- Email: [email protected]
Fibrous dysplasia: Findings in a pre-Columbian skull
Fibrous Dysplasia: Findings in a Pro-Columbian Skull RINALD0 F. CANA~.ZS,M.D.,* ROBERT0 M. ARAGON,M.D.,$ FERNANDO CABIESES, M.D.,~: AND WILLIAM N. HANAFI~1LM.D.w
An ancient Peruvian specimen (600 to 800 years old) with fibrous dysplasia involving the left frontoethmoidal region is discussed. The skull is that of a woman aged 20 to 30 years exhibiting marked deformity of the left frontal bone, ethmoids, orbit, and nasal wall. Plane radiographs and tomographic views demonstrate a heavily calcified, expanding process in the frontal region and replacement of the ethmoids by less dense osseous tissue. This specimen represents the second reported instance of fibrous dysplasia occurring in the Americas, further documenting the antiquity of the disease on this continent. The lesion is probably monostotic and has characteristics s{milar to the process as it is known today.
Ancient pathologic findings often provide important information regarding late results of untreated disease. However, they have stimulated little interest among current day clinicians. Indeed the study of paleopathologic specimens is frequently regarded as a scholarly pastime with no practical value. This attitude is unfortunate, since understanding of a disease is enhanced by knowledge of its past manifestations; moreover, such knowledge permits additional insight into the society in which the disease developed. The scientific value of prehistoric specimens is especially important in the evaluation of pathologic processes involving bone. For the otolaryngologist the existence in the United States of several pro-Columbian skull collections provides a unique source of material for study. Traumatic, neoplastic, congenital, and infectious diseases of the craniofacial structures arc
represented by specimens covering a period of over 1000 years. The authors' opportunity to study the prehistoric skull collection of the Museum of Man in San Diego prompted this initial communication. Its purpose is to describe the findings in an exceptional specimen of fibrous dysplasia involving the frontal and ethmoidal sinuses and to stress the importance of studies of this nature. The specimen has been studied in detail by plane and tomographic radiography at the UCLA Center for the Health Sciences.
SOURCE SITE AND MATERIAL
The prehistoric skull collection of the Museum of Man in San Diego contains over 900 specimens. The majority of these were obtained and classified by Ales Hrdlicka during the first two
Acceptedfor publication July 11, 1979.
*Associate Professor of Surgery, University of California, LosAngeles, School of Mediclne. Chief, Division ofOtolaryngology, Horbor-UCLA Medical Center. Vice Chief, Section of Head and Neck Surgery, UCLA Center for Health Sciences, Torrance, California. tHonora137 Consul of Peru at Los Angeles, California. $Professor of Surgery, Universldad Naeional de San Mareos,Lima, Peru. w of Radiology, University of California, Los Angeles, School of Medicine,Los Angeles, California. American ]ournal of Otoloryngology--Volume 1, Number 2, February 1980
1.3"1
American Journal Otolaryngo]ogy
decades of this century. Many were studied by Moodie":l in the late twenties and thirties, and several interesting otolaryngologic findings were reported by Burton 4 in 1927. Most of these specimens were obtained from several different areas of Peru inhabitated by culturally different human groups. The skull to be described was found in Ancon, an archeologic area located on the Pacific coast 28 miles from the capital city of Lima. In pre-Hispanic times, as today, Ancon was populated by small groups of people whose main occupation is fishing. Although Ancon is in close proximity to areas archeologically rich in architectural findings, the site itself is only a burial ground. Several strata have been uncovered from this cemetery, some dating back 2000 years. 5. The specimens collected from this location are representative of the most superficial layer (approximate depth, 9 m.). In fact, many of the specimens had been exposed by vandals and weekend archeologists seeking ancient pottery and metal artifacts. The specimens therefore represent relatively recent pre-Columbian skulls dating from the Inca period, placing the age of the one to be described at approximately 600 to 800 years.
THE SPECIMEN
Figure 1, Frontal photograph of the specimen. Note pronounced frontoethmoidal deformity with distortion of the left supraorbital ridge. T h e r e is marked septal deviatiox~. The irregular verticat frecturc of the froutal bone ocuurred post martem.
and the vomer are deviated to the right but are not involved. With the exception of slight extension of the lesion across the midline in the frontal region, the right half of the skull is essentially normal.
