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Dental anesthetic management of epidermolysis bullosa: A new approach
oral medicine Editor: JAMES W. LITTLE,
D.M.D.,
M.S.D.
Chairman and Professor Department of Oral Diagnosis and Oral Medicine University of Kentucky Lexington, Kentucky 40506
Dental anesthetic management of epidermolysis bullosa: A new approach DEPARTXEIVT UNIVERSITY
OF OF
PEDODONTICS,
COLLEGE
OF
DENTISTRY,
KESTUCKY
Epidermolysis bullosa is a rare blister-producing skin disorder that has been of some concern to dentists in the past. Dental treatment has been attempted on an outpatient ljasis with local anesthesia. In some of these patients, local anesthesia has caused large intraoral bullne. Other methods of treatment have been sought, including the use of general anesthesia and intramuscular sedation. This is a report of a case in which intravenous k&amine hydrochloride was used with good results. TVe believe this method has much to offer in the treatment of these patients.
E
pidermolysis bullosa is a rare blister-producing dermatologic disorder. These blisters or vesicles appear at sites of mild trauma or friction. The disease is subdivided according to severity : (Weber-Cockayne syndrome) is a less severe 1. The simplex type autosomal dominant type. It does not leave scars and does not affect the mucous membrane. 2. The dystrophic type (Goldschneider’s syndrome) can take several forms, varying in degree of severity : 1. The dominant auosomal type produces scars on healing. The mucous membranes are affected. It does not interfere with growth and development. 2. The recessive form is more severe and leaves scars. This form is usually associated with other congenital defects, including mental retardation and retarded physical growth. *Formerly Associate Director, Dental Services, Children’s Hospital of Pittsburgh, and Assistant Professor, School of Dental Medicine and School of Medicine, University of Pittsburgh. 732
Volume 40 Number 6
Anesthetic
managemeqlt of epidermolysis
bullosa
733
3. The lethal form is rare. The patient usually dies early in the first few months.’ The most notable feature of the disease is the production of bullae or vesicles at sites of slight trauma. The hands, feet, fingers, and elbows are most commonly involved. When a bulla is broken or peeled off, a raw surface is exposed. CASE REPORT On March 20, 1973, a 5?¥r-old Caucasian boy was brought to the dental clinic for routine dental care. The patient had a medical history of epidermolysis bullosa-dystrophic type. An intraoral examination revealed lmlla formation lingual to the lower anterior teeth and in the mucobuccal fold on hoth left nnd right sides. Oral hygiene was poor, with large amounts of plaque present. The mother explained that the child “was afraid to brush because he produced painful blisters in his mouth.” There were numerous carious lesions, and the mandihular deciduous molars were badly Ijroken down, with abscess formation. The child had previously been seen by a local dentist who declined to treat him because of bulla formation after administration of a local anesthetic. Consultations were secured from the Anesthesia, Dermatology, and Pediatrics Departments. A decision was made to carry out treatment with intravenous sedation. The results of laboratory studies were within normal limits. The patient received steroid coverage the day before, the day of, and the day after the procedure. Antibiotic coverage was provided on tile day of the procedure and for 6 days thereafter. The patient weighed 20 kilogrnms. No premeditation was used. The patient was placed on the dental chair in the dental operating room. A precordial stethoscope was placed, and a blood pressure cuff was placed over a gauze-covered arm. An intravenous site was secured, and ketamine hydrochloride was infused along with intravenous fluids. A total of 150 mg. ketamine hydrochloride was used (i.5 mg. per kilogram of body weight). The rubber dam was used carefully. Four deciduous teeth were restored and four mere extracted. The patient’s vital signs were closely monitored. On the advice of the Pediatrics Department, the patient was retained overnight for obsrrvation to be sure that no hullae formed intraorally to compromise his airway. The bed had a lamb’s wool blanket. The postoperative course was uneventful, and the patient was discharged the following morning. The patient was seen 1 week postoperatively. The extraction sites were healing slomly. Numerous oral lmllac were present. Bands were fitted on the mandibular permanent molars, and a lingual arch was constructed. The child was plnced on a 3-month recall. Six months later, as a result of the poor oral hygiene, several new carious lesions had developed. A decision was made to perform another intravenous procedure. The second procedure was carried out exactly the same as the first. Lahoratory studies were secured. Restorations were placed in four deciduous and three permanent teeth. The lingual arch was removed, the teeth were cleaned, fluoride was applied, and the appliance was recemented. A total of 7.5 mg. of ketamine hydrochloride per kilogram (145 mg. total) was used. The postsurgical course was uneventful. The patient was retained for several hours hecause of the long recovery time from ketamine hydrochloride. When the patient was seen 1 week later, numerous painful oral hullae mere present. The patient was placed on a regular 3month recall. The importance of better oral hygiene, conditions permitting, was emphasized to the child and his mother.
