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Hemophilic pseudotumor of the mandible
Hemophilic
pseudotumor of the mandible
Report of a case Grady W. Bryan, DDS,a David G. Leibold, DDS, MD,b and Robert G. Triplett, DDS, PhD,C San Antonio, Texas UNIVERSITY
OF TEXAS
HEALTH
SCIENCE
CENTER
AT
SAN
ANTONIO
Hemophilic pseudotumor of bone is a rarely encountered lesion associated with hemophilia. To date, only eight cases in the mandible have been reported in the literature. A rare case occurring in the mandible of a 2H-year-old child required the fabrication of a customized wound obturator for postoperative wound care afler surgical curettage. The literature is reviewed and the obturator described. (ORALSURG ORAL MEDORALPATHOL 1990;69:550-3)
H
emophilic pseudotumor is an uncommon, but characteristic, manifestation of the rare disease hemophilia. Hemophilic pseudotumor, also known as hemophilic cyst of bone, is an extra-articular destructive osseous lesion causing necrosis of bone by hematoma under pressure.I, * A review of the literature reveals that, to date, only eight cases have been documented in the mandible and none in the maxilla. The first reported case in the mandible was published in 196K3T4 If left untreated, the lesion can cause extensive destruction of bone and surrounding tissues. The purpose of this article is to present a case that occurred in the mandible and to describe the postoperative management. CASE REPORT
In July 1985, a 2%year-old Mexican national boy with diagnosed hemophilia A was brought to Santa Rosa Medical Center, San Antonio, Texas, for evaluation of persistent swelling and bleeding in the region of the right mandibular ramus. An incisional biopsy had been performed in Mexico a week earlier, with a diagnosis of benign dentigerous cyst. Continued oozing and swelling after treatments with cryoprecipitate indicated that inhibitors to factor VIII may have developed in the patient. Radiographic evaluation revealed a radiolucent intraosseous,cystic, expansile lesion of the right mandibular body and ramus approximately 4 cm x 2 cm in diameter (Fig.
aResident, Department of Oral and Maxillofacial Surgery, Uniw-r&v of Texas Health Science Center at San Antonio,
bin private practice, San Antonio, Texas. cProfessor,Department of Oral and Maxillofacial Surgery, University of Texas Health Science Center at San Antonio. 7112111373
550
1). The histopathologic specimen received from Mexico was not diagnostic. Although a notation was made that there was no evidence of neoplasm, the specimen was compatible with a follicular cyst and resorptive characteristics that have been associated with hemorrhagic pseudotumors of hemophilia. The patient was hospitalized and taken to the operating room, where curettage of the mandibular mass was performed. Intraoperative bleeding was routine for the procedure with an estimated blood loss of 125 ml. Over the next
several days, however, the patient continued to have exacerbations of active bleeding. Combined treatment with factor VIII and amino-caproic acid (Amicar) resulted in control of the bleeding. Microscopic examination of the specimenrevealed granulation tissue, hemorrhage, moderate-to-florid fibroblastic proliferation, osteoid production, and new bone formation. The histopathologic diagnosis of pseudotumor of hemophilia was made. Becauseof the patient’s age and recurrent postoperative bleeding, a custom obturator was made to facilitate wound irrigation and management. Nine days after the first surgical procedure, the patient was again taken to the operating room where arch bars were applied to the maxillary and mandibular teeth. An obturator composed of two irrigation tubes and a small acrylic splint was inserted into the osseousdefect (Figs. 2 and 3). One tube was positioned along the dependent portion of the wound and connected to a suction bulb to facilitate drainage. The second tube was positioned superiorly to facilitate irrigation of the wound. The obturator was modified by incrementally adding a soft lining material until a satisfactory fit was obtained. The completed obturator was placed in its proper position, and the terminal portions of the irrigation and drainage tubes were taped to the lateral aspect of the face. It was then possible to irrigate the wound with saline solution while simultaneously evacuating the wound with continuous low suction. The patient
Hemophilic
Volume 69 Number 5
Fig.
1. Radiograph
showing radiolucent
pseudotumor
lesion in region of right mandibular
of mandible
body and ramus.
b
Figs. 2 and 3. Schematic diagram of custom-made obturator placed in mandibular tube. 6, Evacuation tube. c, Self-curing soft liner. d, Acrylic core.
lesion. a, Irrigation
55 I
552
Bryan, Leibold, and Triplett
Fig.
