CONGENITAL ANOMALIES OF THE KIDNEY AND

URINARY TRACT

INTRODUCTION
Congenital anomalies of the kidney and urinary tract (CAKUT) are a group of phenotypically
diverse structural malformations characterized by defects in renal and urinary tract development.
Nearly half of children who develop end-stage renal disease (ESRD) have asymmetric,
irregularly shaped kidneys, often referred to as bilateral renal scarring and frequently associated
with lower urinary tract anomalies, including vesicoureteral reflux (VUR).
CLINICAL PRINCIPLES
Congenital renal tract abnormalities may present in one of the following five settings:
1. Antenatal diagnosis by fetal ultrasound screening
2. Failure to thrive in an infant or young child
3. Investigation of urinary tract infection (UTI)
4. An incidental finding in a child or adult
5. An adult with abnormal urinalysis, stones, hypertension, or renal impairment
DEVELOPMENT OF THE KIDNEY AND URINARY TRACT
 The kidneys and urinary tract develop simultaneously from the cloaca and intermediate
mesoderm .
 Kidney development can be divided into three phases; the pronephros, mesonephros, and
metanephros.
 The pronephros develops 22 days after conception and forms a transient, rudimentary,
and nonfunctioning system that degrades by day 28.
 It elongates caudally to meet the cloaca by day 26, becoming the mesonephric (Wolfian)
duct, which ultimately contributes to the formation of the urinary bladder and male
genital system (epididymis and caudal vas deferens).
 Functioning mesonephric tubules develop from the intermediate mesoderm and start to
excrete urine, although most of these subsequently degenerate.
 By the 5th week of fetal life the ureteral bud branches from the caudal part of the
mesonephric duct into the metanephric mesenchyme to become the metanephros, the
precursor to the adult kidney.

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 This process is mediated by the GDNF/c-RET/Wnt-11 signaling pathway, disruptions of
which can result in varying phenotypes, including renal agenesis.
 Reciprocal induction between the ureteral bud and the metanephric mesenchyme results
in branching morphogenesis and elongation of the ureteral bud to form the collecting
system and mesenchymal epithelial transformation of the metanephric mesenchyme to
generate primitive nephrons.
 The metanephros starts to function 6 to 10 weeks after fertilization, with nephrogenesis
complete by 36 weeks.
 Sixty percent of nephrons are formed in the last trimester, which has important clinical
implications for preterm and low-birth-weight infants, who have increased long-term risk
for chronic kidney disease (CKD).
 The extent to which reduced nephron number contributes to this increased risk relative to
exposure to nephrotoxic insults and acute kidney injury as a neonate is not yet
understood.
 The lower urinary tract is formed from the endodermal cloaca, which is divided by the
urorectal septum into ventral and dorsal parts that develop into the urogenital sinus and
rectum, respectively.
 The urogenital sinus gives rise to the early bladder, the urethra and vestibule of the
vagina in females, and the posterior urethra in males.
 Growth of the anterior abdominal wall between the allantois and the urogenital
membrane is accompanied by an increase in size and capacity of this bladder precursor.
 The allantois remains attached to the apex of the fetal bladder and extends into the
umbilical root, although it loses its patency and persists as the urachal remnant, the
median umbilical ligament, which connects the bladder to the umbilicus.
 By the seventh week, there is a separate opening of the distal mesonephric duct into the
bladder at what will become the vesicoureteral opening and the area known as the
trigone.
 At the same time the paramesonephric (mullerian) ducts start to regress in males and fuse
in females to become the uterovaginal cord, which opens into the urogenital sinus and
will go on to develop into the vagina.