DISCUSSION
Lesions of the mucous membranes of the mouth and tongue are usually present in the dystrophic form .2 Clinically, in the mouth we may see both clear and hemorrhagic bullae, white scars, and a nonspecific gingivitis.3 Other
Oral Hurg. December, 1975
oral structures may bc affected. Tooth brushing may bc painful and may also l~rotlucc oral bullac~. Home carr is a problem. The production of oral bullae is a consideration in clental anesthetic management. A review of the literature reveals past problems associated with dental anesthetic management. Local anesthesia has proved successful in some cases, but other cases have required another form of dental anesthetic management because of behavior problems or excessive oral bulla formation after the administration of a local anesthetic. The use of general anesthesia may involve long hospital stays, a post operative course of concern, and a slow recovery. General anesthesia with various modifications has been accomplishd in several patients with varying results. 4-6 Bullae were usually present postoperatively as a result of anesthetic management or surgical manipulation. The possibility of the formation of large oral bullae so as to compromise the patient’s airway has always been of concern. R,ecently, ketamine has found favor in the treatment of these patients because it can produce good sedation without a reduction in respiration. Most have given the drug by the intramuscular route because of fear of continued trauma with an intravenous site.?? 8 In the case presented here good dental sedation without local anesthesia was accomplished by the intravenous route. We have proposed this route of administration because it offers a more controlled drug dosage and the presence of an intravenous site if necessary. The proper amount of drug can be administered to achieve optimal levels of sedation with the least drug dosage. SUMMARY
We believe that intravenous dental sedation management of patients with epidermolysis bullosa has several advantages to offer over intramuscular or oral sedation management : 1. Local anesthesia is not necessary. 2. The lowest drug dosage that can produce good dental sedation can be given. 3. Increased amounts o’f the drug can easily be added. 4. An intravenous site is available in case of emergency. 5. Patients can be treated on an outpatient basis when reliable home care is available. 6. This method did not cause undue or unexpected bulla formation with the continuous intravenous site. Special thanks are extended to Dr. Joseph Marcy and Dr. Paul Gaffney Hospital of Pittsburgh for their aid and counsel in this case.
of the Children’s
REFERENCES
1. Gorlin, X. J., and Pindborg, J. J.: Syndromes of the Head and Neck, New York, 1964, MCGravv-Hill Book Company, Inc., p. 208. 2. Tobias, H.: Lesions of the Mucous Membrane in a Case of Epidermolysis Bullosa, J. Pediatr. 47: 750-751, 1955. 3. Winstock, Donald: Oral Aspects of Epidermolysis Bullosa, Br. J. Dermatol. 74: 432438, 1962. 4. Boyer, Harold E., and Owens, Robert H.: Epidermolysis Bullosa: A Rare Disease of Dental Interest, ORAL SURG. 14: 1170-1177, 1961.
Volume 40 Number 6
Anesthetic
ma,nagement of epidermolysis
bullosa
735
5. Marshall, B. E.: A Comment on Epidermolysis Bullosa and Its Anesthetic Management for Dental Operations, Br. J. Anaesth. 35: 724-727, 1963. F. Wilson, Frank : Epidermolysis Bullosa : A Rare Disease of Anaesthetic Interest, Br. J. Anaesth, 31: 26-31, 1959. 7. Endriaschat, Albert J., and Keenen, Daniel A.: Anesthetic and Dental Management of a Child With Epidermolysis Bullosn, ORAL SIJRG. 36: 667-671, 1973. 8. Hamann, Richard A., and Cohen, Peter J.: Anesthetic Management of a Patient With Epidermolysis Bullosa Dystrophica, Anesthesiology 34: 389-391, 1971. Reprint requests to : Dr. William C. Morgan Department of Pedodontics College of Dentistry University of Kentucky Lexington, Ky. 40506
D.M.D.,
M.S.D.
