorectal atresia; tracheal; esophageal fistula / atresia; radial / renal; plus uterine (bicornuate) VATER + U. Usually larger in size than an ordinary kidney. Kidney enlargement in renal agenesis is more marked than in secondary compensatory hypertrophy. Renal shadow usually absent on IVU on the affected side. Bladder is usually deformed when there is absence of ureter on the affected side. May be associated with bladder hypoplasia, sacral agenesis, small pelvis and congenital heart disease. SUPERNUMERARY KIDNEY The extra kidney is usually small-sized. Kidney on the ipsilateral side tends to be smaller than the normal kidney on the contralateral side. Diagnosis is clinched by demonstrating a separate blood supply, renal pelvis and ureter.

ANOMALIES OF SIZE-1
DUPLEX OR DOUBLE KIDNEY Occurs when more than one ureteric bud ascends during development. Affected kidney tends to be larger in size than normal. There are usually two collecting systems and two ureters. The two ureters become united or remain separate and drain independently. The ureter draining the upper moiety of a duplex collecting system always inserts distally and medially to the ureter of the lower moeity. Upper moeity ureter may also drain into ectopic locations such as the urethra, vagina and rectum. Collecting system of the upper moiety is prone to obstruction whereas that of the lower pole is predisposed to reflux. An obstructed, dilated and non-functioning upper moiety produces a mass effect which displaces the lower moiety downwards giving rise to the drooping lily sign on IVU. Renal duplication can either be unilateral or bilateral. When double ureters are fused in the upper segment, this may also cause reflux from one moiety to the other.

ANOMALIES OF SIZE-2
HYPOPLASTIC KIDNEY Usually associated with hyperplasia of the opposite kidney. Functions normally as demonstrated on IVU. Calyceal system and renal pelvis are small-sized. On renal angiography, ostium of the renal artery is small. It is important to distinguish it from contracted kidney arising from inflammation or vascular disease as follows: normal sized calyceal system; impaired renal function on IVU; diminished renal parenchymal tissue; normal ostium of the renal artery on angiography. HYPERPLASTIC KIDNEY Present when there is hypoplasia or agenesis of the contralateral kidney. The term compensatory hypertrophy is used to describe a kidney that is enlarged from causes other than contralateral hypoplastic kidney such as: infection, tumour and hydronephrosis or beer drinkers.

ANOMALIES OF FUSION-1
HORSESHOE KIDNEY Most common type of fusion anomaly. Lower poles are united by an isthmus which varies from thick parenchymal tissue to a thin string-like fibrous band. Lower poles are nearer the midline leading to reversal of the renal long axis. One or both kidneys are usually malrotated and it tends to be more on the left. Calyces point postero-medially and backwards instead of laterally. Ureters are stretched over the isthmus and this may lead to partial obstruction, hydrocalycosis, chronic inflammation and stone formation.

ANOMALIES OF FUSION-2
HORSESHOE KIDNEY (CONTD) IVU shows malrotation; kidney enlargement; varying degree of hydrocalycosis which may result in non function; partial obstruction usually at the PUJ; displacement of the upper ureters due to malrotation; and any calculus present. Blood supply to horseshoe kidneys is usually by multiple arteries while the isthmus is supplied by branches from the common iliac arteries. US, CT and MRI are useful to confirm diagnosis of horseshoe kidney.

ANOMALIES OF FUSION-3
CROSSED FUSED ECTOPIA Results from fusion of the kidneys on one side of the body. The ectopic kidney is lower in position, its ureter enters normally into the urinary bladder after crossing to the other side. The two fused kidneys tend to be lower in position and may show malrotation. May be associated with partial obstruction and this leads to inflammation and stone formation. PANCAKE OR DISCOID KIDNEY The discoid or pancake is a variant of crossed ectopia with fusion of the upper and lower poles. There is failure of rotation of the kidneys hence their calyces point posteriorly. Typically a discoid kidney is low positioned, lies around the midline and usually overlies the sacrum. The ureters are short but enter the bladder normally. Blood supply is aberrant and usually from the iliac vessels. Ureteric obstruction results in infection and stone formation. Vulnerable to traumatic injury. Pregnancy can cause compression and obstruction of the ureters.

