Orthopedics

Treatment of Multiple Enchondromatosis (Ollier's Disease) of the Hand

John F Fatti, MD; John F Mosher, MD

Abstract

ABSTRACT: The clinical treatment and results for three hands in two patients with multiple enchondromatosis Ollier's disease) are presented. Diaphyseal resection (total or subtotal) in 27 phalanges and metacarpals preceded autogenous fibular bone grafting to the defects. All bone grafts subsequently healed and were well incorporated. Hand function and cosmesis were restored satisfactorily in each case. There were no infections or recurrences.

Abstract

ABSTRACT: The clinical treatment and results for three hands in two patients with multiple enchondromatosis Ollier's disease) are presented. Diaphyseal resection (total or subtotal) in 27 phalanges and metacarpals preceded autogenous fibular bone grafting to the defects. All bone grafts subsequently healed and were well incorporated. Hand function and cosmesis were restored satisfactorily in each case. There were no infections or recurrences.

Introduction

Enchondromas are the most common tumors arising in the bones of the hand. >·2 They may present as solitary hand lesions or may be multiple in conjunction with the clinical condition known as Ollier's Disease (multiple enchondromatosis).

Enchondromas consist of circumscribed masses of mature hyaline cartilage in the interior of enchondrally formed bones. These expansile masses of cartilage may thin the endosteal surface of the cortex so that only periosteum remains. The cortical thinning may lead to pathologic fractures. The resultant deformity, scarring and limitation of tendon excursion, as well as the significant size obtainable by these cartilagenous tumors, may substantially limit finger joint motion and hand function.

Methods of dealing with enchondromas in the hand have included curettage alone,3 curettage and grafting with autogenous3 or freeze-dried homologous cancellous bone,4 and en bloc diaphysectomy with autogenous corticocancellous strut grafting.3-9

Two cases are presented, one of which was reported previously.9 The methods and results of en bloc diaphysectomy and autogenous fibular strut grafting for treating multiple enchondromatosis of the bones of the hand will be discussed.

Case 1: RJ was first seen at the age of 6, in 1967, with asymptomatic swellings of the index, long, and ring fingers of the right hand. Multiple enchondromas were diagnosed roentgenographically. The patient was followed at yearly intervals and referred to Upstate Medical Center when he was 11, in 1972. The swellings had slowly enlarged and interfered with function of the dominant right hand. Several episodes of sudden pain and swelling in one of the involved fingers were associated with trauma and probably represented pathological fractures.

On physical examination, the only visible or palpable abnormalities were confined to the right hand. The hard, painless masses were in the rays of the index, long, and ring fingers (Fig. 1). The hand was as large and strong as the left and the patient could make a "good" fist.

Roentgenograms showed the expected large lesions in the rays of the index, long, and ring fingers. In addition, a small cortical irregularity was present in the middle third of the left fibula and there was a small defect of the proximal phalanx of the left third toe.

The patient was progressively unable to use the'dominant right hand for heavy manual activity because of the osseous fragility. He had suffered at least three probable fractures in the involved fingers during the 6 months prior to surgery, all accompanied by sudden pain and swelling which resolved over the ensuing weeks. Because of the fragility of the bones, surgical intervention was advised.

Surgical reconstruction of the right hand was begun in April 1973. The proximal phalanges were most severely involved and me diaphyses of these bones were replaced first (Fig. 2). The dorsal surface of the proximal phalanx was approached through a straight longitudinal incision, splitting the central slip of the extensor mechanism. The diaphysis was excised through an incision across the proximal physeal plate and another was made distally at the level of the attachment of the proximal interphalangeal joint capsule to the head of the proximal phalanx. The middle third of the left fibula was excised subperiosteal^. The periosteal sleeve was closed and the fibular bone was cut into plugs and shaped with the power saw. The plugs were then impacted into the defects between the epiphyses and the heads of the proximal phalanges. After closure of the extensors and skin, a compression dressing was applied and me hand was immobilized for 3 weeks. Protected exercises were then started within the confines of a dorsal splint. Within 2 months of surgery, all finger motion had returned to the preoperative range.

