PEDIATRIC DENTISTRY/Copyright © 1990 byThe American Academy of Pediatric Dentistry

Volume 12, Number 4

Epidermoiysis bullosa m dental managementand anesthetic considerations: case report

Patricia A. Lanier, DDS William R. Posnick, DDS, MPH, MS

Kevin J. Donly, DDS, MS

Abstract

Epidermolysis bullosa represents a group of hereditaryskin disorders manifested by an exceptional tendency of theskin and mucosa to form bullae and vesicles as a result oftrauma or friction.

Comprehensive dental care for precooperative-age chil-dren with this disorder frequently is managed in the operatingroom under a general anesthetic.

This case report describes an anesthetic protocol for dentalmanagement which was effective and resulted in minimalpostoperative trauma. Eucerin® cream, Gauztex® bandages,and DuoDerm® pads were used to lubricate and stabilizeanesthetic armamentarium. Preoperative and postoperativeerythromycin antibiotic therapy was instituted to preventinfection of the bullae present.

Early diagnosis and institution of preven rive measures canminimize the need for restorative and surgical management inthese children. However, when anesthetic management isnecessary, the use of appropriate consultants and adjunctscan provide valuable support.

Introduction

Epidermolysis bullosa(EB) represents a group genetically transmitted ves-iculobullous skin disordersof varying severity (Gorlin1971). It is manifested by anexceptional tendency of theskin and mucosa to formbullae and vesicles as a resultof trauma, friction, or sponta-neous eruption. The etiologyof the disorder is due to inef-fective or absent connectivetissue anchoring fibrils, re-suiting in a loosely boundepithelial/subepithelial con-nective tissue interface. The

degree of severity and phenotype of the disorder isrelated to the uniformity and pattern of the localizedstructural abnormalities (Sedano and Gorlin 1989).

Classification of EB can be based on phenotypiccharacteristics, mode of inheritance, or microscopicassessment of the specific tissue defect. Table I presentsa summary of diagnostic criteria of the most commonforms of EB.

Dental management of individuals with EB has beenreported previously by several authors (Howden andOldenburg 1972; Endruschat and Keenen 1973;Crawford et al. 1976; Kahn and Trieger 1976; Album etal. 1977; Carroll et al. 1983; Wright 1984). Dental restora-tions and extractions generally follow a variety of anes-thetic protocols focusing on the need to minimize skinand mucosal tissue trauma.

The following case report presents a multidiscipli-nary approach to comprehensive dental management

TABLE Diagnostic Criteria and Classification of Epidermolysis Bullosa

PhenotypicCharacteristics Dominant Inheritance Recessive Inheritance

Simplex Simplex Dys- Dystrophic JunctionalKoebner Weber- trophic (Letalis)

Type Cockayne Cockayne- HerlitzType Touraine Type

Typeneonatal first to neonatal birth birth

thirddecade

some no rare yes yesno no no yes yesrare rare yes yes yesno no yes yes nogeneral- hands/ general- general- general-ized feet ized ized izedbasal mid- papillary papillary laminalayer epidermis dermis dermis lucida

Age of onset

Oral bullaeDental defectsNail defectsScarringLesion location

Site of primaryBullae forma-tion

246 EPIDERMOLYSIS BULLOSA--CASE REPORT: LANIER, POSNICK AND DONLY

of a child with Cockayne-Touraine type epidermolysisbullosa dystrophica.

Case Report

Preoperative Management

A three-year-old white female, CS, presented to theUniversity of Texas Dental Branch Pediatric DentalClinic for initial examination with multiple cariousteeth and a medical diagnosis of epidermolysis bullosadystrophica.

Past medical history included an uncomplicatedpregnancy and delivery, with a diagnosis of Cockayne-Touraine type epidermolysis bullosa dystrophica madeat birth. During the neonatal period she was placed ona diphenylhydantoin therapy regimen of two monthson/one month off. She remains on this protocol withdosages being monitored and modified. With the excep-tion of the dermatologic disorder and its sequelae, a re-view of systems proved noncontributory. Family his-_ tory revealed that her

father also is affected bythe same disorder.

Upon physical exami-nation, bullae were gen-eralized and in variousstages of eruption andhealing (Figs 1 and 2).

