Prune Belly Syndrome Workup

  • Author: Israel Franco, MD, FAAP, FACS; Chief Editor: Bradley Fields Schwartz, DO, FACS   more...
 
Updated: Jan 23, 2012
 

Laboratory Studies

  • Obtain a sequential multiple analysis (SMA-6; ie, 6 different serum tests and creatinine tests to evaluate renal function in a serial fashion) because many children with prune belly syndrome may have renal compromise.
  • Urinary output and electrolytes must be carefully observed in newborns. A rise in BUN and creatinine levels associated with decreased urine output indicates obstruction.
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Imaging Studies

  • Careful radiologic evaluation is essential before committing to any type of diverting procedure.
  • Radiologic studies should be performed in the newborn period, regardless of whether obstruction is present.
  • Kidney, ureter, and bladder (KUB) test findings can usually be used for diagnosis based on the typical appearance of the bowels hanging over the lateral edge of the abdominal wall.
  • The first study that should be performed is renal and bladder ultrasonography, which is a noninvasive procedure and can be used later to noninvasively monitor the child's progress.
  • Perform ultrasonography of the kidneys as early as possible to evaluate the upper tracts.
  • Renal scan is necessary after renal function stabilizes to evaluate renal function and drainage.
  • Contrast voiding cystourethrography (VCUG) should be performed.
    • VCUG is used to delineate the prostate-membranous urethra and the bladder and to detect the presence of an urachal remnant. In addition, the patient is evaluated for vesicoureteral reflux. Patients with vesicoureteral reflux are placed on antibiotic prophylaxis.
    • Upon evidence of upper-tract dilatation or obstruction, a renal Hippuran or diethylenetriamine pentaacetic acid (DTPA) study should be performed.
    • Children with only megaureter, megacystis, and the prostatic abnormalities who have no evidence of obstruction or reflux can be managed conservatively with close observation.
    • VCUG is necessary to evaluate the bladder size. In addition, urethral stenosis must be ruled out immediately. The presence of a patent urachus is a hint that stenosis is present. The degree and extent of reflux is assessed with this study.
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Contributor Information and Disclosures
Author

Israel Franco, MD, FAAP, FACS  Associate Professor, Department of Urology, Division of Pediatric Urology, New York Medical College; Director of Pediatric Urology, Lincoln Hospital and Medical Center

Israel Franco, MD, FAAP, FACS is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Urological Association, Endourological Society, International Pediatric Endosurgery Group, Medical Society of the State of New York, and Society of Laparoendoscopic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Peter Langenstroer, MD  Associate Professor, Department of Urology, Medical College of Wisconsin

Peter Langenstroer, MD is a member of the following medical societies: American Urological Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Shlomo Raz, MD  Professor, Department of Surgery, Division of Urology, University of California at Los Angeles School of Medicine

Shlomo Raz, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Urological Association, and California Medical Association

Disclosure: Nothing to disclose.

J Stuart Wolf Jr, MD, FACS  The David A Bloom Professor of Urology, Director, Division of Endourology and Stone Disease, Department of Urology, University of Michigan Medical School

J Stuart Wolf Jr, MD, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Catholic Medical Association, Endourological Society, Society for Urology and Engineering, Society of Laparoendoscopic Surgeons, Society of University Urologists, and Society of Urologic Oncology

Disclosure: Nothing to disclose.

Chief Editor

Bradley Fields Schwartz, DO, FACS  Professor of Urology, Director, Center for Laparoscopy and Endourology, Department of Surgery, Southern Illinois University School of Medicine

Bradley Fields Schwartz, DO, FACS is a member of the following medical societies: American College of Surgeons, American Urological Association, Association of Military Osteopathic Physicians and Surgeons, Endourological Society, Society of Laparoendoscopic Surgeons, and Society of University Urologists

Disclosure: Nothing to disclose.

References
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Abnormal bladder in a patient with prune belly syndrome. Note the large size of the bladder and that the most anterior portion flops over the pubic symphysis, leading to partial obstruction.
High-grade reflux and dilated posterior urethra, which are diagnostic of prune belly syndrome.
Child with severe scoliosis and prune-belly abdominal wall: This is the same child with the abnormal bladder in Images 1 and 2.
Laparoscopic view of intra-abdominal testis.
Ten-year-old boy 1 week after abdominal wall reconstruction.
Lateral view of patient with prune belly syndrome.
Laparoscopic abdominal wall plication with the excess tissue plicated and the laparoscope in the abdomen.
The lateral subcostal weakness in this patient is due to absence of the internal and external obliques subcostally.
Laparoscopic view of the fascial defect transilluminated from outside the abdomen. Note that the defect is just a thin layer of attenuated fascia.
Note that the previously attenuated fascial defect has been covered by reapproximated muscle.
Bilateral subcostal incisions exposed the fascial defects.
 
 
 
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