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). In one man with elbow pain (CASE able rheumatoid arthritis. 15), steroid injections and spur removal had been ac­ The most prominent physical finding was decreased complished, while in another patient (CASE 12), epicon­ range of motion in the low back in 10 patients and in the dylitis was successfully treated with local steroids. One cervical spine in 11 patients. The degree of chest ex­ man (CASE 8) had left carpal tunnel syndrome. The pat­ pansion, averaging 2.3 em in this group, correlated well tern of "polyarthralgia" noted in these men was nonin- with the range of motion of the lumbar spine. Dimin- Diagnostic Vol. 115 DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS 517 Radiology

ished range of motion in the peripheral skeleton was ap­ parent in the hips (5 patients), subtalar joints (4 pa­ tients), shoulders (3 patients), knees (3 patients), elbows (2 patients), and ankles (2 patients). One man (CASE 14), had palpable firm, nodular masses adherent to the quadriceps and patellar tendons. Other physical findings included relative obesity (6 pa­ tients) (2), Dupuytren's contractures (2 patients), and neurological deficits (2 patients). No historical or clinical evidence of acromegaly or fluorosis was noted. Laboratory abnormalities included mild elevation of sedimentation rates (6 patients), and alterations in fast­ ing and two-hour post-prandial serum glucose levels and/or glucose tolerance tests suggesting diabetes mel­ litus in 6 others. Rheumatoid factors were positive in 2 of the men. The remainder of the laboratory evaluation was unrewarding.

Radiographic Study (Tables /I and III) Spine: All 21 men had abnormalities of the spine; in 15, cervical, thoracic and lumbar segments of the ver­ tebral column were involved. Sixteen individuals had alterations in the cervical spine; these most frequently involved the fourth through seventh cervical levels. Abnormalities of the third cervi­ cal vertebra were less common and involvement of the second and first, unusual. Hyperostosis varied from an ill-defined increase in density or well-defined triangular outgrowth in front of the disc to a more extensive flow-' ing pattern of new bone formation (Fig. 1, A); mild-to­ moderate abnormalities were present in 7 individuals and moderate-to-severe changes were seen in 9. Fig. 2. Pelvic Abnormalities. D. The iliolumbar (straight Twenty-one patients had alterations in the thoracic closed arrows) and sacrotuberous ligaments (curved arrow) are ossified. A para-articular osteophyte along the inferior portion of the spine, of a mild-to-moderate degree in 6, and moderate­ sacroiliac joint is seen (open arrows) (CASE 19). to-severe in 15. The most commonly involved levels were in the lower thoracic region, particularly between the absence of outgrowths along the posterior aspects the seventh and eleventh vertebrae although involve­ of the vertebral body were noteworthy. Extension of the ment of most of the thoracic region was evident in 9 pa­ anterior exostosis to the right side of the spine was fre­ tients. The radiographic pattern was distinctive at the quent, particularly in the lower thoracic segment. Asso­ thoracic level, with laminated new bone formation along ciated ligamentous ossification, particularly the ligamen­ the anterior aspects of the vertebral body and disc tum nuchae in the cervical region, and supraspinous space (Fig. 1, B and C). The hyperostosis was generally and costotransverse ligaments were noted in several continuous throughout the area of involvement although patients. in 2 individuals, two abnormal segments were separated Pelvis: Radiographic abnormalities of the pelvis by an area of normality. were demonstrated in all 21 patients. Sites of involve­ Nineteen patients had abnormalities at the lumbar ment included the iliac crest in 14, ischial tuberosities in level which were mild-to-moderate in 10 men and mod­ 11, iliolumbar ligaments in 10, lesser trochanters in 9, erate-to-severe in 9. The bony outgrowths, which varied greater trochanters in 8, acetabular margins in 7, sacro­ from small, triangular projections extending superiorly iliac joints in 4, sacrotuberous ligaments in 4, and the from the anterosuperior edges of the vertebral body to symphysis pubis in 2. Alterations were usually symmet­ large, irregular osteophytes bridging one or several lev­ rical and included irregular outgrowths or whiskering els, showed little predilection for any particular lumbar (iliac crest, ischial tuberosities, trochanters) (Fig. 2, A segment (Fig. 1, D). and B), and broad, well-defined para-articular osteo­ In all segments of the spine, preservation of disc phytes (acetabular margins, sacroiliac joints, symphysis height was distinctive; in the lumbar level in 2 individu­ pubis) (Fig. 2, B and C). Extensive ossification in the ilio­ als, slight loss of disc space and vacuum phenomena lumbar and sacrotuberous ligaments was distinctive were noted. The integrity of the apophyseal joints and (Fig. 2, D). 518 DONALD RESNICK AND OTHERS June 1975

Fig. 3. Upper Extremity Abnormalities. A. Prominence of the deltoid tuberosity (curved arrow) and periosteal new bone formation on the medial humeral shaft (straight ar­ rows) are evident (CASE 20). B. Lateral radiograph of the elbow outlines the large olecranon spur (straight arrow). Osteoarthritic spurs are also noted (curved arrows) (CASE 15). C. Peculiar outgrowths on several metacarpals have appeared (straight arrows); similar but smaller excrescences are noted in the proximal phalanges (curved arrow) (CASE 7).

