- Email: [email protected]
Anesthetic and dental management of a child with epidermolysis bullosa dystrophica
Anesthetic and dental managementof a child with epidermolysis bullosa dystrophica Albert J. Endruschat, Ohio CHILDREN’S
HOSPITAL
D.D.S., and Daniel A. Keenen, D.D.S., Cincinnati, MEDICAL
CENTER,
DEPARTMENT
OF PEDIATRIC
DENTISTRY
The debilitated dental condition of a 5-year-old white girl with the rare dermatologie disease, epidermolysis bullosa, dystrophic type, was corrected by complete oral rehabilitation while the patient was under general anesthesia. The preoperative preparation and the problem associated with the administration of general anesthesia, the dental procedure, and the postoperative care are presented.
E
pidermolysis bullosa is a rare dermatologic disease in which bullae or vesicles are produced on the skin and mucous membranes at the sites of the slightest trauma or friction. There are two general hereditary patterns of this disease.lm3 The simplex type, which is an autosomal dominant, does not leave scars and rarely affects the mucous membranes.4 The dyystrophic type has three forms(1) a dominantly inherited form, which leaves scars on healing and is said not to interfere with growth or mental development; (2) a recessive lethal form, in which the bullae do not heal and death usually occurs before the age of 3 months; and (3) a recessive form, which leaves scars, is often associated with retarded mental and physical growth, and may also be associated with ectodermal growth defects of hair, sweat glands, nails, and teeth similar to those defects occurring in hereditary ectodermal dysplasia.596 In 1929, in a classic report of cases of ectodermal dysplasia, Weech? first alluded to this association of similar ectodermal defects in both ectodermal dysplasia and epidermolysis bullosa. A nonhereditary or acquired type of epidermolysis bullosa which leaves scars is well documented by Roenigk and associates.8 CASE REPORT Pnopemlivo
preparation
complete physical examination and history gave a clinical impression of a thin Syear-old white girl with the dystrophie type of epidermolysis bullosa, oral mucous membrane lesions,* A
667
668
En&-us&at
Oral Surg. November, 1973
altd Keewl.
Fig. 1. and dental caries (Fig. 1). This patient was referred to us by her dermatologist. Except aa noted above, the child was in good physical condition and had no history of other serious illnesses. The results of a complete blood count and blood studies were within normal limits. There were some bacteria in an otherwise normal urine. Care was taken in planning the administration of general anesthesia and the dental procedure. The patient was placed on prophylactic penicillin 6 hours preoperatively.3 The operating table was prepared with a B-inch layer of 4 by 4 inch gauze fluffs” and a head ring with extra layers of gauze rolls t (Fig. 2). The patient’s extremities were wrapped lightly with gauze rollst (Fig. 3).
Intramuscular administration of preoperative sedation was avoided, eliminating the possibility of trauma due to the patient’s physical resistance to injection. One hour preoperatively the patient was given 20 mg. of Benadryl and 30 mg. of Phenergan by mouth. The girl weighed 39% pounds. The sedated patient was not moved from her bed to the operating room until she was anesthetized. Rapid intramuscular injection of 40 mg. ketamme hydrochloride into the left thigh gave the desired anesthesia.10 The patient was gently moved to the operating table, with gauze fluffs and light pressure used over large areas of her body. The operating table was in the Trendelenberg position at approximately 15 degrees. A pressure cuff was placed over the gauze-covered right arm and a Medicut catheter was inserted into a vein on the dorsal surface of the hand, delivering Ionosol MB in 5 per cent dextrose and water. The catheter was secured with a gauze roll. The eyes were protected with a towel. The patient was now in position for continuation of anesthesia with intravenous ketamine hydrochloride, Although endotracheal intubation has been used in this type patient,l& 12 in order to prevent the formation of bullae in the airway,2 the anesthesiologist selected twin nasopharyngeal tubes for the delivery of nitrous oxide and oxygen. The patient received nitrous oxide, oxygen, and parenteral ketamine hydrochloride for anesthesia during the procedure. Atropine sulfate was also administered. The patient’s respiration, carotid pulse, and depth of anesthesia were closely monitored. The need to use a rubber dam for the dental procedure was taken into consideration in the selection of nasopharyngeal tubes in the anesthetic technique used for this patient. l Kerlix tKerlix
Fluffs, Kendall, Rolls, Kendall,
Hospital Hospital
Products Products
Division, Division.
