Cleveland Clinic, one of the largest and most respected multispecialty hospitals in the US, has successfully performed its first in-utero foetal surgery to repair a spina bifida birth defect in a nearly 23-week-old foetus.
A multispecialty team of clinicians performed the surgery in February, and the baby, a girl, was later delivered by caesarean section near full term on June 3, making it northern Ohio’s first surgery of its kind. Mother and daughter are doing well, according to a statement by the hospital.
The surgical team, led by Dr Darrell Cass, MD, director of Foetal Surgery in Cleveland Clinic’s Foetal Centre and a specialist who has performed more than 160 foetal surgeries since 2002, included Dr Amanda Kalan, MD, medical director of Cleveland Clinic’s Special Delivery Unit; Dr Violette Recinos, MD, and Dr Kaine Onwuzulike, MD, both paediatric neurosurgeons; Dr Francine Erenberg, MD, foetal cardiologist; and Dr McCallum Hoyt, MD and Dr Tara Hata, MD, obstetric and paediatric anaesthesiologists.
Spina bifida is a birth defect that is most often discovered during the routine anatomy scan typically performed when a foetus is around 18 weeks old. The condition affects the lowest part of the spine and occurs when the neural tube does not fully close, causing the backbone that protects the spinal cord not to form as it should. This often results in damage to the spinal cord and nerves and can even lead to brain damage.
Spina bifida can affect a child’s lower leg strength and their ability to walk and run, as well as their ability to go to the bathroom and urinate adequately. According to the CDC, approximately 1,645 babies are born with spina bifida each year in the United States.
An animation of the in-utero foetal surgery to repair a spina bifida birth defect.
During the foetal repair surgery, a caesarean section-like incision is made and the mother’s uterus is exposed. An ultrasound is then used to locate the placenta and foetus. The uterus is opened 4.5 cm and the back of the foetus is exposed, showing the spina bifida lesion. The surgeons then carefully suture several individual layers of tissue (myofascia, dura and skin) in order to cover the defect. After the uterus is closed back up, the foetus remains in the womb for the remainder of the pregnancy and is ultimately born by caesarean section.
“By successfully repairing the defect before birth, we’re allowing this child to have the best possible outcome and significantly improve her quality of life,” said Dr Cass. “There are different measures of quality in determining success for foetal repairs and in this particular case, all metrics for maximum quality were achieved.”
The success of this surgery was based on two metrics – restoration of normal brain structure and the gestational age at birth.
Prior to foetal repair surgery, the back of the brain herniates down the spinal column – known as an Arnold-Chiari malformation – causing cerebrospinal fluid to back up and build pressure that can cause brain damage. Typically babies with spina bifida need shunts to decompress the built-up fluid after they are born. When a successful foetal surgery repair is completed and the brain is examined one month later, the malformation is reversed and the back of the brain returns to a normal appearance, which was observed in this case.
The surgery was also successful by the second metric – when the baby is born. The average birth after foetal surgery repair is 34 weeks gestation. In this case, the baby was born at 36.5 weeks gestation – exceeding the average birth time by more than two weeks and giving the baby more time to develop and grow.
“Although the surgery was a success, spina bifida is never cured,” said Dr Cass. “Moving forward, the baby will require ongoing supportive care provided by a multidisciplinary team of caregivers in our Spina Bifida Clinic, which will involve neurology, urology, orthopaedics, developmental paediatrics and neurosurgery, among other specialists.”
The surgical team from Cleveland Clinic’s Foetal Centre worked for more than a year to prepare for this first surgery, including making site visits to other centres, conducting simulations, and consulting with other experts in the field.
“I am honoured to work with this amazing team of clinicians and to see our efforts come to fruition after preparing for so long,” said Dr Cass, who joined Cleveland Clinic as director of Foetal Surgery in October 2017 to build its foetal surgery programme after co-founding and co-directing Texas Children’s Foetal Centre in Houston for 17 years. “Families in this region now have more options when it comes to making these types of decisions and we are thrilled to be able to provide the care needed for these complex cases.”
Cleveland Clinic is a non-profit multispecialty academic medical centre that integrates clinical and hospital care with research and education. Located in Cleveland, Ohio, it was founded in 1921 by four renowned physicians with a vision of providing outstanding patient care based upon the principles of cooperation, compassion and innovation. Cleveland Clinic has pioneered many medical breakthroughs, including coronary artery bypass surgery and the first face transplant in the United States. Among Cleveland Clinic’s 66,000 employees are more than 4,200 salaried physicians and researchers and 16,600 nurses, representing 140 medical specialties and subspecialties.
Cleveland Clinic’s health system includes a 165-acre main campus near downtown Cleveland, 11 regional hospitals in northeast Ohio, more than 180 northern Ohio outpatient locations – including 18 full-service family health centres and three health and wellness centres – and locations in southeast Florida; Las Vegas, Nev; Toronto, Canada; Abu Dhabi, UAE; and London, England. In 2018, there were 7.9 million total outpatient visits, 238,000 hospital admissions and observations, and 220,000 surgical cases throughout Cleveland Clinic’s health system. Patients came for treatment from every state and 185 countries.