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 .
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 .
this case report describes the successful anesthetic management of a patient with eb presenting for syndactyly release .
a 6-year - old male child , weighing 14 kg ; a known case of eb since birth presented with congenital left hand syndactyly [ figure 1 ] .
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 .
left hand shows syndactyly and right shows iv canula secured with nonadhesive technique airway assessment shows anticipated difficult airway oral antibiotics were administered preoperatively .
intravenous ( iv ) line 22-g secured with use of emla and fixed with vaseline gauze .
premedication with iv fentanyl 20 g , midazolam 0.4 mg , and dexamethasone 2 mg was given .
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 .
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 ,
with use of lubricated macintosh laryngoscope trachea was intubated with uncuffed et no . 5 and tube secured with nonadhesive lubricated bandage [ 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 .
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 .
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 .
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 .
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 .
iv or intra - arterial access should be secured with bandage , cotton wrap , or suture .
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 .
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 . as our case was of a short duration with no expected major blood loss and hemodynamic shifts , use of nibp and tourniquet
therefore , clinical use of nibp and tourniquet in such patients depends on its merits and demerits .
microstomia , neck contracture , oropharyngeal lesion , ankyloglossia ( decrease mobility of the tongue ) , thickened epiglottis , and possible tracheal stenosis make maintenance of a patent airway challenging .
fiberoptic intubation is less traumatic to the mucosa than direct laryngoscopy and should be the first choice in eb patients with a difficult airway .
laryngeal , tracheal involvement in eb is rare because that tissue is pseudostratified , columnar , ciliated epithelium and whereas oropharyngeal , esophageal mucosa is stratified squamous .
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 .
regional anesthesia should be considered whenever possible but contractures , scars , and infections are the issues associated with regional anesthesia .
perioperative blister should be treated with liquid paraffin , silver sulphadiazine , or steroids . in ambulatory
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 .
minimal mandatory monitoring and no touch principle remains the key to successful anesthesia management of eb cases .
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 .
 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 . 