Gross Description Figures 1, 2, and 3 are representative of the findings. The skull is that of a young female, between 20 and 30 years old. Postmortem fracture of the left molar bone has resulted in loss of this structure, as well as the zygomatic arch and lateral wall of the orbit. The left maxillary antrum, including the rim and orbital floor, is also destroyed, probably the result of vandalism. The mandible is missing. The most obvious antemortern abnormality is a striking asymmetry of the forehead. The left portion of the frontal bone protrudes forward in a smooth rounded tumorlike deformity, 6.8 cm. wide and 5.2 cm. high. An irregular vertical postmortem fracture runs close to the dome of this deformity. The superior orbital fissure is obliterated, but the orbital foramen is uninvolved. The anterior and posterior ethmoidal cells and the anterior wall of the sphenoid are entirely replaced by a porous, finely trabeculated bony mass. The same type of tissue has invaded the lateral wall of the nose, distorting the normal architecture of the turbinates. The perpendicular plate of the ethmoids
13 2
Figt~re 2. Photograph t a k e n in a plane perpendicular to the specimen to illustrate the extreme frontal deformity present.
FIBROUS DYSPLAS[A: FINDINGS IN A PRE-COLUMBIAN SKULl,
postmortem anterior brake and a small linear dehiscence at the midportion of the posterior wall. The latter defect bears no relationship with any of the grossly evident postmortem fractures and could have occurred during life. The clivus and anterior portion of the sphenoid bone show evidence of bone proliferation, but the sphenoidal sinus cavity is aerated. The ethmoids on the left are replaced to a great extent by less dense bone than that encountered in the frontal bone. The remainder of the specimen is radiographically unremarkable except for several postmortem fractures located outside the area under study.
Figure 3. Three-quarter profile of the specimen, Note the porous quality of the lesion in the medial orbital wall and ethmoids.
Radiographic Description Plane views were taken in the standard sinus projections. Tomograms were obtained only in the lateral plane. Figures 4 and 5 exemplify the findings. The lesion is revealed as a markedly expansive process arising from the left frontal bone and sinus and extending inferiorly into the ethmoids and orbit. Except at the ethmoidal level where it loses definition, the periphery of the process has become heavily calcified, acquiring a ground glass appearance. The core of the lesion appears to have been less proliferative and has less radiographic density. There are, however, confluent, disc shaped, heavily calcified centers that are attached to the walls of the left frontal bone. The walls of this cavity have expanded and exhibit a
Volume 1 Number 2
February
1980
Differential Diagnosis Although histologic confirmation of the diagnosis in this specimen is not possible, both gross and radiographic findings are highly compatible with fibrous dysplasia of bone. A few other possibilities, especially Paget's osteitis deformans, have to be excluded. Several facts are against this diagnosis. Paget's disease is extremely infrequent before age 30 to 40 years, whereas the biologic age of the skull presented is between 20 and 30 years, most probably under 25 years. Furthermore, the process under study is likely to have started during childhood and probably represents several years of active disease. Other general facts helpful in excluding the diagnosis of osteitis deformans are its rare occurrence among South Americans and its prevalence among males. At first sight the plane radiographic views may be considered pagetoid because of the presence of multiple spherical densities reminiscent of the "cotton-wool" pattern seen in this disease. Closer observation, however, shows these areas to be the product of active bone pro-
Figure 4. A, Lateral radiograph of the skull showing marked distortion of the left frontal bone and sinus. The normal appearance has been changed by dense, heavily calcified bone. B, Radiograph taken in a piano directly perpendicular to the specimen. Note the expanding character of the lesion. The outer cortex is thinned but intact, a typical feature of libraus dysplasia,
RINALDO F. CANALIS ET AI..
13 3
American
Journal of Otolaryngology
liferation within the core of the lesion, an arrangement frequently noted in mature areas of dysplasia. The smooth appearance of the outer frontal cortex and the diffuse involvement of the left ethmoids are also more compatible with fibrous dysplasia. In Paget's disease the mottled appearance of the skull is secondary to irregular thickening of the outer table and the diplo~. This lesion seems to have expanded the frontal sinus without directly involving the diplo~. An additional point of interest is that in Paget's disease affecting the cranium, involvement of the temporal bones is nearly universal! In this skull the temporal bones appeared to be normal. Besides osteitis deformans, a malignant primary or metastatic lesion may be considered; however, it is hardly possible in the absence of multilocular erosive areas. Mucocele, pneumosinus dilatans, hyperostosis frontalis interna, osteomyelitis, and other inflammatory lesions that may be suspected upon gross examination cannot be radiographically supported.
