Cooley’s anemia-Orthodontic surgical treatment
and
George Silting, D.D.S.,* and Stephen J. Moss, D.D.S., M.S.** New York. N. Y.
T
here are some disease entities which have important orthodontic implications but which are rarely seen in the orthodontic practice. Consequently, little attention is paid to them. Nevertheless, it is important to record whatever experience is accumulated regarding orthodontic diagnosis and treatment for such conditions. Cooley’s anemia (thalassemia, erythroblastic anemia, or Mediterranean anemia) is a blood dyscrasia characterized by a peculiar alteration in skull and long bone structure which produces a Mongoloid facial appearance (Figs. 1 and 2). Other clinical manifestations are icterus (jaundice) and enlargement of the spleen and heart. The disease is congenital and affects almost exclusively persons of Mediterranean extraction, chiefly Greek or Italian. In severe cases the maxilla is grossly enlarged and the malar bones bulge markedly. These bony changes are related to the enlargement of marrow spaces in response to a stimulus for increased production of red cells. Radiographically, these patients exhibit enlarged marrow spaces in the jaws and thinning of the cortical layers (Fig. 3). There is a severe retardation of maturation, which is observable in carpal radiographs, and a widening of the diploic spaces in the skull. As a result of the active marrow hyperplasia, pneumatization of the maxillary sinuses fails to occur at the proper time and there is considerable overgrowth of the maxilla. This causes severe overbite, prominence of the upper incisor teeth, and separation of the orbits.’ The trabeculae of the thickened diploic spaces often become arranged vertically, giving the “hair-on-end” appearance described in the literature. Preventive procedures are now being developed and appear promising. According to one investigator,* the facial disfigurement seen in these patients can be prevented by periodic transfusion therapy, as long as the hemoglobin is kept at an optimal level and therapy is started by the fifteenth month of life. Kaplan and associates3 described dental and oral findings in a study of fifty cases; 64 percent of the patients showed the maxillary deformities previously described, but the DMF rate and gingival inflammation were not unusual. Johnston and Krogman4 studied the patterns of growth of fifty persons with Cooley’s anemia. They concluded that the growth patterns of these children were characterized by a retardation in normal growth *Clinical Professor of Orthodontics, New York University **Professor of Pedodontics and Head, Division of Growth College of Dentistry.
444
College of Dentistry. and Development, New York
0002.9416/78/100444+06$00.60/0
0
University
1978 The C. V. Mosby Co.
Volume 74 Number 4
Cooley’s anemia
445
Fig. 1. Patient C. T., a 17-year-old girl.
Fig. 2. Patient R. C., a W-year-old
boy.
expectations as well as in their rates of growth. A.4~11,~ in a study of the orthodontic aspects of the disease in fifteen patients, noted that at about the age of 8 to 10 years, there is midfacial overgrowth accompanied by incisal prominence and shortness of the mandibular corpus. Asbell comments that “. . . the reaction to pressure brought about by orthodontic appliances has not been fully tested, nor are there any clinical results re-
Am .I. Orrhod. October I978
Fig. 3. Lateral spacing.
plate
radiograph
of Patient
C. T. Note
abnormal
height
of the corpus
and intertrabecular
Table I Lundmark NP-FH N-a-P SN-GoGn SGn-FH S-N-a S-N-b Diff. T to1 it0aP
FMA IMPA FMIA
Patient 79 28 41 71 84 71 13 121 9mm. 39 105 36
C. T.
Patient
R C.
