Anaesthesia for correction of oesophageal stricture in a patient with recessive epidermolysis bullosa dystrophica: case report.
نویسندگان
چکیده
Epidermolysis bullosa dystrophica is a rare hereditary disorder which presents significant anaesthetic problems. These include malnutrition and anaemia; electrolyte imbalance in severe cases; renal failure and amyloidosis in progressive disease; association with porphyria; a history of steroid therapy. Technical problems associated with the necessity to avoid trauma to the skin and mucous membranes include those related to management of the airway and avoidance of regional techniques. Venepuncture may be difficult and oesophageal stricture increases the risk of regurgitation and aspiration. Anaesthetic management of a patient with epidermolysis bullosa dystrophica with oesophageal stricture presenting for colonic interposition is described and the problems associated with this disease are discussed.
منابع مشابه
Epidermolysis bullosa of the oesophagus with oesophageal wed formation.
Marsden, R. A., Sambrook Gowar, F. J., MacDonald, A. F., and Main, R. A. (1974). Thorax, 29, 287-295. Epidermolysis buBosa of the oesophagus with oesophageal web formation. Four members of a family are described with epidermolysis bullosa dystrophica of probable autosomal recessive inheritance. They have shown the typical blistering of the skin and oral mucosa, usually present at birth, which o...
متن کاملManagement of oesophageal stenosis in epidermolysis bullosa dystrophica.
Seven patients with epidermolysis bullosa dystrophica and chronic and recurrent oesophageal lesions such as spasm, strictures, and complete occlusion were studied. Dysphagia could be cured with drugs if it was caused by bullae formation or spasm. If oesophageal strictures were present, endoscopy and bouginage with corticosteroid prophylaxis during the quiescent phase of the disease was a safe a...
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Epidermolysis Bullosa associated with pyloric stenosis: A case report
Epidermolysis bullosa are a group of hereditary skin diseases manifested as blisters on the sites of trauma to the skin. According to the level of blister development, epidermolysis bullosa are divided into the following groups: epidermolysis bullosa simplex, junctional epidermolysis bullosa and dystrophic epidermolysis bullosa. The latter two groups are associated with poorer prognosis and hig...
متن کاملTwelve hour anaesthesia in a patient with epidermolysis bullosa.
Epidermolysis bullosa (EB), an inherited disorder presents clinically with recurrent cutaneous blister formation with possible involvement of mucous membranes and other organs. The sequelae of this disease pose multiple challenges to the anaesthetist and operating room team. Recent literature describes several anaesthetic techniques for the short surgical procedures this patient population may ...
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ورودعنوان ژورنال:
- Canadian Anaesthetists' Society journal
دوره 27 2 شماره
صفحات -
تاریخ انتشار 1980