Extraordinary Care Meets Extraordinary Results in the Lone Star State

from Connexions Spring 2019

Preeclampsia + How it Snuck In

extraordinary care meets extraordinary results in the lone star state

editorial submitted by friend steward partners at Texas Children's Fetal Center

Before Luke Vela was even born, his older brother nicknamed him Tuff because of the many 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 Velas were referred to Texas Children’s Fetal Center®, located within Texas Children’s Pavilion for Women in Houston, Texas, and part of Texas Children’s Hospital, one of the nation’s largest children’s hospitals. Texas Children’s Fetal Center is a national leader in LUTO interventions, including vesicoamniotic shunts, a delicate and complex procedure whereby a surgeon connects a catheter from the fetal bladder to the amniotic cavity to bypass the bladder outlet obstruction.

“When we were first told about Luke’s condition, it felt like being hit by a bus,” Luke’s father Josh Vela said. “As soon as we met with the surgeons at Texas Children’s Fetal Center, there was a newfound hope in us. I remember that within minutes of making the decision to do the surgery, we had every expert you can think of, urology, nephrology, cardiology, coming in and talking to us about what to expect.”

A unique and distinct advantage for all mothers delivering at Texas Children’s Pavilion for Women is its location inside of one of the largest and most renowned children’s hospitals in the world. This means no risk of transfer during critical postnatal periods – if a baby unexpectedly needs a surgery or the highest level of NICU care, they’re already in the best place to receive the best care. And for Texas Children’s Fetal Center patients, it means that the full range of pediatric experts responsible for treating a child after birth are an important part of the care team prior to birth as well.

“When you have a complex patient like Luke, you quickly discover you have many doctors involved,” said Dr. Jimmy Espinoza, Luke’s fetal surgeon. “Not only do you need the best maternalfetal medicine specialists and fetal surgeons, you also need the best pediatric surgeons and pediatric subspecialists who will provide care for the patient long after he or she is born. That makes the list of centers that can offer this sort of care very, very small.”

Pioneering treatments that bring new hope Advances in genetics, diagnostic technology, fetal medicine and fetal surgery mean that early detection of fetal problems and anomalies can often be met with equally sophisticated treatments. Such innovation has helped to change the emotions around a complex pregnancy from those of fear to those of hope.

Since 2001, Texas Children’s Fetal Center has been at the forefront of developing new fetal procedures to treat devastating conditions like lower urinary tract obstruction, congenital diaphragmatic hernia, spina bifida, congenital heart defects, twin-twin transfusion syndrome, and more. Today it is one of only a few centers in the United States to offer the full spectrum of fetal therapies to patients. The center is known for pioneering its own advanced treatments, including EXIT-to-resection procedures and fetoscopic versions of standard open fetal surgeries designed to reduce risk and impact on both mother and baby.

Conditions treated include: abdominal wall defects, amniotic band syndrome, central nervous system lesions, congenital

airway obstructions, congenital diaphragmatic hernia, congenital heart disease, craniofacial abnormalities, duodenal and intestinal atresia, esophageal atresia, fetal cardiac conditions, fetal chylothorax, giant neck masses, lung lesions, maternal immune disorders, myelomeningocele, nonimmune hydrops, small bowel obstruction, sacrococcygeal teratoma, skeletal dysplasia, twin abnormalities and urinary tract obstructions.

Dedicated support for every family Texas Children’s Fetal Center believes that caring for both mothers and unborn infant(s)

cannot be separated from caring for the patient’s whole family. Their treatment model is designed around giving every family enhanced access to

experts at every level, and around explanations of tests, procedures, prognoses, treatment options, details of recovery, and possible outcomes for every situation.

Every family that comes to Texas Children’s Fetal Center for specialized treatment is paired with a clinical coordinator, highly trained in meeting the needs of patients with fetal anomalies. The coordinator is available to patients and their families from the time of arrival until treatment is complete. This service provides patients and families with a direct link to medical information, insight and explanation, and, equally important, a familiar face to serve as a source of support during treatment.

In addition, patients remark about the extraordinary access and level of responsiveness

Luke’s case is one for the history books. Survival with normal renal function in such a severe case of LUTO is extremely rare. – Jimmy Espinoza, MD, PhD, fetal surgeon

of their fetal surgeons, often receiving immediate call backs, even in the middle of the night whenever there was a development in their case. The center is staffed and supported for surgical or medical intervention 24/7, 365 days a year, with senior physicians always available to discuss diagnoses and care plans with patients or their providers. ~~~~~~~~~~~~~~~~~~

In the end, Tuff proved to be a remarkably fitting and prescient nickname for Luke. During his first fetal surgery, he was diagnosed with complete obstruction of the urethra, a rare and severe case of LUTO associated with an expected survival rate of under 10 percent. A multidisciplinary team of surgeons performed an ultrasound-guided fetal bladder tap to alleviate the pressure inside Luke’s bladder, and then placed his vesicoamniotic shunt.

The surgery was a success, but Luke and Kate had to undergo the procedure twice in order to meet the demands of Luke’s growth. At 34 weeks, Luke was delivered via planned cesarean section at Texas Children’s Pavilion for Women.

His completely obstructed urethra made it impossible to catheterize him, so Luke

underwent a vesicotomy at four days old. Afterwards and remarkably, he was found to have normal renal function.

Over 5 years old now, Luke has some challenges, including chro kidney disease, but he continues to live up to his reputation.

“He is thriving and meeting every physical and cognitive milestone,” Kate said. “There are probably more surgeries in our future, but every time I see him smile, I know that we made the best possible choices, and I would do it all over again.”

It’s with great pride and privilege that Texas Children’s Fetal Center cares for patients like the Vela family, delivering the highest-quality care across the full spectrum of needs, for the best possible outcomes.

To learn more about Texas Children’s Fetal Center, please visit women.texas childrens.org/fetal or call 832-822-BABY (2229) with any questions you may have.