Sir, Behcet's disease, a multi-systemic inflammatory process of unknown aetiology is characterised by relapsing episodes of oral aphthous ulcers, genital ulcers, skin lesions and ocular lesions, first described 1937.[1] It is associated with involvement of the cardiovascular system, lungs, central nervous system (CNS), vessels and synovial surfaces. There are very few reports of patients with this disease posted for surgery. A 22-year-old male presented with complaints of swelling in the left scrotal region over the past few months. He gave a history of joint pain and swelling. Diagnosed as a case of ulcerative colitis with multiple strictures years back, he was operated for the same thrice under general anaesthesia. Later he developed oral and penile ulcers, was diagnosed as Behcet's syndrome and started on tablet prednisolone 10 mg, tablet mesalazine 400 mg once daily and tablet colchicine 5 mg twice daily. On examination, he had mild pallor (haemoglobin - 9 g/dl). Mouth opening was one finger breadth [Figure 1] with Mallampati class IV. Ulceration of the oral mucosa, acneiform skin lesions and genital scarring was noted. Echocardiography and magnetic resonance imaging of the brain were normal. The morning dose of oral prednisolone was continued and nil by mouth status and written informed consent confirmed. The patient was posted for hydrocoele and incisional hernia repair under spinal anaesthesia. An intravenous (IV) line was secured using 20 gauge cannula in a single prick and Ringer lactate solution was started. Injection hydrocortisone 50 mg and ondansetron 4 mg were given IV. Subarachnoid block was performed. 3 cc of bupivacaine 0.5% with 30 μg of injection clonidine was administered resulting in a block uptoT6. Pulse rate, blood pressure, respiration, electrocardiogram and oxygen saturation were monitored throughout. Surgery was completed uneventfully.Figure 1: Restricted mouth openingDiagnostic criteria for Behcet's disease as suggested by Mason and Barnes include: Major criteria-buccal ulceration, genital ulceration, eye lesions and skin lesions. Minor criteria-gastrointestinal lesions, cardiovascular lesions, thrombophlebitis, arthritis, CNS lesions and family history. Diagnosis of the disease is made only if three major or two major and two minor criteria are present.[2] Male sex and younger age group are associated with a more severe disease.[1] Since Behcet's disease is an inflammatory process, chronic use of anti-inflammatory and anti-neoplastic drugs is common.[3] Our patient was on tablet mesalazine and tablet colchicine. Mesalamine is a 5-amino salicylic acid based agent indicated for the treatment of ulcerative colitis. Nephrotoxicity can be seen with these compounds. Immunosuppressants reduce the activity of the immune system, thus interrupting the inflammation process that causes symptoms of Behcet's disease. Colchicine, an anti-inflammatory medication is found to be beneficial here. It may lead to haematologic, renal and musculoskeletal side effects on long-term therapy. Thus, a complete haemogram with blood cell count and renal function tests should be obtained pre-operatively. Chronic corticosteroid use can cause suppression of the adrenocortical response leading to cardiovascular collapse intra and post-operatively. Thus, supplemental corticosteroids are necessary on the day of surgery. Since these patients are prone to develop inflammatory skin lesions ultimately leading to ulcer formation, puncture of the skin and mucous membranes should be minimised. IV line should be secured with caution. General anaesthesia should proceed only after a thorough airway examination. Difficult airway could result from active oropharyngeal ulceration or scarring leading to limited mouth opening.[2] When considering awake intubation, anaesthesia of the airway should include topical application of local anaesthetics, not injectable airway blocks because of potential compromise of the airway from an inflammatory response to the local injection. In extreme cases, lesions can severely reduce the lumen of oropharynx and tracheostomy might be necessary for urgent surgery.[2] For elective procedures, awake fibreoptic intubation is usually preferred. The use of laryngeal mask airway can lead to aggravation of the oropharyngeal lesions. Repeated attempts at laryngoscopy and intubation should be avoided as tissue trauma can lead to further development of aphthous ulcers.[4] Regional anaesthesia is less ideal as puncture of the skin or mucous membranes is very likely to result in inflammation and nodular formation. Dangerous parenchymal CNS involvement affecting the brainstem, spinal cord and cerebral hemispheres and non-parenchymal manifestations including intracranial hypertension, aseptic meningitis, cranial neuropathy and cerebrovascular disorders are contraindications for the neuraxial block.[5] However, we preferred spinal anaesthesia considering that CNS involvement was ruled out and the problems associated with difficult airway were far more severe. Clonidine was added to prolong the duration of the neuraxial block. The main pre-operative anaesthetic concerns in Behcet's disease are therefore scarring of oral mucosa leading to difficult airway, inflammatory involvement of the organ systems, inflammatory response to skin prick and chronic immunosuppressant therapy which could cause renal and haematopoietic side effects. The case was successfully managed after a thorough knowledge of the disease and the anaesthetic implications involved.
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Jan 1, 2016
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