Osteomyelitis of the mandible as a result of sickle cell disease Report and literature
review
John V, Shroyer III, DDS,” Daniel Lew, DDS,b Fleurette Abreo, MD,c and Gerald P. Unhold, DMD,d Shreveport, La. LOUISIANA
STATE UNIVERSITY
MEDICAL
CENTER
Only five cases of osteomyelitis of the mandible as a result of sickle disease have been published. We report another case, which uniquely affected not only the osseous portion of the mandible, including the condyle, but also the adjacent musculature. Radical resection of the involved hard and sofl tissue was necessary to cure the patient. Two possible etiologies are discussed. The first states that the sickle cell crisis caused a tissue anoxia in which this tissue became secondarily infected; the second states that the preexisting infection triggered or augmented the sickling phenomena. (ORAL SURC ORAL MED ORAL PATHOL 1991;72:25-8)
S
ickle cell anemia is one of the familial hemoglobinopathies inherited through an autosomal recessive mutant gene. It occurs primarily in the black population, with an incidence of approximately 1 in 400 in the homozygous state (sickle cell anemia) and 1 in 10 in the heterozygous state (sickle cell trait). Osteomyelitis resulting from sickle cell anemia has been recognized since 1925.1The long bonesare most frequently involved, and multiple bone involvement is relatively common.2-4In view of this, the infrequency of facial bone occurrence is striking. Only five cases have been reported in the literature.5-7 The basic abnormality in hereditary sickle cell disease afflicts the globin portion of the hemoglobin molecule. Sickle cell hemoglobin (hemoglobin S) differs from normal adult hemoglobin in that valine is substituted for glutamic acid in the sixth amino acid position in each of the two beta polypeptide chains. This change alters the net electrical charge of the molecules and allows electrophoretic separation. In the deoxygenated state, hemoglobin S is less soluble than hemoglobin A. The insoluble hemoglobin
Bln private practice, Arlington, Tex. bChief, Oral and Maxillofacial Surgery, Louisiana State University Medical Center. CAssistant Professor, Pathology, Louisiana State University Medical Center. din private practice, Gadsden, Ala. 7/12/19502
molecules in the erythrocyte form in needlelike polymers that distort the cell, thus producing the characteristic sickle cell. The deformed cells embrace each other and causethe blood to be more viscid. The blood therefore flows sluggishly through the capillaries, and a chronic stasis of blood in the capillaries results. Wherever stasis is significant, prolonged ischemic necrosis results. The deformed sickle cells have a shortened survival time in the circulation, resulting in a hemolytic anemia. Besides deoxygenation, certain clinical conditions enhance the sickling phenomena. The patient with an acute infection is more liable to undergo a sickling crisis. Dehydration can result in hemoconcentration and increased blood viscosity, ultimately resulting in stasis. With the rise of mean corpuscular hemoglobin concentration, the sickling rate increases. Finally, acidosis facilitates fever as well as sickling. Bones are commonly involved in the infarctive crises.Osteomyelitis may result from marrow infarction. Salmonella is a common causative organism in the long bones. Bennett and Hook* pointed out that, in patients afflicted with sickle cell anemia, Salmonellainduced bacteremias were predominant. Diggs5 demonstrated the impressive statistical correlation between sickle cell diseaseand Salmonella osteomyelitis. Staphylococcus9 and Bacteroides,‘O among other organisms, have also been implicated in the process. Ischemia, subsequent necrosis of bones, and perhaps associated connective tissues form a hospitable 25
26
Shroyer et al.
ORAL SURG ORAL
MED ORAL PATHOL July 1991
Fig. 1. Panoramic x-ray film demonstrating absence of visible bony involvement of left mandible.
Fig. 3. Photomicrograph of section of blood vessel. Lumen contains sickled red blood cells and fibrin. (Hematoxylin-eosin stain; original magnification, X40.)
