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The treatment of haemophilic intraosseous bone cyst of the talus
Original Article
The treatment of haemophilic intraosseous bone cyst of the talus Michael Heim, Amos Schindler, Boaz Lieberman, Yehuda Amit and Uri Martinowitz
Professor Michael Heim MBChB, Department of Orthopaedic Surgery, Sheba Medical Center, affiliated with the Sackler School of Medicine, University of Tel Aviv, Israel; The Israel National Haemophilia Center, Tel Hashomer Hospital, Tel Hashomer 52621, Israel. Tel.: +972 3 5303702; Fax: +972 9 7401208 Amos Schindler MD, Boaz Lieberman MD, Yehuda Amit MD, Department of Orthopaedic Surgery, Sheba Medical Center, affiliated with the Sackler School of Medicine, University of Tel Aviv, Israel Uri Martinowitz MD, The Israel National Haemophilia Center, Tel Hashomer Hospital, Tel Hashomer, Israel
Subchondral bone cysts are commonly noted in radiographs of joints of persons with haemophilia. Articular chondral integrity is partially dependent upon a trans-osseal blood supply. Bone cysts disrupt this physiological pathway. Ensuing chondrolysis may expose the cyst contents to the joint cavity. This article reports upon the clinical and radiological improvement in a child with a subchondral bone cyst of the talus which was surgically treated. One may hypothesise regarding the connection between “open” chondral cysts and chronic intra-articular synovitis which leads to the frequently noted osteoarthropathy. © 2002 Elsevier Science Ltd. All rights reserved
Introduction The talus (astrogalus) has been referred to as the keystone with regard to its biomechanical function. Vertically acting forces down the shin are partially dissipated horizontally and anteriorly within the hindfoot. The osseous keystone not only translates forces but is the essence of ankle motion. The trochea, or dome, articulates superiorly against the tibial plafond and the sides articulate against both malleolii. Movement of the lateral malleolus, during dorsal and planter flexion, ensures that the talus remains well contained and stable within the ankle mortise. The motion of the talus is not in an exact coronal plane and the bone shape changes during skeletal maturity (Davies 1962). The vascular supply of the talus is multiple, very intricate and complicated and, although theoretically abundant, avascular necrosis of the bone is a feared complication, particularly in displaced fractures (Kundel et al. 1995). The reparative potential of the bone is arduous and unpredictable. There are no muscular attachments to the talus and the surface comprises of three major articular surfaces whose chondral articular coverings require healthy
198 The Foot (2002) 12, 198–202 doi: 10.1016/S0958-2592(02)00028-7, available online at http://www.idealibrary.com on
subchondral bone in order to sustain chondral replenishment and nutrition. The ankle joint in haemophilia is one of the most frequently involved in repeated haemarthrosis (Duthie et al. 1972). The free blood within the joint space is the cause for synovial hypertrophy and the proteolytic enzymes released into the joint space together with the free iron molecules originating from the degraded red blood cells have a deleterious effect upon chondral integrity (Stein & Duthie 1981). Subchondral, interosseous bleeds result in cyst formation and this too has a negative effect upon the cartilage’s ability to receive nourishment via the bone. In chondral fractures it has been postulated that the fibrous tissue of the reparative process interferes with blood vessel penetration to the chondal layers (Berndt & Harty 1959, Loomer et al. 1993). This case study reports the successful outcome of the surgical intervention to the talus in a young boy.
Case history Y.O. is an 11-year-old boy with severe haemophilia A. At the age of 2 years it was
© 2002 Elsevier Science Ltd. All rights reserved
Treatment of haemophilic intraosseous bone cyst of the talus
Figs. 1 & 2 The CT scan shows a section through the talus and on the right side a large bone cyst is clearly visible. Figure 1 The cyst is truly intraosseous. Figure 2 There may be erosion into the joint cavity.
noticed that he had a marked calcaneo-valgus of both feet and he was provided with a set of Helfet heel seats. At the age of 4 years he had periodic episodes of bleeding into the right ankle joint and despite adequate on-demand factor infusion developed some synovial hypertrophy within the joint. This was apparent clinically and a bone scan showed a minor uptake in the ankle joint. An aircast (Aircast, Summit, NJ, USA) orthosis was provided (Buzzard & Heim 1995). There was a positive response and the incidence of haemarthrosis decreased. A year later, weekly ankle bleeds occurred and for a long period the child was provided with prophylactic factor VIII infusions. When breakthrough bleeding episodes occurred, despite the adequate amount of factor provided and the lack of an antibody established, it was decided that surgical intervention would be necessary. Radiological investigations revealed a
© 2002 Elsevier Science Ltd. All rights reserved
huge bone cyst within the talus which looked like erosion through the dome of the talus affecting the articular surface (Figs 1 & 2). Under general anaesthesia and with a tourniquet applied to the limb, an incision was made anterior to the ankle joint and the neck of the talus was identified. A small hole was drilled into the talar neck and under radiological guidance an intraosseous canal was developed until it perforated the sclerotic wall and allowed access into the cyst. A second small skin incision was made over the flare of the proximal tibia, a window opened in the bone cortex and cancellous bone was extracted to be used as a bone graft. Fibrin glue was sprayed into the donor site, the cortical window replaced and the wound sutured. The contents of the talar bone cyst were removed by using a small curette and then the autologous cancellous bone, which had been
The Foot (2002) 12, 198–202 199
Treatment of haemophilic intraosseous bone cyst of the talus
Fig. 3 A & B Anterior/posterior and lateral view of the talus 6 weeks after surgery. On the lateral view one can still identify the bone filling of the cyst.