Oral examination re-vealed a complete pri-mary dentition with se-vere dental caries secon-dary to ad libitum andnocturnal bottle feedingof sweetened juice for 21/2 years. Although thebottle feeding had beendiscontinued, a pacifierhabit resulted in a severeanterior open bite andperioral bullae forma-tion. Intraoral soft tissueexamination revealedplaque accumulations,generalized marginalgingivitis, and a distinctlack of oral mucosal bul-lae (Figs 3 and 4).

Clinical examinationand charting was com-pleted, noting that thechild's behavior was dif-ficult to manage. Anunsuccessful attemptwas made at obtaining

Fig 1. Facial bullae.

Fig 3. Preoperative maxillary dentition.

Fig 2. Generalized bullae invarious stages of healing.

Fig 4. Preoperative mandibular dentition.

bite-wing and periapical radiographs. Although no oralbullae erupted as a consequence of the attempt to exposeradiographs, placement of the lead shield resulted inbullae formation on her legs.

At CS's second visit, preventive counseling was ini-tiated, including oral hygiene instructions, diet counsel-ing, and home fluoride therapy. The decision was madeto pursue consultations from pediatric medicine, der-matology, and anesthesiology directed toward provid-ing comprehensive dental care under general anesthe-sia.

Appropriate consultations were obtained, and CSwas scheduled for general anesthesia with specificconsideration for minimizing bullae formation and in-fection.

Hospitalization and Dental TreatmentProphylactic antibiotic coverage, erythromycin (125

mg every 6 hr), was instituted by the pediatric derma-tologist six days prior to surgery to prevent infection ofexisting bullae at the time of admission.

PEDIATRIC DENTISTRY: JULY/AUGUST, 1990 ~ VOLUME 12, NUMBER 4 247

The patient was admitted one day prior to surgery toreview and update the history and physical examina-tion and to verify her preoperative status by all medicalconsultants. On the morning of surgery the patient wastaken to the holding area where 2 mg of diazepam wereadministered orally. She was carried sedated to theoperating room and placed in a supine position onsterile lambswool bedding.

Halothane mask induction was accomplished usinga thick layer of Eucerin® cream (Beiersdorf, Inc., Nor-walk, CT) applied to the mask borders to preventtrauma. Gauztex® gauze (General Bandages, Inc., Mor-ton Grove, IL) was placed on the patient's arm, and ablood pressure cuff was secured. An intravenous linewas started and stabilized with tape applied over Duo-Derm® pads (ER Squibb and Sons, Princeton, NJ, Fig 5).

Fig 5. Stabilized intravenous line.

A precordial stethoscope was lubricated with Eucerincream and stabilized with DuoDerm pads. The eyes

were protected with pe-troleum jelly, and nohead cover was used.Orotracheal intubationwas achieved, and thetube was stabilized withtape to DuoDerm padswhich had been adaptedto protect the circumoraland facial skin (Fig 6).

A throat pack lubri-cated with sterile salinewas placed convention-ally, and intraoral radio-graphs were obtained.Restorative dentistry,

Fig 6. Stabilized orotracheal including pulpotomies,tube. stainless steel crowns,

and composite resin restorations, was performed withrubber dam isolation and a mouth prop for jaw stabili-zation. Maxillary primary incisors were extracted, andhemorrhage was controlled with light pressure andinfiltration of a local anesthetic containing epinephrine.All procedures were performed using normal dentaloperating room protocol, augmented with additionalsensitivity to tissue manipulation and pressure.

Postoperative Care and Follow-upFollowing completion of the dental procedures, the

mouth and oropharyngeal areas were carefully suc-tioned, and the throat pack was removed. All mucosalsurfaces were inspected for bullae formation and nonewas noted. The patient was aroused and extubated inthe operating room and transferred to postoperativeholding. Sterile lambswool blankets were used overeggcrate foam cushions for all postoperative bedding.

The patient's postoperative course was uneventful.The intravenous line was maintained for a 36-hr postop-erative period until adequate oral fluid intake was as-sured. All adhesive materials including DuoDerm padswere kept lubricated with Eucerin cream and atraumati-cally removed approximately 48 hr postoperatively.The patient was discharged 72 hr postoperative with aminimum of treatment-related bullae and was main-tained on erythromycin for one week.

A follow-up oral examination was performed oneweek postoperatively, and normal healing was noted.Preventive measures were reinforced, and the patientwas placed on three-month recall. Two subsequentrecall examinations have taken place with no new den-tal caries and a noticeable improvement in hygiene andbehavior.

