Diagnostic Diffuse Idiopathic Skeletal Hyperostosis (DISH): Radiology Forestier's Disease with Extraspinal Manifestations1 Donald Resnick, M.D., Stephen R. Shaul, M.D., and Jon M. Robins, M.D. The extraspinal manifestations of Forestier's disease are described in 21 consecutive cases; diffuse idiopathic skeletal hyperostosis (DISH) is suggested as a more appropriate description of this ossifying diathesis. Characteristic roentgen abnormalities of the spine were present in all individuals and associated with significant axial clinical complaints. In extraspinal locations, hyperostosis at ligament attachments usually occurs in the pelvis, calcaneus, tarsal bones, ulnar olecranon and patella, and is occasionally associated with clinical signs and symptoms requiring surgery. The radiographic appearance in the pe­ ripheral skeleton is frequently distinctive and allows the radiologist to suggest the correct diagnosis, even in the absence of axial radiographs. INDEX TERMS: Bones, diseases. Foot • Knee • Pelvis, calcification. Sacroiiiac Joint • Soft Tissues, calcification • Spine, diseases • Ulna Radiology 115:513-524, June 1975 N 1950, Forestier and Rotes Querol (10) described phosphatase, uric acid and glucose, and serologic test­ I a peculiar type of ankylosing hyperostosis of the ing for syphilis, rheumatoid factor and antinuclear anti­ spine characterized by ossification of the anterior and body (ANA). right lateral aspects of the vertebral column, particularly Radiographic Evaluation: A radiographic survey of in the thoracic region. The clinical, pathologic, and each patient included the following projections: roentgenographic features of the disorder allowed its 1. Spine: Anteroposterior (AP) and lateral views differentiation from other spinal diseases including an­ of the cervical, thoracic and lumbar vertebrae. kylosing spondylitis and osteoarthrosis. Although occa­ 2. Pelvis including hips: AP. sional reports describe bony outgrowths in extraspinal 3. Femurs: AP, lateral. locations (8), a systematic radiographic study of the en­ 4. Knees: AP, lateral. tire skeleton in patients with Forestier'sdisease has not 5. Forelegs including ankles: AP, lateral. been accomplished. 6. Heels: lateral. In the first of two articles on the roentgenographic 7. Feet: AP. manifestations of diffuse idiopathic skeletal hyperostosis 8. Humeri including shoulders: AP. (DISH), we wish to emphasize the type and location of 9. Elbows: AP, lateral. extraspinal abnormalities in this disorder, and to relate 10. Forearms: AP. these to clinical and laboratory alterations. A second re­ 11. Hands including wrists: Postero-anterior (PA). port correlating the radiographic and pathologic abnor­ In addition, axial views of the patella and lateral pro- malities of the spine is in progress at this time. jections of the skull were available in some individuals. The osseous alterations were recorded and graded as absent, mild-to-moderate, or moderate-to-severe in de­ gree. MATERIAL AND METHODS II. Pathologic material obtained at autopsy in 10 individuals with spinal or extraspinal manifestations of I. Twenty-one consecutive patients with ankylosing hyperostosis of the spine discovered in the Radiology the disease was investigated. A more detailed descrip­ tion of these alterations in the vertebral column will be Departments, Veterans Administration and University reported in