Chicago, Ill.
Epidemolysis
Volume 36 Number 5
bullosa dystropkica
669
Figs. 8 and J. Dental
nhabilitation
The lips were not swollen; nor were there vesicles on them prior to the surgical procedure, although lesions were present on the tongue and mucous membranes. It was thought that any throat packs would cause the formation of bullaex; therefore, all operative procedures were performed under a rubber dam. The rubber dam was used to isolate the field, prevent foreignbody aspiration, stop fluid accumulation in the airway, and, to a degree, make a closed airway for oxygen and nitrous oxide through the nasopharyngeal tubes. The patient’s lips were coated with Bacitracin ointment. The entire maxillary arch was isolated with a rubber dam, and the carious teeth were restored. The entire mandibular arch was then isolated and restored in the same manner. With the rubber dam in place and a Molt mouth prop on the teeth to stabilize the dental arches, multiple earious teeth were restored using stainless steel crowns and silver amalgam fillings in the posterior teeth, while the anterior teeth were restored with composite resin fillings. The operative procedures were carried out in the usual manner for operating room dentistry, except for the extreme caution used in tissue manipulation and the inability to exert pressure in the seating of stainless steel crowns. Dental prophylaxis was not done at this time, thereby minimizing trauma and suctioning. The condition of the oral tissues immediately postoperatively showed some vesicles that had burst, leaving shreds of tissue.11 These shreds were trimmed with scissors and removed. The tongue appeared swollen, but the lips did not exhibit any swelling although there was some evidence of stretching and bulla formation at the commissures of the mouth.13 The procedure lasted 2 hours. Postoperative
cowsc
ond recovery
After the dental procedures were completed, the patient was gently placed on a bed covered with lamb’s wool and taken to the Intensive Care Unit with the twin nasopharyngeal tubes and the intravenous apparatus still in place. Three hours postoperatively, the patient had several episodes of vomiting, which caused respiratory di6iculty and necessitated the use of suction and oxygen for an hour to keep the
670 Endruschat and Keenen
Oral Surg. November, 1973
E
Figs. 4 and 5. airways open. The patient also received a 25 mg. Phenergan suppository for the treatment of nausea and vomiting at this time. Approximately 6 hours postoperatively, the patient became restless and removed one of the nasopharyngeal tubes. Since her color was good and there was no respiratory distress, it was not replaced. Eleven hours postoperatively, the other nasopharyngeal tube wan removed. Fourteen hours postoperatively, the patient’s tongue was less swollen, but the lips and eommissures of the mouth were encrusted and showed a serous exudate (Fig. 4). The attached gingiva was also edematous. Twenty-six hours postoperatively, the patient began taking some oral fluids and was given a 5 C.C. rinse of a mixture of 2 per cent lidocaine viscous (15 c.c.) and Kenalog 10 (0.5 CL), which greatly relieved the pain from the oral lesions. Continued improvement was noted, so that after 38 hours the oral intake was sticient that intravenous fluids could be discontinued. The patient was kept on a regimen of 2 per cent lidocaine viscous and Kenalog rinses twice daily or when needed for pain. Penicillin coverage was continued. Seventy-two hours postoperatively, the patient was discharged. A supply of viscous lidocaine, Kenalog, and penicillin capsules for an additional 10 days was given to the patient at the time of discharge. The patient had uneventful healing with minimal scarring. Three months postoperatively, there was no noticeable constricture of the oral opening due to scarring of the eommissures (Fig. 5). At this time the tongue and palate had lesions; these were due to trauma from recent normal mastication.