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PATHOGENESIS
 Familial clustering, monogenic syndromes associated with urinary tract malformations,
and animal models suggest a strong genetic basis for CAKUT.
 Environmental and epigenetic factors are also thought to contribute to the pathogenesis of
CAKUT
 Pregestational maternal diabetes mellitus has been associated with an increased risk for
kidney and urinary tract anomalies, with hyperglycemia shown to adversely affect
nephron number.
 Transcription factors HNF1B (hepatocyte nuclear factor 1B) and PAX2 (paired box gene
2) are estimated to explain approximately 15% of CAKUT (both syndromic and isolated)
and are associated with cystic kidneys and renal hypodysplasia, respectively.
 HNF1B mediates the development of the kidneys, liver, pancreas, and urinary tract.
 Heterozygous variants can result in renal cysts and diabetes syndrome11 but have also
been identified in a wide range of isolated CAKUT phenotypes, including renal
hypodysplasia, cystic kidneys, single and horseshoe kidneys, and malformations of the
collecting system.

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  PAX2 is expressed in the metanephros, in cell lineages forming nephrons, and in those
destined to differentiate into the ureter, renal pelvis, and branching collecting duct
system.
 Heterozygous variants in PAX2 were first discovered in patients with renal-coloboma
syndrome presenting with renal hypodysplasia, optic nerve abnormalities, and hearing
loss and VUR and multicystic dysplastic kidney (MCDK)
 Teashirt 3 (Tshz3) fails to develop normal smooth muscle in the ureter and have
congenital hydronephrosis without anatomic obstruction.
 Autosomal recessive mutations in heparanase 2 (HPSE2) have been detected in patients
with urofacial syndrome, a congenital disease characterized by grimacing and incomplete
bladder emptying
 Administration of angiotensin-converting enzyme (ACE) inhibitors during pregnancy in
humans can cause hypotension and anuria in the baby with histologic features of renal
tubular dysplasia.
EPIDEMIOLOGY
 CAKUT accounts for 20% to 30% of all developmental anomalies identified in the
antenatal period and has a prevalence of 3 to 6 per 1000 births.
 CAKUT accounts for 40% to 50% of children with CKD
 20% to 50% of patients with a congenital solitary kidney require RRT by the age of 30.
RENAL MALFORMATIONS

Congenitally abnormal kidneys may be large or small, cystic or irregular in outline, and absent or
misplaced.
LARGE KIDNEYS
Enlarged kidneys resulting from congenital problems are usually hydronephrotic or cystic

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POLYCYSTIC KIDNEYS
 Commonest congenital anomalies as inherited autosomal disease.
 It is an complex syndrome,resulting from progressive dilatation of specific portion of the
nephron.
 Diagnosis : IVP , renal angiography
 For poly cystic kidney disease- analgesics, except ibuprofen (Advil), which is not
recommended since it may worsen kidney disease, anti hypertensives, antibiotics
to treat UTIs, a low-sodium diet, diuretics to help remove excess fluid from the body,
surgery to drain cysts and help relieve discomfort.


IRREGULAR KIDNEYS
 Irregularity of the renal outline may result from fetal lobulation or a “dromedary hump,”
neither of which has any functional implications.
 Much more important is the diagnosis of renal dysplasia

RENAL DYSPLASIA
 Abnormal differentiation of renal parenchyma with development of abnormal structures,
including primitive ducts surrounded by collars of connective tissue, metaplastic

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 cartilage, variety of nonspecific malformations such as preglomeruli of fetal type, and
reduced branching of collecting ducts with cystic dilations and primitive tubules.
 Dysplastic kidneys often contain cysts
RENAL HYPOPLASIA
• Significantly reduced renal mass with either normal or reduced (oligomeganephronia)
nephron number without evidence of maldevelopment of parenchyma.
RENAL HYPODYSPLASIA
Reduced renal mass and nephron number with dysplastic features. Previously thought to be
secondary to scarring from reflux or reflux nephropathy, but now increasingly considered to be
primary dysplasia with associated reflux.
RENAL MULTICYSTIC DYSPLASIA
 Severe cystic dysplasia with extremely enlarged kidney full of cystic structures; occurs as
an isolated renal lesion in response to ureteral atresia and urethral obstruction; 10% of
patients have a family history.
 The management is nephrectomy
RENAL AGENESIS
 Absence of the kidney or an identifiable metanephric structure.
 2 types Unilateral Renal Agenesis

