UROGENITAL

ABNORMALITIES.
Defination
These are birth defects and
inherited conditions that affect
the urinary tract or the genital
tract(reproductive organs) also
called the genital urinary
system.
Renal hypoplasia
is a congenitally small kidney
without dysplasia and can be
bilateral or unilateral
Key findings in renal agenesis:
location; unilateral or bilateral;
if bilateral, findings can be
asymmetric e.g agenesis on one
side, hypoplasia on the other
side.
Agenesis/degree of hypoplasia;
kidney absent vs present but
small or rudimentary.
NB; BE SURE NOT TO CONFUSE
THIS CONDITION WITH
MULTICYSTIC RENAL DYSPLASTIC.
Signs and symptoms.
Both types of renal agenesis
are associated with other birth
defects, such as problems with
the;
1.lungs
2. genitals and urinary tract
3. stomach and intestines
4. Heart
5. muscles and bones
6. eyes and ears
Diagnosis.
Prenatal. Renal agenesis can be
diagnosed or strongly suspected
prenatally by U/S but should
always be confirmed
postnatally.
Postnatal. Renal agenesis or
hypoplasia is conclusively
diagnosed only through direct
assessment by abd. U/S, CT
scan or MRI, surgery or autopsy.
Bilateral renal agenesis should be
considered in an infant with
features of potter sequence.
Bilateral renal hypoplasia might or
might not be recognized after
delivery, depending on the
severity and degree of residual
kidney function.
Unilateral renal agenesis or
hypoplasia may be clinically silent
at delivery if the contralateral
kidney is functional, such that the
diagnosis may occur later on
Clinical and epidemiology.
About half of all the cases of
bilateral renal agenesis there are
other structural anomalies e.g.
urogenital cardiac, skeletal, CNS or
syndromes (chromosomal or
genetic).
The non-syndromic multiple anomaly
patterns include the following:
1. VATER/VACTERL( Vertebral, Anus,
Cardiac,Trachea, Oesophagus, Renal,
Limb) association;
2. MURCS association
( Mullerian, Renal,
Cervicothoracic Somite
abnormalities), a developmental
disorder affecting primarily
females and involving mainly
the reproductive and urinary
systems.
3.CAUDAL DYSPLASIA ( also
seen in progestational
diabetes).
TREATMENT
About 4 in 10 fetuses with
bilateral renal agenesis die in
the uterus before child birth
(stillbirth) or lead to premature
birth less than 37wks in
pregnancy.
For infants born alive they
entirely depend on kidney
transplant and dialysis. This
procedure is experimental and
not widely available.