Exodontia for a patient
with sickle cell anemia: report of case
M ichael S. M atson,* D .D .S., M iam i, Fla.
In patients with sickle cell anemia, exo­
dontia and other oral surgery procedures
present risks. Precautions necessary to
prevent crisis include supervision of the
patient by medical consultants, raising of
the hemoglobin content of the blood by
the transfusion of whole blood, regional
rather than general anesthesia, hospitali­
zation and close postoperative observa­
tion. A case report illustrates the treat­
ment procedures.
Sickle cell anemia is a serious malady with
a fatal prognosis,, although some patients
live on for many years. Extraction of
teeth and other oral surgery procedures
for patients suffering from this disease
present definite risks. There are important
factors which must be taken into con­
sideration when planning oral surgery
treatment.
In this form of anemia, death may
occur from infections or as a result of
thrombosis or hemorrhage involving vital
tissues. This form of anemia is now recog­
nized as a clinical entity and many case
histories are on record. Usually it is found
in Negroes. Instances of this type of
anemia have been reported among
Greeks, Italians, and Sicilians, as well as
Negroes, but these instances are rare.
Sickle cell anemia is a hereditary and
familial disease, present and recognizable
in infancy. T h e disease appears in two
forms, latent and active. T h e dentist con­
cerns himself with the complications that
may arise when the patient has the active
form of the disease. This type is most
severe in children. T h e red corpuscles are
elongated and sickle shaped and the
hemoglobin content is proportionately
high. A moderate leukocytosis with a
nearly normal differential count1 is al­
most always present.
Individuals with this type of anemia
show a remarkable adaptation to their
state of chronic anemia and jaundice.
They may have few or no complaints.
Examination may reveal that the sclerae
are greenish-yellow and the mucous mem­
branes are very pale. The physical ap­
pearance of these patients often presents
a characteristic picture. They are usually
underweight, the trunk is short, the ex­
tremities are long, and the body is linear
with narrow hips and shoulders.
Abnormalities in the roentgenographic
appearance of the bones may be found,
especially in the skull, vertebrae, tibia,
and fibula. The earliest change visible in
the skull is the development of a ground
glass appearance. In more advanced
stages a peculiar radial striation is seen.2
A complication which may arise when
surgical procedures are performed is the
development of crisis. This is manifest as
shock. Crisis sometimes occurs in sickle

cell anemia when the oxygen content of
the blood is lowered. The lowered oxygen
tension deoxygenates hemoglobin causing
it to crystallize which in turn distorts the
erythrocyte cell membrane. The mal­
formed cells collect in small blood vessels
causing blockage which results in organic
damage. The same process also causes a
rapid removal of red cells from circula­
tion.The result of this combined mecha­
nism is shock and, in severe cases, death.
Prevention of crisis depends on careful
supervision of the patient by the dentist,
or oral surgeon, and the physician. Pre­
operative examination of the patient’s
blood may reveal a hemoglobin as low as
5 Gm . per 100 cc. of blood. I f this is the
case a transfusion of whole blood will be
necessary to raise the hemoglobin content
to a level which will permit the necessary
; surgical procedures to be undertaken
safely.
The use of general anesthesia is contra­
indicated for these patients because a
lowering of the oxygen tension of the
blood may occur. This will result in a
crisis with all the dangers inherent in this
condition. Regional anesthesia should be
relied upon.
Hospitalization is indicated for the pa­
tient with sickle cell anemia when treat­
ment, such as extensive exodontia, is
necessary. Despite all precautions, these
patients may go into crisis during the
operative or postoperative periods. O xy­
gen should be available for immediate ad­
ministration. A supply of whole blood of
the proper type should be available for
transfusion.
The following case history illustrates
the problems of clinical management en­
countered during the treatment of a 14
year old Negro boy with a history of sickle
cell anemia.
R E P O R T O F CASE
The patient was referred to the oral sur­
gery clinic of the hospital for multiple
tooth extractions.