Bilateral Renal Agenesis

Unilateral Renal Agenesis

 Complete absence of one kidney occurs in 1 in 500 to 1000 births.
 It can be familial and is referred to as hereditary renal aplasia by pediatricians.
 It is an autosomal dominant trait with incomplete penetrance and variable expression and
can be associated with bilateral renal agenesis or severe dysplasia.

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  Typically, there is no ureter, and the ipsilateral half of the bladder trigone is missing.
 The remaining kidney is usually hypertrophic, but it may be ectopic, malrotated, or
hydronephrotic with a megaureter.
 The more severe the dysplasia of the remaining kidney, the earlier is the presentation
 The ipsilateral testis and seminal tract are usually absent, and in 10% of cases, the adrenal
gland is also missing.
 Girls can have an absent fallopian tube or ovary or malformation of the vagina or uterus.
 Other associations include imperforate anus and malformations of the vertebrae and
cardiovascular system.
 Agenesis could result from failure in formation of the metanephros or the ureteral bud;
however, in association with cloacal abnormalities
 Normality of the single kidney should be confirmed by 99mTc-DMSA scintigraphy,
normal isotopic GFR, and absence of proteinuria.
 If theremaining kidney is abnormal or GFR is less than 30 ml/min, lifelong follow-up is
necessary.
 Ultrasound of the kidneys is recommended in all first-degree relatives of individuals with
unilateral or bilateral renal agenesis.
Bilateral renal agenesis
 Bilateral renal agenesis is lethal. It is associated with pulmonary hypoplasia and a
characteristic facial appearance (Potter facies) caused by intrauterine compression, which
is a consequence of oligohydramnios.
 The prevalence is about 1 in 10,000 births, with risk for occurrence in siblings of about
3%, unless there is a family history of agenesis, in which risk rises to 15% to 20%.
MISPLACED KIDNEYS
Renal Ectopia, Malrotation, and Crossed Fused Kidneys
 The starting position of the fetal kidney is deep in the pelvis.
 Kidneys that fail to ascend properly and therefore remain lower than usual occur in 1 in
800 births.
 During development and ascent of the kidney, the renal pelvis comes to face more
medially. The most common anomaly is for the pelvis to face forward.

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  The more ectopic the kidney, the more severe is the rotation and more abnormal the
appearance. In more than 90% of ectopia, there is fusion of both kidneys.
 This is best visualized on CT or MR urography
 Symptoms and complications, if any, are caused by associated reflux or pelviureteral
junction (PUJ) obstruction.

HORSE SHOE KIDNEY

 If both kidneys are low, they may join at the lower pole and are usually drained by two
ureters.
 The kidneys lie lower than normal, and further ascent is prevented by the root of the
inferior mesenteric artery.
 Horseshoe kidney occurs in 1 in 400 to 1800 births and is more common in males (2 : 1)
 Patients present, with complications of reflux, obstruction, or stone formation
 Symptomatic treatment only for Horse shoe kidney

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 CALYCEAL ABNORMALITIES
Hydrocalyx and Hydro calycosis
 Dilated calyces are usually caused by obstruction.
 Focal dilation also can be caused by congenital infundibular stenosis, extrinsic
compression from vessel or tumor, stones, or tuberculosis.
 Moreover, if the GFR is normal and the divided function of the kidneys is 50: 50, surgery
to improve the anatomy should not be attempted.