In January 1974, diaphysectomy of the index, Jong, and ring finger metacarpals was performed. The distal margin of resection was through the physis and the proximal margin was determined by tumor formation. As in the first surgery, only a few fine volar cortical struts of diaphysis remained . The middle third of the right fibula was excised subperiosteally, cut into three fragments, and shaped to fit the defects (Fig. 3). The hand was again immobilized for 3 weeks and protective active motion was begun. Full preoperative motion had returned by 2 months postoperatively.

Fig. IA: Preoperative photograph of the right hand of case 1 (age 12). Note the multiple masses which were easily palpable.

Fig. IA: Preoperative photograph of the right hand of case 1 (age 12). Note the multiple masses which were easily palpable.

In April 1974, diaphysectomy of the middle phalanges of the long and ring fingers was performed and the defects were filled with strong corticocancellous struts from the right iliac crest (Fig. 4). The middle phalanx of the index finger had only a small area of involvement; this was curetted and burred with the power drill and packed with cancellous iliac crest bone. Again, the full preoperative range of motion had returned by 2 months after surgery (Fig. 5, 6).

As of December 1982, the patient had maintained the excellent range of motion evidenced 2 months postoperatively. His right hand was still dominant and its function was completely satisfactory. He was having no problems in carrying out his full-time job with the United States Marines. A phone conversation in April 1984 reconfirmed his continued satisfaction with his right hand.

Case 2: JL presented to her physician on March 3, 1980 with asymptomatic bilateral hand deformities as a 5-year-old white female. Physical examination and x-rays done then revealed multiple enchondromatosis involving many of the bones of the hand as well as subclinical enchondromata in all the long bones of the upper and lower extremities. As she was asymptomatic, and her hands fully functional at this time, she was followed expectantly.

Fig. IB: Preoperative AP radiograph of the hand shows multiple enchondromata, most notably in the proximal phalanges.

Fig. IB: Preoperative AP radiograph of the hand shows multiple enchondromata, most notably in the proximal phalanges.

In November of 1983, at age 8, she began to complain of progressive bothersome enlargement of the enchondromata of her hands as well as intermittent pain. The enlargement and pain interfered with hand function (Fig. 7). The metacarpal phalangeal and interphalangeal joint motion averaged 50% of normal due to the size and pain associated with these masses. There were no neurovascular deficits, but hand x-rays (Fig. 8) revealed some very large enchondromata mainly affecting the proximal and middle phalanges and the metacarpal neck regions of the middle three rays.

Because her left hand was more symptomatic, in December of 1982, she underwent surgical excision of the enchondromata of the diaphyses of the proximal and middle phalanges of the left index, long, ring and little fingers through a dorsal extensor tendon-splitting approach. A 15 cm section of fibula that was subperiosteally resected from the patient's left leg was used as autogenous bone graft. Correctly measured lengths of one-half of the diameter of the fibula were "PnSSs-Uf into the bony diaphyseal defects (Fig. 9) and fixed with Kirschner wires that crossed the DIP and PIP joints of the central three digits. In almost each of the phalanges, the entire diaphysis (including the volar cortex and the physis) was replaced with cartilagenous tumor so that no bony continuity between the proximal and distal aspects of the phalanx remained.

Fig. 2: Postoperative AP radiograph showing diaphysectomy and fibular bone grafting to the proximal phalanges of the middle three rays.

Fig. 2: Postoperative AP radiograph showing diaphysectomy and fibular bone grafting to the proximal phalanges of the middle three rays.

The patient's postoperative splint was changed on day five and metacarpal phalangeal joint motion was begun. The pins crossing the DIP and PIP joints were removed at 4 weeks and IP motion was then begun. Presently, she has full motion at her MP joints and an average of 70° of PIP flexion 18 months postsurgery. There are no rotational or angulatory deformities. There were no infections or recurrences in the eight phalanges done. Incorporation of all eight fibular grafts was complete (Fig. 10). The hand is fully functional and cosmesis is good except for the metacarpal neck enchondromata which are to be resected and grafted in a later operation.

On July 20, 1984, because of the progressive decreasing right hand function due to enlarging enchondromata, the same technique and same section of regenerated left fibula was used to resect and bone graft the enchondromata involving the proximal and middle phalanges of the right index, long, and ring fingers and all five right hand metacarpals. Again, the dorsal approach was used. Thirteen months after this surgery, she has achieved nearly normal MP motion and an average of 60° of PIP motion of the right hand has shown good incorporation and healing of all 11 fibular grafts (Fig. 11). She is completely satisfied with me cosmesis of her right hand.

