May 20, 2014

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Eva and Jay Heintz were enjoying a blissfully uneventful twin pregnancy in Hammond, LA, when their world was turned upside down at Eva’s 19-week check-up.

Her MFM specialist detected polyhydramnios in one twin’s sac and oligohydramnios in the other twin’s sac and quickly made the diagnosis of Twin-Twin Transfusion Syndrome (TTTS). From there, the bad news kept coming. Even though Eva’s ultrasound had been normal just two weeks earlier, the disease had progressed rapidly to an advanced stage of TTTS, (Stage 3) which involved cardiac Doppler abnormalities with selective intrauterine growth restriction. Action needed to be taken quickly if there was any hope of saving Eva’s twins.

Her MFM called Texas Children’s Fetal Center with a referral, and by the time she arrived home from her doctor’s appointment, Jayme Molohon, the nurse coordinator at Texas Children’s Fetal Center who received Eva’s case, had already started to make arrangements for Eva to be seen in Texas the next day.

“The nurses simply could not have been more warm and comforting as I prepared for my surgery,” said Eva.

“Once a referral comes through the Fetal Center, we try to get these patients in as quickly as possible due to the nature of the disease,” said Molohon. My relationship with the family starts with the first phone call I make to introduce myself. You can almost feel their anxiety and fear through the phone because you know they just want someone to help their child. My job is to make these families and patients as comfortable as possible and see that they understand their reasoning for coming all the way to Houston.”

But her role as a nurse coordinator extends far beyond that first call. Prepping for surgery involves a comprehensive initial evaluation. Patients like Eva start with an initial anatomy ultrasound, fetal echocardiogram, genetic counseling MFM consultation and fetal intervention consultation. The entire team on the fourth floor of the Pavilion for Women is a part of the patient’s evaluation. If babies meet criteria for surgery, the patient will meet with pre-anesthesia testing and possibly neonatology, if of viable gestational age. The nurse coordinator is then in charge of scheduling all appointments, verifying insurance authorization and also assisting in housing, if the patient has limited means. She also reviews all prenatal records and obtains any additional records depending on maternal conditions. And according to Molohon, one of her most important roles is to offer the family hope and the knowledge that the Fetal Center staff will fight for them.

“I am with the patient and their family every step of the way,” she said. “I develop relationships with patients that go beyond the fetal surgery. All my patients touch my heart in some shape or form. We experience a bond that can never be shared with anyone else. You may not be related to them by blood, but they will never forget how much of an impact you made in their lives and their children’s lives.

Eva underwent laser ablation surgery led by Dr. Alireza Shamshirsaz – a procedure that is still rare in other hospitals, but has become routine at Texas Children’s where the Fetal Center sees TTTS cases every week. Eva’s surgery was a success, but she still wasn’t in the clear.

“The families that go through fetal intervention always understand there is a risk that their children may not make it after surgery,” said Molohon.

After the procedure, doctors explained that the next important step would be surviving the night after surgery. At 6 a.m. the next morning, a team of doctors entered Eva’s room for the important evaluation – and looks of joy were exchanged as they found two heartbeats.

“When I found out that both babies were alive and made it through surgery, I said a prayer,” said Molohon. “Life is so precious and you always want the best possible outcome for these patients. Eva was such an amazing patient and wanted to do everything right to make sure her babies were okay. To see pictures of her twins now and how beautiful they are makes you appreciate all the hard work and dedication that we do every day in the Fetal Center.”

Eva’s story is a prime example of the high level of care that goes into TTTS cases at Texas Children’s Fetal Center.

“I saw and felt what it is like to have doctors invest themselves in, not only your outcome, but in your future and the future of your family, said Eva. “It felt so personal and special, and I will never forget that feeling. Through all the exhaustion and ultimately joy that this experience has brought me, I often reflect of my time at Texas Children’s Hospital.”

April 15, 2014

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The Texas Children’s Fetal Center proudly announces the birth of baby Cabellotrejo, a boy born healthy at Texas Children’s Pavilion for Women following a complex pregnancy that included open fetal surgery. Early in the pregnancy, doctors diagnosed baby boy Cabellotrejo with a large lung mass, called congenital cystic adenomatoid malformation, or CCAM. Mom and baby were transferred from their hometown of Austin, where the team at the Fetal Center intervened with an open fetal surgery that saved the baby’s life.

During the procedure, which took place on January 16, 2014, the Fetal Center team partially removed the baby from her mother’s womb, opened the baby’s chest and removed the giant mass, returning him safely back into his mother’s womb less than 30 minutes later. The fetal heart failure resolved, and the baby and mother subsequently recovered smoothly; mom remained pregnant for 11(+) weeks before she delivered her health baby boy.

A CCAM is an abnormal growth of malformed lung tissue that is the result of abnormal organ development. The adenomatous overgrowth of terminal bronchioles and reduced number of normal alveoli may cause significant pulmonary effects. It is incredibly rare for these malformations to grow to such a large size as to lead to fetal heart failure, a condition that is very difficult to treat prenatally. The fetus continued to deteriorate despite medical treatment.

