mplant report Jaime Susan
rehabilitation S. Brahim, DDS, F. Rudy, BSN”
in Erdheim-Chester
MS,a Albert
D. Guckes,
Clinical Investigations and Patient Care Branch, National of Dental Research, Bethesda, Md.
rdheim-Chester disease is a rare multisystem histiocytic disorder first described in 1930. Chester1 is credited with characterizing the disease as a histiocytic entity, distinct from Hand-Schiiller-Christian and Niemann-Pick disease. The clinical manifestations range from minor symptoms, such as joint pain and keloid formation,2, 3 to potentially fatal widespread involvement of the heart, pericardium, lungs, retroperitoneal tissues, and bone.3 A diagnosis of Erdheim-Chester disease is made from radiographic and histologic findings.4-6 Radiographs show bilateral symmetric sclerosis of the metaphyses and diaphyses of the long bones. A lytic bony component has been noted in one third of the reported patients.7 Histologic findings include an infiltration of foamy, lipid-laden histiocytes and giant cells, with medullary fibrosis and osteosclerosis. Lymphocytes and plasma cells are occasionally seen.7 Treatment approaches vary and have included radiation, chemotherapy, and steroids. One third of the cases are fatal,” often as a result of cardiac failure.2,7,8 CLINICAL
DDS,
Institutes
MSD,b
disease: A clinical
and
of Health,
National
Institute
removed in 1981 when the patient was 29. At the time of extractions, the surgeon noted that the alveolar bone resembled adipose tissue. Histologic findings, which included chronic lipid granulomatosis with lipid-laden histiocytes, lymphocytes, and plasma cells, were consistent with Erdheim-Chester disease and confirmed the clinical impression. Following extractions, the alveolar ridges resorbed rapidly.g The amount and pattern of resorption complicated treatment with complete dentures. Despite multiple adjustments and relines over 4 years, the patient could not tolerate the mandibular denture. In 1985, the patient was counseled regarding the lack of supporting alveolar bone, and subperiosteal or endosseous implants were recom-
REPORT
A 38-year-old white man had been referred to the National Institutes of Health Clinical Center in 1978 with a provisional diagnosis of Erdheim-Chester disease. Subsequent referral to the Dental Clinic of the National Institute of Dental Research revealed advanced periodontitis requiring extraction of all remaining teeth. The teeth were
Fig.
2. Abnormal resorptive pattern of maxillary residual
ridge.
%enior Staff Oral and Maxillofacial Surgeon. bDeputy Clinical Director, Patient Care and Clinical Studies Section. ‘clinical Coordinator, Oral and Maxillofacial Surgery/ENT 10/l/38582
Fig.
THE
1. Severe
JOURNAL
resorption
OF PROSTHETIC
of mandibular
DENTISTRY
residual
ridge.
Fig.
3. Multiple
fat deposits
in neck.
BRAHIM,
Fig. 4. Patty tissue from site of fixture placement.
Fig,
GUCKES,
AND
RUDY
6. Implant abutments in place.
Fig. ‘7. Maxillary complete denture and mandibular fixed prosthesis before Locking-screw access holes were sealed. 5. Histologic specimen from surgical site demonstrates infiltration of foamy histiocytes.
Fig.
mended. However, the cardiac status precluded elective surgery. Given the history of rapid residual bone resorption and the presumed widespread adipose infiltration of the remaining bone, the response to implants was considered unpredictable. Because of the unknown prognosis, several more unsuccessful attempts were made to provide an acceptable mandibular denture. In 1988, clinica examination showed severe residual ridge atrophy with absence of the vestibule (Figs. 1 and 2). Several fatty deposits were present in the skin of the face and neck (Fig. 3). Medical clearance was obtained to place endosseous implants using the technique described by Brtinemark et aLlo During surgery, it was noted that the anterior mandible was filled with deposits of yellow material resembling adipose tissue (Fig. 4). Samples of soft and bony tissue sent for pathologic examination contained large, foamy histiocytes mixed with inflammatory cells (Fig. 5). Despite the abnormal bone composition, initial mechanical stability of six 10 X 3.75 mm fixtures was achieved. The postoperative
400
course was without complications. Four months later, abutments were connected (Fig. 6) to all six clinically immobile fixtures. Prosthodontic reconstruction followed the Branemark technique lo (Fig. 7). The fixed mandibular denture has been functioning since November 1989 and the fixtures are assumed to be osseointegrated. SUMMARY Successful osseointegration of endosseous titanium implants is thought to be dependent upon close apposition of bone to the implant surface. The integration of implants in this patient was achieved despite the lipid-laden histiocytic infiltration of the bone marrow. Presumably, enough unaffected stromal cells were present to allow sufficient bone formation for osseointegration of the implant fixtures. This result invites speculation regarding both the mechanism of osseointegration and the minimum surface area of boneimplant interface necessary for achieving and maintaining osseointegration of titanium implants. This patient is periodically examined to determine if the loaded fixtures will remain clinically immobile for a prolonged period.
