Orthodontic treatment in a patient with sickle cell anemia

CASE REPORT
Orthodontic treatment in a patient with sickle cell
anemia
Matheus Melo Pithon
Jequie, Bahia, Brazil
Sickle cell anemia is a common hereditary hematologic disease. It affects many systems and tissues in the body,
including the mouth. Delayed tooth eruption, an uncommon degree of periodontitis, alterations in the cells of the
tongue surface, hypomaturation and hypomineralization in enamel and dentin, pulp calcifications, hypercementosis, and bone alterations resulting in maxillary protrusion and formation of a thick trabecular pattern are some
oral manifestations of the disease. The aim of this study was to report the orthodontic treatment of a patient with
sickle cell anemia. Treatment consisted of correcting a Class II dental and skeletal pattern with an extraoral appliance combined with a fixed orthodontic appliance. From the orthodontic point of view, the results were satisfactory: the occlusion was normalized, and the patient’s health was maintained throughout the entire treatment
period. (Am J Orthod Dentofacial Orthop 2011;140:713-9)
S
ickle cell anemia is one of the most common hereditary hematologic diseases worldwide, and it
is usually considered a matter of public health.1
It is a hereditary type of chronic hemolytic anemia,
caused by a genetic mutation of the hemoglobin A molecule, which is called hemoglobin S.2,3
Hemoglobin is a respiratory protein in the erythrocytes with the primary function of transporting oxygen
throughout the body. Molecular biology explains sickle
cell anemia as a mutation of the hemoglobin that leads
to cells that look like sickles.1,4 In situations of low
oxygen tension, the affected hemoglobin molecules
change from their normal disk shape to sickle shape.
Initially, when the oxygen level increases, this sickling
is reversible; however, the constant changes of shape
of the red blood cells harm their cell membranes,
which become rigid and no longer return to their
normal state. This results in decreased capacity to
transport oxygen to the tissues, vaso-occlusion conditions, and diminished lifespan of the red blood cells.
Professor, Southwest Bahia University UESB, Jequie, Bahia, Brazil; doctor of
orthodontics, School of Dentistry, Federal University of Rio de Janeiro - UFRJ,
Brazil; diplomate of Brazilian Board of Orthodontics and Dentofacial
Orthopedics.
The author reports no commercial, proprietary, or financial interest in the products or companies described in this article.
Reprint requests to: Matheus Melo Pithon, Av. Otavio Santos, 395, sala 705, Centro Odontomedico Dr. Altamirando da Costa Lima, Vit
oria da Conquista, Bahia,
Brazil, CEP: 45020-750; e-mail, [email protected].
Submitted, November 2009; revised and accepted, February 2010.
0889-5406/$36.00
Copyright Ó 2011 by the American Association of Orthodontists.
doi:10.1016/j.ajodo.2010.02.039
These physiopathologic events are the determinant in
the origin of most signs and symptoms in the clinical
condition of patients with sickle cell anemia: algic crises,
high susceptibility to infections, hemolytic crises, ulcers
in the lower limbs, splenic sequestration, priapism,
strokes, and chronic compromise of multiple organs,
systems, or processes.5-7
The clinical spectrum of involvement can vary greatly
from patient to patient. The pathologic effects of sickle
cell anemia, demonstrated in mineralized and connective
tissues in other areas of the body, also occur in oral tissues.1,3 The most common findings described in the
literature involving the oral region are paleness of the
oral mucosa, delayed tooth eruption, uncommon degree
of periodontitis, alterations in the cells of the tongue
surface, hypomaturation and hypomineralization in
enamel and dentin, pulp calcifications, hypercementosis,
and bone alterations, resulting in maxillary protrusion
and formation of a thick trabecular pattern.1,3,5,8
There is no specific treatment for sickle cell anemia,
and it is necessary to include preventive measures to
ameliorate its consequences. These measures include
good nutrition, prophylaxis, early diagnosis of and treatment for infections, maintenance of good hydration,
and avoidance of adverse climate conditions. Allied
with the preventive measures, folic acid is indispensable,
because of its fundamental importance in the maturation and speed of red blood cell production, and should
be prescribed for patients with sickle cell disease. In some
cases, it is associated with vitamin B12.9,10
Orthodontists should have knowledge of the oral
manifestations and possible interferences of sickle cell
713
714
Pithon
Fig 1. Pretreatment photographs.
anemia in orthodontic treatment. Nevertheless, no article describing orthodontic treatment in these patients
was found, only cephalometric evaluations in population groups with this pathology and descriptions of the
effects of this pathology on craniofacial growth. With
the intention of filling this gap, the objective of this article was to describe the clinical orthopedic and orthodontic treatment of a patient with sickle cell anemia,
its particularities, and the care to be taken.
DIAGNOSIS AND ETIOLOGY
The patient, a 12-year-old girl, came for orthodontic
consultation with the chief complaint of misaligned and
protruding teeth (Fig 1). Her mother said that she had
sickle cell anemia and was under the care of a hematologist (Fig 2). According to the mother, the girl took supplements of folic acid associated with vitamin B12,
prescribed by the hematologist. Clinically, she patient
had a good state of general health, without any complications because of this pathology.
