- Email: [email protected]
Monostotic Paget’s disease of the calcaneus
The Foot (2000) 10, 84–86 © 2000 Harcourt Publishers Ltd doi: 10.1054/foot.2000.0611, available online at http://www.idealibrary.com on
ORIGINAL ARTICLE
Monostotic Paget’s disease of the calcaneus Evaluation of bone changes by imaging studies M. Koti, F. W. Smith, *R. Bhattacharya† Clinical Research Fellow in Orthopaedic Surgery; *Consultant Radiologist; †Consultant Orthopaedic Physician; Woodend Hospital, Aberdeen, UK SUMMARY. Involvement of the calcaneus in polyostotic Paget’s disease occurs in 3–10% of cases, but monostotic calcaneal involvement is rare. We report the radiographical and magnetic resorance imaging (MRI) appearances in a patient with monostotic Paget’s disease affecting the calcaneus. © 2000 Harcourt Publishers Ltd
subtalar and ankle joint movements. The right heel was normal. He had no other abnormality and systemic examination was normal. Blood biochemistry was normal except for a slight decrease in serum calcium level. Lateral and axial radiographs of both heels showed the right calcaneus to be normal (Fig. 1 A & B). The left calcaneus showed characteristic appearances of Paget’s disease with generalized enlargement, sclerosis and coarsening with formation of thick bundles of weight bearing trabeculae (Fig. 1C). On the axial projection, the left calcaneus showed abnormal irregular cortical splitting, thickening and disorganization of bony architecture (Fig. 1D). The other bones of the foot were normal. An isotope bone scan showed isolated increased uptake in the left calcaneus (Fig. 2).
INTRODUCTION Paget’s disease of the bone is a benign bone disorder associated with progressive and extensive replacement of normal bone tissue by abnormal, excessive bony tissue of unknown aetiology described by Sir James Paget in 1876.1 The incidence of Paget’s disease is 3–4% of individuals over the age of 402,3 and in the majority it is asymptomatic.3,4 Pain is the commonest presenting symptom.5–7 The disease is rarely monostotic, 85–90% of patients having more than one bone involved.3,5,7 Paget’s disease affects the foot in 1–20% of polyostotic cases5,7–10 and the calcaneus in 3–10%.8,11 Isolated calcaneal involvement is unusual. Perrot et al. reported two cases of monostotic Paget’s involving the calcaneus.12 Because of the chronic nature of the disease and delayed appearance of characteristic roetgenographic lesions, the diagnosis of monostotic Paget’s disease in the early stages is difficult. We report a case of monostotic Paget’s disease involving the calcaneus.
CASE REPORT A 47-year-old male research worker presented with several years’ history of constant dull pain in his left heel which had no relation to weight-bearing. He also noticed gradual swelling of the left heel. On examination, he had a broad left heel with callosities over the posterolateral aspect. The left calcaneus was enlarged and thickened and tender on deep pressure, with no evidence of overlying inflammation. He had normal
Fig. 1 (A & B) Radiographs in lateral and axial projections showing the right calcaneus to be normal. (C) Lateral projection of the left calcaneus shows generalised enlargement, sclerosis and coarsening with formation of thick bundles of weight bearing trabeculae. (D) Axial projection of the left calcaneus showing abnormal irregular cortical splitting, thickening and disorganization of bony architecture.
Correspondence to: Mr Manjunath Koti, FRCS (Glas), Clinical Research Fellow in Orthopaedic Surgery, Woodend Hospital, Eday Road, Aberdeen AB15 6XS, UK. Tel: +44 (0) 1224 B681818, ext. 56040; Fax: +44(0) 1224 B404179; E-mail: [email protected] 84
Monostotic Paget’s disease of the calcaneus
85
DISCUSSION
Fig. 2 Bone scan shows isolated increased uptake in the left calcaneus.