COMMENT Fibrous dysplasia is a benign disease of unknown etiology characterized by active replacement of normal elements of bone by fibrous tissue exhibiting varying degrees of osseous metaplasia. According to Eden, s the disease was first described by Menzel in 1872. The specimen herein presented establishes the antiquity of the disease among South Americans, and it is the second report of the prehistoric existence of fibrous dysplasia on this continent. In 1931 Denninger:' described a 1000 year old skeleton with the polyostotic form of the disease encountered in Lewinston, Illinois. The remains were of a man aged 35 years crippled by multiple bone lesions. The skull involvement was limited to the left maxilla, palatine bone, and pterygoid process. Fibrous dysplasia is currently classified into three types. In type I, the monostofic variety, the lesions m a y be single or multiple but are restrict-
Figure 5. Successive lateral tomographic views (from le[t to right) demonstrating the dense osteomatous appearance of the frontal hone in the most peripheral portion of the lesion (A). The expanding character of the lesion is clearly exemplified (B, C). Note confluent, heavily calcified disks attached to the walls of the frontal bone. B, The arrow shows posterior wall fracture, Increased calcification is demonstrated as the right peripheral aspect of file lesion is reached (D to F). The right frontal sinus is aerated (E) and the right ethmoids (F) appear intact.
134
FIBROUS DYSPLASIA: FINDINGS IN A PRE-COLUMBIAN SKULL
ed to one bone. In type II, the po]yostotic variety, the disease involves several bones. In type HI, disseminated lesions are often accompanied by abnormal pigmentation and endocrine dysfunct.ion (Albright's syndrome). A hereditary form producing symmetrical maxillary enlargement known as cherubism has been described by Jones et al.10 Involvement of the skull may occur in any of the three main types, but it most frequently represents monostotic disease. In the series of 47 craniofacial cases presented by Ramsey et al. '1 five were polyostotic, and of these only two showed extracranial involvement. A similar distribution has been noted by others, r~ The lack of other skeletal remains prevents us from accurately classifying the lesion presented here. However, since craniofacial manifestations are frequently isolated, it is likely that it was type I. Frontal and ethmoidal sinus involvement by fibrous dysplasia represents approximately 10 per cent of all craniofacial lesions. '" ~' The rarity of this location and the opportunity to document the results of uncontrolled fibrous dysplasia are important features of the specimen described. In addition it offered the possibility of studying the lesion in continuity, which demonstrates the progressive tendency occasionally seen in this disease. This evidence of continuous growth is interesting, since lesions originating in childhood tend to become stable about the age of puberty.r,. ,3 Although a clinical picture is difficult to outline, some of this prehistoric woman's symptoms may be easily inferred. Proptosis with inferior and lateral displacement, diplopia, and ophthalmoplegia were almost certainly present. Blindness occurred as a result of extreme proptosis, corneal abrasion, and infection. Whether fibrous dysplasia was responsible for the woman's death is impossible to discover. The fracture of the posterior table of the frontal sinus is similar to others described as spontaneously occurring in this disease and could have been inconsequential. ,4 To conclude, the existence of the craniofacial lesions of fibrous dysplasia in ancient times has been documented. Although no environmental factors have been identified as being etiologically important, the disease developed in a rela-
R I N A L D O F. C A N A L I S E T AL.
tively natural environment. The findings suggest that the disease had features similar to those recognized today.
Volume 1 Number 2 February 1980
Acknowledgments The authors are grateful to Rose Tyson, Curator, Physical Anthropology, and Barton Wright, Scientific Director of the San Diego M u s e u m of Man, for their professional assistance and generous help. Tom Lotto p r o v i d e d the photographic material and Tatiana Astroza, editing help.
