fetal anomaly series prune belly syndrome
DESCRIPTION
FETAL ANOMALY SERIES PRUNE BELLY SYNDROMETRANSCRIPT
Dr Arun Gupta Director imaging
Dr Rakhee gupta Dr Vinayak Mittal
Dr Kiran Dr Ritesh Mahajan
Abdominal Muscle Deficiency Syndrome, Congenital Absence of
the Abdominal Muscles, Eagle-Barrett Syndrome, Obrinsky
Syndrome,Fröhlich Syndrome, or rarely, Triad Syndrome.
FETAL ANOMALYSERIESADVANCED USG
LOUNGE
PRUNE BELLY SYNDROME
The prune belly syndrome is a rare anomaly comprising a specific constellation of features. It consists of three major findings :
1. Gross ureteric dilatation2. Anterior abdominal wall underdevelopment
(resulting in the "prune belly" appearance)3. Bilateral undescended testes .
There is often an association with other respiratory, gastrointestinal, musculoskeletal, and
cardiovascular anomalies.
FETUS OF FOURTEEN WKS GESTATION WITH GROSS DISTENSION OF THE
ABDOMEN BY CYSTIC FLUID FILLED DEFINED LESION .
FETAL ABDOMEN
FETAL SPINE
An ultrasound examination that reveals a dilated bladder and/or fetal ureters, a distended fetal abdomen, and oligohydramnios, either alone or in combination, should alert a physician to the possibility of Prune-Belly syndrome.
GORSS MASS EFFECT LEAD TO POOR
DISCRETE VISUALIZATION OF THE REST OF THE
ABDOMINAL VISCERA .
FETAL HEAD
FETAL ABDOMENGROSSS DISTENSIONSEQUAEL TO FETAL
MEGACYSTIS
DORSUM OF FETAL TORSO
RENDERED IMAGE
AC IS CORROBORTAIVE
WITH POG OF 20WKS REST OF THE FETAL
BIOMETRY IS CORROBORATIVE
WITH 14WKS. THIS IS SEQUAEL TO
GROSS ABDOMEN DISTENSION OWING
TO MEGACYSTIS
FETAL SPINE NORMAL CORONAL VIEW
OLIGOHYDRAMNIOS
GROSS DISTENSION OF THE
FETAL ABDOMEN LEADING TO MASS EFFECT ON THE
THORACIC CAVITY
( VOLUME LOSS)
PRUNE BELLY SYNDROME …….BRIEF
A partial or complete lack of abdominal muscles .There may be wrinkly folds of skin covering the abdomen.Undescended testis in malesUrinary 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.
Prune belly syndrome is a rare, genetic birth defect affecting about 1 in 40,000 births.About 97% of those affected are male. Prune belly syndrome is a ,congenital disorder of the urinary system characterized by a triad of symptoms.
PRUNE BELLY SYNDROME ………..
DIFFERENTIAL DIAGNOSIS OF PRUNE-BELLY SYNDROME.
Megacystis-microcolon-intestinal hypoperistalsis syndrome
Posterior urethral valve syndrome
Ureteropelvic junction obstruction
Bladder exstrophy Urachal cyst Enteric duplication cyst
Ascites
SONOGRAPHIC FINDINGS OF PRUNE-BELLY SYNDROME.
Persistent megacystis Persistent dilatation
of the proximal urethra
Thickening of the bladder wall in the setting of oligohydramnios
D/D AND SONOGRPAHIC FINDINGS
REFERENCE
DIAGNOSTICULTRASOUNDFOURTH EDITIONCarol M. Rumack, MD, FACRJ. William Charboneau, MD, FACRDeborah Levine, MD, FACR