An 8-year-old boy with epidermolysis bullosa (EB) presents for the insertion of a gastrostomy tube. He has a severe form of the disease and is completely wrapped in dressings. All his fingers and toes are fused, and his mouth opening is very limited. No veins are visible or palpable.
More than 20 different forms of epidermolysis bullosa exist, with different degrees of severity. All have in common that friction and shearing forces cause blistering, whereas pressure does not. Therefore, blood pressure cuffs are not problematic, but even a gentle chin lift can cause blistering. The blisters then form scars, which lead to deformity.
The scar formation after oral and pharyngeal blistering leads to limited mouth opening, dysphagia, and malnutrition. Esophageal scarring leads to dysmotility and gastroesophageal reflux. Scarring of the digits and toes causes pseudosyndactyly (mitten deformity). Anal fissures lead to chronic constipation. Chronic open wounds lead to anemia and to zinc, vitamin, and iron deficiency. Most patients suffer from chronic pain because of pressure in a new blister or infected skin.
Avoid prolonged mask ventilation to minimize the friction associated with handling the mask. Lubricate the mask.
Laryngoscopy is safe as long as all equipment is lubricated.
Prepare for a difficult intubation.
Remove all adhesive parts from your electrocardiogram stickers and blood oxygen saturation probe. Ear clips or forehead sensors are useful in the absence of fingers and toes.
Obtaining IV access can be difficult, particularly in a child that will blister from moving during cannulation. Consider premedication with midazolam and inhalation of nitrous oxide to facilitate IV placement. Inhalational induction in patients with an expected easy airway can cause less friction than starting an IV in an uncooperative child.
Postoperative monitoring is just as difficult as intraoperative monitoring. A calm and pain-free child is the postoperative goal, since agitation will lead to movements that will lead to blistering.
DOs and DON’Ts
✓ Do insist on adequate monitoring.
✓ Do prepare for a difficult intubation.
⊗ Do not apply friction.
⊗ Do not slide the patient from the bed to the operating room table; lifting avoids friction.
✓ Do use a polyurethane foam dressing coated with silicone (Mepiform) instead of tape.
✓ Do listen to caregivers; they usually know exactly what causes blistering and what does not.
At some centers, bone marrow transplants are performed; their role is still controversial.
Most surgical and dental management is as conservative as possible, because of the concern about blistering of the surgical site. The need for adequate nutrition with a gastrostomy tube needs to be weighed against the surgical and anesthetic risk.