Fig. 2. Photomicrograph of section of bone shows focal necrosis of spicules. Osteolytic lacunae are empty, and spicule has granular appearance.(Hematoxylin-eosin stain; original magnification, X 10.)
environment for bacterial growth, Infection commences in the medullary portion of the bone and
rarely involves the joints. l t Pain and fever are common symptoms heralding the onset of osteomyelitis. Osteomyelitis as a result of sickle cell diseaserarely affects the facial bones. Of these, the mandible is the most commonly affected. Five casesof sickle cell osteomyelitis involving the mandible have been reported in the literature. Walker6 in 1971 was the first to report such a case.The following case report describes massive necrosis and destruction of the ramus and body of the mandible and of the adjacent soft tissue. CASE REPORT
A 22-year-old black man with homozygous sickle cell anemia was seen at the Oral and Maxillofacial Surgery Clinic at Louisiana State University Medical CenterShreveport, on March 11, I987, with pain, trismus, and minimal swelling in the left posterior portion of the mandible. He stated that the pain and swelling had been present for approximately 1 week. He related that he had had a
Fig. 4. Photomicrograph of blood vessel.Lumen contains organizing thrombus. (Hematoxylin-eosin stain; original magnification, X40.)
sickle cell crisis 7 days before his admission. The laboratory values on admission showed a white blood cell count of 14,000/mm3 and a hematocrit of 38.5%. Results of hemoglobin electrophoresis showed a hemoglobin S value 52% of total and hemoglobin C value 48% of total. Rectal temperature on admission was 102.4” F. An intraoral examination revealed pericoronitis associatedwith the partially erupted mandibular left third molar. There was purulent drainage from this area. Moderate left facial edema overlying the lateral ramus and posterior body was evident. The rest of the teeth in the quadrant were in good repair, with no gross decay or periodontal disease noted (Fig. 1). The initial treatment consisted of intravenous fluid therapy together with 2 million U aqueous penicillin G, given intravenously every 4 hours. Analgesics were also given for pain relief. On hospital day 2, the infection was incised and drained through an extraoral approach and the left upper molar and
Osteomyelitis
Volume 72 Number I Table
of mandible in sickle cell disease
27
I. Characteristics of reported cases
Author
Girasole and Lyon’ Case 1 Black/M Case 2
Black/F
Case 3
Black/M
Daramolag
Location
Age (yr)
Mandibular R 3rd molar (pericoronitis) Mandibular R 1st molar Edentulous R posterior mandible Entire mandibular body (L and R) Mandibular R 1st molar and 3rd molar (pericoronitis) Mandibular L 3rd molar (pericoronitis)
22
Race/sex
Black/F
Walker and Black/M Schenck6 Present case Black/M
Crisis at presentation
Organisms cultured
Staphylococcus
Treatment
aureus
34
-
Streptococcus viridans
33
+
Pseudomonas
21
+
Mixed organisms
21
+
Normal oral flora
22
Eikenella corrodens. Bacteroides melangenicus, Peptostreptococcus, mixed Streptococcus and Staphylococcus
Sequestrectomy and curettage Debridementcorticotomy Sequestrectomy and curettage Sequestrectomy and curettage Sequestrectomy Resection of mandible and adjacent muscles
Sickle cell me
Homo Homo Homo Homo Homo Homo
Homo,Homozygous;L. left: R, right
mandibular left third molar were extracted. Drainage of a large amount of brownish black, foul-smelling purulence was obtained from the mandibular extraction site and the extraoral incision, and cultures were submitted for aerobic and anaerobic bacterial and fungal evaluation. A Penrose drain was inserted in the dependent portion of the neck. Metronidazole, 500 mg every 6 hours, was added to the antibiotic regimen. Culture results showed growth of Eikenella corroders, Bacteroides melaninogenicus, Peptostreptococcus, and mixed Streptococcus and Staphylococcus flora organisms. On the basis of the culture and sensitivity report, nafcillin, 1 gm every 4 hours, was begun. The patient’s temperature, left facial edema, and trismus decreased.The Penrosedrain was removed 10days later. Even though the amount of drainage decreasedsubstantially, a moderate amount continued to drain from the site. We therefore decided to explore the area. The wound was explored through a Risdon incision. The entire medical aspect of the mandibular horizontal and ascending rami were found to be devitalized. Involvement of the lateral cortex and the adjacent musculature and periosteum appearedto be minimal. Resection of the lingual cortex and debridement of the cancellous portion of the mandible from the lingula to the mental foramen was carried out. A portion of the lateral pterygoid, mylohyoid, and temporal muscleswere found to be devitalized and were resected.These together with the resectedbone were submitted for biopsy and culture studies (Figs. 2 to 4). Daily dressing changes were performed, and the wound drainage decreased. The patient was discharged 3 weeks after surgery. His wound was managed with daily irrigations and packing changes on an outpatient basis. The wound, however, did not heal, and drainage continued. Six weeks after
