SOUTH KAZAKHSTAN MEDICAL

ACADEMY
DEPARTMENT OF PATHOPHYSIOLOGY

TOPIC:

SUBMITTED BY:
GROUP:

SUBMITTED TO:
SHYMKENT 2021
• Prune belly syndrome is a rare condition that gets its name because weak or
absent abdominal wall muscles make an infant’s belly look wrinkled. Other
condition features include undescended testicles and urinary system problems.
Treatment depends on condition severity.
• Four patients with non-functioning kidneys with hydronephrosis underwent a
nephrectomy and one patient pyeloplasty. We demonstrate that successful
treatment is possible even in cases of serious and complex malformations, such
as those of the Prune-Belly syndrome.
• If your child has mild prune belly syndrome, they may take antibiotics to prevent
urinary tract infections. Some children may need surgery. A vesicostomy is
surgery to make a small opening in the bladder through the belly to empty the
bladder of urine.
CAUSES:
Prune belly syndrome is congenital (which means that they are born
with it). No one knows the exact cause of prune belly syndrome.
Researchers have also seen prune belly syndrome in siblings, which
may mean the problem is genetic (runs in the family). In addition, this
condition has been seen in children with trisomy 18 and trisomy 21
(Down syndrome).
Other Names: Eagle-Barrett syndrome; Abdominal muscles, absence of,
with urinary tract abnormality and cryptorchidism
Categories: Congenital and Genetic Diseases; Kidney and Urinary
Diseases
SYMPTOMS:
The most common symptoms include:
Wrinkled or prune-like appearance of the abdominal wall. This is due to the
absence or poor development of the abdominal muscles.
Large abdomen with loops of intestine that can be seen though the thin skin of the
abdominal wall.
Testicles that fail to descend into the scrotum (undescended testicles, in males)
Urinary system problems including enlarged or blocked ureters, enlarged kidney,
enlarged bladder, vesicoureteral reflux (backflow of urine up the urinary tract).
Stomach and intestinal problems.
Heart structural problems.
Musculoskeletal abnormalities, including scoliosis and club foot.
DIAGNOSIS:
This syndrome can often be found during a fetal ultrasound while you are still
pregnant. Your doctor can usually tell by looking at your baby after he’s born that
he has prune belly syndrome.
TREATMENT:
Your baby’s treatment plan is based on their unique needs. Usually a team of
specialists, depending on the organs affected, assist in developing the treatment
plan. Many factors are considered, including:
Overall health of your baby at the time.
Age of your baby.
Extent of your baby’s medical problems.
Ability of your baby to tolerate/survive certain procedures and treatments.
Your child’s outlook for the future.
REFERENCES:
Eagle JF, Barrett GS (1950). “Congenital deficiency of abdominal
musculature with associated genitourinary abnormalities: A syndrome.
Report of 9 cases”. Pediatrics. 6 (5): 721–36. PMID 14797335.
Obrinsky W (1949). “Agenesis of abdominal muscles with associated
malformation of the genitourinary tract; a clinical syndrome”. Am J Dis
Child. 77 (3): 362–73. doi:10.1001/archpedi.1949.02030040372008.
PMID 18116668.
• Frolich, F. Der Mangel der Muskeln, insbesondere der
Seitenbauchmuskeln. Dissertation: Wurzburg 1839.