MED ORAL PATHOL May 1990
4. Radiograph 4 months after treatment showing extent of healing of osseousdefect.
was then placed in intermaxillary fixation to safeguard the position of the obturator. The patient was discharged. The family provided wound care for the patient, irrigating the wound four times per day. The patient’s coagulopathy was controlled on an outpatient basis. Approximately 1 month later, the patient returned to the operating room where obturator, irrigation tubes, and arch bars were removed to allow examination of the osseousdefect. There was good granulation tissue growing around the bone margins of the wound. The wound was cleaned with minimal debris and no evidence of infection. The patient progressed to normal healing (Fig. 4). DISCUSSION
The cause of hemophilic pseudotumor is not well understood. The first reported case of hemophilic pseudotumor is credited to Starker5 in 1918. It has an estimated incidence of 1% to 2% and has only been reported in persons with severehemophilia.5b7It is most commonly associated with classic hemophilia or factor VIII deficiency but has also been seen in association with factor IX deficiency, PTC deficiency, hemarthrosis in proconvertin deficiency, and acquired hemophilia. 1,6 * The pathogenesis of hemophilic pseudotumor is hq-@~sizei:
ORAL SURC ORAL
to be a pressure necrosis phenomenon
where bleeding into a closed subperiosteal space or interosseous bleeding may cause enough pressure to
induce necrosis of bone. Hemorrhage associatedwith hemophilia will occur into three distinctive anatomic sites: (1) the joints, which leads to arthropathy; (2) soft tissues, which leads to neuropathy; and (3) in the proximity of bone, which leads to pseudotumor. Three types of pseudotumor have been described: (1) bleeding into a large muscle mass adjacent to bone, which destroys underlying tissues by pressure necrosis; (2) subperiosteal hemorrhage, which causes stripping of the periosteum from the cortex and pressure necrosis of the bone; and (3) intraosseous hemorrhage and necrosis.6 The cause of initial hematoma formation is subject to debate. In most reported cases of pseudotumor, there has been a history of trauma.‘O However, often the interval between the trauma and onset of symptoms has been months or years. Once present, the hematoma resolves slowly, if at all, and is followed by gradual cystic enlargement. The masswill continue to increase in size at irregular intervals, often remaining quiescent for months, only to increase rapidly in size, apparently secondary to another episode of hemorrhage.9 A pseudotumor can remain asymptomatic and unchanged for decadesand then suddenly become the source of bleeding and perforation.t2 Repeated hemorrhage into one of these closed spaces is thought to cause the bony changes associated with pseudotumor.’ Radiographically, skeletal
Hemophilic
Volume 69 Number 5
changesconsist of areas of bone destruction and new bone formation. In some instances, the picture resembles that of malignant skeletal tumors, especially osteosarcoma-hence the name, pseudotumar
SUMMARY
The pathogenesis and radiographic features of hemophilic pseudotumor are discussed. A case involving the mandible and a cutomized method of postoperative wound management are presented.
of mandible
553
REFERENCES i 2
11-14
The femur is the most common site of pseudotumor occurrence, accounting for about one third of all reported cases. This is followed, in order, by the pelvis, tibia, bones of feet, and bones in the hand or wrist.9 Treatment of hemophilic pseudotumors has consisted of surgery, supportive therapy, and radiation.2 Before the availability of factor VIII transfusions, surgical intervention was usually fatal. Supportive care consists of immobilization and administration of factor replacement. This management seems to be successful in lesions of recent onset. Radiation therapy is reportedly useful in cases in which an inhibitor to factor VIII has developed and that are, thus, difficult to control hemostatically, even in the presence of replacement therapy. Inhibitor to factor VIII has been reported to develop in 15% of patients with factor VIII deficiency.6 In this case,surgical removal of the lesion, coupled with obliteration of the space with an obturator and internal irrigation, provided an environment that allowed hemostatic initiation of the reparative process. Early use of such a device may assist the surgeon in reducing the morbidity of the postoperative course.
pseudotumor
3 4
5 6 I 8. 9. 10.
Il. 12. 13. 14.
