Successful treatment of complex narrow-skull syndrome (Apert’s Syndrome), it is a rare disease in the world

19/06/2019 09:50

Viet Duc University Hospital said that after 6 times of surgery for 5 years, doctors have save life of a child from the risk of disability, mental retardation, blindness returning to life almost normal. 

 

Unfortunately, N.T.T 6-year-old baby was born with Apert syndrome, a rare complication of facial craniofacial syndrome with the rate of only 1 / 65,000 – 1 / 200,000 live births. This syndrome consists of a combination of malformations from severe narrowing of the skull, lack of eye socket, maxillary bone, arch openings as well as the sticking of all five tips of fingers and toes together.

Ass. Prof. Nguyen Hong Ha – Chief of Maxillofacial, plastic and aesthetic surgery of Viet Duc University Hospital said, for this multi-malformation syndrome, cranial and ocular narrowing are two dangerous and acute problems, necessary to deal first the hand deformities of children. The fact that the skull immediately preceded 1 year did not develop the brain. Many studies show that a child’s brain needs to develop very rapidly during the first 12 months of life. The increase in intracranial pressure will cause compression of the push into the brain impact to tissue. Without plastic surgery to expand the skull, the brain will not develop, leading to serious consequences that should develop children almost normally, then become mental retardation and cannot self-service. The uncovered eyes are at risk of corneal ulcers causing vision loss and blindness. Therefore, it is urgent to require early surgical intervention as soon as the child is less than 12 months old.

N.T.T’s mother said that due to early intervention in Plastic Surgery Department, Viet Duc University Hospital at the appropriate time, when she was 10 months old, she had to recreate her eyes and skull, so now she had to play normal brain development. She can start learning to read, write, practice singing like other children. Currently, family takes her to the hospital to prepare for surgery to repair the fingers in left hand hopefully she can confidently go to school with friends in the next school year.

It is known that Vietnam is located in Southeast Asia which is one of the regions with the highest rate of congenital defects in the skull face in the world. Therefore, the development of a team of medical doctors equipped with adequate knowledge as well as material facilities is essential for medical examination and treatment. Previously, because of medical conditions, the country still had difficulties, so with the complexity of diseases rich families tended to go abroad to have expensive, hard treatment with dozens of surgeries in a long time. Patients without surgical conditions lead to lifelong disability, leaving a huge burden on families and society.

 

Therefore, from the last 10 years, Viet Duc University Hospital has sent many doctors abroad to train in major centers of craniofacial surgery of the UK, France, USA, Canada, and Australia. There are a series of hospitals that have sent a whole team including full doctors specialized in Plastic surgery, Maxillofacial, Center of anesthesia and surgical intensive care, Neurosurgery to create a closed specialized circle, possibly comprehensive treatment for these complex defects.

 

For more than 5 years in the hospital, there has been a very close coordination between specialists. Every Thursday in the afternoon, Center of anesthesia and surgical intensive care surgeons are also invited to join the consultation meeting of the Maxillofacial Surgery to discuss and select the most optimal surgical plan for patients. The hospital has successfully examined and operated for hundreds of children with congenital facial defects.

 

Although successfully deployed many techniques in craniofacial surgery, the hospital has always focused on developing international cooperation to exchange experience with the world’s leading centers for Vietnamese patients. Although there is still access to the most advanced techniques in the country.

 

From May 5, 2019 to May 11, 2019, Maxillofacial-plastic and aesthetic surgery will coordinate with the leading team of Craniofacial Surgery from the UK to examine and operate for children with birth defects in face such as Apert, Crouzon, Goldenhar syndrome, microtia (small ear)… Families who want to register can call the number of Khoa 02438253531 odd number 350 (24 / 24h) to have more information and free examination registration.

 

Social Work Department