DISCUSSION
In reviewing the literature, we have found that most dental procedures for this type of patient have been limited to extractions. It is even more important that these patients receive restorative and preventive dental care because of the characteristics of the disease. The oral health must be considered from the standpoint of esthetics, psychologic effects, and disease-free function. Removable dental prostheses are contraindicated in this disease.14These patients may show an improvement in their dermatologic condition after puberty1 and then can be treated as routine dental patients.
Volume Number
36 5
Epidermolysis bullosa dystrophica
Edward E. Lowe, M.D., of the Department of Anesthesia, Children’s Center, administered the general anesthesia in the case reported here.
Hospital
671 Medical
REFERENCES
1. Gorlin, R. J., and Pindborg, J. J.: Epidermolysis Bullosa. In Gorlin, R. J., and Pindborg, J. J.: Syndromes of the Head and Neck, New York, 1964, McGraw-Hill Book Company, The Blakiston Division, pp. 208-214. Bullosa and Its Anesthetic Management 2. Marshall. B. E.: A Comment on Euidermolysis for Dental Operations, Br. J. Anaesth. 35: 72&727,1963. 3. Burgoon, C. F., Jr.: The Skin. In Nelson, W. E.: Textbook of Pediatrics, ed. 9, Philadelphia, 1969, W. B. Saunders Company, pp. 1393-1394. 4. Mescon, H., Grots, I. A., and Gorlm, R. J.: Muooeutaneous Disorders. In Gorlin, R. J., and Goldman. H. M.: Thoma’s Oral Patholorrv._I, ed. 6.I St. Louis. , 1970., The C. V. Mosbv Company, ~011 2, pp. 679-707. 5. Shafer, W. G., Hine, M. K., and Levy, B. M.: Diseases of the Skin. In Shafer? W. G., Hine. M. K.. and Levv. B. M.: A Textbook of Oral Patholoev.-_, ed. 2.I Philadelphia. 1963, _I W. B. Saunders Compiny, pp. 703-705. Kaslick, R. S., and Brustein, H. C.: Epidermolysis Bullosa; Review of the Literature and Report of a Case, ORAL Suao. 14: 1315-1330,196l. Weech, A. A.: Hereditary Ectedermal Dysplasia (Congenital Ectodermal Defect) : Report of 2 Cases, Am. J. Dis. Child. 37: 766-790, 1929. Roenigk, H. H., Jr., Ryan, J. cf., and Bergfeld, W. F.: Epidermolyais Bullosa Acquisita; Report of Three Cases and Review of All Published Cases, Arch. Dermatol. 103: l-10, 1971. Gorlin, R. J., and Chaudhry, A. P.: Unusual Stomadromes-Pachyonychia Congenita, Lipoid Proteinosis, Reiter’s Syndrome, Epidermolysis Bullosa, Bonnevie-Ullrich-Turner’s Syndrome, and Multiple Hereditary Tel.angiectasia, ORAL SURQ. 13: 257-268, 1960. 10. Hamann, R. A., and Cohen, P. J.: Anesthetic Management of a Patient With Epidermolysis Bullosa Dystrophica, Anesthesiology 34: 389-391, 1971. 11. Boyer, H. E., and Owens, R. H.: Epidermolysis Bullosa: A Rare Disease of Dental Interest; Review of the Literature and Report of a Case, ORAL SURG. 14: 1170-1177, 1961. 12. Wilson, F.: Epidermolysis Bullosa: A Rare Disease of Anesthetic Interest, Br. J. Anaesth. 31: 26-31, 1959. 13. Winstock, D.: Oral Aspects of Epidermolysis Bullosa, Br. J. Dermatol. 74: 431-438, 1962. 14. Haas, C. D. : Epidermolysis Bullosa Dystrophica; Report of a Case, ORAL SURG.26: 291295, 1968.