Abel1 JM, Bailey RW. Hemophilic pseudotumor: two cases occurring in siblings. Arch Surg 1960;81:569-81. Jensen PS, Putnam CE. Hemophilic pseudotumor: diagnosis, treatment, and complications. Am J Dis Child 1975; 129:717-19. Lazarovits P, Griem ML. Radiotherapy of hemophilic pseudotumor. Radiology 1968;91: 1026-27. Brook AH, Bedi R, Chan Lui WY, Yuen PMP. Hemophilic pseudotumors of the mandible: report of a case in a one-yearold child. Br J Oral Maxillofac Surg 1985;23:47-52. Starker L. Knochenusur durch ein hamophiles, subperiostaies hamatom, mitt. Grenzgeb Med Chir 1918;31:381-415. Correra A, Buckley J, Roser S, Schreiber A, Syrop S. Radiotherapy of a pseudotumor in a hemophiliac with Factor VIII inhibitor. Am J Pediatr Hematol Oncol 1984;6:325-27. Mulkev TF. Hemoohilic oseudotumor of the mandible. J Oral Surg lb77;35:561-68. ’ Treble NJ, Henderson NJ, Dassani H. Amputation for pseudotumor in acquired hemophilia. Br Med J [Clin Res] 1984;289: 1349-50. Gilbert MS. Characterizing the hemophilic pseudotumor. Ann NY Acad Sci 1975;240:3<1-15. Marauez JL. Vinaaeras E. Dorantes S. Nussbaumer C. Flares A, Perez Rolon G‘: Hemophilic pseudotumor of the inferior maxilla. ORAL SURC ORAL MED ORAL PATHOL 1982;53:34750. Stoneman DW, Beierl CD. Pseudotumor of hemophilia in mandible. ORAL SURC ORAL MED ORAL PATHOL 1975;40:8 1 I15. Ahlberg AKM. On the natural history of hemophilic pseudotumor. J Bone Joint Surg 1975;57-A:1133-5. Martinez-Lage JL, Sanchez H, Garcia JS, Lorenzo F. A pseudotumor of the mandible in a haemophiliac patient. J Maxillofac Surg 1983;11:171-3. Brant EE, Jordan HH. Radiologic aspects of hemophilic pseudotumors in bone. Am J Roentgen01 1972;115:52539.
Reprint requests to: Dr. Grady W. Bryan Department of Oral and Maxillofacial Surgery University of Texas Health Science Center at San Antonio San Antonio, Texas 78284
pseudotumor of the mandible
Report of a case Grady W. Bryan, DDS,a David G. Leibold, DDS, MD,b and Robert G. Triplett, DDS, PhD,C San Antonio, Texas UNIVERSITY
OF TEXAS
HEALTH
SCIENCE
CENTER
AT
SAN
ANTONIO
Hemophilic pseudotumor of bone is a rarely encountered lesion associated with hemophilia. To date, only eight cases in the mandible have been reported in the literature. A rare case occurring in the mandible of a 2H-year-old child required the fabrication of a customized wound obturator for postoperative wound care afler surgical curettage. The literature is reviewed and the obturator described. (ORALSURG ORAL MEDORALPATHOL 1990;69:550-3)
H
emophilic pseudotumor is an uncommon, but characteristic, manifestation of the rare disease hemophilia. Hemophilic pseudotumor, also known as hemophilic cyst of bone, is an extra-articular destructive osseous lesion causing necrosis of bone by hematoma under pressure.I, * A review of the literature reveals that, to date, only eight cases have been documented in the mandible and none in the maxilla. The first reported case in the mandible was published in 196K3T4 If left untreated, the lesion can cause extensive destruction of bone and surrounding tissues. The purpose of this article is to present a case that occurred in the mandible and to describe the postoperative management. CASE REPORT
In July 1985, a 2%year-old Mexican national boy with diagnosed hemophilia A was brought to Santa Rosa Medical Center, San Antonio, Texas, for evaluation of persistent swelling and bleeding in the region of the right mandibular ramus. An incisional biopsy had been performed in Mexico a week earlier, with a diagnosis of benign dentigerous cyst. Continued oozing and swelling after treatments with cryoprecipitate indicated that inhibitors to factor VIII may have developed in the patient. Radiographic evaluation revealed a radiolucent intraosseous,cystic, expansile lesion of the right mandibular body and ramus approximately 4 cm x 2 cm in diameter (Fig.
