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Hemophilic pseudotumor of the inferior maxilla
Hemophilic pseudotumor of the inferior maxilla Report of a case J. L. Marquez, M.D.,* E. Vinageras, M.D.,** S. Dorantes, M.D.,* C. Nussbaumer, D.D.S.,*** A. Flores, D.D.S.,** and G. Perez Roion, M.D.,**** Mexico, D. F., Mexico HOSPITAL INFANTIL DE MfiXICO
H
emophilic pseudotumor is a risky complication in the hemophilic patient. Its frequency has been estimated at 1 or 2 percent of all cases of severe hemophilia,* but it has also been reported in patients with 2 to 10 percent of factor VIII or IX.? In 1812 Koning described the first hemophilic pseudotumor, and in 1918 Starker3 studied its
pathogenesis. The lesion is characterized by progressive subperiosteal bleeding that eventually affects the neighboring tissues with muscular and even extra-articular osseous destruction. The time required for its formation varies from weeks to several years. In most cases there is a previous history of trauma. CASE REPORT
*Department of Hematology. **Head, Department of Plastic and Reconstructive Surgery. ***Department of Stomatology. ****Department of Radiology.
A 13-year-old boy complained of a progressive’swelling in the jaw of 2 years’ duration, which had been increasing during the last 2 months (Fig. 1). At the age of 7 he had required a blood transfusion because of bleeding gingi-
II III 111-l
ICI
Ill-2
PROPOSI TUS III-3
T+
IV
Fig. 1.
Patient with swelling over left side of face.
(March, 1974.) 0030-4220/82/040347
+ 04$00.40/O @ I982 The C. V. Mosby Co.
A w-1
i IY-2
Fig. 2. Family pedigree. Black squares represent affected members. 347
348
Marquez
et al.
Fig. 3. Orthopantograph several teeth.
Oral Yurg. April. 1982
showing cystic lesion, bone destruction
Fig. 4. Resected maxilla showing extensive struction as seen from its lingual aspect.
bone de-
Fig. 5. Resected maxilla seen from its buccal aspect showing involvement of the ascending branch.
involving
maxilla.
and absence of
Fig. 6. Boyne’s prosthesis with silicone condyle. Folded edges show the portion used.
vae. Bleeding symptoms had been present for the last 12 years. The patient was one of four siblings. A maternal uncle and two sons of the patient’s sister showed abnormal bleeding from minor cuts and easy bruising. The family pedigree is shown in Fig. 2. Examination of the patient revealed facial asymmetry secondary to a 10 by 8 by 3 cm. hard mass on the left side of the inferior maxilla. The mass was slightly painful, with depressed and pressure-resistant areas. It was not movable and was firmly attached to deep structures. A diagnosis of hemophilia was considered. The activated partial thromboplastin test4 disclosed a clotting time of 77 seconds, as compared to 47 seconds for normal plasma. The thromboplastin-generation test was abnormal.” A factor VIII assay,” using a method similar to that described in this laboratory for factor IX, showed a concentration of 2 percent (normal is between 65 and 136 percent).
Volume Number
53 4
Fig. 7. Six months postoperatively the gingival configuration was adequate to receive a dental prosthesis.
X-ray examination revealed a multiloculated tumor with destruction of the left angle of the inferior maxilla. The cortical plate was missing, and spicules were projecting perpendicular to the destroyed area. There were no teeth in this area (Fig. 3). A hemophilic pseudotumor was diagnosed. In March, 1974, the boy was admitted to the Hospital Infantil de Mexico. Conservative treatment was initiated with cryoprecipitate, epsilon aminocaproic acid, immobilization of the mandible, and a liquid diet. Clinical and radiologic improvement was achieved, and the patient was discharged after 120 days. One month after discharge the patient suffered a trauma in the same area. Because of the failure of conservative treatment and the possibility of infection, a hemimaxillary resection was undertaken (Fig. 4). Even though an autologous bone transplant was the best choice for the reconstruction, it would have entailed a second operation and a higher consumption of factor VIII. Therefore, a titanium implant conceived by Boyne’- * was used. A silicone rubber condyle was carved to the measurements of the opposite condyle as obtained by radiologic cephalometry. The titanium implant was molded in order to sustain the gingival mucosa and form a structure able to receive a dental prosthesis. On April 24, 1975,after infusion of 60 units of cryoprecipitate,” a normal kaolin partial thromboplastin time was obtained. With the patient under general anesthesia, a submaxillary incision was made. The left half of the inferior maxilla was dissected, sectioned at the median line, and removed. Figs. 4 and 5 show the destruction of the condyle and the ascending branch where the pseudotumor was located. The temporal glenoid fossa was spared. The titanium implant with the silicone condyle (Fig. 6) was settled and fastened into place with wire and a screw of the same material. The oral floor muscles were then fixed to the prosthesis. Bleeding was minimal during the operation, with the patient requiring only 300 ml. of fresh blood. A drain was left for 24 hours, and 20 units of
Hemophilic pseudotumor of inferior maxilla
349
Fig. 8. Dental prosthesis in place 6 months after surgical procedure.