78 26 49 71 79 68 11 140 9 mm. 40 98 42
ported. ’ ’ A search of the dental literature has failed to reveal any clinical orthodontic reports since Asbell’s paper. Case reports The orthodontic experience with two cases are reported here. The similarities in facial appearances of these patients were startling (Figs. 1 and 2). Another investigator has commented that children with this affliction resemble each other more than they do members of their own families.6 Our cephalograms revealed the classic symptoms of enlarged marrow spaces, overgrowth of the jaws, and procumbence of the anterior alveolar bone (Fig. 4). While cephalometric interpretations appear to have little value in the treatment of these children, it is interesting to note some of the values obtained on analysis (Table I and Fig. 5). Dental occlusion in both cases was similar, with prominent and crowded upper an-
Volume 74 Number 4
Cooley’s
ram of Patient C. T. 13, CqWbgram
anemia
447
of PHieM Ft.
terior teeth and labially positioned canines. Posteriorly, there was an excess of space. In both cases function was satisfactory, and it was thought that the thrust of treatment should be aimed at the improvement of facial and dental appearance. In view of the frequent periods of hospitalization and other medical treatment needed by these patients, it was thought best to limit the orthodontic procedures to the anterior segments. In Patient C. T., a 17-year-old girl of Greek extraction, the prominent upper canines appeared to add measurably to the bizarre facial appearance. It was decided that the canines should be removed. In addition, the oral surgeon was asked to reduce the bony area above these teeth at the time of the extractions in an effort to reduce the prominence in the area of the canine eminences. Orthodontic procedures were then instituted with a Begg appliance in the upper arch. Tooth movement proceeded normally and at a normal rate of speed, with no untoward clinical effects. Soft tissues also responded normally. Treatment lasted 3 months, with marked improvement of the dental esthetics. In Patient R. C., a 12-year-old boy of Italian parentage, an effort was again made to
Am. J. Or&d. Octobw 1978
Fig.
5. A, Cephalometric
Fig. 6. A, Anterior after treatment.
teeth
tracing
of Patient
of Patient
FL C. before
C. T. B, Cephalometric
treatment.
Note
bulging
tracing
of malar
of Patient
bones.
R. C.
8, Patient
R. C.
Volume 14 Number 4
Cooley’s
anemia
449
improve the appearance of the anterior teeth. Although no teeth were extracted in this case, oral surgery was performed to reduce the alveolar bone above the canine and premolar teeth. This patient was treated for 12 months with a Begg appliance. Tooth movement proceeded normally, again with no unusual effects noted. Frequent interruptions of treatment for hospital procedures, not difficulties related to orthodontic tooth movement, were the main cause of slow progress. The dental appearance showed improvement (Fig. 6). Discussion The facial appearance of children with Cooley’s anemia is readily recognizable and is accompanied by grossly procumbent anterior teeth. Orthodontists, pedodontists, and other dental practitioners are obliged to use whatever clinical approaches are available in order to reduce the severe emotional trauma which these children must sustain during childhood and adolescence because of their appearance. It is hoped that modem transfusion techniques will reduce the skull and facial deformities caused by this disease. In two cases orthodontic treatment with multibanded appliances proceeded normally, with no untoward effects in rate of tooth movement or soft-tissue response. Surgical reduction of the areas above the canines helped to improve dental esthetics. REFERENCES 1. Baker, D. H.: Roentgen manifestations of Cooley’s anemia, Ann. N. Y. Acad. Sci. 119: 641-661, 1964. 2. Piomelli, S., Karpatkin, M. H., et al.: Hypertransfusion therapy for patients with Cooley’s anemia, Ann. N. Y. Acad. Sci. 232: 186-192, 1974. 3. Kaplan, R. I., Werther, R., and Castano, F. A.: Dental and oral findings in Cooley’s anemia: A study of fifty cases, Ann. N. Y. Acad. Sci. 119: 664-666, 1964. 4. Johnston, F. E., and Krogman, W. M.: Patterns of growth in children with thalassemia major, Ann. N. Y. Acad. Sci. 119: 667-679, 1964. 5. Asbell, M. B.: Orthodontic aspects of Cooley’s anemia, Ann. N. Y. Acad. Sci. 119: 662-663, 1964. 6. Baty, J. M., Blackfan, K. D., and Diamond. L. K.: Blood studies in infants and children, erythroblastic anemia, Am. J. Dis. Child. 43: 667, 1932.
and
George Silting, D.D.S.,* and Stephen J. Moss, D.D.S., M.S.** New York. N. Y.
