Anemia Falciforme

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Oral complications associated with sickle cell anemia 
A review and case report 
Martin Kelleher, BDS, MSc, FDSRCPS, a 
Karl Bishop, BDS, MScD, DRD, MRD, FDSRCS, b and 
Peter Briggs, BDS, MSc, MRD, FDSRCS, ~ London and Sheffield, U.K. 
KINGS COLLEGE DENTAL HOSPITAL AND CHARLES CLIFFORD DENTAL HOSPITAL 
Sickle cell anemia is a multisystem disease that can affect the oral region. This article reviews previously 
reported oral complications associated with this disease and describes the cause of these problems. A case history 
describes a patient with multiple oral problems after a sickle cell crisis. The diagnostic and management problems of this 
case are highlighted. The need for a multidisciplinary approach to the care of these patients is emphasized. (Oral Surg 
Oral Med Oral Pathol Oral Radiol Endod 1996;82:225-8) 
Sickle cell anemia (SCA) is an inherited multisystem 
disease caused by a defect in chromosome II. If only 
one of the pair of chromosomes is affected then sick- 
le-cell trait develops while SCA reflects the involve- 
ment of both chromosomes. 1 The defect results in an 
amino acid substitution in the [3-hemoglobin chain of 
red blood cells (RBCs). 
Although Afro-Caribbeans are predominantly af- 
fected. Mediterranean, Middle Eastern, and East In- 
dian persons may also be at risk. a In the United States. 
1 in 400 of African-Americans are affected by SCA. 
In areas where malaria is endemic, for example in 
West Africa, up to 30% of persons may be affected) 
The clinical problems associated with SCA are at- 
tributed directly to the defective RBCs. These in- 
clude: hemolytic anemia, bacterial infections, and 
painful vast-occlusive crises. 
HEMOLYTIC ANEMIA AND BACTERIAL 
INFECTIONS 
The deformed cells are recognized as abnormal by 
the reticuloendothelial system and are destroyed. If 
this occurs at a faster rate than new cells can be pro- 
duced, a hemolytic anemia develops. Hyperplasia and 
wadening of bone marrow spaces may occur as the 
body increases hematopoiesis in an attempt to main- 
tain the number of blood cells. 4 The patient may also 
be more prone to infection as macrophages are 
aConsultant in Restorative Dentistry, Kings College Dental Hos- 
pital, London. 
bSenior Registrar in Restorative Dentistry, Charles Clifford Den- 
tal Hospital. Sheffield. 
cConsultant in Restorative Dentistry, Kings College Dental Hos- 
pital. London. 
Received for publication Feb. 14. 1996: returned for revision Mar. 
14. 1996: accepted for publication Apr. 8. 1996. 
Copyright �9 1996 by Mosby-Year Book. Inc. 
1079-2104/96/$5.00 + 0 7/15/74195 
involved in phagocytosis of the defective RBCs and 
may not be available for killing bacteria. 5 Other fea- 
tures of SCA, such as leucocyte defects and hypos- 
plenism, may also cause the patient to be immuno- 
compromised. 5 
PAINFUL VASO-OCC LUSIVE CRISES 
The distorted cells may also occlude the microvas- 
culature and impede bloodflow to an area. This can 
cause tissue anoxia, infarcts, necrosis, and pain. 6 
Clinically, the anoxia results in a sickle cell crisis 
(SCC). Severe pain is experienced for 1 to 2 weeks. 
often accompanied by a low-grade fever and leuco- 
cytosis. 3 An SCC may be precipitated by a number of 
factors including infection, dehydration, acidosis, 
trauma, and strenuous exercise)" 5 
ORAL COMPLICATIONS 
Oral problems have been described, but these are 
not as common as other complications. 4-9 Those that 
have been recorded include: manidbular osteomyeli- 
tis. anesthesia of the mandibular nerve, and asymp- 
tomatic pulpal necrosis. 
Mandibular osteomyelitis 
Studies 5, 6, 9 indicate that osteomyelitis occurs sev- 
eral hundred times more frequently in SCA patients 
than in the rest of the population and that up to 29% 
of SCA patients experience at least one episode of 
osteomyelitis during their lifetime. 1~ 11 Although 
sickle cell osteomyelitis is more common in long 
bones, it can affect facial bones. The mandible is par- 
ticularly at risk because of a relatively poor blood 
supply.5, 6 Intravascular sickling is thought to lead to 
an ischemic infarction and necrosis of bone that cre- 
ates a favorable environment for bacterial growth. 
Oral flora may then invade the area either via the pe- 
riodontal ligament or by hematogenous spread. 5" 9 
225 
226 Kelleher, Bishop, andBriggs ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY 
August 1996 
Fig. 1. Pan•ramicradi•graphatinitialpresentati•n.N•telack•frest•rati•nsandmultil•cularradi••ucency 
in anterior mandible. 