MEGACALYCOSIS
 In megacalycosis, there is bizarre dysplasia of the calyceal system with an increase in the
number of calyces.
 There is no obstruction, and the cause is malformation of renal papillae.
 Megacalycosis is congenital, usually unilateral, and an incidental finding. It is much more
common in males.
CALYCEAL DIVERTICULUM (CALYCEAL CYST)
• A calyceal diverticulum is a cavity peripheral to a minor calyx that is not a closed cyst
but rather is connected to the calyx by a narrow channel.
• It is usually an incidental finding and may manifest with symptoms relating to stones or
infection within the cavity.
• Treatment is Calyceal Diverticulectomy and percutaneous ablation

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BARDET-BIEDL SYNDROME
 Multiple calyceal clubbing and calyceal diverticula are the characteristic features of the
renal dysplasia seen in Bardet-Biedl syndrome (formerly known as Laurence-Moon-Biedl
syndrome).
 This autosomal recessive condition is characterized by retinitis pigmentosa, dysmorphic
extremities (sometimes with polydactyly), obesity, and hypogonadism.
 Malformation is associated with parenchymal dysplasia; renal failure in early adult life is
common.
 It has now been shown that Bardet-Biedl syndrome is caused by a defect of the basal
body of ciliated cells, 21 and mutations in 20 genes coding for different proteins located
in the basal body and cilia of the cell.
PELVIURETERAL JUNCTION OBSTRUCTION
 In children, PUJ obstruction is one of the most frequent causes of obstructive uropathy.
 The condition is usually congenital but can have an acquired mechanical basis caused by
stenosis or external compression from adhesions, aberrant lower pole vessels, or kinking
of the most proximal ureter.
 Associated abnormalities are common, and up to 50% of infants have another urologic
abnormality, such as contralateral PUJ obstruction, contralateral renal dysplastic and
multicystic kidney, minor degrees of VUR, and contralateral renal agenesis.
 Older children can present with an abdominal mass or with flank pain, hematuria
secondary to mild trauma, or UTI.
 Hypertension is unusual but can occur temporarily after surgical correction.

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  Diagnostic procedures need to differentiate between significant obstruction that requires
surgical correction and congenital ectasia of the renal pelvis, in which case surgery is not
indicated.
 Indications for surgical intervention include impairment of renal function, pyelonephritis,
renal stones, and pain. Kidneys with good function can generally be left alone, and
surgery is indicated only when function is clearly deteriorating

 A pyeloplasty is a surgical procedure that is indicated for a pelviureteric junction

(PUJ) obstruction

URETERAL ABNORMALITIES
DUPLEX URETERS
 Duplication of the ureter and the renal pelvis is a common anomaly, with an incidence of
about 1 in 150 births; unilateral duplication is six times more frequent than bilateral.
 It is more common in girls. If duplication has been detected in a patient, the likelihood of
another sibling with duplication rises to 1 in 8.

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  If the ureteral bud bifurcates after its origin from the mesonephric duct but arises at a
normal site, an incomplete ureteral duplication with a Y ureter will develop.
 Complete ureteral duplication occurs if there are two ureteral buds, one in the normal
location and the other in a low position.
 The normal bud ends in a correct site on the trigone in the bladder and is nonrefluxing.
 The lower bud, representing the ureter of the lower pole of the kidney, ends in the bladder
as a lateral orifice with a short submucosal tunnel.
 The lower pole ureter is therefore often associated with VUR, and scarring of the lower
pole can result.
 If there are two ureteral buds, one with a normal location and one with a high position,
the upper ureter is incorporated into the developing bladder, ending more distally and
medial to the normal one.
 Thus the upper pole ureter ends ectopically, and because of obstruction or dysplasia,
there is often severe scarring of the upper pole moiety.
 In most adult patients, ureteral reduplication is asymptomatic and causes no long-term
problems.
 Children with ureteral duplication often have VUR. The spontaneous disappearance of
reflux is less common in duplex ureters than in patients with a single ureter.
 Duplex ureters are best diagnosed by CT urogram. PUJ obstruction of the ureter draining
the lower pole of the kidney can occur.
 Associated conditions, such as ectopic ureters and ureterocele usually cause problems in
early life and therefore have been addressed by adolescence. Upper pole scarring is
associated with an ectopic ureter and lower pole scarring with VUR.
ECTOPIC URETERS
 Ectopic ureters are almost always associated with ureteral reduplication, and 10% are
bilateral.
 The ectopic ureter comes from the upper pole and inserts into the bladder more distally
and toward the bladder neck or opens into the upper urethra.
 Ectopic ureters are rare in males and manifest as UTI. Males are usually continent
because the ureter is proximal to the external sphincter.

