What is the basic histological lesion? Are there different forms of epidermolysis bullosa? What are the broad categories? Of what medical, clinical, and anesthetic significance are the associated comorbidities?

With specific reference to the planned surgery, what unique areas are essential to your preoperative discussion with your surgical colleagues? Nursing colleagues? PACU nurses?

What is the significance of his treatment with anabolic steroids?

How will you evaluate the airway? What are the risks of airway instrumentation/manipulation?

There are four basic types of epidermolysis bullosa (EB) that differ phenotypically, genotypically, and histologically [1]. While milder forms of EB simplex will commonly present in late childhood or even during adult years, pediatric anesthesiologists will encounter earlier onset and more severe forms such as junction and dystrophic EB and Kindler syndrome, with multiple skin levels of blister formation. Mechanically fragile skin with easy blistering or erosions is characteristic. Nails may be dystrophic or absent, and granulation tissue may be exuberant especially periorally. In general, the more severe and widely distributed the blistering on the skin, the more likely it is for extracutaneous sites (trache, esophagus, ocular, corneal) to be involved. Dental enamel hypoplasia is seen in all subtypes of junctional EB. There is an increased risk (44 % by age 55) of developing squamous or basal cell carcinomas and even melanomas [1]. The Herlitz type of junctional EB, which is what this patient has, is present from birth and typically involves the upper airway as well as all skin surfaces. The severity of intraoral blistering (and their inevitable healing pattern which ultimately results in chronic scarring) may eventually narrow the mouth and the laryngeal inlet and also result in ankyloglossia, similar to the glossoptosis in Pierre-Robin syndrome. The cumulative risk of laryngeal stenosis or stricture is 40 % by 6 years of age. Fifty percent of patients die within the first 2 years of life [1]. Children are also often anemic because of the constant injury and healing process and the skin’s metabolic utilization of iron.

Good preoperative rapport between every member of the OR team, the patient, and the parents is crucial in order to smooth the induction of anesthesia. Adequate premedication may be crucial to ensure a smooth induction. Specific induction methods such as intramuscular or rectal medication may be needed to forestall struggling.

Anabolic steroids (testosterone derivatives) are typically given to stimulate appetite and increase muscle mass because it is almost impossible for children to keep up with the constant metabolic challenge of chronic skin injury and repair. Obviously, transdermal delivery systems are contraindicated; parenterally administered anabolic steroids are acceptable. Orally administered anabolic steroids may have associated hepatotoxicity. The risks of anabolic steroids include personality (i.e., aggressiveness) alterations, hypertension, hypercholesterolemia (paradoxical), feminization as a result of suppression of natural testosterone levels, focal segmental glomerulosclerosis, and an increased oxygen consumption (therefore an increased carbon dioxide production and minute ventilation requirement under anesthesia) in the setting of an already increased metabolism from skin turnover.

Airway evaluation begins with physical diagnosis; anticipated problems would include perioral blisters, scarring, microstomia, intraoral blisters and scarring, restricted head and neck motion, esophageal strictures/stenosis, and laryngotracheal stenosis. Any of these can be made worse with airway manipulation following the procedure [3].

Does this patient need an IV in order to facilitate a “quiet” induction? How will you accomplish this?

The medical student suggests an IM preinduction dose of ketamine and midazolam; do you think this is a good idea?

An elderly attending says “in the old days we would use rectal methohexital to get started.” What do you think about this idea?

You proceed with a mask induction with a well-lubricated mask. What airway management considerations do you have? Do you anticipate having any difficulty visualizing the airway?

What are your equipment considerations?

Can this case be done without intubation of the trachea? What are your choices? What are your risk-benefit considerations for those choices?

How will you monitor this patient? For each “routine” monitor, how are your choices influenced by the underlying disease?

What are your positioning considerations?

If a “natural” airway is chosen, how will you maintain anesthesia?

The surgeons would like to do a penile block for the circumcision. What are your recommendations?

The dentists would like to inject local anesthetic with a vasoconstrictor to control bleeding and provide for analgesia post-op. What are your recommendations?

A calm induction is best, no matter how one accomplishes it. A collaborative approach among the OR team and parents is best; generous premedication may help considerably if needed. This can be accomplished orally with midazolam, rectally with methohexital, or even parenterally (spray the skin prep rather than rubbing the skin). If an IV option is chosen, especially in patients who are wrapped in bandages, it will be wise to have the parent remove the bandages, for patient comfort. With skin blistering, scar formation, and possibly pseudosyndactyly in certain forms of EB, the parent will be the best guide for IV placement. The need for restraint must be avoided, however. Securing of an IV can be accomplished with tape over nonadhesive dressings; they may also be sutured in place.

Within the general guidance above, it is an acceptable alternative as long as restraint is avoided, and the area to be injected is not rubbed with a prep pad but rather sprayed with alcohol, betadine, or chlorhexidine.

Again, it is a perfectly acceptable idea as long as the perianal area is carefully examined with regard to blistering and skin irritation [4]. The usual induction dose (methohexital) or 25–30 mg/kg (10 % solution) may have to be increased if the patient is on concurrent drug therapy such as anabolic steroids.