- Email: [email protected]
Melorheostosis of the hand
MELORHEOSTOSIS
OF T H E H A N D
R. SHARMA and F. D. BURKE
From the Pulvertaft Hand Centre, Derbyshire Royal Infirmary, Derby, UK We report a case of melorheostosis of the hand which showed a sclerotomal pattern of involvement, with swelling and pain. Surgical debulking resulted in correction of the deformity and relief of pain.
Journal of Hand Surgery (British and European Volume, 1996) 21B: 3:413 415 Melorheostosis usually affects the long bones of the lower limbs and the hand is a relatively uncommon site of presentation. This benign and hyperostotic disorder of the bone is rare and was first reported by Leri and Joanny (1922), who described it as resembling "wax flowing down the candle". The oldest recorded example of the condition was noted in the fibula of a prehistoric Alaskan skeleton (Lester 1967). Hand involvement has been reported sporadically. It tends to have the following features: a sclerotomal distribution; skin involvement over the bony protuberance; deformity caused by joint contractures or length inequality of the affected ray or digit and pain (Azuma et al, 1992; Pruitt and Manske, 1992; Steffens and Koob 1993). Carpal tunnel syndrome has been reported in a melorheostotic hand (B6stman and Bakalim, 1985). The histopathology is not very distinctive, showing an inconsistent presentation of a thickened periosteum, Haversian canals of unequal diameter, fibrobtast proliferation of the bone (Campbell et al, 1968; Dimar and Campion, 1987; Ippolito et al, 1993) and cartilaginous metaptasia (Kawabata et al, 1984).
The histopathology report noted "reactive change of the periosteum with dense trabecular bone with intertrabecular spaces containing loose spindle cell fibroblasts and lymphoid cells". The postoperative recovery was uneventful with normal wound healing. The cosmetic appearance was better, with no swelling and a linear scar (Fig 4). The patient was relieved of her pain and returned to work in 3 weeks. Three years after operation, there has been some recurrence of mild pain but cosmesis, range of motion of the PIP joint and hand function are preserved.
CASE R E P O R T
A 29-year-old Caucasian woman presented with a complaint of a gradually increasing swelling of her dominant right index finger, which caused her distress in daily activities (Fig 1). Her main problem was pain if she inadvertently struck her hand. The finger had begun to look bent due to the swelling on the radial side of the proximal phalanx. It had been gradually increasing in size for 2 years. On examination, the skin was supple with mild tenderness on pressure over the swelling. There was a full range of motion in all joints of the index finger and no contractures or rotatibnal deformity. X-rays showed unicortical formation of hyperostotic bone on the radial side of metacarpal, proximal and middle phalanges of the index ray (Fig 2). The joints appeared normal and uninvolved. The wrist showed possible involvement of the trapezoid in the form of increased density. The distribution was consistent with a C6 sclerotomal pattern. The lesion was surgically debulked from the proximal phalanx and the bone nibbled to a near normal contour. At operation the periosteum was found to be thickened and the underlying bone very hard and sclerotic (Fig 3).
Fig 1
413
X-ray film of hand showinginvolvementof the middle phalanx, proximal phalanx and metacarpal head of the index ray and the trapezoid.
414
THEJOURNALOF HANDSURGERYVOL.21BNo. 3 JUNE 1996
DISCUSSION Melorheostosis presents rarely in the hand. The extent of involvement is variable. Sometimes there is marked loss of function yet there also may be near normal appearance and function. Review of the literature reveals the condition to be more than an incidental radiological curiosity. Pain and tenderness have been c o m m o n complaints in the majority of cases. Inequality in length of the affected part is somewhat less frequently seen. Pathological fractures and vascular problems have not been reported yet in an affected finger. All the reviewed hand cases were adults but Younge et al (1979) mentioned hand involvement in paediatric patients who had melorheostosis at other sites. The hyperostotic bone does not revert to normal and has not been shown to undergo malignant change. Our case had a sclerotomal distribution of C6. On reviewing ten previous case reports there seems to be an even distribution between C6, 7, and 8 sclerotomes. All these cases were diagnosed radiologically and some also had bone scans. Whyte et al (1978) have shown the value of bone scans in differentiating osteopoikilosis and osteopathia striata from melorheostosis; only
Fig 2
Radial bowing appearance of the index finger.