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  Ectopic ureters are best visualized by CT or MR urography.
 A voiding cystourethrogram shows reflux into the lower pole of the kidney in 50% of
patients.

MEGAURETER
 Isolated dilation of the ureter does not necessarily imply obstruction.
 There are three broad groups of conditions with widely dilated ureters, as follows:
1. Obstruction of the ureter itself. This may be intrinsic (e.g., stone) or extrinsic
(e.g., retroperitoneal fibrosis); it is not associated with reflux.
2. Bladder outflow obstruction, with secondary ureteral obstruction. Examples
include a neuropathic bladder and posterior urethral valves; this may or may not
be associated with reflux.
3. A dilated but non obstructed ureter. This often occurs without reflux, and there
can be normal renal function; this may be caused by an adynamic segment of the
lower ureter.
 In the normal ureter, there is a characteristic helical orientation of muscle fibers.
 When the megaureter is secondary to bladder outlow obstruction, there is muscle
hyperplasia and hypertrophy of the ureteral wall.
 In megaureters with no apparent cause, a variety of abnormalities of muscle orientation
are described or there may be absence of muscle fibers at the proximal end of the
undilated segment.
 Electron microscopy shows an increase in collagen between the muscle bundles at the
level of the obstructing segment.

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  Obstruction appears to be caused by a failure of peristalsis through the distal ureteral
segment.
 Most cases of megaureter associated with obstruction present in childhood with severe
infections, often complicated by septicemia.
 These patients have a high incidence of other congenital abnormalities.
 In less severe cases or with no obstruction, patients can present with abdominal pain, loin
pain, hematuria, and UTI.
 Renal stones can form easily in the dilated systems. The exclusion of obstruction is often
established only by an antegrade pressure-low study (Whitaker test), in which a
nephrostomy is placed in the renal pelvis and contrast material infused at 10 ml/min.
 The typical surgery for megaureters involves putting the ureters back into the bladder
("ureteral reimplantation") and trimming the widened ureter ("ureteral tapering"). If your
child doesn't have a urinary tract infection or decrease in kidney function, the surgery can
be delayed until he/she is 12 months old.

BLADDER AND OUTFLOW DISORDERS

PRUNE-BELLY SYNDROME

 Prune-belly syndrome occurs in males and consists of absence of the muscles of the
anterior abdominal wall, bizarre malformations of the urinary tract with gross dilation of
the bladder and ureters, and bilateral undescended testes.
 When the disorder is diagnosed early, renal outcome is related to the degree of renal
dysplasia.
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  There are incomplete forms of prune-belly syndrome (pseudo-prune). Rarely, a similar
megacystis or megaureter may be seen in a male or female patient.

BLADDER EXSTROPHY( ECTOPIA VESICAE)

 It is a congenital malformation
 Lower portion of the abdominal wall and the anterior wall of the bladder are missing so
that bladder is everted through the opening and may found on the lower abdomen with
continuous passage of urine to the outside.
 Male are more commonly affected.
 Failure of growth of the lower abdominal wall between the allantois and the urogenital
membrane coupled with breakdown of the urogenital membrane leaves a small, open
bladder plate, a low-placed umbilical root, and diastasis of the pubic bones.
 The genital tubercle is probably placed lower in these patients, and the cloacal membrane
ruptures above it, leading to a penis with an open dorsal surface that is continuous with
the bladder plate.
 A midline closure defect causes a failure of fusion of the lower anterior abdominal wall,
including the symphysis pubis, lower urinary tract, and external genitalia.
 Reports of familial clustering and twin studies suggest an as yet undetermined genetic
basis for the disorder.
 In severe cases, the bladder mucosa lies exposed on the lower abdominal wall, with the
bladder neck and urethra laid open.
 The prostate and testes are normal. Most patients have normal kidneys at birth, although
many reports do not record the state of the kidneys at birth.