T
here are some disease entities which have important orthodontic implications but which are rarely seen in the orthodontic practice. Consequently, little attention is paid to them. Nevertheless, it is important to record whatever experience is accumulated regarding orthodontic diagnosis and treatment for such conditions. Cooley’s anemia (thalassemia, erythroblastic anemia, or Mediterranean anemia) is a blood dyscrasia characterized by a peculiar alteration in skull and long bone structure which produces a Mongoloid facial appearance (Figs. 1 and 2). Other clinical manifestations are icterus (jaundice) and enlargement of the spleen and heart. The disease is congenital and affects almost exclusively persons of Mediterranean extraction, chiefly Greek or Italian. In severe cases the maxilla is grossly enlarged and the malar bones bulge markedly. These bony changes are related to the enlargement of marrow spaces in response to a stimulus for increased production of red cells. Radiographically, these patients exhibit enlarged marrow spaces in the jaws and thinning of the cortical layers (Fig. 3). There is a severe retardation of maturation, which is observable in carpal radiographs, and a widening of the diploic spaces in the skull. As a result of the active marrow hyperplasia, pneumatization of the maxillary sinuses fails to occur at the proper time and there is considerable overgrowth of the maxilla. This causes severe overbite, prominence of the upper incisor teeth, and separation of the orbits.’ The trabeculae of the thickened diploic spaces often become arranged vertically, giving the “hair-on-end” appearance described in the literature. Preventive procedures are now being developed and appear promising. According to one investigator,* the facial disfigurement seen in these patients can be prevented by periodic transfusion therapy, as long as the hemoglobin is kept at an optimal level and therapy is started by the fifteenth month of life. Kaplan and associates3 described dental and oral findings in a study of fifty cases; 64 percent of the patients showed the maxillary deformities previously described, but the DMF rate and gingival inflammation were not unusual. Johnston and Krogman4 studied the patterns of growth of fifty persons with Cooley’s anemia. They concluded that the growth patterns of these children were characterized by a retardation in normal growth *Clinical Professor of Orthodontics, New York University **Professor of Pedodontics and Head, Division of Growth College of Dentistry.
444
College of Dentistry. and Development, New York
0002.9416/78/100444+06$00.60/0
0
University
1978 The C. V. Mosby Co.
Volume 74 Number 4
Cooley’s anemia
445
Fig. 1. Patient C. T., a 17-year-old girl.
Fig. 2. Patient R. C., a W-year-old
boy.
expectations as well as in their rates of growth. A.4~11,~ in a study of the orthodontic aspects of the disease in fifteen patients, noted that at about the age of 8 to 10 years, there is midfacial overgrowth accompanied by incisal prominence and shortness of the mandibular corpus. Asbell comments that “. . . the reaction to pressure brought about by orthodontic appliances has not been fully tested, nor are there any clinical results re-
Am .I. Orrhod. October I978
Fig. 3. Lateral spacing.
plate
radiograph
of Patient
C. T. Note
abnormal
height
of the corpus
and intertrabecular
Table I Lundmark NP-FH N-a-P SN-GoGn SGn-FH S-N-a S-N-b Diff. T to1 it0aP
FMA IMPA FMIA
Patient 79 28 41 71 84 71 13 121 9mm. 39 105 36
C. T.
Patient
R C.
