Trismus-pseudocamptodactyly syndrome

Question Question

My child has trismus-pseudocamptodactyly syndrome. We are very concerned about his trismus. We are interested in receiving any information regarding effective treatment for trismus.

Answer Answer

The following information may help to address your question:

What is trismus-pseudocamptodactyly syndrome?
How might trismus-pseudocamptodactyly syndrome be treated?
How can I learn about research involving the treatment of trismus in people with trismus-pseudocamptodactyly syndrome?

What is trismus-pseudocamptodactyly syndrome?

Trismus-pseudocamptodactyly syndrome (TPS) 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] The most serious complications of the condition occur as a result of the limited mobility of the mouth (trismus).^[2] TPS is typically reported to be inherited in an autosomal dominant manner and is caused by mutations in the MYH8 gene.^[1]^[3] Treatment may involve surgical correction and physical therapy.^[1]^[2]

Last updated: 7/21/2016

How might trismus-pseudocamptodactyly syndrome be treated?

There is no one treatment for trismus-pseudocamptodactyly (TPS), however, there are various strategies that can be used to manage symptoms. Physical and/or occupational therapy may be helpful for individuals experiencing difficulties with walking and hand movement. Surgery may be performed to correct certain musculoskeletal symptoms.^[4] There have been reported cases of improvement of mouth mobility following surgery and physical therapy.^[1]^[2]^[5]^[6]

Last updated: 7/21/2016

How can I learn about research involving the treatment of trismus in people with trismus-pseudocamptodactyly syndrome?

To learn about research opportunities, we suggest you review the information available on our get involved in research page.

Last updated: 7/21/2016

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

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GARD Information Specialist

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References References

Carlos R, Contreras E, Cabrera J. Trismus-pseudocamptodactyly syndrome (Hecht-Beals' syndrome): case report and literature review. Oral Dis. 2005 May; http://www.ncbi.nlm.nih.gov/pubmed/15888111.
Lefaivre JF, Aitchison MJ. Surgical correction of trismus in a child with Hecht syndrome. Ann Plast Surg. 2003 Mar; http://www.ncbi.nlm.nih.gov/pubmed/12800911.
Victor A. McKusick. ARTHROGRYPOSIS, DISTAL, TYPE 7; DA7. In: Marla J. F. O'Neill. OMIM. 2/26/2013; http://www.omim.org/entry/158300.
Trismus Pseudocamptodactyly Syndrome. NORD. 2003; http://rarediseases.org/rare-diseases/trismus-pseudocamptodactyly-syndrome/. Accessed 7/21/2016.
Gasparini G, Boniello R, Moro A, Zampino G, Pelo S. Trismus-pseudocamptodactyly syndrome: case report ten years after. Eur J Paediatr Dent. 2008 Dec; http://www.ncbi.nlm.nih.gov/pubmed/19072009.
Balkin, Daniel M. MD, PhD; Chen, Isaac DDS; Oberoi, Snehlata DDS, MS; Pomerantz, Jason H. MD. Bilateral Coronoidectomy by Craniofacial Approach for Hecht Syndrome-Related Trismus. Journal of Craniofacial Surgery. September 2015; 26(6):1954-1956. http://www.ncbi.nlm.nih.gov/pubmed/26335328.

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