Anesthesia of the mandibular nerve 
Permanen t neuropathies affect ing the infer ior den- 
tal nerve after an SCC have been repor ted and resul ted 
in pers is tant anesthes ia for up to 24 months. 7, s The 
loss o f sensat ion is thought to be caused by an infarc- 
t ion o f the mic rovascu la r b lood supply to the infer ior 
denta l nerve or its branches. The infer ior dental nerve 
m a y be par t icular ly vulnerable because it passes 
through a nar row b o n y canal. 7, 8 
Asymptomatic pulpal necrosis 
A n d r e w s et al. 4 obse rved asymptomat i c pulpal ne- 
crosis in o therwise hea l thy teeth in pat ients with SCA. 
They repor ted that this was more c o m m o n in pat ients 
wi th S C A and sugges ted that the s ickled cells caused 
a b l o c k a g e of the b lood vessels supply ing the pulp, 
which resul ted in necrosis . S ickle cel ls have also been 
ident i f ied in teeth wi th a his tory of repeated episodes 
of pu lpa l pain. 4 
Repor ts on oral compl ica t ions have descr ibed sin- 
gle read i ly d iagnosed problems. However , this art icle 
descr ibes a pat ient wi th S C A who deve loped mult i - 
p le oral compl ica t ions and highl ights the diff icul t ies 
in d i agnos i s and managemen t in such circumstances. 
CASE REPORT 
A 41-year-old Ghanaian man was referred to the hospi- 
tal services by his general dental practitioner (GDP) after 
developing oral problems after a recent SCC. The patient 
was unaware of any oral problems before the SCC. 
The initial examination revealed a tenderness over the 
right body of the mandible together with a unilateral anes- 
thesia affecting the lower lip on the same side. Pus was also 
discharging from the periodontal ligament around the par- 
dally erupted right mandibular third molar. The patient was 
afebrile, and there was no evidence of disturbance of the 
central nervous system. The dentition was well maintained 
with good oral hygiene and minimal loss of periodontal at- 
tachment. The findings were in agreement with the GDP's 
observations except that the GDP had also observed a grade 
II mobility associated with the right mandibular first mo- 
lar. 
Radiographic examination (Fig. 1) revealed a widening 
of the periodontal ligament at the apices of the right first 
and second premolar and first molar and a radiolucent area 
associated with the furcation of that first molar. A well-de- 
fined mulfilocular radiolucency in the body of the mandi- 
ble was also noted that extended from the fight first 
premolar to the left second molar. Because all teeth were 
asymptomatic and unrestored, the radiographic features 
were considered normal for a patient with SCA. The loss 
of sensation was thought to be due either to an infarct a 
consequence of the SCC) or possibly pericoronitis. A diag- 
nosis of acute pericoronitis was made and the right man- 
dibular third molar subsequently extracted under local an- 
esthesia. 
Despite the extraction, the patient continued to experi- 
ence discomfort from the right mandibular region. This was 
attributed to delayed healing after the extraction and was 
treated with local measures and systemic antibiotics. Un- 
fortunately, the patient's problems persisted until he re- 
turned to his GDP 4 months later. Considering the history, 
the clinical findings and the previous radiographic appear- 
ances, the GDP concluded that the pulps of the right man-dibular first premolar through to the second molar were 
necrotic. This was confirmed by preparing test cavities after 
which root canal therapy was commenced. The GDP clinically 
confirmed the presence of necrotic pulps in all the teeth 
investigated. The start of root canal therapy resulted in imme- 
diate relief of the symptoms. An panoramic radiograph (Fig. 
2) taken after the preparation of test cavities revealed an ill- 
defined radiolucency in the fight body of the mandible. The 
lesion extended to the apices of first and second molar and 
second premolar and into the furcation of first molar. The le- 
sion appeared quite distinct from the multilocular radiolu- 
cency in the body of the mandible. The apical radiolucency 
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY Kelleher, Bishop, and Briggs 2 2 Z 
Volume 82, Number 2 
Fig. 2. Four months after initial presentation. Note ill-defined radiolucency in right mandible in region of 
first and second molar and second premolar, which extends into the furcation of first molar. Also note loss 
of lamina dura at the apices of first and second molar. Radiolucencies associated with apex of second pre- 
molar and furcation of first molar have increased in size. 
Fig. 3. Ten months after initial presentation. 
associated with the apex of the second premolar and the 
furcation of the second molar also appeared to have 
increased in size when compared with the radiograph taken 
4 months earlier (Fig. 2). 
