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Baby Born With Part Of Brain Outside Skull Defies Odds, Survives First-Ever Operation Of Its Kind

Discussion in 'Pediatrics' started by Hadeel Abdelkariem, Oct 5, 2019.

  1. Hadeel Abdelkariem

    Hadeel Abdelkariem Golden Member

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    A baby born with a part of his brain outside of his skull becomes the first to survive a unique operation, making him the only newborn with the rare, normally fatal condition to survive more than a day after birth.

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    Ten weeks into his mother’s pregnancy, Lucas was diagnosed with exencephaly – a life-threatening neurological condition where the skull does not form properly and leaves the brain exposed to the “intrauterine environment” of a mother’s womb. This exposure causes physical and mental damage, and doctors typically recommend that mothers terminate the pregnancy. Even if the baby is carried to term, most will not survive the day. Maria Santa Maria, Lucas’ mother, decided against an abortion and said she had prepared her three daughters for the birth.

    "When we were in the delivery room because I wanted them to meet their baby brother, so we didn't know what to expect. So, they came in, they were told their baby brother was going to die," she told ABC7.

    Doctors removed half of Lucas' basal ganglia, a part of the brain responsible for the control of movement, that hadn't formed properly. Given young children's high neuroplasticity, doctors believe there is a chance the protected half of Lucas' basal ganglia might reassign duties from the damaged half, reports CNN. Doctors worked to close the skin around his exposed brain tissue and as the skull bone grows in, they will shave small layers in order to fill in sunken areas.

    “After only a week in the NICU, he was able to go home eating and breathing on his own. Each day he has is a blessing,” said operating doctor Tim Vogel in a Facebook post.


    Lucas is now seven months old and has defied the odds against him. However, doctors are not sure how long he will live or what his quality of life will be. Exencephaly is a rare malformation of a hollow structure where the brain and spinal cord eventually form, or the neural tube. Neural tube defects are the third most common congenital abnormality in the US, second only to cardiac malformations. Though facial structures and the base of the brain are still present, babies with exencephaly are characterized by bulging eyeballs and having brain tissue protruding from where the skullcap is missing. The exact cause is not known but the condition occurs in about 3 per 10,000 pregnancies screened, making it an early pregnancy precursor to anencephaly, a serious birth defect where a baby is born without parts of the brain and skull. Exencephaly causes destruction of the developing brain by exposure to amniotic fluid and repeated trauma that, in some cases, can destroy the brain as early as eight weeks gestation.

    Most babies diagnosed with exencephaly are stillborn; however, a live full-term fetus was born in 2009 before dying three hours later. According to an article published in the Journal of Pediatric Neurosciences, an autopsy revealed the brain was covered in a thick membrane, was swollen in several parts, and had “tortuous blood vessels”.

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