The sacroiliac para-articular ossifications favored the als (bilateral in 11) included olecranon spurs (10 pa­ inferior margin (Fig. 2, D); in addition, indistinctness of tients) and hyperostosis along the distal medial humerus the superior portion of the space between the ilium and (2 patients). The former were frequently large, well-de­ sacrum was noted. In one patient, complete bridging of fined, and extended from the olecranon in a superior di­ the fibrocartilaginous joint about the pubis was noted rection (Fig. 3, B). (Fig. 2, C). Forearms: Irregularities at the site of attachment of Shoulders and Humeri: Abnormalities were evident the interosseous membrane along apposing surfaces of in8 patients and were bilateral in 7. Prominence and the radius and ulna were bilaterally evident in 3 patients bony irregularity were observed along the deltoid tuber­ and pronounced in 2. osity in 4 patients, medial humeral shaft was seen in 2 Hands and Wrists: Abnormalities in these sites, patients, inferior glenoid in 3, and inferior distal clavicle noted in 8 patients, were bilateral h, 6. Hyperostosis and coracoclavicular ligament in 2 of the men (Fig. 3, along the distal lateral radius was demonstrated in 4 pa­ A). tients, metacarpal and phalangeal heads in 3, and ter­ Elbows: Alterations about the elbows in 12 individu- minal tufts were seen in 5 patients (Fig. 3, C). In the lat- Diagnostic Radiology

Fig. 4. Lower Extremity Abnormalities. A. Cloud-like new bone formation along a segment of the medial femur (arrows) may be seen (CASE 20). Band C. Photograph and radiograph of the knee reveal extensive calcification of the quadriceps and patellar tendons which has produced a large soft-tissue mass (arrows) (CASE 14). D. An axial view of the patella outlines anterior hyperostosis (arrow) (CASE 16). E. Note the osteophyte extending from the proximal tibia to the fibular head (straight arrow) and hyperostosis of the lateral tibial shaft (curved arrow) (CASE 15). F. Bony excrescences on the distal lateral tibia (straight arrow) and medial malleolus (curved arrow) may be noted (CASE 15). 519 520 DONALD RESNICK AND OTHERS June 1975

Fig. 5. Heel and Foot Abnormalities. A. Calcaneal spurs along posterior and inferior surfaces (straight closed arrows) are associated with ossification adjacent to the cuboid and base of the fifth metatarsal (curved arrows). A small osteophyte on the dorsum of the navicular is apparent (open arrow) (CASE 16). B. Sagittal section of the heel demonstrates calcaneal spurs incorporated into the Achilles tendon (straight arrow) and plantar aponeurosis (curved arrow). C. Hyperostosis in the dorsum of the foot has produced a large talar beak (curved arrow), cortical thickening of the tarsal na­ vicular and a small osteophyte Of the cuneiform (straight arrows) (CASE 19). D. Peculiar new bone formation on the medial navicular (arrow) is typical (CASE 15). ter location, mild overgrowth of the tufts similar to that Forelegs: Abnormalities, bilateral in 4 and unilateral occurring in acromegaly was evident. Some promi­ in 2, were noted in 6 patients, particularly involving the nence of the volar diaphyseal ridges of the proximal and proximal fibula, bony attachments of the interosseous middle phalanges could be noted, but integrity of the ar­ membrane, proximal medial tibia, and ankle. Osseous ticular structures of the hand and wrist was maintained. bridging between the lateral proximal tibia and fibular Femurs and Knees: Hyperostosis along the posteri­ head was seen in 2 patients (Fig. 4, E). About the ankle, or or medial femur was seen in 3 patients. It was cloud­ hyperostosis was most extensive on apposing surfaces like in 2 and well defined in the third (Fig. 4, A). of the tibia and fibula (Fig. 4, F). Bilateral ligamentous ossification about the knee was Heels: Calcaneal spurs, evident in 16 patients, were evident in 6 individuals. Predilection for the superior bilateral in 14 and involved the posterior aspect of 27 margin of the patella at the quadriceps mechanism was calcanea and the inferior aspect of 18; plantar and pos­ noted; inferior pole patellar abnormalities were less fre­ terior spurs were evident in 15 heels. They were fre­ quent. In one man, extensive calcification along the en­ quently large and irregular although well defined (Fig. 5, tire quadriceps mechanism on the right side produced A and B). Associated calcification in the plantar liga­ distinctive clinical and radiographic findings (Fig. 4, 8 ments was noted in one individual. and C). Irregularities of the tibial tuberosity were seen in Feet: Hyperostotic abnormalities of the feet, present 2 patients. In several individuals in whom axial views of in 15 patients, were bilateral in 12. They were most the patella were available, irregular whiskering on the common on the dorsum of the mid and hindfoot (9 pa­ anterior surface of this bone was noted (Fig. 4, D). tients) where outgrowths along the talus and navicular Diagnostic Vol. 115 DIFFUSE IDIOPATHIC SKElETAL HYPEROSTOSIS 521 Radiology