References 1. Moodie, R. L,: Studies in paleopathology. XXI. [njurles to the head among the pro-Columbian Peruvians. Ann. Med. Hist., 9:277-306, 1927. 2. Moodie, R. L.: ArcheoIogioal evidences of the antiquity of disease in South America. Sci. Monthly, 29:193211, 1929, 3. Moodie, R. I,.: Studies in paleopathology. XXIII. Surgery in pro-Columbian Peru, Ann. Mad. Hist., 11:698-728, :1929. 4. Burton, F. A.: Some Considerations on Prehistoric Aural, Nasal, Sinus Pathology and Sm'gery. Santa Fe, New Mexico, E1 Palaeio Press, 1929, pp. 1-38. 5. Uhle, M.: Die Muschelhugel van Ancon. Proceedings of the XVII[ Session of the International Congress of Americanists. London, England, 19't2, pp. 22-45. 6. Kaufman Doig, F.: Hlstoria General de los Paruanos. I: E1 Peru Atltiguo. Lima, Peru, Ediciones Peisa, 1978, pp. 160-168. 7. Nager, G. T.: Paget's disease of the temporal bone, Aml. Oral., 84 (Suppl. 22}: 1-32, 1975. 8. Eden, K. C.: Benign fibre-osseous tumors of the skull and facial bones. Br. J. Surg., 27:275-289, 1939. 9. Denninger, H. S.: Osteitis fibrosa in a skeleton of a prehistoric American Indian. Arch. Pathol., 11:939-946, 1931. 10. Jones, W. A., Gerrie, J., and'Pritchard, J.: Cherubism: a familial fibrosus dysplasia of the jaws. J. Bone Joint Surg., 32B:334-347, 1950. 11. Ramsay, H. E., Skrong, E. W., and Frazell, E. L,: Fibrous dyspiasia of the craniofaciaI bones. Am. J. Surg., 116:542-547, 1968. 12. Georgiade, N., Masters, F., Herren, C., and Pickrele, K.: Ossifying fibromas (fibzous dysplasia) of the facial bones in children and adolescents, J, Pediatr., 46:3641, 1955. 13. Ward, P. H., Alley, C., and Owen, R.: Monostotic fibrous dysplasia of the maxilla. Laryngoscope, 79:12951306, 1969. 14. Sherman, R. S., and Sternberg, W.'. The roentgen appearance of ossifying fibroma of bone. Radiology, 5 0 : 5 9 5 619, 1948. Harbor-UCLA Medical Center 1000 West Carson Street Torrance, California 90509 (Dr, Canalis}
13 5
An ancient Peruvian specimen (600 to 800 years old) with fibrous dysplasia involving the left frontoethmoidal region is discussed. The skull is that of a woman aged 20 to 30 years exhibiting marked deformity of the left frontal bone, ethmoids, orbit, and nasal wall. Plane radiographs and tomographic views demonstrate a heavily calcified, expanding process in the frontal region and replacement of the ethmoids by less dense osseous tissue. This specimen represents the second reported instance of fibrous dysplasia occurring in the Americas, further documenting the antiquity of the disease on this continent. The lesion is probably monostotic and has characteristics s{milar to the process as it is known today.
Ancient pathologic findings often provide important information regarding late results of untreated disease. However, they have stimulated little interest among current day clinicians. Indeed the study of paleopathologic specimens is frequently regarded as a scholarly pastime with no practical value. This attitude is unfortunate, since understanding of a disease is enhanced by knowledge of its past manifestations; moreover, such knowledge permits additional insight into the society in which the disease developed. The scientific value of prehistoric specimens is especially important in the evaluation of pathologic processes involving bone. For the otolaryngologist the existence in the United States of several pro-Columbian skull collections provides a unique source of material for study. Traumatic, neoplastic, congenital, and infectious diseases of the craniofacial structures arc
represented by specimens covering a period of over 1000 years. The authors' opportunity to study the prehistoric skull collection of the Museum of Man in San Diego prompted this initial communication. Its purpose is to describe the findings in an exceptional specimen of fibrous dysplasia involving the frontal and ethmoidal sinuses and to stress the importance of studies of this nature. The specimen has been studied in detail by plane and tomographic radiography at the UCLA Center for the Health Sciences.
SOURCE SITE AND MATERIAL
The prehistoric skull collection of the Museum of Man in San Diego contains over 900 specimens. The majority of these were obtained and classified by Ales Hrdlicka during the first two
Acceptedfor publication July 11, 1979.
