Understanding Congenital Renal Disorders in Newborns

1. CONGENITAL RENAL DISORDERS Mrs.Sharin Neetal D'souza

2. CONGENITAL RENAL DISORDERS Reviewed by: Prof.Umarani J Dr.PriyaReshmaAranha Prepared by: Mrs.SharinNeetalD’souza Lecturer Dept.Child health nursing Yenepoya nursing college Mrs.Sharin Neetal D'souza

3. Learning objectives At the end of the class students will be able to • list down the congenital anomalies and its features • explain the management of congenital anomalies Mrs.Sharin Neetal D'souza

4. RENAL AGENESIS: • Clinically the absence of palpable kidneys ( in the newborn) should arouse a strong index of suspicion of renal anomaly. • Renal agenesis arises from failure of ureteric bud formation or mesenchymal blastema differentiation or final mesenchymal condensation • It may be unilateral or bilateral. Mrs.Sharin Neetal D'souza

5. RENAL AGENESIS Mrs.Sharin Neetal D'souza

6. POTTER’S SYNDROME (BILATERAL RENAL AGENESIS) • It is an uncommon anomaly • Clinical characteristics: Peculiar facial features(low-set malformed ears, hypertelorism, beaked nose, micrognathia) , pulmonary hypoplasia and positional abnormality of the lower limbs. Mrs.Sharin Neetal D'souza

7. RENAL HYPOPLASIA • May be unilateral or bilateral and may be of simple, segmental or oligo-meganephric type. • It is characterized by reduction in the renal mass affecting nephron population mainly . Mrs.Sharin Neetal D'souza

8. RENAL DYSPLASIA: It is characterized by the complete disorganization of the renal parenchyma with immature nephronic and ductal elements resulting in a large or small kidney. Mrs.Sharin Neetal D'souza

9. MULTICYSTIC KIDNEY: Mrs.Sharin Neetal D'souza

10. MULTICYSTIC KIDNEY: • May be May be unilateral or bilateral presenting usually as a unilateral cystic abdominal mass in a newborn male. • Confirmation: by failure of excretion of dye in an IVP and absence of ureteric orifice visualization in cystoscopy • Treatment • Surgery. Mrs.Sharin Neetal D'souza

11. Supernumerary kidney • Thrid kidney is very rare and not to be confused with the relatively common . Unilateral. Mrs.Sharin Neetal D'souza

12. Supernumerary kidney Mrs.Sharin Neetal D'souza

13. Horse shoe kidney: • It results from the fusion of the ureteric buds. • Usually situated at a lower level. • The connecting bridge of tissue may or may not contain nephrons and may lead to kidney of the ureter. Mrs.Sharin Neetal D'souza

14. Clinical confirmation: • Midline firm mass with associated pyuria, albuminuria and vomiting. • IVP demonstrating a single mass with malpositioned pelvis and a bridge of tissue. Treatment: • There is no specific treatment. • Surgery. Mrs.Sharin Neetal D'souza

15. Polycystic kidney: • Polycystic kidney disease (PKD) is a genetic disorder characterized by the growth of numerous cysts in the kidneys.  Mrs.Sharin Neetal D'souza

16. Symptoms • Abdominal pain or tenderness • Blood in the urine • Excessive urination at night • Flank pain on one or both sides • Other symptoms that may occur with this disease include: • Drowsiness • Joint pain • Nail abnormalities Mrs.Sharin Neetal D'souza

17. Treatment • The goal of treatment is to control symptoms and prevent complications. High blood pressure may be hard to control, but controlling it is the most important part of treatment. • Treatment may include: • Blood pressure medicines • Diuretics • Low-salt diet Mrs.Sharin Neetal D'souza

18. Any urinary tract infection should be treated quickly with antibiotics. • Surgery to remove one or both kidneys may be needed. Treatments for end-stage kidney disease may include dialysis or a kidney transplant. Mrs.Sharin Neetal D'souza

19. Polycystic kidney Mrs.Sharin Neetal D'souza

20. Ectopic kidney: • It results from defective ascent during development leading to the kidney being palpable in the pelvis (or) lower abdomen with its malpositioned pelvis and ureter • May be unilateral or crossed. Treatment • No treatment is necessary. Mrs.Sharin Neetal D'souza

21. Ectopic kidney Mrs.Sharin Neetal D'souza

22. Disorders of renal pelvis and ureters: • Duplication of renal pelvis and ureter: • Double and completely separate pelves may occur, draining through separate ureters to separate ureteric orifice in the bladder • There may be two pelves and one ureter or the two ureters may unite in the shape of ‘Y’ during the descent to the bladder. Mrs.Sharin Neetal D'souza

23. Double ureter: Mrs.Sharin Neetal D'souza

24. Mega ureter: • Megaureter, or a wide and dilated ureter may occur without any distal ureteral obstruction or vesicoureteralrefllux. • Four times more common in boys than girls Mrs.Sharin Neetal D'souza

25. Signs and symptoms • Hematuria • UTI • Abndominal pain Mrs.Sharin Neetal D'souza

26. Ureterocele: • Definition: • A ureterocele is a cystic out pouching of the distal ureter into the urinary bladder Mrs.Sharin Neetal D'souza

27. Etiology: • Obstruction of the ureteral orifice • Incomplete muscular development of the intramural ureter • Excessive dilatation of the intramural ureter during the development of bladder. Mrs.Sharin Neetal D'souza