Fig. 6. Spectrum of Abnormality in One Patient (CASE 15). A. Anterior "flowing" osteophytes extending from the fourth through the seventh cervical vertebrae (straight arrows). Smaller outgrowths are apparent on the second and third vertebrae (curved arrows). B. An "armor" of new bone is deposited along most of the thoracic spine (arrows). Note the integrity of the disc spaces. C. Excrescences on the anterior and inferior patella (arrows) are seen. D. Note the prominent calcaneal spurs and similar outgrowth on the dorsum of the foot and on the fifth metatarsal (ar­ rows). were noted (Fig. 5, C). Large talar beaks were seen in 4 5, D). Occasionally, similar abnormalities of the cuboid patients (Fig. 5, C). Hyperostosis along the medial navic­ were present (Fig. 5, A). Additional sites of hyperostosis ular and cuneiform were not infrequent (5 patients) (Fig. in the feet included the base of the fifth metatarsal (7 522 DONALD RESNICK AND OTHERS June 1975

patients) (Fig. 5, A) and the phalanges (3 patients). suit in flowing ankylosis of a few or several vertebrae. In Skull: Radiographs of the skull were available in 7 of the thoracic region, the pattern is linear or laminated, the 21 patients. Mild hyperostosis frontalis interna was and can produce anterior ankylosis extending over a noted in 3 of the men; no other abnormalities of the cra­ longer segment. The integrity of the disc spaces and ap­ nium could be appreciated. ophyseal joints is maintained, and allows differentiation from ankylosing spondylitis and disc degeneration. CASE REPORT The extraspinal manifestations of this disease have not been emphasized or systematically studied but ac­ CASE 15. This 50-year-old Caucasian man, a retired office work­ cording to our observations, they are frequent and simi­ er, presented at the Veterans Administration Hospital in October larly distinctive. Particularly prominent are abnormalities 1973 for evaluation of arthritis. His current difficulties began in 1966 in the following sites: when he experienced neck pain radiating down his left upper arm. Radiographs of the cervical spine revealed "degenerative" changes Pelvis: Osseous or soft-tissue radiographic abnor­ and shoulder x rays demonstrated calcific tendonitis. The patient ex­ malities, present in all 21 patients in this series, have perienced symptomatic improvement following physical therapy and occasionally been reported (6, 9, 15). Osseous "whisk­ no medication was required. In 1968 he noted cervical dysphagia, ering" at sites of ligament attachments (iliac crest, is­ particularly with solid foods, and repeat radiographs showed exten­ chial tuberosity, trochanters), not unlike that occurring in sive anterior cervical osteophytes which encroached upon a barium­ ankylosing spondylitis, and ligament ossification are fre­ filled esophagus. Multiple spurs were surgically removed. Six months quent. The latter has a predilection for the iliolumbar later, right elbow pain and tenderness were evaluated, the radio­ graphs outlining a large olecranon osteophyte which was also re­ and sacrotuberous ligaments. Para-articular osteo­ moved at surgery, with symptomatic relief. Five months prior to his phytes along the inferior aspect of the sacroiliac joint current evaluation, the patient noted the acute onset of right elbow (6), lateral acetabular (8), and superior pubic margins (9) pain, redness, and swelling which responded to local steroid injec­ may apparently restrict motion (17), produce para-artic­ tions. At the time of his initial presentation, the patient's complaints ular osseous bridging and, on rare occasions, intra-ar­ included occasional neck pain and stiffness and left shoulder and ticular bony ankylosis (6). The latter was not apparent in elbow discomfort, with numbness of the hands. our series; in fact, the integrity of the joint space adja­ There was no familial history of diabetes or arthritis. The