Epidermolysis bullosa: Careful monitoring and no touch principle for anesthesia management.

Epidermolysis bullosa (EB) is a rare genetic mechanobullous disorder, with excessive fragility of the skin and mucous membranes. Avoiding mechanical injury to the skin and mucous membranes is essential in the anesthetic management. Shearing forces applied to the skin result in bullae formation, while compressive forces to the skin are tolerated. The challenge is to use monitoring technology without damaging the epithelial surface. Difficult airway, positioning issues, nutritional deficiencies, poor immunity, and carcinogenic potential add to the comorbidities. We managed a child with EB undergoing syndactyly release. Ensuring maximal skin and mucous membrane protection, anesthesia in children with EB can be conducted with few sequelae.

Introduction

Epidermolysis bullosa (EB) is a rare genetic disorder characterized by abnormal fragility of skin and mucosal surface. The separation of skin layers occurs after application of friction or shearing forces and results in intradermal fluid accumulation and bullae formation.[1] This may lead to scarring and debilitating, life-threatening medical conditions.

In addition to considerations associated with positioning, monitoring, infection, and prevention of skin and mucosal trauma, anesthetic management of EB is uniquely challenging because of the effects on the airway. Careful clinical monitoring and no touch principle is the key for safe anesthetic management.

This case report describes the successful anesthetic management of a patient with EB presenting for syndactyly release.

Case Report

A 6-year-old male child, weighing 14 kg; a known case of EB since birth presented with congenital left hand syndactyly [Figure 1]. He was posted for syndactyly release. He required frequent hospital admissions for repeated episodes of chest infection and anemia. Significant preoperative findings were generalized scars, bed sores, pustules, and joint contractures. Airway assessment revealed Mallampatti Class III, microstomia, loose teeth, and poor oral hygiene with thyromental distance 3 cm. Patient also had limited neck extension secondary to scarring [Figure 2].

Figure 1

Left hand shows syndactyly and right shows IV canula secured with nonadhesive technique

Figure 2

Airway assessment shows anticipated difficult airway

Oral antibiotics were administered preoperatively. Informed consent was taken for surgery. Hydrocortisone skin ointment was applied to skin. Intravenous (IV) line 22-G secured with use of EMLA and fixed with vaseline gauze.

Premedication with IV fentanyl 20 μg, midazolam 0.4 mg, and dexamethasone 2 mg was given. Anticholinergics were avoided. Antacid and antibiotic prophylaxis were given with IV ranitidine 1 mg/kg and cefotaxime 50 mg/kg, respectively. Intraoperative heart rate, rhythm, and oxygen saturation were monitored with lubricated clip on pulse oximeter. Noninvasive blood pressure (NIBP) and electrocardiogram monitoring were avoided [Figure 3]. Patient was positioned supine and pressure points were padded with cotton. Due to anticipated difficult airway, a difficult airway cart was prepared with various size masks, laryngoscope, endotracheal tubes (ETs), ventilating bougie, and stylet. Though supraglotic airway devices were inappropriate in this patient they were kept ready for an emergency airway situation.

Figure 3

Minimal monitoring was used intraoperatively

Preoxygenation was followed by induction with IV propofol 20 mg along with oxygen and sevoflurane under gentle mask holding with vaseline gauze. After confirming ventilation, IV atracurium 0.5 mg/kg was given. Minimal pressure for chin lift and head tilt was used for ventilation. Airway was lubricated to prevent mucosal trauma. With use of lubricated Macintosh laryngoscope trachea was intubated with uncuffed ET no. 5 and tube secured with nonadhesive lubricated bandage [Figure 4]. Eye padding was done with cotton to prevent ophthalmic complications.

Figure 4

ET secure with nonadhesive technique

Anesthesia was maintained with oxygen, nitrous oxide, and sevoflurane with manual ventilation using Jackson Rees circuit. As rectal suppositories, subcutaneous and intradermal routes are generally not recommended; postoperative analgesia was provided with IV diclofenac sodium 25 mg 8 hourly. Regional block was avoided due to joint contracture and scars, which made location of anatomical landmarks difficult. Syndactyly release was performed without the use of tourniquet uneventfully [Figure 5]. Neuromuscular blockade was antagonized at the end of the procedure, and trachea extubated when adequate signs of spontaneous recovery were evident. Postoperatively, oral examination revealed small intra-oral blister formation not requiring active management. Patient was observed for 2 h in recovery room for signs of respiratory obstruction.

Figure 5

Syndactyly release performed without use of tourniquet

Discussion

EB encompasses an array of autosomal dominant and recessive conditions that may have either localized or generalized dermatological manifestations. The loss or absence of normal intracellular bridges is due to a collagen abnormality, which makes patient susceptible for blister formation by friction/shearing forces and subsequent scarring. Clinical, pathophysiological, and molecular criteria define more than 30 subtypes of EB.[2]

Anesthetic management is challenging, as airway manipulations can be hazardous. Equipment and techniques routinely used in the induction and maintenance of general anesthesia can be the source of serious postoperative complications.

