DENTAL AND ORAL FINDINGS IN COOLEY’S ANEMIA:

A STUDY OF FIFTY CASES

Robert I. Kaplan
School of Dentistry, University of Penmylvania and Children’s
Hospital of Philadelphia, Pkiladslphia, Pa.
Raymond Werther
Pedodontic Clinic, School of Dentistry, University of Pennsylvania
and Divisions of Dentistry and Orthodontics, Children’s Hospital
of Philadelphia, Philadelphia, Pa.
Francis A. Castano
Children’s Hospital of Philadelphia, Philadelphia, Pa.

Fifty patients with Cooley’s anemia (thalassemia major) were studied

a t Children’s Hospital of Philadelphia, by means of clinical dental examina-
tions, roentgenographs, dental study models and photographs. Observa-
tions were made regarding maxillary deformities, the prevalence of dental
caries and gingival inflammation.
The individuals studied ranged in age from three to 28 years. They
shaved dental and oral changes of varying degrees of severity, from normal
to grossly abnormal.
The most noticeable changes that take place in the oral structures are
associated with maxillary enlargement. The maxilla is composed mainly of
cancellous bone containing marrow spaces which are a part of the hema-
topoietic system. Under the influence of the disorder, these spaces and the
entire maxilla go through a progressive enlargement producing the typical
facial appearance, with high and bulging cheek bones, retraction of the
upper lip, protrusion of the anterior teeth, spaces between the teeth, over-
bite, or open bite and varying degrees of malocclusion. In severe cases, the
maxillary tuberosity areas become widened and bulbous. The dentofacial
appearance has been described as having a more of a “rodent” than a
“mongoloid” cast. Baty’ remarks that patients with thalassemia major re-
semble each other more than they resemble the other members of their
own families. However, not all patients demonstrate these changes. In this
survey, 32 (64 per cent) showed maxillary enlargement. Eighteen (36 per
cent) had deep overbite; 15 (30 per cent) showed an open-bite condition,
and four (8 per cent) had some degree of cross bite. Twenty-five (50 per
cent) showed more than one dentofacial deformity, and 10 (20 per cent)
had apparently normal occlusion.
The mandible ordinarily undergoes less enlargement than the maxilla.
If the patient survives to adolescence, Cohen’ states that there is usually

664
 Kaplan et al. : Dental and Oral Findings
gome regression of the bony overgrowth and a more normal facial appear-
ance may return.
Dental defects in some patients are quite severe. Venturai theorizes that
the endocrine dysfunction caused by thalassemia is responsible for an in-
crease in dental caries prevalence, although such an increase has not been
evident in the majority of cases observed in this study. Dental caries in
these patients appears to be associated primarily with dental neglect. The
parents are more concerned with the serious physical problems, paying
lesser attention to the dental ailments, and only seek dental care when the
child is in pain. On the other hand, many of the patients were receiving
routine dental care and had teeth in good state of repair.
DMF studies (decayed, missing and filled teeth) showed that in the 50
persons observed there was a total of 110 carious primary teeth (average
2.2 per patient), 125 carious permanent teeth (average 2.5) 26 missing
permanent teeth (average 0.5) 38 filled primary teeth (0.75 average) and
126 filled permanent teeth (average 2.5 per patient). There were also 10
patients (20 per cent) who showed teeth in perfect condition with no carious
areas and no restorations. The overall DMF rate was 8.3 per patient. A
comparable study done on 5,663 children of military personnel at Fort Dix
and McGuire Air Base, ages 5 to 18 years, disclosed a DMF rate of 7.0
per child.
The oral mucosa frequently displays a characteristic pallor in thalas-
semia major. The gingival tissues may appear thin and pale, but are often
otherwise normal. The incidence of gingivitis in children with the disease
seems likely related to local factors such as poor mouth hygiene, maloc-
clusion and drying of the gingivae through the patient’s inability to close
his mouth over the protruding teeth. The chronic anoxemia may in some
cases predispose to gingival disorders.
In the 50 cases studied, 16 (or 32 per cent) showed some degree of gin-
gival inflammation, five (10 per cent) had one or more periodontal pockets,
five (10 per cent) showed supra and subgingival calculus and two (4 per
cent) showed only supragingival calculus. Where the bite relationship was
normal, only one case showed gingival inflammation.
Roentgenographically the maxilla and mandible showed a coarsened
trabecular pattern in 43 cases (86 per cent), with increased radiolucency,
enlarged marrow spaces and thinning of the cortical layer of bone.
Summarg
A study of 50 patients with Cooley’s anemia shows a high prevalence
(64 per cent) of maxillary deformity, and a dental defect rate (8.3 per
patient) slightly higher than a comparable group of persons (7.0). Thirty-
two per cent showed some degree of gingival inflammation.
666 Annals New York Academy of Sciences
Although the maxillary deformity was considered to be a concomitant
of the disease, the dental defects and gingival inflammation were thought
to be not directly related to it.
Acknowledgments
Grateful appreciation is expressed to Dr. Irving R. Wolman, Chief of the
Department of Hematology, Children’s Hospital of Philadelphia for the
opportunity to engage in this study.
References
1. BATY,J. M., K. D. BLACKFAN & L. K. DIAMOND.1932. Blood studies in in-
fants and children, erythioblastic anemia. Am. J. Dis. Child. 43: 667.
2. COHEN,M. M. & J. M. BATY.1945. Oral manifestations of eiythroblastic
anemia. J.A.D.A. 32: 1396.
3. VENTURA, B. 1965. Cooley’s anemia: Its dental aspects. Clin. Odontoiatr
(Roma) . l o : 342350. (D. Abs. 1 :148.1956).