Fetal anomaly series prune belly syndrome

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