Airway management in child with epidermolysis bullosa dystro
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Epidermolysis bullosa (EB) is a rare disorder characterized by fragility of the epidermis involving the formation of bullae with shearing force (‘Nikolsky’ sign), as well as ulcers and erosions after relatively minor trauma. These blisters form on the mucocutaneous surfaces due to inherited deficiencies in connective tissue structures (keratin, collagen, laminin).

The patient is a 12 year old left handed boy (1.27 m, 22.8 kg) with recessive DEB presenting with recurrent syndactyly and flexion contractures after syndactyly release surgery 5 years prior. He was scheduled for de-cocooning of the right hand with finger flexion contracture release and full thickness skin grafting (Figure 1). Pre-operatively, on examination of the airway the patient was noted to have limited mouth opening, and was a Mallampati II without limitation of neck extension.15mg of oral midazolam is given to patient to decrease anxiety. Temperature was monitored with a lubricated probe in the axilla..

A lubricated mask was used for induction, with 70% nitrous oxide and increasing concentrations of sevoflurane. After induction, the eyes were lubricated generously for protection. Intravenous access was obtained with a 22g catheter, which was wrapped with Vaseline gauze and KlingTM rolled gauze, covered with CobanTM..

The patient was then given 30 mg (1.3 mg/kg) of propofol and 30 mg (1.3 mg/kg) of rocuronium to facilitate endotracheal intubation after mask induction.gentle direct laryngoscopy with a Macintosh 2 blade to open the pharynx was performed by first provider while the second provider placed the FOB (Olympus Exera BF-XP160F 2.8mm pediatric fiberoptic bronchoscope), loaded with a 4.5 mm cuffed endotracheal tube, into the oropharynx and through the vocal cords. A few shallow pre-existing ulcers were noted in the oropharynx, and the carina was visualized before the ETT was advanced. Tracheal placement was confirmed with end tidal CO2 and chest rise. Optimal placement was confirmed 2 cm above the carina with the FOB. ETT was secured with a lubricant-soaked twill tie over Mepilex TM to distribute pressure. All points of contact between the ETT security system and the skin were lined with Vaseline gauze .

Anesthesia was maintained with sevoflurane and a dexmedetomidine infusion titrated from 0.7 to 2.2 mcg/kg/hr based on heart rate and blood pressure. The patient was ventilated on pressure control settings. Vital signs remained stable without need for vasoactive medications or additional monitors.

Prior to extubation, all anesthetics were discontinued and the effects of the muscle relaxant were reversed with sugammadex. With the patient still deeply anesthetized, the FOB was passed through the endotracheal tube to visualize the airway structures during extubation. The ETT cuff was then deflated and both the ETT and FOB were carefully removed. With the fiberoptic scope just past the tip of the ETT, the carina, trachea, larynx, and pharynx were assessed for any new trauma or lesions from baseline assessment.He was stable and discharged home the same day with plan for follow up surgery in two weeks.

Conclusively ,For patients with epidermolysis bullosa, even with a favorable airway exam, it is prudent to have a fiberoptic bronchoscope available should a difficult airway arise. Simple oral placement of the fiberoptic scope with direct laryngoscopic assistance may be a practical method to secure an airway, avoid extended mask ventilation and minimize oropharyngeal trauma. This technique also has the benefit of airway visualization, documenting lesions before and after the case, and confirm endotracheal placement prior to the start of surgery.