Orthodontics and dentistry for the hemophilic patient Richard
C. Grossman,
D.M.D.
Woodland Hills, Calif.
A
number of years ago the staff of a regional center for treatment of hemophilic children, knowing of my interest in handicapped persons,l-g referred a hemophilic patient to me for treatment of his severe malocclusion. I attempted to discourage orthodontic treatment for the patient because I was fearful of initiating hemorrhages that would be difficult to control. However, the determination of the patient and his mother gave impetus to a search for improved methods of management of dental problems associated with the disease. The advent of new techniques since my previous publicationl” necessitates this updated report.
One bleeds when one or more blood vessels are cut. The usual reactions which stop the bleeding are: (1) The severed vessel contracts. (2) Blood platelets adhere to each other and the cut surfaces of the vessel, causing a soft platelet plug to form in response to the release of adenosine diphosphate.14> I5 (These are the primary methods for closing superficial blood vessels; in classic hemophilia the bleeding time is normal following cuts of superficial vessels if a platelet plug forms.) (3) The stopping of bleeding in larger or deeper vessels requires that a fibrin clot be formed after the formation of the soft platelet plug. (4) The fibrin clot then retracts because platelets form knots at the intersections of fibrin strands and pull the clot mass into a tight unit. For all this to occur, the presence of thrombin is necessary for fibrin to be formed from fibrinogen. The hemophilic patient differs from other persons, however, in that the necessary blood factors for conversion of prothrombin to thrombin are absent. Thus, without blood supplementation or replenishment, the hemophilic person does not form substantial blood clots. Approximately 80 per cent of affected persons have classic hemophilia, otherwise known as hemophilia A, in which antihemophilic blood plasma factor VIII (AHF) is missing. Another 15 per cent have Christmas disease, or hemophilia B, in which a different protein, factor IX, is absent from the blood. These other391
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wise similar disease cntitics comprise the bulk of the hernophilic population and have an incidence of one in JO thousand males or one in 20 thousantl persons. There are several other types of hemophilia, each of which represents I or ‘L per cent of all hemophilia. Hemophilia C, evident among ;lewish persons, is the result of a deficiency of PTA, otherwise known as factor XT. This is a milder form of the disease and is considered to be more rare because it is not diagnosed as frequently. Finally, the fourth relatively common form of hemophilia, Von Willebrand’s disease, also hereditary, affects both males and females, and is due to a deficiency of factor VIII as well as an absence o-f the cofactor which makes platelets sticky. Hemophilia A and hemophilia B are sex-linked diseases transmitted to the child by a gene on one of his mother’s two X chromosomes. Males have an X and a. Y chromosome, and all male offspring of a male hemophiliac receive his Y chromosome and consequently are normal with respect to this disease. However, the X chromosome of a male hemophiliac is donated to all of his daughters, who thus become carriers of the disorder just as if they received an affected X chromosome from their carrier mother. It should also be recognized that it is statistically possible for all of the female offspring and 50 per cent of the male offspring of a female carrier to be born free of hemophilia. Females not only carry and transmit the responsible genes to their offspring; they may suffer from the disease, but to milder degrees, and therefore are usually not recognized as bleeders. Severe classic hemophiliacs (hemophilia A) usually have less than 2 per cent of the normal circulating antihemophilic factor (AHF) and suffer from internal bleeding and hemorrhage into joints without being aware of any accident which has caused such bleeding. The person with moderate classic hemophilia has more than 2 per cent antihemophilic globulin and suffers joint hemorrhage only in instances of trauma. Persons with mild classic hemophilia have even more antihemophilic globulin and bleed only when subjected to surgical procedures. Since factors VIII and IX do not cross the placental barrier, there is no transfer of protection against hemorrhage from the mother to the infant. Therefore, if the child survives the birth experience without bleeding, he will probably be in no difficulty until he starts learning to walk. Repeated minor falls with attendant bleeding and extravasation of blood into the joints and other body sites are the basis for significant future problems for these patients. Although hemorrhage into the kidneys, gastrointestinal tract, and other internal sites is a major problem for the hemophiliac, brain hemorrhage is the most common cause of death in these persons. Bleeding into joints and soft tissues is a serious chronic and often progressively disabling problem for the hemophiliac. Blood is a foreign body in tissues outside blood vessels and causes irritation of the tissues into which it has leaked, even though it is ultimately resorbed. Thus, recurrent episodes of bleeding serve as chronic insults to the host tissues, resulting in the formation of scar tissue. The progressive scarring of joints often becomes very apparent as the child begins to attend school and matures further. Similarly, repeated extravasation of blood into flexor muscle regions may cause contracture due to scarring.
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Paralysis of muscle may even occur on the occasion of a single severe hemorrhage into a muscle, which causes entrapment of a nerve and consequent pressure on it, or which presses the nerve against a ligament or bone. The first of two kinds of soft-tissue hemorrhage is less important and occurs where pressure from swelling is released by itself (for example, bleeding into the orbit). The second, however, is hemorrhage into a closed space, as in the anterior space of the legs, which can necrose muscles, resulting in scar tissue, contraction, and permanent flexure. In addition, with the constant irritation of repeated bleeding into the leg, its length could be increased unless the bleedings are near a growth center, in which case shortening is the result. It becomes obvious that hemophilia is a crippling disease because it creates difficulty in moving as a result of joint bleeding. Such a patient cannot. use crutches because if one knee hurts, and he favors it, the other knee may bleed. If he uses crutches, his elbows bleed. If his knee is painful, he flexes to decrease the pain. The bleeding stops when the capsule is so distended that it equalizes the blood pressure, which causes ischemia at some points in the joint. Repeated insults cause more and more deformity, which becomes worse and worse, causing the cartilage to grow thinner and thinner. Simultaneously, joint fluid becomes thinner and lubricates less and less. Therefore, the joint becomes rough, deformed, swollen, and flexed; the flexor muscles become tight and short; the extensors become weakened, too long, and atrophied. The advent of antihemophilic concentrates has slightly decreased the severity of the problems described, but repeated insults still cause chronic synovitis, resulting in swollen joints without pain and accompanied by gradual joint destruction. Intraosseous or subperiosteal bleeding within the mandible or long bones also often results in cortical plate thinning and pseudotumors. In general, in order to supplement the deficient level of antihemophilic factor on occasions of bleeding due to accidental trauma or anticipated major surgery, clinicians aim for a 50 per cent AHF blood level when providing supplements of this factor. Thirty per cent AHF will suffice in many instances ; however, a higher blood level will more greatly ensure correction. Since AHF is quickly metabolized, a large amount of AI-IF must be administered at one time to stop bleeding, and this is usually maintained until it is certain that adequate healing has taken place. If one increases the AHF level in a severely deficient patient above the 2 per cent level, fairly good prophylaxis for physical therapy and similar activities is achieved. For purposes of measurement and replenishment therapy, one speaks of AHF units, wherein one AHF unit is equivalent to that amount of antihemophilic factor which is found in 1 cc. of fresh, average, normal blood plasma. In replenishment therapy prior to surgical intervention or on occasions of bleeding insults, the patient’s AHF blood level is increased to that amount necessary t,o halt bleeding. In general, a minimum of 30 per cent of the normal factor level is necessary for hemostasis. Concentrates of AHF are necessary hecause if blood plasma were to be used to bring AHF to appropriate levels, inordinately large volumes of plasma would need to be administered. Another reason often given for avoiding the administration of plasma is that 5 to 8 per
cent of patients with classic hemophilia tl~rc~lop ;Intibotlics (know11 as inhibitors i to AHF as a result of antigens found in plasma. (Inhibitor prohlcms arc worsts in children than in older persons.) I”or these patients. plasma transfusions should not he administered; concentrated forms of XI-IF’ supplc‘lncntatioIl shoul~l be used instead. Two types of concentrates are available for treatment of hemophilia A. Cryoprecipitates, the first type, riced to he kept frozen hut have the advantage of coming totally from one donor, thus diminishing the risk of transmitting the virus of hepatitis to the recipient. The second t,vpe of caoncentrate is freeze-dried from the pooled plasma of 2,000 persons. The latter concentrate need not be refrigerated and, thus, may be kept at the hemophilic patient’s home for immediate administration upon hemorrhaging. The risk of including hepatitis virus in plasma pooled from 2,000 persons is quibe high and must be borne in mind when freeze-dried concentrate is used. In this connection, patients who have severe hemophilia develop an attitude of fatalism with regard to hepatitis under the assumption that they will contract the disease, at least in subclinical form, at some time during their lives. However, for patients with mild hemophilia, who may need concentrate administration on only a few occasions in a lifetime, it is logical to use the cryoprecipitate concentrate preparations in order to reduce the risk of hepatitis transmission. Only one concentrate exists for th(j t,reatment of hemophilia B, and this is from large pools of plasma. Therefore, the risk of hepatitis from its use is great. Since it is freeze-dried, its storage and administration are simplified. It is also possible to treat bleeding episodes in patients with hemophilia B with fresh frozen plasma. If the patient started with a 10 per cent cairculating AIIF level, it may he possible in these instances to raise the circulating level bp administration of concentrates to the 30 or 30 per cent level. There is no concentrate available for the treatment of hemophilia C. In Van Willebrand’s disease infusion of plasma has a beneficial effect on platelet adhesion, but only for 6 hours; however, it stimulates the patient’s mechanisms to synthesize his own factor VIII for days. In all instances of significant blood loss, oral iron supplements are beneficial to replace iron for hemoglobin lost due to hemorrhage. Contraindications
