MnlkAXI QH

MANISHA
M.SC. NURSING
 Congenital anomalies of the kidney and urinary tract (CAKUTs) occur in 3–6
per 1000 live births and are responsible for 34–59% of chronic kidney
disease (CKD) and for 31% of all cases of end-stage kidney disease (ESKD).
 All children with ESKD require renal replacement therapy and up to 70% of
them develop hypertension.
 survival rate of children with ESKD is about 30 times lower than that of
healthy children.
 New strategies are needed to prevent CAKUT, preserve renal function, and
reduce associated cardiovascular morbidity.
(Ihor V. Yosypiv, 2012)
 With improved prenatal screening, many cases of CAKUT are diagnosed by
antenatal ultrasonography performed on 18–20 weeks of gestation.
 Most common antenatal manifestations of CAKUT include oligohydramnios
or variations in gross morphology of the kidney, ureter, or bladder.
 Postnatal manifestations of CAKUT may include presence of palpable
abdominal mass or single umbilical artery, feeding difficulties, decreased
urine output, deficient abdominal wall musculature, and undescended testes
in a male infant or multiorgan birth defects.

(Ihor V. Yosypiv, 2012)

Disorders of kidney and urinary tract are commonly seen in pediatric units as
medical and surgical problems.

Incidence: 3 to 6 per 1000 live births

 These problems usually required surgical correction.

 Some of them are producing no clinical symptoms.

 25% cases of CRF are due to congenital anomalies

 8 to 10 % of children affected with congenital anomalies of urinary tract.

 Kidney and Ureter Bladder and Urethra
•Renal agenesis •Ectopia vesicaehus
•Renal hypoplasia •Patent urachus
•Horse-shoe kidney •Bladder and neck obstruction
•Poly cystic renal disease •Posterior urethral valves
•Ectopic kidney •Neurogenic bladder
•Duplication of renal pelvis and •Hypospadias
ureter •Epispadias
•Hydronephrosis •Phimosis
•Pelvi-ureteric obstruction •Urethral stenosis
•Congenital renal neoplasm •Meatal stenosis
•ureterocele
Anomalies of number
-Agenesis Unilateral
Bilateral Anomalies of ascent
-Supernumerary kidney
Anomalies of volume and structure Simple ectopia
Hypoplasia Cephalad ectopia
Multicystic kidney Thoracic kidney
Polycystic kidney
Other cystic disease & Medullary cystic disease
 Anomalies of rotation

Crossed ectopia with and without Incomplete

fusion Excessive
 Unilateral fused kidney (inferior
ectopia) Reverse
 Sigmoid or S-shaped kidney
 Lump kidney
 L-shaped kidney
 Disc kidney
 Unilateral fused kidney (superior
ectopia)
 Horseshoe kidney
It comprises a diversity of abnormalities, ranging from:
complete absence kidney
supernumerary Kidney
aberrant location, orientation, and shape of the kidney
aberrations of the collecting system
aberrations of blood supply
 Definition: It is the absence of kidney due to failure of ureteric bud
formation.
Incidence : 1: 1400 births

Type: Bilateral
Unilateral
Embryology :Complete absence of a ureteric bud or aborted ureteral
development prevents maturation of the metanephric blastema into adult
kidney tissue.
Asymptomatic

Unilateral renal agenesis is much more common than bilateral one.

Malformations associated with unilateral renal agenesis:
1. Single umbilical artery
2. Absent ureter
3. Absent ipsilateral vas deferens
4. Contralateral vesico-ureteral reflex.
Diagnosis : U/S or IVU,CT scan: absent kidney on that side +
compensatory hypertrophy of the contralateral kidney
Treatment: no specific treatment
Prognosis: no evidence that they have an increased susceptibility to other
diseases
Bilateral agenesis: rare, incompatible with life
 Supernumerary kidney is an additional
kidney to the number which is a rare
congenital anomaly; fewer than 100 cases
have been reported over the years.
 It occurs due to reduction of renal mass affecting the nephron.
 Small size, non-dysplastic, calyces and nephrons are less than normal.
 It may be unilateral or bilateral
 It may be segmental, simple.
 Types:-
 Unilateral: Incident diagnosis ( another urinary tract problem or HTN).

 Bilateral: CRF manifestations. It’s compatible with life.

 Segmental Hypoplasia.
 Multicystic dysplastic kidney (MCDK) is the most common cystic disease in
infants.
 It is also the most common cause of abdominal mass in neonates; although
the mass is non-palpable at birth in majority of cases.
 MCDK is a congenital condition, in which the kidney is replaced by cysts and
the entire kidney is dysplastic; so it does not function.
 It is usually unilateral (also called unilateral renal dysplasia).
 When it is bilateral, it is incompatible with life.
 Risk of Wilm’s tumor arising from an MCDK is approx. 1/333.
 It may be multicystic, hypo plastic or aplastic
 When it is bilateral, it usually presents with signs and symptoms of chronic
renal failure.
Investigations & diagnosis:
1 US. Can differentiate between it and hydronephrosis.
2 DMSA (no function in the affected side) , hypertrophy
of contra-lateral kidney.
3 MCUG : contra-lateral VUR (20%).

