Int J Clin Exp Med 2015;8(8):13417-13420 www.ijcem.com /ISSN:1940-5901/IJCEM0011336
Original Article Three different methods for treating multiple enchondromatosis in one hand Hui Lu1*, Qiang Chen1, Hui Shen1, Xiang-Qian Shen1, Shou-Cheng Wu1, Xiang-Jin Lin2* Departments of 1Hand Surgery, 2Orthopedics, The First Affiliated Hospital, College of Medicine, Zhejiang University, #79 Qingchun Road, Hangzhou 310003, Zhejiang Province, P. R. China. *Equal contributors. Received June 12, 2015; Accepted August 2, 2015; Epub August 15, 2015; Published August 30, 2015 Abstract: Ollier’s disease remains comparatively rare, and is a non-hereditary cartilage dysplasia of bone. It is usually associated with problems such as deformity and fracture. Three different methods were used in a one-hand of 15-year-old boy reporting his pain in the left hand and swellings. After the curettage of tumor, regarding as the differences of all parts of the bone structure reconstruction in the patient’s hand, we chose three following methods for this boy, i.e. fixed by the locking plate with calcium phosphate cement, filled with allograft bone, curetted the tumor without any bone graft. After the surgery, the patient was able to perform full motion of the operated hand. No evidence of recurrence was noted four years after surgery. To choose the different ways with bone grafts or not that relies on the patients’ conditions for bone structure reconstruction. However, patients with large osseous defects or pathological fracture, we demand full bone graft and reliable internal fixation. After surgery, early exercises can reach a desirable result and functional recovery. Keywords: Ollier’s disease, enchondromatosis, bone graft
Background Enchondromatosis is a form of osteochondrodysplasia characterized by a proliferation of enchondromas. Multiple enchondromatosis (Ollier’s disease), originally reported by Ollier in 1899, is a rare and non-hereditary cartilage dysplasia of bone, consisting of multiple, asymmetrically distributed, intraosseous cartilaginous foci and subperiosteal deposition of cartilage, either exclusively or predominantly involving one side of the body. It arises in the medullary cavity and grows in the cortex, forming a prominent endogenous mass of the bone. Some patients will not accept the medical treatment until a pathological fracture occurs. And also the enchondroma will not be discovered until some patients take radiographs after trauma. We report a 15-year-old boy’s case with three different treatments in one hand. There have not been any articles reporting different treatments that are used on the same patient. Case presentation A 15-year-old teenage boy came to the Hand Clinic in the First affiliated hospital, Zhejiang
University, complaining of the pain that was lasting for half a year in his left hand. We found swellings and deformities on his dorsal of hand and in his proximal phalanx of middle and ring fingers respectively. There was no history of injury, systemic symptoms or operation. The skin and tendon were unaffected. Laboratory studies were within normal findings. Radiographs showed expansion of bone and thinning of cortex, endosteal scalloping, cortical thinning in the 3rd~4th metacarpal shaft and the proximal and middle phalanx of the middle and ring fingers respectively. The lesions caused deformities of the 3rd~4th metacarpal shaft and the 4th metacarpal shaft had a pathologic fracture. The middle phalanx of the index finger had only thinning of one side cortex. The radiographs findings showed no signs of aggressive activity (Figure 1). Based on the clinical presentation and radiographic findings, we diagnosed this case as a benign tumor. An incisional biopsy procedure could not be performed in order to avoid the wound contamination. A dorsal skin incision was made under general anesthesia so as that the overlying extensor tendon was exposed longitudinally and mobilized medially or laterally. A cortical window was
Three different methods for treating Ollier’s disease in one hand fixation was removed and the patient’s functions presented normally. No evidence of recurrence was noted four years after surgery. Bone regeneration was noted both radiographically and clinically (Figure 3). Discussion
Figure 1. Preoperative radiograph of the left hand, showing. The middle phalanx of the index finger had only thinning of one side cortex. The 3rd~4th metacarpal shaft and the proximal and middle phalanx of the middle and ring fingers had expansion and thinning of the cortices. The 4th metacarpal shaft had a pathologic fracture.