For adequate orthodontic treatment, it is indispensable to know the medical history of a patient with sickle
cell disease, as well as his or her family history, to gain
knowledge of the degree of systemic compromise of
the disease.
The patient had an Angle Class II dental relationship,
12 mm of overjet, and an exaggerated anterior overbite
(Fig 3). The gingivae showed discrete paleness, particularly
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Fig 2. Blood smear slide demonstrating the morphology
of the patient’s hemoglobin.
in the posterior region. Enamel hypoplasia was noted on
the maxillary incisors on the right side (Fig 1).
Cephalometrically, the patient had a Class II skeletal
relationship (ANB, 6 ) with maxillary protrusion (SNA,
85 ) and a slightly retruded mandible (SNB, 79 ), a trend
toward a vertical facial growth pattern (SnGoGn, 34 ),
vestibularization and protrusion of the maxillary and
mandibular incisors (1.NA, 30 ; 1-NA, 8 mm; 1.NB,
38 ; 1-NB, 7 mm), and a convex facial profile (LS-S, 4
mm; LI-S, 7 mm) (Figs 4 and 5).
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Fig 3. Pretreatment dental models.
TREATMENT OBJECTIVES
The goals were to correct the skeletal Class II relationship, improve the Class II dental relationship, harmonize
the facial profile, improve the esthetics of the smile, and
maintain the patient’s general health.
TREATMENT ALTERNATIVES
The treatment alternatives were to (1) extract the first
premolars and retract the maxillary anterior teeth; (2) wait
for cessation of craniofacial growth and plan for future
orthodontic-surgical treatment with posterior repositioning of the maxilla and anterior repositioning of the mandible; and (3) correct the skeletal and dental Class II
relationships with a combined extraoral traction appliance.
0.016-, and 0.018-in stainless steel archwires. With the
0.018-in maxillary archwire, retraction of the maxillary
second premolars began to create space for the distalized
molars produced by the extraoral appliance. After this,
the first premolars and the canines were moved distally.
Once the canines were well positioned in a Class I relationship, a 0.019 3 0.026-in maxillary retraction archwire was made with inverted drop-shaped loops, to
achieve retraction of the maxillary anterior teeth.
When retraction was achieved, 0.019 3 0.026-in
ideal arches were made to conclude the orthodontic
treatment. Then the appliances were removed, with every care to control transitory bacteremia (antibiotic prophylaxis). After this, a mandibular canine-to-canine
bonded lingual bar and a maxillary circumferential retainer were given to the patient.
TREATMENT PROGRESS
The treatment plan chosen was the use of an extraoral appliance combined with a fixed orthodontic appliance. Initially, orthodontic bands were placed on the
maxillary first molars, followed by fitting the extraoral
traction appliance. After this, the fixed orthodontic
brackets (0.022 3 0.030-in brackets; Morelli, Sorocaba,
Brazil) were bonded to the remaining teeth. All banding
appointments were preceded by antibiotic prophylaxis
with 2 g of amoxicillin 1 hour before the procedure.
After the appliance was fitted, the dental alignment
and leveling stage began, with a sequence of 0.014-,
TREATMENT RESULTS
The results obtained with orthopedic-orthodontic
treatment were the correction of the dental and skeletal
Class II malocclusion with reduction of maxillary protrusion and an improved relationship between the bony bases (ANB, 4 ). The facial profile improved with the
reduction in labial protrusion (LS-S, 13 mm; LI-S, 14
mm) and the passive lip seal at rest (Figs 6-10).
The maxillary incisors were better positioned in the
bony base of the maxilla (1.NA, 15 mm; 1-NA, 3 mm).
There was a discrete increase of the mandibular plane
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Fig 5. Pretreatment cephalometric tracing.
Fig 4. Pretreatment radiographs.
in a clockwise direction, which contributed to a slight increase in the lower third of the face (54%).
DISCUSSION
Sickle cell anemia is a common hereditary hematologic diseases worldwide, affecting a significant portion
of the Brazilian population. It is usually pointed out as
a question of public health. Thus, the objective of this article was to describe the orthodontic treatment of a patient with sickle cell anemia.
The oral manifestations in patients with sickle cell
anemia are not pathognomonic of the disease but could
help the dentist to identify it. The paleness of the oral
mucosa is the result of chronic anemia or jaundice resulting from hemolysis of the red blood cells. In this patient, paleness of the mucosa was not noted, since she
was under the constant care of a hematologist, thus preventing any anemic condition. Nor was any delay in
tooth eruption seen. Nevertheless, there were defects
in enamel mineralization affecting the crowns of the
maxillary central and lateral incisors (Fig 1).