Fig. 3: The right fibular strut seen in place, keyed into the ends of the index metacarpal that were not involved with tumor. The metacarpals of the three middle rays were grafted during this procedure.

Fig. 3: The right fibular strut seen in place, keyed into the ends of the index metacarpal that were not involved with tumor. The metacarpals of the three middle rays were grafted during this procedure.

Fig. 4: AP radiograph of the right hand (case 1) in April 1974, prior to surgery on the middle phalanges. This film shows good incorporation of the fibular grafts replacing the proximal phalanges and metacarpals previously operated.

Fig. 4: AP radiograph of the right hand (case 1) in April 1974, prior to surgery on the middle phalanges. This film shows good incorporation of the fibular grafts replacing the proximal phalanges and metacarpals previously operated.

Fig. 5: AP radiograph in 1979, 5 years after completion of all surgical procedures. There have been no recurrences of enchondromata.

Fig. 5: AP radiograph in 1979, 5 years after completion of all surgical procedures. There have been no recurrences of enchondromata.

Fig. 7: Hand photographs of case 2 (age 8).

Fig. 7: Hand photographs of case 2 (age 8).

The pathological slides in both hand procedures showed multiple curetted fragments of hyaline cartilage with mild cellular atypia consistent with the diagnosis of multiple enchondromatosis (Ollier's disease) (Fig. 12).

Fig. 6A, B: Photographs of the right hand postoperatively (case 1).

Fig. 6A, B: Photographs of the right hand postoperatively (case 1).

Fig. 8: Case 2 radiographs preoperati vely.

Fig. 8: Case 2 radiographs preoperati vely.

Discussion

Ollier's Disease (multiple enchondromatosis) is a congenital, non-hereditary rare disease usually affecting one side of the body more than the other. It usually presents between the ages of 5 and 7 when stunted growth and hand deformities are noted. The multiple enchondromata bring potential problems to these patients. Leg length discrepancy, angular deformity of long bones, pathologic fractures, and malignant degeneration of enchondromata to chondrosarcomas are the main clinical problems.

Malignant degeneration of enchondromas to chondrosarcomas, while occurring in the long bones at rates quoted between 1% and 25%,10 is very rare in the hand." There are only 21 cases of chondrosarcoma in the hand bones reported in the literature. In only three of these cases could a pre-existing benign enchondroma be histologically documented. Characteristics such as pain, rapid swelling in a pre-existing lesion, concomitant development of a soft tissue mass, and a newly found lytic, ill-defined tumor area in a previously benign looking x-ray pattern should be taken as ominous signs of malignant degeneration of an enchondroma.

Fig. 9A, B: Intraoperative photographs left hand, December 1983 (case 2).

Fig. 9A, B: Intraoperative photographs left hand, December 1983 (case 2).

Subtotal diaphysectomy and autogenous corticocancellous fibular bone grafting was found to be a definitive and successful way to treat enchondromas of the hand in patients with multiple enchondromatosis. The results in three hands in two patients (27 phalanges and metacarpals) treated in this manner showed good functional and cosmetic restoration of the hand with no problems referable to infection, recurrence, angular or rotatory deformity, or bone graft nonunion. There are several technical points that deserve mentioning. The section of fibula used for bone graft can be cut into sections comprising one-half the diameter of the fibula, this substantially reduced the length of fibula necessary to complete the bone grafting for each operation. In the second case the full diameter of the fibula was too large to substitute for her phalangeal diaphyses, and sagittal sectioning was imperative. Also, there was no problem in harvesting the same section of regenerated fibula during subsequent operations. In case 2, the midportion of the left fibula was used twice and will be used a third time to replace the enchondromata in the metacarpals of the left hand. We now recommend the use of the same fibula for serial bone grafting procedures provided that regeneration is radiologically proven preoperatively.

