Pycnodysostosis is an uncommon autosomal recessive sclerosing bone disorder which is characterized by short stature and generalized diffuse osteosclerosis. Patients usually have a large head with separated sutures, open fontanels, aplasia of frontal sinuses, obtuse mandibular gonial angle, and acroosteolysis of the distal phalanges. This case report showed a 25-year-old female with features pathognomonic of pycnodysostosis. The emphasis is mainly on the early diagnosis as it has an important role in the general health of such patients and prevention of complications.
Chronic diffuse sclerosing osteomyelitis (CDSO) is a disease that presents a rather unusual clinicoradiographic picture creating diagnostic and therapeutic problems. Involvement of the temporomandibular joint is not frequently observed in CDSO. We report a unique case of a 37-year-old male with cyclic episodes of insidious swelling and progressive trismus for the past 4 years. Each episode was followed by spontaneous remission after 2 weeks. Radiographic examination detected unilateral coarse trabeculae with ground-glass appearance of the left hemimandible and resorption of ipsilateral condyle. Computed tomography demonstrated endosteal sclerosis, cortical thickening, and condylar resorption, while magnetic resonance imaging revealed altered marrow intensity with postcontrast enhancement of the surrounding musculature. Bone histopathology was consistent with the diagnosis of osteomyelitis. Palliative antibiotic therapy and regular follow-up were recommended. Of particular interest in the present case is ipsilateral condylar resorption not associated with any dermato-skeletal conditions, which is uncommon in CDSO of the mandible.
Various techniques have been described to restore acquired maxillary defects. The aim of this paper is to review the contemporary treatment modalities that have been explored to rehabilitate and reconstruct partial or complete maxillectomy in dentulous and edentulous patients. MEDLINE/PUBMED search was conducted for dental literature published from January 2000 to December 2018. Literature study revealed various methods and materials used to treat patients with acquired maxillary defects. These include modifications in method and materials used for the fabrication of conventional obturators (using precision and semi-precision attachments, telescopic copings, magnets, silicone based materials, titanium and its alloys, PEEK, thermoplastic resin); implant-supported obturators supported on conventional, zygomatic and pterygoid implants using different attachments (ERA, magnet, bar attachment, locator, ball attachment); use of CAD/CAM technology for presurgical planning and fabrication of obturator prosthesis; surgical reconstruction with microvascular free tissue flaps; and bone engineering. Significant attention has been devoted to refining current methods and developing better methods to successfully rehabilitate maxillectomy patients. The use of osseointegrated implants, newer prosthesis materials, advanced laboratory procedures, inclusion of CAD/CAM technology, advances in surgical reconstructive techniques and tissue engineering has revolutionized the traditional treatment options.
International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations.IJCRI publishes Review Articles, Case Series, Case Reports, Case in Images, Clinical Images and Letters to Editor.
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