MISCELLANEOUS RENAL ANOMALIES AND CONDITIONS-1

BENIGN CORTICAL NODULE (HYPERTROPHY) A normal variant due to presence of renal cortical tissue than usually seen in a part of the kidney. Depending on its location, three types of cortical nodules are recognized and are namely: hilar, subcapsular, and column (septa) of Bertin. During IVU, the differential diagnoses are: cyst and tumour; US helps to confirm a cyst but CT is necessary to distinguish b/w a normal cortical nodule or a tumour mass.
ABERRANT PAPILLA In normal circumstances, a renal papilla projects into a minor calyx.

An aberrant papilla projects directly into the lumen of an infundibulum. It appears round or oval shaped when seen en face. Multiple papillae may enter a single minor calyx and resemble blood clot or radiolucent calculus.
PERSISTENT FETAL LOBULATION

Present when fetal lobulation of the kidney remains till adult life. Should be distinguished from pyelonephritic scarring which is always present opposite a calyx .

MISCELLANEOUS RENAL ANOMALIES AND CONDITIONS-2

SOLITARY RENAL CALYX A very rare anomaly in which the whole kidney is drained by a single calyx which empties into bulbous tube-like renal pelvis. One or both kidneys may be involved . Morphologically, the kidneys of many other mammals have a solitary calyx. The affected kidney usually functions normally hence its presence does not actually indicate renal disease. MEGACALYCES Refers to anomalous enlargement of calyces inside one or both kidneys.

Usually associated with underdevelopment of the renal pyramids. Renal function is normal and there is no obstruction. Large calyceal size promotes stasis which predisposes to calculus formation and it in turn leads to infection.
DROMEDARY (OR SPLENIC) HUMP

Occurs on the lateral margin of the left kidney. Results from impression of the spleen on the kidney.

ANOMALIES OF THE RENAL PELVIS-1

PELVI-URETERIC JUNCTION (PUJ) DYSFUNCTION It is the most common congenital anomaly encountered in the urinary tract. Tends to be bilateral but not usually symmetrical. Usually more severe on the left than the right. May result in failure of peristaltic waves to pass affected part. Could cause severe renal enlargement in neonates.

PELVI-URETERIC JUNCTION (PUJ) OBSTRUCTION

Appears to be due to an intrinsic wall defect that is more functional in nature than anatomical. Injection of diuretic (frusemide) during IVU or radionuclide study establishes if the PUJ narrowing is of functional significance. Usually results from extrinsic cause such as a fibrous band or aberrant vessel. Cause of the extrinsic impression tends to angulate and fix the ureter at its origin thus making it to dilate. Has a strong association with other renal anomalies hence the need to thoroughly examine the opposite kidney. An intrinsic mucosal fold or web may be present as a rare association.

ANOMALIES OF THE RENAL PELVIS -2 EXTRA RENAL PELVIS Renal pelvis lies outside the kidney. Tends to be more dilated than normal especially during IVU. Does not normally show obstructive symptoms. On injection of diuretic (frusemide) during IVU, the pelvis drains normally into the ureter. DUPLICATION OF RENAL PELVIS (BIFID COLLECTING SYSTEM) May occur alone in mild form (duplex collecting system) and is of no clinical significance. May be associated with double kidney (different from supernumerary kidney) and double ureter. Multiple renal pelves may be rarely present and are associated with multiple ureters (e.g. triplex ureters).

