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congenital anomalies of renal system


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congenital anomalies of renal system

  1. 1. Company LOGO CONGENITAL ABNORMALITIES Kidney and Urinary Tract
  2. 2. COMPANY LOGO Abnormalities during development: 1.Dysgenesis of the Kidney a- Renal Agenesis(absent Kidney) b- Renal Hypoplasia c-Renal dysplasia 2.Abnormalities in shape & position: a- Ectopic Kidney b- Fusion Anomalies  horseshoe Kidney  crossed fused Ectopia 2
  3. 3. COMPANY LOGO 3. Abnormalities of the collecting system: • Hydronephrosis • Bladder extrophy • PUV • Patent Urachus 3
  4. 4. 1.Dysgenesis of the Kidney a- Renal Agenesis b- Renal Hypoplasia c-Renal dysplasia 4
  5. 5. RENAL AGENESIS • Kidney is 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 5
  6. 6. ETIOLOGY • There is no 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. 6
  8. 8. a) Unilateral Renal Agenesis  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. 8
  9. 9. CLINICAL MANIFESTATIONS  no other 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 9
  10. 10. b) Bilateral renal agenesis  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.  It is more 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. 10
  11. 11. CLINICAL MANIFESTATIONS They may have 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) 11
  12. 12. DIAGNOSIS In a fetal ultrasound there will be a lack of amniotic fluid. It is detected by US at 12th wk of gestation. 12
  13. 13. 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. 13
  14. 14. 2.Long-term treatment  Protect the 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. 14
  15. 15. NURSING MANAGEMENT  Protecting the kidney function.  low dose of an antibiotic to take once a day to prevent kidney infection and damage.  Blood pressure should be carefully monitored and elevations treated.  Dialysis or kidney transplant is preferred when the solitary kidney has ceased to function. 15
  16. 16. COMPANY LOGO b)Renal hypoplasia This appears 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. 16
  17. 17. COMPANY LOGO c)Renal dysplasia Multicystic dysplastic kidney results from 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. 17
  18. 18. COMPANY LOGO TYPES • Bilateral • Unilateral INCIDENCE • the disease is found to be bila teral in 19% to 34% of cases. 18
  19. 19. COMPANY LOGO CLINICAL MANIFESTATIONS • Those with bilateral disease often have other s evere 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) 19
  20. 20. DIAGNOSIS Antenatal ultrasound about 28 weeks, with a range of 21 to 35 weeks. 20
  21. 21. COMPANY LOGO TREATMENT • It is not treatable. • observe periodically for the first few years to ensure the healthy kidney is functioning properly and that the unhealthy kidney is not causing adverse effects. • In case of renal hypertension or malignant transformation, the un healthy kidney is removed entirel y. 21
  22. 22. COMPANY LOGO CONSERVATIVE MANAGEMENT • cysts < 5cm , high chance of involution, or cause no prob lems. • reviewed annually for: - BP - urinary protein. - US for cysts involution, of MCDK. growth of contra-lateral kidney. Up to 2yrs of age then at 5yrs of age if normal. • Nephrectomy: 1- no involution by 2 yrs of age. 2- HTN 3- infections 22
  23. 23. COMPANY LOGO COMPLICATIONS • Malignancy: Wilm's’ tumor, adenocarcinoma& embryonic carcinoma. • HTN: cured by nephrectomy. • Infection, bleeding into, or rupture of cysts if large. 23
  24. 24. 2.Abnormalities in shape & position: a- Ectopic Kidney b- Fusion Anomalies  horseshoe Kidney  crossed fused Ectopia 24
  25. 25. a)Ectopic kidey Renal ectopia or ectopic kidney describes a kidney that is not located in its usual position. It results from the kidney failing to ascend from its origin in the true pelvis or from a superiorly ascended kidney located in the thorax. 25
  26. 26. b)Fusion anomalies 1. Horse Shoe Kidney It develops when the lower poles of the kidneys are fused in the midline due to fusion of ureteric buds during fetal development. These kidneys are more prone to develop wilms tumour than general. Diagnosis could be done with IVP. Surgery is indicated when uncontrolled urinary infections result in pyelonephritis. 26
  27. 27. 2.Crossed fused ectopia • In this abnormality, both the kidneys are located on the same side with two separate ureters arising from the respective kidneys. The ureter arising from the crossed over kidney travels back to the opposite side and inserts in the bladder. • This congenital anomaly is the result of the abnormal development of the ureteric bud and metanephric blastema during the fourth to eighth weeks of gestation. 27
  28. 28. 3. Abnormalities of the collecting system: • Hydronephrosis • Bladder extrophy • PUV • Patent Urachus 28