Reprint requests to : Dr. Albert J. Endruschat Department of Pediatric Dentistry Children’s Hospital Medical Center Cincinnati, Ohio 45229
HOSPITAL
D.D.S., and Daniel A. Keenen, D.D.S., Cincinnati, MEDICAL
CENTER,
DEPARTMENT
OF PEDIATRIC
DENTISTRY
The debilitated dental condition of a 5-year-old white girl with the rare dermatologie disease, epidermolysis bullosa, dystrophic type, was corrected by complete oral rehabilitation while the patient was under general anesthesia. The preoperative preparation and the problem associated with the administration of general anesthesia, the dental procedure, and the postoperative care are presented.
E
pidermolysis bullosa is a rare dermatologic disease in which bullae or vesicles are produced on the skin and mucous membranes at the sites of the slightest trauma or friction. There are two general hereditary patterns of this disease.lm3 The simplex type, which is an autosomal dominant, does not leave scars and rarely affects the mucous membranes.4 The dyystrophic type has three forms(1) a dominantly inherited form, which leaves scars on healing and is said not to interfere with growth or mental development; (2) a recessive lethal form, in which the bullae do not heal and death usually occurs before the age of 3 months; and (3) a recessive form, which leaves scars, is often associated with retarded mental and physical growth, and may also be associated with ectodermal growth defects of hair, sweat glands, nails, and teeth similar to those defects occurring in hereditary ectodermal dysplasia.596 In 1929, in a classic report of cases of ectodermal dysplasia, Weech? first alluded to this association of similar ectodermal defects in both ectodermal dysplasia and epidermolysis bullosa. A nonhereditary or acquired type of epidermolysis bullosa which leaves scars is well documented by Roenigk and associates.8 CASE REPORT Pnopemlivo
preparation
complete physical examination and history gave a clinical impression of a thin Syear-old white girl with the dystrophie type of epidermolysis bullosa, oral mucous membrane lesions,* A
667
668
En&-us&at
Oral Surg. November, 1973
altd Keewl.
Fig. 1. and dental caries (Fig. 1). This patient was referred to us by her dermatologist. Except aa noted above, the child was in good physical condition and had no history of other serious illnesses. The results of a complete blood count and blood studies were within normal limits. There were some bacteria in an otherwise normal urine. Care was taken in planning the administration of general anesthesia and the dental procedure. The patient was placed on prophylactic penicillin 6 hours preoperatively.3 The operating table was prepared with a B-inch layer of 4 by 4 inch gauze fluffs” and a head ring with extra layers of gauze rolls t (Fig. 2). The patient’s extremities were wrapped lightly with gauze rollst (Fig. 3).
Intramuscular administration of preoperative sedation was avoided, eliminating the possibility of trauma due to the patient’s physical resistance to injection. One hour preoperatively the patient was given 20 mg. of Benadryl and 30 mg. of Phenergan by mouth. The girl weighed 39% pounds. The sedated patient was not moved from her bed to the operating room until she was anesthetized. Rapid intramuscular injection of 40 mg. ketamme hydrochloride into the left thigh gave the desired anesthesia.10 The patient was gently moved to the operating table, with gauze fluffs and light pressure used over large areas of her body. The operating table was in the Trendelenberg position at approximately 15 degrees. A pressure cuff was placed over the gauze-covered right arm and a Medicut catheter was inserted into a vein on the dorsal surface of the hand, delivering Ionosol MB in 5 per cent dextrose and water. The catheter was secured with a gauze roll. The eyes were protected with a towel. The patient was now in position for continuation of anesthesia with intravenous ketamine hydrochloride, Although endotracheal intubation has been used in this type patient,l& 12 in order to prevent the formation of bullae in the airway,2 the anesthesiologist selected twin nasopharyngeal tubes for the delivery of nitrous oxide and oxygen. The patient received nitrous oxide, oxygen, and parenteral ketamine hydrochloride for anesthesia during the procedure. Atropine sulfate was also administered. The patient’s respiration, carotid pulse, and depth of anesthesia were closely monitored. The need to use a rubber dam for the dental procedure was taken into consideration in the selection of nasopharyngeal tubes in the anesthetic technique used for this patient. l Kerlix tKerlix
Fluffs, Kendall, Rolls, Kendall,
Hospital Hospital
Products Products
Division, Division.
