Five-Year-Old Boy Becomes First in World to Undergo Minimally Invasive Procedure
Keisha Bishop went to her routine ultrasound appointment during her twelfth week of pregnancy filled with excitement at the thought of seeing her unborn baby for the first time. She recalls marveling at seeing her baby suck his thumb. But very quickly after that, she knew that something was wrong with her baby.
"Nobody was saying anything at first and then the nurse practitioner performing the exam brought high-risk obstetricians in to study the screen," Bishop said.
One of the physicians brought in to examine Bishop's baby was former University of Chicago obstetrician and maternal-fetal specialist, Judith Hibbard, MD, who told her that the ultrasound revealed an alarming abnormality.
"Dr. Hibbard showed me on the monitor a huge space of fluid in the middle of his bladder and there was urine all around his heart and compressing all of his organs because his kidneys weren't functioning properly," Bishop says. "She explained all of the options to me and cautioned me that babies with such severe kidney dysfunction often don't live through pregnancy or die shortly after birth."
Bishop was determined to see her pregnancy through and decided to maintain a positive attitude, turning to prayer and meditation for strength.
In order to home in on the cause of the excess urine, a condition known as hydronephrosis, Hibbard performed a percutaneous abdominal aspiration. Using a long needle inserted through Bishop's abdomen and into her baby's abdomen, Hibbard drew out as much urine as possible. In addition to retrieving a sample for pathology testing, the procedure also removed enough fluid to achieve decompression of the baby's organs.
The pathology testing didn't reveal the cause of the hydronephrosis and when Bishop returned for an ultrasound only a week later, enough fluid had returned that Hibbard decided to perform another aspiration. This time, she was able to remove nearly all of the fluid and a week later, an ultrasound exam showed that the baby's kidneys were doing better.
Bishop was monitored with ultrasounds every week for the rest of her pregnancy. When she reached full-term, her labor was medically induced and she had a normal delivery at the University of Chicago.
"When he came out, he peed on everybody and we all laughed a little in relief," Bishop said.
Bishop's baby, who she named Graham, was diagnosed almost immediately with Prune Belly Syndrome, a very rare condition that occurs in about 1 out of 100,000 males. Babies with Prune Belly Syndrome usually have distended abdomens as a result of deficient or absent abdominal wall musculature, urinary tract anomalies and undescended testicles. Graham also had clubbed feet.
"Babies with Prune Belly Syndrome suffer from a host of medical problems because of the characteristics of their disease," says Mohan Gundeti, MD, director of pediatric urology. "Because they have no abdominal muscles, they can't breathe well and are prone to chest infections and are unable to empty their bladders, putting them at risk for recurrent urinary tract infections."
Graham was only two months old when Christopher Sullivan, MD, associate professor of surgery at the University of Chicago and an expert in pediatric muscle and skeletal disorders, performed a surgery to correct Ahmad's clubbed feet. At six months old, a pediatric urologist who was Gundeti's predecessor at the University of Chicago Medicine performed a circumcision and brought down his testicles in a procedure that took eight hours.
While Graham never suffered a chest infection, he required catheterization four to five times each day to empty his bladder. By the time he was five years old, he was experiencing recurrent urinary tract infections (UTI) and had been hospitalized for a related blood infection.
When preventive use of antibiotics administered daily failed to prevent the UTIs from returning, Gundeti told Bishop about a surgical procedure, showing her video footage and providing her with as much information as he could to help her understand the disease. Bishop says the procedure gave her hope that her son would have a more normal childhood and she signed Graham up for surgery.
"Graham's kidneys were showing evidence of scarring because of the infections," Gundeti said. "The surgical option that I proposed has been performed for other conditions but always through large incisions in an open approach."
For Graham, Gundeti asked Bishop if she would be amenable to consenting to a minimally invasive robotic approach to spare Graham some of the cosmetic and recovery challenges that go along with the more traditional approach.
"Dr. Gundeti's warmth, sincerity and concern for Graham's well-being always were front and center of all our discussions about his treatment and I trusted him implicitly," Bishop said. "So I didn't really have many reservations about letting him use a minimally-invasive approach to perform the procedure. And the thought of my son being able to wear underwear like a normal little boy outweighed any reservations I might have had."
As such, Graham became the first patient in the world to undergo the procedure laparoscopically.
During the five-hour surgery, Gundeti took Graham's appendix, which is a hollow muscular tube with an intact blood supply, detached it from the cecum and joined it to one end of the bladder, bringing the other end through an opening, or stoma, in the abdominal wall. This conduit is supported by the bladder muscle so when the bladder is full, there is no leakage. Although Graham would have to learn to catheterize the stoma, he would be fully continent.
Because of the expertise in laparoscopic and robotic surgery, the University of Chicago Medicine has been performing more and more complex cases, Gundeti said. "While Graham's case was particularly challenging because of his anatomy, we felt the benefits of using minimally invasive approaches more than justified our taking on the challenge."
Graham quickly learned how to catheterize himself. The six-year-old recently graduated from kindergarten. He takes the bus to school and participates in most of the activities a boy his age would, although he wears a brace to protect his vulnerable abdomen.
"I thank God for Dr. Gundeti," said Bishop. "Early on, I had a hard time finding enough reliable, accurate information about Prune Belly Syndrome. When I met Dr. Gundeti, and realized that I had an expert available to us whose main focus was improving the quality of life for my son, I was overjoyed. Every time I see him, I am struck by how much he loves what he does."