“Fetal surgery was the only hope for this baby boy who was sure to die without surgical intervention. The good outcome achieved in this case is the result of great teamwork, including the contributions by our expert fetal radiologists, cardiologists and maternal fetal surgery team. I am so pleased this baby has recovered fully and now has the hope of a completely normal life,” said Dr. Cass, co-director of Texas Children’s Fetal Center, and lead surgeon on this case.

In addition to an expert OR team, nurse coordinators and pediatric anesthesiologists, a multidisciplinary team of specialists, including: Dr. Darrell Cass, Dr. Oluyinka O. Olutoye, Dr. Wesley Lee, Dr. Michael Belfort, Dr. Nancy Ayres, Dr. Rodrigo Ruano, Dr. Christopher Cassady and Dr. Alireza Shamshirsaz worked together on the case.

To date, only two other centers in the world have been successful at treating this rare and complex medical condition. Texas Children’s Hospital has performed 59 open fetal surgeries since 2003, but seeing that healthy baby enter the world never gets old.

“Baby Cabellotrejo is a fighter and a survivor. We are ecstatic that his parents have delivered a healthy baby boy,” said Cass, of the Fetal Center’s latest patient success.

April 1, 2014

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On February 23, 2012, the Fetal Center announced the birth of baby Charlotte, the first baby at Texas Children’s to receive in-utero surgery to treat spina bifida. Now, a little more than two years later, the center is celebrating another milestone with 20 cases completed.

After years of treating spina bifida by a neurosurgical closure of the spine defect during the first days of life, research breakthroughs prompted the Texas Children’s Fetal Center team to begin performing in-utero surgery on patients with a prenatal diagnosis of spina bifida.

Myelomeningocele, also known as spina bifida or open neural tube defect (NTD), is a developmental defect in which the spine is improperly formed and the spinal cord is open to and fused with the skin; it is usually associated with hydrocephalus, which requires surgical treatment to drain the fluid via a shunt. Myelomeningocele occurs in 3.4 out of every 10,000 live births in the U.S. and is the most common permanently disabling birth defect for which there is no known cure.

Advancements in fetal imaging and early prenatal diagnosis allow the team to gain access to a fetus while still inside a mother’s womb. Texas Children’s Fetal Center has developed extensive screening and diagnostic algorithms for pregnancies with fetal spina bifida. These algorithms determine which patients are appropriate candidates for the in-utero surgery.

The surgery is a huge, multi-disciplinary effort, bringing together a team of specialized experts, who all play a vital role in the medical treatment of both mom and baby. The team includes maternal-fetal specialists, led by Dr. Michael Belfort, fetal surgeons, led by Dr. Darrell Cass and Dr. Oluyinka Olutoye, and a neurosurgery team, led by Dr. William Whitehead. Once the fetus is exposed, Dr. Whitehead and his team surgically close the spinal defect before fetal surgeons return the baby to the womb. This procedure reduces the risk of hydrocephalus and may improve motor function in select patients.

A NICHD-funded study entitled the Management of Myelomeningocele Study (MOMS) published in the New England Journal of Medicine demonstrated a significant decrease in the risk of hydrocephalus for select patients undergoing fetal closure of the spine, as well as possible improvement in lower extremity function, compared to patients who underwent standard closure after birth. The MOMS trial is the second fetal intervention that has proved beneficial through a multi-center randomized clinical trial. The first was the Euro FETUS trial for laser ablation in the treatment of twin-to-twin transfusion syndrome (TTTS).

“Breakthrough studies like the MOMS trial are exciting and reaffirm our commitment to advancing fetal medicine and giving babies with complications and anomalies the healthiest possible start to life,” said Dr. Darrell Cass, co-director of Texas Children’s Fetal Center. “Our program has grown exponentially since we completed our first NTD repair in 2012, and we remain cautious, but it seems as though our center’s results are exceeding even those of the trial.”

The final piece to this puzzle is Dr. Kathryn Ostermaier, clinic chief of the Spina Bifida Clinic. It is under her guidance that the hospital provides long term care to surgical patients. The hospital’s Spina Bifida Clinic is a specialized program that serves only those patients with spina bifida, and includes several specialty services: orthopedics, neurosurgery, pediatrics, physical medicine and rehabilitation and urology.

The team that cares for NTD patients before birth, after birth and into childhood includes physicians from maternal fetal medicine, pediatric surgery, neurosurgery, anesthesiology, neonatology, pediatric radiology, cardiology, orthopedics, urology, physical therapy, and a highly dedicated group of specialized nurses, ultrasound technologists and genetic counselors.

“The confirmation that fetal surgery may decrease the physical challenges some of these babies face is not only a ray of hope for families, it is also a significant achievement for fetal medicine,” said Cass.