discharge the patient was readmitted to the hospital and a technetium Tc 99m scan was performed. On the basisof the physical findings and the positive scan, a decision to reexplore the infection site was made. The patient again underwent an exploration of the left mandibular area through the previous Risdon incision. At this time the lateral cortex of the left mandible and the adjacent lateral and medial soft tissue were found to be infected. A left hemimandibulectomy was performed together with resection of all infected muscle tissue, which involved a portion of the masseter muscle and the deep portions of the lateral pterygoid, temporal, and mylohyoid muscles.The patient was given cephapirin (Cefadyl), 1 gm every 6 hours. Daily dressing changes of the extraoral wounds were undertaken, and 3 weeks after surgery the patient underwent a delayed primary closure of the wound. Healing progressedwithout complications (Table I). DISCUSSION
The present case has certain elements in common with previously reported cases5 to 7 (Table I). The initial symptoms of pain and swelling were present as they were in the other five cases.Our patient’s age, like those of the patients cited in the other studies, was beyond the preteen years, the period during which osteomyelitis resulting from sickle cell disease most commonly occurs. We hypothesize that two mechanisms may be involved for the development of osteomyelitis in this disease.A crisis can cause a sterile area in bone that can then be invaded by blood-borne or local bacteria. The secondpossibility is that a systemic infection can
ORAL SURC ORAL MED ORAL PATHOL July 1991
tic. The extent of osteomyelitis and the subsequent resection in this case were greater than in earlier reported cases. In addition, the involvement of the condyle was not seenin previous reports. The patient refused reconstruction or the removal of the Ivy loops. A vestigial mandible subsequently developed (Fig. 5). SUMMARY
Fig. 5. Panoramic x-ray film demonstrating extent of resection of left mandible and regeneration of mandibular bone.
trigger a crisis, which in turn leads to an infarct. Infarcts of the long bones after a crisis do not reveal themselves at the time of onset, but lysis usually appears a couple of weeks later. The crisis is initially manifested by painful episodes.One of Girasole and Lyon’s patients’ initially had pain as his only symptom. Two weekslater swelling accompanied the painful area. Our patient demonstrated a similar history. On the other hand, the patient reported by Daramola9 and one of Girasole and Lyon’s patients’ were in crisis and infected on presentation. This could indicate that the infection was the initiating phenomenon that helped trigger the sickling crisis. Diggs5 has stated that “no one has ever demonstrated infarcts and necrosis in muscles and tendons or connective tissue.” It seemsreasonable that, as he further stated, stasis and ischemia can effect these structures as well as any others. We did not obtain a biopsy specimenof the anterior deep temporal, mylohyoid, masseteric, or pterygoid arteries; however, the extensive necrosis of the temporal, mylohyoid, masseter, and interanal pterygoid muscles undoubtedly could have been due to a direct invasion of oral organisms infecting structures whose arterial supply was compromised. That would certainly account for the massivenecrosisseenduring the two debridement episodes. The inferior alveolar artery was probably thrombosed. An attempt was made to maintain the continuity of the mandible by resecting only the apparently involved lingual cortex from the linguale to the mental foramen. The resection included the inferior alveolar artery and the associated marrow. Unfortunately, it was not clear at the time that the lateral cortex had also becomeinfected and that the bone was unable to ward off the infection, because its two sourcesof blood supply, namely the inferior alveolar artery and the adjacent musculature, were not absent. Clearly, resection of the involved bone was therapeu-
