Radiological features of Gorham’s disease

Aim To describe the key findings on plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI) of Gorham’s disease. Materials and methods Eight children diagnosed with Gorham’s disease between 1999 and 2009 were included. All imaging studies performed on each patient were revi...

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Veröffentlicht in:Clinical radiology 2012-08, Vol.67 (8), p.782-788
Hauptverfasser: Kotecha, R, Mascarenhas, L, Jackson, H.A, Venkatramani, R
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container_end_page 788
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container_title Clinical radiology
container_volume 67
creator Kotecha, R
Mascarenhas, L
Jackson, H.A
Venkatramani, R
description Aim To describe the key findings on plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI) of Gorham’s disease. Materials and methods Eight children diagnosed with Gorham’s disease between 1999 and 2009 were included. All imaging studies performed on each patient were reviewed with special attention to the extent of bone, soft tissue, and visceral involvement. Results All patients had bone lesions at diagnosis, most commonly in the vertebrae. CT showed generalized osteopenia, multiple lytic lesions, and heterogeneous bone density. MRI demonstrated altered signal intensity in bone marrow that was hyperintense on T1 imaging. Seven patients had soft-tissue lymphangiomatous lesions adjacent to identified osseous lesions. Four patients had chylous pleural effusions: three with bilateral and one with unilateral involvement. The spleen was involved in six patients. Conclusion Splenic lesions and soft-tissue involvement are common in patients with Gorham’s disease. The presence of extra-osseous lesions along with characteristic bone lesions on plain radiography may be pathognomonic of Gorham’s disease.
doi_str_mv 10.1016/j.crad.2012.01.009
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Materials and methods Eight children diagnosed with Gorham’s disease between 1999 and 2009 were included. All imaging studies performed on each patient were reviewed with special attention to the extent of bone, soft tissue, and visceral involvement. Results All patients had bone lesions at diagnosis, most commonly in the vertebrae. CT showed generalized osteopenia, multiple lytic lesions, and heterogeneous bone density. MRI demonstrated altered signal intensity in bone marrow that was hyperintense on T1 imaging. Seven patients had soft-tissue lymphangiomatous lesions adjacent to identified osseous lesions. Four patients had chylous pleural effusions: three with bilateral and one with unilateral involvement. The spleen was involved in six patients. Conclusion Splenic lesions and soft-tissue involvement are common in patients with Gorham’s disease. The presence of extra-osseous lesions along with characteristic bone lesions on plain radiography may be pathognomonic of Gorham’s disease.</description><identifier>ISSN: 0009-9260</identifier><identifier>EISSN: 1365-229X</identifier><identifier>DOI: 10.1016/j.crad.2012.01.009</identifier><identifier>PMID: 22424931</identifier><identifier>CODEN: CLRAAG</identifier><language>eng</language><publisher>Amsterdam: Elsevier Ltd</publisher><subject>Adolescent ; Biological and medical sciences ; Child ; Diseases of the osteoarticular system ; Female ; Follow-Up Studies ; Humans ; Investigative techniques, diagnostic techniques (general aspects) ; Lung Diseases - complications ; Lung Diseases - diagnosis ; Lung Diseases - diagnostic imaging ; Magnetic Resonance Imaging - methods ; Male ; Medical sciences ; Miscellaneous. Osteoarticular involvement in other diseases ; Osteolysis, Essential - complications ; Osteolysis, Essential - diagnosis ; Osteolysis, Essential - diagnostic imaging ; Pleural Effusion - complications ; Pleural Effusion - diagnosis ; Pleural Effusion - diagnostic imaging ; Radiology ; Radionuclide Imaging ; Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects) ; Splenic Diseases - complications ; Splenic Diseases - diagnosis ; Splenic Diseases - diagnostic imaging ; Tomography, X-Ray Computed - methods</subject><ispartof>Clinical radiology, 2012-08, Vol.67 (8), p.782-788</ispartof><rights>The Royal College of Radiologists</rights><rights>2012 The Royal College of Radiologists</rights><rights>2015 INIST-CNRS</rights><rights>Copyright © 2012 The Royal College of Radiologists. Published by Elsevier Ltd. 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Materials and methods Eight children diagnosed with Gorham’s disease between 1999 and 2009 were included. All imaging studies performed on each patient were reviewed with special attention to the extent of bone, soft tissue, and visceral involvement. Results All patients had bone lesions at diagnosis, most commonly in the vertebrae. CT showed generalized osteopenia, multiple lytic lesions, and heterogeneous bone density. MRI demonstrated altered signal intensity in bone marrow that was hyperintense on T1 imaging. Seven patients had soft-tissue lymphangiomatous lesions adjacent to identified osseous lesions. Four patients had chylous pleural effusions: three with bilateral and one with unilateral involvement. The spleen was involved in six patients. Conclusion Splenic lesions and soft-tissue involvement are common in patients with Gorham’s disease. The presence of extra-osseous lesions along with characteristic bone lesions on plain radiography may be pathognomonic of Gorham’s disease.