Baby Born with Exencephaly First to Survive, Thrive, After History-Making Surgery

Oradell, NJ – October 14, 2019 – A baby born with exencephaly, a rare condition where the baby’s skull doesn’t fully develop and the brain is outside the skull, is the first with the diagnosis to survive after a life-saving surgery performed by a New Jersey Brain and Spine surgeon.

Babies with exencephaly who survive birth are generally given palliative care and may only live for minutes or hours. Exencephaly is caused by a neural tube defect, like spina bifida, where the spinal column fails to close somewhere along its length. These defects occur in about 5 out of 10,000 live births. With exencephaly, the unprotected brain usually deteriorates from exposure to the amniotic fluid and the baby’s movement within the womb. There may be only a layer or two of cells – a thin membrane – protecting the brain.

Maria and Augusto Santa Maria received their baby’s exencephaly diagnosis at 10 weeks, after their first ultrasound at Hackensack University Medical Center (HUMC). They were told their baby would die before or shortly after birth, and they began working with the HUMC team to prepare for palliative care. Midway through their pregnancy, Tim Vogel, M.D., chief of pediatric neurosurgery at New Jersey Brain and Spine, heard about the diagnosis and conferred with the HUMC neonatal team.

“I told the HUMC team that if the baby survived birth, I might be able to surgically intervene and improve the baby’s exencephaly situation,” said the NJBS team. “Baby Lucas was born at 35 weeks with no skull above his eyebrows and ears, but there was a large sac of fluid protecting his brain. It was delicate, yet he was in a stable condition. I met with the Santa Marias and they just wanted to be able to bring their baby home. While there were risks to consider, they decided to proceed with the surgery to remove the sac of fluid, stabilize Lucas’ brain, and close the open skull cavity.”

The team of physicians at HUMC performed the five-hour surgery four days after Lucas’ birth. Much of his brain had not formed properly and they had to carefully remove one hemisphere that was damaged along with part of the malformed cerebellum. He was able to use existing dura, the thickest of the three membranes covering the brain and spinal cord, to close most of the gap across the top of Lucas’ head.

The exencephaly procedure was a success. After only a week in the neonatal intensive care unit, Lucas was able to go home with his parents and three older sisters, eating and breathing on his own. Now seven months old, he is developmentally on par with other babies his age, eating soft solid foods, cooing when awake, and holding his head up during tummy time. Next year, the NJBS team expects to perform a second surgery to replace the missing portion of Lucas’ skull with a bone graft.

“We don’t really know what to expect developmentally, because Lucas is the only exencephaly survivor in the world. We’re in uncharted territory with him. As his parents said to me, every day is a miracle. For the medical and neurosurgical community, this is a significant advancement in medicine, and for parents everywhere who might face this rare condition, there is hope.”

About New Jersey Brain & Spine

For over 20 years, the physicians and surgeons of NJBS have restored children and adults with a wide range of brain, spine and other neurological conditions to optimal health. Leaders in their field and respective subspecialties, NJBS physicians begin with conservative care and extend to highly advanced, minimally invasive procedures as needed, achieving unparalleled outcomes when treating complex neurosurgical conditions, including strokes, aneurysms, brain and spine tumors, spine pain and disorders, and movement disorders such as Parkinson’s disease. NJBS accepts most insurances and practice at the area’s finest hospitals. For more information please visit www.njbrainspine.com.