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CASE REPORT
Year : 2011  |  Volume : 29  |  Issue : 2  |  Page : 155-160
 

Dental and anesthetic management of a child with epidermolysis bullosa


Department of Pediatric and Preventive Dentistry, Kanchi Kamakoti Childs Trust Hospital, Nugambakam, Chennai, Tamil Nadu, India

Date of Web Publication9-Sep-2011

Correspondence Address:
P Rekka
Department of Pediatric and Preventive Dentistry, Kanchi Kamakoti Childs Trust Hospital, Nungambakam, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.84690

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   Abstract 

Epidermolysis bullosa (EB) is a wide spectrum of rare genetic disorders characterized by marked fragility of the skin and mucous membranes in which vesculobullous lesions occur in response to trauma, heat, or no apparent cause. This case report deals with the dental and anesthetic management of a 10-year-old girl with dystrophic EB.


Keywords: Dystrophic EB, dermolytic EB, epidermolysis bullosa


How to cite this article:
Prabhu V R, Rekka P, Ramesh, Swathi S. Dental and anesthetic management of a child with epidermolysis bullosa. J Indian Soc Pedod Prev Dent 2011;29:155-60

How to cite this URL:
Prabhu V R, Rekka P, Ramesh, Swathi S. Dental and anesthetic management of a child with epidermolysis bullosa. J Indian Soc Pedod Prev Dent [serial online] 2011 [cited 2019 Dec 14];29:155-60. Available from: http://www.jisppd.com/text.asp?2011/29/2/155/84690



   Introduction Top


Epidermolysis bullosa (EB) is a wide spectrum of rare genetic disorders characterized by marked fragility of the skin and mucous membranes in which vesculobullous lesions occur in response to trauma, heat, or no apparent cause. The pathophysiology of EB varies, depending on the specific defect in the epithelial/subepithelial connective tissue, with varying severity of blistering up to potentially debilitating scar formation and premature death. More than 20 distinct variants of EB have been identified and classified according to the phenotypic characteristics, mode of inheritance, and the ultrastructural level at which the blisters occur. [1] These have further been classified into three major subgroups based on specific level of tissue cleavage following mechanical trauma to the skin: epidermolytic (simplex EB) usually is dominantly inherited with cytolysis of the basal layer of epidermis and is characterized by intraepidermal blistering with relatively blistering of skin and mucous membranes. Lesions typically heal without scarring. Lamina lucidolytic (junctional EB) is recessively inherited and lesions occur in the lamina lucida or epidermal-dermal interface with variable hemidesmosomal abnormalities. Dermolytic (dystrophic EB) is inherited in a dominant as well as recessive form and is usually associated with the cleavage in the sublamina lucida plane. There is excessive collangenolysis resulting in reduced or absent collagen VII, a fibrous protein that is the main component of the anchoring filaments. The anchoring fibrils appear abnormal, reduced, or altogether absent and there is diminished epidermal adherence. [1],[2],[3]

The ultrastructural level of separation in blistered tissue is determined using transmission electron microscopy and/or immunofluorescence antigenic mapping. Characterizing morphologic features - including the hemidesmosomes, anchoring fibrils, and subbasal dense plates and the relative expression of numerous basement membrane-specific antigens, such as type VII collagen, GB3, 19DEJ-1, and the chondroitin 6-sulfate proteoglycan are also useful diagnostic aids in further delineating EB types and subtypes. [4]

The mode of inheritance and clinical features, such as the severity and distribution of cutaneous and extracutaneous findings, are also considered in the final classification of each EB type. Hereditary EB subtypes may exhibit autosomal dominant or recessive modes of transmission, with the possible exception of the Mendes Da Costa variant of EB simplex, which is reported to be X linked. [4]

During the 1980s, the National Institute of Health established four regional institutional centers that were designated as clinical sites for the National Epidermolysis Bullosa Registry (NEBR). These centers were charged on new developments and research of and understanding the clinical, diagnostic, and laboratory characteristics of EB. As a result of these and other investigations, knowledge concerning the different characteristics and oral manifestations of the 23 different EB subtypes has increased markedly. [4],[5]