Fig 3
lntraoperative view of the prominent hyperostotic cortex.
Fig 4
Postoperative appearance.
MELORHEOSTOSIS
melorheostosis shows increased focal uptake on bone scan.
There has been no clinical or radiological progression of disease in our case over a 3-year follow-up. Most of the reported cases who underwent surgical debulking of the hyperostotic cortex and release of contractures did well symptomatically. This treatment is thus recommended. Ray amputation is an option for late cases with stiffness and deformity. Long-term follow-up by Siler (1954) and Caudle and Stern (1987) are notable for reporting progressive deformity and pain over the years. Pruitt and Manske (1992) stressed the importance of training the patients early on to use the unaffected extremity in a dominant fashion if the affected side has severe contractures. The aetiology of melorheostosis is still unclear. The theories of "congenital vascular disturbance and inflammatory vascular obliteration" (Morris et al, 1963) have been challenged by Campbell et al (1968), who suggested the theory of a congenital disorder of a mesodermal cell line instead. This theory is consistent with the distribution in sclerotomes (Murray and McCredie, 1979) and we feel that this appears the most acceptable theory of aetiology. References A Z U M A H, S A K A D A T, TANABE H and H A N D A M (1992). Melorheostosis of the hand: a report of two cases. Journal of H a n d Surgery, 17A: 1076 1078. BOSTMAN O M and BAKALIM G E (1985). Carpal tunnel syndrome in a melorheostotic limb. Journal of H a n d Surgery, 10B: 101-102. C A M P B E L L C J, PAPADEMETRIOU T and B O N F I G L I O M (1968).
415 Melorheostosis: a report of the clinical, roentgenographic, and pathological findings in 14 cases. Journal of Bone and Joint Surgery, 50A: 1281-1304. C A U D L E R J and STERN P J (1987). Melorheostosis of the hand. Journal of Bone and Joint Surgery, 69A: 1229-1231. D I M A R J R and C A M P I O N T S (1987). Melorheostosis: two case presentations and review of the literature. Orthopaedic Review, 16: 615/27-621/33. IPPOLITO V, M I R R A J M, M O T T A C, C H I O D E R A P and BONETTI M F (1993). Case report 771: melorheostosis in association with desmoid tumour. Skeletal Radiology, 22: 284-288. KAWABATA H, T S U Y U G U C H I Y, KAWAI H and YASUI N (1984). Melorheostosis of the upper limb: a report of two cases. Journal of Hand Surgery, 9A: 871 876. LERI A and J O A N N Y L (1922). Une affection non d6crite de os: hyperostose "en coul6e" sur toute a la longueur d'un membre ou "melorh6ostose". Bulletins et memoires de ta Societe Medicale des Hopitaux de Paris, 46: 1141-1145. LESTER C W (1967). Melorheostosis in a prehistoric Alaskan skeleton. Journal of Bone and Joint Surgery, 49A: 142 143. MORRIS J M, SAMILSON R L and C O R L E Y C L (1963). Melorheostosis: review of the literature and report of an interesting case with a 19-year follow-up. Journal of Bone and Joint Surgery, 45A: 1191-1206. M U R R A Y R O and M c C R E D I E J (1979). Melorheostosis and sclerotomes: a radiological con'elation. Skeletal Radiology, 4: 57-71. PRUITT D L and M A N S K E P R (1992). Soft tissue contractures from melorheostosis involving the upper extremity. Journal of H a n d Surgery, 17A: 90 93. SILER V E (1954). Unusual tumors of the forearm and hand: a report on melorheostosis and neurofibroma. American Surgeon, 20:43 48. STEFFENS K and KOOB E (1993), Melorheostose der Hand. Zeitschrift fur Orthop~tdie und Ihre Grenzgebiete, 131: 340-343. W H Y T E M P, M U R P H Y W A and SIEGAL B A (1978). 99mTc-pyrophosphate bone imaging in osteopoikilosis, osteopathia striata, and melorheostosis. Radiology, 127: 439-443. Y O U N G E D, D R U M M O N D D, H E R R I N G J and CRUESS R L (1979). Metorheostosis in children. Journal of Bone and Joint Surgery, 61B: 415-418.
