Congenital genito urinary disorders

1. CONGENITAL GENITO URINARY DISORDERS

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

3. 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.

4. 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

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

6. 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

7. 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

8. 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

9. 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.

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

11. 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 Management : Surgical removal or pyeloplasty,percutaneous nephrostomy

12. Posterior urethral valve 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

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

14. Exstrophy of bladder( 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

15. 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

16. 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

17. 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

18. Management : surgical correction 1.5 to 2 years of age for penile lengthening, elongation of urethral strip and chordee correction. operation done at least 6 months after first stage for urethral reconstruction 3 to 4 years of age for bladder neck reconstruction and correction of VUR Cystoplasty Supportive nursing care

19. 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.

20. Problems related to hypospadias : Painful downward curvature of penis Deflected stream of urine Inability void urine while standing Management : surgical reconstruction Meatotomy Chordee correction urethroplasty

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

22. 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

23. Wilm’s tumor (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

24. 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

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

26. 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

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

28. 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

29. 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

30. Prevention of congenital genito urinary 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

31. Nursing management of the child with urologic 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

32. Thank you…