made to allow the entire tumor to be exposed, and through the window it was curetted. Due to the early functional exercises requirements, the pathologic forth metacarpal fracture and the intramedullary canal were filled with calcium phosphate cement (Wright, USA). The dorsal metacarpal shaft were fixed by the locking plate(depuysynthes, USA). Proximal and middle phalanx of the middle and ring fingers were filled with allograft bone. The middle phalanx of the index finger was only curetted without any bone graft. Considering the patient’s age, we didn’t use autogenous bone as the material of bone graft. Then the wounds were covered. Histologic findings revealed benign cartilaginous patterns of multiple enchondromatosis. There was no evidence of hemangiomas, which was consistent with the diagnosis of Ollier’s disease. Three days after the surgery, the patient was able to perform full motion of the operated hand. Three months after the surgery, the patient could do all weight exercises (Figure 2). One year after the surgery, the locking plate 13418
Ollier’s disease, a rare non-hereditary skeletal disorder, is characterized by multiple enchondromas with a predilection for unilateral cosmetic problems such as deformity and fracture. The enchondromas grow large with progressive skeletal growth, become more evident and disfigure. After the cessation of growth and development, the enchondromas do not enlarge [1]. The rate of occurrence of this disease is 1/100,000, which is equal between men and women [2]. In general, enchondromas are involved in the short tubular bones of the hands and feet, and also found incidentally in the distal femurs, and proximal humerus. They undergo malignant transformation in less than 1% of cases [3]. Malignant transformation and development of extra skeletal cancers are the main complications of multiple enchondromatosis. It has up to 30% risk of malignant chondrosarcomatous transformation [4]. Patients with enchondromas of the small bones of the limbs have the lowest risk to develop malignancy (14%), whereas enchondromas affecting the long axial bones show the highest risk (44%-55%) [5]. Multiple enchondromas and hemangiomas of soft tissue constitute a condition known as Maffucci’s Syndrome. It is less common than Ollier disease, but has a higher risk of chondrosarcomatous change. Other cartilaginous lesions include bone infarct, low-grade chondrosarcoma, epidermoid inclusion cyst, chondromyxoid fibroma, unicameral bone cyst, giant cell tumor and fibrous dysplasia. Our case didn’t destruct the cortex or enter the soft tissues, so benign tumors should be firstly considered. Surgery remains the only effective solution to remove the enchondroma and prevent malignant transformation. Surgical resection to multiple enchondromas of the hand includes curettage, curettage with autografts or allografts and amputations [6-8]. According to Klausmeyer MA et al. and Hasselgren G et al., despite large bony deficits after resection, bony regrowth occurs under the circumstance of without bone autograft [9, 10]. Int J Clin Exp Med 2015;8(8):13417-13420
Three different methods for treating Ollier’s disease in one hand Figure 3. After four years postoperative radiograph of the left hand, showing No evidence of recurrence was noted. Bone regeneration repairs bone defect.
Our case obviously showed bony defects and pathological fracture. Concerned about stable fixation and the initiation of early rangeof-motion exercises, we chose the calcium phosphate cement to fill with the defect of metacarpal shafts and then the metacarpal shafts were fixed with the locking plate. Proximal phalanx of the middle finger, proximal and middle phalanx of ring fingers were filled with allograft bone. The middle phalanx of the index finger was only curettage of the intramedullary. Three different treatments in one hand had a satisfactory result. Figure 2. After three months postoperative radiograph of the left hand, showing Pathologic fracture has healed, internal fixation has presented stable and bone grafting is without bone absorption.
Conclusions
This case report emphasizes that according to the patient’s conditions of bone structure, we choose different treatments including bone grafts and without bone grafts. If patients have large osseous defects or pathological fracture, we demand bone graft and reliable internal fixation. After surgery, early exercises can reach a desirable result and functional recovery. Acknowledgements First and foremost, I would like to show my deepest gratitude to my colleagues Dr. Qiang Chen, Dr. Hui Shen, Dr. Xiang-Qian Shen, Dr. Shou-Cheng Wu, Dr. Xiang-Jin Lin who have provided me with valuable assist in every stage of writing this paper. Meanwhile, I also appreciate Zhejiang Medicine and Hygiene Research Program for sponsoring our research (the grant number 2015KYA100). Last but not least, I’d like to thank all my friends, especially my lovely wife for her encouragement and support. Zhejiang Medicine and Hygiene Research Program (2015KYA100) supports the work.
