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Treacher Collins Syndrome

Treacher Collins Syndrome is a cluster of genetic disorders that affect the structure, size, and positioning of the jaws, eyes, ears, and cheekbones. Treacher Collins Syndrome can affect a child’s eating, breathing, or hearing ability.

The disorder is rare, ranges from mild to severe, and is treated with corrective surgeries and lifelong medical care.

Symptoms

Common symptoms of Treacher Collins Syndrome include:

  • A partially blocked airway
  • A downward slant to the eyelids
  • Cleft palate, an opening in the roof of the mouth
  • Small or abnormally flat cheeks
  • Small ears or lower jawbones
  • Hearing loss due to structural abnormalities of the outer ear
  • Sleep apnoea
  • Narrow nasal passages
  • Abnormal development of the teeth
  • Crossed eyes
  • Loss of eyelashes
  • Narrow tear ducts
  • Developmental delays that affect motor movement
  • Eyes set far apart
  • Congenital heart defects
  • Developmental abnormalities in the digestive system

The symptoms that someone with Treacher Collins Syndrome exhibits vary from person to person. They are present at birth and usually affect both sides of the face. Treacher Collins Syndrome affects speech, language, hearing, and breathing abilities the most and rarely affects cognitive abilities.

Causes & Risk Factors

The only known cause of Treacher Collins Syndrome is genetic mutations. 60% of genetic mutations linked to Treacher Collins Syndrome are not inherited. However, mutations linked to Treacher Collins Syndrome can be inherited from a parent. The four genetic mutations linked to Treacher Collins Syndrome occur on the TCOF1, POLR1B, POLR1C, and POLR1D genes.

The mutations can be inherited in an autosomal dominant (only one parent needs a mutation) or autosomal recessive (both parents need a mutation) pattern. Autosomal dominant mutations occur on POLR1B, POLR1D, and TCOF1, and autosomal recessive mutations occur on TCOF1, rarely, and POLR1D.

The only risk factor for Treacher Collins Syndrome is having a family member with the disorder. If there is a family history of Treacher Collins Syndrome, you may want to consider genetic testing when trying to conceive.

Diagnosis & Complications

Diagnosis is usually made while your child is a newborn. A detailed medical history and physical exam findings may lead a doctor to believe your child has Treacher Collins Syndrome. X-ray testing to visualise skull and facial abnormalities and genetic testing will be done to make a definitive diagnosis.

Complications associated with Treacher Collins Syndrome include severe respiratory illness, heart disease, and malnutrition.

Treatment & Management

Treatment for Treacher Collins Syndrome depends on your child’s symptoms. Here are some common treatments for different aspects of the disorder.

  • Narrow nasal passages/blocked nose – surgery to clear the airway
  • Abnormally angled lower jaw – side sleeping, CPAP machine, surgery
  • Cleft palate – surgery
  • Facial abnormalities – facial reconstruction surgery to help support the child’s breathing and treat other symptoms
  • Eyes that slant downwards – surgery
  • Small or absent cheekbones – bone grafts or implants
  • Jaw abnormalities – surgery to support breathing and eating
  • Structural abnormalities of the outer ears – surgery, often bone graft surgery
  • Hearing loss – surgery to build out the outer ear, speech therapy, bone implants

Children with Treacher Collins Syndrome cannot be cured and will need lifelong medical support from specialists. However, they typically have an average life expectancy. There is no way to prevent Treacher Collins Syndrome in a child. Genetic testing can be done in early pregnancy to see if your child has the disorder. Many children and families caring for children with Treacher Collins Syndrome benefit from going to local support groups.

 
 
 

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