BENGALURU: Doctors in the city have come across a one in a million occurrence, with an eight-year-old boy having a rare condition called nasal encephalocele. Due to this, his brain grew down through a crack in the skull into his nose. An encephalocele is a rare congenital disorder where the bones of a baby’s skull do not close completely in the mother’s uterus. This later creates an opening through which the brain tissue and cerebrospinal fluid protrude out of the head in a sac-like structure, and poses a huge risk of brain infection for the patient.
The hanging brain matter impacted the boy’s life for several years. Every day, his parents helplessly watched their son struggling, with a part of his brain hanging like a pendulum inside his mouth. The condition obstructed the child’s vision and had completely deformed his nose and face. He was also suffering from a cleft lip (congenital split in the upper lip on one or both sides of the centre) and a cleft palate (congenital split in the roof of the mouth).
Though his parents visited several hospitals, doctors who saw him couldn’t ensure his survival because of the potential complications involved in the operative procedure. Finally, doctors at Aster CMI Hospital performed a six-and-a-half hour surgery. Dr Ravi Gopal Varma, Lead Consultant Neurosurgeon & Chief of Neuro Sciences, Aster, Bengaluru, said it was a complex case which had to be operated in two stages. The child presented to them with a cleft lip, cleft palate, and a swelling inside the mouth. The pulsating mass in the child’s mouth looked ominous, moving with each heartbeat.
“The child’s brain had descended down as a pouch, and the bone between his brain and eyes was deficient. Though there was no impact on his brain activity and he was a very bright boy, there were chances that the encephalocele could rupture and cerebrospinal fluid could flow out. This, in turn, could have caused an infection -- a life-threatening scenario for the child,” Dr Varma said.
The doctors opened his skull and retracted his brain. They isolated the normal brain from the hanging sac of non-functioning brain matter. They then placed an artificial bone between the eyes and another bone on that to ensure the brain does not fall into the nasal cavity again. In the next stage, they repaired his cleft lip and put him under speech therapy. In three months, he will undergo repair of the cleft palate.