Fig. 9. Roentgenogram showing Boyne’s prosthesis in place.
cryoprecipitate were infused every 8 hours for 10 days. The postoperative course was uneventful. The fibrinogen level oscillated from 350 to 600 mg. per 100 ml. No increase of fibrinolytic activity and no factor VIII antibodies were detected. Serum protein levels remained within normal limits. Six months later the mobility of the temporomandibular joint was normal and, as shown in Figs. 7 and 8, the gingival configuration was adequate to receive a dental prosthesis. The patient has been seen every 6 months for 5 years, and no pathologic changes have been detected in the mandible. X-ray films taken 1 year after the surgical procedure show anatomic continuity and good tolerance of the implant (Fig. 9). Fig. 10 shows the patient’s facial appearance 18 months postoperatively. DISCUSSION
There are several reports in the literature of surgical attempts to replace segments of the mandible. Various techniques and materials have been used to repair it immediately if the condition of the
350
Marquez
et al.
Oral Surg. April. I982
was settled and fastened into place, and a esthetic and functional result was obtained. patient has been followed every 6 months years, and an excellent tolerance has observed.
good The for 5 been
REFERENCES
5. 6.
7.
Fig. 10. Results 1% years postoperatively.
8.
superficial layers was satisfactory. Most of the inorganic materials used have been abandoned because of complications or have been used only as temporary prostheses. The best results have been obtained with the autologous bone transplant. Ollier and Bardenheuer and Sykoff used rib and tibia transplants; latter Ivy and other British surgeons used ilium because of its characteristics. Time has shown the advantages of this method.“‘-‘7 Because of the special problems that this case presented with respect to obtaining a bone graft, a titanium and silicone implant was used with good tolerance to date (5 years’ follow-up). Seventy-five cases of hemophilic pseudotumor have been reported, but only two were located in the mandible.‘“-“’ Arias and Goiiis’ studied a third patient with the same problem. Their patient was a boy with less than 1 percent of factor IX. When he was 8 years old he developed a hemophilic pseudotumor in the distal portion of the thigh, which required amputation. Three years later he presented with a pseudotumor at the left horizontal part of the mandible; a curettage was performed and a bone “bank graft implant” was placed. A good esthetic and functional result was obtained.”
9.
10.
I I.
12.
13.
14.
15.
16. 17
18. 19
Ahlberg, Ake: On the Natural History of Hemophilic Pseudotumor, J. Bone Joint Surg. 57.A:1133-1135, 1975. Abell, J.M., Jr. and Bayley, R.W.: Haemophilic Pseudotumor, Arch. Surg. 81:569-581, 1960. Starker, L.: Knochenusur dunch ein hamophiies subperiostales Hamatom. Mitt. Grenzgeb. Med. Chir. 31:318, 1918. Proctor. R.R., and Rapaport. S.1.: The Partial Thromboplastin Time With Kaolin. Am. J. Clin Pathol. 36:212-219. 1965. Biggs, R., and Douglas, AS.: Thromboplastin Generation Test, J. Clin. Pathol. 6:23. 1953. Dorantes, S.. Barron, I.. Areas. N.. Vazquez, J., and Soto. R.: Pathogenesis of Purpura in the Child With Severe Malnutrition, J. Pediatr. 65:438, 1964. Boyne, P.J.: Restoration of Osseus Defects in maxillo-facial casualties, J. Am. Dent, Assoc. 78:767. 1969. Boyne, P.J.: Implants and Transplants; Review of Recent Research in the Area of Oral Surgery. J. Am. Dent. Assoc. 87:1074. 1973. Pool, J.G.. and Shannon, A.E.: Production of High-Potency Concentrates of Antihemophilic Globulin in a Closed-Bag System Assay In Vitro and In Vivo. N. Engl. J. Med. 273:1443. 1965. Converse. J.M.: Restoration of Facial Contour by Bone Graft Introduced Through Oral Cavity. Plast. Reconstr. Surg. 6~295. 1950. Fernandez De Valderrama, J.A.. and Matthews, J.M.: The Haemophilic Pseudotumor or Haemophilic Subperiosteal Haematoma. J. Bone Joint Surg. 47B:256-265, 1965. Ivy. R.H.: Iliac Bone Graft to Bridge Mandibular Defect; 49-Year Clinical and Radiological Follow-up, Plast. Reconstr. Surg. 50:483-486. 