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Most children with Crouzon’s Syndrome can have a normal life expectancy if diagnosed early and treated timely.
Crouzon's Syndrome is a rare genetically inherited disorder with an incidence rate of 1 in 60,000 newborns worldwide. Cranial sutures or anatomical lines are the fibrous tissue bands that join the bony plates of the skull together. In Crouzon Syndrome, the sutures, in an infant, merge prematurely affecting the normal growth of the skull and head leading to an alteration in the shape and therefore the development of the skull. The seriousness of craniosynostosis does vary from one child to another. The symptoms primarily include abnormalities of the face and head.
Dr Nitin Mokal, Paediatric Plastic Surgeon at SRCC-NH Children's Hospital said, "The syndrome is characterized by Craniosynostosis and many other features. This can cause an abnormal shape of the head and face with babies having a short and wide or long and narrow head, enlarged foreheads, shallow orbits (eye sockets) leading to bulging eyeballs, flattened cheeks, poorly developed upper jaw, protruding lower jaw, and curved, beak-like nose."
The other features associated with this syndrome include inflammation of the cornea (exposure keratitis) due to bulging eyeballs (proptosis), squint, vision loss, hearing loss, dentition problems, cleft in the lip/palate, narrow airways leading to respiratory problems and sleep apnoea. According to experts, there can be increased pressure in the brain and accumulation of excessive fluid in the brain (hydrocephalus).
Most patients can have normal life if diagnosed and treated early
Dr Uday Andar, Paediatric Neurosurgeon at SRCC-NH Children's Hospital, called for increasing awareness about Crouzon's Syndrome.
An expert in Congenital scoliosis - Spinal dysraphism - Craniofacial anomalies, Dr Andar said that most of these children can have a normal life expectancy if diagnosed early and treated timely by an experienced team.
He added, "The social stigma around abnormal facial appearance and lack of awareness about the condition leads to many children being devoid of appropriate and timely diagnosis and treatment. The treatment of this syndrome requires a multidisciplinary team approach consisting of specialists like Neurosurgeons, Plastic Surgeons, ENT Surgeons, Ophthalmologists, Dental Surgeons, Paediatric Anaesthetists, Speech and Language Therapists etc. Such children may require more than one surgical procedure during their lifetime."
Such children if referred to and treated by a team experienced in the management of craniofacial disorders can have a normal life and future, the experts at the SRCC-NH Children's Hospital noted.
Treatment of Crouzon Syndrome: A case study
Yusuf Ali, a 17-year-old teen with Crouzon Syndrome (abnormal brain structure) was having serious issues with breathing, including sleep apnoea. Besides, his bulging eyeballs were at risk of exposure to keratitis. On Yusuf's birth, his parents were told that the baby would never make it past his childhood and that if he did, he would grow up with severe mental retardation and disability. He had undergone two surgeries as a baby, but as he grew up, the problem continued, and his brain potential was at stake.
He was recently admitted and treated by a highly experienced craniofacial team at Narayana Health's SRCC Children's Hospital. He successfully underwent Monobloc Advance Surgery, which helped moved abnormal brain structure which was hampering his brain potential and gave him back his love for history, literature and the future.
The complex procedure was performed by a team of craniofacial surgeons comprising plastic surgeons: Dr Nitin Mokal, Dr Amita Hiremath, and Neurosurgeons Dr Uday Andar & Dr Saurav Samantray.
As explained by the experts, the surgery was performed to bring Yusuf's forehead and mid-face forwards so as to make space for his eyeballs and increase the diameter of his airways through the nose and mouth. The surgery is called Monobloc Advancement using a RED (Rigid External Distractor) frame.
Dr Saurav Samantray, Paediatric Neurosurgeon at SRCC-NH Children's Hospital, said, "This frame was attached to the sides of his skull, forehead, cheek and jaw bones with wires. The frame was then used to advance the forehead and face by 1 mm/day, starting one week after surgery. The frame stayed on the patient's head for 2-3 months after that it was removed, confirming adequate advancement on imaging. This process of slow advancement leads to new bone formation in the gap generated by pulling the forehead and face forwards."
Yusuf's mother noticed a discernible change in his smile and eyes immediately after the surgery. The teen was able to eat, drink, sleep, read, walk and do every other routine activity within a week of the surgery.
Dr Amita Hiremath, Craniofacial Surgeon, SRCC-NH Children's Hospital, highlighted that Yusuf's story proves the need for awareness and sensitivity towards a condition like Crouzon's Syndrome.
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