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According to Franco et al,1 certain characteristics are
found on the periapical radiographs of subjects with
sickle cell anemia, including the formation of a thick trabeculated pattern, attributed to the erythroblastic hyperplasia and medullary hypertrophy that result in loss
of the thin trabeculated bone and formation of wide
medullary spaces. There is greater alteration in alveolar
bone trabeculation, and the stepladder trabecular pattern is found more frequently in the posterior teeth.
These characteristics of thick trabeculated bone were
seen in this patient, particularly in the mandibular premolar region (Figs 4 and 8). The evident bone
alterations of sickle cell anemia alert one to the
importance of the radiographic examination as an aid
in diagnosis of the disease.
Although the oral manifestations are not pathognomonic of the disease, the cephalometric alterations of the
bone tissues are characteristic. The process of red blood
cell sickling results in a lower capacity of oxygen transport to the tissues, circulatory difficulties, and a reduction
in their lifespan, which falls from 120 days to approximately 20 days, when they are removed from circulation
by the spleen. The early destruction of red blood cells
makes it necessary for them to be constantly produced,
causing hyperplasia and compensatory bone marrow expansion, which might result in changes in the bony structures that can be observed radiographically.11
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Fig 6. Posttreatment photographs.
The gnathopathy resulting from the exaggerated
growth of the midface was first described by Brown and
Sebes12 who observed expansion of the maxilla and protrusion of the middle third of the face in patients with
sickle cell anemia. The author attributed this fact to medullary hyperplasia and found maxillary protrusion in 31.6%
of anemic patients in Ghana.12 These skeletal alterations
were seen in the patient of this report. Cephalometrically,
she had a Class II skeletal relationship with maxillary protrusion (SNA, 85 ) and slight mandibular retrusion (SNB,
79 ) (Fig 5). These findings agree with the cephalometric
measurements for Brazilians with sickle cell anemia
(SNA, 85 ; SNB, 78 ) found by Souza et al3 in 2008.
The issues in treating these patients are concentrated
on avoiding bleeding during orthodontic procedures. If
it is not possible to prevent bleeding, the procedures
should be performed under prophylactic antibiotic therapy. Habitual orthodontic procedures that might generate some type of bacteremia would be the placement and
removal of orthodontic separators and orthodontic miniplates. Therefore, every orthodontic separation procedure was preceded by antibiotic prophylaxis with 2 g
of amoxicillin, as suggested by Franco et al.1 The use
of mini-implants was not necessary because an extraoral
appliance was used to correct the skeletal Class II relationship.
The choice of treatment was strictly related to the pathology; ie, any more aggressive procedure was avoided.
The option was to correct the Class II malocclusion with
an extraoral appliance instead of extractions, miniimplants, or orthognathic surgery.
Our choice of treatment without tooth extractions
was necessary because extraction of asymptomatic teeth
is contraindicated in patients with sickle cell anemia. In
these patients, teeth should be extracted only when it is
impossible to perform adequate restorative procedures.
Special care was taken throughout the treatment period, which included periodic consultations with the
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Fig 7. Posttreatment dental models.
hematologist, strict control of oral hygiene, and antibiotic prophylaxis during all procedures that could lead
to a bacteremic condition. Other concerns would be to
avoid treatment during the chronic stage of the disease
and avoid appointments during algic crises, except in
cases of emergency.
The major cause of death in patients with sickle cell
anemia is infection.1 Pneumonia, renal infection, and
osteomyelitis occur with greater frequency in children
and adults with sickle cell anemia, caused by the functional splenectomy resulting from continuous fibrosis
of this organ over the years.13 Episodes of fever must
be regarded as situations of risk, requiring immediate
therapy.4 Oral surgeries are invasive procedures involving high risk; therefore, they must be judiciously planned
so that the intervention will be safe. Susceptibility to infection justifies the use of prophylactic antibiotic therapy before invasive procedures that could cause
bleeding and promote bacteremia. When surgery is necessary, the dental surgeon must request a complete hemogram of the patient.
CONCLUSIONS
Performing orthodontic treatment on a patient with
sickle cell anemia is a reality, requiring special care
from both the local and the systemic points of view. Because we took special care, our results were satisfactory,
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with correction of the malocclusion and maintenance
the patient’s general health.
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3. Souza PHG, Oliveira RSF, Rocha JM, Gravina MA, Vitral RWF. Craniofacial skeletal alterations in sickle cell anemia patients of Juiz
de Fora. HU Revista 2008;34:85-91.
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Fig 9. Posttreatment cephalometric tracing.
Fig 8. Posttreatment radiographs.
11. Mourshed F, Tuckson CR. A study of the radiographic features of
the jaws in sickle-cell anemia. Oral Surg Oral Med Oral Pathol
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12. Brown DL, Sebes JI. Sickle cell gnathopathy: radiologic assessment. Oral Surg Oral Med Oral Pathol 1986;61:653-6.
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and costs of care of patients with sickle-cell anemia in the state of
Maryland in the era of hydroxyurea. Am J Hematol 2006;81:927-32.
Fig 10. Superimposed cephalometric tracings.
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