• Korean Journal of Radiology
• /
• v.1 no.3
• /
• pp.172-174
• /
• 2000

We present a case of medullary sclerosis of the appendicular skeleton in a patient with chronic renal insufficiency for whom MR imaging findings were characteristic. T1- and T2-weighted MR images showed multiple vertical lines (medullary streaks) of low signal intensity in the metaphyses and diaphyses of the distal femur and proximal tibia.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• v.47 no.2
• /
• pp.141-144
• /
• 2021

Osteopathia striata with cranial sclerosis (OS-CS) is a bone dysplasia characterized by a linear striated pattern of sclerosis, especially in the long bones, and cranial sclerosis. It has variable clinical findings but distinctive radiological findings. Multiple oral and dental findings have been associated with this disease and can be seen during dental and/or medical imaging of the head and neck. Dentists and clinicians must be familiar with these signs to differentiate them from pathosis or erroneous radiographs. In the following case, we present a patient with OS-CS that presented at The University of Florida College of Dentistry with multiple craniofacial manifestations of this syndrome that were seen on a panoramic radiograph, which is one of the most commonly requested radiographs by dentists.

• Imaging Science in Dentistry
• /
• v.30 no.2
• /
• pp.117-122
• /
• 2000

Fibrous dysplasia is a benign disorder of bone consisting of intramedullary proliferation of fibrous tissue and irregularly distributed, poorly developed bone. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. We reported a extensive case of polyostotic fibrous dysplasia with involvement of craniofacial bones, mandible, ribs and extremities. A 18-year-old man showed remarkable right facial swelling who had been treated on right femur 3 years ago with a bone graft for pathologic fracture and he recognized facial swelling 5 years ago. Extraoral radiograms and computed tomogram showed diffuse sclerosis with a ground glass appearance of the most cranial bones, facial bones. The right mandibular lesion showed very expansile lesion with mottled appearance. Bone scans showed mutifocal increased uptakes in craniofacial bones, right mandible, bilaterally in ribs, humerus, femur, tibia and characteristic varus deformity of right femur (shepherd's crook defomity). This case showed exceptionally bilateral, extensive nature of bone lesion and didn't show any features of skin pigmentation and endocrine disturbances.

• Imaging Science in Dentistry
• /
• v.48 no.4
• /
• pp.283-287
• /
• 2018

Craniometaphyseal dysplasia (CMD) is a rare hereditary disorder characterized by hyperostosis of the craniofacial bones and flared metaphyses of the long bones. Although some reports have described the dentomaxillofacial characteristics of CMD, including increased density of the jaw, malocclusion, and delayed eruption of the permanent teeth, only a few studies have reported the distinct imaging features of CMD on panoramic radiography. This report presents 2 cases of confirmed CMD patients with an emphasis on panoramic imaging features. The patients' images revealed hyperostosis and sclerosis of the maxilla and mandibular alveolar bone, but there was no change in the mandibular basal bone. In both cases, the mandibular condyle heads exhibited a short clubbed shape with hyperplasia of the coronoid process. For patients without clear otorhinolaryngological symptoms, common radiologic features of CMD could be visualized by routinely-taken panoramic radiographs, and further medical examinations and treatment can be recommended.

• Imaging Science in Dentistry
• /
• v.41 no.4
• /
• pp.177-181
• /
• 2011

Pyknodysostosis is a rare autosomal recessive disorder characterized by the post natal onset of short limbs, short stature, and generalized hyperostosis along with acro-osteolysis with sclerosis of the terminal phalanges, a feature that is considered essentially pathognomonic. Other features include persistence of fontanelles, delayed closure of sutures, wormian bones, absence of frontal sinuses, and obtuse mandibular gonial angle with relative mandibular prognathism. We report a case of 17-year-old girl who presented with a chief complaint of retention of deciduous teeth. General physical examination demonstrated short stature, frontal and parietal bossing, depressed nasal bridge, beaked nose, hypoplastic midface, wrinkled skin over the finger tips, and nail abnormalities. Radiographs showed multiple impacted permanent and supernumerary teeth, hypoplastic paranasal sinuses with acro-osteolysis of terminal phalanges, and open fontanelles, and sutures along with wormian bones in the lambdoidal region.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• v.11 no.2
• /
• pp.61-66
• /
• 1989

Osteopetrosis (Albers-Schonberg disease) is a rare disease characterized by generaized sclerosis of bones, hepatoslenomegaly, pancytopenia, multiple fracture, blindness. The disease shows characteristic radiographic feature and two rather well-defined pattern have been recognized. We experienced a case of osteopetrosis in a 31 year old man, who has had pus discharge and fetid odor after extraction of upper maxillary molar. We made a brief review of literature.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.36 no.4
• /
• pp.619-624
• /
• 2009

Pyknodysostosis(PKND) is a rare sclerosing bone disorder that has an autosomal recessive trait, also known as Toulouse-Lautrec syndrome. Deficiency of the cathepsin enzyme K in the osteoclasts of PKND patients results in continuous endosteal bone deposits without osteoclastic resorption or remodeling. This causes a generalized increase in sclerosis and fragility of bones. Osteomyelitis in the mandible and recurrent fracture of the long bones are characteristic complicatons of PKND. The patients present typical features of PKND, such as short stature under 150 cm, open cranial suture and fontanelle, club-shaped phalanges, and underdevelopment of midface. This is a case of a 7-year-old girl with PKND, who visited our clinic with the chief complaint of anterior Open-bite and generalized crowding. The patient had been diagnosed as PKND by an orthopedist and manifested characteristic clinical and radiographic features, such as open cranial suture and fontanelle, obtuse madibular gonial angle, frontal and occipital bossing, grooved palate, club-shaped phalanges, and short stature. Orthodontic treatment was not considered because patients with PKND show abnormal bone resorption and remodeling. Instead, removal of deciduous teeth near exfoliation and TFA were performed, and periodic check-up is planned to maintain good oral hygiene.