patient had lived in western Pennsylvania for 27 years and in California for cent to the exostosis was frequently striking. the last 5 years. Heel: Spurs on the posterior and/or inferior surface On physical examination, he was 6 feet (180 cm) tall and weighed of the calcaneus were common, and occasionally have 230 pounds (104 kg). Dental evaluation was unremarkable. Chest been noted in previous reports (8). They occurred in 16 expansion was 2.0 cm. There was decreased range of motion in the of our 21 patients (76 %); evaluation of the calcaneus in thoracic and lumbar spine, particularly in flexion. The right sacroiliac "controls" indicates spur formation in 16% (1). The cal­ joint was tender to palpation. Neurologic evaluation was normal. Laboratory values were normal for serum electrolytes. The fasting caneal outgrowths in our patients were of variable size, blood sugar was 114 mgt100 ml. A two-hour postprandial serum but were frequently large. Occasionally, multiple spurs sugar was 164 mgt 100 ml with 4+ glucosuria noted at one and two on either surface of the calcaneus were apparent. They hours. Serum rheumatoid and ANA titers were negative. Radio­ were well defined, without adjacent reactive sclerosis or graphs revealed extensive anterior osteophytes of the cervical (Fig. erosions, and differed from the calcaneal spurs which S, A), thoracic (Fig. 6, B), and lumbar spine (Fig. 1, D). Moderate accompany the granulomatous arthritides (4). The cal­ "whiskering" along the iliac crests was noted. Mild cortical irregulari­ caneal outgrowths were at the attachments of the ties on the inferior aspect of the distal right and left clavicles and bi­ lateral extensive olecranon spurs were apparent (Fig. 3, B). Osseous Achilles tendon and plantar aponeurosis. A similar infe­ outgrowths were also seen about both knees (Fig. 6, C), forelegs rior or plantar spur has been noted in obese men (17); it (Fig. 4, E), ankles (Fig. 4, F), and feet (Figs. 5, D and 6, D). Large cal­ may result from mechanical stress on the longitudinal caneal spurs were also revealed (Fig. 6, D). arch of the foot. All 6 obese patients in our series had Treatment with aspirin brought clinical improvement. In January calcaneal spurs; they were bilateral in 5, and on the 1974, a steroid injection was administered for left shoulder bursitis plantar surface in 9 of the 12 calcanea. but the pain recurred. He has been taking 6.to 8 aspirin tablets each day and intermittently wears a cervical collar for neck pain. Foot: Distinctive bony outgrowths occurred along the dorsal surface of the talus, dorsal and medial tarsal, DISCUSSION navicular, and lateral and plantar portions of the cuboid and base of the fifth metatarsal. At the latter sites, they In the classic descriptions of ankylosing hyperostosis may represent calcification in the plantar aponeurosis of the spine (8, 10), distinctive radiographic abnormali­ or resemble variations of the peroneal sesamoid (17). ties are emphasized. Bony outgrowths, predominating in Large talar "beaks" simulate those occurring with tarsal the thoracolumbar area, form along the anterior and coalition (5) or athletic injuries (17); special radiographic right lateral aspects of the vertebral column; their evolv­ projections to detect talocalcaneal fusion were not ing roentgenographic pattern differs somewhat accord­ available in our 4 patients with talar beaking. ing to their level of development (8). In the cervical and Elbow: Olecranon spurs are frequent, well defined, lumbar regions, early thickening of the bone along the and occasionally of considerable size. anterior surface of the vertebral bodies eventually be­ Other Sites: Although scattered osseous outgrowths comes pronounced and spur-like and may ultimately re- occur at other sites of ligament attachment in the body, Diagnostic Vol. 115 DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS 523 Radiology

