Abstract
Hecht Syndrome is an autosomal dominant distal arthrogryposis caused by mutation in the MYH8 locus characterized by trismus and pseudocamptodactyly. Hecht-associated trismus is thought to result from bilateral hyperplasia of the mandibular coronoid processes. Although several interventions to address trismus have been pursued, no consensus exists regarding optimal management. In this report, the authors present a 7-month-old male with Hecht Syndrome referred for management of trismus. By age 2, interincisal opening had progressively decreased from 12 to 5 mm despite physical therapy. Nutrition was limited to liquids, oral hygiene was compromised, and aspiration risk was present. Computed tomography examination revealed enlarged coronoid processes extending medially and superiorly to the zygomatic arches. To release bony impaction of the coronoid processes against the zygoma and to prevent reossification of the temporalis tendon insertion, resection of the enlarged coronoids and distal temporalis muscles as well as placement of Alloderm spacers were performed via an open craniofacial transzygomatic approach. Jaw motion rehabilitation was used following surgery. Two years postoperatively, the patient had no signs of recurrence an...Continue Reading
References
Oct 1, 1990·The Journal of Cell Biology·G E LyonsM Buckingham
Feb 15, 1985·Science·E Bandman
Oct 1, 1974·The Journal of Pediatrics·C C MabryH W Sorenson
Oct 1, 1973·Oral Surgery, Oral Medicine, and Oral Pathology·S L HorowitzA J Chabora
Mar 1, 1974·Journal of Medical Genetics·B G ter Haar, R F van Hoof
Jan 1, 1971·Humangenetik·J G De Jong
Aug 1, 1984·Journal of Pediatric Orthopedics·P J O'BrienJ G Hall
Jan 1, 1996·Annual Review of Cell and Developmental Biology·A Weiss, L A Leinwand
Aug 6, 1999·Journal of Clinical Anesthesia·J Seavello, G B Hammer
Aug 14, 1999·Paediatric Anaesthesia·O NagataK Hanaoka
Feb 19, 2000·Developmental Dynamics : an Official Publication of the American Association of Anatomists·S MachidaK Momma
Apr 4, 2002·Dental Update·P J Dhanrajani, O Jonaidel
Jun 13, 2003·Annals of Plastic Surgery·Jean-Francois Lefaivre, Mary J Aitchison
Jun 18, 2003·Development·Loren D SacksFrank E Stockdale
Jul 30, 2004·The New England Journal of Medicine·Mark VeugelersCraig T Basson
Sep 4, 2004·The Journal of Craniofacial Surgery·Adrian M Skinner, Martin J W Rees
May 13, 2005·Oral Diseases·R CarlosJ Cabrera
Apr 28, 2006·Nature Genetics·Reha M ToydemirMichael J Bamshad
Citations
Oct 20, 2018·The Journal of Craniofacial Surgery·Marco FarronatoCinzia Maspero
May 26, 2020·Journal of Stomatology, Oral and Maxillofacial Surgery·M BénardA Depeyre
Feb 23, 2021·Cranio : the Journal of Craniomandibular Practice·Małgorzata Kulesa-MrowieckaAndrzej Myśliwiec