Anesthesia is frequently required for multiple surgeries like daily dressings, dental procedure, esophageal dilatation, gastrostomy, contracture release, and syndactyly release. Malnutrition, anemia, and decreased immunity are present in patients due to decrease oral intake secondary to oropharyngeal and esophageal lesions. Malnutrition can leads to hypoprotienemia, anemia and electrolyte imbalance which may affect pharmacokinetic effects of anesthetic agents. Infection is common as patients often have both poor immunity and long-term corticosteroid treatment. Perioperative hydrocortisone is required to compensate adrenal suppression.[3] Antacid prophylaxis is required due to history of reflux, regurgitation, or esophageal stricture.

Patients with EB are prone for ophthalmic complications like corneal erosion, conjunctival symblepharon, and ectropion. Dental problems like cleaning difficulty, poor eating patterns, enamel dysplasia, carious, and loose teeth are frequently associated.

Sedative premedication can be useful in younger patients to avoid restlessness and injury. EMLA cream can be used successfully but occlusive dressings should be avoided.[4] IV or intra-arterial access should be secured with bandage, cotton wrap, or suture. Difficult venous access necessitates inhalational induction.[4]

In general, management of patients with EB is one of a “no touch” principle.[35] All persons involved in handling these children must be aware of the extreme vulnerability of skin. During transport or mobilization of the patient, the most important task is to maintain the integrity of the skin and avoid friction and trauma. Trolleys and operating tables should be well padded. Patients should be allowed to move themselves on the operating table.

Minimal monitoring is advocated for the patient with EB.[346] Standard monitoring may not be possible. Electrocardiogram monitors should be secured with bandage and adhesive portion should be removed. Pulse oximetry is the preferred method of monitoring heart rate. Direct pressure to the skin is not as damaging as frictional or shearing forces, so NIBP and tourniquet can be used with pressure and duration limitations.[7] As our case was of a short duration with no expected major blood loss and hemodynamic shifts, use of NIBP and tourniquet were avoided as chances of bullae formation still persist. Therefore, clinical use of NIBP and tourniquet in such patients depends on its merits and demerits.

Induction of anesthesia should be smooth.[8] Propofol is preferred over thiopental as EB is linked with porphyria. Ketamine and etomidate are associated with excitation and involuntary movements. Suxamethonium should be used with caution due to hyperkalemic potential.[3] Use of inhalational agents is safe.

Microstomia, neck contracture, oropharyngeal lesion, ankyloglossia (decrease mobility of the tongue), thickened epiglottis, and possible tracheal stenosis make maintenance of a patent airway challenging.[9] Oxygen masks with sharp edges should be strictly avoided. Facemasks, oropharyngeal, nasopharyngeal airways, and laryngoscope should be lubricated.[8] Chin lift and head tilt should be minimal and gentle. Small-size ET should be used and should be fixed with nonadhesive technique. Fiberoptic intubation is less traumatic to the mucosa than direct laryngoscopy and should be the first choice in EB patients with a difficult airway.[1] Laryngeal, tracheal involvement in EB is rare because that tissue is pseudostratified, columnar, ciliated epithelium and whereas oropharyngeal, esophageal mucosa is stratified squamous.[10] Hence, tracheal intubation is safe.[7] Pharyngeal suctioning should be nontraumatic. Supraglottic airway increases bullae formation but can be used in difficult airway scenario with appropriate care. After application of moisturizing ophthalmic gel, eyes should be covered with moistened gauze to protect from mechanical trauma.[39] Urinary tract is also affected so urinary catheters should be well lubricated before insertion.[11]

Excellent analgesia is important to prevent excessive movements and new skin trauma. A multimodal approach using nonsteroidal analgesics and opioids is the most convenient method.[12] Regional anesthesia should be considered whenever possible but contractures, scars, and infections are the issues associated with regional anesthesia. Rubbing of skin with antiseptic solution should be avoided. Local skin infiltration should not be given.[10] Rectal suppositories are not recommended because of the risk of rectal lesion.[9]

Perioperative blister should be treated with liquid paraffin, silver sulphadiazine, or steroids. In ambulatory setting, small procedures can be performed under analgosedation without airway manipulations.[11]

Careful monitoring, transport and positioning was instituted in the present case to avoid undue skin trauma. Atraumatic ventilation and intubation was possible with use of vaseline gauge and gentle mask holding. Mucosal harm was limited with the use of lubricated laryngoscope and airway. Minimal mandatory monitoring and no touch principle remains the key to successful anesthesia management of EB cases.

Conclusion

Patients with EB present a unique challenge for all anesthesia-care providers. With maximal skin and mucous membrane protection, anesthesia in children with EB can be conducted with few sequelae. Hence, meticulous execution of preoperative planning is indispensable for ensuring a favorable intraoperative and postoperative course.