General findings

Cutaneous findings vary considerably and may include blisters, crusted erosions, milia, scarring, granulation tissue, pigmentation changes, cicatricial alopecia, and the absence or dystrophy of nails. Extra-cutaneous involvement may include eyes, teeth, oral mucosa, esophagus, intestinal tract, anus, genitourinary tract, and/or musculoskeletal system. Extra-cutaneous involvement can be so severe that it significantly alters an individual's life style. [4],[5]

EB simplex is the least serious form of the disease. In EB simplex, blister formation occurs within basal keratinocytes due to mutations in the keratin 5 and 14 genes. In most patients, blisters are mild and do not scar after they heal. Some forms of EB simplex affect just the hands and feet (simplex localized with hypodontia, "Kallin syndrome"); the other form of EBs simplex can lead to more widespread blistering, as well as hair loss and missing teeth (simplex herpetiformis, "Dowling-Meara type"). Recurrent blistering is annoying but not life threatening. [5]

In the second form of EB, junctional EB, blisters arise within the lamina lucida of the basement membrane, and several mutations have been described in gene 3 that encodes the anchoring filament lamin 5. In most patients, it does not lead to scarring. However, skin is the area prone to blistering, such as the skin of elbows and the knees often shrinks. In one variation of junctional EB, called Gravis junctional EB (Herlitz type), the blistering can be so severe that affected infants may not survive due to massive infection and dehydration. [5]

The third form of EB, dystrophic EB causes blisters to form on the superficial papillary dermis as a consequence of mutations in type VII collagen gene. It varies greatly in terms of severity, but more typically affects the arm and legs. In one variation called Hallopeau-Siemens EB, repeated blistering and scarring of hands and feet causes the fingers and toes to fuse, leaving them mitten-like and dysfunctional. The disease is also observed at birth or shortly after. [5]

Oral findings

The character and extent of oral involvement varies greatly from one EB type to the next. In the milder forms of inherited EB, the oral mucosa may suffer only occasional blistering with small discrete vesicles that heal rapidly without scarring and do not significantly alter the patient's life. In most severe cases, however, the entire oral mucosa is affected and may be characterized by severe intraoral blistering with subsequent scar formation, microstomia, obliteration of the oral vestibule, and ankyloglossia. [2],[4]

Oral mucosa fragility, with subsequent development of intraoral soft tissue lesions, is common to all major EB types. EB simplex patients develop intraoral blistering, these lesions tend to be small in size (<1 cm), and tend to heal without scarring. While oral involvement in EB simplex appears to occur more commonly during the perinatal period, some individuals experience continued blistering into or beyond infancy or even late childhood. [4]

Most individuals with junctional or dominant dystrophic forms of inherited EB develop lesions involving their oral mucosa that are characteristically larger (>1 cm), and more numerous than those of observed in EB simplex, often more erosive, and are usually quite painful. Despite this propensity to develop clinically significant oral lesions, patients with these two major inherited EB forms usually heal without scarring and, therefore do not typically develop ankyloglossia or vestibular obliteration. [4]

Individuals with generalized recessive dystrophic EB exhibit the most severe oral involvement, which is characterized by a complete obliteration of the vestibule and ankyloglossia. With the increasing age, structures such as palatal rugae and lingual papillae typically become unrecognizable because of the presence of continuous blister formation and scarring. In contrast, localized or mildly affected generalized recessive dystrophic cases do not show the same severity in oral scarring, loss of lingual papillae, and/or ankyloglossia observed in the severe generalized forms. Squamous cell carcinoma of the tongue has been reported in several cases of recessive dystrophic EB, presumably due to the analogous tendency for such tumors to arise on skin sites subjected to repeated ulceration and re-epithelization. [4],[6]

Dental involvement also varies according to different subtypes. In the simplex form, the teeth are not affected; however, the dystrophic forms often display malformed teeth with early caries development. The enamel changes observed resembles those seen in amelogenesis imperfecta. [4],[5],[7]