13419
Int J Clin Exp Med 2015;8(8):13417-13420
Three different methods for treating Ollier’s disease in one hand Disclosure of conflict of interest None. Address correspondence to: Hui Lu, Department of Hand Surgery, The First Affiliated Hospital, College of Medicine, Zhejiang University, #79 Qingchun Road, Hangzhou 310003, Zhejiang Province, P. R. China. E-mail: [email protected]; Xiang-Jin Lin, Department of Orthopedics, The First Affiliated Hospital, College of Medicine, Zhejiang University, #79 Qingchun Road, Hangzhou 310003, Zhejiang Province, P. R. China. E-mail: drlinxiangjin@hotmail. com
References [1] [2] [3] [4]
Heckman JA. Ollier’s disease. AMA Arch Surg 1951; 63: 861-865. Silve C and Juppner H. Ollier disease. Orphanet J Rare Dis 2006; 1: 37. Noble J and Lamb DW. Enchondromata of bones of the hand. A review of 40 cases. Hand 1974; 6: 275-284. Vazquez-Garcia B, Valverde M and San-Julian M. [Ollier disease: benign tumours with risk of malignant transformation. A review of 17 cases]. An Pediatr (Barc) 2011; 74: 168-173.
13420
[5]
Verdegaal SH, Bovee JV, Pansuriya TC, Grimer RJ, Ozger H, Jutte PC, San Julian M, Biau DJ, van der Geest IC, Leithner A, Streitburger A, Klenke FM, Gouin FG, Campanacci DA, MarecBerard P, Hogendoorn PC, Brand R and Taminiau AH. Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients. Oncologist 2011; 16: 1771-1779. [6] Tordai P, Hoglund M and Lugnegard H. Is the treatment of enchondroma in the hand by simple curettage a rewarding method? J Hand Surg Br 1990; 15: 331-334. [7] Miyawaki T, Kinoshita Y and Iizuka T. A case of Ollier’s disease of the hand. Ann Plast Surg 1997; 38: 77-80. [8] Uzunismail A. Multiple enchondroma. Plast Reconstr Surg 1995; 95: 598-599. [9] Klausmeyer MA, Cohen MJ and Kulber DA. Reconstruction of Ollier disease in a severely involved hand. Ann Plast Surg 2013; 71: 646648. [10] Hasselgren G, Forssblad P and Tornvall A. Bone grafting unnecessary in the treatment of enchondromas in the hand. J Hand Surg Am 1991; 16: 139-142.
Int J Clin Exp Med 2015;8(8):13417-13420
Original Article Three different methods for treating multiple enchondromatosis in one hand Hui Lu1*, Qiang Chen1, Hui Shen1, Xiang-Qian Shen1, Shou-Cheng Wu1, Xiang-Jin Lin2* Departments of 1Hand Surgery, 2Orthopedics, The First Affiliated Hospital, College of Medicine, Zhejiang University, #79 Qingchun Road, Hangzhou 310003, Zhejiang Province, P. R. China. *Equal contributors. Received June 12, 2015; Accepted August 2, 2015; Epub August 15, 2015; Published August 30, 2015 Abstract: Ollier’s disease remains comparatively rare, and is a non-hereditary cartilage dysplasia of bone. It is usually associated with problems such as deformity and fracture. Three different methods were used in a one-hand of 15-year-old boy reporting his pain in the left hand and swellings. After the curettage of tumor, regarding as the differences of all parts of the bone structure reconstruction in the patient’s hand, we chose three following methods for this boy, i.e. fixed by the locking plate with calcium phosphate cement, filled with allograft bone, curetted the tumor without any bone graft. After the surgery, the patient was able to perform full motion of the operated hand. No evidence of recurrence was noted four years after surgery. To choose the different ways with bone grafts or not that relies on the patients’ conditions for bone structure reconstruction. However, patients with large osseous defects or pathological fracture, we demand full bone graft and reliable internal fixation. After surgery, early exercises can reach a desirable result and functional recovery. Keywords: Ollier’s disease, enchondromatosis, bone graft
Background Enchondromatosis is a form of osteochondrodysplasia characterized by a proliferation of enchondromas. Multiple enchondromatosis (Ollier’s disease), originally reported by Ollier in 1899, is a rare and non-hereditary cartilage dysplasia of bone, consisting of multiple, asymmetrically distributed, intraosseous cartilaginous foci and subperiosteal deposition of cartilage, either exclusively or predominantly involving one side of the body. It arises in the medullary cavity and grows in the cortex, forming a prominent endogenous mass of the bone. Some patients will not accept the medical treatment until a pathological fracture occurs. And also the enchondroma will not be discovered until some patients take radiographs after trauma. We report a 15-year-old boy’s case with three different treatments in one hand. There have not been any articles reporting different treatments that are used on the same patient. Case presentation A 15-year-old teenage boy came to the Hand Clinic in the First affiliated hospital, Zhejiang