they are particularly striking about the patella (24) and ance of DISH. Additionally, digital clubbing, absence of distal tibia and fibula. In the former location, they are spinal involvement and the frequent presence of pulmo­ noted on the superior and inferior margins of the patella; nary pathology allow accurate diagnosis of hypertrophic axial views may identify peculiar hyperostosis of this osteoarthropathy. bone (27). Widespread calcification of the quadriceps It would appear that diffuse idiopathic skeletal hyper­ and patellar tendons occurred in one of our patients; ostosis is a common "ossifying diathesis" occurring in clinical tendinitis was apparent in 3 individuals. Addition­ middle-aged and elderly patients in which bony hyperos­ ally, exostosis about the shoulder (8), knee and phalan­ tosis results at tendon and ligament attachments; al­ ges or metacarpals (11), and hyperostosis of the skull though such involvement predominates in the axial skel­ (8, 11) may be noted. eton, initial or concomitant involvement at extra-axial Axial symptoms and signs dominate the clinical pic­ locations occurs. The spinal osteophyte production in ture in this disorder. Mid or low back pain and de­ our group of patients was generally widespread and ex­ creased range of motion are prominent and cervical tensive. Localized outgrowths, particularly in the thorac­ spine osteophytosis may produce dysphagia due to ic spine, are frequently encountered, demonstrating typ­ compression of the esophageal lumen (19). Peripheral ical Forestier-like characteristics (flowing pattern of an­ musculoskeletal complaints, present in 8 of the 21 pa­ kylosis and absence of disc space narrowing, vacuum tients, may be the initial clinical manifestation of the dis­ phenomena and end-plate sclerosis) and may be unas­ ease. These symptoms are "para-articular" with pain, sociated with axial symptoms or signs. Pathologically, tenderness and decreased range of motion and surgical these smaller osteophytes are similar in all respects ex­ removal of osteophytes may be necessary. Joint inflam­ cept size and represent one end of the disease spec­ mation is characteristically absent although concomitant trum. Although we have not specifically investigated the osteoarthritis in these elderly patients is not infrequent. extra-axial manifestations in a group of patients with The etiology of this disorder is obscure. Its associa­ typical but localized spinal changes, we would suspect tion with hyperglycemia and obesity (16, 21) and its oc­ that the incidence of such abnormalities would be con­ currence in cats with hypervitaminosis A (22) have been siderably lower than in the present group of patients and noted. Ligament ossification in adult human beings with that the degree of alteration would similarly be less vitamin A poisoning has also been demonstrated (12). striking. Several additional patients seen at the Veter­ Its resemblance in some respects to acromegaly may ans Administration Hospital during this period demon­ be appreciated but assays of growth hormones in af­ strated similar widespread extraspinal radiographic al­ 'tected patients have not been rewarding (3). Toxic ex- terations without spinal abnormality. These patients are posure to fluorine has also been suggested as an etio­ being carefully observed to determine their eventual logic factor. proclivity to spinal involvement. The roentgen features of DISH are distinctive. Ank­ ylosing spondylitis produces squaring of the vertebral ADDENDUM: Axial and extra-axial radiographs of 21 age­ bodies, thin bony syndesmophytes, and apophyseal and matched male controls (who had been examined for meta­ sacroiliac articular alterations. Acromegaly may result static bone disease) were reviewed. In each instance, x-ray in bony deposition along the anterior and lateral aspects views comparable to those obtained in the patients with of the vertebral bodies (7, 20) with preservation of disc Forestier's disease were available, including radiographs of height, but the .'flowing" pattern of new bone is not the peripheral joints. In the pelvis, small, irregular outgrowths seen, posterior scalloping of the vertebral bodies may were present at the iliac crests, ischial tuberosities and/or be noted (26), and typical alterations in the skull and pe­ trochanters in 5 patients. Additional findings included olecra­ ripheral skeleton are apparent. The prominent tufts of non spurs (2 cases), calcaneal spurs (4 cases), and anterior the terminal phalanges noted in several of our patients patellar spurs (1 case). Slight hyperostosis of the distal medial humerus was noted in 1 patient. Hyperostosis was mild and with DISH were mild compared to those occurring in localized in all of these men. acromegalics. Fluorosis (23) may produce spinal osteo­ phytosis, ligament ossification, pelvic "whiskering," and ACKNOWLEDGMENTS: I wish to gratefully acknowledge the peripheral periostitis (25), but increased skeletal density assistance of Sue Brown for the photography and Janet is apparent. Pertinent geographic history and clinically Zatlokowicz for her secretarial work. evident dental abnormalities may allow accurate diagno­ sis. Pachydermoperiostosis (14, 18) may produce per­ Department of Radiology iosteal new bone formation involving the distal ends of Veterans Administration Hospital long bones which may simulate the extraspinal manifes­ 3350 La Jolla Village Drive tations of DISH. The presence of digital clubbing, San Diego, Calif. 92161 coarsening of the facial features, and furrowing and oili­ REFERENCES ness of the skin, and the absence of radiographic ab­ normalities of the spine allow its differentiation. Hyper­ 1. Bassiouni M: Incidence of calcaneal spurs in osteo-arth­ trophic osteoarthropathy (13) produces diaphyseal and rosis and rheumatoid arthritis, and in control patients. Ann Rheum metaphyseal periostitis which differs from the appear- Dis 24:490-493, Sep 1965 524 DONALD RESNICK AND OTHERS June 1975

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