Rampant dental caries is frequently seen in patients with severe generalized recessive dystrophic EB despite appearing to have normal dentition development. [8] It has been hypothesized that excessive dental caries is a result of presence and severity of the soft tissue involvement, which leads to alterations in diet (soft and frequently high carbohydrate consumption) and increased oral clearance time (secondary limited to tongue mobility and vestibular constriction), and creates an abnormal tooth/soft tissue relationship (buccal and lingual mucosa) which firmly positions against the tooth. Furthermore, these individuals often lack the ability to routinely practice normal preventive measures such as oral hygiene or the use of oral rinses. [4],[5],[7]

The examination of salivary flow rates and salivary antibody titers in a large EB population indicates that there is no diminution in the salivary function that predisposes these individuals to dental caries. This leaves the clinician unable to accurately predict the degree of expected oral involvement, and therefore, unable to formulate a specific therapeutic approach based on risk factors associated with each EB subtype. [5],[7]

The dental management of individuals with EB has been reported previously by several authors (Howden and Oldenburg 1972 [9] ; Endruschat and Keenen 1973 [10] ; Crawford et al. 1976 [11] ; Kahn and Trieger 1976 [12] ; Album et al. 1977 [13] ; Carroll et al. 1983 [14] ; Wright 1984 [15] ). Dental restorations and extractions generally follow a variety of anesthetic protocols focusing on the need to minimize skin and mucosal tissue trauma. [3]


   Case Report Top


A 10-year-old girl reported to the pediatric dental op with the chief complaint of pain and swelling in her right lower back teeth region. She has been already diagnosed with dystrophic EB. There was no family history of EB. She had numerous blistering on her hands, elbows, knees, feet, trunk, scalp, nails, and face [Figure 1],[Figure 2],[Figure 3],[Figure 4].
Figure 1: Blistering seen on the (a) back, (b) hands, and (c) foot

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Figure 2: Absence of nails due to repeated blistering and scarring of feet

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Figure 3: Lateral cephalogram revealed radiolucency involving pulp in 46 with periapical radiolucency

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Figure 4: Skin sloughing on the right side of the forehead during the chin lift-head tilt procedure while intubation

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On intraoral examination, there was dental caries with dentoalveolar abscess in relation to 46. The extra-oral swelling was seen in relation to the right lower border of the mandible extending anteriorly till the corner of mouth. Due to repeated intraoral blistering with subsequent scar formation, the girl had limited mouth opening. So a lateral oblique radiograph was taken to assess the dental caries involvement of 46. The radiograph revealed radiolucency involving pulp in 46 with periapical radiolucency [Figure 3]. It was decided to extract 46 under general anesthesia as the child was very apprehensive on dental examination.

Anesthetic management included orotracheal intubation and careful lubrication of the facemask and laryngoscope. A throat pack was placed. Despite careful handling of the patient, there was a slight skin sloughing on the right side of the forehead during the chin lift-head tilt procedure while intubation [Figure 4]. The girl subsequently had her 46 removed. A gauze pack was placed on the extraction site and the extraction site was observed until clot formed. Following this procedure, the girl was extubated from GA successfully.