1972. Leake. K.L.: Mandibular Reconstruction With a New Type of Alloplastic Tray: A Preliminary Report, J. Oral Surg. 32~23. 1974. Manchester. W.M.: Immediate Reconstruction of the Mandible and Temporal Mandibular Joint, Br. J. Plast. Surg. l&291. 1965. Bnegeser. H.L.: Late Reconstruction of Large Maxillary Defects After Tumor Resection, J. Maxilla-Fat. Surg. 1:19, 1973. Post, Melvin. and Telfer. Margaret C.: Surgery in Hemophilic Patients. J. Bone Joint Surg. 57A:I 136, 1975. Rosenthal. Robert L.. Graham. Jacob J.. and Selirio. Eva: Incision of Femur in Hemophilia, J Bone Joint Surg. 55A:827. 1973. Jensen. Pamela S.. and Putman, Charles E.: Hemophilic Pseudotumor. Am J. Dis. Child. 129717-719, 1975. Stoneman. Douglas W.. and C. Beierl. C. Douglas: Pseudotumor of Hemophilia in the Mandible, ORAL SURG. 4031 I815. 1975.
SUMMARY
20. Mulkey Thomas F.: Hemophilic Pseudotumor of the Mandible, J. Oral Surg. 35:561, 1977. 21 Arias. J.. and Goni, A.: Personal communication.
A hemophilic pseudotumor of the inferior maxilla is reported in a 13-year-old boy. Because of the failure of conservative treatment (cryoprecipitate, epsilon aminocaproic acid, and immobilization of the mandible), a hemimaxillary resection was performed. A titanium implant with a silicone condyle
Reprint requests to: Dr. Enrique Vinageras Guameros Plastic and Reconstructive Surgery Department Hospital Infantil de Mexico Federico Gomez Dr. Marquez No. 162 Mexico 7. D.F . Mexico
H
emophilic pseudotumor is a risky complication in the hemophilic patient. Its frequency has been estimated at 1 or 2 percent of all cases of severe hemophilia,* but it has also been reported in patients with 2 to 10 percent of factor VIII or IX.? In 1812 Koning described the first hemophilic pseudotumor, and in 1918 Starker3 studied its
pathogenesis. The lesion is characterized by progressive subperiosteal bleeding that eventually affects the neighboring tissues with muscular and even extra-articular osseous destruction. The time required for its formation varies from weeks to several years. In most cases there is a previous history of trauma. CASE REPORT
*Department of Hematology. **Head, Department of Plastic and Reconstructive Surgery. ***Department of Stomatology. ****Department of Radiology.
A 13-year-old boy complained of a progressive’swelling in the jaw of 2 years’ duration, which had been increasing during the last 2 months (Fig. 1). At the age of 7 he had required a blood transfusion because of bleeding gingi-
II III 111-l
ICI
Ill-2
PROPOSI TUS III-3
T+
IV
Fig. 1.
Patient with swelling over left side of face.
(March, 1974.) 0030-4220/82/040347
+ 04$00.40/O @ I982 The C. V. Mosby Co.
A w-1
i IY-2
Fig. 2. Family pedigree. Black squares represent affected members. 347
348
Marquez
et al.
Fig. 3. Orthopantograph several teeth.
Oral Yurg. April. 1982
showing cystic lesion, bone destruction
Fig. 4. Resected maxilla showing extensive struction as seen from its lingual aspect.
bone de-
Fig. 5. Resected maxilla seen from its buccal aspect showing involvement of the ascending branch.
involving
maxilla.
and absence of
Fig. 6. Boyne’s prosthesis with silicone condyle. Folded edges show the portion used.
vae. Bleeding symptoms had been present for the last 12 years. The patient was one of four siblings. A maternal uncle and two sons of the patient’s sister showed abnormal bleeding from minor cuts and easy bruising. The family pedigree is shown in Fig. 2. Examination of the patient revealed facial asymmetry secondary to a 10 by 8 by 3 cm. hard mass on the left side of the inferior maxilla. The mass was slightly painful, with depressed and pressure-resistant areas. It was not movable and was firmly attached to deep structures. A diagnosis of hemophilia was considered. The activated partial thromboplastin test4 disclosed a clotting time of 77 seconds, as compared to 47 seconds for normal plasma. The thromboplastin-generation test was abnormal.” A factor VIII assay,” using a method similar to that described in this laboratory for factor IX, showed a concentration of 2 percent (normal is between 65 and 136 percent).