mixed in fibrin glue, was compressed into the cyst via the canal. The cartilaginous surface of the talar dome covering the cyst had only a very small aperture and the compressed bone, with the glue, did not escape through this aperture. The bone graft plugged the hole. A wound drain was left in situ, the subcutaneous tissues and skin were closed and the limb immobilised in a plaster of Paris cast. Figure 3A & B show the radiological findings 6 weeks after the surgery when the plaster was removed and physiotherapy initiated. The passive range of motion of the ankle joint improved quickly but the child persisted with a poor gait pattern for months. The incidence of bleeding dropped dramatically and 3 years later the child had no limp upon walking. On examination, there was loss of about 40% of ankle dorsiflexion when compared to the contra lateral side. Figure 4A–C show the present state of the talus 3 years after the surgery.
Discussion In this surgical procedure the cyst was accessed via an anterior ankle arthrotomy. In the literature
200 The Foot (2002) 12, 198–202
reports, access has been achieved to the medial side of the talar dome by osteotomising the medial malleolus and displacing it distally (Draper & Fallat 2000). The former procedure mentioned is, in our opinion, far less traumatic. The authors found no reports of a true osteochondral bone graft. Many examples of bone grafts exist (Monroe & Manoli 1999). Draper and Fallat (2000) reported an osteochondral bone graft, but the cartilage used to cover the bone-grafted defect was the chondrolytic cartilage that was present within the joint. No report was found wherein a bone graft that contained its integral chondral surface was found. This would obviously be ideal in cases where a large chondral defect exists. A bone trephine could be used to extract a cartilage-covered bone peg from a non-weight bearing area in the knee and this could then be transplanted into the talus. This case report indicates that intraosseal bone cysts are not as innocuous as previously presumed and when there are recurrent haemarthroses in a joint where a subchondral bone cyst is present, one should suspect that the cyst may be involved in the pathogenesis of hypertrophic synovitis.
© 2002 Elsevier Science Ltd. All rights reserved
Treatment of haemophilic intraosseous bone cyst of the talus
Fig. 4 A–C Anterior/posterior and mortise view of the ankle joint shows no cysts or deformity of the talus, but in the lateral view bone cysts can be seen anterior and posterior to the site of the previously treated pathology. These are non-weight bearing areas.
© 2002 Elsevier Science Ltd. All rights reserved
The Foot (2002) 12, 198–202 201
Treatment of haemophilic intraosseous bone cyst of the talus
References Berndt A L, Harty M 1959 Transchondral fractures (osteochondritis dissecans) of the talus. J Bone Joint Surg 41A: 988–1020 Buzzard B, Heim M 1995 A study to evaluate the effectiveness of ‘Air-Stirrup’ splints as a means of reducing the frequency of ankle haemarthroses in children with haemophilia A and B. Haemophilia 1: 131–136 Draper S D, Fallat L M 2000 Autogenous bone grafting for the treatment of talar bone lesions. J Foot Ankle Surg 39(1): 15–23 Duthie R B, Mathews J M, Rizza C R, Steel W M 1972 The Management of Musculo Skeletal Problems in
202 The Foot (2002) 12, 198–202
the Haemophilias. Oxford: Blackwell Scientific Publication, 29–51 Davies D V (ed) 1962 Gray’s Anatomy, Davies F.33 edn. New York: Longman, 455–458 Kundel K, Braun W, Scherer A 1995 Late results of central talus injuries. Unfallchirurg 98(3): 124–129 Loomer R, Fisher C, Lloyd-Smith R, Sisler J, Cooney T 1993 Osteochondral lesions in the talus. Am J Sport Med 21: 13–19 Monroe M T, Manoli A 1999 Osteotomy for malunion of a talar neck fracture. A case report. Foot Ankle Int 20(3): 192–195 Stein H, Duthie R B 1981 The pathogenesis of chronic haemophilic arthropathy. J Bone Joint Surg 63B: 601–609
© 2002 Elsevier Science Ltd. All rights reserved
The treatment of haemophilic intraosseous bone cyst of the talus Michael Heim, Amos Schindler, Boaz Lieberman, Yehuda Amit and Uri Martinowitz
Professor Michael Heim MBChB, Department of Orthopaedic Surgery, Sheba Medical Center, affiliated with the Sackler School of Medicine, University of Tel Aviv, Israel; The Israel National Haemophilia Center, Tel Hashomer Hospital, Tel Hashomer 52621, Israel. Tel.: +972 3 5303702; Fax: +972 9 7401208 Amos Schindler MD, Boaz Lieberman MD, Yehuda Amit MD, Department of Orthopaedic Surgery, Sheba Medical Center, affiliated with the Sackler School of Medicine, University of Tel Aviv, Israel Uri Martinowitz MD, The Israel National Haemophilia Center, Tel Hashomer Hospital, Tel Hashomer, Israel