28. Signs and symptoms: • Urinary tract infection • Urosepsis • Obstructive voiding symptoms • Urinary retention • Hematuria • Cyclic abdominal pain • Ureteral calculus. Mrs.Sharin Neetal D'souza

29. Treatment • Endoscopic puncture • Incision (or) transureteral / on roofing of the ureterocele • Upper pole heminephrectomy • Excision of ureterocele • nephroureterectomy Mrs.Sharin Neetal D'souza

30. Ectopic ureteral orifice: • Ectopic ureter (or ureteralectopia) is a medical condition where the ureter, rather than terminating at the urinary bladder, terminates at a different site. • In males this site is usually the urethra, in females this is usually the urethra or vagina • It is usually occurs with ureterocele and duplication of the ureter but single ectopic ureters are seen. • Boys may present with epididymitis as the ureter drains directly into the vas deferens or seminal vesicle • Girls normally present with incontinence with continual dripping despite normal voiding. Mrs.Sharin Neetal D'souza

31. Clinical manifestations • Urinary tract infection • Infants exhibit failure to thrive • In females, the ureteral orifice is located in vagina. Septum or urethra. • Urine drips from the meatus Mrs.Sharin Neetal D'souza

32. Diagnostic evaluation • Renal function and electrolye levels must be monitored • Ultrasonography • Intravenous urography • Voiding cystourethrography • Axial imaging Mrs.Sharin Neetal D'souza

33. Treatment: • Antibiotic therapy • Endoscopic decompression • Open reconsrtruction at bladder level. Laproscopic reconstruction • Open reconstruction at renal level. Mrs.Sharin Neetal D'souza

34. Bladder exstrophy: • Definition: • Extrophy of bladder is a congenital malformation in which the lower portion of the abdominal wall and the anterior wall of the bladder are missing, so that the bladder is exerted through the opening and may found on the lower abdomen just above the symphysis pubis, with continuous passage of urine to outside. Mrs.Sharin Neetal D'souza

35. Bladder exstrophy Mrs.Sharin Neetal D'souza

36. Cont.. • Exstrophy of bladder usually associated with number of congenital anomalies related to urogenital tract, musculoskeletal system, and sometimes of GI system. • Male children are more commonly affected. Mrs.Sharin Neetal D'souza

37. Clinical manifestation: • Constant urinary dribbling through the defect • Skin excoriation • Infection • Ulceration of bladder mucosa • Unsteady gait Mrs.Sharin Neetal D'souza

38. Diagnostic evaluation: • Cystoscopic examination • X_ray • USG • IVP • Urodynamic test – to detect the extent of anomaly and other associated problems Mrs.Sharin Neetal D'souza

39. Therapeutic management: • Extrophy of bladder can be managed by surgical closure of the bladder with in 48hrs. • Urinary diversion may be needed in some cases before reconstructive surgery • Complete correction of the malformation can be done in stages by reconstruction of the defect. • Orthopedic surgery may be needed in some cases with associate musculoskeletal problems. Mrs.Sharin Neetal D'souza

40. Nursing management: • Pre-operative management: • Special attention should be given for protection of bladder area from infection and trauma. • These can be obtained by avoiding irritating clothing and linen over the exposed bladder. • Positioning the infant on back (or ) side. Mrs.Sharin Neetal D'souza

41. Cont….. • Humidifying the exposed bladder by covering with wet gauze. • Maintaining aseptic precautions and general hygienic measures along with other routine care • Preparation of parents and child for planned reconstructive surgery is important aspect of nursing care. Mrs.Sharin Neetal D'souza

42. Post –operative management: • Close monitoring of child’s condition, vital signs, features of infection, intake-output etc… • Special attention should be given for care of urinary catheters, its position, drainage and aseptic precautions. • Instructions to be given to parents about necessary precautions for prevention of infection, dislodgement (or) leakage, Mrs.Sharin Neetal D'souza

43. Routine post-operative care should be provided to promote early recovery • Necessary information and demonstration to be given to the parents regarding home based care, follow-up and next probable data of operative interventions. • Teaching about signs of complication and their precautions are important. Mrs.Sharin Neetal D'souza

44. Bladder neck obstruction • Bladder neck obstruction is a condition where the bladder neck does not open enough during voiding. • This has been described as an important lesion in girls with recurrent urinary tract infection and is presumed to be congenital Mrs.Sharin Neetal D'souza

45. Diagnostic evaluation • Cystoscopy • Cystography Mrs.Sharin Neetal D'souza

46. Treatment • Operative enlargement of the bladder outlet. Mrs.Sharin Neetal D'souza

47. Neurogenic bladder: Neurogenic bladder refers to dysfunction of the urinary bladder due to disease of the central nervous system or peripheral nerves involved in the control of micturition (urination). (OR) Faulty innervations of the bladder neck • Causes: • Spinal cord trauma • Tumors • Congenital defects and infections • Spina bifida with a sacral (or) lumbo sacral myelocele. Mrs.Sharin Neetal D'souza

48. TREATMENT • While neurogenic bladder can’t be cured, necessarily, it can most definitely be managed. • Most cases of neurogenic bladder can be managed with medication and intermittent catheterization. • The minority of children with the condition need major reconstructive surgery. Mrs.Sharin Neetal D'souza

49. Complications: • Hydronephrosis • Hypertension • Renal failure. Mrs.Sharin Neetal D'souza

50. Anomalies of the penis and urethra: • Apenia: • Congenital absence of penis is very rare • More than 50% of patient’s have associated genitourinary anomalies. • Eg: cryptorchitism, renal agenesis, dysplasia. Mrs.Sharin Neetal D'souza