Volume Number
53 4
Fig. 7. Six months postoperatively the gingival configuration was adequate to receive a dental prosthesis.
X-ray examination revealed a multiloculated tumor with destruction of the left angle of the inferior maxilla. The cortical plate was missing, and spicules were projecting perpendicular to the destroyed area. There were no teeth in this area (Fig. 3). A hemophilic pseudotumor was diagnosed. In March, 1974, the boy was admitted to the Hospital Infantil de Mexico. Conservative treatment was initiated with cryoprecipitate, epsilon aminocaproic acid, immobilization of the mandible, and a liquid diet. Clinical and radiologic improvement was achieved, and the patient was discharged after 120 days. One month after discharge the patient suffered a trauma in the same area. Because of the failure of conservative treatment and the possibility of infection, a hemimaxillary resection was undertaken (Fig. 4). Even though an autologous bone transplant was the best choice for the reconstruction, it would have entailed a second operation and a higher consumption of factor VIII. Therefore, a titanium implant conceived by Boyne’- * was used. A silicone rubber condyle was carved to the measurements of the opposite condyle as obtained by radiologic cephalometry. The titanium implant was molded in order to sustain the gingival mucosa and form a structure able to receive a dental prosthesis. On April 24, 1975,after infusion of 60 units of cryoprecipitate,” a normal kaolin partial thromboplastin time was obtained. With the patient under general anesthesia, a submaxillary incision was made. The left half of the inferior maxilla was dissected, sectioned at the median line, and removed. Figs. 4 and 5 show the destruction of the condyle and the ascending branch where the pseudotumor was located. The temporal glenoid fossa was spared. The titanium implant with the silicone condyle (Fig. 6) was settled and fastened into place with wire and a screw of the same material. The oral floor muscles were then fixed to the prosthesis. Bleeding was minimal during the operation, with the patient requiring only 300 ml. of fresh blood. A drain was left for 24 hours, and 20 units of
Hemophilic pseudotumor of inferior maxilla
349
Fig. 8. Dental prosthesis in place 6 months after surgical procedure.
Fig. 9. Roentgenogram showing Boyne’s prosthesis in place.
cryoprecipitate were infused every 8 hours for 10 days. The postoperative course was uneventful. The fibrinogen level oscillated from 350 to 600 mg. per 100 ml. No increase of fibrinolytic activity and no factor VIII antibodies were detected. Serum protein levels remained within normal limits. Six months later the mobility of the temporomandibular joint was normal and, as shown in Figs. 7 and 8, the gingival configuration was adequate to receive a dental prosthesis. The patient has been seen every 6 months for 5 years, and no pathologic changes have been detected in the mandible. X-ray films taken 1 year after the surgical procedure show anatomic continuity and good tolerance of the implant (Fig. 9). Fig. 10 shows the patient’s facial appearance 18 months postoperatively. DISCUSSION
There are several reports in the literature of surgical attempts to replace segments of the mandible. Various techniques and materials have been used to repair it immediately if the condition of the
350
Marquez
et al.
Oral Surg. April. I982
was settled and fastened into place, and a esthetic and functional result was obtained. patient has been followed every 6 months years, and an excellent tolerance has observed.
good The for 5 been
REFERENCES
5. 6.
7.
Fig. 10. Results 1% years postoperatively.
8.
superficial layers was satisfactory. Most of the inorganic materials used have been abandoned because of complications or have been used only as temporary prostheses. The best results have been obtained with the autologous bone transplant. Ollier and Bardenheuer and Sykoff used rib and tibia transplants; latter Ivy and other British surgeons used ilium because of its characteristics. Time has shown the advantages of this method.“‘-‘7 Because of the special problems that this case presented with respect to obtaining a bone graft, a titanium and silicone implant was used with good tolerance to date (5 years’ follow-up). Seventy-five cases of hemophilic pseudotumor have been reported, but only two were located in the mandible.‘“-“’ Arias and Goiiis’ studied a third patient with the same problem. Their patient was a boy with less than 1 percent of factor IX. When he was 8 years old he developed a hemophilic pseudotumor in the distal portion of the thigh, which required amputation. Three years later he presented with a pseudotumor at the left horizontal part of the mandible; a curettage was performed and a bone “bank graft implant” was placed. A good esthetic and functional result was obtained.”
