Airway management in patients with pemphigoid lesions has anaesthetic implications. We report a case of a 23 years old female with bullous pemphigoid who presented with laryngeal stenosis and critical airway narrowing. The airway was initially managed with jet ventilation. Anaesthesia was maintained with propofol infusion and ventilation was performed by introducing a size 10 French gauge suction catheter through the stenotic laryngeal orifice. Thirty minutes into anaesthesia, she developed subcutaneous emphysema and decreased air entry on right side of the chest but remained hemodynamically stable. The airway was further managed by tracheostomy. This case report highlights complications that can occur during the anaesthetic management of such cases

Khan, Fauzia Anis

Yasir, Mohammad

English

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eCommons@AKUDepartment of Anaesthesia Medical College, PakistanAugust 2013Airway management in a patient with bullouspemphigoidMohammad YasirFauzia Anis KhanAga Khan University, fauzia.khan@aku.eduFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_anaesthPart of the Anesthesia and Analgesia Commons, and the Anesthesiology CommonsRecommended CitationYasir, M., Khan, F. A. (2013). Airway management in a patient with bullous pemphigoid. Journal of the College of Physicians andSurgeons Pakistan, 23(8), 583-585.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_anaesth/155INTRODUCTIONBullous pemphigoid is a chronic autoimmune blisteringdisorder of skin and mucous membrane. Females areaffected more than males.1 It is characterized by tensebullae on the skin and mucous membrane, mostcommonly in the intertriginous areas but may alsoinvolve other regions.2 The diagnosis is made on thebasis of clinical presentation, history, and immune-pathological studies. Oral mucosa can be involved inone-third of patients.3 In approximately 50% of patientswith oral pemphigoid the disease can progress to extra-oral sites such as the eye, larynx, pharynx oresophagus.3We report a case of bullous pemphigoid that presentedwith laryngeal stenosis and critical airway narrowing thatwas initially managed with jet ventilation. Airwaymanagement difficulties in such a case are describedand discussed.CASE REPORTA 23 years old female patient weighing 53 kilogramspresented with 3 years history of progressive bullouspemphigoid. She was on oral steroid 15 mg twice a day.She presented to the dermatology clinic with complaintsof multiple blisters over the body and inability to openboth eyes. She was referred to ophthalmology clinicwhere she was found to have bilateral blepharospasmand chemosis. On further enquiry and examination, shehad significant cough, dyspnea and hoarseness for thepast 5 months. Pulmonary function tests showed severeobstruction without reversibility (FEVI/FVC = 32% pre-bronchodilation and 33% postbronchodilation). She hadno previous history of asthma. Bronchoscopy reportmentioned inability to pass the adult fibreopticbronchoscope beyond the vocal cord and a scarredlaryngeal inlet. She was referred to the ENT surgeonwho performed an awake fibreoptic examination whichrevealed laryngeal oedema with excessive supraglottictissue. The CT scan report revealed laryngeal stenosisat the level of glottis with lumen opening approximately5 mm and normal vocal cords below the lesion (Figure 1).She was planned for micro-laryngoscopic excision ofsupraglottic lesion and blepharoplasty. The patient wasthen assessed by a consultant anaesthetist.Pre-anaesthetic assessment revealed anaemia (Haemo-globin level of 10.8 mg/dl), skin blisters and restrictedfunctional activity. Chest was normal shaped. A wheezewas audible but this appeared to be due to extra-thoracic obstruction at the level of laryngeal inlet. ChestX-ray was normal. She was not pre-medicated withbenzodiazepine but was administered 100 mg intra-venous hydrocortisone half hour before anaesthesia.Journal of the College of Physicians and Surgeons Pakistan 2013, Vol. 23 (8): 583-585 583CASE REPORTAirway Management in a Patient with Bullous PemphigoidMohammad Yasir and Fauzia Anis KhanABSTRACTAirway management in patients with pemphigoid lesions has anaesthetic implications. We