4. 1.Dysgenesis of the Kidney
a- Renal Agenesis
b- Renal Hypoplasia
c-Renal dysplasia
4
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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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
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
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. 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. COMPANY LOGO
COMPLICATIONS
• Malignancy: Wilm's’ tumor, adenocarcinoma&
embryonic carcinoma.
• HTN: cured by nephrectomy.
• Infection, bleeding into, or rupture of cysts if
large.
23
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. 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. 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. 3. Abnormalities of the collecting system:
• Hydronephrosis
• Bladder extrophy
• PUV
• Patent Urachus
28
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
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. MANAGEMENT
Surgical removal or pyloplasty is done and
in case of complication nephrectomy or
percutaneous nephrostomy is indicated.
33
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
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. 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. 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. 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
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. 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. 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
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. 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. 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
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. 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. 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. 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
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. 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. 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
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. 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. 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. 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. 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. 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. 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. 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
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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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
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. 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. TREATMENT
The type of treatment depends on the severity of the
symptoms.
Vesicostomy
83
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