We report a case of osteomyelitis of the mandible that resulted from a sickle cell crisis in a black man with homozygous sickle cell disease. This rare case was more extensive than previously reported casesin its destruction of the mandible and involved the condyle. A biopsy of the adjacent muscles demonstrated the presence of sickle cells in a vessel lumen as well as the presenceof an infarct. The diagnosis of homozygous sickle cell diseaseshould alert one to the possibility of periodontal or periapical infections contributing to the development of osteomyelitis of the jaws. The extent of the lesion is rarely discernible during the acute phase. Significant necrosis of the adjacent musculature and soft tissue, as well as thrombosis of the inferior alveolar artery, demanded the extirpation of the ascending and horizontal ramus of the mandible and of a significant amount of adjacent soft tissue in the region. REFERENCES 1. Smith WS. Sickle cell anemia and Salmonella osteomyelitis. Ohio Med J 1953;49:692-5. 2. Hook EW. Salmonellosis: certain factors influencing the interaction of Salmonellu and the human host. Bull N Y Acad Med 1961;37:499-512. 3. Silver HK, Simon JL, Clement OH. Snlmonella osteomyelitis and abnormal hemoglobin disease.Pediatrics 1957;20:439-47. 4. Hughes JG, Carroll DS. Salmonellu osteomyelitis complicating sickle cell disease. Pediatrics 1957;19:184-91. 5. Diggs LW. Bone and joint lesions in sickle cell disease. Clin Orthop 1967;52:119-43. 6. Walker RD, Schenck KL. Infarct of the mandible in sickle cell anemia: report of case. J Am Dent Assoc 1973;87:661-4. 1. Girasole RV, Lyon ED. Sickle ceil osteomyelitis of the mandible: report of three cases.J Oral Surg 1977;35:231-4. 8. Bennett IL Jr, Hook EW. Infectious diseases(some aspectsof salmonellosis). Ann Rev Med 1959;10:1-20. 9. Daramola JO. Massive osteomyelitis of the mandible complicating sickle cell disease: report of case. J Oral Surg 1981; 391144-6. 10. Sares HE. Osteomyelitis complicating sickle cell thalassemia syndrome. Tex Ren Biol Med 1964:22:356-66. 11. Statman N, Spitzel S. Bucteroides, ‘septecemia and osteomyelitis: report of a case complicating sickle cell anemia. Ohio Med J 1962;58:1374-7.
Reprint requests:
Daniel Lew, DDS Louisiana State University Medical Center 1501 Kings Highway Shreveport, LA 7 1130
review
John V, Shroyer III, DDS,” Daniel Lew, DDS,b Fleurette Abreo, MD,c and Gerald P. Unhold, DMD,d Shreveport, La. LOUISIANA
STATE UNIVERSITY
MEDICAL
CENTER
Only five cases of osteomyelitis of the mandible as a result of sickle disease have been published. We report another case, which uniquely affected not only the osseous portion of the mandible, including the condyle, but also the adjacent musculature. Radical resection of the involved hard and sofl tissue was necessary to cure the patient. Two possible etiologies are discussed. The first states that the sickle cell crisis caused a tissue anoxia in which this tissue became secondarily infected; the second states that the preexisting infection triggered or augmented the sickling phenomena. (ORAL SURC ORAL MED ORAL PATHOL 1991;72:25-8)
S
ickle cell anemia is one of the familial hemoglobinopathies inherited through an autosomal recessive mutant gene. It occurs primarily in the black population, with an incidence of approximately 1 in 400 in the homozygous state (sickle cell anemia) and 1 in 10 in the heterozygous state (sickle cell trait). Osteomyelitis resulting from sickle cell anemia has been recognized since 1925.1The long bonesare most frequently involved, and multiple bone involvement is relatively common.2-4In view of this, the infrequency of facial bone occurrence is striking. Only five cases have been reported in the literature.5-7 The basic abnormality in hereditary sickle cell disease afflicts the globin portion of the hemoglobin molecule. Sickle cell hemoglobin (hemoglobin S) differs from normal adult hemoglobin in that valine is substituted for glutamic acid in the sixth amino acid position in each of the two beta polypeptide chains. This change alters the net electrical charge of the molecules and allows electrophoretic separation. In the deoxygenated state, hemoglobin S is less soluble than hemoglobin A. The insoluble hemoglobin
Bln private practice, Arlington, Tex. bChief, Oral and Maxillofacial Surgery, Louisiana State University Medical Center. CAssistant Professor, Pathology, Louisiana State University Medical Center. din private practice, Gadsden, Ala. 7/12/19502