SEPTEMBER
1992
VOLUME
68
NUMBER
3
IlMPLAiVTS
Ii?’ ERDHEIM-CHESTER
DISEASE
We thank Dr. Lorren Zech and Dr. Leah Royce for their assistance with the medical management of this patient, and Mr. Victor Morgan for assistance with the dental laboratory procedures. REFERENCES 1. Chester W. Lipoid granulomatosis. Virchows Arch 1930;279:561-602. 2. Resnick D, Greenway G, Genant H, Brewer A, Haghighi P, Emmett M. Erdheim-Chester disease. Radiology 1982;142:289-95. 3. Atkins HL, Klopper JP, Ansari AN, Iwai J. Lipid (cholesterol) granulomatosis (Chester-Erdheim disease) and congential megacalices. Clin Nucl Med 1978;3:324-7. 4. Brewer AC, Worsham GF, Dudley, AH. Erdheim-Chester disease. A distinct lipoidosis or part of the spectrum of histiocytosis? Radiology 1986;151:35-8. 5. Freyochmidt J, Ostertag H, Lang W. Case report 365. Skeletal Radio1 1986;15:316-22. 6. Waite RJ, Doherty PW, Liepman M, Woda B. Langerhsns’ cell histio-
THE
JOURNAL
OF PROSTHETIC
DENTISTRY
cytosis with the radiographic findings of Erdheim-Chester disease. AJR 1988;150:869-71. 7. Miller RL, Sheeler LR, Bauer TN, Burowski TM. Erdheim-Chester disease.Casereportandreviewoftheliterature.Am JMed1986;80:1230-6. 8. Ferrans VJ, Rodriguez ER, McAllister HA. Granulomatous inflammation of the heart. Heart Vessels 1985;(suppl 1):262-70. 9. Valdez IH, Katz RW, Travis WD. Premature alveolar bone loss in Erdheim-Chester disease. Oral Surg Oral Med Oral Pathol 1990;70:294-6. 10. Brinemark PI, Zarb GA, Albrektsson T. Tissue integrated prosthesis. Chicago: Quintessence Publishing Co, 1985:211-82. Reprint requests to: DR. JAIME BRAHIM PATIENT CARE AND CLINICAL STUDIES SECTION, CIPCB NATIONAL INSTITUTE OF DENTAL REGWZCH NATIONAL INSTITUTES OF HEALTH BUILDING 10, ROOM 112 BETHESDA, MD 20892
401
rehabilitation S. Brahim, DDS, F. Rudy, BSN”
in Erdheim-Chester
MS,a Albert
D. Guckes,
Clinical Investigations and Patient Care Branch, National of Dental Research, Bethesda, Md.
rdheim-Chester disease is a rare multisystem histiocytic disorder first described in 1930. Chester1 is credited with characterizing the disease as a histiocytic entity, distinct from Hand-Schiiller-Christian and Niemann-Pick disease. The clinical manifestations range from minor symptoms, such as joint pain and keloid formation,2, 3 to potentially fatal widespread involvement of the heart, pericardium, lungs, retroperitoneal tissues, and bone.3 A diagnosis of Erdheim-Chester disease is made from radiographic and histologic findings.4-6 Radiographs show bilateral symmetric sclerosis of the metaphyses and diaphyses of the long bones. A lytic bony component has been noted in one third of the reported patients.7 Histologic findings include an infiltration of foamy, lipid-laden histiocytes and giant cells, with medullary fibrosis and osteosclerosis. Lymphocytes and plasma cells are occasionally seen.7 Treatment approaches vary and have included radiation, chemotherapy, and steroids. One third of the cases are fatal,” often as a result of cardiac failure.2,7,8 CLINICAL
DDS,
Institutes
MSD,b
disease: A clinical
and
of Health,
National
Institute
removed in 1981 when the patient was 29. At the time of extractions, the surgeon noted that the alveolar bone resembled adipose tissue. Histologic findings, which included chronic lipid granulomatosis with lipid-laden histiocytes, lymphocytes, and plasma cells, were consistent with Erdheim-Chester disease and confirmed the clinical impression. Following extractions, the alveolar ridges resorbed rapidly.g The amount and pattern of resorption complicated treatment with complete dentures. Despite multiple adjustments and relines over 4 years, the patient could not tolerate the mandibular denture. In 1985, the patient was counseled regarding the lack of supporting alveolar bone, and subperiosteal or endosseous implants were recom-
REPORT
A 38-year-old white man had been referred to the National Institutes of Health Clinical Center in 1978 with a provisional diagnosis of Erdheim-Chester disease. Subsequent referral to the Dental Clinic of the National Institute of Dental Research revealed advanced periodontitis requiring extraction of all remaining teeth. The teeth were
Fig.
2. Abnormal resorptive pattern of maxillary residual
ridge.
