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Dysplasia epiphysealis hemimelica: radiographic and magnetic resonance imaging features and clinical outcome of complete and incomplete resection

Objectives The objectives of this communication were to discuss radiographic and magnetic resonance (MR) imaging manifestations and clinical outcome after complete and incomplete resection of the mass of dysplasia epiphysealis hemimelica (DEH). Materials and methods Clinical records, radiographs, an...

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Published in:Skeletal radiology 2010, Vol.39 (1), p.85-90
Main Authors: Bahk, Won-Jong, Lee, Han-Yong, Kang, Yong-Koo, Park, Jung-Mi, Chun, Kyeong-A, Chung, Yang-Guk
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description Objectives The objectives of this communication were to discuss radiographic and magnetic resonance (MR) imaging manifestations and clinical outcome after complete and incomplete resection of the mass of dysplasia epiphysealis hemimelica (DEH). Materials and methods Clinical records, radiographs, and MR images of eight patients with DEH were retrospectively examined. Six patients were treated by complete excision of the lesional mass, and two patients were treated by incomplete resection at our University Hospitals during the period from 1980 to 2006. Results We found that, unlike in osteochondroma, DEH was radiographically not clearly separable from the underlying or host bone with preserved cortical bone and marrow continuity. The finding in the talus distinguished DEH from (osteochondroma-like) parosteal osteosarcoma, in which a radiolucent demarcation line clearly separated the tumor from the host bone. The DEH mass had a well-defined low to intermediate signal intensity on T1-weighted images and an intermediate to high signal intensity on T2-weighted images, with irregularity of the articular surface. Simple excision was performed in all patients. The excision was complete in six patients and incomplete in two patients whose lesions was juxta-articular in the ankle and articular in the knee, respectively. The residual mass slowly absorbed and vanished, resulting in mild flaring of the affected portion of the epiphysis. No local recurrence or complication was seen in any of the eight patients. Conclusions Although the radiographic signs of DEH are characteristic, (osteochondroma-like) parosteal osteosarcoma should be differentiated from DEH when there is a radiolucent separation line between the mass and host bone in the talus. Simple excision was effective in the management of DEH if the deformity was not complicated. Incompletely excised masses resolved and vanished with time.
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Materials and methods Clinical records, radiographs, and MR images of eight patients with DEH were retrospectively examined. Six patients were treated by complete excision of the lesional mass, and two patients were treated by incomplete resection at our University Hospitals during the period from 1980 to 2006. Results We found that, unlike in osteochondroma, DEH was radiographically not clearly separable from the underlying or host bone with preserved cortical bone and marrow continuity. The finding in the talus distinguished DEH from (osteochondroma-like) parosteal osteosarcoma, in which a radiolucent demarcation line clearly separated the tumor from the host bone. The DEH mass had a well-defined low to intermediate signal intensity on T1-weighted images and an intermediate to high signal intensity on T2-weighted images, with irregularity of the articular surface. Simple excision was performed in all patients. The excision was complete in six patients and incomplete in two patients whose lesions was juxta-articular in the ankle and articular in the knee, respectively. The residual mass slowly absorbed and vanished, resulting in mild flaring of the affected portion of the epiphysis. No local recurrence or complication was seen in any of the eight patients. Conclusions Although the radiographic signs of DEH are characteristic, (osteochondroma-like) parosteal osteosarcoma should be differentiated from DEH when there is a radiolucent separation line between the mass and host bone in the talus. Simple excision was effective in the management of DEH if the deformity was not complicated. Incompletely excised masses resolved and vanished with time.</description><identifier>ISSN: 0364-2348</identifier><identifier>EISSN: 1432-2161</identifier><identifier>DOI: 10.1007/s00256-009-0803-x</identifier><identifier>PMID: 19813010</identifier><identifier>CODEN: SKRADI</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Adolescent ; Biological and medical sciences ; Care and treatment ; Case Report ; Child ; Child, Preschool ; Clinical outcomes ; Diseases of the osteoarticular system ; Dysplasia ; Epiphyses - diagnostic imaging ; Epiphyses - surgery ; Female ; Fibrous Dysplasia of Bone - diagnostic imaging ; Fibrous Dysplasia of Bone - surgery ; Humans ; Imaging ; Investigative techniques, diagnostic techniques (general aspects) ; Magnetic Resonance Imaging ; Male ; Medical sciences ; Medicine &amp; Public Health ; Nuclear Medicine ; Orthopedics ; Osteoarticular system. Muscles ; Pathology ; Patient outcomes ; Postoperative Complications ; Radiodiagnosis. Nmr imagery. 