molecules in the erythrocyte form in needlelike polymers that distort the cell, thus producing the characteristic sickle cell. The deformed cells embrace each other and causethe blood to be more viscid. The blood therefore flows sluggishly through the capillaries, and a chronic stasis of blood in the capillaries results. Wherever stasis is significant, prolonged ischemic necrosis results. The deformed sickle cells have a shortened survival time in the circulation, resulting in a hemolytic anemia. Besides deoxygenation, certain clinical conditions enhance the sickling phenomena. The patient with an acute infection is more liable to undergo a sickling crisis. Dehydration can result in hemoconcentration and increased blood viscosity, ultimately resulting in stasis. With the rise of mean corpuscular hemoglobin concentration, the sickling rate increases. Finally, acidosis facilitates fever as well as sickling. Bones are commonly involved in the infarctive crises.Osteomyelitis may result from marrow infarction. Salmonella is a common causative organism in the long bones. Bennett and Hook* pointed out that, in patients afflicted with sickle cell anemia, Salmonellainduced bacteremias were predominant. Diggs5 demonstrated the impressive statistical correlation between sickle cell diseaseand Salmonella osteomyelitis. Staphylococcus9 and Bacteroides,‘O among other organisms, have also been implicated in the process. Ischemia, subsequent necrosis of bones, and perhaps associated connective tissues form a hospitable 25
26
Shroyer et al.
ORAL SURG ORAL
MED ORAL PATHOL July 1991
Fig. 1. Panoramic x-ray film demonstrating absence of visible bony involvement of left mandible.
Fig. 3. Photomicrograph of section of blood vessel. Lumen contains sickled red blood cells and fibrin. (Hematoxylin-eosin stain; original magnification, X40.)
Fig. 2. Photomicrograph of section of bone shows focal necrosis of spicules. Osteolytic lacunae are empty, and spicule has granular appearance.(Hematoxylin-eosin stain; original magnification, X 10.)
environment for bacterial growth, Infection commences in the medullary portion of the bone and
rarely involves the joints. l t Pain and fever are common symptoms heralding the onset of osteomyelitis. Osteomyelitis as a result of sickle cell diseaserarely affects the facial bones. Of these, the mandible is the most commonly affected. Five casesof sickle cell osteomyelitis involving the mandible have been reported in the literature. Walker6 in 1971 was the first to report such a case.The following case report describes massive necrosis and destruction of the ramus and body of the mandible and of the adjacent soft tissue. CASE REPORT
A 22-year-old black man with homozygous sickle cell anemia was seen at the Oral and Maxillofacial Surgery Clinic at Louisiana State University Medical CenterShreveport, on March 11, I987, with pain, trismus, and minimal swelling in the left posterior portion of the mandible. He stated that the pain and swelling had been present for approximately 1 week. He related that he had had a
Fig. 4. Photomicrograph of blood vessel.Lumen contains organizing thrombus. (Hematoxylin-eosin stain; original magnification, X40.)
sickle cell crisis 7 days before his admission. The laboratory values on admission showed a white blood cell count of 14,000/mm3 and a hematocrit of 38.5%. Results of hemoglobin electrophoresis showed a hemoglobin S value 52% of total and hemoglobin C value 48% of total. Rectal temperature on admission was 102.4” F. An intraoral examination revealed pericoronitis associatedwith the partially erupted mandibular left third molar. There was purulent drainage from this area. Moderate left facial edema overlying the lateral ramus and posterior body was evident. The rest of the teeth in the quadrant were in good repair, with no gross decay or periodontal disease noted (Fig. 1). The initial treatment consisted of intravenous fluid therapy together with 2 million U aqueous penicillin G, given intravenously every 4 hours. Analgesics were also given for pain relief. On hospital day 2, the infection was incised and drained through an extraoral approach and the left upper molar and
Osteomyelitis
Volume 72 Number I Table
of mandible in sickle cell disease
27
I. Characteristics of reported cases
Author
Girasole and Lyon’ Case 1 Black/M Case 2
Black/F
Case 3
Black/M
Daramolag
Location
Age (yr)
Mandibular R 3rd molar (pericoronitis) Mandibular R 1st molar Edentulous R posterior mandible Entire mandibular body (L and R) Mandibular R 1st molar and 3rd molar (pericoronitis) Mandibular L 3rd molar (pericoronitis)