aResident, Department of Oral and Maxillofacial Surgery, Uniw-r&v of Texas Health Science Center at San Antonio,
bin private practice, San Antonio, Texas. cProfessor,Department of Oral and Maxillofacial Surgery, University of Texas Health Science Center at San Antonio. 7112111373
550
1). The histopathologic specimen received from Mexico was not diagnostic. Although a notation was made that there was no evidence of neoplasm, the specimen was compatible with a follicular cyst and resorptive characteristics that have been associated with hemorrhagic pseudotumors of hemophilia. The patient was hospitalized and taken to the operating room, where curettage of the mandibular mass was performed. Intraoperative bleeding was routine for the procedure with an estimated blood loss of 125 ml. Over the next
several days, however, the patient continued to have exacerbations of active bleeding. Combined treatment with factor VIII and amino-caproic acid (Amicar) resulted in control of the bleeding. Microscopic examination of the specimenrevealed granulation tissue, hemorrhage, moderate-to-florid fibroblastic proliferation, osteoid production, and new bone formation. The histopathologic diagnosis of pseudotumor of hemophilia was made. Becauseof the patient’s age and recurrent postoperative bleeding, a custom obturator was made to facilitate wound irrigation and management. Nine days after the first surgical procedure, the patient was again taken to the operating room where arch bars were applied to the maxillary and mandibular teeth. An obturator composed of two irrigation tubes and a small acrylic splint was inserted into the osseousdefect (Figs. 2 and 3). One tube was positioned along the dependent portion of the wound and connected to a suction bulb to facilitate drainage. The second tube was positioned superiorly to facilitate irrigation of the wound. The obturator was modified by incrementally adding a soft lining material until a satisfactory fit was obtained. The completed obturator was placed in its proper position, and the terminal portions of the irrigation and drainage tubes were taped to the lateral aspect of the face. It was then possible to irrigate the wound with saline solution while simultaneously evacuating the wound with continuous low suction. The patient
Hemophilic
Volume 69 Number 5
Fig.
1. Radiograph
showing radiolucent
pseudotumor
lesion in region of right mandibular
of mandible
body and ramus.
b
Figs. 2 and 3. Schematic diagram of custom-made obturator placed in mandibular tube. 6, Evacuation tube. c, Self-curing soft liner. d, Acrylic core.
lesion. a, Irrigation
55 I
552
Bryan, Leibold, and Triplett
Fig.
MED ORAL PATHOL May 1990
4. Radiograph 4 months after treatment showing extent of healing of osseousdefect.
was then placed in intermaxillary fixation to safeguard the position of the obturator. The patient was discharged. The family provided wound care for the patient, irrigating the wound four times per day. The patient’s coagulopathy was controlled on an outpatient basis. Approximately 1 month later, the patient returned to the operating room where obturator, irrigation tubes, and arch bars were removed to allow examination of the osseousdefect. There was good granulation tissue growing around the bone margins of the wound. The wound was cleaned with minimal debris and no evidence of infection. The patient progressed to normal healing (Fig. 4). DISCUSSION
The cause of hemophilic pseudotumor is not well understood. The first reported case of hemophilic pseudotumor is credited to Starker5 in 1918. It has an estimated incidence of 1% to 2% and has only been reported in persons with severehemophilia.5b7It is most commonly associated with classic hemophilia or factor VIII deficiency but has also been seen in association with factor IX deficiency, PTC deficiency, hemarthrosis in proconvertin deficiency, and acquired hemophilia. 1,6 * The pathogenesis of hemophilic pseudotumor is hq-@~sizei:
ORAL SURC ORAL
to be a pressure necrosis phenomenon
where bleeding into a closed subperiosteal space or interosseous bleeding may cause enough pressure to
induce necrosis of bone. Hemorrhage associatedwith hemophilia will occur into three distinctive anatomic sites: (1) the joints, which leads to arthropathy; (2) soft tissues, which leads to neuropathy; and (3) in the proximity of bone, which leads to pseudotumor. Three types of pseudotumor have been described: (1) bleeding into a large muscle mass adjacent to bone, which destroys underlying tissues by pressure necrosis; (2) subperiosteal hemorrhage, which causes stripping of the periosteum from the cortex and pressure necrosis of the bone; and (3) intraosseous hemorrhage and necrosis.6 The cause of initial hematoma formation is subject to debate. In most reported cases of pseudotumor, there has been a history of trauma.‘O However, often the interval between the trauma and onset of symptoms has been months or years. Once present, the hematoma resolves slowly, if at all, and is followed by gradual cystic enlargement. The masswill continue to increase in size at irregular intervals, often remaining quiescent for months, only to increase rapidly in size, apparently secondary to another episode of hemorrhage.9 A pseudotumor can remain asymptomatic and unchanged for decadesand then suddenly become the source of bleeding and perforation.t2 Repeated hemorrhage into one of these closed spaces is thought to cause the bony changes associated with pseudotumor.’ Radiographically, skeletal
Hemophilic
Volume 69 Number 5
changesconsist of areas of bone destruction and new bone formation. In some instances, the picture resembles that of malignant skeletal tumors, especially osteosarcoma-hence the name, pseudotumar
SUMMARY
The pathogenesis and radiographic features of hemophilic pseudotumor are discussed. A case involving the mandible and a cutomized method of postoperative wound management are presented.