*Associate Professor of Surgery, University of California, LosAngeles, School of Mediclne. Chief, Division ofOtolaryngology, Horbor-UCLA Medical Center. Vice Chief, Section of Head and Neck Surgery, UCLA Center for Health Sciences, Torrance, California. tHonora137 Consul of Peru at Los Angeles, California. $Professor of Surgery, Universldad Naeional de San Mareos,Lima, Peru. w of Radiology, University of California, Los Angeles, School of Medicine,Los Angeles, California. American ]ournal of Otoloryngology--Volume 1, Number 2, February 1980
1.3"1
American Journal Otolaryngo]ogy
decades of this century. Many were studied by Moodie":l in the late twenties and thirties, and several interesting otolaryngologic findings were reported by Burton 4 in 1927. Most of these specimens were obtained from several different areas of Peru inhabitated by culturally different human groups. The skull to be described was found in Ancon, an archeologic area located on the Pacific coast 28 miles from the capital city of Lima. In pre-Hispanic times, as today, Ancon was populated by small groups of people whose main occupation is fishing. Although Ancon is in close proximity to areas archeologically rich in architectural findings, the site itself is only a burial ground. Several strata have been uncovered from this cemetery, some dating back 2000 years. 5. The specimens collected from this location are representative of the most superficial layer (approximate depth, 9 m.). In fact, many of the specimens had been exposed by vandals and weekend archeologists seeking ancient pottery and metal artifacts. The specimens therefore represent relatively recent pre-Columbian skulls dating from the Inca period, placing the age of the one to be described at approximately 600 to 800 years.
THE SPECIMEN
Figure 1, Frontal photograph of the specimen. Note pronounced frontoethmoidal deformity with distortion of the left supraorbital ridge. T h e r e is marked septal deviatiox~. The irregular verticat frecturc of the froutal bone ocuurred post martem.
and the vomer are deviated to the right but are not involved. With the exception of slight extension of the lesion across the midline in the frontal region, the right half of the skull is essentially normal.
Gross Description Figures 1, 2, and 3 are representative of the findings. The skull is that of a young female, between 20 and 30 years old. Postmortem fracture of the left molar bone has resulted in loss of this structure, as well as the zygomatic arch and lateral wall of the orbit. The left maxillary antrum, including the rim and orbital floor, is also destroyed, probably the result of vandalism. The mandible is missing. The most obvious antemortern abnormality is a striking asymmetry of the forehead. The left portion of the frontal bone protrudes forward in a smooth rounded tumorlike deformity, 6.8 cm. wide and 5.2 cm. high. An irregular vertical postmortem fracture runs close to the dome of this deformity. The superior orbital fissure is obliterated, but the orbital foramen is uninvolved. The anterior and posterior ethmoidal cells and the anterior wall of the sphenoid are entirely replaced by a porous, finely trabeculated bony mass. The same type of tissue has invaded the lateral wall of the nose, distorting the normal architecture of the turbinates. The perpendicular plate of the ethmoids
13 2
Figt~re 2. Photograph t a k e n in a plane perpendicular to the specimen to illustrate the extreme frontal deformity present.
FIBROUS DYSPLAS[A: FINDINGS IN A PRE-COLUMBIAN SKULl,
postmortem anterior brake and a small linear dehiscence at the midportion of the posterior wall. The latter defect bears no relationship with any of the grossly evident postmortem fractures and could have occurred during life. The clivus and anterior portion of the sphenoid bone show evidence of bone proliferation, but the sphenoidal sinus cavity is aerated. The ethmoids on the left are replaced to a great extent by less dense bone than that encountered in the frontal bone. The remainder of the specimen is radiographically unremarkable except for several postmortem fractures located outside the area under study.
Figure 3. Three-quarter profile of the specimen, Note the porous quality of the lesion in the medial orbital wall and ethmoids.
