Dutch-kennedy syndrome is a rare inherited disorder characterized by short muscles and tendons that result in limited mobility of the hands, legs, and mouth. The most serious complication of this condition is the inability to completely open the mouth (trismus), which causes difficulty with chewing. Short muscles and tendons in the fingers cause the fingers to bend or curve (camptodactyly) when the hand is bent back at the wrist. However, the fingers are not permanently bent or curved, so this particular finding is called “pseudocamptodactyly”.
DUTCH-KENNEDY SYNDROME is an autosomal dominant disorder caused by changes in the MYH8 gene. All cases of DUTCH-KENNEDY SYNDROME studied to date were found to have the same change in the MYH8 gene. This gene is involved in making the instructions for the development of the limb skeletal muscles and the muscles in the face.
1. The treatment of this disorder is directed toward the specific symptoms seen in each individual.
2. Treatment may require the efforts of a team of specialists who work together to plan a child’s care.
3. Such specialists may include pediatricians, those who diagnose and treat skeletal disorders (orthopedists), orthopedic surgeons, dentists, anesthesiologists, physicians who specialize in disorders of the digestive tract (gastroenterologists), nutritionists, and physical and occupational therapists.
4. In some people, jaw surgery may be performed to help increase the opening of their mouth.
Genetic counseling is recommended for affected individuals and their families.
Short muscles and tendons that result in limited mobility of the hands, legs, and mouth
Inability to completely open the mouth (trismus), which causes difficulty with chewing