Subchondral bone cysts are commonly noted in radiographs of joints of persons with haemophilia. Articular chondral integrity is partially dependent upon a trans-osseal blood supply. Bone cysts disrupt this physiological pathway. Ensuing chondrolysis may expose the cyst contents to the joint cavity. This article reports upon the clinical and radiological improvement in a child with a subchondral bone cyst of the talus which was surgically treated. One may hypothesise regarding the connection between “open” chondral cysts and chronic intra-articular synovitis which leads to the frequently noted osteoarthropathy. © 2002 Elsevier Science Ltd. All rights reserved
Introduction The talus (astrogalus) has been referred to as the keystone with regard to its biomechanical function. Vertically acting forces down the shin are partially dissipated horizontally and anteriorly within the hindfoot. The osseous keystone not only translates forces but is the essence of ankle motion. The trochea, or dome, articulates superiorly against the tibial plafond and the sides articulate against both malleolii. Movement of the lateral malleolus, during dorsal and planter flexion, ensures that the talus remains well contained and stable within the ankle mortise. The motion of the talus is not in an exact coronal plane and the bone shape changes during skeletal maturity (Davies 1962). The vascular supply of the talus is multiple, very intricate and complicated and, although theoretically abundant, avascular necrosis of the bone is a feared complication, particularly in displaced fractures (Kundel et al. 1995). The reparative potential of the bone is arduous and unpredictable. There are no muscular attachments to the talus and the surface comprises of three major articular surfaces whose chondral articular coverings require healthy
198 The Foot (2002) 12, 198–202 doi: 10.1016/S0958-2592(02)00028-7, available online at http://www.idealibrary.com on
subchondral bone in order to sustain chondral replenishment and nutrition. The ankle joint in haemophilia is one of the most frequently involved in repeated haemarthrosis (Duthie et al. 1972). The free blood within the joint space is the cause for synovial hypertrophy and the proteolytic enzymes released into the joint space together with the free iron molecules originating from the degraded red blood cells have a deleterious effect upon chondral integrity (Stein & Duthie 1981). Subchondral, interosseous bleeds result in cyst formation and this too has a negative effect upon the cartilage’s ability to receive nourishment via the bone. In chondral fractures it has been postulated that the fibrous tissue of the reparative process interferes with blood vessel penetration to the chondal layers (Berndt & Harty 1959, Loomer et al. 1993). This case study reports the successful outcome of the surgical intervention to the talus in a young boy.
Case history Y.O. is an 11-year-old boy with severe haemophilia A. At the age of 2 years it was
© 2002 Elsevier Science Ltd. All rights reserved
Treatment of haemophilic intraosseous bone cyst of the talus
Figs. 1 & 2 The CT scan shows a section through the talus and on the right side a large bone cyst is clearly visible. Figure 1 The cyst is truly intraosseous. Figure 2 There may be erosion into the joint cavity.
noticed that he had a marked calcaneo-valgus of both feet and he was provided with a set of Helfet heel seats. At the age of 4 years he had periodic episodes of bleeding into the right ankle joint and despite adequate on-demand factor infusion developed some synovial hypertrophy within the joint. This was apparent clinically and a bone scan showed a minor uptake in the ankle joint. An aircast (Aircast, Summit, NJ, USA) orthosis was provided (Buzzard & Heim 1995). There was a positive response and the incidence of haemarthrosis decreased. A year later, weekly ankle bleeds occurred and for a long period the child was provided with prophylactic factor VIII infusions. When breakthrough bleeding episodes occurred, despite the adequate amount of factor provided and the lack of an antibody established, it was decided that surgical intervention would be necessary. Radiological investigations revealed a
© 2002 Elsevier Science Ltd. All rights reserved
huge bone cyst within the talus which looked like erosion through the dome of the talus affecting the articular surface (Figs 1 & 2). Under general anaesthesia and with a tourniquet applied to the limb, an incision was made anterior to the ankle joint and the neck of the talus was identified. A small hole was drilled into the talar neck and under radiological guidance an intraosseous canal was developed until it perforated the sclerotic wall and allowed access into the cyst. A second small skin incision was made over the flare of the proximal tibia, a window opened in the bone cortex and cancellous bone was extracted to be used as a bone graft. Fibrin glue was sprayed into the donor site, the cortical window replaced and the wound sutured. The contents of the talar bone cyst were removed by using a small curette and then the autologous cancellous bone, which had been
The Foot (2002) 12, 198–202 199
Treatment of haemophilic intraosseous bone cyst of the talus
Fig. 3 A & B Anterior/posterior and lateral view of the talus 6 weeks after surgery. On the lateral view one can still identify the bone filling of the cyst.
