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.