Accepted: 23 October 1995 F, D. Burke, Pulvertaft Hand Centre, Derbyshire Royal Infirmary, London Road, Derby DE1 2QY, UK. © 1996 The British Society for Surgery of the Hand
OF T H E H A N D
R. SHARMA and F. D. BURKE
From the Pulvertaft Hand Centre, Derbyshire Royal Infirmary, Derby, UK We report a case of melorheostosis of the hand which showed a sclerotomal pattern of involvement, with swelling and pain. Surgical debulking resulted in correction of the deformity and relief of pain.
Journal of Hand Surgery (British and European Volume, 1996) 21B: 3:413 415 Melorheostosis usually affects the long bones of the lower limbs and the hand is a relatively uncommon site of presentation. This benign and hyperostotic disorder of the bone is rare and was first reported by Leri and Joanny (1922), who described it as resembling "wax flowing down the candle". The oldest recorded example of the condition was noted in the fibula of a prehistoric Alaskan skeleton (Lester 1967). Hand involvement has been reported sporadically. It tends to have the following features: a sclerotomal distribution; skin involvement over the bony protuberance; deformity caused by joint contractures or length inequality of the affected ray or digit and pain (Azuma et al, 1992; Pruitt and Manske, 1992; Steffens and Koob 1993). Carpal tunnel syndrome has been reported in a melorheostotic hand (B6stman and Bakalim, 1985). The histopathology is not very distinctive, showing an inconsistent presentation of a thickened periosteum, Haversian canals of unequal diameter, fibrobtast proliferation of the bone (Campbell et al, 1968; Dimar and Campion, 1987; Ippolito et al, 1993) and cartilaginous metaptasia (Kawabata et al, 1984).
The histopathology report noted "reactive change of the periosteum with dense trabecular bone with intertrabecular spaces containing loose spindle cell fibroblasts and lymphoid cells". The postoperative recovery was uneventful with normal wound healing. The cosmetic appearance was better, with no swelling and a linear scar (Fig 4). The patient was relieved of her pain and returned to work in 3 weeks. Three years after operation, there has been some recurrence of mild pain but cosmesis, range of motion of the PIP joint and hand function are preserved.
CASE R E P O R T
A 29-year-old Caucasian woman presented with a complaint of a gradually increasing swelling of her dominant right index finger, which caused her distress in daily activities (Fig 1). Her main problem was pain if she inadvertently struck her hand. The finger had begun to look bent due to the swelling on the radial side of the proximal phalanx. It had been gradually increasing in size for 2 years. On examination, the skin was supple with mild tenderness on pressure over the swelling. There was a full range of motion in all joints of the index finger and no contractures or rotatibnal deformity. X-rays showed unicortical formation of hyperostotic bone on the radial side of metacarpal, proximal and middle phalanges of the index ray (Fig 2). The joints appeared normal and uninvolved. The wrist showed possible involvement of the trapezoid in the form of increased density. The distribution was consistent with a C6 sclerotomal pattern. The lesion was surgically debulked from the proximal phalanx and the bone nibbled to a near normal contour. At operation the periosteum was found to be thickened and the underlying bone very hard and sclerotic (Fig 3).
Fig 1
413
X-ray film of hand showinginvolvementof the middle phalanx, proximal phalanx and metacarpal head of the index ray and the trapezoid.
414
THEJOURNALOF HANDSURGERYVOL.21BNo. 3 JUNE 1996
DISCUSSION Melorheostosis presents rarely in the hand. The extent of involvement is variable. Sometimes there is marked loss of function yet there also may be near normal appearance and function. Review of the literature reveals the condition to be more than an incidental radiological curiosity. Pain and tenderness have been c o m m o n complaints in the majority of cases. Inequality in length of the affected part is somewhat less frequently seen. Pathological fractures and vascular problems have not been reported yet in an affected finger. All the reviewed hand cases were adults but Younge et al (1979) mentioned hand involvement in paediatric patients who had melorheostosis at other sites. The hyperostotic bone does not revert to normal and has not been shown to undergo malignant change. Our case had a sclerotomal distribution of C6. On reviewing ten previous case reports there seems to be an even distribution between C6, 7, and 8 sclerotomes. All these cases were diagnosed radiologically and some also had bone scans. Whyte et al (1978) have shown the value of bone scans in differentiating osteopoikilosis and osteopathia striata from melorheostosis; only
Fig 2
Radial bowing appearance of the index finger.
Fig 3
lntraoperative view of the prominent hyperostotic cortex.