Chairman and Professor Department of Oral Diagnosis and Oral Medicine University of Kentucky Lexington, Kentucky 40506
Dental anesthetic management of epidermolysis bullosa: A new approach DEPARTXEIVT UNIVERSITY
OF OF
PEDODONTICS,
COLLEGE
OF
DENTISTRY,
KESTUCKY
Epidermolysis bullosa is a rare blister-producing skin disorder that has been of some concern to dentists in the past. Dental treatment has been attempted on an outpatient ljasis with local anesthesia. In some of these patients, local anesthesia has caused large intraoral bullne. Other methods of treatment have been sought, including the use of general anesthesia and intramuscular sedation. This is a report of a case in which intravenous k&amine hydrochloride was used with good results. TVe believe this method has much to offer in the treatment of these patients.
E
pidermolysis bullosa is a rare blister-producing dermatologic disorder. These blisters or vesicles appear at sites of mild trauma or friction. The disease is subdivided according to severity : (Weber-Cockayne syndrome) is a less severe 1. The simplex type autosomal dominant type. It does not leave scars and does not affect the mucous membrane. 2. The dystrophic type (Goldschneider’s syndrome) can take several forms, varying in degree of severity : 1. The dominant auosomal type produces scars on healing. The mucous membranes are affected. It does not interfere with growth and development. 2. The recessive form is more severe and leaves scars. This form is usually associated with other congenital defects, including mental retardation and retarded physical growth. *Formerly Associate Director, Dental Services, Children’s Hospital of Pittsburgh, and Assistant Professor, School of Dental Medicine and School of Medicine, University of Pittsburgh. 732
Volume 40 Number 6
Anesthetic
managemeqlt of epidermolysis
bullosa
733
3. The lethal form is rare. The patient usually dies early in the first few months.’ The most notable feature of the disease is the production of bullae or vesicles at sites of slight trauma. The hands, feet, fingers, and elbows are most commonly involved. When a bulla is broken or peeled off, a raw surface is exposed. CASE REPORT On March 20, 1973, a 5?¥r-old Caucasian boy was brought to the dental clinic for routine dental care. The patient had a medical history of epidermolysis bullosa-dystrophic type. An intraoral examination revealed lmlla formation lingual to the lower anterior teeth and in the mucobuccal fold on hoth left nnd right sides. Oral hygiene was poor, with large amounts of plaque present. The mother explained that the child “was afraid to brush because he produced painful blisters in his mouth.” There were numerous carious lesions, and the mandihular deciduous molars were badly Ijroken down, with abscess formation. The child had previously been seen by a local dentist who declined to treat him because of bulla formation after administration of a local anesthetic. Consultations were secured from the Anesthesia, Dermatology, and Pediatrics Departments. A decision was made to carry out treatment with intravenous sedation. The results of laboratory studies were within normal limits. The patient received steroid coverage the day before, the day of, and the day after the procedure. Antibiotic coverage was provided on tile day of the procedure and for 6 days thereafter. The patient weighed 20 kilogrnms. No premeditation was used. The patient was placed on the dental chair in the dental operating room. A precordial stethoscope was placed, and a blood pressure cuff was placed over a gauze-covered arm. An intravenous site was secured, and ketamine hydrochloride was infused along with intravenous fluids. A total of 150 mg. ketamine hydrochloride was used (i.5 mg. per kilogram of body weight). The rubber dam was used carefully. Four deciduous teeth were restored and four mere extracted. The patient’s vital signs were closely monitored. On the advice of the Pediatrics Department, the patient was retained overnight for obsrrvation to be sure that no hullae formed intraorally to compromise his airway. The bed had a lamb’s wool blanket. The postoperative course was uneventful, and the patient was discharged the following morning. The patient was seen 1 week postoperatively. The extraction sites were healing slomly. Numerous oral lmllac were present. Bands were fitted on the mandibular permanent molars, and a lingual arch was constructed. The child was plnced on a 3-month recall. Six months later, as a result of the poor oral hygiene, several new carious lesions had developed. A decision was made to perform another intravenous procedure. The second procedure was carried out exactly the same as the first. Lahoratory studies were secured. Restorations were placed in four deciduous and three permanent teeth. The lingual arch was removed, the teeth were cleaned, fluoride was applied, and the appliance was recemented. A total of 7.5 mg. of ketamine hydrochloride per kilogram (145 mg. total) was used. The postsurgical course was uneventful. The patient was retained for several hours hecause of the long recovery time from ketamine hydrochloride. When the patient was seen 1 week later, numerous painful oral hullae mere present. The patient was placed on a regular 3month recall. The importance of better oral hygiene, conditions permitting, was emphasized to the child and his mother.