MRI examination in T1-weighted and double echo steady state (DESS) sequences showed trabecular and cortical thickening with clear visualization of bone marrow fat (high-intensity signal) in the intertrabecular spaces and no soft tissue abnormality within the calcaneus, thus excluding a malignant tumour (Fig. 3A&B). In the short T1 inversion recovery (STIR) sequence, most of the calcaneus showed moderate- to high-signal intensity, suggesting increased vascularity (Fig. 3C). As the patient did not have significant pain and the serum alkaline phosphatase (SAP) was within the normal range, we did not attempt to treat the disease and the patient was kept under observation.
A
B
Paget’s disease is primarily a destructive bone lesion followed by repair during which an excess of abnormal new bone of lamellar type is laid down.13 Radiographically, three stages of the disease are classically described as lytic, mixed and sclerotic. A solitary focus should not be regarded as an early stage.2,14 The normal trabecular pattern of the calcaneum has been described by Lockhart et al.15 In the sagittal section, the trabeculae are arranged in two groups corresponding to compression and tensile stresses and it appears to be packed with cancellous bony tissue. The structural changes of Pagetic bone are extremely influenced by the physical stresses and the resulting strains on the weakened bone.16,17 The remodelled trabecular bone usually shows selective thickening of the weight-bearing trabeculae and relative atrophy of the non-weight-bearing ones, as seen in this patient (Fig. 1C). Bone scanning is a reliable method for analysis of the skeletal distribution of Paget’s disease of bone because the high remodelling of Pagetic sites induces an intense uptake. Bone scan demonstrates more pagetic sites than plain radiographs.8 Asymptomatic patients do not require treatment other than periodic monitoring. The primary indication for treatment of Paget’s disease is persistent pain. Other indications including suppression of the disease, which may benefit the patient are nerve compression, fractures and a raise in SAP twice the normal level, with the disease in weightbearing bone.18 Perrot et al.12 used bisphosphonates to treat two patients with painful calcaneal Paget’s disease and found long-lasting benefit. Sarcoma is a rare but well-known complication of Paget’s disease and occurs in not more than 1% of affected patients.4
C
Fig. 3 (A & B) T1-weighted and double echo steady state (DESS) sequences showing trabecular and cortical thickening with clear visualization of bone marrow fat (high-intensity signal). (C) Short T1 inversion recovery sequence (STIR), shows most of the calcaneus to have moderate- to high-signal intensity suggesting increased vascularity.
© 2000 Harcourt Publishers Ltd
The Foot (2000) 10, 84–86
86
The Foot
CONCLUSION We describe a rare case of monostotic Paget’s disease of the calcaneus. MRI is a potentially useful tool to further enhance conformation of the diagnosis and to exclude sarcomatous changes. ACKNOWLEDGEMENTS Sincere thanks to Mr N. Maffulli PhD, FRCS (Orth) for assistance with this manuscript. REFERESNCES 1. Paget J. On a form of chronic inflammation of bones (osteitis deformans). Trans Roy Med Chir Soc London 1876; 60: 37–64. 2. Schmorl G. Uber osteitis deformans Paget. Virchow’s Arch 1932; 283: 694–751. 3. Collins D H. Paget’s disease of bone. Lancet 1956; 2: 51–57. 4. Krane S M. Paget’s disease of bone. In: Wilson J D, Braunwald E, Isselbacher K J et al., eds. Harrison’s Principles of Internal Medicine, 12th edn. New York: McGraw-Hill, 1991: 1938–1941, Ch. 344. 5. Gutman A B, Kasabach H. Paget disease (osteitis deformans). Am J Med Sci 1956; 191: 361–380. 6. Rosenkrantz J A, Wolf J, Kaicher J J. Paget’s disease (osteitis deformans). Review of one hundred eleven cases. Arch Intern Med 1952; 90: 610–633.