ANOMALIES OF THE URETER-1

DUPLICATION OF THE URETER When incomplete, the ureters unite before reaching the bladder. Complete ureteric duplication leads to each ureter emptying separately into the bladder. Upper pole ureter is ventral to the lower pole ureter. Upper pole ureter crosses the lower ureter to empty at a lower position (Weigert-Meyer rule) in the bladder or ectopic location e.g. urethra, vagina or rectum. Upper pole ureter may be associated with ectopic ureterocele. Lower pole ureter is prone to vesico-ureteric reflux. ANOMALOUS POSITION OF URETERIC ORIFICE In males an abnormal ureter may open into the urethra, vas deferens, seminal vesicles or ejaculatory duct. In females, an ectopic ureter may open into the vagina, lateral vulvar wall, rectum or uterus. All patients with complete ureteric duplication have an ectopic insertion. Occasionally ectopic insertion may be seen in a single ureter. In females, ectopic ureteric insertion is below the urinary sphincteric mechanism and usually results in urinary incontinence. In males, ectopic ureteric insertion is above the urinary sphincteric mechanism and usually does not result in urinary incontinence. Cystoscopy is better able to identify an anomalous ureteric orifice compared to radiography.

ANOMALIES OF THE URETER-2

SIMPLE URETEROCELE Results from intravesical dilatation of the distal ureter immediately before its orifice in the bladder. Due to ureteric orifice stenosis combined with deficiency in connective tissue attachment of the ureter to the bladder. Gives a cobra-head appearance. Usually smaller than an ectopic ureterocele. Equal incidence in males and females. Usually discovered incidentally. Differential diagnosis is that of pseudoureterocele due to a bladder tumour. When filled with contrast during IVU, it shows a radiolucent halo. When not filled with contrast during IVU, it apears as a radiolucent mass (filling defect) in the bladder. Usually associated with double ureter. A calculus inside a vesical ureterocele does not move with change in patients position.

ANOMALIES OF THE URETER-3 ECTOPIC URETEROCELE Commoner in males than in females with a ratio of 6-7 : 1. Almost always associated with double ureter. Results from submucosal passage of the distal ureter which then terminates in the urethra instead of the bladder. It is due to dilatation of the intramural part of the ureter. Tends to be larger than the simple type of ureterocele. Usually extends to the anterior bladdder wall when viewed laterally. Differential diagnosis includes hydrometrocolpos.

ANOMALIES OF THE URETER-4

RETROCAVAL URETER Present only on the right side except in cases of situs inversus. May cause partial ureteric obstruction. Ureter moves to the left behind the IVC then to the right and then passes downwards in the normal position. S-type, J-type or fish-hook variants of the deformity may be encountered. When present, the site of narrowing or obstruction is proximal to the IVC. Diagnosis is made on IVU. Diagnosis can be confirmed at inferior vena cavography by placing an opaque catheter in the ureter. Easily demonstrated by CT which also helps to distinguish it from ureteric displacement by retroperitoneal masses e.g. lymphoma. Differential diagnoses include: retroperitoneal fibrosis and aortic aneurysm which both cause medial deviation of the ureter.

ANOMALIES OF THE URETER-5

URETERIC DIVERTICULUM A single ureteric diverticulum is believed to congenital. Multiple diverticula are almost always acquired and result from previous infection. A diverticulum appears as an outpouching . When multiple, they vary in size and number. A diverticulum is best demonstrated by retrograde pyelogram but may also be well defined on IVU. URETERIC JET Seen on US, CT and IVU. When present, it rules out the possibility of vesico-ureteric reflux or ureteric obstruction. TRANSVERSE URETERIC FOLDS May have a corkscrew appearance on IVU in infants caused by thin transverse folds. Are of no clinical significance. URETERIC VALVES Usually unilateral. May be annular with a pin-point. Commoner in the lower ureter but may also occur in other segments. Aetiology is uncertain. Transverse folds of ureteric mucosa contain smooth muscle fibres. There is obstruction above the valve but the ureter below it is normal.

ANOMALIES OF THE URETER-6

VERTICAL URETEROPELVIC STRIATIONS Occasional observed in renal pelvis and upper ureter in association with reflux. Appears to be a minor anatomical variant. Usually demonstrated by IVU or retrograde pyelogram. PRIMARY MEGA URETER It is due to functional abnormality of the distal ureter. Distal third of the ureter appears dilated on IVU. Affected ureter is not obstructed. Fluoroscopy demonstrates increased peristalsis. The proximal ureter and pelvicalyceal system are not usually dilated.

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