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Congenital abnormalities of kidney and urinary system
This document discusses congenital abnormalities of the kidney and urinary system (CAKUT). It begins by describing the normal anatomy of the urinary system, including the kidneys, ureters, urinary bladder, and urethra. It then discusses various abnormalities that can occur during development, including dysgenesis of the kidneys resulting in renal agenesis, hypoplasia, or dysplasia. Other abnormalities mentioned include anomalies in kidney shape and position, abnormalities of the collecting system, and abnormalities of the bladder and urethra. Specific conditions like renal agenesis, Potter's syndrome, and multicystic dysplastic kidney are then explained in more detail.
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Congenital abnormalities of kidney and urinary system
- 1. CONGENITAL ABNORMALITIES OF KIDNEYAND URINARY SYSTEM (CAKUT) SUNIL KUMAR.P Haematology& Transfusion medicine 2/3/2018 1SUNIL KUMAR P
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- 4. • The urinarysystem consist of the ….. • 1.Kidneys • 2.Ureters • 3.Uriary bladder • 4.Urethra 2/3/2018 4SUNIL KUMAR P
- 5. Urinary system….. • UpperPart : • Located within the ABD and consist of the • 1.Kidneys • 2.A large portions of the ureters 2/3/2018 5SUNIL KUMAR P
- 6. Urinary system….. • LowerPart: • Constitutes the Pelvic organs includes • 1.Ureters • 2.Urinay bladder • 3.Urethra 2/3/2018 6SUNIL KUMAR P
- 7. Functions of UrinarySystem… • Filtering and eliminating wastes from the body • Also Maintains the……. • Homeostasis of water, • Ions • pH • BP • Production of hormones….EPO, Renin, Calcitriol 2/3/2018 7SUNIL KUMAR P
- 8. • The ureters,urinary bladder, and urethra together form the urinary tract…….. which acts as a plumbing system to drain urine from the kidneys ,store, it and then release it during urination. 2/3/2018 8SUNIL KUMAR P
- 9. KIDNEYS • Kidneys arepair of bean- shaped excretory organs situated along the Posterior wall of the abdominal cavity. • The Lt.Kidney is located slightly higher than right kidney because the right side of the liver is much larger than the left side. 2/3/2018 SUNIL KUMAR P 9
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- 11. Functions of Kidneys…. •The kidneys filter metabolic wastes , excess ions and chemicals. 2/3/2018 11SUNIL KUMAR P
- 12. Ureters • The uretersare a pair of tubes that carry urine from the kidneys to the urinary bladder. • The ureters about 10-12 inches long and run on the left and right sides of the body parallel to the vertebral Coolum. 2/3/2018 SUNIL KUMAR P 12
- 13. • The endsof the ureters extend slightly in to the urinary bladder and are sealed at the point of entry to the bladder by ureterovesical valves. • The valves prevent urine from flowing back towards the kidney. 2/3/2018 SUNIL KUMAR P 13
- 14. Urinary Bladder…. • Theurinary bladder is a sac like hollow organ used for the storage of urine. • The urinary bladder is located along the body’s midline at the inferior end of the pelvis. • Urine entering the urinary bladder from the ureters slowly fills the hollow space of the bladder and stretches it’s elastic walls. 2/3/2018 14SUNIL KUMAR P
- 15. • The wallsof the bladder allow it to stretch to hold any where from 600-800 ml of urine 2/3/2018 SUNIL KUMAR P 15
- 16. urethra • The urethrais the tube through which urine passes from the bladder to the exterior of the body. • The female urethra is around 2inches long and ends inferior to the clitoris and superior to the vaginal opening. • In males the urethra is around 8-10 inches long and ends at the tip of the penis. 2/3/2018 16SUNIL KUMAR P
- 17. Abnormalities during development: •1.Dysgenesis of the Kidney • a- Renal Agenesis(absent Kidney) • b- Renal Hypoplasia • c-Renal dysplasia • D-Cystic dysplasia • E-Solitary kidney disease 2/3/2018 17SUNIL KUMAR P
- 18. 2.Abnormalities in shape& position • a- Ectopic Kidney • b- Fusion Anomalies • - horseshoe Kidney • - crossed fused Ectopia 2/3/2018 18SUNIL KUMAR P
- 19. 3. Abnormalities ofthe collecting system: • Hydronephrosis • Bladder extrophy • PUV • Patent Urachus 2/3/2018 19SUNIL KUMAR P
- 20. 4.Abnormalities in Bladder •1.Ureterocele • 2.Vesicoureteral reflex (VUR) 2/3/2018 SUNIL KUMAR P 20
- 21. 5.Abnormalities in Urethra •PUV ( Posterior Urethral Valves) 2/3/2018 SUNIL KUMAR P 21
- 22. 1.Dysgenesis of theKidney • a- Renal Agenesis • b- Renal Hypoplasia • c-Renal dysplasia 2/3/2018 22SUNIL KUMAR P