of mandible
553
REFERENCES i 2
11-14
The femur is the most common site of pseudotumor occurrence, accounting for about one third of all reported cases. This is followed, in order, by the pelvis, tibia, bones of feet, and bones in the hand or wrist.9 Treatment of hemophilic pseudotumors has consisted of surgery, supportive therapy, and radiation.2 Before the availability of factor VIII transfusions, surgical intervention was usually fatal. Supportive care consists of immobilization and administration of factor replacement. This management seems to be successful in lesions of recent onset. Radiation therapy is reportedly useful in cases in which an inhibitor to factor VIII has developed and that are, thus, difficult to control hemostatically, even in the presence of replacement therapy. Inhibitor to factor VIII has been reported to develop in 15% of patients with factor VIII deficiency.6 In this case,surgical removal of the lesion, coupled with obliteration of the space with an obturator and internal irrigation, provided an environment that allowed hemostatic initiation of the reparative process. Early use of such a device may assist the surgeon in reducing the morbidity of the postoperative course.
pseudotumor
3 4
5 6 I 8. 9. 10.
Il. 12. 13. 14.
Abel1 JM, Bailey RW. Hemophilic pseudotumor: two cases occurring in siblings. Arch Surg 1960;81:569-81. Jensen PS, Putnam CE. Hemophilic pseudotumor: diagnosis, treatment, and complications. Am J Dis Child 1975; 129:717-19. Lazarovits P, Griem ML. Radiotherapy of hemophilic pseudotumor. Radiology 1968;91: 1026-27. Brook AH, Bedi R, Chan Lui WY, Yuen PMP. Hemophilic pseudotumors of the mandible: report of a case in a one-yearold child. Br J Oral Maxillofac Surg 1985;23:47-52. Starker L. Knochenusur durch ein hamophiles, subperiostaies hamatom, mitt. Grenzgeb Med Chir 1918;31:381-415. Correra A, Buckley J, Roser S, Schreiber A, Syrop S. Radiotherapy of a pseudotumor in a hemophiliac with Factor VIII inhibitor. Am J Pediatr Hematol Oncol 1984;6:325-27. Mulkev TF. Hemoohilic oseudotumor of the mandible. J Oral Surg lb77;35:561-68. ’ Treble NJ, Henderson NJ, Dassani H. Amputation for pseudotumor in acquired hemophilia. Br Med J [Clin Res] 1984;289: 1349-50. Gilbert MS. Characterizing the hemophilic pseudotumor. Ann NY Acad Sci 1975;240:3<1-15. Marauez JL. Vinaaeras E. Dorantes S. Nussbaumer C. Flares A, Perez Rolon G‘: Hemophilic pseudotumor of the inferior maxilla. ORAL SURC ORAL MED ORAL PATHOL 1982;53:34750. Stoneman DW, Beierl CD. Pseudotumor of hemophilia in mandible. ORAL SURC ORAL MED ORAL PATHOL 1975;40:8 1 I15. Ahlberg AKM. On the natural history of hemophilic pseudotumor. J Bone Joint Surg 1975;57-A:1133-5. Martinez-Lage JL, Sanchez H, Garcia JS, Lorenzo F. A pseudotumor of the mandible in a haemophiliac patient. J Maxillofac Surg 1983;11:171-3. Brant EE, Jordan HH. Radiologic aspects of hemophilic pseudotumors in bone. Am J Roentgen01 1972;115:52539.
Reprint requests to: Dr. Grady W. Bryan Department of Oral and Maxillofacial Surgery University of Texas Health Science Center at San Antonio San Antonio, Texas 78284