The Foot (2000) 10, 84–86
7. Newman F W. Paget’s disease: A statistical study of eightytwo cases. J Bone Joint Surg 1946; 28A: 798–804. 8. Meunier P J, Salson C, Mathieu L et al. Skeletal distribution and biochemical markers of Paget’s disease. Clin Orthop 1987; 217: 37–44. 9. Claustre J, Blotman F, Simon L. Les atteintes du pied au cours de la maladie osseuse de Paget. Rev Rhum Mal Osteoartic 1976: 43–49. 10. Guyer P B. Paget’s disease of bone: The anatomical distribution. Metab Bone Dis Rel Res; 1981; 4&5: 239–242. 11. Shirazi P H, Ryan W G, Fordham E W. Bone scanning in the evaluation of Paget’s disease of the bone. Clin Radiol Nucl Med 1974; 5: 523–558. 12. Perrot S, Mortier E, Renoux M, Job-Deslandre C, Memkes CJ. Monostotic Paget’s disease involving the calcaneus, diagnostic and therapeutic problems: two case reports. Rev Rhum (Engl Ed) 1995 62; 1: 45–47. 13. Krane S M, Simon L S. Metabolic consequences of bone turnover in Paget’s disease of the bone. Clin Orthop 1987; 217: 26–36. 14. Groh J A. Mono-osteitic Paget’s disease as a clinical entity: Roentgenologic observations in nine cases. Am J Roentgenol 1943; 50: 230–243. 15. Lockhart R D, Hamilton G F, Fyfe F W. Anatomy of human body, 2nd edn. London: Faber & Faber 1965: 12. 16. Edeiken J, De Palma A F, Hodes P J. Paget’s disease: osteitis deformans. Clin Orthop 1966; 46: 141–153. 17. Grainger R G, Laws J W. Paget’s disease-active or quiscent? Br J Radiol 1957; 30: 120–124. 18. Flynn W F Jr, Lane J M, Cornell C N. Metabolic bone disease. In: Chapman M W, ed. Operative Orthopaedics 2nd edn. Vol. 4. Philadelphia: J B Lippincott, 1993; 3471–3472.
© 2000 Harcourt Publishers Ltd
ORIGINAL ARTICLE
Monostotic Paget’s disease of the calcaneus Evaluation of bone changes by imaging studies M. Koti, F. W. Smith, *R. Bhattacharya† Clinical Research Fellow in Orthopaedic Surgery; *Consultant Radiologist; †Consultant Orthopaedic Physician; Woodend Hospital, Aberdeen, UK SUMMARY. Involvement of the calcaneus in polyostotic Paget’s disease occurs in 3–10% of cases, but monostotic calcaneal involvement is rare. We report the radiographical and magnetic resorance imaging (MRI) appearances in a patient with monostotic Paget’s disease affecting the calcaneus. © 2000 Harcourt Publishers Ltd
subtalar and ankle joint movements. The right heel was normal. He had no other abnormality and systemic examination was normal. Blood biochemistry was normal except for a slight decrease in serum calcium level. Lateral and axial radiographs of both heels showed the right calcaneus to be normal (Fig. 1 A & B). The left calcaneus showed characteristic appearances of Paget’s disease with generalized enlargement, sclerosis and coarsening with formation of thick bundles of weight bearing trabeculae (Fig. 1C). On the axial projection, the left calcaneus showed abnormal irregular cortical splitting, thickening and disorganization of bony architecture (Fig. 1D). The other bones of the foot were normal. An isotope bone scan showed isolated increased uptake in the left calcaneus (Fig. 2).