- 23. RENAL AGENESIS • Kidneyis either absent or undeveloped. • It usually causes no symptoms and is found incidental • It is due to failure of ureteric bud formation or mesenchymal blastoma differentiation of final mesenchymal condensation. • 1:500 – 1:3200 live births 2/3/2018 23SUNIL KUMAR P
- 24. ETIOLOGY • There isno family history, but in 20-36% of cases, there is a genetic cause. • The risk of recurrence in future pregnancies is 3% unless one parent has unilateral renal agenesis, in which case the risk is about 15%. • Women with uncontrolled diabetes in pregnancy may deliver a baby with bilateral renal agenesis. 2/3/2018 24SUNIL KUMAR P
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- 26. a) Unilateral RenalAgenesis • It is common and not usually of any major health consequence, as long as the other kidney is healthy. • It is associated with an increased incidence of mullerian duct abnormalities which are abnormalities of the development of the female reproductive tract and can be a cause of infertility. • People with this condition are advised to approach contact sports with caution. 2/3/2018 26SUNIL KUMAR P
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- 28. CLINICAL MANIFESTATIONS • noother symptoms at all. • premature birth. • low-set ears (This is because the ears and kidneys are formed at the same time in fetal development) • The ureters may also be abnormal 2/3/2018 28SUNIL KUMAR P
- 29. b) Bilateral renalagenesis • It is the uncommon and serious failure of both a fetus' kidneys to develop during gestation. • The malformations associated to this is known as Potters Syndrome. • This absence of kidneys causes oligohydramnios, which can place extra pressure on the developing baby and cause further malformations. • The condition is frequently, but not always the result of a genetic disorder. 2/3/2018 29SUNIL KUMAR P
- 30. • It ismore common in infants born to one or more parents with a malformed or absent kidney. • Males are more commonly affected and most infants that are born alive do not live beyond four hours. 2/3/2018 30SUNIL KUMAR P
- 31. CLINICAL MANIFESTATIONS • They mayhave a number of unique characteristics: • dry loose skin • wide-set eyes • prominent folds at the inner corner of each eye • sharp nose • large low-set ears with lack of ear cartilage • underdeveloped lungs • absent urinary bladder • anal atresia • esophageal atresia • unusual genitals • The lack of amniotic fluid causes some of the problems (undeveloped lungs, sharp nose, clubbed feet) 2/3/2018 31SUNIL KUMAR P
- 32. DIAGNOSIS • In afetal ultrasound there will be a lack of amniotic fluid. It is detected by US at 12th wk of gestation. 2/3/2018 32SUNIL KUMAR P
- 33. TREATMENT • 1.Short-term treatment •Bilateral renal agenesis is fatal. • If one kidney is present, the child will develop normally. The remaining kidney, if properly functioning, can very effectively remove the wastes from the blood and keep the body entirely healthy. • Once detected, families where renal agenesis has occurred will be offered genetic counseling because of the possibility of recurrence in future pregnancies. 2/3/2018 33SUNIL KUMAR P
- 34. 2.Long-term treatment • Protectthe remaining kidney from infection or injury. • Periodic examinations of the kidney and prompt treatment of any urinary tract infection is required. • Counselling to avoid contact sports where the kidney could be injured. 2/3/2018 34SUNIL KUMAR P
- 35. b)Renal hypoplasia • Thisappears as one small kidney with the other one larger. • It occurs due to the partial development of kidney. Small kidneys also have small arteries and are associated with hypertension requiring nephrectomy. 2/3/2018 35SUNIL KUMAR P
- 36. c)Renal dysplasia • Multicysticdysplastic kidney results the malformation of the kidney during fetal development. • The kidney consists of irregular cysts of varying sizes and has no function. • It is the most common type of renal cystic disease, and it is one of the most common causes of an abdominal mass in infants. 2/3/2018 36SUNIL KUMAR P
- 37. TYPES TYPES • Bilateral • Unilateral •INCIDENCE • the disease is found to be bilateral in 19% to 34% of cases 2/3/2018 37SUNIL KUMAR P
- 38. CLINICAL MANIFESTATIONS • Thosewith bilateral disease often have other severe deformities . • In bilateral cases, the newborn has the classic abnormal facies • Oligohydramnios • Characteristic of Potter's syndrome • Contralateral ureteropelvic junction • Hypertension • (Malignant transformation to Wilm's tumor has been reported) 2/3/2018 38SUNIL KUMAR P
- 39. DIAGNOSIS • Antenatal ultrasoundabout 28 weeks, with a range of 21 to 35 weeks. 2/3/2018 39SUNIL KUMAR P