Radiographic Description Plane views were taken in the standard sinus projections. Tomograms were obtained only in the lateral plane. Figures 4 and 5 exemplify the findings. The lesion is revealed as a markedly expansive process arising from the left frontal bone and sinus and extending inferiorly into the ethmoids and orbit. Except at the ethmoidal level where it loses definition, the periphery of the process has become heavily calcified, acquiring a ground glass appearance. The core of the lesion appears to have been less proliferative and has less radiographic density. There are, however, confluent, disc shaped, heavily calcified centers that are attached to the walls of the left frontal bone. The walls of this cavity have expanded and exhibit a
Volume 1 Number 2
February
1980
Differential Diagnosis Although histologic confirmation of the diagnosis in this specimen is not possible, both gross and radiographic findings are highly compatible with fibrous dysplasia of bone. A few other possibilities, especially Paget's osteitis deformans, have to be excluded. Several facts are against this diagnosis. Paget's disease is extremely infrequent before age 30 to 40 years, whereas the biologic age of the skull presented is between 20 and 30 years, most probably under 25 years. Furthermore, the process under study is likely to have started during childhood and probably represents several years of active disease. Other general facts helpful in excluding the diagnosis of osteitis deformans are its rare occurrence among South Americans and its prevalence among males. At first sight the plane radiographic views may be considered pagetoid because of the presence of multiple spherical densities reminiscent of the "cotton-wool" pattern seen in this disease. Closer observation, however, shows these areas to be the product of active bone pro-
Figure 4. A, Lateral radiograph of the skull showing marked distortion of the left frontal bone and sinus. The normal appearance has been changed by dense, heavily calcified bone. B, Radiograph taken in a piano directly perpendicular to the specimen. Note the expanding character of the lesion. The outer cortex is thinned but intact, a typical feature of libraus dysplasia,
RINALDO F. CANALIS ET AI..
13 3
American
Journal of Otolaryngology
liferation within the core of the lesion, an arrangement frequently noted in mature areas of dysplasia. The smooth appearance of the outer frontal cortex and the diffuse involvement of the left ethmoids are also more compatible with fibrous dysplasia. In Paget's disease the mottled appearance of the skull is secondary to irregular thickening of the outer table and the diplo~. This lesion seems to have expanded the frontal sinus without directly involving the diplo~. An additional point of interest is that in Paget's disease affecting the cranium, involvement of the temporal bones is nearly universal! In this skull the temporal bones appeared to be normal. Besides osteitis deformans, a malignant primary or metastatic lesion may be considered; however, it is hardly possible in the absence of multilocular erosive areas. Mucocele, pneumosinus dilatans, hyperostosis frontalis interna, osteomyelitis, and other inflammatory lesions that may be suspected upon gross examination cannot be radiographically supported.
COMMENT Fibrous dysplasia is a benign disease of unknown etiology characterized by active replacement of normal elements of bone by fibrous tissue exhibiting varying degrees of osseous metaplasia. According to Eden, s the disease was first described by Menzel in 1872. The specimen herein presented establishes the antiquity of the disease among South Americans, and it is the second report of the prehistoric existence of fibrous dysplasia on this continent. In 1931 Denninger:' described a 1000 year old skeleton with the polyostotic form of the disease encountered in Lewinston, Illinois. The remains were of a man aged 35 years crippled by multiple bone lesions. The skull involvement was limited to the left maxilla, palatine bone, and pterygoid process. Fibrous dysplasia is currently classified into three types. In type I, the monostofic variety, the lesions m a y be single or multiple but are restrict-
Figure 5. Successive lateral tomographic views (from le[t to right) demonstrating the dense osteomatous appearance of the frontal hone in the most peripheral portion of the lesion (A). The expanding character of the lesion is clearly exemplified (B, C). Note confluent, heavily calcified disks attached to the walls of the frontal bone. B, The arrow shows posterior wall fracture, Increased calcification is demonstrated as the right peripheral aspect of file lesion is reached (D to F). The right frontal sinus is aerated (E) and the right ethmoids (F) appear intact.