a subsequent article. hospitals, San Diego, underwent extensive clinical, labo­ ratory and roentgenographic evaluation.2 Clinical and Laboratory Evaluation: A complete RESULTS rheumatologic history and physical examination was ob­ Clinical and Laboratory Study (Table I) tained for each patient, including weight, chest expan­ sion, spinal mobility, and tenderness and crepitus over The 21 men studied ranged in age from 49 to 80 axial and peripheral joints. Laboratory determinations in­ years, with a mean age of 66 years. There were two cluded hematocrit, leukocyte count, Westergren sedi­ blacks (CASES 4 and 11); the remainder were white. mentation rate, serum calcium, phosphorus, alkaline Family and geographic histories were nonrewardihg; 11 1 From the Departments of Radiology (D. R.) and Internal Medicine (5. R. 5), University Hospital, University of California. San Diego and the Veterans Administration Hospital, San Diego; and the Department of Radiology (J. M. R.), Alvarado Community Hospital, San Diego, Calif. Re­ vised manuscript accepted for publication in February 1975. shan 2 One patient was included whose films from-a neighboring community hospital were seen in consultation during this period at the Veterans Administration Hospital. shan 513 514 DONALD RESNICK AND OTHERS June 1975 TABLE I TABLE III MAJOR SITES OF CLINICAL INVOLVEMENT RADIOGRAPHIC ABNORMALITIES AGE DURATION IVRSI SXS/SIGNS PT. SEX (VRS) C·SPINE T·L SPINE SHOULDERS ELBOWS KNEES HEELS MISC.SITES SITE NUMBEA OF PATIENTS 1 74M 24 - DPM PM A·P C; L·C - - - SPINE 21 2 85M <1 - -- R·P A·L·P A· P HANDS cervical 16 3 81M 30 P P -- R·C - TIBIAL TUBEROSITY thoracic 21 lumbar 19 4 49M 5 OM P - - - L·HIP;R·AC JOINT - PELVIS 21 5 79M 10 PM - -- - - - FEMUR 3 6 79M ? N.A. N.A. N.A. N.A. N.A. N.A. N.A. KNEE 6 7 79M 4 M P A·P C - L· PM - ~.~~~r,;, A. FORELEG. ANKLE 6 8 56M 32 M P - -- - FEET;R·HIP HEEL 16 9 59M 9 OM PM A·M - - - l·HIP FOOT 15 8 10 12M 44 PM PM A·L·C - A·L·C - HIPS SHOULDER. HUMERUS ELBOW 12 11 51M 12 PM - A·M R·M - - HANDS FOREARM 3 12 51M 18 P P L·C A·L·P - - - HAND. WRIST 8 13 74M ASX - - - - - - - 14 HANDS.WR ISIS. SSM 25 D - - A·L·M L· P - ANKLESSUBTALARS 15 50M 8 DP M A·L·P A·L·P - - R· SI quired a moderate degree of physical activity were a 16 69M 12 PM PM A·L·P M C - R·L·PMC HANDS.R·L·HIP RANKLE. construction worker, 2 ranchers, 2 janitors, a butcher 17 58M I M PM - - - R· P R·SUBTALAR 18 80M 38 - PM A·C - - - - and a roofer. 19 88M 32 PM PM L·C - - - - Eighteen of the 21 patients had a history of significant 20 IBM ASX - - - - - -- 21 11M 2 DPMC PM - - A·L·M - R·SUBTALAR musculoskeletal complaints. Although their duration was A· = AIGHT L· = LEFT ASX = ASYMPTOMATIC N.A. = NOT AVAILA8LE variable, symptoms in many of the patients had been D = DYSPHAGIA C = CAEPITUS M = DECAEASED AANGE OF MOTION P = PAIN OR TENDEANESS 0 or P = INITIAL MANIFESTATION present for longer than 10 years. Mid or low back pain was an initial complaint in 12 patients and an eventual patients had lived in California for the last 10 years or one in 14. It was characteristically dull, non-radiating more. Among the 12 individuals whose occupations re- and associated with "stiffness," varying in severity from TABLE II MAJOR SITES OF RAOIOGRAPHIC INVOLVEMENT SPINE SHOULDERS. HANDS. FEMURS. FORELEGS. PT C·SPINE T·SPINE L-SPINE PELVIS HUMERI ELBOWS FOREARMS WRISTS KNEES ANKLES HEELS FEET 1 + ++ ++ ++ R·L + - - L + - - R-L + R-L + 2 ++ ++ + + - - - L + R-L ++ - L + L ++ 3 ++ ++ + + - R·L + - - - - R-L + - 4 - + + ++ - R-L ++ - -- R-L + R ++;L+ R +;L++ 5 - ++ + + R-L ++ - - R-L + -- R·L + - 6 + ++ ++ ++ - - - - - - - - 7 ++ ++ ++ ++ R +;L ++ R·L ++ R·L + R+;L ++ - L ++ R ++;L + R-L + 8 - ++ ++ ++ - - - --- - R ++ 9 ++ ++ + + L + R·L + - - - L + - R + 10 ++ ++ + + - R·L + -- - R·L + - R·L + - 11 + + + ++ - L + - R·L + - - R-L ++ R·L ++ 12 ++ ++ + + - R·L + - - - - R-L + R-L + 13 + ++ ++ ++ - - - R-L + - - R-L + - 14 - + - ++ - R·L ++ - - R +;L-t-+ R-L ++ - R-L + 15 ++ ++ ++ ++ R·L + R-L ++ -- R-L ++ R ++;L + R-L ++ R·L ++ 16 ++ ++ ++ ++ - R-L + - R-l + R·L ++ - R-L ++ R·L ++ 17 - + + + - - - -- - R ++;L + - 18 + ++ + + R-L + - R-L ++ - - - L ++ R·L + 19 ++ ++ ++ ++ - R-L + - - - - - R·L ++ 20 + + ++ ++ R-L ++ R·L + R +;L ++ R-L + R ++;L + R-L + R·L ++ R-L ++ 21 + + - + R-L + -- - - - R +;L ++ R-L + R ~ RIGHT L = LEFT + = MILD TO MODERATE ABNORMALITY ++ = MODERATE TO SEVERE ABNORMALITY Fig. 1. Spinal Abnormalities. A. Cervical spine: In a lateral radiograph of the cervical spine, extensive ossification along the anterior margins of the vertebral bodies (closed arrows) has resulted in ankylosis. The heights of the discs in the upper cervical region and the apophyseal joint spaces (open arrows) have been relatively maintained (CASE 10). Band C. Thoracic spine: A sagittal section and corresponding radiograph reveal a flowing pattern of new bone deposi­ tion along the anterior margins of the vertebral bodies and disc spaces (closed arrows). Disc-space height is preserved (open arrows) (CASE 2). D. lumbar spine: Osteophytes are most prominent on the anterosuperior margins of the vertebral bodies (closed ar­ rows) and extend proximally to unite with those of the adjacent vertebra. The disc spaces are maintained (open arrows) (CASE 15). Diagnostic Radiology (For legend to Fig. 1, see opposite page.) 515 516 DONALD RESNICK AND OTHERS June 1975 Fig. 2. Pelvic Abnormalities. A. Irregular projections of bone (curved arrows) extend from the iliac crest and the lateral margins of the ilium (CASE 11). B. Alterations include ischial tuberosity (curved arrows) and lesser trochanter "whiskering" (open arrow), and a large para­ acetabular osteophyte (closed straight arrow). Prostatic calcification is apparent (CASE 16). C. Osseous bridging across the superior margin of the symphysis pubis is noted (arrow) (CASE 13). (Fig. 2, D is on opposite page.) mild to incapacitating. Cervical pain, an initial symptom flammatory except in one man (CASE 2) in whom rheu­ in one individual, eventually appeared in 8 others. Cervi­ matoid arthritis apparently developed a short time be­ cal dysphagia was historically present in 6 patients, an fore death (positive rheumatoid factor, symmetrical initial complaint in one, and required surgical removal of proximal interphalangeal joint tenderness and swelling in spinal osteophytes in 2 patients (CASES 1 and 15). the hands). Although peripheral symptoms were the ini­ Peripheral musculoskeletal complaints, significant in tial finding in 4 patients (CASES 2, 7, 14, 17), only 2 indi­ 8 of the men, most commonly involved the shoulders (4 viduals (CASES 2 and 14) had isolated extraspinal symp­ patients), knees (4 patients), elbows (3 patients), and tomatology, and one of these was the patient with prob­ heels (2 patients).