Chicago, Ill.
Epidemolysis
Volume 36 Number 5
bullosa dystropkica
669
Figs. 8 and J. Dental
nhabilitation
The lips were not swollen; nor were there vesicles on them prior to the surgical procedure, although lesions were present on the tongue and mucous membranes. It was thought that any throat packs would cause the formation of bullaex; therefore, all operative procedures were performed under a rubber dam. The rubber dam was used to isolate the field, prevent foreignbody aspiration, stop fluid accumulation in the airway, and, to a degree, make a closed airway for oxygen and nitrous oxide through the nasopharyngeal tubes. The patient’s lips were coated with Bacitracin ointment. The entire maxillary arch was isolated with a rubber dam, and the carious teeth were restored. The entire mandibular arch was then isolated and restored in the same manner. With the rubber dam in place and a Molt mouth prop on the teeth to stabilize the dental arches, multiple earious teeth were restored using stainless steel crowns and silver amalgam fillings in the posterior teeth, while the anterior teeth were restored with composite resin fillings. The operative procedures were carried out in the usual manner for operating room dentistry, except for the extreme caution used in tissue manipulation and the inability to exert pressure in the seating of stainless steel crowns. Dental prophylaxis was not done at this time, thereby minimizing trauma and suctioning. The condition of the oral tissues immediately postoperatively showed some vesicles that had burst, leaving shreds of tissue.11 These shreds were trimmed with scissors and removed. The tongue appeared swollen, but the lips did not exhibit any swelling although there was some evidence of stretching and bulla formation at the commissures of the mouth.13 The procedure lasted 2 hours. Postoperative
cowsc
ond recovery
After the dental procedures were completed, the patient was gently placed on a bed covered with lamb’s wool and taken to the Intensive Care Unit with the twin nasopharyngeal tubes and the intravenous apparatus still in place. Three hours postoperatively, the patient had several episodes of vomiting, which caused respiratory di6iculty and necessitated the use of suction and oxygen for an hour to keep the
670 Endruschat and Keenen
Oral Surg. November, 1973
E
Figs. 4 and 5. airways open. The patient also received a 25 mg. Phenergan suppository for the treatment of nausea and vomiting at this time. Approximately 6 hours postoperatively, the patient became restless and removed one of the nasopharyngeal tubes. Since her color was good and there was no respiratory distress, it was not replaced. Eleven hours postoperatively, the other nasopharyngeal tube wan removed. Fourteen hours postoperatively, the patient’s tongue was less swollen, but the lips and eommissures of the mouth were encrusted and showed a serous exudate (Fig. 4). The attached gingiva was also edematous. Twenty-six hours postoperatively, the patient began taking some oral fluids and was given a 5 C.C. rinse of a mixture of 2 per cent lidocaine viscous (15 c.c.) and Kenalog 10 (0.5 CL), which greatly relieved the pain from the oral lesions. Continued improvement was noted, so that after 38 hours the oral intake was sticient that intravenous fluids could be discontinued. The patient was kept on a regimen of 2 per cent lidocaine viscous and Kenalog rinses twice daily or when needed for pain. Penicillin coverage was continued. Seventy-two hours postoperatively, the patient was discharged. A supply of viscous lidocaine, Kenalog, and penicillin capsules for an additional 10 days was given to the patient at the time of discharge. The patient had uneventful healing with minimal scarring. Three months postoperatively, there was no noticeable constricture of the oral opening due to scarring of the eommissures (Fig. 5). At this time the tongue and palate had lesions; these were due to trauma from recent normal mastication.
