Ekstrofia Buli

definition
Bladder exstrophy is a congenital abnormality in which
a baby is born with the bladder exposed . This disorder
can be seen clearly and occurs due to failure to close
the anterior abdominal wall infraumbilikus

epidemology
Bladder exstrophy is a rare disorder . The number of
events is only about 1 : 10,000 to 1 : 50,000 live births.
This disorder occurs 3 to 6 times more often in men
than in women. Supported the possibility of genetically
related increase in the incidence becomes berkiras 1 :
275 births in orangtuayang have a child with this
disorder will have a child with the same disorder

 Bladder exstrophy exact cause is unknown, but is believed to have terjadigangguan

establishment and fetal organ development during pregnancy. In perkembangannormal,
mesodermal growth between the ectoderm and endoderm layers of membrane cloacal
bilaminar produce a muscle formation lower abdomen and pelvis. After the course of
mesenchymal growth, growth down from membagikloaka rectal septum into the bladder
and rectum on the front with back at the early stage of development, there is no
separation between the urinary tract and gastrointestinal tract. Membrankloaka form
the caudal region of the fetus. At the caudal end of the cloaca, there ektodermtepat
above endoderm forming a thin membrane of the cloaca. With growth, sebuahseptum
formed (folds Toureux s) that divides the rear with ruanganterior intestine, which is the
urogenital sinus. This septum formed to caudal. Two jaringanmelipat formed from the
lateral portion of the cloaca (fold Rathke's). These folds medialsaling moving closer to
complete separation of the rear intestine urogenital.Lipatan Tourneux's sinus and fold
together to form a Rathke's uro-rectal septum. In gestation harikesepuluh, bladder
cylindrical. The cylinder cranial part meruncingmembentuk vesico-alantoik canal, which
then closes perfectly, leaving ligamenumbilikus medially

The division of the cloaca into the urogenital sinus and

rectum . A. at the end of the fifth week . Bminggu to seven .
C.minggu to eight

clinical symptoms
In patients with bladder ekstropi will happen
incontinence total but generally does not occur during a
urinary tract infection urine flow is not disrupted.
Usually the baby's growth is not disrupted despite
severe disorder and wound in the abdominal wall wet
with urine darimuara ureter . Should be given vaseline
on the edge of the skin to prevent dermatitis due to wet
urine. Can cause chronic irritation of the bladder
danmetaplasia residual fibrosis that may be the basis
carcinoma later in life

Conts
Defects in bone
Defects in the pelvic wall
defect in the abdominal wall
Defect anorectal

Diagnosis
Diagnosis Prenatal = USG
DiagnosisPascanatal = fenotip

Governance
Surgery
Reconstructive surgery is needed to repair bladder exstrophy . Type of
management depends on the type and degree of abnormality that occurs .
Currently available management includes several types of surgical
procedures performed within a period of several years . This action is known
as functional reconstruction stages

Non-Surgery
Management begins by addressing the general state of the patient . Cover
open urinariayang bladder by using a clean plastic cover . Avoid lembabdan
emergence of the state of water droplets, which can irritate the bladder
mucosa is thin . Antibiotic therapy can be started immediately after
delivery , and resume immediately after the surgery performed .
Prophylactic antibiotics are given every day after the closing act bladder

Prognosis
The prognosis of patients with bladder exstrophy very
well after surgery