After completion of root canal therapy the apical 
radiolucencies associated with these three teeth decreased 
in size (Fig. 3). The furcation lesion at the second molar 
also appeared to have resolved. During this period the pa- 
tient also developed acute pain in the left maxillary canine, 
which was accompanied by radiographic changes. As a re- 
sult, this tooth was also root treated. 
One year after the initial presentation, the patient was still 
asymptomatic although sensation in the lower lip had not 
returned. He was advised to continue visiting his GDP for 
his routine care and arrangements were made for a 6-month 
review in the hospital services. He was instructed to return 
earlier if problems developed. 
DISCUSSION 
Al though previous reports of oral p rob lems associ- 
ated with S C A have been descr ibed, in each case a 
single compl ica t ion has occurred in one person. 4-9 
This case highl ights the diagnost ic p rob lems when 
mul t ip le oral p rob lems deve lop in the same person. 
The pa t ien t ' s symptoms sugges ted the presence of 
an acute infect ion and an init ial d iagnos is of per i- 
coronit is associa ted with the r ight mand ibu la r third 
molar was made. Because S C A pat ients are suscep- 
t ible to infection, the cont inued p rob lems after the 
extract ion were thought to be caused by a postextrac- 
t ion osteit is . This is a reasonable conclus ion, but the 
symptoms were not re l i eved by convent iona l therapy. 
This should have made the cl inicians quest ion both 
the ini t ial and subsequent diagnoses . 
228 Kelleher, Bishop, and Briggs ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY 
August 1996 
The other possible cause of the acute infection was 
a necrotic pulp. Unfortunately, this was not initially 
considered because the teeth were unrestored and 
there was no history of trauma. Furthermore, the pa- 
tient 's perception and localization of pain was also 
compromised because of the concurrent loss of sen- 
sation in the region. The significance of this factor is 
highlighted by the fact that the patient developed 
classic symptoms when the pulp of the canine tooth 
became necrotic. However, the clinicians failed to 
realize that pulps can become necrotic in the absence 
of any visible damage and this is common in SCA 
patients. The infarct, which probably caused the loss 
of sensation in the area, could also have caused pul- 
pal necrosis if the blood supply to the teeth was in- 
terrupted. Furthermore, many teeth could be affected 
if a large blood vessel was involved. Even if this fact 
was realized, disruption of the sensory nerve supply 
meant that conventional vitality tests were of little 
value in assessing the condition of the pulp. 12 Alter- 
native methods of assessing pulp vitality, such as la- 
ser Doppler flowmetry, which evaluates the pulpal 
blood flow directly, may have been more applicable. 
Unfortunately, such techniques have not been fully 
assessed clinically and are not widely available. 12 
Necrotic pulps often produce radiographic changes 
that can be used to confirm a diagnosis. Unfortu- 
nately, the radiographic changes associated with SCA 
may mask those alterations normally associated with 
a nonvital pulp. Although the radiographic appear- 
ance in this case was considered normal for a patient 
with SCA, the apical changes associated with these 
teeth were quite distinct from the multilocular lesion 
observed in the body of the mandible (Figs. 1 and 2). 
These changes, therefore, warranted further investi- 
gation and questioning of the initial diagnosis. 
Although intra-oral periapical radiographs may 
have provided additional information, only OPGs 
were available before the start of root canal therapy. 
However, these radiographs gave a clear indication of 
the changes that occurred. The GDP felt that little ad- 
ditional information on the pulpal status of the four 
teeth would be provided by further radiographs, par- 
ticularly considering the ambiguity of the appearance 
in SCA patients. 
In the circumstances, the only method available to 
confirm the status of the pulp was by preparing test 
cavities. This was undertaken by the GDP who rec- 
ognized that multiple oral complications made con- 
ventional methods of diagnosis difficult. 
Root canal therapy is usually successful if per- 
formed to a satisfactory technical standard. 13 How- 
and Olujohungbe. reported a SCC af- ever, Gregory 8 
ter a molar root canal therapy and considered that this 
was the result of a localized inflammation caused by 
the root filled tooth. For this reason the patient was 
made aware of the implications of the root fillings at 
the second molar and the second premolar (Fig. 3). 
CONCLUSION 
This case illustrates a number of diagnostic and 
management difficulties when acute infections occur 
in patients with SCA and when a SCC results in mul- 
tiple oral problems. In such situations it is important 
that all possible causes for symptoms are fully con- 
sidered and eliminated. I f response to therapy is not 
predictable then an alternative diagnosis should be 
considered with opinions from other specialists 
sought. 
Ill-defined radiolucencies in mandibular right re- 
gion and at furcation of first molar appear to have re- 
solved compared with Fig. 1. Note also the completed 
root filling in the canine tooth. 
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Reprint requests: 
Karl Bishop 
Department of Restorative Dentistry 
Charles Clifford Dental Hospital 
Wellesly Road 
Sheffield, UK