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Materials and methods Clinical records, radiographs, and MR images of eight patients with DEH were retrospectively examined. Six patients were treated by complete excision of the lesional mass, and two patients were treated by incomplete resection at our University Hospitals during the period from 1980 to 2006. Results We found that, unlike in osteochondroma, DEH was radiographically not clearly separable from the underlying or host bone with preserved cortical bone and marrow continuity. The finding in the talus distinguished DEH from (osteochondroma-like) parosteal osteosarcoma, in which a radiolucent demarcation line clearly separated the tumor from the host bone. The DEH mass had a well-defined low to intermediate signal intensity on T1-weighted images and an intermediate to high signal intensity on T2-weighted images, with irregularity of the articular surface. Simple excision was performed in all patients. The excision was complete in six patients and incomplete in two patients whose lesions was juxta-articular in the ankle and articular in the knee, respectively. The residual mass slowly absorbed and vanished, resulting in mild flaring of the affected portion of the epiphysis. No local recurrence or complication was seen in any of the eight patients. Conclusions Although the radiographic signs of DEH are characteristic, (osteochondroma-like) parosteal osteosarcoma should be differentiated from DEH when there is a radiolucent separation line between the mass and host bone in the talus. Simple excision was effective in the management of DEH if the deformity was not complicated. Incompletely excised masses resolved and vanished with time.</description><subject>Adolescent</subject><subject>Biological and medical sciences</subject><subject>Care and treatment</subject><subject>Case Report</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Clinical outcomes</subject><subject>Diseases of the osteoarticular system</subject><subject>Dysplasia</subject><subject>Epiphyses - diagnostic imaging</subject><subject>Epiphyses - surgery</subject><subject>Female</subject><subject>Fibrous Dysplasia of Bone - diagnostic imaging</subject><subject>Fibrous Dysplasia of Bone - surgery</subject><subject>Humans</subject><subject>Imaging</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Medicine &amp; Public Health</subject><subject>Nuclear Medicine</subject><subject>Orthopedics</subject><subject>Osteoarticular system. 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Materials and methods Clinical records, radiographs, and MR images of eight patients with DEH were retrospectively examined. Six patients were treated by complete excision of the lesional mass, and two patients were treated by incomplete resection at our University Hospitals during the period from 1980 to 2006. Results We found that, unlike in osteochondroma, DEH was radiographically not clearly separable from the underlying or host bone with preserved cortical bone and marrow continuity. The finding in the talus distinguished DEH from (osteochondroma-like) parosteal osteosarcoma, in which a radiolucent demarcation line clearly separated the tumor from the host bone. The DEH mass had a well-defined low to intermediate signal intensity on T1-weighted images and an intermediate to high signal intensity on T2-weighted images, with irregularity of the articular surface. Simple excision was performed in all patients. The excision was complete in six patients and incomplete in two patients whose lesions was juxta-articular in the ankle and articular in the knee, respectively. The residual mass slowly absorbed and vanished, resulting in mild flaring of the affected portion of the epiphysis. No local recurrence or complication was seen in any of the eight patients. Conclusions Although the radiographic signs of DEH are characteristic, (osteochondroma-like) parosteal osteosarcoma should be differentiated from DEH when there is a radiolucent separation line between the mass and host bone in the talus. Simple excision was effective in the management of DEH if the deformity was not complicated. Incompletely excised masses resolved and vanished with time.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>19813010</pmid><doi>10.1007/s00256-009-0803-x</doi><tpages>6</tpages></addata></record>
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subjects Adolescent
Biological and medical sciences
Care and treatment
Case Report
Child
Child, Preschool
Clinical outcomes
Diseases of the osteoarticular system
Dysplasia
Epiphyses - diagnostic imaging
Epiphyses - surgery
Female
Fibrous Dysplasia of Bone - diagnostic imaging
Fibrous Dysplasia of Bone - surgery
Humans
Imaging
Investigative techniques, diagnostic techniques (general aspects)
Magnetic Resonance Imaging
Male
Medical sciences
Medicine & Public Health
Nuclear Medicine
Orthopedics
Osteoarticular system. Muscles
Pathology
Patient outcomes
Postoperative Complications
Radiodiagnosis. Nmr imagery. Nmr spectrometry
Radiography
Radiology
Retrospective Studies
Surgery
Treatment Outcome
Tumors of striated muscle and skeleton
title Dysplasia epiphysealis hemimelica: radiographic and magnetic resonance imaging features and clinical outcome of complete and incomplete resection
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