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Lincoln B. Taylor, DDS, MS, Arthur J. Nowak, DMD, Roger H.

Giller, MD,
Paul S. Casamassimo, DDS, MS

Sickle cell anemia: A review of the dental concerns and a retrospec-


tive study of dental and bony changes

ickle cell anemia (homozygous tions of severe hypoxia.


This paper is a review of the me&
SS disease) is a hereditary Sickle cell crises are experienced
ical concerns pertlnent to dental
hemolytic disease. Eight per- by SS patients and consist of recur-
care and a preliminary study of den-
cent of American Blacks are silent car- rent attacks of pain in the abdomen
tal findings of the sickle cell anemia
riers of the sickle hemoglobin gene. and limbs sometimes associated with
(SS) patient. The dental characteris-
These individuals have one normal fever and the passage of dark or red
tics observed in 21 dental patients
gene and one mutant sickle hemo- urine, weakness, jaundice, pallor, car-
with SS are described. Radiographic
globin gene, a condition referred to as diomegaly, splenomegaly, increased
findings included “stepladder” tra-
sickle trait. Approximately 1 in 500 risk of infections due to loss of
beculae pattern (70%),enamel
American Blacks inherit two defective splenic function, and leg ulcers. The
hypomineralization (24%),calcified
sickle hemoglobin genes and manifest crises are promoted by a number of
canals (5%),increased overbite ( 3 0
the clinical disorder of sickle cell ane- factors such as hypoxia, dehydration,
80%),and increased ovetjet (56%).
mia.’,* acidosis, and stre~s.’*~,~-”
Comparisons are made with other
The distortion of the erythrocyte Frequently, the first manifestation
studies of the sickle cell patient, and
into a sickle shape is the result of low of SS disease is dactylitis (hand and
the need for further study Is suggest-
oxygen tension (oxygen partial pres- foot syndrome): symmetric nonpit-
ed.
sure of 50 to 60 mm Hg), which caus- ting edema of the hands and feet,
es partial crystallization of the accompanied by warmth and tender-
Hemoglobin S and realignment of the n e s 7 It is most common in children
defective hemoglobin molecules.3 under the age of 4 years7 Hand and
The distorted sickle cell has increased foot syndrome should alert the clini-
cellular rigidity, causing membrane cian to evaluate the patient for under-
damage as the cell traverses the lying sickle cell disease.
body’s microvasculature. Thus, red Infarction of the mandible has
cell life span is shortened due to been recognized as part of a painful
hemolysis. Sickling promotes ery- crisis9 Abnormal sensation associat-
throstasis, increased blood viscosity, ed with the mental nerve has been
reduced blood flow velocity, and, in reported.’O Mental nerve neuropathy
turn, tissue hypoxia and further sick- is an oral manifestation which pro-
ling4 duces severe pain in the mandible
Sickle cell anemia patients have and usually results in lip
many medical problems which can be paresthesia.6 Paresthesia of the men-
induced during dental treatment. tal nerve appears to be secondary to a
These potential medical complications vascular occlusive episode involving
increase the need for precautionary the inferior alveolar artery at or near
measures during dental treatment. In the mental foramen.6The pathologi-
contrast, individuals with sickle cell cal basis appears to be nerve com-
trait possess a heterozygous genetic pression associated with mandibular
pattern (SA) and may be treated as bone involvement, though this is still
normal healthy patients. Individuals ~ n c l e a rRecovery
.~ of sensation may
with sickle trait are asymptomatic be slow, with the paresthesia lasting
carriers of a single recessive sickle as long as 18 months.6
gene and do not experience clinically Bony deformities are commonly
relevant sickling except under condi- observed in sickle cell disease

