2. INTRODUCTION
Genitourinary is a word that refers to the urinary and
genital organs .
Urology is the branch of medicine concerned with the
urinary tract in both genders and genital tract of the
reproductive system in males.
Nephrology is the the branch of medicine concerned
with the kidney.
4. INCIDENCE
Congenital disorder of genitourinary system occurs 1 out of 500
newborns and constitute approx 20-30%of all anomalies identified in
the prenatal period.
Account for 1/3rd of all anomalies detected by routine fetal
ultrasonography.
15%of congenital urogenital anomalies are secondary form
underlying chromosomal disorders.
In children 20% of chronic renal failure is due to renal dysplasia
orthypoplasia.
In adult 10% of chronic renal failure is due to adult polycystic kidney
disease.
6. 1. GENETIC FACTORS
Gene play an important role in many congenital
disorders.
*CHROMOSOMAL ABNORMALITIES:
-late maternal age the time of pregnancy leads to
chromosomal dysfunction.
-Virus like German muscles
-Some chemical agents as anti -mitotic drugs.
7. SINGLE GENE DISORDER
-AUTOSOMAL INHERITANCE :
DOMINANT TRAITS : one affected parents
RECESSIVE TRAITS: both parents
-LINKED OR SEX LINKED INHERITANCE :
DOMINANT TRAITS: daughter affected
RECESSIVE TRAITS: son affected
POLYGENIC OR MULTIFACTIONAL INHERITANCE
Polygenic refers to the sum total of many genes, whereas multifactorial
also includes the interaction of environmental and genetic
determinants.
8. 2. ENVIRONMENTAL FACTORS
1.INTRA UTERINE INFECTION -STORCH
-Syphilis ,Rubella, Taxoplasmoids
2.X-RAY EXPOSURE TO PREGNANCY:
-Exposure to a large dose of x ray can
lead to microcephaly spinal bifida or
cleft palate.
9. 3. MATERNAL DISEASE :
-Diabetes mellitus , endocrine abnormalities
,iodine deficiency, malnutrition
4.MATERNAL ADDICTION:
-Use of alcohol , smoking , tobacco
3.UNKNOWN ETIOLOGY
Although approx 50% of all congenital disorder
cannot be linked to specific cause.
14. 1.RENAL AGENESIS
Presence of normal urethral bud is necessary for renal development ,
therefore an abnormality of urethral
bud during development is responsible for renal
agenesis.
SIGN AND SYMPTOMS :
Absence of kidney and or urine in baby.
Excess and dehydrated skin
Deformities of hands & feet
In female, absence of uterus and upper vagina .
In male ,absence of seminal vesicles & spermatic duct
15. MANAGEMENT:
Keep balance of Na,K and other electrolytes
in the blood.
Assess the patient peripheral edema in every
shift
Measure the wt of pt. Daily.
Nurse should educate the pt about foods and
diet plan.
16. 2.SMALL KIDNEY
Kidney may be small either due to dysplasia or can be small in size
without any alternation in renal parenchyma and normal function.
SIGN & SYMPTOMS:
Pain while passing urine
Pain in abdomen
Blood in urine
Feeling tired
Loss of appetite
Itchy skin
Muscle cramps
17. MANAGEMENT:
A surgery Nephrectomy is surgery to remove a kidney.
POST OP:
-provide and ensure understanding of discharge instruction’
-monitor for bleeding from the insertion site or biopsy site .
-enforce bed rest for pt until stable.
18. 3.ECTOPIA AND FUSION ANOMALIES :
Renal ectopy and fusion are common congenital anomalies of the
kidney and the urinary tract and result from normal embryology
migration of the kidney.
TYPES:
SIMPLE RENAL ECTOPIA
CROSSED RENAL ECTOPIA
HORSE SHOE KIDNEY
19. SIGN AND SYMPTOMS :
1. RENAL ECTOPIA:
UTI
Belly pain
Kidney stone
Hypertension
2.HORSE SHOE KIDNEY
Hank pain
Blood in urine
Nausea
Vomiting
20. Management
1. Blood pressure should be carefully monitored
and elevation treated.
2. Protecting the kidney function
3. Treatment of sepsis
4. Temporary relieve of pressure
21. 4. BLADDER EXSTROPHY
>It is rare birth defect in which the bladder develops
outside the foetus.
SIGN AND SYMPTOMS :
The uretheral not forming
completely
Abnormal genitalia development
Belly button displacement or umbilical hernia
22. MANAGEMENT
1.Complete primary repair of bladder exstrophy .Complete repair surgery
is performed in a single procedure that closes the bladder and abdomen
and repairs the urethra and outer sex organs.
2.Urinary diversion.
3.Protection from bladder infections.
3. Avoid irritating clothes over exposed bladder.
23. 5. ABDOMIANL WALL MUSCLE
DEFICIENCY SYNDROME
PRUNE BELLY SYNDROME
It is rare genetic disorder in which there is a complete or partial
absence of abdominal muscles causing the belly to appear wrinkled
or prune like.
SIGN AND SYMPTOMS
Large abdomen with loops of intestine
Urinary system problems
stomach and intestinal problem
Testicles that fails to descend into the scrotum.