78 26 49 71 79 68 11 140 9 mm. 40 98 42
ported. ’ ’ A search of the dental literature has failed to reveal any clinical orthodontic reports since Asbell’s paper. Case reports The orthodontic experience with two cases are reported here. The similarities in facial appearances of these patients were startling (Figs. 1 and 2). Another investigator has commented that children with this affliction resemble each other more than they do members of their own families.6 Our cephalograms revealed the classic symptoms of enlarged marrow spaces, overgrowth of the jaws, and procumbence of the anterior alveolar bone (Fig. 4). While cephalometric interpretations appear to have little value in the treatment of these children, it is interesting to note some of the values obtained on analysis (Table I and Fig. 5). Dental occlusion in both cases was similar, with prominent and crowded upper an-
Volume 74 Number 4
Cooley’s
ram of Patient C. T. 13, CqWbgram
anemia
447
of PHieM Ft.
terior teeth and labially positioned canines. Posteriorly, there was an excess of space. In both cases function was satisfactory, and it was thought that the thrust of treatment should be aimed at the improvement of facial and dental appearance. In view of the frequent periods of hospitalization and other medical treatment needed by these patients, it was thought best to limit the orthodontic procedures to the anterior segments. In Patient C. T., a 17-year-old girl of Greek extraction, the prominent upper canines appeared to add measurably to the bizarre facial appearance. It was decided that the canines should be removed. In addition, the oral surgeon was asked to reduce the bony area above these teeth at the time of the extractions in an effort to reduce the prominence in the area of the canine eminences. Orthodontic procedures were then instituted with a Begg appliance in the upper arch. Tooth movement proceeded normally and at a normal rate of speed, with no untoward clinical effects. Soft tissues also responded normally. Treatment lasted 3 months, with marked improvement of the dental esthetics. In Patient R. C., a 12-year-old boy of Italian parentage, an effort was again made to
Am. J. Or&d. Octobw 1978
Fig.
5. A, Cephalometric
Fig. 6. A, Anterior after treatment.
teeth
tracing
of Patient
of Patient
FL C. before
C. T. B, Cephalometric
treatment.
Note
bulging
tracing
of malar
of Patient
bones.
R. C.
8, Patient
R. C.
Volume 14 Number 4
Cooley’s
anemia
449
improve the appearance of the anterior teeth. Although no teeth were extracted in this case, oral surgery was performed to reduce the alveolar bone above the canine and premolar teeth. This patient was treated for 12 months with a Begg appliance. Tooth movement proceeded normally, again with no unusual effects noted. Frequent interruptions of treatment for hospital procedures, not difficulties related to orthodontic tooth movement, were the main cause of slow progress. The dental appearance showed improvement (Fig. 6). Discussion The facial appearance of children with Cooley’s anemia is readily recognizable and is accompanied by grossly procumbent anterior teeth. Orthodontists, pedodontists, and other dental practitioners are obliged to use whatever clinical approaches are available in order to reduce the severe emotional trauma which these children must sustain during childhood and adolescence because of their appearance. It is hoped that modem transfusion techniques will reduce the skull and facial deformities caused by this disease. In two cases orthodontic treatment with multibanded appliances proceeded normally, with no untoward effects in rate of tooth movement or soft-tissue response. Surgical reduction of the areas above the canines helped to improve dental esthetics. REFERENCES 1. Baker, D. H.: Roentgen manifestations of Cooley’s anemia, Ann. N. Y. Acad. Sci. 119: 641-661, 1964. 2. Piomelli, S., Karpatkin, M. H., et al.: Hypertransfusion therapy for patients with Cooley’s anemia, Ann. N. Y. Acad. Sci. 232: 186-192, 1974. 3. Kaplan, R. I., Werther, R., and Castano, F. A.: Dental and oral findings in Cooley’s anemia: A study of fifty cases, Ann. N. Y. Acad. Sci. 119: 664-666, 1964. 4. Johnston, F. E., and Krogman, W. M.: Patterns of growth in children with thalassemia major, Ann. N. Y. Acad. Sci. 119: 667-679, 1964. 5. Asbell, M. B.: Orthodontic aspects of Cooley’s anemia, Ann. N. Y. Acad. Sci. 119: 662-663, 1964. 6. Baty, J. M., Blackfan, K. D., and Diamond. L. K.: Blood studies in infants and children, erythroblastic anemia, Am. J. Dis. Child. 43: 667, 1932.
