Smallpox has been eradicated completely but its unique pathology and sequlae are still found in routine clinical practice. We present a report of a patient having osteomyelitis variolosa in both elbow joints and wrist with fracture of humerus. The condyles were typically elongated as central portion of distal humerus absorbed. The fracture united uneventfully following stabilization with dynamic compression plate and bone grafting. Patient had satisfactory elbow function at the end of the last follow-up.
Keywords: Joint deformity, osteomyelitis, smallpox, variola virus
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Singh PK. Osteomyelitis variolosa with fracture: A unique case report. Ann Trop Med Public Health 2012;5:124-6
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Singh PK. Osteomyelitis variolosa with fracture: A unique case report. Ann Trop Med Public Health [serial online] 2012 [cited 2021 Mar 4];5:124-6. Available from: https://www.atmph.org/text.asp?2012/5/2/124/95968
Smallpox has already been eradicated but because of its consequences the sufferers still have its effect on their faces. Musculoskeletal complication following smallpox has been reported especially in young children. Smallpox is known for damaging the epiphysis and growth plate and causing subsequent joint deformity.  Deformities consisted of a bilateral, symmetrical osteomyelits with arthritis predominantly affecting the upper limb. Osteomyelitis variolosa is the accepted terminology for the skeletal involvement which appears during the recovery period from the smallpox eruption. The clinical and radiological features of osteomyelits variolosa have been very well-described by Cockshott and MacGregor in their reports.  We report a case of osteomyelitis variolosa and associated deformity which was further complicated by fracture. Operative intervention was done to treat the fracture and surprisingly the fracture union was uneventful
A 41-year-old right-handed Indian male school teacher presented to our OPD at NSCB Medical College, Jabalpur, India in January 2004 with history of fall while walking. He sustained a fracture of his left arm. He stated that he was from an area where an epidemic of smallpox had occurred when he was seven years old. Two of his elder siblings contracted the disease but no deformity. Deformities were gradual onset and progressive as he grew up. Deformities involved his fingers, wrist, elbows, and ankles. On physical examination he had residual facial stigmata of variola pustules, and he walked with a limp. His left humerus had abnormal mobility and bony crepitus. Transmitted movements were lost. Movement at the left elbow could not be elicited as it was very painful. He was not able to demonstrate any movements at the left elbow due to recent trauma. Right elbow was grossly unstable and had 20-degree fixed flexion deformity with further flexion up to 110 degrees. Both his wrists were grossly deformed, deviated towards te radial side and unstable. His middle finger was short on the left side. There was normal movement at the small joints of the hand with normal hand grip on both sides. He demonstrated good dexterity and was able to write fluently with his right hand. There was normal sensation including touch and temperature.
The hemogram was within normal limits with erythrocyte sedimentation rate (ESR) 16 mm at the end of the first hour. Blood chemistry clotting profile, liver and kidney profile revealed no abnormality.
Antero-posterior radiograph of the right elbow joint demonstrated bone absorption of the central portion of the trochlea and capitulum, with elongation of both the medial and lateral condyle [Figure 1]. Shortening of the radius with deformed radius was evident. Lateral radiograph demonstrated maintained congruity of joint. Radiograph of wrist showed distal radio-ulnar joint disruption with radio-carpal derangement with normal hand bones [Figure 2]. Left humerus fractured in distal 3ird of diaphysis with milder degree of bone absorption of trochlea and capitulum and deformity [Figure 3]. Left wrist radiograph showed radio-carpal derangement and deformity with shortened third metacarpals of hands [Figure 4] with disturbance of the third metacarpophalangeal joints. Other irregular changes were noted in the phalanges.
|Figure 1: Antero-posterior radiograph of arm and forearm of right upper limb of 41-year-old patient who contracted smallpox at the age of seven years. Several years later radiograph revealed absorption of trochlea and capitulum with elongation of condyles
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|Figure 2: Antero-posterior radiograph of wrist of 41-year-old shows radio-carpal derangement with distal radio-ulnar disruption
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|Figure 3: Radiograph of left arm and forearm shows fracture of the distal third of the humerus with radio-carpal anatomical disruption
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|Figure 4: Radiograph of wrist and hand shows shortening of third metacarpal with third carpometacarpal arthritic changes
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The patient was operated under general anesthesia. Open reduction internal fixation with bone grafting was performed. Fracture was fixed by seven-hole dynamic compression stainless steel plate which had greater biomechanical stability as compared to nail device for distal humerus fracture [Figure 5]. Postoperative recovery was uneventful. He regained his previous functional status within 12 weeks and returned to his job.
|Figure 5: Postoperative radiograph shows good anatomical reduction and stabilization with plate
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It has been 30 years since the World Health Organization (WHO) declared the world free from smallpox.  Still some complications are being confronted and reported. Recent reports in the literature are from tropical countries where the smallpox epidemic was more severe. , Though smallpox vaccination has been reported to cause bone lesions.  Osteomyelitis variolosa is a rare complication of smallpox and has been estimated to occur in 0.25-0.5% of all patients and in 2-5% of affected children. , Most of the osteoarticular complications involved the elbow followed by the wrist, hand and ankle. The skull, spine, and pelvis have also been reported to get involved on rare occasions.
Few autopsy based study formulate pathophysiological evidences which demonstrates pathological foci in the bone marrow of victims of smallpox. , Eventually, these lesions lead to destruction of the perimetaphyseal structures, epiphyseal separation and to bony changes in diaphysis.  Subsequent deformities may result from reparative ossification and distortion, cessation of the longitudinal growth of bones, probably due to destruction of the physeal growth plate. Abnormal mechanical axis and juxtradeformity stress riser at the bone could be the explanation for fracture as in our case.
Osteomyelitis variolosa deserves to be differentiated from pathologies having similar clinical and radiological presentation. Bone lesion needs to be differentiated from infantile hyperostosis disease and salmonella osteitis in sickle cell anemia.  Gross pathological finding includes irregular bony surface, thickening of cortices and sclerosis which has been mentioned in the literature.  The radiological changes as depicted in our case must not be confused with those seen in leprosy,  which will have a unique clinical picture along with bony changes. Differential diagnosis of the deformities of smallpox includes achondroplasia, dysplasia, and sequelae of septic arthritis. ,, Fracture union and functional outcome did not show any abnormality despite gross bony alteration.
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Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]