DiscussionA disorder such as epidermolysis bullosa greatly

compromises a dentist's ability to provide necessarydental treatment. The successful delivery of dental careinvolves consultation and cooperation with multiplemedical specialists and knowledge and appreciation ofthe characteristics of the various categories of the disor-der.

Dental care for the young uncooperative child withdystrophic epidermolysis bullosa can be delivered ef-fectively in the operating room with inhalation anesthe-sia. The use of Eucerin cream, DuoDerm pads, andGauztex gauze enable conventional anesthesia tech-niques, including mask induction, intravenous lineplacement, and stabilization of monitoring devices, tobe used with minimum tissue trauma. In addition, oraland pharyngeal protection can be assured by carefulplacement of a throat pack and rubber dam.

The benefits of early consultation, preventive meas-ures, and ongoing patient monitoring must be stressed

248 EPIDERMOLYSIS BULLOSA—CASE REPORT: LANIER, POSNICK AND DONLY

to minimize the need for restorative therapy.

Dr. Lanier is in private practice in pediatric dentistry in Houston, TX.Dr. Posnick is in private practice in pediatric dentistry in Galveston,TX, and is adjunct professor, pediatric dentistry at the University ofMinnesota School of De/~tistry. Dr. Donly is assistant professor,Department of Pediatric Dentistry at the University of Texas DentalBranch, Houston, TX. Reprint requests should be sent to: Dr. WilliamR. Posnick, 2501 65th Street - Suite A, Galveston, TX 77551.

Album MM, Gaisin A, Lee KWT, Buck BE, Sharrar WG, Gill FM:Epidermolyis bullosa dystrophica polydysplastica: a case of anes-thetic management in oral surgery. Oral Surg Oral Med OralPathol 43:859-72, 1977.

Carroll DL, Stephan MJ, Hays GL: Epidermolysis bullosa--reviewand report of case. J Am Dent Assoc 107:749-51, 1983.

Crawford EG Jr., Burkes EJ, Briggaman RA: Hereditary epidermolysisbullosa: oral manifestations and dental therapy. Oral Surg OralMed Oral Patho142:490-500, 1976.

Endruschat AJ, Keenen DA: Anesthetic and dental management of achild with epidermolysis bullosa dystrophica. Oral Surg Oral MedOral Patho136:667-71, 1973.

Gorlin RJ: Epidermolysis bullosa. Oral Surg Oral Med Oral Pathol32:760-66, 1971.

Howden EF, Oldenburg TR: Epidermolysis bullosa dystrophica:report of two cases. J Am Dent Assoc 85:1113-18, 1972.

Kahn SB, Trieger N: Epidermolysis bullosa hereditaria letalis--a casereport with special emphasis on oral manifestations. J Oral Med31:32-35, 1976.

Sedano HO, Gorlin RJ: Epidermolysis bullosa. Oral Surg Oral MedOral Pathol 67:555-63, 1989.

Wright JT: Epidermolysis bullosa: dental and anesthetic managementof two cases. Oral Surg Oral Med Oral Pathol 57:155-57, 1984.

Healthy Snacks

According to a recent report by the U.S. Surgeon General, what you eat may affect your long-termhealth. On the average, about 40% of our daily calories come from fat. Experts say that figure should be

30% or less. You can help your children develop good eating habits, and improve your own, by choosinghealthy snacks.

Examples of high-calorie, high-fat foods to avoid:

Salted peanuts (1 oz.)

Potato chips (1 oz)

Corn chips (1 oz)

Candy bar (1-1/2 oz.)Ice cream (1/2 cup, chocolate)195

Pie (1/8 pie, apple)

Cheddar cheese (1 oz.)

Cheesecake (1 piece)

Examples of low-fat, low-calorie alternatives

Popcorn (1 cup, plain)

Three graham crackers

One apple

One pear

One carrot

Fruit yogurt (6 oz.)

Mozzarella (1 oz.)

Calories Fat(gm)

170 15

150 !0

160 10

150 8-10

16

181 11114 9.4

257 16.3

23 2

120 3

80 0.5

98 0.7

42 0.2

190 4

72 0.7

PEDIATRIC DENTISTRY: JULY/AuGuST, 1990 - VOLUME 12, NUMBER 4 249