Gurugram: 33-year-old with rare condition undergoes open-skull surgery to remove tumour

GURUGRAM: A 33-year-old woman with a rare genetic condition that triggers tumours on her face and around her skull underwent extensive open-skull surgeries that are expected to improve her quality of life after years of suffering, her doctors said on Wednesday.
The woman was diagnosed with plexiform neurofibromatosis - in which tumours form along the nerves, on the face and around the eye - in her early childhood.
At just six months old, she lost vision in her left eye after being diagnosed with glaucoma. Her condition deteriorated over the last 15 years, and at least three rounds of surgeries in 2006, 2010 and 2014 across hospitals could only partially remove the tumour, which would grow back.
Doctors at the Medanta Hospital, where she came for treatment last year, said she was emotionally and psychologically drained.
It took eight months of counseling to convince her to undergo invasive surgeries that could remove the tumour near her eye socket and improve the facial disfigurement caused by it.
Doctors decided on a multidisciplinary approach as the patient's left was bulging from the socket and her facial skin was sagging.
"This is a very rare case as determining the tumours was difficult because they had spread across her brain, eyes and face. We used CT scans and MRIs to find out the extent and location of the benign tumours. It was then decided that the surgeries would be conducted in two phases. First, plastic surgeons and neurosurgeons would work together to surgically remove the excess tissue and trace the tumour back to the brain, as far as possible, without compromising the patient's functions," said Dr VP Singh, chairman at the hospital's Institute of Neurosciences.
Doctors were to reconstruct the bone in her skull that had to be cut for access to the tumours. This, they said, would also prevent the tumour from growing again.
"The bone was re-constructed using a titanium mesh... In the second stage of the surgery, the non-functional left-eye was removed, and in its place, an artificial eye was inserted to block a tumour from developing again. The result was reasonably positive," Dr Singh added.
Before the surgeries this week, the doctors used computer-made models to study the patient's skull. AN incision on her scalp was their only way to access the tumours.
But to reach the tumour, near her eye, they had to remove the cranial bone. Once they did so, they could cut out the tumour and create space for her eye to return to its position, they said.
"Having achieved that, the titanium mesh was used to separate the orbital cavity (near her eye) from the cranial cavity. Post-surgery the patient showed remarkable improvement," said Dr Singh.
The woman, who belongs to the city, still needs to be operated on to work on her facial appearance, and a portion of the tumour that still exists at the base of her skull will be treated with radiology to prevent a relapse, the doctors said.
She is expected to recover from the surgeries in two weeks, and has been discharged from the hospital.