  29. 29. a) Hydronephrosis It is the dilatation of the renal pelvis which may be found as unilateral or bilateral. It may be due to obstruction of urine flow in the distal urinary tract or reflux of urine up the ipsilateral ureter or due to bladder neck obstruction or urethral obstruction. 29
  30. 30. ETIOLOGY Uretero Pelvic Junction Obstruction Vesico Urethral Reflux Megaureter Ureterocoele PUV 30
  31. 31. CLINICAL MANIFESTATIONS urinary infections large abdominal mass abdominal pain failure to thrive anemia hypertension Hematuria renal failure. 31
  32. 32. DIAGNOSIS  Antenatal US ( 18-20 WKS) - severity of antenatal US - Unilateral vs. bilateral - Renal parenchyma thin - Bladder - Amniotic fluid  Post natal Physical exam: Abdominal mass, palpable bladder  USG  IVP  MCU  diuretic isotope renography 32
  33. 33. MANAGEMENT Surgical removal or pyloplasty is done and in case of complication nephrectomy or percutaneous nephrostomy is indicated. 33
  34. 34. b) Posterior urethral valve (PUV) It is the most frequent cause of distal urinary tract obstruction. The valves are found usually at the point of junction of posterior urethra with anterior urethra. 34
  35. 35. 35
  36. 36. CLINICAL MANIFESTATION • dribbling of urine • abnormal urine stream • palpable bladder • recurrent urinary tract infections • vomiting • failure to thrive • pulmonary hypoplasia. • Poor urinary stream • Voiding dysfunction. • Urosepsis. 36
  37. 37. DIAGNOSIS  US suggestive at < 24 wks gestation  MCU  USG  Endoscopy 37
  38. 38. MANAGEMENT • It could be done by urinary catheterization • Defenitive management is by transurethral destruction of valvular leaflet by balloon catheter. • In some cases temporary urinary diversion is done. NURSING MANAGEMENT • correction of electrolytes. • Treatment of sepsis. • Resp.distress • Temporary relieve of pressure 38
  39. 39. c) Exstrophy of bladder In this the lower portion of abdominal wall and the anterior wall of the bladder are missing, so that the bladder is everted through the opening and may found on the lower abdomen just above the symphysis pubis, with continuous passage of urine to outside. It occurs as a result of altered, not arrested embryogenesis. 39
  40. 40. INCIDENCE: -It is the most common congenital anomaly of lower urinary and genital tracts. -It occurs in 1 in 30,000 to 40,000 live births. -it occurs frequently in males than in females. 40
  41. 41. CLINICAL MANIFESTATION: • This condition is diagnosed on inspection at birth. -Urinary dribbling through defect -skin excoriation -infection & ulceration of bladder mucosa -ambigous genetalia -wadling unsteady gait -UTI -Growth failure 41
  42. 42. DIAGNOSIS - physical examination -cystoscopy -X-ray -USG -IVP -urodynamic testing 42
  43. 43. MANAGEMENT: -surgical closure of bladder within 48 hours -urinary diversion -complete correction in stages by reconstruction -orthopedic surgery in case of musculoskeletal problems 43
  44. 44. NURSING MANAGEMENT: Supporting nursing care is important before and after reconstructive surgery to prevent complications. • Pre-operative care:- -Protection of bladder area from infections and trauma -avoid irritating clothing over exposed bladder -position by back or side -humidifying with wet gauze -Preparation of parents and child for surgery • Post-operative care:- -close monitoring of child’s condition -special attention to urinary catheter, drainage -teaching the parents regarding follow up care, complications and prevention 44
  45. 45. The urachus is a remnant of allantois, a channel between the bladder and the umbilicus (belly button) where urine initially drains in the fetus during the 1st trimester of pregnancy. The channel of the urachus usually seals off and obliterates around the 12thweek of gestation and all that is left is a small fibrous cord between the bladder and umbilicus called the median umbilical ligament. 45
  46. 46. 46
  47. 47. A patent urachus occurs when the urachus did not seal off and there is a connection between the bladder and the umbilicus. A patent urachus can cause varying amounts of clear urine to leak at the umbilicus. If the urachussss remains open all the way to the bladder, there is the danger that bacteria will enter the bladder through the open tube and cause infection. For this reason, the patent urachus of the infant must be removed 47
  48. 48. A surgical incision is made in the baby's abdomen and the patent urachus is removed, then the opening to the bladder is closed. 48
  49. 49. Pelvi-ureteric junction stenosis 49 It is the narrowing of the ureter at the junction between the ureter and renal pelvis of the kidney. It produces blockage of urine drainage from the kidney (hour glass appearance).
  50. 50. 50 It produces increased backpressure on the kidney and can cause impaired kidney function and ultimately long term potential damage to the kidney itself. It is found as a cause of hydronephrosis and it can be associated with ectopic or horse shoe kidney.