%enior Staff Oral and Maxillofacial Surgeon. bDeputy Clinical Director, Patient Care and Clinical Studies Section. ‘clinical Coordinator, Oral and Maxillofacial Surgery/ENT 10/l/38582
Fig.
THE
1. Severe
JOURNAL
resorption
OF PROSTHETIC
of mandibular
DENTISTRY
residual
ridge.
Fig.
3. Multiple
fat deposits
in neck.
BRAHIM,
Fig. 4. Patty tissue from site of fixture placement.
Fig,
GUCKES,
AND
RUDY
6. Implant abutments in place.
Fig. ‘7. Maxillary complete denture and mandibular fixed prosthesis before Locking-screw access holes were sealed. 5. Histologic specimen from surgical site demonstrates infiltration of foamy histiocytes.
Fig.
mended. However, the cardiac status precluded elective surgery. Given the history of rapid residual bone resorption and the presumed widespread adipose infiltration of the remaining bone, the response to implants was considered unpredictable. Because of the unknown prognosis, several more unsuccessful attempts were made to provide an acceptable mandibular denture. In 1988, clinica examination showed severe residual ridge atrophy with absence of the vestibule (Figs. 1 and 2). Several fatty deposits were present in the skin of the face and neck (Fig. 3). Medical clearance was obtained to place endosseous implants using the technique described by Brtinemark et aLlo During surgery, it was noted that the anterior mandible was filled with deposits of yellow material resembling adipose tissue (Fig. 4). Samples of soft and bony tissue sent for pathologic examination contained large, foamy histiocytes mixed with inflammatory cells (Fig. 5). Despite the abnormal bone composition, initial mechanical stability of six 10 X 3.75 mm fixtures was achieved. The postoperative
400
course was without complications. Four months later, abutments were connected (Fig. 6) to all six clinically immobile fixtures. Prosthodontic reconstruction followed the Branemark technique lo (Fig. 7). The fixed mandibular denture has been functioning since November 1989 and the fixtures are assumed to be osseointegrated. SUMMARY Successful osseointegration of endosseous titanium implants is thought to be dependent upon close apposition of bone to the implant surface. The integration of implants in this patient was achieved despite the lipid-laden histiocytic infiltration of the bone marrow. Presumably, enough unaffected stromal cells were present to allow sufficient bone formation for osseointegration of the implant fixtures. This result invites speculation regarding both the mechanism of osseointegration and the minimum surface area of boneimplant interface necessary for achieving and maintaining osseointegration of titanium implants. This patient is periodically examined to determine if the loaded fixtures will remain clinically immobile for a prolonged period.
SEPTEMBER
1992
VOLUME
68
NUMBER
3
IlMPLAiVTS
Ii?’ ERDHEIM-CHESTER
DISEASE
We thank Dr. Lorren Zech and Dr. Leah Royce for their assistance with the medical management of this patient, and Mr. Victor Morgan for assistance with the dental laboratory procedures. REFERENCES 1. Chester W. Lipoid granulomatosis. Virchows Arch 1930;279:561-602. 2. Resnick D, Greenway G, Genant H, Brewer A, Haghighi P, Emmett M. Erdheim-Chester disease. Radiology 1982;142:289-95. 3. Atkins HL, Klopper JP, Ansari AN, Iwai J. Lipid (cholesterol) granulomatosis (Chester-Erdheim disease) and congential megacalices. Clin Nucl Med 1978;3:324-7. 4. Brewer AC, Worsham GF, Dudley, AH. Erdheim-Chester disease. A distinct lipoidosis or part of the spectrum of histiocytosis? Radiology 1986;151:35-8. 5. Freyochmidt J, Ostertag H, Lang W. Case report 365. Skeletal Radio1 1986;15:316-22. 6. Waite RJ, Doherty PW, Liepman M, Woda B. Langerhsns’ cell histio-
THE
JOURNAL
OF PROSTHETIC
DENTISTRY
cytosis with the radiographic findings of Erdheim-Chester disease. AJR 1988;150:869-71. 7. Miller RL, Sheeler LR, Bauer TN, Burowski TM. Erdheim-Chester disease.Casereportandreviewoftheliterature.Am JMed1986;80:1230-6. 8. Ferrans VJ, Rodriguez ER, McAllister HA. Granulomatous inflammation of the heart. Heart Vessels 1985;(suppl 1):262-70. 9. Valdez IH, Katz RW, Travis WD. Premature alveolar bone loss in Erdheim-Chester disease. Oral Surg Oral Med Oral Pathol 1990;70:294-6. 10. Brinemark PI, Zarb GA, Albrektsson T. Tissue integrated prosthesis. Chicago: Quintessence Publishing Co, 1985:211-82. Reprint requests to: DR. JAIME BRAHIM PATIENT CARE AND CLINICAL STUDIES SECTION, CIPCB NATIONAL INSTITUTE OF DENTAL REGWZCH NATIONAL INSTITUTES OF HEALTH BUILDING 10, ROOM 112 BETHESDA, MD 20892
401