22
Race/sex
Black/F
Walker and Black/M Schenck6 Present case Black/M
Crisis at presentation
Organisms cultured
Staphylococcus
Treatment
aureus
34
-
Streptococcus viridans
33
+
Pseudomonas
21
+
Mixed organisms
21
+
Normal oral flora
22
Eikenella corrodens. Bacteroides melangenicus, Peptostreptococcus, mixed Streptococcus and Staphylococcus
Sequestrectomy and curettage Debridementcorticotomy Sequestrectomy and curettage Sequestrectomy and curettage Sequestrectomy Resection of mandible and adjacent muscles
Sickle cell me
Homo Homo Homo Homo Homo Homo
Homo,Homozygous;L. left: R, right
mandibular left third molar were extracted. Drainage of a large amount of brownish black, foul-smelling purulence was obtained from the mandibular extraction site and the extraoral incision, and cultures were submitted for aerobic and anaerobic bacterial and fungal evaluation. A Penrose drain was inserted in the dependent portion of the neck. Metronidazole, 500 mg every 6 hours, was added to the antibiotic regimen. Culture results showed growth of Eikenella corroders, Bacteroides melaninogenicus, Peptostreptococcus, and mixed Streptococcus and Staphylococcus flora organisms. On the basis of the culture and sensitivity report, nafcillin, 1 gm every 4 hours, was begun. The patient’s temperature, left facial edema, and trismus decreased.The Penrosedrain was removed 10days later. Even though the amount of drainage decreasedsubstantially, a moderate amount continued to drain from the site. We therefore decided to explore the area. The wound was explored through a Risdon incision. The entire medical aspect of the mandibular horizontal and ascending rami were found to be devitalized. Involvement of the lateral cortex and the adjacent musculature and periosteum appearedto be minimal. Resection of the lingual cortex and debridement of the cancellous portion of the mandible from the lingula to the mental foramen was carried out. A portion of the lateral pterygoid, mylohyoid, and temporal muscleswere found to be devitalized and were resected.These together with the resectedbone were submitted for biopsy and culture studies (Figs. 2 to 4). Daily dressing changes were performed, and the wound drainage decreased. The patient was discharged 3 weeks after surgery. His wound was managed with daily irrigations and packing changes on an outpatient basis. The wound, however, did not heal, and drainage continued. Six weeks after
discharge the patient was readmitted to the hospital and a technetium Tc 99m scan was performed. On the basisof the physical findings and the positive scan, a decision to reexplore the infection site was made. The patient again underwent an exploration of the left mandibular area through the previous Risdon incision. At this time the lateral cortex of the left mandible and the adjacent lateral and medial soft tissue were found to be infected. A left hemimandibulectomy was performed together with resection of all infected muscle tissue, which involved a portion of the masseter muscle and the deep portions of the lateral pterygoid, temporal, and mylohyoid muscles.The patient was given cephapirin (Cefadyl), 1 gm every 6 hours. Daily dressing changes of the extraoral wounds were undertaken, and 3 weeks after surgery the patient underwent a delayed primary closure of the wound. Healing progressedwithout complications (Table I). DISCUSSION
The present case has certain elements in common with previously reported cases5 to 7 (Table I). The initial symptoms of pain and swelling were present as they were in the other five cases.Our patient’s age, like those of the patients cited in the other studies, was beyond the preteen years, the period during which osteomyelitis resulting from sickle cell disease most commonly occurs. We hypothesize that two mechanisms may be involved for the development of osteomyelitis in this disease.A crisis can cause a sterile area in bone that can then be invaded by blood-borne or local bacteria. The secondpossibility is that a systemic infection can
ORAL SURC ORAL MED ORAL PATHOL July 1991
tic. The extent of osteomyelitis and the subsequent resection in this case were greater than in earlier reported cases. In addition, the involvement of the condyle was not seenin previous reports. The patient refused reconstruction or the removal of the Ivy loops. A vestigial mandible subsequently developed (Fig. 5). SUMMARY
Fig. 5. Panoramic x-ray film demonstrating extent of resection of left mandible and regeneration of mandibular bone.