for
aspirin,‘4-2t
alcohol,
and
buttock
injections
The normal life of a human blood platelet is 10 days, and during that lo-day period the ingestion of aspirin permanently impairs a platelet’s ability to function in plug formation, Therefore, especially in a hemophilic person, where the lack of factor VIII causes failure of fibrin strands to form, the tendency to bleed without clot formation is greatly increased by the use of aspirin. For similar reasons, products containing aspirin are contraindicated for all persons with bleeding diatheses for at least 1 week prior to any surgical procedure. Pain relief in these patients map be achieved by administration of acetaminophen; for more severe forms of pain, arctaminophen with codeine is available; for extreme pain, Demerol is recommended for hemophilic patients. Of those patients on replenishment therapy, approximately 1.3 per cent will
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contract hepatitis within any given year. Most of these instances will be subclinical cases. Since hepatitis could readily lead to cirrhosis, it is wise to regard alcohol as in a class with hepatotoxic agents contraindicated for these persons. A further admonition is to avoid injections of antibiotics or other substances into the buttocks of all pediatric patients, not only hemophiliacs, because of the ease of perforating a blood vessel in the buttock region. Recommended alternate sites for injections in pediatric patients are the arm or anterior thigh. Oral
surgical
considerations22-24
My apprehension about undertaking orthodontic treatment for my first hemophilic patient was in part due to his need for extractions to achieve a desirable treatment result. My concern proved valid, but for the wrong reason. At that time all severe classic hemophiliacs were hospitalized for extraction procedures, and my patient was no different. He was admitted to a fine university teaching hospital and cared for by the professor of oral surgery, who had extensive experience in treating hemophiliacs; he was given concentrates for many days before and after surgery; he developed hepatitis, which kept him hospitalized for many weeks. The regimen for hemophilic patients today is quite different, in most instances precluding the necessity for hospitalization associated with oral surgery. The procedures devised and employed by Dr. Thomas Mulkey since 1966 have brought these developments about and deserve mention here, because many of our oral surgery colleagues with whom we must collaborate closely in treating these patients are not fully cognizant of the details of the procedures now available to simplify and make management of hemophiliacs more effective. Obviously, of first importance is a complete medical and dental history. In addition to the routine medical history questions which should be asked of the patient, it is important to know if the patient has had surgical procedures or injury involving the mouth or nose. These areas present a most severe challenge to patients with bleeding disorders in the form of days of oozing following any such episode, which presents a significant negative psychological block in the patient’s development. A recent onset of bleeding problems, including bruising, is probably an indication of a lowered level of blood platelets (thrombocytopenia) versus a long history which may be indicative of hemophilia. A medical consultation is required to answer any questions pertaining to type of hemophilia, concentrate used previously, past experiences, etc. A discussion with the patient and his family of the total procedure to be followed is imperative. It is necessary to be aware of the presence of allergies and allergic reactions, medications administered in the past, previous response to plasma concentrates, etc. All roentgenographic information which would be required prior to surgery in otherwise normal persons is required in this instance as well. Whereas formerly all dental extraction patients were hospitalized for this procedure, at the present time extractions are routinely performed on an outpatient basis. On the day of surgery the patient is transfused with plasma or concentrates to elevate the deficient factor blood level. For oral surgery the desirable AHF
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level (50 per cent) can usually be rcachcd by intravenous supl,lelrlentatiotI within half an hour before surgery, and then it is not necessary to tuaint,ain that level unless the patient has a bleeding problem. If there should be an episode of hemorrhaging after extraction, it is possible to provide intravenous AHF concentrate infusions to t,he postextraction patient as an outpatient. The Hemophilia Rehabilitation Center at Orthopaedic Hospital in Los Angeles has even trained a number of individuals to provide such intravenous concentrate infusions for themselves at home at the onset of any bleeding incident. By the time the patient subsequently arrives at the hospital, the hemorrhage is usually already under control. Oxydized cellulose (Surgicel) which has been precut into 1 by :!L$inch strips is broken up into smaller pieces and dipped into and coated with powdered dry bovine thrombin. These are made ready in advance for packing the apical third of extraction sockets. A 30-gauge dental hypodermic needle with bevel against the tooth is used for injection into the periodontal membrane space under pressure. This is done at four points : mesiobuccally, distobuccally, distolingually, and mesiolingually. Anesthesia is immediate and the tooth is removed as atraumatically as possible without reflection of a flap and without. severing the periodontal atta.chments. As in nonhemophilic patients, local anesthesia is not effective where infection is present around the root surfaces of the teeth. In such instances of infection and other occasions requiring general anesthesia, anesthesia is achieved by means of intravenous Brevital sodium without nasoendotracheal intubation, which could cause serious epistaxis and possibly bleeding elsewhere in the respiratory tract. When general anesthesia is employed in the absence of peridental infection, supplementary paracemental local anesthetic injections are used. Because they are all irritants, no packs, splints, or sutures are employed at the surgical site. The dressing described earlier (oxydized regenerated cellulose and bovine thrombin) is placed in the extraction socket and the patient bites on gauze dressing for 8 hours, swallowing all of his own saliva, without rinsing. Caution should be used to avoid administering this agent at times of bleeding in internal organs, such as the bra.in or genitourinary system, where long retention of a clot could lead to serious consequences from sca,r tissue formation. On awakening, the patient is given 5 Crm. of epsilon-aminocaproic acid (Amicar, Lederle) and then 2.5 Cm. every 6 hours for 10 days. The patient is awakened from sleep to maintain this schedule. This drug blocks the body’s nat,ural fibrinolytic mechanisms and is used to promote clotting and to preserve clots longer than they would normally survive in hemophilic patients. Amicar comes in several dosage forms, the easiest of which to administer is a 25 per cent syrup. The 5 Cm. initial dosage level for adults can be achieved merely by taking four teaspoons of the 25 per cent syrup, then two teaspoons subsequently every 6 hours to achieve the 2.5 Cm. dosage required. The dosage employed for children is one half that described for adults. For the first 24 hours following extraction, the patient is given nothing to eat or drink except Amicar and sufficient water for other medicines. For the next 48 hours, cold clear liquids may be taken without flushing or rinsing around
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the mouth. Specifically, water, soft drinks, cool coffee, ancl cool tea are permissible. However, the patient must not ingest anything hot or anything which leaves a sediment. No dairy products may be ingested, because they leave a sediment which tends to break down the clot. The diet during the fourth day and for the next 6 days (a IO-day total) may be pureed or consist of anything which can be swallowed without chewing. The patient shou7d wet be permitted to do any cheuGg whatsoever. Following the initial IO-day period, the patient is allowed to return gradually to his normal dietary habits. No suture has been placed in the extraction site, and no splint protects the clot. Clot protection is solely by diet and Amicar. The clot, can be expected to grow out of the extraction socket in 2 to 3 days until it retracts at or about 7 days postoperatively. If, during the course of the 10 postextraction days, a problem develops with the clotting or clot retent,ion, the diet reverts back to the cold, clear liquid stage and the level of the antihemophilic factor responsible for the particular type of hemophilia possessed by the patient is raised to 50 per cent. During the course of healing, it is not necesary to confine the patient to bedrest. However, limitation of activity is required. The oral surgeon should follow up on the patient’s progress each day by telephone during the healing process. As of one year ago, the foregoing procedure had been used by Dr. Mulkey in 225 patients, with only 46 inpatients, and, of