INTRODUCTION Paget’s disease of the bone is a benign bone disorder associated with progressive and extensive replacement of normal bone tissue by abnormal, excessive bony tissue of unknown aetiology described by Sir James Paget in 1876.1 The incidence of Paget’s disease is 3–4% of individuals over the age of 402,3 and in the majority it is asymptomatic.3,4 Pain is the commonest presenting symptom.5–7 The disease is rarely monostotic, 85–90% of patients having more than one bone involved.3,5,7 Paget’s disease affects the foot in 1–20% of polyostotic cases5,7–10 and the calcaneus in 3–10%.8,11 Isolated calcaneal involvement is unusual. Perrot et al. reported two cases of monostotic Paget’s involving the calcaneus.12 Because of the chronic nature of the disease and delayed appearance of characteristic roetgenographic lesions, the diagnosis of monostotic Paget’s disease in the early stages is difficult. We report a case of monostotic Paget’s disease involving the calcaneus.
CASE REPORT A 47-year-old male research worker presented with several years’ history of constant dull pain in his left heel which had no relation to weight-bearing. He also noticed gradual swelling of the left heel. On examination, he had a broad left heel with callosities over the posterolateral aspect. The left calcaneus was enlarged and thickened and tender on deep pressure, with no evidence of overlying inflammation. He had normal
Fig. 1 (A & B) Radiographs in lateral and axial projections showing the right calcaneus to be normal. (C) Lateral projection of the left calcaneus shows generalised enlargement, sclerosis and coarsening with formation of thick bundles of weight bearing trabeculae. (D) Axial projection of the left calcaneus showing abnormal irregular cortical splitting, thickening and disorganization of bony architecture.
Correspondence to: Mr Manjunath Koti, FRCS (Glas), Clinical Research Fellow in Orthopaedic Surgery, Woodend Hospital, Eday Road, Aberdeen AB15 6XS, UK. Tel: +44 (0) 1224 B681818, ext. 56040; Fax: +44(0) 1224 B404179; E-mail: [email protected] 84
Monostotic Paget’s disease of the calcaneus
85
DISCUSSION
Fig. 2 Bone scan shows isolated increased uptake in the left calcaneus.
MRI examination in T1-weighted and double echo steady state (DESS) sequences showed trabecular and cortical thickening with clear visualization of bone marrow fat (high-intensity signal) in the intertrabecular spaces and no soft tissue abnormality within the calcaneus, thus excluding a malignant tumour (Fig. 3A&B). In the short T1 inversion recovery (STIR) sequence, most of the calcaneus showed moderate- to high-signal intensity, suggesting increased vascularity (Fig. 3C). As the patient did not have significant pain and the serum alkaline phosphatase (SAP) was within the normal range, we did not attempt to treat the disease and the patient was kept under observation.
A
B
Paget’s disease is primarily a destructive bone lesion followed by repair during which an excess of abnormal new bone of lamellar type is laid down.13 Radiographically, three stages of the disease are classically described as lytic, mixed and sclerotic. A solitary focus should not be regarded as an early stage.2,14 The normal trabecular pattern of the calcaneum has been described by Lockhart et al.15 In the sagittal section, the trabeculae are arranged in two groups corresponding to compression and tensile stresses and it appears to be packed with cancellous bony tissue. The structural changes of Pagetic bone are extremely influenced by the physical stresses and the resulting strains on the weakened bone.16,17 The remodelled trabecular bone usually shows selective thickening of the weight-bearing trabeculae and relative atrophy of the non-weight-bearing ones, as seen in this patient (Fig. 1C). Bone scanning is a reliable method for analysis of the skeletal distribution of Paget’s disease of bone because the high remodelling of Pagetic sites induces an intense uptake. Bone scan demonstrates more pagetic sites than plain radiographs.8 Asymptomatic patients do not require treatment other than periodic monitoring. The primary indication for treatment of Paget’s disease is persistent pain. Other indications including suppression of the disease, which may benefit the patient are nerve compression, fractures and a raise in SAP twice the normal level, with the disease in weightbearing bone.18 Perrot et al.12 used bisphosphonates to treat two patients with painful calcaneal Paget’s disease and found long-lasting benefit. Sarcoma is a rare but well-known complication of Paget’s disease and occurs in not more than 1% of affected patients.4
C
Fig. 3 (A & B) T1-weighted and double echo steady state (DESS) sequences showing trabecular and cortical thickening with clear visualization of bone marrow fat (high-intensity signal). (C) Short T1 inversion recovery sequence (STIR), shows most of the calcaneus to have moderate- to high-signal intensity suggesting increased vascularity.
