Anesthetic management of adults with Epidermolysis bullosa
Melissa Brooks Peterson MD and Judit Szolnoki MD
Epidermolysis bullosa (EB) is a group of rare epithelial disorders marked by extreme skin fragility and caused by abnormal or absent structural proteins at the epidermal-dermal junction. Its severity is very variable; some patients can lead relatively normal lives others die in infancy. In last week’s PAAD, I reviewed the original article Anesthetic Management and Outcomes of Patients with Epidermolysis Bullosa: Experience at a Tertiary Referral Pediatric Center. Anesth Analg. 2021 Sep 30. doi: 10.1213/ANE.0000000000005749. Online ahead of print. PMID: 34591805. I’ve asked Melissa Brooks Peterson, a regular contributor to the PAAD and the previous paper’s first author, and Judit Szolnoki, the senior author of that paper to write today’s PAAD which discusses the anesthetic management of adult patients with EB. Rather than reviewing the pathophysiology of EB, I’ve asked them to zero in on the anesthetic management differences as described in these papers. Myron Yaster MD
Original article
Mittal BM, Goodnough CL, Bushell E, Turkmani-Bazzi S, Sheppard K. Anesthetic Management of Adults With Epidermolysis Bullosa. Anesth Analg. 2022 Jan 1;134(1):90-101. PMID: 34403382
Mittal et al. stress the importance of the preoperative evaluation and underscore the need to communicate with everyone involved perioperatively (nurses, trainees, surgical technicians, etc.) of the patient’s extreme vulnerability. They also stress the need to allow extra surgical time when scheduling these patients and to make them first starts of the day when possible. We absolutely agree!
In the preoperative physical examination section, as in our paper, Mittal et al. stress the importance of assessing mouth opening which often is very limited (maybe only a millimeter or 2 due to stricture formation at the oral orifice as the disease progresses). They also assess for hoarseness or inspiratory stridor to detect laryngeal involvement. To be honest, we rarely (if ever) find laryngeal involvement to be an issue. Additionally, they seek a history of urethral stenosis for procedures where a foley catheter may be considered. Again, the need for a Foley is very rare in our experience, and in our estimation the placement of a Foley can cause more sloughing, blister and wound formation, which is why we avoid Foley placement if at all possible. Finally, they state that “many EB patients have chronic pain from blistering, denuded skin, and contractures that can result in immobility. A detailed medication reconciliation should be done to assess chronic pain requirements and antidepressant therapy”. Although, we did not mention this in our paper, this approach makes a lot of sense. We agree that expecting tolerization to pain and sedation medications should be expected, and a close assessment of a patient’s medication baseline can help guide appropriate pain and sedation medication titration.
Perhaps not surprisingly, Mittal et al. are adult anesthesiologists and they recommend placing an awake IV and using it for anesthetic induction. They also state that “Intravenous induction is preferred in adults with EB to avoid facial trauma and an excitatory phase during inhalational induction”. We agree that for adults with EB, it may be most prudent to place an IV awake – but this approach (vs. an inhaled induction in an adult) must be weighed against the degree of difficulty of IV placement and the risk of multiple attempts, ‘blown’ veins that then cannot be used again, and the trauma of many attempts. One “compromise” that we would frequently consider would be to bring the patient to the OR and utilize inhaled nitrous oxide for comfort during a challenging or prolonged IV attempt, and then proceed with IV induction. Regardless of the approach, IV placement in EB patients can be one of the most difficult aspects of the anesthetic and ultrasound guidance is often required. We also agree with avoidance of infiltration of local for PIV placement, and subcutaneous injection will cause blister and wound formation almost immediately.
Regardless of the method of induction (which should be tailored to each patient), one key for safe and atraumatic mask ventilation regardless of when it occurs is the use of special lubrication for creating a seal without generating any sheer force on the skin: 3 packets of surgilube + 1 tube of eye ointment to lubricate the mask, gloves of the airway operator, and anything else that comes into contact with the patient’s skin.
The biggest – and most important – differences in the 2 papers involves airway management. Although both papers agree that airway management is one of the most challenging aspects of anesthetic management, Mittal et al. recommend and demonstrate oral intubation techniques. We firmly disagree and believe that nasal fiberoptic intubation is the BEST way to atraumatically manage the airway of a patient with EB. Unlike Mittal et al., in our experience, approaching the oral cavity of an EB patient with any device (DL, VL, hyperangulated VL), oral airways, and (especially) supraglottic airways will always cause trauma – because even the most atraumatic oral manipulation causes trauma to the oral mucosa of a patient with EB. Additionally, most oral devices are actually quite difficult to use atraumatically because of limited mouth opening, with subsequent potential damage to oral mucosa and teeth. Bottom line: the nasal mucosa is histologically different than oral mucosa and therefore far less prone to the blistering and wounds common with EB, and the best approach is to avoid the most “at risk” mucosa entirely and opt instead for the nasal approach as routine.
Finally, Mittal et al., demonstrate a novel method on how to secure the tracheal tube. They recommend placing a surgical mask under the patient’s occiput, bringing the ties up past the cheeks (over padding), and knotting them securely around the endotracheal tube. In our paper, and as discussed in a previous PAAD a nasal RAE tube can be secured indirectly and, when using the method we have developed over the last 3 decades, does not need to be taped or tied directly in order to provide a secure airway. This is another benefit of using a nasal RAE tube, because it lends itself to indirect securement better than any oral tube.
Mittal et al., recommend a smooth emergence with minimal coughing and patient movement. As we all know, there are many ways to accomplish this in our field. Obviously in EB patients agitation, excessive movement and bucking may produce new blisters or airway bleeding due to rupture of blisters. The team describes “a low-dose remifentanil infusion to facilitate a smooth awake tracheal extubation”. We suspect that this will work well, however, in our practices we rely on careful titration of a potent pain medication that will continue to provide analgesia throughout emergence, extubation and recovery, (usually fentanyl), we consider dexmedetomidine to augment a calm emergence. Importantly, we recommend an awake extubation in the operating room environment so that the anesthesiology team can entirely manage the patient throughout emergence, as a way to ensure continuous, meticulous skin protection and atraumatic airway management.
Finally, Mittal et al. discuss how to use regional anesthetics and catheters in EB patients. We only have limited experience with this (see case report of a four-quarter amputation, PMID 28459718) because the majority of the procedures that we do do not benefit from or require regional anesthetic techniques. We agree that offering regional is important for patients with EB, with application of the principles of touch-free/ atraumatic approaches to performing regional. Their recommendations of securing catheters with Mepitac (Mölnlycke Health Care) mirror our approach to IV securement, appear very reasonable, and we are appreciative of their thoughtfulness about non-opioid and regional analgesia for this patient population.
Melissa Brooks Peterson MD, Judit Szolnoki MD