mixed in fibrin glue, was compressed into the cyst via the canal. The cartilaginous surface of the talar dome covering the cyst had only a very small aperture and the compressed bone, with the glue, did not escape through this aperture. The bone graft plugged the hole. A wound drain was left in situ, the subcutaneous tissues and skin were closed and the limb immobilised in a plaster of Paris cast. Figure 3A & B show the radiological findings 6 weeks after the surgery when the plaster was removed and physiotherapy initiated. The passive range of motion of the ankle joint improved quickly but the child persisted with a poor gait pattern for months. The incidence of bleeding dropped dramatically and 3 years later the child had no limp upon walking. On examination, there was loss of about 40% of ankle dorsiflexion when compared to the contra lateral side. Figure 4A–C show the present state of the talus 3 years after the surgery.
Discussion In this surgical procedure the cyst was accessed via an anterior ankle arthrotomy. In the literature
200 The Foot (2002) 12, 198–202
reports, access has been achieved to the medial side of the talar dome by osteotomising the medial malleolus and displacing it distally (Draper & Fallat 2000). The former procedure mentioned is, in our opinion, far less traumatic. The authors found no reports of a true osteochondral bone graft. Many examples of bone grafts exist (Monroe & Manoli 1999). Draper and Fallat (2000) reported an osteochondral bone graft, but the cartilage used to cover the bone-grafted defect was the chondrolytic cartilage that was present within the joint. No report was found wherein a bone graft that contained its integral chondral surface was found. This would obviously be ideal in cases where a large chondral defect exists. A bone trephine could be used to extract a cartilage-covered bone peg from a non-weight bearing area in the knee and this could then be transplanted into the talus. This case report indicates that intraosseal bone cysts are not as innocuous as previously presumed and when there are recurrent haemarthroses in a joint where a subchondral bone cyst is present, one should suspect that the cyst may be involved in the pathogenesis of hypertrophic synovitis.
© 2002 Elsevier Science Ltd. All rights reserved
Treatment of haemophilic intraosseous bone cyst of the talus
Fig. 4 A–C Anterior/posterior and mortise view of the ankle joint shows no cysts or deformity of the talus, but in the lateral view bone cysts can be seen anterior and posterior to the site of the previously treated pathology. These are non-weight bearing areas.
© 2002 Elsevier Science Ltd. All rights reserved
The Foot (2002) 12, 198–202 201
Treatment of haemophilic intraosseous bone cyst of the talus
References Berndt A L, Harty M 1959 Transchondral fractures (osteochondritis dissecans) of the talus. J Bone Joint Surg 41A: 988–1020 Buzzard B, Heim M 1995 A study to evaluate the effectiveness of ‘Air-Stirrup’ splints as a means of reducing the frequency of ankle haemarthroses in children with haemophilia A and B. Haemophilia 1: 131–136 Draper S D, Fallat L M 2000 Autogenous bone grafting for the treatment of talar bone lesions. J Foot Ankle Surg 39(1): 15–23 Duthie R B, Mathews J M, Rizza C R, Steel W M 1972 The Management of Musculo Skeletal Problems in
202 The Foot (2002) 12, 198–202
the Haemophilias. Oxford: Blackwell Scientific Publication, 29–51 Davies D V (ed) 1962 Gray’s Anatomy, Davies F.33 edn. New York: Longman, 455–458 Kundel K, Braun W, Scherer A 1995 Late results of central talus injuries. Unfallchirurg 98(3): 124–129 Loomer R, Fisher C, Lloyd-Smith R, Sisler J, Cooney T 1993 Osteochondral lesions in the talus. Am J Sport Med 21: 13–19 Monroe M T, Manoli A 1999 Osteotomy for malunion of a talar neck fracture. A case report. Foot Ankle Int 20(3): 192–195 Stein H, Duthie R B 1981 The pathogenesis of chronic haemophilic arthropathy. J Bone Joint Surg 63B: 601–609
© 2002 Elsevier Science Ltd. All rights reserved