9.
10.
I I.
12.
13.
14.
15.
16. 17
18. 19
Ahlberg, Ake: On the Natural History of Hemophilic Pseudotumor, J. Bone Joint Surg. 57.A:1133-1135, 1975. Abell, J.M., Jr. and Bayley, R.W.: Haemophilic Pseudotumor, Arch. Surg. 81:569-581, 1960. Starker, L.: Knochenusur dunch ein hamophiies subperiostales Hamatom. Mitt. Grenzgeb. Med. Chir. 31:318, 1918. Proctor. R.R., and Rapaport. S.1.: The Partial Thromboplastin Time With Kaolin. Am. J. Clin Pathol. 36:212-219. 1965. Biggs, R., and Douglas, AS.: Thromboplastin Generation Test, J. Clin. Pathol. 6:23. 1953. Dorantes, S.. Barron, I.. Areas. N.. Vazquez, J., and Soto. R.: Pathogenesis of Purpura in the Child With Severe Malnutrition, J. Pediatr. 65:438, 1964. Boyne, P.J.: Restoration of Osseus Defects in maxillo-facial casualties, J. Am. Dent, Assoc. 78:767. 1969. Boyne, P.J.: Implants and Transplants; Review of Recent Research in the Area of Oral Surgery. J. Am. Dent. Assoc. 87:1074. 1973. Pool, J.G.. and Shannon, A.E.: Production of High-Potency Concentrates of Antihemophilic Globulin in a Closed-Bag System Assay In Vitro and In Vivo. N. Engl. J. Med. 273:1443. 1965. Converse. J.M.: Restoration of Facial Contour by Bone Graft Introduced Through Oral Cavity. Plast. Reconstr. Surg. 6~295. 1950. Fernandez De Valderrama, J.A.. and Matthews, J.M.: The Haemophilic Pseudotumor or Haemophilic Subperiosteal Haematoma. J. Bone Joint Surg. 47B:256-265, 1965. Ivy. R.H.: Iliac Bone Graft to Bridge Mandibular Defect; 49-Year Clinical and Radiological Follow-up, Plast. Reconstr. Surg. 50:483-486. 1972. Leake. K.L.: Mandibular Reconstruction With a New Type of Alloplastic Tray: A Preliminary Report, J. Oral Surg. 32~23. 1974. Manchester. W.M.: Immediate Reconstruction of the Mandible and Temporal Mandibular Joint, Br. J. Plast. Surg. l&291. 1965. Bnegeser. H.L.: Late Reconstruction of Large Maxillary Defects After Tumor Resection, J. Maxilla-Fat. Surg. 1:19, 1973. Post, Melvin. and Telfer. Margaret C.: Surgery in Hemophilic Patients. J. Bone Joint Surg. 57A:I 136, 1975. Rosenthal. Robert L.. Graham. Jacob J.. and Selirio. Eva: Incision of Femur in Hemophilia, J Bone Joint Surg. 55A:827. 1973. Jensen. Pamela S.. and Putman, Charles E.: Hemophilic Pseudotumor. Am J. Dis. Child. 129717-719, 1975. Stoneman. Douglas W.. and C. Beierl. C. Douglas: Pseudotumor of Hemophilia in the Mandible, ORAL SURG. 4031 I815. 1975.
SUMMARY
20. Mulkey Thomas F.: Hemophilic Pseudotumor of the Mandible, J. Oral Surg. 35:561, 1977. 21 Arias. J.. and Goni, A.: Personal communication.
A hemophilic pseudotumor of the inferior maxilla is reported in a 13-year-old boy. Because of the failure of conservative treatment (cryoprecipitate, epsilon aminocaproic acid, and immobilization of the mandible), a hemimaxillary resection was performed. A titanium implant with a silicone condyle
Reprint requests to: Dr. Enrique Vinageras Guameros Plastic and Reconstructive Surgery Department Hospital Infantil de Mexico Federico Gomez Dr. Marquez No. 162 Mexico 7. D.F . Mexico