The first detailed description of congenital facial paralysis was reported by Moebius in 1888. It is characterized by either unilateral or bilateral paralysis of the facial muscles and an associated abducens palsy. The present report is of two patients with Moebius syndrome, who were also diagnosed with trismus at birth. Each patient also demonstrated bilateral hypertrophy of the coronoid process of the mandible. In effect, the zygoma obstructed the excursion of the mandible because of a "coronoid block." A three-dimensional computed tomography scan demonstrated normal temporomandibular joints but bilateral hypertrophy of the coronoid processes and micrognathia. Both patients demonstrated less than 10 mm of oral excursion. Bilateral coronoidectomies were performed through an intraoral approach. The oral excursions after surgery increased to at least 20 mm. In each of these patients, the coronoid process was enlarged relative to the zygoma, which was of normal size and configuration. The trismus was associated with blocking of the coronoid by the anterior zygoma, preventing open or full excursion of the hypoplastic mandibles. Moebius syndrome can have a variable presentation at birth. In two patients, the authors describe a new finding of hypertrophy of the coronoid process and trismus secondary to obstruction of the coronoid by the hypertrophic zygomas during oral excursions. Each patient is described, and a review of the literature is discussed.
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J Orthop Case Rep
August 2023
Department of Pathology, Government Medical College, Haldwani, Uttarakhand, India.
Introduction: The elbow pain and restricted movement is a nagging problem and elbow arthropathies need to be excluded. On rare instances, uncommon etiology like a benign lesion is the culprit and the diagnosis would require judicious clinicoradiological correlation. Osteoid osteoma in the intra- or juxta-articular region is reported in the literature as rare, sporadic report.
View Article and Find Full Text PDFIndian J Otolaryngol Head Neck Surg
June 2023
Institute- Atal Bihari Vajpayee Institute of Medical Sciences and Dr Ram Manohar Lohia Hospital, New Delhi, India.
Background: Jacob's disease is a rare pathology characterized by elongation/ enlargement of coronoid process of mandible with formation of pseudoarticulation with zygomatic arch. It presents clinically as restricted mouth opening and is often misdiagnosed as temporomandibular joint pathology.
Case Presentation: We performed cross sectional imaging and evaluation of Jacob's disease in a 14-year-old girl with restricted mouth opening, CT images including Multiplanar Reconstruction and Volume Rendered Technique revealed enlarged left coronoid process of mandible while open mouth CT images and MR images concluded the presence of joint between enlarged coronoid process and zygomatic arch.
J Clin Med
December 2022
Jagiellonian University Medical College, Department of Rehabilitation in Internal Diseases, Faculty of Health Science, Institute of Physiotherapy, 8 Skawińska Str., 31-066 Krakow, Poland.
Temporomandibular disorder is a musculoskeletal disease with complex, multifactorial etiology regarding improper functioning of the stomatognathic system (masticatory muscles, temporomandibular joints, and surrounding structures). This article presents medical emergencies occurring among patients treated for temporomandibular disorders, which tend to constitute a severe difficulty for practitioners during their clinical practice. Examples of the most common emergencies of this type are disc displacement without reduction and a sudden contraction of the inferior part of the lateral pterygoid muscle.
View Article and Find Full Text PDFJ Stomatol Oral Maxillofac Surg
February 2023
INSERM U1229 - Regenerative Medicine and Skeleton RMeS, Nantes, France; Nantes University, CHU Nantes, Department of Oral and Maxillofacial Surgery, Nantes, France.
Background: The objective of this study was to evaluate the mouth opening (MO) in patients with Langenbeck or Jacob diseases after a multimodal treatment combining the coronoidectomy and a self or assisted postoperative rehabilitation.
Methods: This observational retrospective study included patients who had clinically impacted MO limitation. All patients underwent unilateral or bilateral coronoidectomy and then physical therapy for at least 3 months.
Mol Genet Genomic Med
December 2022
Department of Pediatric Neurology, Developmental Medicine, Social Pediatrics, Dr. von Hauner Children's Hospital, LMU - University Hospital, Ludwig Maximilians Universität, Munich, Germany.
Background: Mutations in SOX6 have recently been recognized as a new molecular cause of neurodevelopmental disorders characterized by intellectual disability, behavioral changes, and nonspecific facial and digital skeletal abnormalities. To date, <25 cases have been reported in the literature.
Methods And Findings: Here we report a new case of SOX6-associated neurodegeneration and expand the phenotype to include ceratoconus.
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