M A T S O N . . . V O L U M E 62, JU N E 1961 • 79/705
Complaint and Present Illness • He was
conscious of sensitivity to thermal stimuli
in several sections of his dentition. Clin­
ical examination revealed that the man­
dibular first molars, the maxillary first
molars, and a maxillary second bicuspid
were unrestorable. Roentgenographic ex­
amination confirmed this diagnosis as
regions of apical involvement were visible
and decay involved the bifurcation of the
molars.
Past History • The patient was a school­
boy in apparent good health. H e had been
admitted to the hospital several times for
“stomach ache” which apparently had
been sickle cell crisis. O n these occasions
he had received transfusions of whole
blood. H e apparently had not received
adequate dental care and was negligent
about oral hygiene.
Family History • The patient’s mother
had died of sickle cell anemia. His older
brother, age 17 years, was suffering from
the same disease. Tw o older sisters had
died from this disease at the ages of 20
and 13 years. The father was alive and in
apparent good health.
Physical Examination • The boy was
poorly developed for his age. He was
underweight, with a small and linear
physique. He appeared to be mentally
sluggish. O n admittance to the hospital
his blood pressure was 1 0 8 /6 8 , his oral
temperature 99.2 °F ., his pulse 78, and his
respiration 22.
Examination by the medical service re­
vealed the presence of a sinus arrhythmia
in the heart with “blowing systoles,” that
is, a functional murmur heard when the
heart is in systolic function, over the apex.
The liver and tonsils were enlarged.
Laboratory Data • Consultation with the
hematological service was obtained. C om ­
plete laboratory examination of the blood
and urine was ordered.
The results of the laboratory tests
 80/704 • THE J O U R N A L O F THE A M E R IC A N D EN T A L A S S O C IA T IO N
were: red blood cells 2,690,000, hemo­
globin 7.3, hematocrit 22.5, mean corpus­
cular volume 83.7, mean corpuscular
hemoglobin volume 32.5, mean corpus­
cular hemoglobin 27.1, reticulocytes 10.5,
icterus index 15, and white blood cell
count 14,250.
T h e results were positive for the sickle
cell test. T h e serological test for syphilis
was negative. The urine was negative for
sugar and albumin, the specific gravity
was 1.010, and the color was clear and
yellow. Cross match and typing was Rh
positive, Group A.
Course and Treatment • As a result of
consultation with the hematological, anes­
thesiology, and medical services, the fol­
lowing suggestions were made for the
contemplated removal of the necessary
teeth.
The hematology report said :
Patient with sickle cell anemia with no his­
tory o f crisis during the past two years. T he
bleeding time is normal, the Rum pee Leede is
negative and the prothrombin time is normal.
N o contraindications for dental extractions
are present from the hematological viewpoint.
Recom m end transfusing with tw o units o f
whole blood prior to surgery.
The anesthesiology report said:
In light o f the sickle cell anemia, it would
be advisable to use some form o f local or block
anesthesia. A lowering o f the oxygen content
o f the blood or loss o f blood during surgery
_ w ould certainly be better tolerated if the pa­
tient is not under general anesthesia.
The medicine service report said:
R eading o f the chest film revealed a very
slight decrease in the size o f the heart. There
is coarsening of the trabecular markings
throughout the ribs consistent with sickle cell
disease. T h e lungs are clear. T h e cardiac
status o f the patient presents n o contraindica­
tions for the extraction o f teeth with the use
o f local anesthetic agents.
Operative Procedures • Removal of the
teeth was scheduled for three days after
admission to the hospital. O n the day
prior to surgery a transfusion of one pint
of blood was given. O n the morning of
surgery an additional 250 cc. of whole
blood was transfused. The result o f the
test for the hemoglobin content o f the
blood showed a rise to 9.3 G m . per 100
cc. of blood. One pint of blood was made
available for use, if necessary, during sur­
gery. T w o hours before the operation the
patient received 50 mg. of promethazine
hydrochloride intramuscularly, and one
hour later 75 mg. of meperidine hydro­
chloride intramuscularly.
The patient was brought to the operat­
ing room in a completely relaxed and
sedated condition. A medical consultant
was preseiit. Oxygen with proper equip­
ment for administration and the pint of
whole blood ordered previously were on
hand.
