Cases reported "Esophageal Stenosis"

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11/23. Perioperative considerations in esophageal replacement for epidermolysis bullosa: report of two cases successfully treated by colon interposition.

    Esophageal stricture commonly occurs in patients with epidermolysis bullosa dystrophica recessive (EBDR), but esophageal replacement is considered a high risk procedure because of limited exposure of the airway, malnutrition, and postoperative skin bullae to secondary infection. Recent innovations in care, including preoperative parenteral nutrition, topical care for bullae and skin ulceration, fiberoptic tracheal intubation, electrocardiogram monitoring with metallic pacer leads, and an overall concern to protect the fragile intact skin, have improved the results of esophageal replacement. Utilizing these adjunctive measures, ileocolonic substernal interposition has been successfully performed in a 26-yr-old male and a 19-yr-old female at our institution. Despite cervical anastomotic stricture requiring resection in one, and an obstructive cervical esophageal bullous developing acutely 5 yr after operation in the second, both patients now gum or chew a solid diet. The first patient also benefited from total esophagectomy for squamous dysplasia detected at the time of esophageal replacement. Multidisciplinary management by the surgeon, gastroenterologist, anesthesiologist, and dermatologist makes esophageal replacement available for younger patients with epidermolysis bullosa dystrophica recessive and esophageal strictures.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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12/23. Dermolytic (dystrophic) epidermolysis bullosa inversa.

    We treated four patients with an inverse form of recessive dermolytic (dystrophic) epidermolysis bullosa. The distinguishing features of the disease are (1) early generalized skin involvement with blisters and erosions that heal with superficial, atrophic scars; (2) persistence into adulthood, although milder; (3) severity in flexural areas, especially the inguinal folds, perineum, axillae, submammary area, posterior and lateral aspects of the neck, and often the lower parts of the abdomen and back; (4) normal stature and general development; (5) severe oral and esophageal mucosal involvement; (6) normal teeth; (7) normal or minimally involved fingernails, but mild to moderately dystrophic or atrophic toenail changes; and (8) microscopic findings similar to those of the Hallopeau-Siemens form of epidermolysis bullosa.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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13/23. The spectrum of epidermolysis bullosa acquisita.

    A patient has an acquired, scarring, bullous eruption. The severity of the cutaneous, ocular, esophageal, and laryngeal scarring was suggestive of cicatricial pemphigoid or the severely dystrophic forms of epidermolysis bullosa. Clinical features and histologic and immunofluorescence and electron microscopic study led to the diagnosis of epidermolysis bullosa acquisita. The spectrum of epidermolysis bullosa acquisita includes cases that closely approximate the severity of disease previously recognized only for the recessive form of epidermolysis bullosa dystrophica.
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ranking = 1.3333333333333
keywords = epidermolysis bullosa, epidermolysis, bullosa
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14/23. Oesophageal reconstruction for complete stenosis due to dystrophic epidermolysis bullosa.

    Oesophageal involvement in epidermolysis bullosa is discussed. Two patients, a brother and sister, with stricture of the oesophagus due to the disease are described. The strictures were treated by resection and end-to-end oesophageal anastomosis and the patients have been relieved of dysphagia for the subsequent three years.
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ranking = 0.38244510058681
keywords = epidermolysis bullosa, epidermolysis, bullosa
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15/23. Management of esophageal stenosis in recessive dystrophic epidermolysis bullosa.

    Total replacement of the esophagus by colonic interposition has been recommended as the treatment of esophageal obstruction in recessive dystrophic epidermolysis bullosa. We report our experience in the conservative management of esophageal blisters, strictures, and complete occlusion in 5 patients (aged 2-61 yr). Our therapy consists of a combination of the following principles: (a) inhibition of collagenase formation by oral phenytoin to reduce epithelial detachment; (b) pureed or semiliquid food because minor trauma by hard food particles may induce blistering and result in scarring of the upper esophagus, and larger food particles may obstruct an esophageal stricture; (c) avoidance of tangential shearing forces induced by bougienage and endoscopy and instead use of inflatable dilatator balloons which produce vertical pressure that seems to be less harmful; and (d) long-term nasogastric tube feeding, which may relieve even tight strictures. Our observations suggest that successful long-term conservative management of esophageal stenoses in dystrophic epidermolysis bullosa is possible.
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ranking = 0.88727794181337
keywords = epidermolysis bullosa, epidermolysis, bullosa
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16/23. Subarachnoid and epidural anaesthesia for patients with epidermolysis bullosa.

    Successful anaesthetic management of two patients with severe epidermolysis bullosa was accomplished using subarachnoid and epidural blockade. In order to avoid complications of regional anaesthesia, antiseptic preparation of the skin should not involve scrubbing nor should adhesive tape be used to secure catheters. In addition local infiltration of the skin is best avoided. The potential complications of skin and oral mucosal damage associated with mask or endotracheal inhalation anaesthesia was avoided.
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ranking = 0.83333333333333
keywords = epidermolysis bullosa, epidermolysis, bullosa
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17/23. Epidermolysis bullosa and its E.N.T. manifestations. Two case reports.

    The present paper describes two rare cases of epidermolysis bullosa dystrophica (recessive), one with oesophageal stricture and another with laryngeal stenosis, along with other usual features of the disease. Different views on the line of treatment of the oesophageal stricture as a complication of this dreadful disease are discussed. Replacement of the strictured oesophagus by coloplasty may be contemplated in the first case, if the patient develops increasing dysphagia. In the second case, with cicatrical stenosis of the larynx and hoarseness of voice which is a rare complication of the disease, not reported in the literature so far, a tracheostomy is contemplated when the child develops acute respiratory distress. These two cases are under observation.
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ranking = 0.20425069583011
keywords = epidermolysis bullosa, epidermolysis, bullosa
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18/23. Esophageal involvement in epidermolysis bullosa dystrophica.

    The clinical and radiographic findings in four cases of epidermolysis bullosa dystrophica of the esophagus are presented. The patients were 5-67 years old. All had typical skin lesions and dysphagia. The most impressive radiographic finding was bulla formation in virtually any part of the esophagus. The bullae would resolve or ulcerate. Scar formation with resultant stricture of the esophagus was a common sequela of this disease.
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ranking = 0.83333333333333
keywords = epidermolysis bullosa, epidermolysis, bullosa
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19/23. Esophageal involvement in epidermolysis bullosa dystrophica: clinical and roentgenographic manifestations.

    Epidermolysis bullosa is a rare hereditary mechanobullous skin disorder. Four patients with esophageal involvement are reported. These cases and review of reported cases of epidermolysis bullosa dystrophica-recessive (EBD-R) showed distinct clinical and roentgenographie features of esophageal involvement: diffuse inflammatory changes, motility disorders, small blisters or bullae seen as constant nodular-filling defects, esophageal ulcers, scars, pseudodiverticula, transverse and circumferential webs, various size strictures, shortening of the esophagus with development of traction hiatal hernia and gastroesophageal reflux, perforation, and complete obstruction of the esophageal lumen. Dysphagia is reversible when caused by bullae or webs and permanent when due to cicatrizing strictures. The strictures may remain unchanged in size for many years despite variations in dysphagic symptoms.
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ranking = 0.84272934062419
keywords = epidermolysis bullosa, epidermolysis, bullosa
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20/23. Anaesthesia for correction of oesophageal stricture in a patient with recessive epidermolysis bullosa dystrophica: case report.

    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.
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ranking = 0.84272934062419
keywords = epidermolysis bullosa, epidermolysis, bullosa
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