trigger a crisis, which in turn leads to an infarct. Infarcts of the long bones after a crisis do not reveal themselves at the time of onset, but lysis usually appears a couple of weeks later. The crisis is initially manifested by painful episodes.One of Girasole and Lyon’s patients’ initially had pain as his only symptom. Two weekslater swelling accompanied the painful area. Our patient demonstrated a similar history. On the other hand, the patient reported by Daramola9 and one of Girasole and Lyon’s patients’ were in crisis and infected on presentation. This could indicate that the infection was the initiating phenomenon that helped trigger the sickling crisis. Diggs5 has stated that “no one has ever demonstrated infarcts and necrosis in muscles and tendons or connective tissue.” It seemsreasonable that, as he further stated, stasis and ischemia can effect these structures as well as any others. We did not obtain a biopsy specimenof the anterior deep temporal, mylohyoid, masseteric, or pterygoid arteries; however, the extensive necrosis of the temporal, mylohyoid, masseter, and interanal pterygoid muscles undoubtedly could have been due to a direct invasion of oral organisms infecting structures whose arterial supply was compromised. That would certainly account for the massivenecrosisseenduring the two debridement episodes. The inferior alveolar artery was probably thrombosed. An attempt was made to maintain the continuity of the mandible by resecting only the apparently involved lingual cortex from the linguale to the mental foramen. The resection included the inferior alveolar artery and the associated marrow. Unfortunately, it was not clear at the time that the lateral cortex had also becomeinfected and that the bone was unable to ward off the infection, because its two sourcesof blood supply, namely the inferior alveolar artery and the adjacent musculature, were not absent. Clearly, resection of the involved bone was therapeu-
We report a case of osteomyelitis of the mandible that resulted from a sickle cell crisis in a black man with homozygous sickle cell disease. This rare case was more extensive than previously reported casesin its destruction of the mandible and involved the condyle. A biopsy of the adjacent muscles demonstrated the presence of sickle cells in a vessel lumen as well as the presenceof an infarct. The diagnosis of homozygous sickle cell diseaseshould alert one to the possibility of periodontal or periapical infections contributing to the development of osteomyelitis of the jaws. The extent of the lesion is rarely discernible during the acute phase. Significant necrosis of the adjacent musculature and soft tissue, as well as thrombosis of the inferior alveolar artery, demanded the extirpation of the ascending and horizontal ramus of the mandible and of a significant amount of adjacent soft tissue in the region. REFERENCES 1. Smith WS. Sickle cell anemia and Salmonella osteomyelitis. Ohio Med J 1953;49:692-5. 2. Hook EW. Salmonellosis: certain factors influencing the interaction of Salmonellu and the human host. Bull N Y Acad Med 1961;37:499-512. 3. Silver HK, Simon JL, Clement OH. Snlmonella osteomyelitis and abnormal hemoglobin disease.Pediatrics 1957;20:439-47. 4. Hughes JG, Carroll DS. Salmonellu osteomyelitis complicating sickle cell disease. Pediatrics 1957;19:184-91. 5. Diggs LW. Bone and joint lesions in sickle cell disease. Clin Orthop 1967;52:119-43. 6. Walker RD, Schenck KL. Infarct of the mandible in sickle cell anemia: report of case. J Am Dent Assoc 1973;87:661-4. 1. Girasole RV, Lyon ED. Sickle ceil osteomyelitis of the mandible: report of three cases.J Oral Surg 1977;35:231-4. 8. Bennett IL Jr, Hook EW. Infectious diseases(some aspectsof salmonellosis). Ann Rev Med 1959;10:1-20. 9. Daramola JO. Massive osteomyelitis of the mandible complicating sickle cell disease: report of case. J Oral Surg 1981; 391144-6. 10. Sares HE. Osteomyelitis complicating sickle cell thalassemia syndrome. Tex Ren Biol Med 1964:22:356-66. 11. Statman N, Spitzel S. Bucteroides, ‘septecemia and osteomyelitis: report of a case complicating sickle cell anemia. Ohio Med J 1962;58:1374-7.
Reprint requests:
Daniel Lew, DDS Louisiana State University Medical Center 1501 Kings Highway Shreveport, LA 7 1130