these, only 30 had been admitted to the hospital primarily for extractions. From the latter group, 165 teeth were extracted. Of the 46 inpatients, 16 were persons admitted primarily for other reasons who had teeth extracted as an adjunct. From the 179 outpatients, 379 teeth were extracted. Of these 1’79, only five patients were hospitalized postsurgically, and these were for atypical responses. Pain control is achieved by administration of acetaminophen and codeine. As a general routine, multiple extractions on an outpatient basis are limited to four single-rooted teeth or two multirooted teeth (thus providing a maximum of four bleeding sites to be controlled). If more extractions are necessary, they are done on several outpatient occasions or on an inpatient basis under oral endotracheal anesthesia all at one time. Also, if the procedure requires hospitalization and reflection of a flap is necessary, silk sutures are used and the patient’s factor level is raised to 50 per cent that of the normal. For patients possessing inhibitors, corticosteroids are used for their nonspecific beneficial effects. Steroids are otherwise not routinely effective. Surgery for inhibitor patients is done routinely on an inpatient basis, employing paracemental injections for anesthesia. It is necessary to check the patient for hematuria in these instances (and whenever surgical procedures are performed for any hemophiliac) and to administer steroids and Amicar. It is now possible to make removal of an impacted third molar an elective procedure; removal of four impacted third molars at one time, or even mandibular resection when necessary, is no longer a procedure to be avoided or advised against in hemophilic patients. The treatment of choice in instances of lacerations of the tongue in hemophiliacs is the use of Amicar and concentrates rather than cauterization or
suturing. Suturing may7 be appropriate in deep lip lacerations, but attempts to preserve friable clots within the mouth are not recommended. These merely break away, leaving a wound or cxtractioir site to bleed again. It should be noted that after the age of 28 dentistry is regarded by hemophilic patients as their most important problem. We must therefore continue to strive for improved techniques in hemophilic dental management in order to provide better service than in the past and t,o alleviate such concern. In addition to providing routine dental care, we must be concerned with soft-tissue bleeding and traumatic injury as well as with bleeding on the occasion of the extraction of deciduous and permanent teeth. Consequently, in all instances of head and neck bleeding and infection, there should be no watchful waiting. Treatment must be started early. Whenever bleeding into the floor of the mouth is noted, we should be extremely concerned about the possibility of occlusion of the airway by blood finding its way back into the pharyngeal region and causing respiratory embarrassment. In such circumstances, the patient should be immediately referred for infusions of concentrate. Orthodontic
and
other
dental
proceduresz5-*’
For years we shied away from anything but emergency care for hemophilic patientsZ4, 25 I recall a statement in one of our dental texts of a number of years ago indicating that we should avoid treating hemophilic persons for solely orthodontic reasons, but I could not find the source of that statement when searching the literature for this publication. However, new discoveries and procedures within medicine, dentistry, and orthodontics during the past 10 years invalidate any formerly acceptable reason to avoid orthodontic treatment for the hemophilic patient. Cringival laceration upon formation of pinch bands was apparently never a real hazard (though many of us feared it), but even that minor problem was eliminated with the advent of preformed bands; now successful direct bonding of brackets without bands at all has removed orthodontic appliance fixation almost totally from contact with the gingiva. Stainless and spring steel wires and auxiliary springs make possible less frequent adjustments by acting over longer spans of time. Materials developed through plastics research, which possess strength and elasticity without the need for tucking sharp ends or edges, can now be used instead of wires as ligatures and space closers. In an informal survey of orthodontists in Southern California who were known to have treated one or more hemophilic patients, there was unanimity of opinion that the hemophilic persons in their practices exhibit no greater degree of gingival bleeding as a result of orthodontic treatment than do nonhemophilic patients. We found, however, that hemophilic patients do not, as a rule, maintain good oral hygiene, possibly out of fear of inducing gingival hemorrhage with the toothbrush. Another common observation is that gingival tissue reacts differently from other soft tissues in the hemophilic patient, so that gingival bleeding is really not a problem if good home care is maintained. Finally, the survey disclosed that bleeding caused by an accidental scratch or minor laceration of the gingiva can be stopped by pressure and that clotting usually takes place in approximately 5 minutes. We have employed hypnosis to relax hemophilic patients, and to increase
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their sense of well-being at the beginning of successive treatment visits, in the hope that these mood changes would have a salutary effect on gingival bleeding. Nitrous oxide and oxygen analgesia was used with other hemophiliacs for its suggestive potential in achieving similar favorable changes of mood. Using these means, I have been able to halt gingival bleeding in hemophiliacs within 10 seconds. It appears, however, that such exercises and the use of hypnosis and analgesia are unnecessary in routine dental and orthodontic treatment of hemophiliacs. The salutary effects of hypnosis and analgesia have been replaced in my practice by establishment of a unique rapport with the hemophilic patient, continually inspiring his confidence and reassuring him that WC have been successful in stopping intraoral bleeding “very quickly” in the past, and that if he has any future instances of oral soft-tissue bleeding, we can and will do the same for him. This program of positive suggestion and instilling confidence throughout orthodontic treatment has been a most effective adjunct in rapidly stopping ongoing minor hemorrhages. Despite the availability of the foregoing methods, it is desirable to prevent gingival bleeding before it occurs. Establishing and maintaining healthy gingival tissue is the best means of averting gingival bleeding. To achieve such healthy gingivae, excellent oral hygiene and tissue stimulation are essential. In fact, more brushing and gingival massage are appropriate when gingival bleeding occurs rather than avoidance of the area. The presence of the orthodontic arch wire prevents the normal stimulation of the gingiva by deflecting the food bolus during mastication and often makes access by the toothbrush to the marginal gingiva difficult. Therefore, frequent daily use of the Butler stimulator after brushing for additional gingival stimulation and also for removal of residual materia alba is recommended. Two advantages of the particular stimulator recommended are: (1) that the rubber tip is conical and, therefore, lacking the irritating corners or ribs evident on other stimulators, and (2) that the tip is operative from the end of a thin, angulated neck of a narrow round handle to afford better access than can be provided by a rubber tip at the end of a toothbrush. ChandaZ5 recommended a soft toothbrush, but I have found that my patients derive more benefit from a bristle of medium stiffness along with a method of brushing whereby the handle of the brush rotates apico-occlusally from the mucobuccal fold, so that the bristles stimulate the gingiva and push food debris toward the occlusal surface to be flushed away with vigorous rinsing. IJse of dental floss is not taught to patients with fixed orthodontic appliances in place, but it is taught to hemophiliacs who are nonorthodontic patients. Failure of the hemophiliac to maintain adequate interproximal gingival stimulation may result in oozing or weeping of a tag, strand, or miniflap of gingival papilla between teeth undergoing orthodontic manipulation. This oozing has been observed to persist for more than a week and, therefore, can be frightening to the patient. In these instances a small drop of 25 per cent solution of zinc chloride deftly applied to shrink the tissue and cauterize the oozing site has been successful in alleviating the problem and the patient’s apprehension. The ultrasonic scaler is used as much as possible to remove orthodontic
cement and accumulated calculus hwanse it is less tl*i~nmatic to the gillgi\.il than scaling hp hand. However, hand curettage has its l)ropcr play it1 trchatitrg hemophiliacs when concentrates an(l the preparations matlc i’or surgical procedures are employed. Orthodontic evaluation is usually first appropriate t’ollowing t~mcrgcncc of the mandibular anterior teeth, since the lateral growth of the mandibular dental arch begins to cease about that time (when use of sul,cr~ositiol~illg of cc~phalometric roentgcnographs to show tooth IIl(JveIneI~t apart fUJIn growth changes bccomes valid), and since there is littlc dentally significant further growth of the anterior portion of the mandibular body to be expcctccl. Tt is also clcsirable to evaluate patients from the standpoint of possibly averting or prcvcnt,ing a significant malocclusion at any time that a parent, or dentist recognizes the possibility of a malocclusion developing. It is also important to continue ingcstion of fluoride ion in the diet until the permanent tooth crowns arc totally formed (at approximately age 10 years) and to continue topical fluoride applications until about age 18. These rules of thumb apply to all persons but arc of especially great importance to hemophiliacs because prevention of both tooth decay and malocclusion, which would otherwise result in the necessity of anesthetic injections for dental restorations or extractions, tends to diminish the frequency of these injections which have been a major problem for hemophilic persons. In the past it was advocated that local anesthesia for operative dentistry on the deciduous dentition be avoided2” r 27 because nerve blocks can