Findings -Prune Belly syndrome with Dextrocardia wtih TOF
Also known as Eagle Barret syndrome. This syndrome has derived its name from the wrinkled prune like appearance of the abdominal wall
It a congenital nonhereditary multisystem disorder that occurs almost exclusively in males.
It’s a triad of :
- Abdominal wall muscle deficiency (“ prune belly “)
- Non obstructed markedly distended redundant ureters +/- hydronephrosis and variable degree of renal dysplasia.
- Bilateral undescended testis (cryptorchidism)
Associated abnormalities
GI tract
Anomalies include malrotation with mesenteric defect, imperforate anus, gastroschisis, Hirschsprung disease, and constipation.
Cardiac
Anomalies include ventriculoseptal defect or atrial septal defect or Tetralogy of Fallot in 10% of patients.
Pulmonary
Pulmonary anomalies include hypoplasia resulting from the oligohydramnios, compression from the large abdomen, and Potter syndrome (pneumothorax, pneumomediastinum). Respiratory compromise secondary to abdominal muscular deficiency may be seen. Pneumonia and atelectasis also are observed occasionally.
Extremities
Skin dimples over the patient's joints and lower extremity deficiencies are sometimes seen, such as clubfoot and developmental dysplasia of the hip from fetal crowding by oligohydramnios.
Radiographic findings are as follows:
- Chest: Hypoplastic lungs, flared lower ribs secondary to the distended abdomen are seen
- Abdomen: Diffusely distended flanks are seen. Mass like areas in the abdomen represent a hydronephrotic urinary tract Kidneys: Dilated, clubbed, and dysplastic calyces are seen. Renal parenchyma often is hyperechoic with US and shows poor development and differentiation of the calyces. Often, small cysts may be seen in the subcapsular cortex.
- Ureters: Ureters are markedly dilated and tortuous with involvement more severe distally. Usually, refluxing ureters are seen.
- Bladder: The bladder is vertical and trabeculated, with a urachal remnant at the dome. The base of the bladder is triangular with a relaxed internal sphincter
- Urethra: A wide and long posterior urethra is seen with a utricular remnant. The posterior urethra may be triangular in appearance secondary to the absence of the posterior lobe of the prostate. Corporal absence can result in megalourethra of the anterior urethra, rendering it fusiform in appearance).
- Cryptorchidism: The testes are in the abdomen or inguinal canals. Often, the testes appear hypoplastic, although they may be normal size.