Severe angioneurotic oedema causing acute airway obstruction.

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544 Journal of the Royal Society of Medicine Volume 81 September 1988. Case reports. Case presented to. Clinical Section,. 13 March 1987.
544

Journal of the Royal Society of Medicine Volume 81 September 1988

Case reports

Case presented to Clinical Section, 13 March 1987

Severe angioneurotic oedema causing acute airway obstruction

F Self MB ChB

G J E M Bates BSc FRCS

A Drake-Lee FRCS Department of Otolaryngology, The Royal United Hospital, Coombe Park, Bath BAl 3NG Keywords: angioneurotic oedema; flexible bronchoscope; flexible laryngoscope; intubation; Benylin expectorant

A patient with severe angioneurotic oedema of the tongue and floor of mouth is presented. Two aspects of this case are emphasized. Firstly, the management of the airway obstruction by intubating the patient using the flexible bronchoscope. Secondly, the possible aetiology of the angioneurotic oedema which may have occurred because of a reaction to Benylin expectorant.

Case report A 77-year-old retired publican was admitted to casualty at 07.00 h with severe swelling of his tongue (Figure 1). The previous evening the patient had gargled with Benylin expectorant for 20 min. He woke at 0500 h with a grossly swollen tongue. The oedema rapidly progressed to include the floor of his mouth and upper larynx. He was intubated by passing an endotracheal tube over a flexible bronchoscope, using a 'railroading' technique. Relevant past history was that in March 1985 the patient was diagnosed as having a centroblastic/ centrocytic lymphoma. He was treated with chemotherapy and since July 1985 has been in remission. There was no history of allergy or asthma and there were no previous episodes of angioneurotic oedema. After 48 h, the oedema subsided and the patient was extubated. In outpatients, the patient was given stanozolol 5 mg once daily for a week. This androgen derivitive is believed to stimulate depressed complement levels. The effects of the drug on the patient's complement levels can be seen in Figure 2. Four months after his admission, the patient remains well.

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Discussion Within 30 min of being in hospital, the patient required an alternative airway. The intubation technique used was to 'railroad' the endotracheal tube over a flexible bronchoscope which had been passed through the vocal cords. This method is best performed with the patient sitting upright. The mucosa of the widest nostril is anaesthetized by painting on 25% cocaine paste. It is important to then pass the endotracheal tube down the length of the nostril to make sure it will fit through the posterior choana. The tube is then left in this half way position. The bronchoscope is fed down the endotracheal tube so that it protrudes

out through the end of the tube into the postnasal space, from where a good view of the larynx can be obtained. From this position the scope is passed through the vocal cords and the endotracheal tube is advanced in a 'railroading' fashion into the larynx. The importance of this technique is that in certain patients it will allow a tracheostomy to be avoided. Angioneurotic oedema is a swelling in the submucosal or subcutaneous tissues caused by increased capillary permeability. The aetiology can be classffied according to whether there is an immunological basis or not.

Journal of the Royal Society of Medicine Volume 81 September 1988

The immunological group contains reactions which occur because of complement abnormalities; an example is the condition hereditary angioneurotic oedema. This is a dominantly inherited disorder and is characterized by recurring attacks of angioneurotic oedema. There is a functional deficiency of the first complement factor inhibitor (Cl INH). The Cl component is present in normal concentrations. Without this Cl inhibitor, Cl activation readily occurs and this results in cleavage products which have kinin activity. It is these products which cause the angio-oedema1. An acquired form of complement mediated angioneurotic oedema was described in 19722. It was noted to occur in patients with lymphomas. The clinical picture is indistinguishable from the hereditary form, but there is no family history. The biochemical difference is that the Cl component is low as well as the other complement abnormalities found in the hereditary form of the disease. Many of these patients have been noted to have an abnormal paraprotein2 and it is thought to be this which activates and so depletes the complement pathway. The non-immunological mechanisms for generating angioneurotic oedema are probably brought about by

the direct action of drugs on the mast cell membrane'. In this way vaso-active mediators are released which increase capillary permeability. Well known examples of drugs able to do this are radiocontrast media, opiates, azo-dyes and benzoates"3. It is possible, in view of our patient's lymphoma and abnormal complement levels, that he had the acquired form of angioneurotic oedema. There may also be another explanation in that amongst the 20 constituents of Benylin, there is an azo-dye (Ponceau 4RE124) and a benzoate. Either of these 2 drugs could have precipitated the life-threatening oedema.

Behfet's colitis with oesophagael ulceration treated with sulphasalazine and cyclosporin

at 34 g/l (normal 35-45), but other tests of hepatic and renal function were normal. The erythrocyte sedimentation rate was raised to 65 mm/h and immune complexes containing Clq, IgG and IgM were present in the serum. At endoscopy there were many large, discrete ulcers in the transverse and descending colon and linear ulcers in the oesophagus. Histology of these lesions showed non-specific inflammation. A Schilling test was normal, showing that the terminal ileum was not diseased, but xylose absorbtion was reduced (3 h urinary xylose excretion following a 5 g oral dose was 0.53 g (normal >2.5 g) suggesting that the small intestine was damaged). A diagnosis of Behcet's disease with oesophageal and colonic involvement was made. Her condition improved slightly with prednisolone 60 mg per day, but her diarrhoea persisted, despite concurrent

G R Foster MRCP St Charles Hospital, Exmoor Street, London W10 6DZ Keywords: Behqet's disease; oesophagus; colitis; sulphasalazine; cyclosporin

Behcet's disease is a multisystem disorder which causes gastroenterological symptoms in about half the cases'. In most patients these symptoms are minor, transient disturbances of bowel function. A minority of patients develop colitis2 or oesophagitis3, the latter being rare. The treatment of the gastroenterological complications of Behcet's disease is controversial. We have recently seen an unusual case of Behcet's disease with colonic and oesophageal involvement which was treated successfully with sulphasalazine and

cyclosporin. Case report A 61-year-old women presented in 1985 with conjunctivitis, arthritis and myalgia. Her symptoms resolved with non-steroidal anti-inflammatory drugs. Four months later she developed buccal ulceration which healed without therapy. In June 1986 she presented with oro-genital ulceration, folliculitis and bloody diarrhoea. Investigations showed a microcytic anaemia, haemoglobin 9.4 g/l (mean corpuscular volume 77 fl) with a low serum iron of 11.3 iemolfl (normal 13-32) and an iron binding capacity of 48 Amol/l (normal 45-78). Her albumin was reduced

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References 1 Soter NA, Wasserman SI. Angioedema associated with complement abnormalities. In: Fitzpatrick T, Eisen A Z, Wolff K, Freedberg I, Austen K, eds. Dermatology in general medicine. London: McGraw-Hill, 1979:542-8 2 Caldwell JR, Ruddy S, Schur PH and Auste KF. Acquired Cl inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol 1972;1:39-52 3 Simon RA. Adverse reactions to drug additives. JAllergy Clin Immunol 1984;74:623-9

(Accepted 15 July 1987. Correspondence to Dr Bates, ENT Department, Bristol Royal Infirmary)

Case presented to Clinical Section, 10 April 1987

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Figure 1. Response to therapy in a case of Behget's colitis. *', Erythrocyte sedimentation rate mm/h; +, serum albumin gi/l; o, presence or absence of diarrhoea (defined as >4 bowel motions per day)

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