cause dissecting hemorrhage and because it is possible to complete restorations s~wcessfully on 50 per cent of deciduous teeth without discomfort, since thcg~ have a higher pain threshold than permanent teeth. This is especially true for Class 1, Class III, Class V, and buccal pit lesions prepared with new and sharp hurs. high speed, and light pressure. If the patient experiences discomfort, one of the following alternative approaches is employed : (1) premeditation with analgesics (not for painful procedures) ; (2) paracemental injections for inhibitor patients (may not give profound enough anesthesia for operative procedures) ; (3) infiltration anesthesia is most often used and, where necessary, block anesthesia is administered in the mandible and posterior tuberosity. The latter approaches are used only if the deficient antihemophilic factor is first restored to 50 per cent of the normal level. If a hematoma develops, replacement therapy should continue, with ice on the affected site and administration of Amicar. Occasionally intrapulpal anesthesia is emploged without administration of concentrates. There is, therefore, no longer any need to compromise on performing operative procedures for hemophilic persons. A rubber dam is used t(J retract the gingiva, lips, and tongue, which not only provides a dry, operative field without contamination but also tends to minimize soft-tissue injury. Prior to making cavity preparations, a wedge is placed interproximally, which serves as a, papilla retractor. Where there is excessive soft tissue which could be injured during cavity preparation, gingival retraction cord should also be employed. In use of the ultrasonic scaler, caution should be employed to prevent t,hc
Orthodontics
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open tip of the high-speed vacuum aspirator from sucking up the floor of the mouth, thus causing hematoma formation. The use of a rubber tip is advocated to prevent this from happening. During pulpal therapy, no anesbhetit is necessary with necrotic pulps. However, with exposed vital pulps, an injection intrapulpally should provide sufficiently profound anesthesia. When chronically inflamed tissue in association with loose exfoliating deciduous teeth is evident, these teeth should be extracted and Amicar 1.25 Gm. (1 teaspoonful every 6 hours) should be given. All patients with hemophilia should be regarded as having hepatitis and, therefore, all instruments which get into the tissue and bloodstream during their treatment should be autoclaved. Summary
In this presentation we have contrasted the normal blood-clotting mechanisms with the failure to form blood clots in hemophiliacs due to the absence of protein factors necessary for conversion of prothrombin to thrombin. The statistics, hereditary basis, and long-term disabling consequences of hemophilia to the severely affected patient are described. The systemic means of minimizing severe joint disabilities and serious internal bleeding hazards by employing concentrates of antihemophilic factors to reverse the bleeding defects are discussed. Availability and advantages of the types of concentrates are explained. The fatalistic attitude of hemophiliacs toward hepatitis is discussed, along with admonitions to avoid the use of aspirin, alcohol, and buttock injections. Alternative medications for pain are recommended, and injection sites for pediatric patients are suggested. The details of simplified oral surgical management of hemophilic patients without hospitalization are described, including local anesthetic injection technique, method of performing extractions, general anesthesia technique when indicated, materials for packing of extraction sockets, regimen and precautions in use of Amicar administration for clot maintenance, postoperative diet, and postsurgical activity guidelines. Also noted is the self-administration of intravenous concentrate infusions at home in the event of hemorrhaging, so that bleeding is on the way to being controlled even before the patient reaches the hospital. We avoided orthodontic treatment of hemophilic patients in the past; however, recently developed bracket-fixation techniques and auxiliary aids, along with an enlightened understanding that gingival bleeding is not to be feared, have changed our attitude, and we now treat hemophilic patients in much the same manner as otherwise normal orthodontic patients. Oral hygiene is usually a problem in hemophiliacs. Therefore, much attention is paid to techniques for maintaining gingival health as the best mechanism to avert gingival bleeding. Mention is made of my use of hypnosis and nitrous oxide and oxygen analgesia and their salutary effect on reducing gingival bleeding from 5 minutes to 10 seconds. The beneficial effects of these modalities have been replaced by routinely establishing a unique doctor-patient relationship which inspires the patient’s confidence and relaxes and reassures him. Also
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previously unreported is a method for shrinking an oozing or weeping tag, strand, or miniflap of gingival tissue and cauterizing a11 oozing site between teeth undergoing orthodontic manipulation. Appropriate timing for orthodontic evaluation is discussed, along with arguments in favor of topical and dietary fluoride ion. It is stressed that prevention of tooth decay and malocclusion decreases the frequency of need for dental restorations and extractions and, therefore, decreases the number of local anesthetic injections which have been a major problem for hemophilics. Finally, a discussion of anesthesia for operative dentistry is presented, along with a recommendation that use of a. rubber dam tends to minimize soft-tissue injury and permits the operator to do better dentistry. With use of the current techniques as described in this article, hemophilia should no longer be considered as a contraindication to orthodontic procedures; nor should it be regarded, at least in a dental and orthodontic sense, as a handicapping disease. REFERENCES
1. Grossman, R. C., and Bosma, J. F.: Experimental oral sensory prosthesis, J. Dent. Res. 42: 891, 1963. 2. Grossman, R. C.: Methods for evaluating surface sensory experience in the mouth, J. Dent. Res. 43: 301, 1964. 3. Grossman, B. C.: The sensory innervation of the oral mucosae, J. S. Calif. State Dent. Assoc. 32: X8-133, 1964. experience, Arch. Oral Biol. 4. Grossman, R. C., Hattis, B. F., and Ringel, R. L.: Oral tactile 10: 691-705, 1965. 5. Grossman, R. C., and Hattis, B. F.: The sensory innervation of the oral mucosae, in Bosma, J. F., Editor: Symposium on oral sensation and perception, Springfield, Ill., 1967, Charles C Thomas Publisher, chap. 1, pp. 5-62. 6. Grossman, R. C.: Methods of determining oral tactile experience, in Bosma, J. F., editor: Symposium on oral sensation and perception, Springfield, Ill., 1967, Charles C Thomas, Publisher, chap. 8, pp. 161-181. 7. Bosma, J. F., Grossman, R. C., and Kavanagh, J. F.: Impairment of somesthetic perception and motor function. Chapter 18 in Bosma., J. F., editor: Symposium on oral sensation and perception, Springfield, Ill., 1967, Charles C Thomas, Publisher, chap. 8, pp. 318-335. 8. Bosma, J. F., Grossman, R. C., and Kavanagh, J. F.: Impairment of somesthetic perception and motor function in the oral and pharyngeal area, Neurology 17: 649-658, 1967. 9. Kavanagh, J. F., Bosma, J. F. and Grossman, R. C.: Troubles de l’alimentation et de la parole associes a des defectuosites de la sensation et de la perception buccale, Med. Hyg. (Geneve) 26: 175-177, 1968. 10. Grossman, R. C.: Hemophilia and orthodontic treatment, in Album, M. M., editor: Proceedings of the First International Congress on Dentistry for the Handicapped, Atlantic City, 1971, American Society of Dentistry for the Handicapped, pp. 113-119. 11. Kasper, C. K.: Hematology of hemophilia, Presented at Hemophilia Comprehensive Care Institute, Orthopaedic Hospital, Los Angeles, Calif., February, 1970. 12. Kasper, C. K.: Coagulation disorders, diagnosis and hematologic management, Presented at Seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic Hospital, Los Angeles, Calif., November, 1973. 13. Kasper, C. K.: Hemophilia and hemophilioid disorders, Curr. Ther., pp. 258-263, 1974. 14. Spaet, T. H., and Zucker, M. B.: Mechanism of platelet plug formation and role of adenosine diphosphate, Am. J. Physiol. 206: 1267-1274, 1964. 15. Haslam, R. J.: Role of adenosine diphosphate in the aggregation of human blood platelets by thrombin and by fatty acids, Nature (Lond.) 202: 765-768, 1964.
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16. Woodbury, D. M.: Analgesics and antipyretics, in Goodman, L. S., and Gilman, A., editors: The pharmacological basis of therapeutics, New York, 1965, The Macmillan Company, chap. 17, pp. 312-344. Platelet disfunction-Diff erentiation of a newly 17. Sahud, M. A., and Aggeler, P. M.: recognized primary type from that produced by aspirin, N. Engl. J. Med. 280: 453, 1969. 18. Weiss, H. J., and Aledort, L. M.: Impaired platelet connective tissue reaction in man after aspirin ingestion, Lancet 2: 495-497, 1967. 19. Weiss, H. J., Aledort, L. M., and Cochwa, 5.: The effect of salicylate on the hemostatic properties of platelets in man, J. Clin. Invest. 47: 2169-2180, 1968. 26. O’Brien, J. R.: Effects of salicylates on human platelets, Lancet 1: 779-783, 1968. 21. Kaneshiro, M. M., Milkie, C. H., Jr., Kasper, C. K., and Rapaport, S. I.: Bleeding time after aspirin in disorders of intrinsic clotting, N. Engl. J. Med. 231: 1039-1042, 1969. 22. Mulkey, T. F.: Oral surgery, Presented at Hemophilia Comprehensive Care Institute, Orthopaedic Hospital, Los Angeles, Calif., February, 1970. 23. Mulkey, T. F.: Oral surgery, Presented at Seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic Hospital, Los Angeles, Calif., November, 1973. 24. Mulkey, T. F., and Boone, D. C.: Hemophilia and the dentist’s contribution, J. S. Calif. Dent. Assist. Assoc., pp. 1-4, 1973. 25. Chanda, L.: Orthodontics, Presented at Seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic hospital, Los Angeles, Calif., November, 1973. 26. Chiono, 0.: Operative dentistry, Presented at Hemophilia Comprehensive care Institute, Orthopaedic Hospital, Los Angeles, Calif., February, 1970. 27. Powell, D.: Operative dentistry, Presented at seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic Hospital, Los Angeles, Calif., November, 1973. 6365
Topanga
Canyon
Blud.