24. MANAGEMENT
BP should be carefully monitored and elevation
treated.
Pain should be assessed continuously and
should be acted upon.
GI function should be monitored to assess
response to interventions.
25.
26. 1.POLYCYSTIC KIDNEY
>It is an inherited disorder in which cluster of cysts develop
within kidneys causing them to enlarge and lose function
overtime.
SIGN AND SYMPTOMS
>High BP
>back at side pain
>blood in urine
>headache
>kidney stone
27. MANAGEMENT
KIDNEY TRANSPLANTATION is the best
treatment for ADPKD pt’s with kidney failure.
Nurses should control BP and abnormalities
related to renal failure of patient.
Nurses should treat hematuria by analgesics plus
copius oral hydration.
28. 2.RENAL CYSTS
Simple renal cysts are rare in children .It is a benign
lesion situated in cortex and lined by simple epithelium
.Renal cysts may be simple or multiple.
SIGN& SYMPTOMS
Fever
Pain or tenderness
between ribs and pelvis
UpperPain abdominal pain
Hematuria
29. MANAGEMENT
Monitor fluid and electrolytes balance
Promote pulmonary function
Provide safety measures
Prevent the infection
31. PELVIURETRIC JUNCTION OBSTRUCTION
It is when a part of the kidney is blocked at renal pelvis . The
blockage slows or stops the flow of urine out of the kidney. Urine
Can then build up and
damage kidney.
SIGN & SYMPTOMS
Abdominal mass
UTI
Flank pain
Kidney stones
vomiting
32. MANAGEMENT
PYELOPLASTY surgery is done to treat this
anomalies.
POST –OP
Close monitoring of child condition.
Special attention to UTI
Avoid irritating clothing
Check for any palpable abdominal mass
33.
34. 1.Cryptochidism
It is the most common birth defect
of the genitourinary tract in male babies .
Normally ,the testicle develop in the
abdomen in the male fetus and descend to the scrotum before
birth,but in this it does not happen.
SIGN & SYMPTOMS
Not feeling or seeing a testicle where you would expect it to be in
scrotum
Scrotum is empty on the affected side.
35. MANAGEMENT
Determine the child’s expectation regarding illness and
hospitalization through discussion &plan therapy
Monitor daily intake & output
Encourage adequate fluids & monitor daily weight .
Continue ressaurance about appearance of genitilia
Administer fluid as ordered , monitor vital signs for
hypotension or tachycardia.
36. 2.URETERO CELEO URETERAL
DUPLICATION
When a child has a ureterocele the portion of the ureterocele , the ureter closest to the
bladder becomes enlarged because the ureter opening is very tiny and obstruct urine flow
. As the urine flow is obstructed , urine backs up in the ureter tube .
SIGN & SYMPTOMS
Side , back or abdominal pain
UTI
Fever
Painful urination
Foul – smelling urine
Excessive urination
37. MANAGEMENT
Asses the patient’s pattern of elimination
Palpate the client bladder every 4 hours
Encourage the client to void every 2- 3 hrs
38. 3.HYPOSPADIAS
It is congenital condition in males
in which the opening of urethra is on
underside of penis
SIGN & SYMPTOMS
Downward curve of penis
Opening of urethra at a location other than tip of penis .
Abnormal spraying during urination
Fertility problems
39. MANAGEMENT
Assist in the identification of defects in complete
newborn exam
Prevent potential complication
Promote parenteral understanding & attachment
Promote normal voiding pattern
40.
41. 1.BIOPSY : .This Technique is usually done as skin
biopsy through needle insertion into lower lobe
of kidney.
CYSTOSCOPY: It is indispensable for
assessing and treating prostate &urologic
problems. It is used to determine the
cause of urinary manifestation such as
those related to prostatic hypermtropy &
to obtain specimens
42. 3. CYSTOGRAPY : Bladder cystography ,also called
voiding cystourethrogram {VCUG} or micturating
cystourethrogram {MCUG } helps doctor’s diagnose
urethra abnormalities or urinary dysfunction ,UTI’s.
4.LAPROSCOPY: used to identify and diagnose the
source or abdominal pain &
to diagnose endometriosis ,
ovarian cysts , fibroids .
Eg: cryptochidism
43. 5.PELVIC ULTRASOUND : It is used the bladder
prostate gland ,seminal vesicles , and for
diagnosis of bladder, tumor , stones.
6.PELVIC MAGNETIC RESOSNACE IMAGING : In
In female –to find problems such as tumours in
ovaries ,rectum , uterus .
In male – to check man prostate & seminal
vesicles & to examine wheather it has spread.
46. SUMMARY
Today we all have discussed together on the topic
congenital disorders. Here we all get to know what is meant
by congenital disorders ,its etiology , epidemiology, types
,clinical manifestation and diagnostic test related to them,
the management of genitourinary system disorders.
47. BIBLIOGRAPHY
BRUNNERS AND SIDDHARTH “TEXTBOOK OF MED –
SURG NURSING ”
LEWIS MEDICAL SURGICAL NURSING “ CHINTAMANI”
WWW. MAYOCLINIC .ORG