</description><subject>Adolescent</subject><subject>Biological and medical sciences</subject><subject>Child</subject><subject>Diseases of the osteoarticular system</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Humans</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Lung Diseases - complications</subject><subject>Lung Diseases - diagnosis</subject><subject>Lung Diseases - diagnostic imaging</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Miscellaneous. Osteoarticular involvement in other diseases</subject><subject>Osteolysis, Essential - complications</subject><subject>Osteolysis, Essential - diagnosis</subject><subject>Osteolysis, Essential - diagnostic imaging</subject><subject>Pleural Effusion - complications</subject><subject>Pleural Effusion - diagnosis</subject><subject>Pleural Effusion - diagnostic imaging</subject><subject>Radiology</subject><subject>Radionuclide Imaging</subject><subject>Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects)</subject><subject>Splenic Diseases - complications</subject><subject>Splenic Diseases - diagnosis</subject><subject>Splenic Diseases - diagnostic imaging</subject><subject>Tomography, X-Ray Computed - methods</subject><issn>0009-9260</issn><issn>1365-229X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kd9qFDEUh0NR2m3tC_Si7I0glJnmZGYyGxBBilahIPgHvAtnMieadXZSc3aE3vkavl6fxAy7VvDCq5OE73cI30-IM5AlSNCX69Il7EslQZUSSinNgVhApZtCKfP5kVjI_FQYpeWROGZez9da1YfiSKk8TQULcfEe-xCH-CU4HJaecDsl4mX0y-uYvuLm_ucvXvaBCZmeiMceB6bT_TwRn16_-nj1prh5d_326uVN4eoatkXXGt86kl6vHFU91I3uXEurjlpABJRupaFu0ZMxRuMKNDmD0nemqVRTd9WJeLbbe5vi94l4azeBHQ0DjhQntiBVpYzWTZNRtUNdisyJvL1NYYPpLkN2lmTXdpZkZ0lWgs0Kcuh8v3_qNtQ_RP5YycDTPYCctfiEowv8l9Og2rauMvd8x1G28SNQsuwCjY76kMhtbR_D___x4p-4G8I4F_GN7ojXcUpj9mzBcs7YD3N_c5ug8km3svoN-Q-Zew</recordid><startdate>20120801</startdate><enddate>20120801</enddate><creator>Kotecha, R</creator><creator>Mascarenhas, L</creator><creator>Jackson, H.A</creator><creator>Venkatramani, R</creator><general>Elsevier Ltd</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20120801</creationdate><title>Radiological features of Gorham’s disease</title><author>Kotecha, R ; Mascarenhas, L ; Jackson, H.A ; Venkatramani, R</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c441t-b79f7ce0f68ce3d1456bc7e8be71aa1a0c86147afe9996a816ec9a0fb953254b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Adolescent</topic><topic>Biological and medical sciences</topic><topic>Child</topic><topic>Diseases of the osteoarticular system</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Humans</topic><topic>Investigative techniques, diagnostic techniques (general aspects)</topic><topic>Lung Diseases - complications</topic><topic>Lung Diseases - diagnosis</topic><topic>Lung Diseases - diagnostic imaging</topic><topic>Magnetic Resonance Imaging - methods</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Miscellaneous. Osteoarticular involvement in other diseases</topic><topic>Osteolysis, Essential - complications</topic><topic>Osteolysis, Essential - diagnosis</topic><topic>Osteolysis, Essential - diagnostic imaging</topic><topic>Pleural Effusion - complications</topic><topic>Pleural Effusion - diagnosis</topic><topic>Pleural Effusion - diagnostic imaging</topic><topic>Radiology</topic><topic>Radionuclide Imaging</topic><topic>Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects)</topic><topic>Splenic Diseases - complications</topic><topic>Splenic Diseases - diagnosis</topic><topic>Splenic Diseases - diagnostic imaging</topic><topic>Tomography, X-Ray Computed - methods</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kotecha, R</creatorcontrib><creatorcontrib>Mascarenhas, L</creatorcontrib><creatorcontrib>Jackson, H.A</creatorcontrib><creatorcontrib>Venkatramani, R</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical radiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kotecha, R</au><au>Mascarenhas, L</au><au>Jackson, H.A</au><au>Venkatramani, R</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Radiological features of Gorham’s disease</atitle><jtitle>Clinical radiology</jtitle><addtitle>Clin Radiol</addtitle><date>2012-08-01</date><risdate>2012</risdate><volume>67</volume><issue>8</issue><spage>782</spage><epage>788</epage><pages>782-788</pages><issn>0009-9260</issn><eissn>1365-229X</eissn><coden>CLRAAG</coden><abstract>Aim To describe the key findings on plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI) of Gorham’s disease. Materials and methods Eight children diagnosed with Gorham’s disease between 1999 and 2009 were included. All imaging studies performed on each patient were reviewed with special attention to the extent of bone, soft tissue, and visceral involvement. Results All patients had bone lesions at diagnosis, most commonly in the vertebrae. CT showed generalized osteopenia, multiple lytic lesions, and heterogeneous bone density. MRI demonstrated altered signal intensity in bone marrow that was hyperintense on T1 imaging. Seven patients had soft-tissue lymphangiomatous lesions adjacent to identified osseous lesions. Four patients had chylous pleural effusions: three with bilateral and one with unilateral involvement. The spleen was involved in six patients. Conclusion Splenic lesions and soft-tissue involvement are common in patients with Gorham’s disease. 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subjects Adolescent
Biological and medical sciences
Child
Diseases of the osteoarticular system
Female
Follow-Up Studies
Humans
Investigative techniques, diagnostic techniques (general aspects)
Lung Diseases - complications
Lung Diseases - diagnosis
Lung Diseases - diagnostic imaging
Magnetic Resonance Imaging - methods
Male
Medical sciences
Miscellaneous. Osteoarticular involvement in other diseases
Osteolysis, Essential - complications
Osteolysis, Essential - diagnosis
Osteolysis, Essential - diagnostic imaging
Pleural Effusion - complications
Pleural Effusion - diagnosis
Pleural Effusion - diagnostic imaging
Radiology
Radionuclide Imaging
Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects)
Splenic Diseases - complications
Splenic Diseases - diagnosis
Splenic Diseases - diagnostic imaging
Tomography, X-Ray Computed - methods
title Radiological features of Gorham’s disease
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