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Extraordinary Outcome for Devastating LUTO Case
Before Luke Vela was even born, his big brother nicknamed him Tuff because of the challenges he’d already overcome.
At 18 weeks gestation, Luke was diagnosed with a fetal congenital lower urinary tract obstruction (LUTO) that was so severe his bladder took up the entire uterine cavity. It pressed against his heart, causing cardiac distress and decreasing amniotic fluid to a dangerously low level. The condition required Luke to undergo three fetal surgeries and one shortly after birth.
In the first procedure, a multidisciplinary team of surgeons performed an ultrasound-guided vesicocentesis (fetal bladder tap) and drained 700 ccs of fetal urine, alleviating the pressure inside Luke’s bladder.
“We then performed a fetal cystoscopy and diagnosed a possible urethral atresia (complete obstruction of the urethra),” said Jimmy Espinoza, MD, PhD, Luke’s fetal surgeon. “We placed a fetal vesicoamniotic shunt, which improved the amount of amniotic fluid and decreased the pressure inside Luke’s renal system."
As Luke grew inside the womb, his shunt had to be surgically replaced twice.
At 34 weeks, Luke was born by cesarean section and immediately taken to the NICU. Postnatal examination confirmed a completely obstructed urethra that was impossible to catheterize, necessitating a vesicostomy at 4 days old to protect the kidneys from further damage. However, he was found to have normal renal function.
After Luke spent about one month in the NICU, his family was finally able to take him home.
In utero, Luke developed prune belly features, or a flaccid abdominal wall.
“Many physicians confuse this with prune belly syndrome, a genetic disease in which a primary muscular weakness causes a flaccid abdominal wall without bladder outlet obstruction,” Dr. Espinoza said.
“Luke’s case is one for the history books,” said Dr. Espinoza. “Survival with normal renal function in such a severe case of urethral atresia is extremely rare.”
Despite Luke’s challenges, including chronic kidney disease, his mother Kate remains positive.
“Luke is our miracle baby,” she says. “He is thriving and continues to meet every physical and cognitive milestone. We still have a long road ahead of us because he will need many reconstructive surgeries and there are many unknown variables. But every time I see him smile, I know that we made the best possible choices, and I would do it all over again.”
Three years after his brother first called him Tuff, he continues to live up to the name.
“Luke has enormous strength and will to live,” Kate said. “He wanted to be here in this world and was as determined as we were not to give up.”
Fetal Surgery for LUTO
Bladder outlet obstruction, a type of lower urinary tract obstruction, is an uncommon birth defect that occurs most often in males.
The flow of urine from the urethra is blocked, causing urine to back up and enlarge the bladder. This lessens the amount of amniotic fluid and sets up the fetus for a host of possible problems, including lung and heart problems, kidney damage and deformities of the face and extremities. It usually is diagnosed by routine ultrasound in the second trimester of pregnancy.
If the defect is severe, a vesicoamniotic shunt may be necessary. Texas Children’s Fetal Center is one of the nation’s leaders in performing this delicate and complex procedure.
Guided by ultrasound, surgeons place a small shunt (catheter) from the fetal bladder to the amniotic cavity. The objective is to bypass the bladder outlet obstruction, releasing the pressure inside the bladder and the renal system as well as increasing the amount of amniotic fluid.
This procedure is indicated for fetuses with complete bladder outlet obstruction whose odds of survival are less than 10 percent (a 90 percent chance of end stage renal disease) without fetal intervention.
At Texas Children’s, the one-year survival rate after fetal vesicoamniotic shunt placement is 79 percent.
“Our success is based on a prenatal multidisciplinary evaluation that helps us plan the best prenatal treatment and management, the best moment to deliver, and the best postnatal therapy and follow-up,” said Jimmy Espinoza, MD, PhD, co-director of Texas Children’s Fetal Center. We have the most up-to-date equipment and technology, and we have developed:
- Protocols to treat only fetuses that need and will benefit from this therapy
- Algorithms based on our experiences and literature studies
- A staging system to classify cases according to severity of the disease