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Baby boy undergoes complex operation to correct Prune Belly Syndrome

31.10.2002

About as complex as it gets—that’s how pediatric urologist Andrew Freedman, M.D., director of pediatric urology at Cedars-Sinai Medical Center’s Endourology Institute, describes the surgery he performed this summer on then 17- month-old Jalen Brown, born with Prune Belly Syndrome. That surgery required reconstructing the toddler’s urinary system in a nearly 10-hour procedure.

Prune Belly Syndrome, also known as Eagle-Barrett Syndrome, is a very rare occurrence: just one in 80,000 births. An estimated 99 percent of those affected are boys. The cause is still unknown, though some theories suggest urinary tract obstructions and resulting distention or developmental defects in utero.

Like Jalen, most born with Prune Belly Syndrome exhibit three main features: 1) an absence of abdominal muscles, contributing to a “wrinkled” appearance of the skin and a “pot belly” profile, 2) dilated urinary tract and associated problems, and 3) intra-abdominal (undescended) testicles.

These trademark characteristics are typically discovered either prenatally via ultrasound or at birth. Associated medical problems—primarily related to renal function—can be life-threatening. “We weren’t sure he was going to make it,” says Dr. Freedman, citing concerns at birth. For Jalen—and most others with the syndrome—survival depends on medical intervention.

An estimated 20 percent of those affected are stillborn, while another 30 percent die within their first two years. The remaining 50 percent suffer from urinary system disorders of varying severity. Another 10 percent face serious cardiovascular problems such as atrial or septal defects. Musculoskeletal problems afflict 20 percent to 50 percent, including hip dislocation, scoliosis and clubfoot, which Jalen was born with and had surgically corrected.

Jalen’s health issues were diagnosed prenatally when an ultrasound at six months picked up problems. “The ultrasound test was taking so long because they were waiting on the baby to pee,” says Laura, also mom to older sister Jasmine, 6. “The next day I got a call saying I needed to see a perinatalogist. I went through a couple of doctors before the problem was officially diagnosed at Cedars-Sinai, where the urologist said it was probably Prune Belly Syndrome.” When she found out, Laura was actually relieved after worrying about even worse conditions. “There was hope,” she explains.

Jalen’s first surgery, performed by Dr. Freedman when the baby was five days old, was a temporary, initial remedy to his renal problems. The surgeon removed obstructions in the left and right ureters and attached them to the skin just below the rib cage. He spent about a month in the hospital, much of that time under the watchful eye of Neonatal Intensive Care Unit staff.

“Urine would drain into his diapers,” explains mom Laura. “Jalen had to wear big diapers to cover the openings, and we had to change them like clockwork. He couldn’t even have a bath.” But the baby rebounded. Despite prenatal concerns about left kidney function, both of Jalen’s kidneys proved to be working at 100 percent.

In June 2002, Jalen underwent the much-anticipated second phase of surgery, scheduled to coincide with the baby’s growth in size and strength.

“When Jalen was about one, we started looking forward to the surgery—he was healthy enough and it would be time for him to walk soon,” says Laura. And walking he is, following the intricate procedure that required Dr. Freedman to reconstruct and reattach the left ureter to the kidney, join the left and right ureters, and then plug the right into the bladder. At the same time, he performed an abdominoplasty to tighten the abdomen and remove loose skin. He also repositioned the testicles.

“Jalen’s in pretty good shape now,” says Dr. Freedman. “He may never be a sit-up champion, but he’ll be able to ride a bike and play Little League.” And that’s enough for Jalen’s family, who are already seeing the fruits of Dr. Freeman’s labors.

After the surgery, Jalen was able to urinate normally, through his penis, for the first time. “It was a beautiful thing,” says his mother of the experience.

Though Jalen’s prognosis is positive, the extent of kidney damage sustained in utero or as a result of infection is unknown. “We’ll see how many nephrons God gave him,” says Dr. Freedman, alluding to the tiny structures within the kidney that control blood composition and renal function. v For now, Jalen wears a “binder” for support, since he lacks abdominal muscles on the right side. When he’s a teen, Jalen can consider other, cosmetic options. “But that’s his decision,” says his mom. “Right now, we just feel very lucky.”

Cedars-Sinai is one of the largest nonprofit academic medical centers in the Western United States. For the fifth straight two-year period, Cedars-Sinai has been named Southern California’s gold standard in health care in an independent survey. It is internationally renowned for its diagnostic and treatment capabilities and its broad spectrum of programs and services, as well as breakthrough biomedical research and superlative medical education. Named one of the 100 "Most Wired" hospitals in health care in 2001, the Medical Center ranks among the top 10 non-university hospitals in the nation for its research activities.

Sandy Van | Cedars-Sinai Public Relations
Further information:
http://www.csmc.edu/

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