Another technical point of some importance concerns the need for resection of the growth plate during diaphysectomy. In our cases, it was almost universally necessary to include the phalangeal and metacarpal physes with the resected enchondromata. The tumor and growth plate were often directly adjacent to one another with no distinguishing demarcation zone. It is thought that enchondromata originate and grow from aberrancies in their respective physes.12 We recommend that the physis be resected with the adjacent diaphyseal enchondroma if close proximity is demonstrated. Though this sacrifices longitudinal bone growth, we feel this helped reduce the chances of recurrence.

Other authors have used this method of diaphyseal resection and bone grafting to deal with large enchondromas in the hand. Takigawa3 used this method on five bones in two patients with one excellent and one fair result. His graft material was corticocancellous iliac crest. Giannikas5 had good results in all three bones in me three hands that he performed this procedure upon. He used a cortical bone strip from the proximal ulna in each of his cases. With the major amount of bony reconstruction necessary in this type of procedure, we were surprised at the return of near normal joint motion and tendon gliding. The main reason for this probably was the lack of tumorous involvement in the finger joints in these two cases.

Fig. 1OA, B, C: Postoperative radiographs (case 2), left hand, showing the incorporation of the bone grafts.

Fig. 1OA, B, C: Postoperative radiographs (case 2), left hand, showing the incorporation of the bone grafts.

Fig. 11: AP radiograph (case 2) of the right hand 8 weeks postoperatively. Note the early incorporation of the fibular grafts.

Fig. 11: AP radiograph (case 2) of the right hand 8 weeks postoperatively. Note the early incorporation of the fibular grafts.

Our recommendations for treatment of patients with multiple enchondromatosis of the hands are: 1 ) clinical observation for young patients with asymptomatic multiple hand enchondromata (as long as there are no symptoms and no fractures, expectant treatment will preserve longitudinal growth in the bones of the hand); 2) skeletal survey at a young age to discover other subclinical enchondromata and to initiate a search for future leg length discrepancy; 3) for small symptomatic phalangeal or metacarpal enchondromata. curettage and autogenous cancellous or bone bank graft is the treatment of choice: and 4) for extensive enchondromata that involve the major portion of the bone in question, a subtotal or total diaphysectomy and autogenous fibular bone grafting achieved good results as seen in the two patients presented.

Fig. 12: Pathological section from one of the enchondromas (case 2) of the left hand. One notes bone in the lower part of the photograph and a cellular cartilagenous matrix above consistent with Ollier's Disease.

Fig. 12: Pathological section from one of the enchondromas (case 2) of the left hand. One notes bone in the lower part of the photograph and a cellular cartilagenous matrix above consistent with Ollier's Disease.

References

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2. Coley BL. Higinboiham NL: Tumors primary in the bones of the hands and fed. Surgery 1939; 5:1 12-128.

3. Taktgawa K: Chondroma of the hones of the hand, a review of I (Q cases. / Bone Joint Surg 1971: 53A: 1591-1600.

4. Jewusiak EM. Spence KF. Sell KW: Solitary benign cnchondroma of the long bones of the hand. Results of curettage and packing with freeze-dried cancellous bone allograft. J Bone Joint Surg 1971: 53A:!587-!590.

5. Giannikas AC: Treatment of metacarpal enchondromata. Report of three cases. J Bone Joint Surg 1966; 48B:333-335.

6. Miyakawa G: Replacement of the shaft of the phalanx with iliac hone. J Bone Joint Surg 1961; 43A:905-907.

7. O'Brien B: Solitary enchondroma of the thumb. Aust NZ J Surg 1968; 37:249-252.

8. Scngupta A: Resection of the fourth metacarpal in a late case of cnchondroma with cosmetic and functional reconstruction. Intern Surgery 1968: 50:164-166.

9. Mosher JF: Multiple enchondromatosis of the hand. J Bone Joint Surg 1976; 58A:717-7I9.

10. Dahlin DC: Bone Tumors, ed 2. Springfield. Charles C. Thomas. 1973.

11. Culver JE. Sweet DE. McCue FC: Chondrosarcoma of the hand arising from a prc-existcnt benign solitary enchondroma. Clin Orthop 1975: 113: 128- 131.

12. Bogumill GP. Schwamm HA: Orthopedic Pathology. Philadelphia. WB Saunders Co.. 1984.

10.3928/0147-7447-19860401-09

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