Injections of 2 per cent lidocaine were
made into the mucous membrane of the
four quadrants of the mouth. T h e con­
demned teeth were removed without diffi­
culty. T h e patient remained quiet during
the procedures. Clotting appeared to be
normal. The patient was returned to the
ward where instructions were given for
the vital signs to be checked every ten
minutes for four hours.
Result • The patient slept comfortably
for 14 hours after surgery. There was a
minimum of bleeding from the sockets.
O n the day after surgery the patient ap­
peared to be comfortable and without
pain. There was no facial swelling. He
was alert and friendly. Three days after
the extractions he was asymptomatic and
was discharged from the hospital. H e
was instructed to seek dental treatment
for the restoration of the decayed teeth in
his dentition.
SUMMARY
The precautions necessary to prevent
crisis during exodontic procedures on a
patient with sickle cell anemia are dis­
cussed. These precautions included ade­
quate supervision of the patient by medi­
cal consultants prior to surgery. T h e
 M A T SO N . . . V O L U M E 62, JU N E 1961 • 81/707

hemoglobin content of the blood was
raised by the transfusion of whole blood
before the extraction of the teeth.
Regional anesthesia after adequate pre­
medication with promethazine hydro­
chloride and meperidine hydrochloride
was used because general anesthesia was
contraindicated. Additional whole blood
for transfusing and oxygen with adequate
equipment for administration were avail­
able for emergency treatment, if needed,
during the operation.
7657 Biscayne Boulevard
*Former chief resident in oral surgery, department
of dentistry, Kings County Hospital Center, Brooklyn.
1. Todd, James Campbell, and Sanford, Arthur
Hawley. Clinical diagnosis by laboratory methods; a
working manual of cJ/nical pathology, ed. 9. Phila­
delphia, W. B. Saunders and Co., 1939.
2. Wintrobe, Maxwell M. Clinical hematology, ed. 3
Philadelphia, Lea & Febiger, 1951.

N o Plateau in R esearch • R e se a rch in the m e d ica l an d b io lo g ica l area needs to be increased

w ith greater usage o f the physical, ch e m ica l an d m ath em a tical tools. L est w e b e c o m e to o m u ch
satisfied w ith o u r r e c e n t accom p lish m en ts an d think n o m ore m on ey is necessary, let us take
a look at o u r d e ficit. In the field o f these o r g a n ic afflictions o f the brain an d nervous system,
w e are barely b e y o n d the p o in t o f n a m in g th em a ccu rately, an d, unless th ey are cau sed by
in ju ry , in fectiou s a g e n t, or a ca n ce r, w e k n o w n o th in g really ab ou t their origin . T h e w h ole
array o f m ental d iso rd e rs w ill p ro b a b ly som e d ay yield to a b ioch e m ica l-b io p h y sica l expla n ation ,
b u t as yet ou r b o o k o f k n ow ledg e ab ou t this, the m ost p revalen t o f disorders, is still in the
p re fa ce stage. In th e field o f in fection s w e h ave m a d e the m ost n otab le strides, bu t here again
the in fectiou s agen ts, like all liv in g things, eventually learn to fig h t b a ck , an d m ore an d m ore
p a th o g e n ic strains are gradu ally ex ertin g a greater stubbornness a b ou t y ie ld in g to m ed ica tion .
A n an tib io tic has n o t been fo u n d fo r a virus in fe ctio n , w e have n ot su cceed ed in fo r c in g the
co m m o n c o ld to y ie ld , and there are p ro b a b ly literally dozens o f y et-u n d iscov ered n ew strains
o f virus p athogens.
Lest y o u think I am d e p reca tin g o r m in im izin g o u r fo rw a rd m a rch in m ed ica l research, let
m e assure y ou the o p p o site is true. T h e p o in t I w ish to m ake is— this is no tim e to p latea u our
research p ro g ra m . I n view o f the g o v e rn m e n t’ s interest in m ed ica l research, m an y h ave asked,
c a n industry a ffo rd to m aintain o r en h an ce its research p ro g ra m ? T o m e these w ord s are in
in a p p rop ria te o r d e r . T h e real qu estion is, ca n ind ustry afford n ot t o ? L ow ell T. Coggeshall.
C on text 1 :7 Spring 1961.