Fig 4
Postoperative appearance.
MELORHEOSTOSIS
melorheostosis shows increased focal uptake on bone scan.
There has been no clinical or radiological progression of disease in our case over a 3-year follow-up. Most of the reported cases who underwent surgical debulking of the hyperostotic cortex and release of contractures did well symptomatically. This treatment is thus recommended. Ray amputation is an option for late cases with stiffness and deformity. Long-term follow-up by Siler (1954) and Caudle and Stern (1987) are notable for reporting progressive deformity and pain over the years. Pruitt and Manske (1992) stressed the importance of training the patients early on to use the unaffected extremity in a dominant fashion if the affected side has severe contractures. The aetiology of melorheostosis is still unclear. The theories of "congenital vascular disturbance and inflammatory vascular obliteration" (Morris et al, 1963) have been challenged by Campbell et al (1968), who suggested the theory of a congenital disorder of a mesodermal cell line instead. This theory is consistent with the distribution in sclerotomes (Murray and McCredie, 1979) and we feel that this appears the most acceptable theory of aetiology. References A Z U M A H, S A K A D A T, TANABE H and H A N D A M (1992). Melorheostosis of the hand: a report of two cases. Journal of H a n d Surgery, 17A: 1076 1078. BOSTMAN O M and BAKALIM G E (1985). Carpal tunnel syndrome in a melorheostotic limb. Journal of H a n d Surgery, 10B: 101-102. C A M P B E L L C J, PAPADEMETRIOU T and B O N F I G L I O M (1968).
415 Melorheostosis: a report of the clinical, roentgenographic, and pathological findings in 14 cases. Journal of Bone and Joint Surgery, 50A: 1281-1304. C A U D L E R J and STERN P J (1987). Melorheostosis of the hand. Journal of Bone and Joint Surgery, 69A: 1229-1231. D I M A R J R and C A M P I O N T S (1987). Melorheostosis: two case presentations and review of the literature. Orthopaedic Review, 16: 615/27-621/33. IPPOLITO V, M I R R A J M, M O T T A C, C H I O D E R A P and BONETTI M F (1993). Case report 771: melorheostosis in association with desmoid tumour. Skeletal Radiology, 22: 284-288. KAWABATA H, T S U Y U G U C H I Y, KAWAI H and YASUI N (1984). Melorheostosis of the upper limb: a report of two cases. Journal of Hand Surgery, 9A: 871 876. LERI A and J O A N N Y L (1922). Une affection non d6crite de os: hyperostose "en coul6e" sur toute a la longueur d'un membre ou "melorh6ostose". Bulletins et memoires de ta Societe Medicale des Hopitaux de Paris, 46: 1141-1145. LESTER C W (1967). Melorheostosis in a prehistoric Alaskan skeleton. Journal of Bone and Joint Surgery, 49A: 142 143. MORRIS J M, SAMILSON R L and C O R L E Y C L (1963). Melorheostosis: review of the literature and report of an interesting case with a 19-year follow-up. Journal of Bone and Joint Surgery, 45A: 1191-1206. M U R R A Y R O and M c C R E D I E J (1979). Melorheostosis and sclerotomes: a radiological con'elation. Skeletal Radiology, 4: 57-71. PRUITT D L and M A N S K E P R (1992). Soft tissue contractures from melorheostosis involving the upper extremity. Journal of H a n d Surgery, 17A: 90 93. SILER V E (1954). Unusual tumors of the forearm and hand: a report on melorheostosis and neurofibroma. American Surgeon, 20:43 48. STEFFENS K and KOOB E (1993), Melorheostose der Hand. Zeitschrift fur Orthop~tdie und Ihre Grenzgebiete, 131: 340-343. W H Y T E M P, M U R P H Y W A and SIEGAL B A (1978). 99mTc-pyrophosphate bone imaging in osteopoikilosis, osteopathia striata, and melorheostosis. Radiology, 127: 439-443. Y O U N G E D, D R U M M O N D D, H E R R I N G J and CRUESS R L (1979). Metorheostosis in children. Journal of Bone and Joint Surgery, 61B: 415-418.
Accepted: 23 October 1995 F, D. Burke, Pulvertaft Hand Centre, Derbyshire Royal Infirmary, London Road, Derby DE1 2QY, UK. © 1996 The British Society for Surgery of the Hand