  51. 51. INCIDENCE 51  Ureteropelvic junction stenosis and obstruction is the most common cause of kidney blockage or obstruction in children.  It is the most common site of obstruction in the upper urinary tract  It occurs nearly 1 in 500 to 1:1250 live births.
  52. 52. ETIOLOGY 52  The two main causes of PUJ obstruction are:  intrinsic muscular defect causing impaired peristalsis and urine drainage  extrinsic obstruction caused by an aberrant or accessory vascular stalk leading to the lower pole of the kidney and crossing anteriorly to the PUJ or upper ureter.
  53. 53. CLINICAL MANIFESTATIONS 53  recurrent renal colic o flank or abdominal pain o nausea o vomiting  flank mass without symptoms  often associated with UTI  upper abdominal pain
  54. 54. DIAGNOSIS 54  Prenatal ultrasonography  USG  IVP  Renal scan  Renal function test
  55. 55. MANAGEMENT 55 Pyloplasty is indicated to remove obstruction and to avoid complications. The indications for conservative or surgical therapy of PUJ obstruction are still developing. PUJ obstruction by crossing renal vessels is essential in choosing the appropriate surgical approach.
  56. 56. Epispadiasis It is the congenital abnormal urethral opening on the upper surface (dorsum) of the penis. Urethra is displaced dorsally due to the abnormal development of the infraumbilical wall and upper wall of the urethra. It is associated with extrophy of bladder and ambiguous genetalia.The corresponding defect in females is a fissure in the upper wall of the urethra and is quite rare. 56
  57. 57. TYPES: In male child Anterior with normal continence Posterior epispadiasis Male infants have short and broad penis with dorsal curvature. In female child Bifid clitoris with no incontinence of urine Subsymphyseal with incontinence of urine A cleft extends along the roof or entire urethra, involving the bladder neck. 57
  58. 58. DIAGNOSIS diagnosed at birth itself IVP MCU vesicourethral reflux bladder capacity 58
  59. 59. MANAGEMENT Surgical correction  1st stage - it is done about 1.5 to 2 years of age for penile lengthening, elongation of urethral strip and chordee correction.  2nd stage – it is done atleast 6 months after 1st stage urethral reconstruction  3rd stage – it is done about 3 - 4 years of age for bladder neck reconstruction and correction of VUR. Cytoplastycan be done to enhance the bladder capacity after 2 – 3 years of 3rd stage operation. Supportive nursing care should emphasize on prevention of infection. 59
  60. 60. Hypospadiasis Hypospadiasis is a congenital defect, primarily of males, in which the urethra opens on the underside (ventrum) of the penis. The corresponding defect in females is an opening of the urethra into the vagina and is rare. It may be found in females as urethral opening in the vagina with dribbling of urine. 60
  61. 61. INCIDENCE • It is a commonest malformation in a male child occurs 1-3 • Occurs in 1-3 males per 1000 live births • Close relatives of the patients are most likely to have compared to other population 61
  62. 62. TYPES Anterior -65-70% -May be found as glandular or coronal or on distal penile shaft. Middle -10-15% -Penile shaft hypospadiasis Posterior -20% -May be found on proximal penile shaft or as penoscrotal, scrotal or perineal type 62
  63. 63. DIAGNOSIS: • Mostly observed at birth • observe for any abnormal voiding pattern • observe for inability of the boy to stand to urinate, he must sit to void. 63
  64. 64. MANAGMENT: -surgical reconstruction to obtain straight penis at erection, to form urethral meatus at the tip of glans penis. -meatotomy is done at any age after birth. -chordee correction and advancement of prepuce can be done at 2 – 3 years of age. -urethroplasty is done 3 – 4 months after chordee correction. (Surgical correction should be completed before admission to school.) 64
  65. 65. SURGICAL COMPLICATIONS Surgical complication rates depend on the chosen procedure, and include urethrocutaneous fistula, meatal stenosis, urethral strictures, urethral diverticula, complete breakdown, skin necrosis, residual or recurrent curvature and hypospadiac cripple. 65
  66. 66. Phimosis Phimosis refers to the narrow opening of the prepuse that prevents it being drawn back over the glans penis. It also can be acquired by the inflammation of glans or prepuse. The term may also refer to clitoral phimosis in women, whereby the clitoral hood cannot be retracted, limiting exposure of the glans clitoridis. 66
  67. 67. TYPES Different authors calssify it differently • Pathological • Physiological • Physical 67
  68. 68. ETIOLOGY • balanitis (inflammation of the glans penis) • Preputial stenosis or narrowness that prevents retraction, by fusion of the foreskin with the glans penis in children • unusual masturbation practices • secondary to chronic inflammation • repeated catheterization • forcible foreskin retraction • Untreated diabetics due to the presence of glucose in their urine giving rise to infection in the foreskin 68
