Congenital genito urinary disorders

CONGENITAL GENITO URINARY DISORDERS

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

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.

enKidney 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

Renal agenesis

Definition :It is the absence of kidney due to failure of ureteric bud formation.

Type: Bilateral Unilateral

Renal hypoplasia and dysplasia

it occurs due to reduction of renal mass affecting the nephron.

It may be unilateral or bilateralIt may be segmental, simpleRENAL 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

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

Polycystic kidneys

Commonest congenital anomalies as inherited autosomal disease.

It is an complex syndromeResulting from progressive dilatation of

specific portion of the nephron.Types : infantile adult Diagnosis : IVP , renal angiography

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.

Pelvi – ureteric junction stenosis

Unilateral or bilateralUrinary tract infections and upper abdominal

painDiagnosis : ultrasound, IVP , renal function test

Management:Surgery is indicated for removal of

obstruction

hydronephrosis

It is the dilatation of renal pelvisUnilateral or bilateralDue to obstruction of urine flow in the distal

urinary tractMales > femalesAbdominal pain, failure to thrive , anemia,

hypertension, hematuria, renal failure.Diagnosis : USG, IVP ,MCUManagement :Surgical removal or pyeloplasty,percutaneous

nephrostomy

Posterior urethral valve

Distant urinary tract obstructionDribbling of urine, abnormal urine stream,

palpable bladder, recurrent urinary tract infections, vomiting and failure to thrive

Diagnosis : MCU, USG and endoscopyManagement : urinary catheterization, baloon

catheter or endoscopic fulgration, cutaneous vesicostomy, pyelostomy

Meatal stenosis

Congenital abnormality Due to meatal ulcer and scaringMeatal dilatation or meatoplasty

Exstrophy of bladder( ectopia vesicae)

It is a congenital malformationLower 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

Clinical manifestations: Urinary dribblingSkin excoriationInfection and ulceration or the bladder mucosaAmbiguous genitaliaUTIGrowth failure

Diagnosis :Cystoscopic examinationX-rayUSGIVP

Management :Surgical closure of the bladder within 48

hoursUrinary conversion before reconstructive

surgeryOrthopedic surgerySupportive nursing carePre operative carePost operative careFollow up

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 femalesClassification : anterior epispadias posterior epispadias female epispadias – bifid clitoris subsymphyseal with

incontinence of urine

Management : surgical correction1. 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

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 shaftMiddle (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 penis2. Deflected stream of urine3. Inability void urine while standingManagement : surgical reconstruction Meatotomy Chordee correction urethroplasty

phimosis

Narrow opening of the prepuce that prevents it being drawn back over the glans penis.

Management:Circumcision apply Betamethasone cream

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 : edematousSevere pain

Wilm’s tumor (nephroblastoma)

Max wilm’s , German surgeon described this most common renal tumor of childhood.

Chromosomal deletions 11 and 16Highly malignant embryonal tumorTumor develops in kidney parenchyma ,invading the

surrounding tissues.Clinical features :abdominal massMicroscopic hematuriaFeverPallorSuperficial 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 :HistoryPhysical examinationX-ray abdomen ,chestUSGCT scanMRIRenal function testManagement :NephrectomyChemotherapyradiotherapy

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 :RetractilePalpableimpalpable

Diagnosis :USGLaproscopyMRIComplications :TraumaMalignantPsychological traumaManagement :OrcheopexyHormonal treatment

Vesico – urethral reflux

Retrograde flow of bladder urine up the ureter during voiding.

Causes:Insertion of ureter in to the bladderInfectionEdemaClinical features:DysuriaUrinary frequency and urgencyUrine retentionCloudy or blood tinged urine

Management :Correction of structural anomaliesAdministration of low dose antibiotics

Detecting kidney and urinary tract abnormalities before birth

Ultrasound examinationAntenatal screeningScreening of high risk groups

Prevention of congenital genito urinary anomalies

Advice to future parentsTo minimize exposure of pregnant women to

risk factorsTo prevent over weight/ obesityTo promote effective information on dietTo improve folate statusTo avoid contraindicated vaccinationTo include school education programmes

Nursing management of the child with urologic surgery

Promoting understanding of parentsPreparing for diagnostic proceduresInvolving the parents in child careMonitoring intake and outputPreventing infectionsProviding comfortProviding adequate nutritionTeaching the parents about related care

Thank you…