(91364)
C. Grossman,
D.M.D.
Woodland Hills, Calif.
A
number of years ago the staff of a regional center for treatment of hemophilic children, knowing of my interest in handicapped persons,l-g referred a hemophilic patient to me for treatment of his severe malocclusion. I attempted to discourage orthodontic treatment for the patient because I was fearful of initiating hemorrhages that would be difficult to control. However, the determination of the patient and his mother gave impetus to a search for improved methods of management of dental problems associated with the disease. The advent of new techniques since my previous publicationl” necessitates this updated report.
One bleeds when one or more blood vessels are cut. The usual reactions which stop the bleeding are: (1) The severed vessel contracts. (2) Blood platelets adhere to each other and the cut surfaces of the vessel, causing a soft platelet plug to form in response to the release of adenosine diphosphate.14> I5 (These are the primary methods for closing superficial blood vessels; in classic hemophilia the bleeding time is normal following cuts of superficial vessels if a platelet plug forms.) (3) The stopping of bleeding in larger or deeper vessels requires that a fibrin clot be formed after the formation of the soft platelet plug. (4) The fibrin clot then retracts because platelets form knots at the intersections of fibrin strands and pull the clot mass into a tight unit. For all this to occur, the presence of thrombin is necessary for fibrin to be formed from fibrinogen. The hemophilic patient differs from other persons, however, in that the necessary blood factors for conversion of prothrombin to thrombin are absent. Thus, without blood supplementation or replenishment, the hemophilic person does not form substantial blood clots. Approximately 80 per cent of affected persons have classic hemophilia, otherwise known as hemophilia A, in which antihemophilic blood plasma factor VIII (AHF) is missing. Another 15 per cent have Christmas disease, or hemophilia B, in which a different protein, factor IX, is absent from the blood. These other391
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wise similar disease cntitics comprise the bulk of the hernophilic population and have an incidence of one in JO thousand males or one in 20 thousantl persons. There are several other types of hemophilia, each of which represents I or ‘L per cent of all hemophilia. Hemophilia C, evident among ;lewish persons, is the result of a deficiency of PTA, otherwise known as factor XT. This is a milder form of the disease and is considered to be more rare because it is not diagnosed as frequently. Finally, the fourth relatively common form of hemophilia, Von Willebrand’s disease, also hereditary, affects both males and females, and is due to a deficiency of factor VIII as well as an absence o-f the cofactor which makes platelets sticky. Hemophilia A and hemophilia B are sex-linked diseases transmitted to the child by a gene on one of his mother’s two X chromosomes. Males have an X and a. Y chromosome, and all male offspring of a male hemophiliac receive his Y chromosome and consequently are normal with respect to this disease. However, the X chromosome of a male hemophiliac is donated to all of his daughters, who thus become carriers of the disorder just as if they received an affected X chromosome from their carrier mother. It should also be recognized that it is statistically possible for all of the female offspring and 50 per cent of the male offspring of a female carrier to be born free of hemophilia. Females not only carry and transmit the responsible genes to their offspring; they may suffer from the disease, but to milder degrees, and therefore are usually not recognized as bleeders. Severe classic hemophiliacs (hemophilia A) usually have less than 2 per cent of the normal circulating antihemophilic factor (AHF) and suffer from internal bleeding and hemorrhage into joints without being aware of any accident which has caused such bleeding. The person with moderate classic hemophilia has more than 2 per cent antihemophilic globulin and suffers joint hemorrhage only in instances of trauma. Persons with mild classic hemophilia have even more antihemophilic globulin and bleed only when subjected to surgical procedures. Since factors VIII and IX do not cross the placental barrier, there is no transfer of protection against hemorrhage from the mother to the infant. Therefore, if the child survives the birth experience without bleeding, he will probably be in no difficulty until he starts learning to walk. Repeated minor falls with attendant bleeding and extravasation of blood into the joints and other body sites are the basis for significant future problems for these patients. Although hemorrhage into the kidneys, gastrointestinal tract, and other internal sites is a major problem for the hemophiliac, brain hemorrhage is the most common cause of death in these persons. Bleeding into joints and soft tissues is a serious chronic and often progressively disabling problem for the hemophiliac. Blood is a foreign body in tissues outside blood vessels and causes irritation of the tissues into which it has leaked, even though it is ultimately resorbed. Thus, recurrent episodes of bleeding serve as chronic insults to the host tissues, resulting in the formation of scar tissue. The progressive scarring of joints often becomes very apparent as the child begins to attend school and matures further. Similarly, repeated extravasation of blood into flexor muscle regions may cause contracture due to scarring.
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Paralysis of muscle may even occur on the occasion of a single severe hemorrhage into a muscle, which causes entrapment of a nerve and consequent pressure on it, or which presses the nerve against a ligament or bone. The first of two kinds of soft-tissue hemorrhage is less important and occurs where pressure from swelling is released by itself (for example, bleeding into the orbit). The second, however, is hemorrhage into a closed space, as in the anterior space of the legs, which can necrose muscles, resulting in scar tissue, contraction, and permanent flexure. In addition, with the constant irritation of repeated bleeding into the leg, its length could be increased unless the bleedings are near a growth center, in which case shortening is the result. It becomes obvious that hemophilia is a crippling disease because it creates difficulty in moving as a result of joint bleeding. Such a patient cannot. use crutches because if one knee hurts, and he favors it, the other knee may bleed. If he uses crutches, his elbows bleed. If his knee is painful, he flexes to decrease the pain. The bleeding stops when the capsule is so distended that it equalizes the blood pressure, which causes ischemia at some points in the joint. Repeated insults cause more and more deformity, which becomes worse and worse, causing the cartilage to grow thinner and thinner. Simultaneously, joint fluid becomes thinner and lubricates less and less. Therefore, the joint becomes rough, deformed, swollen, and flexed; the flexor muscles become tight and short; the extensors become weakened, too long, and atrophied. The advent of antihemophilic concentrates has slightly decreased the severity of the problems described, but repeated insults still cause chronic synovitis, resulting in swollen joints without pain and accompanied by gradual joint destruction. Intraosseous or subperiosteal bleeding within the mandible or long bones also often results in cortical plate thinning and pseudotumors. In general, in order to supplement the deficient level of antihemophilic factor on occasions of bleeding due to accidental trauma or anticipated major surgery, clinicians aim for a 50 per cent AHF blood level when providing supplements of this factor. Thirty per cent AHF will suffice in many instances ; however, a higher blood level will more greatly ensure correction. Since AHF is quickly metabolized, a large amount of AI-IF must be administered at one time to stop bleeding, and this is usually maintained until it is certain that adequate healing has taken place. If one increases the AHF level in a severely deficient patient above the 2 per cent level, fairly good prophylaxis for physical therapy and similar activities is achieved. For purposes of measurement and replenishment therapy, one speaks of AHF units, wherein one AHF unit is equivalent to that amount of antihemophilic factor which is found in 1 cc. of fresh, average, normal blood plasma. In replenishment therapy prior to surgical intervention or on occasions of bleeding insults, the patient’s AHF blood level is increased to that amount necessary t,o halt bleeding. In general, a minimum of 30 per cent of the normal factor level is necessary for hemostasis. Concentrates of AHF are necessary hecause if blood plasma were to be used to bring AHF to appropriate levels, inordinately large volumes of plasma would need to be administered. Another reason often given for avoiding the administration of plasma is that 5 to 8 per
cent of patients with classic hemophilia tl~rc~lop ;Intibotlics (know11 as inhibitors i to AHF as a result of antigens found in plasma. (Inhibitor prohlcms arc worsts in children than in older persons.) I”or these patients. plasma transfusions should not he administered; concentrated forms of XI-IF’ supplc‘lncntatioIl shoul~l be used instead. Two types of concentrates are available for treatment of hemophilia A. Cryoprecipitates, the first type, riced to he kept frozen hut have the advantage of coming totally from one donor, thus diminishing the risk of transmitting the virus of hepatitis to the recipient. The second t,vpe of caoncentrate is freeze-dried from the pooled plasma of 2,000 persons. The latter concentrate need not be refrigerated and, thus, may be kept at the hemophilic patient’s home for immediate administration upon hemorrhaging. The risk of including hepatitis virus in plasma pooled from 2,000 persons is quibe high and must be borne in mind when freeze-dried concentrate is used. In this connection, patients who have severe hemophilia develop an attitude of fatalism with regard to hepatitis under the assumption that they will contract the disease, at least in subclinical form, at some time during their lives. However, for patients with mild hemophilia, who may need concentrate administration on only a few occasions in a lifetime, it is logical to use the cryoprecipitate concentrate preparations in order to reduce the risk of hepatitis transmission. Only one concentrate exists for th(j t,reatment of hemophilia B, and this is from large pools of plasma. Therefore, the risk of hepatitis from its use is great. Since it is freeze-dried, its storage and administration are simplified. It is also possible to treat bleeding episodes in patients with hemophilia B with fresh frozen plasma. If the patient started with a 10 per cent cairculating AIIF level, it may he possible in these instances to raise the circulating level bp administration of concentrates to the 30 or 30 per cent level. There is no concentrate available for the treatment of hemophilia C. In Van Willebrand’s disease infusion of plasma has a beneficial effect on platelet adhesion, but only for 6 hours; however, it stimulates the patient’s mechanisms to synthesize his own factor VIII for days. In all instances of significant blood loss, oral iron supplements are beneficial to replace iron for hemoglobin lost due to hemorrhage. Contraindications