report a case of a 23 years oldfemale with bullous pemphigoid who presented with laryngeal stenosis and critical airway narrowing. The airway wasinitially managed with jet ventilation. Anaesthesia was maintained with propofol infusion and ventilation was performed byintroducing a size 10 French gauge suction catheter through the stenotic laryngeal orifice. Thirty minutes into anaesthesia,she developed subcutaneous emphysema and decreased air entry on right side of the chest but remainedhemodynamically stable. The airway was further managed by tracheostomy. This case report highlights complications thatcan occur during the anaesthetic management of such cases. Key Words: Bullous pemphigoid.   Jet ventilation.   Tracheostomy.   Laryngeal stenosis.   Critical airway narrowing.   Airway management.Department of Anaesthesia, The Aga Khan University Hospital,Karachi.Correspondence: Dr. Fauzia Anis Khan, Department ofAnaesthesia, The Aga Khan University Hospital,P. O. Box. 3500, Stadium Road, Karachi-74800.E-mail: fauzia.khan@aku.eduReceived: June 02, 2012;   Accepted: October 10, 2012.Figure 1: CT Scan image taken at the level of 4th cervical vertebrae showingslit like airway. Local anaesthesia to the respiratory tract was providedwith 4 mls of 4% nebulized lignocaine. Standardmonitors were applied with extreme caution in order toprevent skin injury and to avoid further blister formation.Her baseline respiratory rate was 20 breaths per minutewith peripheral SpO2 of 97% on room air. Pre-oxygenation was then started with 100% oxygen usingthe circle system.She underwent inhalation induction with sevoflurane,nitrous oxide and oxygen. After checking adequacy ofmask ventilation, a bolus dose of 50 mg succinylcholinewas administered. Gentle laryngoscopy was performedwith Macintosh laryngoscope size three blade. Vocalcords were visualized and a size 10 Nelaton catheterwas introduced in the trachea.Manual jet ventilation was started with clinical monitoringof chest expansion and airway pressure. No wheezewas audible on chest auscultation. Anaesthesia wasmaintained with propofol infusion 6 mg/kg/hour. Fentanyl(50 microgram bolus) was administered for analgesia.When the patient reversed from the effects ofsuxamethonium, 25 mg atracurium was administered.Approximately 30 minutes after the start of surgery,subcutaneous emphysema was observed by theanaesthetist on the right side of neck. On chestauscultation, there was decreased air entry on the rightside, Nelaton catheter was pulled back but air entry stillremained less. Hemodynamically, the patient remainedstable. Trauma to lung was suspected and this wascommunicated to the surgeon. The surgeon decided toperform a tracheostomy and a size 6 tracheostomy tubewas inserted. Airway pressure was kept less than 20cmH2O. The rest of surgical procedure was completeduneventfully. At the end of procedure, both supine andlateral decubitus chest X-ray was done which showedsubcutaneous emphysema but no pneumothorax.Fibreoptic bronchoscopy revealed no obvious lesion andshowed normal area below tracheostomy site. Patient was shifted to recovery room on tracheostomymask maintaining normal oxygen saturation where sheremained stable throughout. Her subcutaneous emphy-sema settled and she was discharged 2 days later.DISCUSSIONThis case report discusses the management of difficultairway with jet ventilation in a patient with bullouspemphigoid. This therapy is generally used as bridgingtherapy in the management of laryngeal airwayobstruction until a definitive therapy is available.4 In thiscase, use of jet ventilation was associated with thecomplication of subcutaneous emphysema.Pemphigoid disease includes subgroups like bullouspemphigoid, cicatricial pemphigoid, localized scaringpemphigoid and Herpes gestations.2 Though similar toepidermolysis bullosa, the disease has its own uniquefeatures. Bullous pemphigoid is a skin disorder, andpredominantly a disease of fifth decade of life.2 There isextensive literature on anaesthesia with epidermolysisbullosa but literature describing anaesthetic manage-ment of both bullous or cicatricial pemphigous is rare.Cicatricial pemphigoid is a variant of bullous pemphigoidwhich primarily