Cases reported "Deglutition Disorders"

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1/10. Dysphagia in a patient with lupus and review of the literature.

    Dysphagia is not infrequent in patients with connective tissue diseases such as scleroderma, polymyositis or systemic lupus erythematosus (SLE). It is usually the result of gastro-oesophageal reflux but dysmotility can equally be responsible. A case of dysphagia is described in a patient with SLE, who had developed a rare variety of bullous mucous disease affecting the whole length of oesophagus with spontaneous extrusion of an oesophageal cast. Histological features were suggestive of a variant of rare form of bullous disease in SLE called epidermolysis bullosa acquisita (EBA). This rare association of SLE and EBA involving the oesophagus has not been described in the literature.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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2/10. Fluoroscopically guided endoluminal balloon dilatation of esophageal stricture due to epidermolysis bullosa dystrophica.

    epidermolysis bullosa dystrophica is a hereditary blistering disorder in which the mucosal surface of the esophagus is frequently involved in addition to skin. blister formation after minor trauma leads to erosions, ulcerations, scarring, and stricture formation in the esophagus and causes dysphagia. There is no definitive medical management for esophageal lesions. Colonic interposition has considerable mortality and morbidity, while surgical or endoscopic bougienage is not recommended because it causes further trauma to the esophagus, which accelerates stricture formation, and has a high risk of perforation. Herein we report a case of esophageal stricture successfully treated with repeated balloon dilatations.
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ranking = 4.0554989590457
keywords = epidermolysis bullosa, epidermolysis, bullosa
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3/10. Effective esophageal balloon dilation for esophageal stenosis in recessive dystrophic epidermolysis bullosa.

    Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited disorder of squamous epithelium that results in dystrophic scarring of the skin after minor trauma. RDEB is classified into two subtypes: Hallopeau-Siemens (HS) and non-Hallopeau-Siemens (nHS). Although severe scarring of the skin is the most common and obvious complication of the disease, esophageal scarring with formation of long strictures may also develop. Treatment options for esophageal stenosis in patients with RDEB include steroids, hyperalimentation, esophageal dilation and replacement. This report describes a child who was dilated immediately after diagnosis of severe esophageal stenosis subsequent to nHS-RDEB and managed successfully. Endoscopic esophageal balloon dilation under fluoroscopic control was very useful for detecting the region of stenosis and bougienage. The literature on such injuries is reviewed here, and the problems associated with the treatment of children with esophageal stenosis associated with RDEB are discussed.
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ranking = 5
keywords = epidermolysis bullosa, epidermolysis, bullosa
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4/10. epidermolysis bullosa acquisita (EBA) and esophageal webs: a new association.

    epidermolysis bullosa acquisita (EBA) is a well-defined, blistering disorder of the skin associated with autoantibodies to type VII collagen. Although esophageal pathology is common in children with hereditary dystrophic forms of epidermolysis bullosa, esophageal problems have not been reported previously in patients with bona fide EBA. In this report, a 71-yr-old white female with longstanding EBA presented with recurrent dysphagia and multiple esophageal webs that responded to esophageal dilatation.
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ranking = 1.2774947952283
keywords = epidermolysis bullosa, epidermolysis, bullosa
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5/10. Management 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 and useful procedure. We have also given corticosteroids, which reduced the oedema caused by bullae formation and oral phenytoin, which reduced epithelial detachment by inhibiting collagenase activity. verapamil counteracted oesophageal spasm and pureed food during periods of dysphagia reduced blistering of the upper oesophagus.
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ranking = 5
keywords = epidermolysis bullosa, epidermolysis, bullosa
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6/10. 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 = 6
keywords = epidermolysis bullosa, epidermolysis, bullosa
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7/10. Gastrointestinal manifestations of epidermolysis bullosa in children.

    The medical and surgical management of the chronic and recurrent esophageal and anal lesions of recessive dystrophic epidermolysis bullosa pose challenging problems for the physician. Various therapeutic approaches are discussed, and the case histories of four problem patients are reviewed.
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ranking = 5
keywords = epidermolysis bullosa, epidermolysis, bullosa
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8/10. 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 = 2.336809404003
keywords = epidermolysis bullosa, epidermolysis, bullosa
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9/10. 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 = 5.0554989590457
keywords = epidermolysis bullosa, epidermolysis, bullosa
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10/10. Esophageal web: a previously unrecognized complication of epidermolysis bullosa.

    The course of the esophageal disease in four patients with epidermolysis bullosa dystrophica recessive is examined. Three of four patients experienced web formation, a previously unrecognized finding, and the significance of this lesion in the evaluation and management of these patients is emphasized. One of four patients underwent a successful colonic interposition for severe esophageal disease and the role of this potentially life-saving procedure is discussed.
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ranking = 5
keywords = epidermolysis bullosa, epidermolysis, bullosa
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