38 Special Care In Dentistry, V o l 1 5 No 1 1995


patients. Compensatory expansion of mine similarities in dental findings. hypomineralization (five patients)
the erythroid (red cell) marrow pro- Information regarding the hemoglo- and the calcified canal (asymptomatic
duces certain bony changes, such as bin genotype was obtained from the tooth #25, coronally). However, the
overgrowth of the anterior part of the medical history reported. No same patient with the asymptomatic
maxilla with increased angulation matched group (control) for age or calcified canal had a cariously
and separation of the incisor teeth.7 sex was considered at the time the involved tooth (#30) which required
Also, distinct bony trabecular pattern data were collected. root canal therapy. This tooth had the
that are usually noted during clinical The radiographic features listed in apical third of the distal canal calci-
and radiographic e~amination.~ the results were obtained from peri- fied.
Clinicians noting the combined apical and occlusal films. Normally, When considering the presence of
occurrence of these bony changes the incisors are in contact, with the malocclusion with overbite and over-
should have the patient evaluated for upper incisors ahead of the lower by jet, we noted oral habits as a possible
hemolytic disorders such as sickle only the thickness of the upper edges etiology. Only one patient (mentally
cell anemia. (ie., 2 to 3 mm overjet is the normal retarded) was shown to have an oral
Mucosal pallor, delayed eruption, relati~nship).’~ Thus, abnormal over- habit (thumb-sucking) which may
dental hypoplasia, and radiographic jet is defined as the horizontal dis- have contributed to the malocclusion.
changes are other common oral find- tance of more than 3 mrn.I2 All the patients were on daily
ings associated with SS disease.” Normally, the lower incisal edges antibiotics as a precautionary mea-
Other dental findings observed in come into contact with the lingual sure against any subsequent infec-
Nigerian sickle cell anemia patients surfaces of the upper incisors at or tions. The regimen was penicillin 250
included the intrinsic opacity of enam- above the cingulum (i.e., normally, mg twice daily, and in the case of
el, malocclusion (overjet and overbite), the overbite is 1 to 2 rnm).I4Okafor et allergy to penicillin, erythromycin
decreased dental caries, and diastema- a l l 2 considered excessive overbite to 250 mg twice daily. Some patients
ta (gaps between the teeth).12It was be the vertical distance greater than were also given folic acid and iron
noted, however, that the decrease in 2.5 mm between the tips of the upper supplements. The accelerated ery-
dental caries among the Nigerians was and lower incisors. This retrospective thropoiesis increases folic acid
attributed to the widespread avoid- study had data recorded as percent- requirements in patients with sickle
ance of sweets or candies, which they age coverage for which excessive cell anemia.l” Iron deficiency may
believe weaken their blood.12Also, overbite is considered to be greater occur, resulting in decreased reticulo-
Okafor et a1.12did not discuss staining than 30% relative to the lower incisal cyte and hemoglobin levels.16On the
but rather only mentioned the intrinsic coronal coverage by the upper other hand, iron levels in patients
opacity noted on the cusps of the teeth. incisors. with sickling disorders are often high
Despite these findings, it should be and must be monitored due to the
noted that there was no sigruficant dif- Results tendency for overloading due to
ference in dental age between sickle The Table summarizes dental find- transfusions and increased dietary
cell anemia patients and normal ings noted on the dental charts of 21 iron absorption.
patients.13This is of note, since puber- patients with sickle cell anemia. The Pneumonia appeared to be the
ty is commonly delayed in patients 21 patients represented all the sickle most common infection among the 21
with sickle cell anemia. cell anemia dental patients at patients. There were seven patients
A preliminary study involving a Children’s Hospital in Columbus, for whom pneumonia had occurred
retrospective dental chart review of Ohio. The most prevalent condition at some time in their lives. Only two
all sickle cell anemia dental patients common to these patients was the patients‘ records contained no indica-
at the Children’s Hospital in radiographic trabeculae “stepladder” tion of their past medical history.
Columbus, Ohio, was conducted. The pattern (70%). Other conditions Other medical problems were sinus
purpose of this study was to deter- observed included overjet of 3-10 mm infection, gallstone removal (two),
mine if there was any similarity to (56%),overbite of 30-80% (~OYO), cal- hand-foot syndrome, back pain, joint
the dental findings described in the cified canal (5%), and hypomineral- pain (two), and abdominal pain.
Nigerian SS study conducted by ization of enamel (24%).The maloc-
Okafor et al.12 clusion does not provide conclusive Discussion
evidence that bimaxillary protrusion Despite the lack of a matched group
Materials and methods is unique to sickle cell patients, since for age and sex, some of the dental
A retrospective dental chart the black population does tend to findings in the SS patients in Ohio are
review was conducted on all (21) exhibit this characteristic feature. similar to those obtained in the
sickle cell anemia patients (ages 8 to In some cases, as indicated in the Nigerian study reported by Okafor et
31 years of age) at the Children’s Table, there was no documentation ~ 1 . ’ ~In. that study, clinical evalua-
Hospital Dental Clinic in Columbus, or radiographs. Also, there were no tions were conducted on 37 consecu-
Ohio. The data collected from routine other etiological factors documented tive SS patients, aged from 14 to 33
follow-up visits were used to deter- in the records regarding enamel years, on routine follow-up visits.12