affects mucosal surfaces but 25% ofpatients have cutaneous lesion.5Airway management in patients with bullous pemhigoidlesion is of particular concern. Airway management canbe difficult because of pre-existing bullous lesions in theoropharynx, or ulcerations, adhesions or deformities oforopharyngeal mucosa or vocal cords as a consequenceof these lesions. Patients can present with stridor ordyspnea due to upper airway obstruction when thelaryngeal orifice is narrowed to three or four millimetersand some can present with acute respiratory distress inemergency.6 This patient had a history of dyspnea andhoarseness but not acute stridor. There was narrowingin the supraglottic area just above the cords and therewas inability to pass the fibreoptic bronchoscope beyondthe narrowed laryngeal inlet. One option was a pre-operative tracheostomy under local anaesthesia,however, the surgeon was reluctant to take this routeinitially because of the high incidence of scarring andstenosis at the tracheostomy site.Airway management itself may also be difficult in thesecases. Airway instrumentation can cause acutebullae formation and there is risk of haemorrhage.Recommended precautions are liberal lubrication of thelaryngoscope blade, and the tracheal tube as well asgentle laryngoscopy and use of smaller sized tubes.7Since this patient had severe supraglottic airwaynarrowing, we opted for jet ventilation through a size 10Nelaton catheter for airway management along with totalintravenous anaesthesia. Unfortunately, it resulted insubcutaneous emphysema, a known complication of jetventilation.8 The probable cause was insufficient outflowdue to narrowing at the inlet which lead to excessiveairway pressure and surgical emphysema. Because ofthe complication, the patient ended up with tracheo-stomy which was initially avoided. The patient was lostto follow-up post-discharge because she had come fromanother city.Carbon dioxide laser technique is now used for removalof supraglottic scarring, but this is not available in ourinstitution. One of the advantages of laser technique isthe reduction in surgical time. Availability of laser couldhave decreased the surgical time in this patient and thismay have resulted in preventing the morbidity which onlyoccurred after 30 minutes into surgery.In conclusion, this case highlights the problem of airwaymanagement in a patient with bullous pemphigoidand complications that can occur with jet ventilation viathe catheter technique. Management of these casesMohammad Yasir and Fauzia Anis Khan584 Journal of the College of Physicians and Surgeons Pakistan 2013, Vol. 23 (8): 583-585requires constant vigilance on the part of anaesthetistwho should be aware of such potential complication.REFERENCES1. Budimir J, Mihic LL, Situm M, Bulat V, Persic S, Tomljenovic-Veselski M. Oral lesion in patient with pemphigus vulgaris andbullous pemphigoid. Acta Clinica Croatica 2008; 48:13-8. 2. Prasad KK, Chen L. Anaesthetic management of a patient withbullouspemphigoid. Anesth Analg 1989; 69:537-40.3. Iqbal M, Ahmed R, Kashef SH, Bahame PS. Laryngealinvolvement in a patient with bullous pemphigoid. Ann SaudiMed 2006; 26:152-4.4. Ort SA, Goldenberg D. Transoral management of obstructingtumour of upper airway. Operat Tech Otolaryngol 2007; 18:156-9.5. Drenger B, Zidenbaum M, Reifen E, Leitersdorf E. Severeupper airway obstruction and difficult intubation in cicatricialpemphigoid. Anaesthesia 1986; 41:1029-31.6. Lazor JB, Varvares MA, Montgomery WW, Goodman ML,Makool BT. Management of airway obstruction in cicatricialpemphigoid. Laryngoscope 1996; 106:1014-7.7. Mahalingham TG, Kathirvel S, Sodhi P. Anaesthesia manage-ment of a patient with pemphigus vulgaris for emergencylaparotomy. Anaesthesia 2000; 55:160-2.8. Evans E, Biro P, Bedforth N. Jet ventilation. Cont EducAnaesth Crit Care Pain 2007; 7:2-5.Airway management in a patient with bullous pemphigoidJournal of the College of Physicians and Surgeons Pakistan 2013, Vol. 23 (8): 583-585 585

Airway management in a patient with bullous pemphigoid

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Airway management in a patient with bullous pemphigoid

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