This document discusses congenital genitourinary disorders that commonly affect the kidneys and urinary tract in children. It outlines various abnormalities that can occur in the kidneys, ureters, bladder and urethra. Some of the disorders discussed include renal agenesis, horseshoe kidney, posterior urethral valves, hypospadias, and Wilm's tumor. The document provides details on the symptoms, diagnosis and management of these various congenital genitourinary disorders that can present in pediatric patients.

1. CONGENITAL GENITO URINARYDISORDERS

2. introduction
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

4. Congenital abnormalities of the kidney and urinary tract
⚫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.

5. en
Kidney and Ureter Bladder and Urethra
•Renal agenesis
•Renal hypoplasia
•Horse-shoe kidney
•Poly cystic renal disease
•Ectopic kidney
•Duplication of renal
pelvis and ureter
•Hydronephrosis
•Pelvi-ureteric
obstruction
•Congenital renal
neoplasm
•ureterocele
•Ectopia vesicaehus
•Patent urachus
•Bladder and neck
obstruction
•Posterior urethral valves
•Neurogenic bladder
•Hypospadias
•Epispadias
•Phimosis
•Urethral stenosis
•Meatal stenosis

6. Renal agenesis
Definition :It is the absence of kidney due to failure of
ureteric bud formation.
Type: Bilateral
Unilateral

7. Renal hypoplasia and dysplasia
⚫it occurs due to reduction of renal mass affecting the
nephron.
⚫It may be unilateral or bilateral
⚫It may be segmental, simple
RENAL DYSPLASIA :
⚫Disorganization of lung parenchyma with immature
nephron and ductal elements resulting in large or
small kidney.
⚫It may be multicystic , hypo plastic or aplastic

8. Horse – shoe kidney
⚫It develops when lower poles of the kidneys are fused
in the midline due to fusion of ureteric buds during
fetal development.
⚫Child presents with pyuria, albuminuria, vomiting
⚫Surgery is indicated

9. Polycystic kidneys
⚫Commonest congenital anomalies as inherited
autosomal disease.
⚫It is an complex syndrome
⚫Resulting from progressive dilatation of specific
portion of the nephron.
Types : infantile
adult
Diagnosis : IVP , renal angiography

10. Obstructive lesions of the urinary tract
⚫It mainly caused by congenital abnormalities like
pelvi-ureteric junction obstruction and posterior
urethral valves which may lead to irreversible renal
damage.

11. Pelvi– uretericjunctionstenosis
⚫Unilateral or bilateral
⚫Urinary tract infections and upper abdominal pain
Diagnosis : ultrasound, IVP , renal function test
Management:
⚫Surgery is indicated for removal of obstruction

12. hydronephrosis
⚫ 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 ,MCU
micturating cysto-
urethrogram (
Management :
⚫ Surgical removal or pyeloplasty,percutaneous
nephrostomy

14. Posteriorurethralvalve
⚫Distant urinary tract obstruction
⚫Dribbling of urine, abnormal urine stream, palpable
bladder, recurrent urinary tract infections, vomiting
and failure to thrive
Diagnosis : MCU, USG and endoscopy
Management : urinary catheterization, baloon catheter
or endoscopic fulgration, cutaneous vesicostomy,
pyelostomy

15. Meatal stenosis
⚫ Congenital abnormality
⚫ Due to meatal ulcer and scaring
⚫Meatal dilatation or meatoplasty

16. Exstrophyof bladder(ectopiavesicae)
⚫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

17. 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

18. 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

19. epispadias
⚫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

21. Management : 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 Vesicoureteral reflux
⚫ Cystoplasty
⚫ Supportive nursing care

22. hypospadias
⚫ 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.

23. 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

24. phimosis
⚫Narrow opening of the prepuce that prevents it being
drawn back over the glans penis.
Management:
⚫Circumcision
⚫ apply Betamethasone cream

25. paraphimosis
⚫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

26. Wilm’stumor(nephroblastoma)
⚫ 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

27. 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

28. Diagnostic evaluation :
⚫History
⚫Physical examination
⚫X-ray abdomen ,chest
⚫USG
⚫CT scan
⚫MRI
⚫Renal function test
Management :
⚫Nephrectomy
⚫Chemotherapy
⚫radiotherapy

29. Undescended testis
⚫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

30. Diagnosis :
⚫USG
⚫Laproscopy
⚫MRI
Complications :
⚫Trauma
⚫Malignant
⚫Psychological trauma
Management :
⚫Orcheopexy
⚫Hormonal treatment

31. Vesico– urethral reflux
⚫ 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

32. Management :
⚫Correction of structural anomalies
⚫Administration of low dose antibiotics
Detectingkidney and urinary tract abnormalities beforebirth
⚫Ultrasound examination
⚫Antenatal screening
⚫Screening of high risk groups

33. Prevention of congenital genitourinary anomalies
⚫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

34. Nursing management of thechild withurologic surgery
⚫Promoting understanding of parents
⚫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