University, complaining of the pain that was lasting for half a year in his left hand. We found swellings and deformities on his dorsal of hand and in his proximal phalanx of middle and ring fingers respectively. There was no history of injury, systemic symptoms or operation. The skin and tendon were unaffected. Laboratory studies were within normal findings. Radiographs showed expansion of bone and thinning of cortex, endosteal scalloping, cortical thinning in the 3rd~4th metacarpal shaft and the proximal and middle phalanx of the middle and ring fingers respectively. The lesions caused deformities of the 3rd~4th metacarpal shaft and the 4th metacarpal shaft had a pathologic fracture. The middle phalanx of the index finger had only thinning of one side cortex. The radiographs findings showed no signs of aggressive activity (Figure 1). Based on the clinical presentation and radiographic findings, we diagnosed this case as a benign tumor. An incisional biopsy procedure could not be performed in order to avoid the wound contamination. A dorsal skin incision was made under general anesthesia so as that the overlying extensor tendon was exposed longitudinally and mobilized medially or laterally. A cortical window was
Three different methods for treating Ollier’s disease in one hand fixation was removed and the patient’s functions presented normally. No evidence of recurrence was noted four years after surgery. Bone regeneration was noted both radiographically and clinically (Figure 3). Discussion
Figure 1. Preoperative radiograph of the left hand, showing. The middle phalanx of the index finger had only thinning of one side cortex. The 3rd~4th metacarpal shaft and the proximal and middle phalanx of the middle and ring fingers had expansion and thinning of the cortices. The 4th metacarpal shaft had a pathologic fracture.
made to allow the entire tumor to be exposed, and through the window it was curetted. Due to the early functional exercises requirements, the pathologic forth metacarpal fracture and the intramedullary canal were filled with calcium phosphate cement (Wright, USA). The dorsal metacarpal shaft were fixed by the locking plate(depuysynthes, USA). Proximal and middle phalanx of the middle and ring fingers were filled with allograft bone. The middle phalanx of the index finger was only curetted without any bone graft. Considering the patient’s age, we didn’t use autogenous bone as the material of bone graft. Then the wounds were covered. Histologic findings revealed benign cartilaginous patterns of multiple enchondromatosis. There was no evidence of hemangiomas, which was consistent with the diagnosis of Ollier’s disease. Three days after the surgery, the patient was able to perform full motion of the operated hand. Three months after the surgery, the patient could do all weight exercises (Figure 2). One year after the surgery, the locking plate 13418
Ollier’s disease, a rare non-hereditary skeletal disorder, is characterized by multiple enchondromas with a predilection for unilateral cosmetic problems such as deformity and fracture. The enchondromas grow large with progressive skeletal growth, become more evident and disfigure. After the cessation of growth and development, the enchondromas do not enlarge [1]. The rate of occurrence of this disease is 1/100,000, which is equal between men and women [2]. In general, enchondromas are involved in the short tubular bones of the hands and feet, and also found incidentally in the distal femurs, and proximal humerus. They undergo malignant transformation in less than 1% of cases [3]. Malignant transformation and development of extra skeletal cancers are the main complications of multiple enchondromatosis. It has up to 30% risk of malignant chondrosarcomatous transformation [4]. Patients with enchondromas of the small bones of the limbs have the lowest risk to develop malignancy (14%), whereas enchondromas affecting the long axial bones show the highest risk (44%-55%) [5]. Multiple enchondromas and hemangiomas of soft tissue constitute a condition known as Maffucci’s Syndrome. It is less common than Ollier disease, but has a higher risk of chondrosarcomatous change. Other cartilaginous lesions include bone infarct, low-grade chondrosarcoma, epidermoid inclusion cyst, chondromyxoid fibroma, unicameral bone cyst, giant cell tumor and fibrous dysplasia. Our case didn’t destruct the cortex or enter the soft tissues, so benign tumors should be firstly considered. Surgery remains the only effective solution to remove the enchondroma and prevent malignant transformation. Surgical resection to multiple enchondromas of the hand includes curettage, curettage with autografts or allografts and amputations [6-8]. According to Klausmeyer MA et al. and Hasselgren G et al., despite large bony deficits after resection, bony regrowth occurs under the circumstance of without bone autograft [9, 10]. Int J Clin Exp Med 2015;8(8):13417-13420
Three different methods for treating Ollier’s disease in one hand Figure 3. After four years postoperative radiograph of the left hand, showing No evidence of recurrence was noted. Bone regeneration repairs bone defect.