for
aspirin,‘4-2t
alcohol,
and
buttock
injections
The normal life of a human blood platelet is 10 days, and during that lo-day period the ingestion of aspirin permanently impairs a platelet’s ability to function in plug formation, Therefore, especially in a hemophilic person, where the lack of factor VIII causes failure of fibrin strands to form, the tendency to bleed without clot formation is greatly increased by the use of aspirin. For similar reasons, products containing aspirin are contraindicated for all persons with bleeding diatheses for at least 1 week prior to any surgical procedure. Pain relief in these patients map be achieved by administration of acetaminophen; for more severe forms of pain, arctaminophen with codeine is available; for extreme pain, Demerol is recommended for hemophilic patients. Of those patients on replenishment therapy, approximately 1.3 per cent will
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contract hepatitis within any given year. Most of these instances will be subclinical cases. Since hepatitis could readily lead to cirrhosis, it is wise to regard alcohol as in a class with hepatotoxic agents contraindicated for these persons. A further admonition is to avoid injections of antibiotics or other substances into the buttocks of all pediatric patients, not only hemophiliacs, because of the ease of perforating a blood vessel in the buttock region. Recommended alternate sites for injections in pediatric patients are the arm or anterior thigh. Oral
surgical
considerations22-24
My apprehension about undertaking orthodontic treatment for my first hemophilic patient was in part due to his need for extractions to achieve a desirable treatment result. My concern proved valid, but for the wrong reason. At that time all severe classic hemophiliacs were hospitalized for extraction procedures, and my patient was no different. He was admitted to a fine university teaching hospital and cared for by the professor of oral surgery, who had extensive experience in treating hemophiliacs; he was given concentrates for many days before and after surgery; he developed hepatitis, which kept him hospitalized for many weeks. The regimen for hemophilic patients today is quite different, in most instances precluding the necessity for hospitalization associated with oral surgery. The procedures devised and employed by Dr. Thomas Mulkey since 1966 have brought these developments about and deserve mention here, because many of our oral surgery colleagues with whom we must collaborate closely in treating these patients are not fully cognizant of the details of the procedures now available to simplify and make management of hemophiliacs more effective. Obviously, of first importance is a complete medical and dental history. In addition to the routine medical history questions which should be asked of the patient, it is important to know if the patient has had surgical procedures or injury involving the mouth or nose. These areas present a most severe challenge to patients with bleeding disorders in the form of days of oozing following any such episode, which presents a significant negative psychological block in the patient’s development. A recent onset of bleeding problems, including bruising, is probably an indication of a lowered level of blood platelets (thrombocytopenia) versus a long history which may be indicative of hemophilia. A medical consultation is required to answer any questions pertaining to type of hemophilia, concentrate used previously, past experiences, etc. A discussion with the patient and his family of the total procedure to be followed is imperative. It is necessary to be aware of the presence of allergies and allergic reactions, medications administered in the past, previous response to plasma concentrates, etc. All roentgenographic information which would be required prior to surgery in otherwise normal persons is required in this instance as well. Whereas formerly all dental extraction patients were hospitalized for this procedure, at the present time extractions are routinely performed on an outpatient basis. On the day of surgery the patient is transfused with plasma or concentrates to elevate the deficient factor blood level. For oral surgery the desirable AHF
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level (50 per cent) can usually be rcachcd by intravenous supl,lelrlentatiotI within half an hour before surgery, and then it is not necessary to tuaint,ain that level unless the patient has a bleeding problem. If there should be an episode of hemorrhaging after extraction, it is possible to provide intravenous AHF concentrate infusions to t,he postextraction patient as an outpatient. The Hemophilia Rehabilitation Center at Orthopaedic Hospital in Los Angeles has even trained a number of individuals to provide such intravenous concentrate infusions for themselves at home at the onset of any bleeding incident. By the time the patient subsequently arrives at the hospital, the hemorrhage is usually already under control. Oxydized cellulose (Surgicel) which has been precut into 1 by :!L$inch strips is broken up into smaller pieces and dipped into and coated with powdered dry bovine thrombin. These are made ready in advance for packing the apical third of extraction sockets. A 30-gauge dental hypodermic needle with bevel against the tooth is used for injection into the periodontal membrane space under pressure. This is done at four points : mesiobuccally, distobuccally, distolingually, and mesiolingually. Anesthesia is immediate and the tooth is removed as atraumatically as possible without reflection of a flap and without. severing the periodontal atta.chments. As in nonhemophilic patients, local anesthesia is not effective where infection is present around the root surfaces of the teeth. In such instances of infection and other occasions requiring general anesthesia, anesthesia is achieved by means of intravenous Brevital sodium without nasoendotracheal intubation, which could cause serious epistaxis and possibly bleeding elsewhere in the respiratory tract. When general anesthesia is employed in the absence of peridental infection, supplementary paracemental local anesthetic injections are used. Because they are all irritants, no packs, splints, or sutures are employed at the surgical site. The dressing described earlier (oxydized regenerated cellulose and bovine thrombin) is placed in the extraction socket and the patient bites on gauze dressing for 8 hours, swallowing all of his own saliva, without rinsing. Caution should be used to avoid administering this agent at times of bleeding in internal organs, such as the bra.in or genitourinary system, where long retention of a clot could lead to serious consequences from sca,r tissue formation. On awakening, the patient is given 5 Crm. of epsilon-aminocaproic acid (Amicar, Lederle) and then 2.5 Cm. every 6 hours for 10 days. The patient is awakened from sleep to maintain this schedule. This drug blocks the body’s nat,ural fibrinolytic mechanisms and is used to promote clotting and to preserve clots longer than they would normally survive in hemophilic patients. Amicar comes in several dosage forms, the easiest of which to administer is a 25 per cent syrup. The 5 Cm. initial dosage level for adults can be achieved merely by taking four teaspoons of the 25 per cent syrup, then two teaspoons subsequently every 6 hours to achieve the 2.5 Cm. dosage required. The dosage employed for children is one half that described for adults. For the first 24 hours following extraction, the patient is given nothing to eat or drink except Amicar and sufficient water for other medicines. For the next 48 hours, cold clear liquids may be taken without flushing or rinsing around
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the mouth. Specifically, water, soft drinks, cool coffee, ancl cool tea are permissible. However, the patient must not ingest anything hot or anything which leaves a sediment. No dairy products may be ingested, because they leave a sediment which tends to break down the clot. The diet during the fourth day and for the next 6 days (a IO-day total) may be pureed or consist of anything which can be swallowed without chewing. The patient shou7d wet be permitted to do any cheuGg whatsoever. Following the initial IO-day period, the patient is allowed to return gradually to his normal dietary habits. No suture has been placed in the extraction site, and no splint protects the clot. Clot protection is solely by diet and Amicar. The clot, can be expected to grow out of the extraction socket in 2 to 3 days until it retracts at or about 7 days postoperatively. If, during the course of the 10 postextraction days, a problem develops with the clotting or clot retent,ion, the diet reverts back to the cold, clear liquid stage and the level of the antihemophilic factor responsible for the particular type of hemophilia possessed by the patient is raised to 50 per cent. During the course of healing, it is not necesary to confine the patient to bedrest. However, limitation of activity is required. The oral surgeon should follow up on the patient’s progress each day by telephone during the healing process. As of one year ago, the foregoing procedure had been used by Dr. Mulkey in 225 patients, with only 46 inpatients, and, of these, only 30 had been admitted to the