   Discussion Top


The comprehensive care of children with dystrophic EB can be difficult because of severe limitations imposed by this condition. This can impact not only dental management but all other specialities as well. Special accommodations need to be provided from an anesthetic standpoint when these children are to be administered general anesthesia prior to oral rehabilitation. [1],[2],[3],[16] The general rule for management is the "no touch" principle. [17] Face masks can be used but must be prepared with several layers of Vaseline gauze. More gauze must be placed below the anesthetists' fingers while applying minimal chin lift. Head tilt can be achieved by the gentle manipulation of the head with a hand below the occiput. [18] Great care should be exercised to avoid the use of tape on any skin surface to avoid shearing forces on the skin when moving the patient. Adhesives surfaces are removed from the ECG electrodes which are held on the extremities with Webril or on the chest and abdominal surface using Meplix. In cases of severe skin involvement, ECG monitoring may be omitted, with the heart rate sampled from the pulse oximeter. Mepilex is an absorbent, atraumatic dressing made of polyurethane foam. Although it adheres to the skin, it has no adhesive surface. Pulse oximetry may be accomplished with an adult clip-on probe (not possible if the patient has total pseudosyndactyly of fingers and toes), or with an adhesive strip placed over a clear plastic bag covering the hand or foot, and then wrapped with Webril or Coban. The IV can be secured with Meplix and then wrapped with Coban. [1] Care should be taken to protect the eyes. Simple eye ointments are used abundantly as soon as the patient is anesthetized. [18] In our case, the orotracheal tube and the IV was secured in place with gauze pads and stabilized with the gauze tape tied over them [Figure 5]. Eyes were covered with gauze pads to prevent exposure to keratitis [Figure 6] Although limited mouth opening in many patients with EB necessitates nasotracheal intubation, this route is a good route of choice for all patients with EB, even with a good mouth opening, as oral intubation causes more trauma to and bulla formation on the tongue and oral mucosa because of the pressure of the laryngoscope on the supraglottic area. The nasal mucosa is composed of pseudostratified cylindrical ciliated epithelium with goblet cells (respiratory epithelium), stratified cuboidal epithelium, and stratified squamous nonkeratinized epithelium, the first two of which are less vulnerable to bulla formation than the stratified squamous keratinized and nonkeratinized epithelium of the oral mucosa, and the tube intubated nasally may be secured more easily without a tape than the one done orallly . Care should be taken to use a tube small enough to avoid pressure on the skin surface at the entrance to the naris. The tube should be softened by soaking it in a warm saline and generously lubricated with a water-based lubricant prior to the insertion in the naris. Oral rehabilitation procedures are also more easily accomplished with the nasotracheal tube. Despite these facts, some authors prefer oral intubation in patients with EB. [16]
Figure 5: IV was secured in place with gauze pads and stabilized with the gauze tape tied over them

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Figure 6: Eyes were covered with gauze pads to prevent exposure keratitis

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These children have been reported to have a dental maturity delay of 2 1/4 months based on Dermirjian's system when compared to healthy children. The reported delay in dental formation supports the possibility of a similar delay in the eruption of teeth in patients with dystrophic EB and may be of importance in children where serial extractions are being considered. [19]

The increased prevalence of dental caries in junctional and recessive dystrophic EB necessitates that aggressive preventive and rehabilitative measures be taken. Diet control is difficult because of the high caloric requirement for tissue repair, which involves the increased intake of carbohydrates. In fact, dietary recommendations sometimes include the consumption of liquid sucrose. [20] The dentist must make recommendations for oral health care while working within the constraints of the soft tissue fragility, enamel hypoplasia, and limited dietary modification. Optimal care will be possible only through the efforts aimed at maintaining dentition through oral hygiene, fluoride therapy, and restorative care, which may require general anesthesia. A review of fourteen cases demonstrates that the comprehensive oral health can be delivered safely and effectively to even the most severely affected persons with EB, with the use of endotracheal intubation. [2]

In the past, extractions have been considered the treatment of choice in recessive dystrophic EB; however, with current anesthetic techniques, functional dentitions can be maintained in the most severely affected

persons. [2] In patients prone to oral blistering, oral hygiene may be best accomplished with a soft-headed tooth brush. In addition to the systemic administration of fluorides, fluoride rinses may also help control caries. However, many EB patients with mucosal lesions are sensitive to the strong flavoring agents and alcohol in most rinses; specially formulated rinses lacking these ingredients may be required. Chlorhexidine (CHX) mouth rinses may also help control dental caries, but again the patient may be sensitive to the high alcohol content of commercially available rinses. This may be overcome by swabbing the CHX directly on teeth. [4],[5],[7]

An increased fluid intake while eating may enhance the oral clearance of food debris in dystrophic EB patients with severe intraoral scarring and restricted tissue mobility. Topical applications of neutral sodium fluoride and high-dose fluoride varnish may be preferable. [7]

Maintaining the dentition can not only reduce the potential for soft tissue trauma to the mucosa, and possibly the esophagus through more efficient mastication, but also may allow better nutrition. There is no question that dentists have the ability to help these patients keep a positive self-image by providing them with optimal oral health.

