oshiba prune belly syndrome.pptx

prune belly syndrome

1. Prune belly syndrome

2. Triad of bilateral cryptorchidism, deficiency of the abdominal wall musculature, and a dilated, dysmorphic urinary tract. William Osler is credited with giving the syndrome its name “prune belly” in 1901 Eagle-Barrett syndrome, urethral obstruction malformation complex, the triad syndrome, abdominal muscle deficiency syndrome, and mesenchymal dysplasia syndrome. Prune belly morphology is not confined to the male gender. Females constitute about 3%–5% (pseudo prune). 1/29,000 to 1/40,000 live births. The majority are sporadic.

3. Classification

4. Theories Fetal Outlet Obstruction. Theory of Mesodermal Arrest. The Yolk Sac Theory.

5. Fetal outlet obstruction

6. Theory of Mesodermal Arrest

7. Clinical features

8. Extra genitourinary manifestations

9. Abdominal wall Wrinkled, floppy abdominal wall, deficiency of underlying muscles, difficult sitting, difficult walking, chronic constipation, respiratory problems. Ventral and lateral muscles: transversus abdominis, rectus abdominis below the umbilicus, internal oblique, external oblique and rectus abdominis above the umbilicus.

10. Genitourinary symptoms

11. kidney Renal dysplasia, hydronephrosis. Renal dysplasia of the prune belly syndrome is due to a combination of a ureteric bud and metanephric defect. The renal parenchyma is often well preserved despite a grossly abnormal drainage system. Urinary infection rather than obstruction represents the greatest threat to the renal parenchyma.

12. Ureters Elongated, tortuous, and dilated. The lower one third of the ureter is more profoundly affected than the proximal portion. VUR: up to 85% of patients The upper ureter is potentially best suited for definitive reconstruction, so no ureterostomy to be done.

13. Bladder Thick walled and grossly enlarged, absent trabeculations. A patent urachus with urethral atresia or microurethra . Splayed trigone. Laterally separated UO. Wide BN. Efficient low-pressure storage and good compliance.

14. Posterior urethra Posterior urethra is dilated, elongated, and tapered at the membranous urethra. Utricular diverticulum. Vas deferential reflux and small or absent verumontanum. True obstructive lesions at the junction of the prostate and membranous urethra have been described in 20% of infants. Stenosis, true valves, atresia, diaphragms, and diverticula. Prostatic hypoplasia is one of the etiologies of infertility in this syndrome.

15. Anterior urethra Urethral atresia, megalourethra, Surviving patients with urethral atresia or microurethra have a patent urachus. Scaphoid and fusiform megalourethra. Fusiform type is the worst.

16. Testis Bilateral cryptorchidism intra-abdominal, overlying the ectatic ureters at the pelvic inlet. Risk of malignancy: 30–50 times. The gubernaculum is normally attached proximally to the tail of the epididymis, travels via the inguinal canal, and attaches distally at the pubic tubercle. Vas deferens obstruction and epididymal abnormalities associated with maldescent affect sperm delivery and maturation.

17. Management

18. Prenatal evaluation As early as 11 weeks of gestation on ultrasound. Difficult to distinguish from other causes of obstructive uropathy like PUV. In utero intervention ????

19. Neonatal evaluation Abdominal wall appearance is diagnostic. Cardiac and pulmonary evaluation is a must. X - ray to rule out pneumothorax and pneumomediastinum. 48-72 hours serum creatinine, if >1 in term infant or >1.5 in preterm >>>> indicate renal insufficiency. Starting chemoprophylaxis.

20. Investigations Serum electrolytes. serum creatinine. Urine analysis and culture. Renal and bladder sonogram. VCUG. MAG 3 renogram vs DMSA. MRU.

21. Initial management Watchful waiting to immediate surgical reconstruction of the urinary tract. Early surgical intervention in the neonate is avoided, unless a rising creatinine or infection occurs, requiring early vesicostomy. Category I: oligohydramnios, pulmonary hypoplasia, usually die , if passes need for high urinary diversion like ureterostomy, pyelostomy . Category II: if urinary tract infection, vesicostomy, urinary tract reconstruction, orchiopexy, abdominoplasty is preferred before the age of 1 year. Category III: chemoprophylaxis, usually no need for urinary reconstruction, urodynamics, early orchiopexy and abdominoplasty improves the voiding and defection mechanism.

22. In utero intervention Vesicoamniotic shunting in patients with suspected bladder outlet obstruction does not improve renal outcomes but would assist in pulmonary maturation and function.

23. Anesthetic consideration Careful preoperative pulmonary assessment. An antecedent history of recurrent respiratory infections warrants aggressive physiotherapy, postural drainage, and intermittent positive pressure breathing treatments. Specific antibiotic therapy. Postoperative analgesics.

24. Vesicourethral dysfunction Internal urethrotomy: trans urethral incision Assessment of lower urinary tract dynamics, routine urinary flow rates, and the amount of residual urine. If urinary tract infection or deteriorating renal function in the perinatal period , a cutaneous vesicostomy is the drainage procedure of choice.

25. Reduction cystoplasty Bladder volume: 3 L. Removal of a dilated urachal diverticulum. Some authors do not recommend reduction cystoplasty in light of the effective and safe application of clean intermittent catheterization except for the removal of a urachal pseudodiverticulum.

26. Anterior urethra Micro urethra: need for regular dilatation and hydrodistension. Megalourethra: hypospadias repair.

27. Upper urinary tract VUR 85%. Vesicoureteral obstruction. Ureteral valve. Decreasing renal function, urinary tract infection. Reduction and reimplantation.

28. Orchiopexy Open or laparoscopic. Early before the age of 1 year. Microvascular testicular auto transplantation , The spermatic vessels are anastomosed to the inferior epigastric vessels, with microsurgical vascular techniques.

29. Abdominal wall reconstruction Improve voiding, defecation and sensation of bladder fullness. Preservation of the lateral and upper parts of the abdominal wall, the sites of most normal musculature. Ehrlich and Monfort techniques: Abdominal wall reconstruction, utilizing an ellipsoid midline incision with extensive subcutaneous dissection, and a pants over vest closure. This technique preserves the umbilicus, uses the full thickness of abdominal wall, and provides narrowing at the waist. Randolph technique.

30. Comprehensive reconstruction Reduction cystoplasty, resection of the distal ureter and bilateral ureteral tapered reimplantation. Combined with the performance of the abdominoplasty and bilateral orchiopexy, before the age of 2 years. Antibiotic prophylaxis.

31. Renal transplantation Bilateral nephroureterectomies . Urodynamic assessment of the lower urinary tract is recommended to insure absence of obstruction and balanced voiding. The use of CIC to empty the decompensated bladder is not a contraindication to renal transplantation. Antibiotic prophylaxis. Allograft torsion is an unusual complication, graft loss, was a result of lack of abdominal wall tone, Nephropexy is recommended.