The main anesthetic management point to
remember is to use a nontouch technique to prevent induction of blisters.
Venous access can be difficult to obtain, and securing the intravenous
cannula is an issue because dressing can cause blistering. Therefore use of
Vaseline gauzes over and around the cannula followed by a light bandage
around the insertion site is recommended. Monitoring can cause new bullae,
and care must be taken to avoid adhesive electrode pads directly on the skin
whenever possible and to wrap the underlying skin with Vaseline gauze before
applying the blood pressure cuff. Needle electrodes for the
electrocardiogram have been used successfully instead. Airway management can
be difficult because of scarring around the mouth (microstomia) and of the
tongue, so a padded (air-cushion type, if possible) and well-lubricated
(Vaseline) face mask and laryngoscope blade must be used. Difficult airway
management is associated with a higher risk of new bullae formation. In
severe cases, fiberoptic intubation is needed because of restricted mouth
opening. Oral intubation is preferred, and an endotracheal tube half to one
size smaller than predicted should be used. Hoarse voice may be an indicator
for laryngeal bullae/scarring, also potentially requiring a smaller
endotracheal tube. Again, endotracheal tube securing may be complicated, and
suturing it in place is often the best choice. The lips should be well
lubricated too, especially where they come in contact with the endotracheal
tube. Laryngeal mask airway use with few complications from new bullae
formation has been reported. In addition, tracheal lesions do not appear to
occur frequently after intubation (may be explained by respiratory columnar
epithelium instead of squamous epithelium). Oropharyngeal suctioning should
be avoided or done under direct vision only (also to check for new bullae
before extubation) because it can easily cause bullae formation. Regional
anesthesia is not contraindicated if the skin over the region is unaffected,
but use of a sterile technique is imperative because these patients are prone
to infections. Avoid rubbing the skin for disinfection; use a spray such as
aqueous chlorhexidine instead. Finally, because of esophageal scarring,
affected patients are at higher risk for regurgitation and aspiration, so
antacid prophylaxis is recommended prior to surgery. Esophageal involvement
also leads to malnutrition as a result of poor feeding and sometimes
requires correction prior to elective surgery. Infections are more common
because of the broken epidermal barrier and should be treated prior to the
patient going to the operating room. Blood for transfusion should be easily
available in the operating room, particularly if the hemoglobin level
already is borderline. Administering a sedative premedication prior to
taking young children to the operating room may be wise to prevent them from
struggling during induction, which may lead to new bullae formation. Be
very careful while positioning these patients because inappropriate handling
may cause new bullae formation. Ideally, the patient should position
him/herself on a well-padded operating table. Corneal abrasions are common
among affected patients secondary to scarring of the eyelids with poor
coverage of the cornea, so lubricant eye ointment and Vaseline gauzes should
be applied. However, in small children, eye ointment resulting in blurred
vision may trigger eye rubbing with new blister formation postoperatively.
Avoid nasopharyngeal, esophageal, or rectal temperature probes. These
patients are often already in significant pain preoperatively, which will be
even worse postoperatively. Adequate pain relief is mandatory. Regional
anesthesia is an elegant and effective way to achieve this goal; however,
not all procedures are suitable for this approach. The oral and rectal
routes may not be options in these patients (dysphagia and anal
blisters/fissures, respectively), leaving the intravenous route as the only
option. Patient-, parent-, or nurse-controlled analgesia has been used
successfully in these cases.