 
   References Top

1.Lindemeyer R, Wadenya R, Maxwell L. Dental and anesthetic management of children with dystrophic epidermolysis bullosa. Int J Paediatr Dent 2009;19:127-34.   Back to cited text no. 1
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2.Wright JT. Comprehensive dental care and general anesthetic management of hereditary epidermolysis bullosa: A review of fourteen cases. Oral Surg Oral Med Oral Pathol 1990;70:573-8.   Back to cited text no. 2
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3.Lanier PA, Posnick WR, Donly KJ. Epidermoiysis bullosa dental management and anesthetic considerations: Case report. Pediatr Dent 1990;12:246-9.  Back to cited text no. 3
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4.Wright JT, Fine JD, Johnson L. Hereditary epidermolysis bullosa: Oral manifestations and dental management. Pediatr Dent 1993;15:242-7.  Back to cited text no. 4
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5.Pekiner FN, Yücelten D, Ozbayrak S, Sezen EC. Oral-clinical and management of epidermolysis bullosa. J Clin Pediatr Dent 2005;30:59-66.  Back to cited text no. 5
    
6.Wright JT, Childers NK, Evans KL, Johnson LB, Fine JD. Salivary functions of persons with hereditary epidermolysis bullosa. Oral Surg Oral Med Oral Pathol 1991;71:553-9.   Back to cited text no. 6
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7.Wright JT, Fine JD, Johnson L. Dental caries risk in hereditary bullosa. Pediatr Dent 1994;16:427-32.  Back to cited text no. 7
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8.Wright J, Capps J, Fine JD, Johnson L. Dental caries variation in the different epidermolysis bullosa disease. J Dent Res 1989;68:416.  Back to cited text no. 8
    
9.Howden EF, Oldenburg TR. Epidermolysis bullosa dystrophica: report of two cases. J Am Dent Assoc 1972;85:1113-8.  Back to cited text no. 9
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10.Endruschat AJ, Keenen DA. Anesthetic and dental management of a child with epidermolysis bullosa dystrophica. Oral Surg Oral Med Oral Pathol 1973;36:667-71.  Back to cited text no. 10
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11.Crawford EG Jr., Burkes EJ, Briggaman RA. Hereditary epidermolysisv bullosa: oral manifestations and dental therapy. Oral Surg Oral Med Oral Pathol 1976;42:490-500.  Back to cited text no. 11
    
12.Kahn SB, Trieger N. Epidermolysis bullosa hereditaria letalis--a case report with special emphasis on oral manifestations. J Oral Med 1976;31:32-5.  Back to cited text no. 12
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13.Album MM, Gaisin A, Lee KWT, Buck BE, Sharrar WG, Gill FM. Epidermolysis bullosa dystrophica polydysplastica: a case of anesthetic management in oral surgery. Oral Surg Oral Med Oral Pathol 1977;43:859-72.  Back to cited text no. 13
    
14.Carroll DL, Stephan MJ, Hays GL: Epidermolysis bullosa-review and report of case. J Am Dent Assoc 1983;107:749-51.  Back to cited text no. 14
    
15.Wright JT: Epidermolysis bullosa: dental and anesthetic management of two cases. Oral Surg Oral Med Oral Pathol 1984;57:155-7.  Back to cited text no. 15
    
16.Herod J, Denyer J, Golman A. epidermolysis bullosa in children: Pathophysiology, anesthesia and pain management. Pediatr Anaesth 2002;12:388-97.  Back to cited text no. 16
    
17.Siddiqui KM, Khan S. Anesthetic management of an infant with Epidermolysis Bullosa undergoing inguinal hernia repair. J Pak Med Assoc 2010;60:497-8.  Back to cited text no. 17
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18.Ames WA, Mayou BJ, Williams KN. Anesthetic management of epidermolysis bullosa. Br J Anaesth 1999;82:746-51.  Back to cited text no. 18
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19.Kostara A, Roberts GJ, Gelbier M. Dental maturity in children with Dystrophic Epidermolysis bullosa. Pediatr Dent 2000;22:385-8.  Back to cited text no. 19
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20.Nutritional management in the EB child. EB Currents (DEBRA Newsletter) 1988;9:1-3.  Back to cited text no. 20
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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