TURNER’S SYNDROME

What is affected in the syndrome? Common symptoms of Turner’s syndrome include:
• Short stature
• Lymphedema of the hands and feet
• Broad chest (shield chest) and widely-spaced nipples
• Low hairline
• Low-set ears
• Reproductive sterility
• Rudimentary ovaries gonadal streak (underdeveloped gonadal structures)
• Amenorrhea
• Increased weight, obesity
• Shield shaped thorax of heart
• Shortened metacarpal IV (of hand)
• Small fingernails
• Characteristic facial features
• Webbing of the neck
• Coarctation of the aorta and other cardiovascular defects (bicuspid aortic valve)
• Poor breast development
• Horseshoe kidney
• Visual impairments
• Ear infections and hearing loss
• Micrognathia
• Cubitus valgus (turned-out elbows)
• Drooping eyelids and palmar crease
• Narrow maxilla
Why? Turner’s syndrome (XO syndrome) or Ullrich-Turner syndrome encompasses several conditions, of which monosomy X is the most common. It is a chromosomal disorder affecting females in which all or part of one of the X chromosomes is absent. Occurring in 1 out of every 2500 girls, the syndrome manifests itself in a number of ways. Approximately 98% of all fetuses with Turner’s syndrome result in miscarriage.
When in the child’s life is the diagnosis made? Turner’s syndrome may be diagnosed by amniocentesis during pregnancy. Sometimes, fetuses with Turner’s syndrome are identified by abnormal ultrasound, findings (i.e. heart defect, kidney abnormality, cystic hygroma, ascites). In the postnatal period, Turner’s syndrome can be diagnosed by karotyping.
Why are we interested? Patients with Turner’s syndrome often present with a narrow, high, arched maxilla and the mandible tends to be wide and micrognathic. There is a high incidence of anterior and posterior open bite and posterior crossbite. Other dental abnormalities include early eruption of the permanent teeth, thinner enamel, less dentin, and shorter roots with a greater potential for root resorption during orthodontic treatment.
What are other systemic/medical conditions associated with the syndrome that are critical concerns if patient needs dental care? Prophylactic antibiotics should be used in patients with Turner’s syndrome with known cardiac malformations.
Information that has been learned recently: It has recently been found that there is a broader spectrum of cardiovascular system abnormalities in TS than previously known. There is a generalized dilation of major vessels in women with TS; the distal extent of this dilated vasculopathy is unknown.
Items of interest: The majority of girls and women have normal intelligence; however, they show some other functional deficits – problem solving, visuospatial organization, social cognition, and motor deficits. Many girls treated with growth hormones can attain normal or near-normal stature, and many women with TS are now able to carry pregnancies to full term due to advances in fertility treatments.