134
FIBROUS DYSPLASIA: FINDINGS IN A PRE-COLUMBIAN SKULL
ed to one bone. In type II, the po]yostotic variety, the disease involves several bones. In type HI, disseminated lesions are often accompanied by abnormal pigmentation and endocrine dysfunct.ion (Albright's syndrome). A hereditary form producing symmetrical maxillary enlargement known as cherubism has been described by Jones et al.10 Involvement of the skull may occur in any of the three main types, but it most frequently represents monostotic disease. In the series of 47 craniofacial cases presented by Ramsey et al. '1 five were polyostotic, and of these only two showed extracranial involvement. A similar distribution has been noted by others, r~ The lack of other skeletal remains prevents us from accurately classifying the lesion presented here. However, since craniofacial manifestations are frequently isolated, it is likely that it was type I. Frontal and ethmoidal sinus involvement by fibrous dysplasia represents approximately 10 per cent of all craniofacial lesions. '" ~' The rarity of this location and the opportunity to document the results of uncontrolled fibrous dysplasia are important features of the specimen described. In addition it offered the possibility of studying the lesion in continuity, which demonstrates the progressive tendency occasionally seen in this disease. This evidence of continuous growth is interesting, since lesions originating in childhood tend to become stable about the age of puberty.r,. ,3 Although a clinical picture is difficult to outline, some of this prehistoric woman's symptoms may be easily inferred. Proptosis with inferior and lateral displacement, diplopia, and ophthalmoplegia were almost certainly present. Blindness occurred as a result of extreme proptosis, corneal abrasion, and infection. Whether fibrous dysplasia was responsible for the woman's death is impossible to discover. The fracture of the posterior table of the frontal sinus is similar to others described as spontaneously occurring in this disease and could have been inconsequential. ,4 To conclude, the existence of the craniofacial lesions of fibrous dysplasia in ancient times has been documented. Although no environmental factors have been identified as being etiologically important, the disease developed in a rela-
R I N A L D O F. C A N A L I S E T AL.
tively natural environment. The findings suggest that the disease had features similar to those recognized today.
Volume 1 Number 2 February 1980
Acknowledgments The authors are grateful to Rose Tyson, Curator, Physical Anthropology, and Barton Wright, Scientific Director of the San Diego M u s e u m of Man, for their professional assistance and generous help. Tom Lotto p r o v i d e d the photographic material and Tatiana Astroza, editing help.
References 1. Moodie, R. L,: Studies in paleopathology. XXI. [njurles to the head among the pro-Columbian Peruvians. Ann. Med. Hist., 9:277-306, 1927. 2. Moodie, R. L.: ArcheoIogioal evidences of the antiquity of disease in South America. Sci. Monthly, 29:193211, 1929, 3. Moodie, R. I,.: Studies in paleopathology. XXIII. Surgery in pro-Columbian Peru, Ann. Mad. Hist., 11:698-728, :1929. 4. Burton, F. A.: Some Considerations on Prehistoric Aural, Nasal, Sinus Pathology and Sm'gery. Santa Fe, New Mexico, E1 Palaeio Press, 1929, pp. 1-38. 5. Uhle, M.: Die Muschelhugel van Ancon. Proceedings of the XVII[ Session of the International Congress of Americanists. London, England, 19't2, pp. 22-45. 6. Kaufman Doig, F.: Hlstoria General de los Paruanos. I: E1 Peru Atltiguo. Lima, Peru, Ediciones Peisa, 1978, pp. 160-168. 7. Nager, G. T.: Paget's disease of the temporal bone, Aml. Oral., 84 (Suppl. 22}: 1-32, 1975. 8. Eden, K. C.: Benign fibre-osseous tumors of the skull and facial bones. Br. J. Surg., 27:275-289, 1939. 9. Denninger, H. S.: Osteitis fibrosa in a skeleton of a prehistoric American Indian. Arch. Pathol., 11:939-946, 1931. 10. Jones, W. A., Gerrie, J., and'Pritchard, J.: Cherubism: a familial fibrosus dysplasia of the jaws. J. Bone Joint Surg., 32B:334-347, 1950. 11. Ramsay, H. E., Skrong, E. W., and Frazell, E. L,: Fibrous dyspiasia of the craniofaciaI bones. Am. J. Surg., 116:542-547, 1968. 12. Georgiade, N., Masters, F., Herren, C., and Pickrele, K.: Ossifying fibromas (fibzous dysplasia) of the facial bones in children and adolescents, J, Pediatr., 46:3641, 1955. 13. Ward, P. H., Alley, C., and Owen, R.: Monostotic fibrous dysplasia of the maxilla. Laryngoscope, 79:12951306, 1969. 14. Sherman, R. S., and Sternberg, W.'. The roentgen appearance of ossifying fibroma of bone. Radiology, 5 0 : 5 9 5 619, 1948. Harbor-UCLA Medical Center 1000 West Carson Street Torrance, California 90509 (Dr, Canalis}
13 5