DISCUSSION
Lesions of the mucous membranes of the mouth and tongue are usually present in the dystrophic form .2 Clinically, in the mouth we may see both clear and hemorrhagic bullae, white scars, and a nonspecific gingivitis.3 Other
Oral Hurg. December, 1975
oral structures may bc affected. Tooth brushing may bc painful and may also l~rotlucc oral bullac~. Home carr is a problem. The production of oral bullae is a consideration in clental anesthetic management. A review of the literature reveals past problems associated with dental anesthetic management. Local anesthesia has proved successful in some cases, but other cases have required another form of dental anesthetic management because of behavior problems or excessive oral bulla formation after the administration of a local anesthetic. The use of general anesthesia may involve long hospital stays, a post operative course of concern, and a slow recovery. General anesthesia with various modifications has been accomplishd in several patients with varying results. 4-6 Bullae were usually present postoperatively as a result of anesthetic management or surgical manipulation. The possibility of the formation of large oral bullae so as to compromise the patient’s airway has always been of concern. R,ecently, ketamine has found favor in the treatment of these patients because it can produce good sedation without a reduction in respiration. Most have given the drug by the intramuscular route because of fear of continued trauma with an intravenous site.?? 8 In the case presented here good dental sedation without local anesthesia was accomplished by the intravenous route. We have proposed this route of administration because it offers a more controlled drug dosage and the presence of an intravenous site if necessary. The proper amount of drug can be administered to achieve optimal levels of sedation with the least drug dosage. SUMMARY
We believe that intravenous dental sedation management of patients with epidermolysis bullosa has several advantages to offer over intramuscular or oral sedation management : 1. Local anesthesia is not necessary. 2. The lowest drug dosage that can produce good dental sedation can be given. 3. Increased amounts o’f the drug can easily be added. 4. An intravenous site is available in case of emergency. 5. Patients can be treated on an outpatient basis when reliable home care is available. 6. This method did not cause undue or unexpected bulla formation with the continuous intravenous site. Special thanks are extended to Dr. Joseph Marcy and Dr. Paul Gaffney Hospital of Pittsburgh for their aid and counsel in this case.
of the Children’s
REFERENCES
1. Gorlin, X. J., and Pindborg, J. J.: Syndromes of the Head and Neck, New York, 1964, MCGravv-Hill Book Company, Inc., p. 208. 2. Tobias, H.: Lesions of the Mucous Membrane in a Case of Epidermolysis Bullosa, J. Pediatr. 47: 750-751, 1955. 3. Winstock, Donald: Oral Aspects of Epidermolysis Bullosa, Br. J. Dermatol. 74: 432438, 1962. 4. Boyer, Harold E., and Owens, Robert H.: Epidermolysis Bullosa: A Rare Disease of Dental Interest, ORAL SURG. 14: 1170-1177, 1961.
Volume 40 Number 6
Anesthetic
ma,nagement of epidermolysis
bullosa
735
5. Marshall, B. E.: A Comment on Epidermolysis Bullosa and Its Anesthetic Management for Dental Operations, Br. J. Anaesth. 35: 724-727, 1963. F. Wilson, Frank : Epidermolysis Bullosa : A Rare Disease of Anaesthetic Interest, Br. J. Anaesth, 31: 26-31, 1959. 7. Endriaschat, Albert J., and Keenen, Daniel A.: Anesthetic and Dental Management of a Child With Epidermolysis Bullosn, ORAL SIJRG. 36: 667-671, 1973. 8. Hamann, Richard A., and Cohen, Peter J.: Anesthetic Management of a Patient With Epidermolysis Bullosa Dystrophica, Anesthesiology 34: 389-391, 1971. Reprint requests to : Dr. William C. Morgan Department of Pedodontics College of Dentistry University of Kentucky Lexington, Ky. 40506