Special Care In Dentistry, Vol15 No 1 1SSS 39


Table 1. Dental findings of 2 1 subjects at Children’s Hospital, Columbus, Ohio. commonly caused by salmo-
nella infections;
(5)distinct areas of radiopacity;
Abnormalities Subjects (N = 21) Percentage and
(6) the stepladder appearance
Trabeculae with stepladder 11/20‘ 70 created in the interdental
alveolar bone by horizontal
Enamel hypomineralization 5/21 24 rows of trabeculation.”
Calcified canal 1/21 5 These findings are attributed to
erythroblastic hyperplasia and
Overbite (30-80‘2,) 8/16 50 medullary hypertrophy, with the
Ovejet (3-10 mrn) 9/16 56 resultant loss of fine trabeculae and
the formation of larger bone marrow
spaces..’ Expansion o f the red-cell-
‘The denominator represents the total number of patients with documented informa-
producing marrow develops as a
tion in each category obtained from their dental records.
compensatory mechanism for the
underlying hemolytic anemia.
Malocclusion in the Nigerian SS lower border of the mandible is Seventy percent (70%))of the patients
patients was reported to be 35%, in mainly supplied by the periosteal in our study had the stepladder tra-
contrast to 16.6% in controls. The blood supply, in contrast to the rest beculae pattern. This was evident in
overjet and overbite occurrence in of the mandible-such a s the coro- the bitewing radiographs around the
our study was 56% and 50%, respec- noid process, ascending ramus, and first molar region.
tively, despite the lack of documenta- the angle-which are more adequate- Other changes include hyperce-
tion in 5 charts. ly supplied by the arteries to the mentosislHand distinct intact lamina
Other studies have indicated a attached muscles.IYThe inferior alve- dura. These changes, although typi-
tendency for a more protrusive max- olar artery supplies the teeth and cal, should not be considered as
illa and a more retruded incisors.I7 It alveolar process, together with part definitive diagnostic criteria for sickle
was considered that an increase in of the mandible. An occlusion of the cell anemia. Similar changes may be
bone marrow activity in patients with artery will have its effects mainly in seen in normal individuals as well as
sickle cell disease may explain the the alveolar process and teeth. This is in other systemic disorders such as
maxillary protrusion. Retrusion of more critical in the molar region than thalassemia, estrogen imbalance,
the anterior teeth is most likely t o be in the more anterior regions.IYThe hyperparathyroidism, Paget’s dis-
the result of the increased lip pres- lower anterior teeth are supplied by ease, and metabolic bone disease.h
sure due to the protrusive m a ~ i l l a . ’ ~ anastomoses involving the incisive As previously stated, the dental
and mental branches of the inferior characteristics given here are not to
Pulp calcificatioo be considered as unique diagnostic
alveolar arteries from both rieht
” and
The presence of peculiar foreign par- left sides, as well as branches of the features of sickle cell anemia. The
ticles in the dental tubules indicates facial arteries. Infarction, necrosis, Occurrence of a calcified canal is quite
infection in the canal.IHAlso, the and osteomyelitis are therefore more common even in normal healthy con-
presence of denticle-like hardened likely to occur in the posterior region trol subjects and thus is not necessari-
bodies as a result of calcification in of the mandible.” ly a definite feature. A sickle cell ane-
the pulp suggests the occurrence of mia patient may have one or more of
blood vessel thrombosis.IHThese ~~ ~
Radlographlc features the features listed in the Table. If a
findings are not pathognomonic for Radiographic changes in the jaw combination of features Occurs, the
sickle cell anemia patients, but sick- occur in 79% to 100% of patients, in clinician should be alerted to investi-
ling has an effect on the aforemen- all age groups, and in both the gate the patient‘s medical history. In
tioned changes.In As noted in our mandible and maxilla.1H some instances, the patient may not
study, 5% of the patients had calci- Radiographic changes in sickle cell alert the clinician to his or her med-
fied canals. anemia patients are: ical condition, since in the patient’s
estimation, the dentist should be con-
Mandibular odeomyelitis cerned only with teeth and not the
(1) a n increase in radiolucency
Chronic hemolysis and secondarily of the jaws d u e to the patient’s general health. In addition,
increased erythropoiesis result in decreased number of trabec- it is helpful to realize that certain con-
expansion of the bone marrow. The ulae; ditions-such a s intrinsic opacity,
skeleton is also vulnerable to vaso- (2) coarse trabecular pattern; ovejet, and overbite-are the norm
occlusive crises, especially where the ( 3 )thin inferior border of the rather than the exception in SS
blood supply is relatively limited or mandible; patients.
tissue is poorly vascularized.Iy The (4) generalized osteoporosis The responsibility of the dentist is