Complications:

1- Malignancy: Wilm's’ tumor, adenocarcinoma& embryonic

carcinoma.
2- HTN: cured by nephrectomy.
3- Infection, bleeding into, or rupture of cysts if large and
Retention.
Conservative:
 cysts < 5cm , high chance of involution, which means the kidney is
shrinking, or cause no problems.
 If > 5cm, it most likely will not involute so follow up the patients for any
complication, and if they develop complications do a nephrectomy.
 reviewed annually for: BP, urinary protein, growth of contra-lateral kidney.
Up to 2yrs of age then at 5yrs of age if normal.
 US for cysts involution, of MCDK.

Nephrectomy
 Commonest congenital anomalies as inherited autosomal disease.
 It is an complex syndrome
 Resulting from progressive dilatation of specific portion of the nephron.

Two types:
 AUTOSOMAL RECESSIVE ("INFANTILE")
 AUTOSOMAL DOMINANT ("ADULT")
Diagnosis : IVP , renal angiography
PATHOPHYSIOLOGY
Control hypertension
 Non-opioid analgesics to control pain
 Surgical/ percutaneous drainage of cysts to control pain/ infection
Nephrectomy in case of intractable symptoms/ symptoms due to mass effect of
enlarged kidneys
 Dialysis: Hemodialysis/ peritoneal dialysis
 Renal transplantation in ESRDpatients
Abnormalities in shape &
position

Ectopic Kidney:
Failure of ascent of the kidney during
embryogenesis.
Incidence 1;900. most commonly in
pelvis. It can also be thoracic (very rare),
iliac or crossed.
 It develops when lower poles of the kidneys are
fused in the midline due to fusion of ureteric buds
during fetal development.
 Commonest form of fusion (95%).
 The lower poles of both kidneys unite across the
midline
 The isthmus of horse shoe kidney lie at the level of
L4-L5, is more susceptible to trauma (avoid severe
aggressive sports)
 More common in male, Turner syndrome (always
look for horseshoe kidney), trisomy 18

 Child presents with pyuria, albuminuria, vomiting

 Surgery is indicated
Complications:
-50% VUR, abnormal vascular supply.
- Stone (ureter is no longer positioned
anterolaterally, and kidney causes pressure on
it, leading to hydronephrosis 2° PUJO.
- Wilm’s tumor
- HTN due to vascular abnormality.
Diagnosis:
DMSA, MCUG ,DTPA (to assess the function of
the ureter) can be missed by U/S.
 Unilateral or bilateral
 Urinary tract infections and upper abdominal pain

 Detected antenatal, most frequent cause of hydronephrosis

 More common in Left side, Ectopic, malrotation, horseshoe

kidney
 Bilateral 40%

 Present: mass, UTI, Pain, Hematuria

Diagnosis : ultrasound, IVP, renal function test

Management:
 Surgery is indicated for removal of obstruction
 It is the dilatation of renal pelvis
 Unilateral or bilateral

 Due to obstruction of urine flow in the distal urinary tract

 Males > females

 Abdominal pain, failure to thrive , anemia, hypertension, hematuria,

renal failure.
Diagnosis : USG, IVP Management :
 Surgical removal or pyeloplasty, percutaneous nephrostomy
• A ureterocele is a cystic out-
pouching of the distal ureter
into the urinary bladder.

• Ureterocele occurs due to a

combination of an abnormality
in the submucosal part ofureter
and stenosis of ureteric orifice.
According to renal collecting system:

1. Single-system ureteroceles: Those

associated with a single kidney,
collecting system and ureter.

2. Duplex-system ureteroceles: Those

associated with kidneys that have
completely duplicated ureters.
 Distant urinary tract obstruction
 Dribbling of urine, abnormal urine stream, palpable bladder, recurrent urinary tract
infections, vomiting and failure to thrive
 Congenital valve in the posterior urethra due to persistent urogenital membrane.
 Associated with renal dysplasia ( Back pressure,
 common developmental insult).

 Risk of perinatal mortality & risk of chronic kidney disease:

1- US suggestive at < 24 wks gestation.
2- Severe bilateral hydronephrosis.
3- Oligohydramnios.
4- Echogenic kidneys.

Diagnosis : MCU, USG and endoscopy

Management : urinary catheterization, baloon catheter or endoscopic fulgration,
cutaneous vesicostomy, pyelostomy
 Congenital abnormality
 Due to meatal ulcer and scaring
 Meatal dilatation or meatoplasty
 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

 Multiple abnormalities in pelvis, bladder, urethra, & external genitalia.