  69. 69. CLINICAL MANIFESTATIONS • inability to retract the foreskin during routine cleaning or bathing • ballooning of the prepuce during urination • painful erections • Hematuria • recurrent urinary tract infections • weakened urinary stream • Physical Phimosis, the foreskin cannot be retracted proximally over the glans penis. • Physiologic phimosis, the preputial orifice is unscarred and healthy appearing. • Pathologic phimosis, a contracted white fibrous ring may be visible around the preputial orifice 69
  70. 70. MANAGEMENT: • Phimosis in infancy needs to be treated only if it is causing obvious problems such as urinary discomfort or obstruction. If phimosis in older children or adults is not causing acute and severe problems, nonsurgical measures may be effective. 1.Non surgical methods include: • Steroid therapy • Manual stretching 70
  71. 71. 2.Surgical methods It range from the complete removal of the foreskin to more minor operations to relieve foreskin tightness: • Circumcision • Dorsal slit (superincision) • Ventral slit (subterincision) • Preputioplasty 71
  72. 72. PROGNOSIS The most acute complication is paraphimosis. In this condition, the glans is swollen and painful, and the foreskin is immobilized by the swelling in a partially retracted position. The proximal penis is flaccid. 72
  73. 73. Paraphimosis It may develop in phimotic child. It is an uncommon medical condition where the foreskin becomes trapped behind the glans penis, and cannot be reduced (that is, pulled back to its normal flaccid position covering the glans penis). It is the retraction of a phimotic foreskin, behind coronal sulcus forming a tight constricting ring around the glans. The foreskin is retracted behind the glans penis and cannot be replaced to its normal position.73
  74. 74. CLINICAL MANIFESTATIONS • painful, swollen glans penis in the uncircumcised or partially circumcised patient • irritability • Flaccidity of the penile shaft proximal to the area of paraphimosis is seen • Erythematous and edematous glans • The glans penis is initially its normal pink hue and soft to palpation. As necrosis develops, the color changes to blue or black and the glans becomes firm to palpation. 74
  75. 75. ETIOLOGY The foreskin may be retracted during penile examination, penile cleaning, urethral catheterization, or cystoscopy; if the foreskin is left retracted for a long period, some of the foreskin tissue may become edematous (swollen with fluid), which makes subsequent reduction of the foreskin difficult. 75
  76. 76. PREVENTION • Paraphimosis can be avoided by bringing the foreskin back into its reduced position after retraction is no longer necessary • Phimosis is a risk factor for paraphimosis; treat pathologic phimosis via long-term stretching or elective surgical techniques 76
  77. 77. MANAGEMENT • Manual manipulation of the swollen foreskin tissue This involves compressing the glans and moving the foreskin back to its normal position, perhaps with the aid of a lubricant, cold compression, and local anesthesia as necessary. • Dorsal slit • Circumcision • The Dundee technique 77
  78. 78. Prune belly syndrome ( Eagle-Barrett syndrome, Triad syndrome) It is a rare, genetic birth defect characterized by a triad of symptoms. 1.Deficiency or absence of anterior abdominal wall musculature. 2.Bilateral cryptorchidism 3.Ureter, bladder,& urethral abnormalities( megacystis, Megaureter, 2°dysplasia) 78
  79. 79. Other names for the syndrome include Abdominal Muscle Deficiency Syndrome, Congenital Absence of the Abdominal Muscles, Eagle-Barrett syndrome, Obrinsky Syndrome, Frohlich Syndrome,or Triad Syndrome 79
  80. 80. INCIDENCE About 1 in 40,000 births About 97% of those affected are male 80
  81. 81. SYMPTOMS  A partial or complete lack of abdominal muscles. There may be wrinkly folds of skin covering the abdomen.  Undescended testicles in males  Urinary tract abnormality such as unusually large ureters, distended bladder, accumulation and backflow of urine from the bladder to the ureters and the kidneys  Frequent urinary tract infections due to the inability to properly expel urine.  Later in life, a common symptom is post-ejaculatory discomfort. Most likely a bladder spasm, it lasts about two hours. 81
  82. 82. DIAGNOSIS  Via ultrasound while a child is still in-utero.  An abnormally large abdominal cavity resembling that of an obese person is the key indicator, as the abdomen swells with the pressure of accumulated urine.  In young children, frequent urinary tract infections  Blood tests to check kidney function  Voiding cystourethrogram  Orthopedic evaluation 82
  83. 83. TREATMENT The type of treatment depends on the severity of the symptoms.  Vesicostomy 83
  84. 84. COMPLICATIONS  distending and enlarging of internal organs such as the bladder and intestines  Surgery is often required but will not return the organs to a normal size.  Also many complications can come from enlarged/malformed kidneys  Musculoskeletal abnormalities include pectus excavatum, scoliosis, and congenital dislocations including the hip. 84