hospital primarily for extractions. From the latter group, 165 teeth were extracted. Of the 46 inpatients, 16 were persons admitted primarily for other reasons who had teeth extracted as an adjunct. From the 179 outpatients, 379 teeth were extracted. Of these 1’79, only five patients were hospitalized postsurgically, and these were for atypical responses. Pain control is achieved by administration of acetaminophen and codeine. As a general routine, multiple extractions on an outpatient basis are limited to four single-rooted teeth or two multirooted teeth (thus providing a maximum of four bleeding sites to be controlled). If more extractions are necessary, they are done on several outpatient occasions or on an inpatient basis under oral endotracheal anesthesia all at one time. Also, if the procedure requires hospitalization and reflection of a flap is necessary, silk sutures are used and the patient’s factor level is raised to 50 per cent that of the normal. For patients possessing inhibitors, corticosteroids are used for their nonspecific beneficial effects. Steroids are otherwise not routinely effective. Surgery for inhibitor patients is done routinely on an inpatient basis, employing paracemental injections for anesthesia. It is necessary to check the patient for hematuria in these instances (and whenever surgical procedures are performed for any hemophiliac) and to administer steroids and Amicar. It is now possible to make removal of an impacted third molar an elective procedure; removal of four impacted third molars at one time, or even mandibular resection when necessary, is no longer a procedure to be avoided or advised against in hemophilic patients. The treatment of choice in instances of lacerations of the tongue in hemophiliacs is the use of Amicar and concentrates rather than cauterization or
suturing. Suturing may7 be appropriate in deep lip lacerations, but attempts to preserve friable clots within the mouth are not recommended. These merely break away, leaving a wound or cxtractioir site to bleed again. It should be noted that after the age of 28 dentistry is regarded by hemophilic patients as their most important problem. We must therefore continue to strive for improved techniques in hemophilic dental management in order to provide better service than in the past and t,o alleviate such concern. In addition to providing routine dental care, we must be concerned with soft-tissue bleeding and traumatic injury as well as with bleeding on the occasion of the extraction of deciduous and permanent teeth. Consequently, in all instances of head and neck bleeding and infection, there should be no watchful waiting. Treatment must be started early. Whenever bleeding into the floor of the mouth is noted, we should be extremely concerned about the possibility of occlusion of the airway by blood finding its way back into the pharyngeal region and causing respiratory embarrassment. In such circumstances, the patient should be immediately referred for infusions of concentrate. Orthodontic
and
other
dental
proceduresz5-*’
For years we shied away from anything but emergency care for hemophilic patientsZ4, 25 I recall a statement in one of our dental texts of a number of years ago indicating that we should avoid treating hemophilic persons for solely orthodontic reasons, but I could not find the source of that statement when searching the literature for this publication. However, new discoveries and procedures within medicine, dentistry, and orthodontics during the past 10 years invalidate any formerly acceptable reason to avoid orthodontic treatment for the hemophilic patient. Cringival laceration upon formation of pinch bands was apparently never a real hazard (though many of us feared it), but even that minor problem was eliminated with the advent of preformed bands; now successful direct bonding of brackets without bands at all has removed orthodontic appliance fixation almost totally from contact with the gingiva. Stainless and spring steel wires and auxiliary springs make possible less frequent adjustments by acting over longer spans of time. Materials developed through plastics research, which possess strength and elasticity without the need for tucking sharp ends or edges, can now be used instead of wires as ligatures and space closers. In an informal survey of orthodontists in Southern California who were known to have treated one or more hemophilic patients, there was unanimity of opinion that the hemophilic persons in their practices exhibit no greater degree of gingival bleeding as a result of orthodontic treatment than do nonhemophilic patients. We found, however, that hemophilic patients do not, as a rule, maintain good oral hygiene, possibly out of fear of inducing gingival hemorrhage with the toothbrush. Another common observation is that gingival tissue reacts differently from other soft tissues in the hemophilic patient, so that gingival bleeding is really not a problem if good home care is maintained. Finally, the survey disclosed that bleeding caused by an accidental scratch or minor laceration of the gingiva can be stopped by pressure and that clotting usually takes place in approximately 5 minutes. We have employed hypnosis to relax hemophilic patients, and to increase
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their sense of well-being at the beginning of successive treatment visits, in the hope that these mood changes would have a salutary effect on gingival bleeding. Nitrous oxide and oxygen analgesia was used with other hemophiliacs for its suggestive potential in achieving similar favorable changes of mood. Using these means, I have been able to halt gingival bleeding in hemophiliacs within 10 seconds. It appears, however, that such exercises and the use of hypnosis and analgesia are unnecessary in routine dental and orthodontic treatment of hemophiliacs. The salutary effects of hypnosis and analgesia have been replaced in my practice by establishment of a unique rapport with the hemophilic patient, continually inspiring his confidence and reassuring him that WC have been successful in stopping intraoral bleeding “very quickly” in the past, and that if he has any future instances of oral soft-tissue bleeding, we can and will do the same for him. This program of positive suggestion and instilling confidence throughout orthodontic treatment has been a most effective adjunct in rapidly stopping ongoing minor hemorrhages. Despite the availability of the foregoing methods, it is desirable to prevent gingival bleeding before it occurs. Establishing and maintaining healthy gingival tissue is the best means of averting gingival bleeding. To achieve such healthy gingivae, excellent oral hygiene and tissue stimulation are essential. In fact, more brushing and gingival massage are appropriate when gingival bleeding occurs rather than avoidance of the area. The presence of the orthodontic arch wire prevents the normal stimulation of the gingiva by deflecting the food bolus during mastication and often makes access by the toothbrush to the marginal gingiva difficult. Therefore, frequent daily use of the Butler stimulator after brushing for additional gingival stimulation and also for removal of residual materia alba is recommended. Two advantages of the particular stimulator recommended are: (1) that the rubber tip is conical and, therefore, lacking the irritating corners or ribs evident on other stimulators, and (2) that the tip is operative from the end of a thin, angulated neck of a narrow round handle to afford better access than can be provided by a rubber tip at the end of a toothbrush. ChandaZ5 recommended a soft toothbrush, but I have found that my patients derive more benefit from a bristle of medium stiffness along with a method of brushing whereby the handle of the brush rotates apico-occlusally from the mucobuccal fold, so that the bristles stimulate the gingiva and push food debris toward the occlusal surface to be flushed away with vigorous rinsing. IJse of dental floss is not taught to patients with fixed orthodontic appliances in place, but it is taught to hemophiliacs who are nonorthodontic patients. Failure of the hemophiliac to maintain adequate interproximal gingival stimulation may result in oozing or weeping of a tag, strand, or miniflap of gingival papilla between teeth undergoing orthodontic manipulation. This oozing has been observed to persist for more than a week and, therefore, can be frightening to the patient. In these instances a small drop of 25 per cent solution of zinc chloride deftly applied to shrink the tissue and cauterize the oozing site has been successful in alleviating the problem and the patient’s apprehension. The ultrasonic scaler is used as much as possible to remove orthodontic
cement and accumulated calculus hwanse it is less tl*i~nmatic to the gillgi\.il than scaling hp hand. However, hand curettage has its l)ropcr play it1 trchatitrg hemophiliacs when concentrates an(l the preparations matlc i’or surgical procedures are employed. Orthodontic evaluation is usually first appropriate t’ollowing t~mcrgcncc of the mandibular anterior teeth, since the lateral growth of the mandibular dental arch begins to cease about that time (when use of sul,cr~ositiol~illg of cc~phalometric roentgcnographs to show tooth IIl(JveIneI~t apart fUJIn growth changes bccomes valid), and since there is littlc dentally significant further growth of the anterior portion of the mandibular body to be expcctccl. Tt is also clcsirable to evaluate patients from the standpoint of possibly averting or prcvcnt,ing a significant malocclusion at any time that a parent, or dentist recognizes the possibility of a malocclusion developing. It is also important to continue ingcstion of fluoride ion in the diet until the permanent tooth crowns arc totally formed (at approximately age 10 years) and to continue topical fluoride applications until about age 18. These rules of thumb apply to all persons but arc of especially great importance to hemophiliacs because prevention of both tooth decay and malocclusion, which would otherwise result in the necessity of anesthetic injections for dental restorations or extractions, tends to diminish the frequency of these injections which have been a major problem for hemophilic persons. In the past it was advocated that local anesthesia for operative dentistry on the deciduous dentition be avoided2” r 27 because nerve blocks can cause dissecting hemorrhage and because