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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Trismus-pseudocamptodactyly syndrome


Other Names for this Disease

  • Arthrogryposis distal type 7
  • Distal arthrogryposis type 7
  • Dutch-Kentucky syndrome
  • Hecht syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is trismus-pseudocamptodactyly syndrome?

What are the signs and symptoms of trismus-pseudocamptodactyly syndrome?

How might trismus-pseudocamptodactyly syndrome be treated?

What is trismus-pseudocamptodactyly syndrome?

Trismus-pseudocamptodactyly syndrome is a disorder of muscle development and function.[1] It is characterized by short muscles and tendons resulting in limited range of motion of the hands, legs, and mouth.[1] Both sporadic occurrence and autosomal dominant inheritance have been reported in the medical literature.[1] The most serious complications of the condition occur as a result of the limited mobility of the mouth.[2] Treatment may involve surgical correction and physical therapy.[1][2] 
Last updated: 4/13/2011

What are the signs and symptoms of trismus-pseudocamptodactyly syndrome?

While the symptoms of trismus-pseudocamptodactyly syndrome vary from patient to patient, characteristic symptoms include the inability to open the mouth wide (e.g., less than 6 mm, just under 1/4th of an inch) and shortened muscles, including of the hamstrings and calf muscles. As a result of shortened muscles some infants with trismus-pseudocamptodactyly syndrome have closed or clinched fists, club foot, metatarsus adductus, and calcaneovalgus (where the foot bends sharply at the ankle) at birth.[1][2] Children with this syndrome may crawl on their knuckles.[2] In adulthood the syndrome may cause reduced hand dexterity,[1] however hand limitation does not often interfere with normal function.[2] The most serious complications of the condition occur as a result of the limited mobility of the mouth, including impairment of adequate calorie intake, speech development, dental care, and difficulty with intubation.[2]
Last updated: 4/13/2011

How might trismus-pseudocamptodactyly syndrome be treated?

While the best treatment options for trismus-pseudocamptodactyly syndrome have not been well established[1][2] cases of improvement of mouth mobility following surgery and physical therapy have been reported in the medical literature.[1][2] We recommend that you speak with your healthcare provider to learn more about specific treatment options.
Last updated: 4/13/2011

References
  1. Carlos R, Contreras E, Cabrera J. Trismus-pseudocamptodactyly syndrome (Hecht-Beals' syndrome): case report and literature review. Oral Dis. 2005 May;
  2. Lefaivre JF, Aitchison MJ. Surgical correction of trismus in a child with Hecht syndrome. Ann Plast Surg. 2003 Mar;


Other Names for this Disease
  • Arthrogryposis distal type 7
  • Distal arthrogryposis type 7
  • Dutch-Kentucky syndrome
  • Hecht syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.