Our case obviously showed bony defects and pathological fracture. Concerned about stable fixation and the initiation of early rangeof-motion exercises, we chose the calcium phosphate cement to fill with the defect of metacarpal shafts and then the metacarpal shafts were fixed with the locking plate. Proximal phalanx of the middle finger, proximal and middle phalanx of ring fingers were filled with allograft bone. The middle phalanx of the index finger was only curettage of the intramedullary. Three different treatments in one hand had a satisfactory result. Figure 2. After three months postoperative radiograph of the left hand, showing Pathologic fracture has healed, internal fixation has presented stable and bone grafting is without bone absorption.
Conclusions
This case report emphasizes that according to the patient’s conditions of bone structure, we choose different treatments including bone grafts and without bone grafts. If patients have large osseous defects or pathological fracture, we demand bone graft and reliable internal fixation. After surgery, early exercises can reach a desirable result and functional recovery. Acknowledgements First and foremost, I would like to show my deepest gratitude to my colleagues Dr. Qiang Chen, Dr. Hui Shen, Dr. Xiang-Qian Shen, Dr. Shou-Cheng Wu, Dr. Xiang-Jin Lin who have provided me with valuable assist in every stage of writing this paper. Meanwhile, I also appreciate Zhejiang Medicine and Hygiene Research Program for sponsoring our research (the grant number 2015KYA100). Last but not least, I’d like to thank all my friends, especially my lovely wife for her encouragement and support. Zhejiang Medicine and Hygiene Research Program (2015KYA100) supports the work.
13419
Int J Clin Exp Med 2015;8(8):13417-13420
Three different methods for treating Ollier’s disease in one hand Disclosure of conflict of interest None. Address correspondence to: Hui Lu, Department of Hand Surgery, The First Affiliated Hospital, College of Medicine, Zhejiang University, #79 Qingchun Road, Hangzhou 310003, Zhejiang Province, P. R. China. E-mail: [email protected]; Xiang-Jin Lin, Department of Orthopedics, The First Affiliated Hospital, College of Medicine, Zhejiang University, #79 Qingchun Road, Hangzhou 310003, Zhejiang Province, P. R. China. E-mail: drlinxiangjin@hotmail. com
References [1] [2] [3] [4]
Heckman JA. Ollier’s disease. AMA Arch Surg 1951; 63: 861-865. Silve C and Juppner H. Ollier disease. Orphanet J Rare Dis 2006; 1: 37. Noble J and Lamb DW. Enchondromata of bones of the hand. A review of 40 cases. Hand 1974; 6: 275-284. Vazquez-Garcia B, Valverde M and San-Julian M. [Ollier disease: benign tumours with risk of malignant transformation. A review of 17 cases]. An Pediatr (Barc) 2011; 74: 168-173.
13420
[5]
Verdegaal SH, Bovee JV, Pansuriya TC, Grimer RJ, Ozger H, Jutte PC, San Julian M, Biau DJ, van der Geest IC, Leithner A, Streitburger A, Klenke FM, Gouin FG, Campanacci DA, MarecBerard P, Hogendoorn PC, Brand R and Taminiau AH. Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients. Oncologist 2011; 16: 1771-1779. [6] Tordai P, Hoglund M and Lugnegard H. Is the treatment of enchondroma in the hand by simple curettage a rewarding method? J Hand Surg Br 1990; 15: 331-334. [7] Miyawaki T, Kinoshita Y and Iizuka T. A case of Ollier’s disease of the hand. Ann Plast Surg 1997; 38: 77-80. [8] Uzunismail A. Multiple enchondroma. Plast Reconstr Surg 1995; 95: 598-599. [9] Klausmeyer MA, Cohen MJ and Kulber DA. Reconstruction of Ollier disease in a severely involved hand. Ann Plast Surg 2013; 71: 646648. [10] Hasselgren G, Forssblad P and Tornvall A. Bone grafting unnecessary in the treatment of enchondromas in the hand. J Hand Surg Am 1991; 16: 139-142.
Int J Clin Exp Med 2015;8(8):13417-13420