40 Specla1 Cam in Dentistry, Vol16 No 1 lo05


to ensure that a proper referral sys- cell anemia patients are categorized (1)Hemoglobin levels should
tem exists for these patients if they as an ASA 111anesthetic risk.20Dental be above 10 gm/100 mL.21
are unable or unwilling to deliver the treatment for the SS patient is best Packed erythrocyte transfu-
necessary dental care. Avoidance by performed on an ambulatory basis sions may be administered
the dentist does not eradicate the with relevant precautions.21Local so that an acceptable hemo-
patient's problem and may have anesthesia is more desirable than globin level can be obtained,
avoidable medical consequences. general anesthesia when possible, i.e., 10-12 gm%.
The dentist has no need to fear because local anesthesia has less (2) Use of preoxygenation
treating the sickle cell patient once potential to lower the oxygenation of before induction may mini-
medical consultation is sought. When the bl00d.~However, numerous den- mize the dangers from air-
the patient is in a non-crisis state, tal appointments and lengthy proce- way obstruction or an unex-
dental treatment within the above- dures may be too stressful for the pectedly difficult intubation.
listed guidelines may be rendered. patient, leading to lack of coopera- (3) The maintenance of oxy-
During all forms of dental treatment, tion, and thus oral rehabilitation genation with the monitor-
care to preserve normal oxygenation under general anesthesia is more ing of oxygen delivery and
must be taken to avoid systemic sick- beneficial under such circumstances. oxygen saturation.22
ling complications. Consultation with Oral sedation is an alternative to (4) Inhalation of 30-50'/0 oxygen
an anesthesiologist experienced in help decrease preoperative anxiety for at least 4 hours postoper-
the care of SS patients may be helpful levels prior to local or general anes- atively should guard against
in this regard. thesia. Low doses should be used; hypoxemia due to hypoven-
Preventive dental care is impor- however, if moderate levels are need- t i l a t i ~ nFollowing
.~~ surgery,
tant, since oral infection may lead to ed, additional oxygen by nasal can- complications are more com-
osteomyelitis or tissue n e c r ~ s i s . ~ nula is suggested.20Similarly, nitrous mon than intraoperatively.
Dental counseling should begin at an oxide sedation must occur with ade- Thus, the recovery period is
early age and should be maintained quate assurance that oxygenation of most critical.
throughout life.7Antibiotic prophy- the patient is maintained at all times (5) Hydration must be main-
laxis is recommended before and during the procedure. The use of tained during all phases of
after all dental procedures (including nitrous oxide is contr~versial.~ the procedure to prevent
routine prophylaxis) which are likely However, if proper oxygenation is hemoconcentration and
to cause gingival bleeding, because of provided and carefully monitored, resultant s i ~ k l i n g . ~ ~
the added risk of infection and sys- nitrous oxide is not contraindicated. (6) Further transfusion may be
temic ba~teremia.'~ Patients with an For an adequate margin of safety, necessary if reduction in the
acute infection-for example, an 50% oxygen concentration must be HbS level (< 30%) is deemed
abscess-should receive intramuscu- maintained along with high flow rate advisable to avoid perioper-
lar or intravenous antibiotics instead and proper ~entilation.',~,~ With any , ative sickling problems.22
of oral medi~ati0n.l~ The parenteral form of sedation, continuous moni- The extent of the dental
route will give faster and more con- toring of oxygen saturations should surgery, the length of gener-
sistent blood levels of the antibiotic. be done. al anesthesia required, and
Antibiotic therapy for established The use of local anesthesia with a the patient's past history of
infections should be determined in vasoconstrictor is contr~versial.~,~ sickle-related and other
consultation with pediatric medical Some clinicians claim that vasocon- medical problems should be
consultants. strictors may impair circulation local- considered by the consulting
Routine dental treatment may be ly and lead to infarction, while others hematologist and anesthesi-
rendered during noncrisis periods; maintain that vasoconstrictors have ologist in making their rec-
however, therapy during crisis no effect on the local tissue oxygena- ommendations regarding
should be directed toward tion. transfusion.
palliati~n.~,~,Patients with fevers of For dental treatment with general (7) Temperature hemostasis
unknown origin should be evaluated anesthesia, all sickle cell anemia should be maintained
to rule out a possible dental ~ a u s e . ~ , ~ ,patients must be referred to both a aggressively with the aid of
Minimizing physical stress hematologist and an anesthesiologist heating blankets, warmed
reduces the risk of a crisis. Thus, care who are experienced in the care of intravenous fluids, humified
should be given in consideration of sickle cell anemia patients. The choice inspired gases, and warm
the manner in which dental treat- of anesthetic technique is less impor- ambient temperature.22
ment should be provided and the tant than the care with which it is
length of treatment. The treatment administered. As previously dis- The guidelines for treatment of SS
options include the traditional dental cussed, important considerations for patients in the dental office should
setting (1)with only local anesthesia the general anesthesia for dental pro- include the relevant list above but
or (2) under general anesthesia. Sickle cedures include: also the following:

Special Care In Dentlstry, Vol15 No 1 1995 41


(1)Schedule dental appoint- educating the parents and providing case. J Am Dent Assoc 87661-4,1973.
ments during the morning both comprehensive and preventive 10. Konotey-Ahulu FID. Mental-nerve neu-
ropathy: a complication of sickle cell cri-
with minimum treatment for dental treatments, dentists can sis. Lancet 2388,1972.
a brief since stress achieve these goals. 11. Cox GM, Soni NN. Pathological effects of
and overexertionll are Further studies are being pursued sickle cell anemia on the pulp. J Dent
thought to be contributing to expand on the findings of Okafor Child 51:128-72,1984.
factors in a sickle cell crisis. et a1.12 and this retrospective study. 12. Okafor LA, Nonnoo DC, qehanon PI,
Aikhionbare 0.Oral and dental complica-
(2) Prescribe CNS depressants These studies suggest that there are tions of sickle cell disease in Nigeria.
judici~usly.~~ some distinct‘changes in both the Angiology 37672-5,1986.
(3) Use acetaminophen for treat- dental and bony structures in sickle 13. Sears RS,Nazif MN, Zullo T. The effects
ment of pain, because high cell anemia patients. of sickle cell disease on dental and skele-
doses of salicylates may tal maturation. J Dent Child 48275-7,
1981.
induce acidosis and increase 14. Proffit WR, Fields HW Jr. Malocclusion
bleeding4 Dr. Taylor is assistant professor of pediatric
dentistry, University of Tennessee, Dunn and dentofacial deformity in contempo-
(4) Avoid elective surgery, such rary society. In:Contemporary orthodon-
Dental Building, Memphis, Tennessee. Dr.
as the removal of asympto- Nowak is professor of pediatric dentistry and tics. 2nd ed. St. Louis: C.V. Mosby Co., 7-
matic impacted teethJ5 pediatrics, University of Iowa, College of 16,1993.
(5) Ensure appropriate home Dentistry, Iowa City, Iowa. Dr. Giller is 15. Smith DB, Gelbman J. Dental manage-
associate professor of pediatrics, University of ment of the sickle cell anemia patient.
fluoride therapy and routine Clin Prev Dent 821-3,1986.
Colorado School of Medicine, Division of
dental recall visits as in any Hematology, Oncology & Bone Marrow 16. Seakins M, Gibbs WN, Milner PF, Bertles
general preventive dental Transplant, The Children’s Hospital, Denver, JF. Erythrocytes HbS concentration an
treatment regimen, to mini- Dr. Casamassimo is professor and chairman important factor in the low oxygen affini-
mize undesirable dental pro- of pediatric dentistry, Children’s Hospital, ty of blood in sickle cell anemia. J Clin
Columbus, Ohio. Invest 52422-32,1973.
cedure~.~~ 17. Shnorhokian HI, Chapman DC, Nazif
MM, Zullo TG. Cephalometric study of
Thus, greater awareness of clinical 1. Bamfiart MI, Henry L, Lusher JM. Sickle American black children with sickle-cell
findings should enable clinicians to cell. 17th ed. Kalamazoo (MI): Scope disease. J Dent Child 51:431-3,1984.