DISCUSSION
In reviewing the literature, we have found that most dental procedures for this type of patient have been limited to extractions. It is even more important that these patients receive restorative and preventive dental care because of the characteristics of the disease. The oral health must be considered from the standpoint of esthetics, psychologic effects, and disease-free function. Removable dental prostheses are contraindicated in this disease.14These patients may show an improvement in their dermatologic condition after puberty1 and then can be treated as routine dental patients.
Volume Number
36 5
Epidermolysis bullosa dystrophica
Edward E. Lowe, M.D., of the Department of Anesthesia, Children’s Center, administered the general anesthesia in the case reported here.
Hospital
671 Medical
REFERENCES
1. Gorlin, R. J., and Pindborg, J. J.: Epidermolysis Bullosa. In Gorlin, R. J., and Pindborg, J. J.: Syndromes of the Head and Neck, New York, 1964, McGraw-Hill Book Company, The Blakiston Division, pp. 208-214. Bullosa and Its Anesthetic Management 2. Marshall. B. E.: A Comment on Euidermolysis for Dental Operations, Br. J. Anaesth. 35: 72&727,1963. 3. Burgoon, C. F., Jr.: The Skin. In Nelson, W. E.: Textbook of Pediatrics, ed. 9, Philadelphia, 1969, W. B. Saunders Company, pp. 1393-1394. 4. Mescon, H., Grots, I. A., and Gorlm, R. J.: Muooeutaneous Disorders. In Gorlin, R. J., and Goldman. H. M.: Thoma’s Oral Patholorrv._I, ed. 6.I St. Louis. , 1970., The C. V. Mosbv Company, ~011 2, pp. 679-707. 5. Shafer, W. G., Hine, M. K., and Levy, B. M.: Diseases of the Skin. In Shafer? W. G., Hine. M. K.. and Levv. B. M.: A Textbook of Oral Patholoev.-_, ed. 2.I Philadelphia. 1963, _I W. B. Saunders Compiny, pp. 703-705. Kaslick, R. S., and Brustein, H. C.: Epidermolysis Bullosa; Review of the Literature and Report of a Case, ORAL Suao. 14: 1315-1330,196l. Weech, A. A.: Hereditary Ectedermal Dysplasia (Congenital Ectodermal Defect) : Report of 2 Cases, Am. J. Dis. Child. 37: 766-790, 1929. Roenigk, H. H., Jr., Ryan, J. cf., and Bergfeld, W. F.: Epidermolyais Bullosa Acquisita; Report of Three Cases and Review of All Published Cases, Arch. Dermatol. 103: l-10, 1971. Gorlin, R. J., and Chaudhry, A. P.: Unusual Stomadromes-Pachyonychia Congenita, Lipoid Proteinosis, Reiter’s Syndrome, Epidermolysis Bullosa, Bonnevie-Ullrich-Turner’s Syndrome, and Multiple Hereditary Tel.angiectasia, ORAL SURQ. 13: 257-268, 1960. 10. Hamann, R. A., and Cohen, P. J.: Anesthetic Management of a Patient With Epidermolysis Bullosa Dystrophica, Anesthesiology 34: 389-391, 1971. 11. Boyer, H. E., and Owens, R. H.: Epidermolysis Bullosa: A Rare Disease of Dental Interest; Review of the Literature and Report of a Case, ORAL SURG. 14: 1170-1177, 1961. 12. Wilson, F.: Epidermolysis Bullosa: A Rare Disease of Anesthetic Interest, Br. J. Anaesth. 31: 26-31, 1959. 13. Winstock, D.: Oral Aspects of Epidermolysis Bullosa, Br. J. Dermatol. 74: 431-438, 1962. 14. Haas, C. D. : Epidermolysis Bullosa Dystrophica; Report of a Case, ORAL SURG.26: 291295, 1968.
Reprint requests to : Dr. Albert J. Endruschat Department of Pediatric Dentistry Children’s Hospital Medical Center Cincinnati, Ohio 45229