• Korean Journal of Radiology
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• 제1권3호
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• pp.172-174
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• 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
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• 제47권2호
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• pp.141-144
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• 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
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• 제30권2호
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• pp.117-122
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• 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
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• 제48권4호
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• pp.283-287
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• 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
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• 제41권4호
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• pp.177-181
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• 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
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• 제11권2호
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• pp.61-66
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• 1989

Osteopetrosis는 드물게 볼 수 있는 질환으로 일명, $Albers-Sch{\"{o}}nberg$ disease 혹은 Marble bone disease라고도 하며 그 주요한 특징은 전반적인 골경화증, 임파선의 증대, 간비 증대, 범혈구 감소증, 다발성 골절과 실명 등이다. 이 질환은 X-선상에서 독특한 소견을 보이며 임상적으로 악성형과 양성형으로 나눌 수 있다. Dominant form은 양성형으로써 다양하게 표현되어 단독 X-선상에서 이상을 나타내는 것부터 골절과 뇌신경을 침범하기까지 다양하게 나타나며, Recessive form은 악성형으로 자궁내에서 진단되어지고 빈혈이 심하며 골수양화생이 있으며, 감염 특히 하악의 골수염으로 대개는 첫 1년 이내에 사망 한다고 보고되어 있다. 이에 저자들은 개인치과에서 상악 좌측 제2대구치 발거후 골수염이 발생한 31세 남자 골석화증 환자를 치험하였기에 문헌고찰과 함께 보고하는 바이다.

• 대한소아치과학회지
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• 제36권4호
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• pp.619-624
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• 2009

Pyknodysostosis(PKND)는 파골세포의 기능 이상으로 인해 발생하는 상염색체 열성의 매우 드문 경화성 골질환으로 Toulouse-Lautrec syndrome으로 불리기도 한다. PKND의 원인으로는 파골세포 내 cathepsin K의 결핍으로 인해 파골세포의 골개조와 골흡수 기능의 실패가 생기게 되어 연골의 축적과 과도한 골의 광화가 나타나는 것으로 알려져 있는데, 이로 인해 골수염의 위험이 높고 빈번한 골의 파절이 나타난다. 150 cm 이하의 작은 신장, 개방된 천문과 두개골 봉합의 실패, 곤 봉형의 손가락과 중안모의 발달이 저하된 특징적인 안모를 보인다. 본 증례는 전반적인 총생과 전치부 개방교합을 주소로 본과에 내원한 7세 1개월의 여아로, 정형외과에서 PKND로 진단받은 상태였다. 임상 및 방사전 사진 검사상 천문과 두개골 봉합 폐쾌의 실패, 곤봉모양의 손가락, 구개의 고랑,짧은사지와 작은 신장 등의 PKND의 전형적인 임상적 특성을 나타내고 있었다. 비정상적인 골흡수와 골재생기능의 문제로 주소인 총생과 개방교합에 대한 교정적 처치는 시행하지 못했다. 탈락 시기의 유치 발거와 불소도포를 시행한 후 정기 검진을 시행 중인 상태로 PKND에 대한 문헌고찰과 함께 본 증례를 보고하고자 한다.