design appropriate prophylactic mea- Publications, Upjohn Co., 9-58,1979. 18. Soni NN. Microradiographic study of
2. Honig GR. Sickle cell anemia. In: Birth dental tissues in sickle cell anaemia. Arch
sures for those in whom incipient defects encyclopedia. Buyse ML, Ed. Oral Biol2:561-4,1966.
abnormalities are detected. Sickle cell Cambridge (MA): Blackwell Scientific 19. Iwu CO. Osteomyelitis of the mandible in
anemia patients cannot risk eliminat- Publications, 135-7,1990. sickle cell homozygous patients in
ing dental evaluation, since, in the 3. Sanger RB, McTigue DJ. Sickle cell ane- Nigeria. Br J Oral Maxillofac Surg 27429-
event of an oral infection, their health mia-its pathology and management. J 34,1989.
Dent Handicapp 3:9-21,1978. 20. Malamed SF. Sedation: A guide to patient
is greatly compromised compared 4. Smith HB, McDonald DK, Miller RI. management. St. Louis: C.V. Mosby Co.,
with that of the normal healthy per- Dental management of patients with sick- 565,1989.
son. le cell disorders. J Am Dent Assoc 114:85- 21. Demas DC, Cantin RY, Poole A, Thomas
Until a completely successful 7,1987. HF. Use of general anesthesia in dental
treatment is identified, sickle cell ane- 5. Primley DM, Oatis GW, Grisins RJ. care of the child with sickle cell anemia.
Complications of sickle cell anemia in a Oral Surg Oral Med Oral Pathol66:190-3,
mia patients will still have to cope dental patient. US Navy Med 7322-6, 1988.
with the complications and conse- 1982. 22. Esseltine DW, Baxter MRN, Bevan JC.
quences of their disease. The dentist 6. Rose LF, Kaye D. Internal medicine for Sickle cell states and the anesthetist. Can J
plays a vital role in the health care dentistry. 1st ed. St. Louis: C.V. Mosby Anaesth 35:385-403,1988.
required by these patients and can CO., 416-7,1983. 23. Howells TH, Huntsman RG, Boys JE,
7. Sejeant GR. Sickle cell disease. Bone and Mahmood A. Anaesthesia and sickle-cell
provide reassurance to the patients. joint lesions. New York Oxford haemoglobin: with a case report. Br J
Parents have enough of a problem University Press, 168-80,1985. Anaesth 44:975-87,1972.
coping with the disease and need not 8. Seakins M, Gibbs WN, Milner PF, Bertles 24. Primley DM, Oatis GW, Grisins RJ.
be further frustrated by their chil- JF. Erthrocyte HbS concentration an Complications of sickle cell anemia in a
dren’s dental care situation. Quality important factor in the low oxygen affini- dental patient. US Navy Med 7322-6,
ty of blood in sickle cell anemia. Clin 1982.
of life can be enhanced through the Invest 52422-32,1973. 25. Rouse LF, Hays GL. Dental considera-
elimination of dental factors which 9. Walker D, Schenck KL. Infarct of the tions in sickle cell anemia. Gen Dent
may trigger a crisis. Thus, through mandible in sickle cell anemia: report of a 2718-9,1979.

42 Special Care In Dentistry, Vol15 No 1 1995

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