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  Surgical closure of the bladder within 48 hours and urinary conversion before
reconstructive surgery for Exstrophy of bladder.

NEUROPATHIC BLADDER

 In childhood, the most common cause of a neuropathic bladder is myelomeningocele,

 A neuropathic bladder also may be seen without associated neurologic or other obvious
causes.
 The principal consequences are incontinence, infection, and reflux with upper tract
dilation and subsequent renal failure.
 Early urodynamic assessment is essential. Three different patterns of bladder behavior are
seen: contractile, intermediate, and acontractile
 Contractile Behavior- An overactive detrusor (hyperreflexia) can produce some bladder
emptying (incontinence). Unfortunately, 95% of patients have sphincter dyssynergia
(inability to relax the urethral sphincter), which results in no relaxation and incomplete
emptying of the bladder.
 Intermediate Behavior- These patients have some detrusor activity, but not sufficient to
empty the bladder. These intermediate bladders are poorly compliant, and patients have
no voluntary control of their sphincters.
 The neural tube normally forms as the neural folds close over and fuse, starting in the
cervical region and progressing caudally. It is believed that the embryologic defect is an

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 incomplete tubularization of the neural tube, with inadequate mesodermal invagination
and subsequent arrest of vertebral arch formation.
 The incidence of myelodysplasia varies from 1 to 5 in 1000 live births, but there are wide
geographical variations.
 Myelomeningocele accounts for more than 90% of myelodysplastic infants. Folic acid
supplements taken during the first trimester reduce the incidence of myelodysplasia by
52%.
BLADDER NECK OBSTRUCTION
 Congenital bladder neck obstruction is rare and is usually caused by a neuropathic
bladder, posterior urethral valves, or an ectopic ureterocele.
Posterior Urethral Valves
 Posterior urethral valves are the most common cause of severe subvesical obstruction in
the male infant (but account for only 10% of neonatal hydronephrosis).
 As a result, bilateral hydronephrosis and megaureter occur.
 Obstruction is caused by a diaphragm that extends from the floor to the roof of the
urethra at the apex of the prostate.
 Valves appear as mucosal folds in the posterior urethra below the verumontanum.
 There is dilation of the proximal urethra and bladder wall hypertrophy and trabeculation.
Above the valves, the prostatic urethra dilates, undermining the bladder neck.
 The valves obstruct flow only in one direction, and therefore a catheter can be passed
without difficulty.
 The urethra develops in two parts: differentiation of the urogenital sinus part (posterior
urethra) and tubularization of the urethral plate (anterior urethra).
 Early obstruction during renal development can result in severe renal dysplasia.
 Most cases of posterior urethral valves are now detected antenatally on ultrasound as
evidenced by bilateral hydronephrosis, dilated bladder, posterior urethra (keyhole sign),
and, in severe cases, oligohydramnios.
 Infants present with a palpably distended bladder and enlarged kidneys, abnormal urine
stream, or failure to thrive as a result of renal failure.
 At diagnosis, 30% to 52% of children also have VUR. Children with less severe disease
present with poor stream, hematuria, incontinence, acute UTI, or renal failure.

