Baby Lucas Survives Exencephaly


HACKENSACK, N.J. (Ivanhoe Newswire)— Exencephaly is a rare birth defect that causes the brain to develop outside the skull, exposing the tissue to amniotic fluid in the womb. The condition has been universally fatal … until now. Neurosurgeons developed a procedure that allowed a newborn identified with the condition during his mom’s pregnancy to survive after birth and continue to thrive.

Lucas Santa Marie is now two years old. An adorable little brother to his three big sisters and by all accounts, a medical miracle.

“We were gambling for when we decided to give birth to him, just a few minutes to hold him. Now we’ve already got two years,” exclaimed Maria Santa Maria and AJ Santa Maria, Lucas’ parents.

Maria’s doctors induced her at 35 weeks. Lucas came into the world at six and a half pounds and 19 inches long–breathing on his own. A team of doctors developed a first-of-its-kind, six-hour surgery that drained extra fluid in his brain, removed dying brain tissue, and provided his healthy brain with protection by using skin that had developed at the base of his skull.

“We provided him with a scalp and what happens is the layerings of the brain actually formed bone. So he was able to form bone around a good portion of his skull,” explained Timothy Vogel, MD, Pediatric Neurosurgeon, Joseph Sanzani Children’s Hospital.

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The family knew that Lucas could die at any time, but he proved everyone wrong and left the NICU after one week.

“We always say God has a plan for everyone and I guess he had something bigger for Lucas and for us,” shared AJ.

“When we look at the future, it’s a little scary to realize that maybe medically, he might not do much more. But then I always remember that medically he’s not supposed to be here,” said Maria.

Dr. Vogel says since Lucas is the first to survive the condition, no one is sure how he will continue to develop, or what his future holds. His parents Maria and AJ say Lucas doesn’t speak phrases, but he coos, and does say “mom.”  Doctors will perform a third surgery around his fifth birthday to give his brain more room as he grows.

Contributors to this news report include: Cyndy McGrath, Executive Producer; Kirk Manson, Videographer; Roque Correa, Editor.

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REPORT:       MB #4930

BACKGROUND: Lucas had a condition called exencephaly; it’s a condition where the brain actually is on the outside of the skull. Exencephaly is a rare malformation of the neural tube with a large amount of protruding brain tissue and absence of calvarium. It is considered to be an embryological precursor of anencephaly where the facial structures and the base of brain are always present. The incidence of exencephaly is 3 per 10,000 pregnancies and the absence of the calvaria causes brain destruction by exposing the developing brain to amniotic fluid and repeated trauma.


DIAGNOSING: What causes exencephaly is the failure of the anterior neuropore to close during the 4th week of embryonic development. The underlying defect is due to a failure in mesenchymal migration. In pathologic studies, the exencephalic brain is noted to be covered by a highly vascular epithelial layer. The two relatively equivalent cerebral hemispheric remnants are present within a reddish mass of disorganized tissues, remnants of deep cerebral neural elements, blood vessels, fibrous tissues, and fluid-filled spaces The remaining brain has been termed the “anencephalic area cerebrovasculosa.” In exencephalic brain tissue, the gyri and sulci are shallow, flattened, and disorganized.


NEW TECHNOLOGY:  Dr. Tim Vogel, chief of pediatric neurosurgery at North Jersey Brain and Spine Center was able to have a successful surgery on Lucas. Vogel’s team also had to preserve the spinal fluid cavity so that the brain would be protected once they were done, and prevent any infection from setting in. Vogel explained that the dura, which is the brain’s outermost membrane, has the ability to form bones in children under the age of 18 months. Since the operation, Vogel said bone is starting to form a protective layer around the brain, and that the scalp has grown over it, complete with hair. Future surgeries will include taking the bone currently growing and shaping it around other areas that are lacking, which makes the timing tricky.





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Doctor Q and A

Read the entire Doctor Q&A for Timothy Vogel, MD, Pediatric Neurosurgeon

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