© 2000 Harcourt Publishers Ltd
The Foot (2000) 10, 84–86
86
The Foot
CONCLUSION We describe a rare case of monostotic Paget’s disease of the calcaneus. MRI is a potentially useful tool to further enhance conformation of the diagnosis and to exclude sarcomatous changes. ACKNOWLEDGEMENTS Sincere thanks to Mr N. Maffulli PhD, FRCS (Orth) for assistance with this manuscript. REFERESNCES 1. Paget J. On a form of chronic inflammation of bones (osteitis deformans). Trans Roy Med Chir Soc London 1876; 60: 37–64. 2. Schmorl G. Uber osteitis deformans Paget. Virchow’s Arch 1932; 283: 694–751. 3. Collins D H. Paget’s disease of bone. Lancet 1956; 2: 51–57. 4. Krane S M. Paget’s disease of bone. In: Wilson J D, Braunwald E, Isselbacher K J et al., eds. Harrison’s Principles of Internal Medicine, 12th edn. New York: McGraw-Hill, 1991: 1938–1941, Ch. 344. 5. Gutman A B, Kasabach H. Paget disease (osteitis deformans). Am J Med Sci 1956; 191: 361–380. 6. Rosenkrantz J A, Wolf J, Kaicher J J. Paget’s disease (osteitis deformans). Review of one hundred eleven cases. Arch Intern Med 1952; 90: 610–633.
The Foot (2000) 10, 84–86
7. Newman F W. Paget’s disease: A statistical study of eightytwo cases. J Bone Joint Surg 1946; 28A: 798–804. 8. Meunier P J, Salson C, Mathieu L et al. Skeletal distribution and biochemical markers of Paget’s disease. Clin Orthop 1987; 217: 37–44. 9. Claustre J, Blotman F, Simon L. Les atteintes du pied au cours de la maladie osseuse de Paget. Rev Rhum Mal Osteoartic 1976: 43–49. 10. Guyer P B. Paget’s disease of bone: The anatomical distribution. Metab Bone Dis Rel Res; 1981; 4&5: 239–242. 11. Shirazi P H, Ryan W G, Fordham E W. Bone scanning in the evaluation of Paget’s disease of the bone. Clin Radiol Nucl Med 1974; 5: 523–558. 12. Perrot S, Mortier E, Renoux M, Job-Deslandre C, Memkes CJ. Monostotic Paget’s disease involving the calcaneus, diagnostic and therapeutic problems: two case reports. Rev Rhum (Engl Ed) 1995 62; 1: 45–47. 13. Krane S M, Simon L S. Metabolic consequences of bone turnover in Paget’s disease of the bone. Clin Orthop 1987; 217: 26–36. 14. Groh J A. Mono-osteitic Paget’s disease as a clinical entity: Roentgenologic observations in nine cases. Am J Roentgenol 1943; 50: 230–243. 15. Lockhart R D, Hamilton G F, Fyfe F W. Anatomy of human body, 2nd edn. London: Faber & Faber 1965: 12. 16. Edeiken J, De Palma A F, Hodes P J. Paget’s disease: osteitis deformans. Clin Orthop 1966; 46: 141–153. 17. Grainger R G, Laws J W. Paget’s disease-active or quiscent? Br J Radiol 1957; 30: 120–124. 18. Flynn W F Jr, Lane J M, Cornell C N. Metabolic bone disease. In: Chapman M W, ed. Operative Orthopaedics 2nd edn. Vol. 4. Philadelphia: J B Lippincott, 1993; 3471–3472.
© 2000 Harcourt Publishers Ltd