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  However, late presentation is also associated with worse outcome.
URETHRAL DIVERTICULUM
 It is a rare condition in which a pocket, sac, or pouch forms in the urethra.
 The urethra is a small tube through which urine passes to exit your body. Because this sac
is in the urethra, it can fill with urine and sometimes pus.
 Multiple open surgical and endoscopic approaches have been described for the treatment
of urethral diverticula, including the following : Transurethral saucerization of the
diverticulum.
 Marsupialization of the diverticular sac into the vagina, excision of the diverticulum.

BLADDER OUTFLOW OBSTRUCTION

UROFACIAL SYNDROME
 Uro facial syndrome or Ochoa syndrome is an autosomal recessive congenital disorder
characterized by an association of a lower urinary tract and bowel dysfunction with a
typical facial expression: When attempting to smile, the patient seems to be crying or
grimacing

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  Patients present with enuresis and UTI and all the features of a neuropathic bladder
together with dilated upper tracts.
 They are at risk for renal failure. Mutations of heparanase-2 (HPSE2) and leucine-rich
repeats and immunoglobulin-like domains 2 (LRIG2) have been reported in urofacial
syndrome, and the proteins they encode are expressed in the fetal bladder.
 It is proposed that they play an important role in the neural control of bladder function.
 Urofacial syndrome is part of a spectrum of congenital bladder disorders that include
non-neurogenic neurogenic bladder or Hinman syndrome.
DIAGNOSTIC EVALUATION
 History collection
 Physical examination
 USG- Can differentiate between it and hydronephrosis.
 MCUG(micturating cystoerethrograme) : contra-lateral VUR (20%).
 IVP
 Renal angiography
 Cystoscopic examination
 X-ray
 Urodynamic studies
 Renal function test
COMPLICATIONS
 Urinary tract infection
 Glomerular hyperfiltration
 Proteinuria and progressive renal failure
 Hypertension
 Calculi
 Tubular dysfunction
 Polyuria
 Salt depletion
 Acidosis
 Bone disease- osteomalacia

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NURSING MANAGEMENT
 Thorough history collection and physical examination
 Monitor vital signs, renal function, intake and output chart
 Personal hygiene
 Catheter care
 Wound dressing
 Dialysis when the solitary kidney has ceased to function
CONCLUSION
Congenital anomalies of kidney and urinary tract (CAKUT) is a group of abnormalities affecting
the kidneys or other structures of the urinary tract. The additional parts of the urinary tract that
may be affected include the bladder, the tubes that carry urine from each kidney to the bladder
(the ureters), and the tube that carries urine from the bladder out of the body (the urethra).
CAKUT results from abnormal development of the urinary system and is present from birth
(congenital), although the abnormality may not become apparent until later in life.

Individuals with CAKUT have one or more kidney or urinary tract abnormalities. For
paired structures, like the kidneys and ureters, one or both may be affected. Many different
developmental abnormalities are classified as CAKUT, including underdevelopment or absence
of a kidney (renal hypodysplasia or agenesis), a kidney formed of fluid-filled sacs called cysts
(multicystic dysplastic kidney), buildup of urine in the kidneys (hydronephrosis), an extra ureter
leading to the kidney (duplex kidney or duplicated collecting system), a blockage in a ureter
where it joins the kidney (ureteropelvic junction obstruction), an abnormally wide ureter
(megaureter), backflow of urine from the bladder into the ureter (vesicoureteral reflux), and an
abnormal membrane in the urethra that blocks the flow of urine out of the bladder (posterior
urethral valve).

CAKUT varies in severity. The abnormalities can result in recurrent urinary tract
infections or a buildup of urine in the urinary tract, which may damage the kidneys or other
structures. Severe CAKUT can result in life-threatening kidney failure and end-stage renal
disease.

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BIBLIOGRAPHY
1. Richard J. Johnson & John Feehally & Jurgen Floege & Marcello Tonelli.
Comprehensive Clinical Nephrology, .6th Edition.
2. Lewis MA. Demography of renal disease in childhood. Semin Fetal Neonatal Med. 2008
3.

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