A six-year-old girl from Oman who developed a rare genetic disorder, characterised by distinctive malformation of the skull and facial region, has been given a new lease of life after a breakthrough surgery at a Delhi hospital.
According to doctors at Fortis Healthcare, Lyaan came with a condition called Crouzon syndrome -- a rare genetic disorder found one in 60,000 live births (or 16.5 cases in one million live births) and suffered with a severely deformed upper part of the face.
"Crouzon syndrome is a rare genetic disorder that may be evident at birth (congenital) or during infancy. It is characterised by distinctive malformations of the skull and facial region. Such abnormalities may vary greatly in range and severity from case to case, including among affected family members," said Rashmi Taneja, Senior Consultant (Plastic & Cosmetic Surgery) at Fortis Hospital in Vasant Kunj, in a statement.
Though genetic, none of her older or younger siblings suffered from this disorder.
According to her father, Ali Halthy, Lyaan was born normal, but developed the deformity in the upper part of the face and a retruded upper jaw, when she was 4-5 months old.
Later, Lyaan also developed breathing problems and underwent tracheostomy -- an incision in the windpipe made to relieve an obstruction to breathing.
Since her head was not growing to the required standard size, she also had to undergo cranial surgery at the age of one in Oman.
"Our team performed the Lefort III Osteotomy, where the mid-face was detached from the skull bone with application of rigid external distractors, also called RED Device, as part of the treatment on the patient," Taneja explained.
Four days following the complex surgery, performed in February this year, her mid-face and upper jaw was pulled forward at the rate of 1 mm a day.
The upper jaw was thus, pulled 35 mm in 35 days, after which the family returned to Oman, the doctors said.
After the complete healing of her bones, the ENT team also removed her tracheostomy.
"Lyaan has started breathing normally through her nose like any other child. She will have to undergo another set of surgery at age 16 to align her jaw," added Gagan Sabharwal, Maxillofacial Surgeon at Fortis Memorial Research Institute in Gurgaon.
--IANS
rt/rn/vt