it is possible to complete restorations s~wcessfully on 50 per cent of deciduous teeth without discomfort, since thcg~ have a higher pain threshold than permanent teeth. This is especially true for Class 1, Class III, Class V, and buccal pit lesions prepared with new and sharp hurs. high speed, and light pressure. If the patient experiences discomfort, one of the following alternative approaches is employed : (1) premeditation with analgesics (not for painful procedures) ; (2) paracemental injections for inhibitor patients (may not give profound enough anesthesia for operative procedures) ; (3) infiltration anesthesia is most often used and, where necessary, block anesthesia is administered in the mandible and posterior tuberosity. The latter approaches are used only if the deficient antihemophilic factor is first restored to 50 per cent of the normal level. If a hematoma develops, replacement therapy should continue, with ice on the affected site and administration of Amicar. Occasionally intrapulpal anesthesia is emploged without administration of concentrates. There is, therefore, no longer any need to compromise on performing operative procedures for hemophilic persons. A rubber dam is used t(J retract the gingiva, lips, and tongue, which not only provides a dry, operative field without contamination but also tends to minimize soft-tissue injury. Prior to making cavity preparations, a wedge is placed interproximally, which serves as a, papilla retractor. Where there is excessive soft tissue which could be injured during cavity preparation, gingival retraction cord should also be employed. In use of the ultrasonic scaler, caution should be employed to prevent t,hc
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open tip of the high-speed vacuum aspirator from sucking up the floor of the mouth, thus causing hematoma formation. The use of a rubber tip is advocated to prevent this from happening. During pulpal therapy, no anesbhetit is necessary with necrotic pulps. However, with exposed vital pulps, an injection intrapulpally should provide sufficiently profound anesthesia. When chronically inflamed tissue in association with loose exfoliating deciduous teeth is evident, these teeth should be extracted and Amicar 1.25 Gm. (1 teaspoonful every 6 hours) should be given. All patients with hemophilia should be regarded as having hepatitis and, therefore, all instruments which get into the tissue and bloodstream during their treatment should be autoclaved. Summary
In this presentation we have contrasted the normal blood-clotting mechanisms with the failure to form blood clots in hemophiliacs due to the absence of protein factors necessary for conversion of prothrombin to thrombin. The statistics, hereditary basis, and long-term disabling consequences of hemophilia to the severely affected patient are described. The systemic means of minimizing severe joint disabilities and serious internal bleeding hazards by employing concentrates of antihemophilic factors to reverse the bleeding defects are discussed. Availability and advantages of the types of concentrates are explained. The fatalistic attitude of hemophiliacs toward hepatitis is discussed, along with admonitions to avoid the use of aspirin, alcohol, and buttock injections. Alternative medications for pain are recommended, and injection sites for pediatric patients are suggested. The details of simplified oral surgical management of hemophilic patients without hospitalization are described, including local anesthetic injection technique, method of performing extractions, general anesthesia technique when indicated, materials for packing of extraction sockets, regimen and precautions in use of Amicar administration for clot maintenance, postoperative diet, and postsurgical activity guidelines. Also noted is the self-administration of intravenous concentrate infusions at home in the event of hemorrhaging, so that bleeding is on the way to being controlled even before the patient reaches the hospital. We avoided orthodontic treatment of hemophilic patients in the past; however, recently developed bracket-fixation techniques and auxiliary aids, along with an enlightened understanding that gingival bleeding is not to be feared, have changed our attitude, and we now treat hemophilic patients in much the same manner as otherwise normal orthodontic patients. Oral hygiene is usually a problem in hemophiliacs. Therefore, much attention is paid to techniques for maintaining gingival health as the best mechanism to avert gingival bleeding. Mention is made of my use of hypnosis and nitrous oxide and oxygen analgesia and their salutary effect on reducing gingival bleeding from 5 minutes to 10 seconds. The beneficial effects of these modalities have been replaced by routinely establishing a unique doctor-patient relationship which inspires the patient’s confidence and relaxes and reassures him. Also
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previously unreported is a method for shrinking an oozing or weeping tag, strand, or miniflap of gingival tissue and cauterizing a11 oozing site between teeth undergoing orthodontic manipulation. Appropriate timing for orthodontic evaluation is discussed, along with arguments in favor of topical and dietary fluoride ion. It is stressed that prevention of tooth decay and malocclusion decreases the frequency of need for dental restorations and extractions and, therefore, decreases the number of local anesthetic injections which have been a major problem for hemophilics. Finally, a discussion of anesthesia for operative dentistry is presented, along with a recommendation that use of a. rubber dam tends to minimize soft-tissue injury and permits the operator to do better dentistry. With use of the current techniques as described in this article, hemophilia should no longer be considered as a contraindication to orthodontic procedures; nor should it be regarded, at least in a dental and orthodontic sense, as a handicapping disease. REFERENCES
1. Grossman, R. C., and Bosma, J. F.: Experimental oral sensory prosthesis, J. Dent. Res. 42: 891, 1963. 2. Grossman, R. C.: Methods for evaluating surface sensory experience in the mouth, J. Dent. Res. 43: 301, 1964. 3. Grossman, B. C.: The sensory innervation of the oral mucosae, J. S. Calif. State Dent. Assoc. 32: X8-133, 1964. experience, Arch. Oral Biol. 4. Grossman, R. C., Hattis, B. F., and Ringel, R. L.: Oral tactile 10: 691-705, 1965. 5. Grossman, R. C., and Hattis, B. F.: The sensory innervation of the oral mucosae, in Bosma, J. F., Editor: Symposium on oral sensation and perception, Springfield, Ill., 1967, Charles C Thomas Publisher, chap. 1, pp. 5-62. 6. Grossman, R. C.: Methods of determining oral tactile experience, in Bosma, J. F., editor: Symposium on oral sensation and perception, Springfield, Ill., 1967, Charles C Thomas, Publisher, chap. 8, pp. 161-181. 7. Bosma, J. F., Grossman, R. C., and Kavanagh, J. F.: Impairment of somesthetic perception and motor function. Chapter 18 in Bosma., J. F., editor: Symposium on oral sensation and perception, Springfield, Ill., 1967, Charles C Thomas, Publisher, chap. 8, pp. 318-335. 8. Bosma, J. F., Grossman, R. C., and Kavanagh, J. F.: Impairment of somesthetic perception and motor function in the oral and pharyngeal area, Neurology 17: 649-658, 1967. 9. Kavanagh, J. F., Bosma, J. F. and Grossman, R. C.: Troubles de l’alimentation et de la parole associes a des defectuosites de la sensation et de la perception buccale, Med. Hyg. (Geneve) 26: 175-177, 1968. 10. Grossman, R. C.: Hemophilia and orthodontic treatment, in Album, M. M., editor: Proceedings of the First International Congress on Dentistry for the Handicapped, Atlantic City, 1971, American Society of Dentistry for the Handicapped, pp. 113-119. 11. Kasper, C. K.: Hematology of hemophilia, Presented at Hemophilia Comprehensive Care Institute, Orthopaedic Hospital, Los Angeles, Calif., February, 1970. 12. Kasper, C. K.: Coagulation disorders, diagnosis and hematologic management, Presented at Seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic Hospital, Los Angeles, Calif., November, 1973. 13. Kasper, C. K.: Hemophilia and hemophilioid disorders, Curr. Ther., pp. 258-263, 1974. 14. Spaet, T. H., and Zucker, M. B.: Mechanism of platelet plug formation and role of adenosine diphosphate, Am. J. Physiol. 206: 1267-1274, 1964. 15. Haslam, R. J.: Role of adenosine diphosphate in the aggregation of human blood platelets by thrombin and by fatty acids, Nature (Lond.) 202: 765-768, 1964.
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16. Woodbury, D. M.: Analgesics and antipyretics, in Goodman, L. S., and Gilman, A., editors: The pharmacological basis of therapeutics, New York, 1965, The Macmillan Company, chap. 17, pp. 312-344. Platelet disfunction-Diff erentiation of a newly 17. Sahud, M. A., and Aggeler, P. M.: recognized primary type from that produced by aspirin, N. Engl. J. Med. 280: 453, 1969. 18. Weiss, H. J., and Aledort, L. M.: Impaired platelet connective tissue reaction in man after aspirin ingestion, Lancet 2: 495-497, 1967. 19. Weiss, H. J., Aledort, L. M., and Cochwa, 5.: The effect of salicylate on the hemostatic properties of platelets in man, J. Clin. Invest. 47: 2169-2180, 1968. 26. O’Brien, J. R.: Effects of salicylates on human platelets, Lancet 1: 779-783, 1968. 21. Kaneshiro, M. M., Milkie, C. H., Jr., Kasper, C. K., and Rapaport, S. I.: Bleeding time after aspirin in disorders of intrinsic clotting, N. Engl. J. Med. 231: 1039-1042, 1969. 22. Mulkey, T. F.: Oral surgery, Presented at Hemophilia Comprehensive Care Institute, Orthopaedic Hospital, Los Angeles, Calif., February, 1970. 23. Mulkey, T. F.: Oral surgery, Presented at Seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic Hospital, Los Angeles, Calif., November, 1973. 24. Mulkey, T. F., and Boone, D. C.: Hemophilia and the dentist’s contribution, J. S. Calif. Dent. Assist. Assoc., pp. 1-4, 1973. 25. Chanda, L.: Orthodontics, Presented at Seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic hospital, Los Angeles, Calif., November, 1973. 26. Chiono, 0.: Operative dentistry, Presented at Hemophilia Comprehensive care Institute, Orthopaedic Hospital, Los Angeles, Calif., February, 1970. 27. Powell, D.: Operative dentistry, Presented at seminar on Dental Treatment of the Hemophilic Patient, Orthopaedic Hospital, Los Angeles, Calif., November, 1973. 6365
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