 Associated abnormalities:

1- VUR
2- Incontinence
3- Repeated UTI
Clinical manifestations:
 Urinary dribbling
 Skin excoriation
 Infection and ulceration or the bladder mucosa
 Ambiguous genitalia
 UTI
 Growth failure

Diagnosis :
 Cystoscopic examination
 X-ray
 USG
 IVP
Management :
 Surgical closure of the bladder within 48 hours
 Urinary conversion before reconstructive surgery
 Orthopedic surgery
 Supportive nursing care
 Pre operative care
 Post operative care
 Follow up
 Abnormal urethral opening on the dorsal aspect of penis.
 Due to abnormal development of the infraumbilical wall and upper wall of
urethra.
 Rare in females

Classification : anterior epispadias

posterior epispadias
female epispadias – bifid clitoris
- subsymphyseal with incontinence of urine
 Surgical correction
1. 1.5 to 2 years of age for penile lengthening, elongation of urethral strip and
chordee correction.
2. operation done at least 6 months after first stage for urethral
reconstruction
3. 3 to 4 years of age for bladder neck reconstruction and correction of VUR
 Cystoplasty
 Supportive nursing care
 It is the congenital abnormal urethral opening on the ventral aspect
(under surface) of the penis.
 Common in male children.

Classification :
 Anterior hypospadias (65 to 70%) : it may found as glandular or coronal
or on distal penile shaft
 Middle (10-15%) penile shaft hypospadias.

 Posterior hypospadias (20%) : it may be found on proximal penile shaft

or as penoscrotal, scortal or perineal type.
 Problems related to hypospadias :
1. Painful downward curvature of penis
2. Deflected stream of urine
3. Inability void urine while standing
 Management :
 surgical reconstruction
 Meatotomy
 Chordee correction
 urethroplasty
A condition in which foreskin can not be retracted from around the tip of
penis.
Management:
 Circumcision

 apply Betamethasone cream

 It is the retraction of a phimotic foreskin, behind coronal sulcus.

 It may develop phimotic child which also need for surgical management
by circumcision or reduction with application of lubricant under deep
sedation.
Clinical features :
 edematous

 Severe pain
 Max wilm’s , German surgeon described this most common renal tumor
of childhood.
 Chromosomal deletions 11 and 16
 Highly malignant embryonal tumor
 Tumor develops in kidney parenchyma ,invading the surrounding
tissues.
Clinical features :
 abdominal mass
 Microscopic hematuria
 Fever
 Pallor
 Superficial vein engorgement
Clinical staging :

Stage 1 - limited to kidney and can be fully

excised
Stage 2 - Regional extension of tumor by
penetration through renal capsule.
Stage 3 – non hematogenous extension of
the tumor confined to the abdomen
following surgery.
Stage 4 – hematogenous metastasis to
distant organs
Stage 5 – bilateral renal involvement
Diagnostic evaluation :
 History

 Physical examination

 X-ray abdomen ,chest

 USG

 CT scan

 MRI

 Renal function test

Management :
 Nephrectomy

 Chemotherapy

 radiotherapy
 Testis cannot be made to reach the bottom of the scrotum
 It is also known as Cryptochordism

 5% of full term male infants may have unilateral or bilateral testis.

Types :
 Retractile

 Palpable

 impalpable
Diagnosis :
 USG
 Laproscopy
 MRI
Complications :
 Trauma
 Malignant
 Psychological trauma
Management :
 Orcheopexy
 Hormonal treatment
Prune belly syndrome ( Eagle-Barrett syndrome,
Triad syndrome)
Triad of:
1- Deficiency or absence of anterior abdominal wall musculature.
2- Bilateral cryptorchidism
3- bilateral Ureter ,bladder,& urethral abnormalities( megacystis, Megaureter 2°
dysplasia.

-Other systemic abnormalities in 75%:

1- GI : malrotation, gastroschisis.
2- Heart: CHD
3- Skeletal: talipes equinovarus, CDH.
4- Pulmonary hypoplasia
 Retrograde flow of bladder urine up the ureter during voiding.
Causes:
 Insertion of ureter in to the bladder

 Infection

 Edema

Clinical features:
 Dysuria

 Urinary frequency and urgency

 Urine retention

 Cloudy or blood tinged urine

Management :
 Correction of structural anomalies
 Administration of low dose antibiotics

Detecting kidney and urinary tract abnormalities

before birth

 Ultrasound examination
 Antenatal screening
 Screening of high risk groups
 Advice to future parents
 To minimize exposure of pregnant women to risk factors
 To prevent over weight/ obesity
 To promote effective information on diet
 To improve folate status
 To avoid contraindicated vaccination
 To include school education programmes
 Risk for Ineffective Protection
 Risk for Impaired Skin Integrity
 Deficient Knowledge
 Excess Fluid Volume
 Acute Pain
 Impaired Renal Tissue Perfusion
 Impaired Urinary Elimination
 Imbalanced Nutrition: Less than Body Requirements
 Risk for Decreased Cardiac Output
 History
 Diagnostic assessment
 Promoting understanding of parents/psychological support
 Preparing for diagnostic procedures
 Involving the parents in child care
 Monitoring intake and output
 Preventing infections
 Providing comfort
 Providing adequate nutrition
 Teaching the parents about related care