Cases reported "Esophageal Stenosis"

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1/23. Balloon dilation of an esophageal stenosis in a patient with recessive dystrophic epidermolysis bullosa.

    We report a 13-year-old boy with recessive dystrophic epidermolysis bullosa who had dysphagia due to esophageal stenosis. A balloon dilation was successfully performed under flexible endoscopic and fluoroscopic control. The early and long-term follow-up was characterized by the disappearance of dysphagia, weight gain, and improvement of his skin lesions.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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2/23. colon interposition for esophageal stenosis in a patient with epidermolysis bullosa.

    Epidermolysis bullosa (EB) is a disease with 3 forms, most hereditary, characterized by spontaneous blistering lesions. The autosomally inherited form, epidermolysis bullosa dystrophica recessive (EBDR), is responsible for esophageal lesions consisting of web or stenosis. The authors could find only 9 cases treated by various esophageal replacement procedures in the literature, and the experience with 1 case treated by colon interposition is presented. J Pediatr Surg 36:1861-1863.
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ranking = 1.011275208749
keywords = epidermolysis bullosa, epidermolysis, bullosa
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3/23. Anesthetic implications in epidermolysis bullosa dystrophica.

    Epidermolysis bullosa is a genetic mechanobullous disease of the stratified squamous keratinizing epithelium that affects the skin and mucous membranes. Its primary feature is the formation of blisters after minor shearing trauma to the skin or mucous membranes that can result in debilitating, even life-threatening scarring. The disease presents special problems for the anesthesia provider because the equipment used to deliver anesthesia and monitor vital signs may cause serious postoperative complications. The challenge is to maintain patency of the airway and use monitoring technology without damaging epithelial surfaces, which could result in permanent scarring. Successful anesthetic management of a patient with epidermolysis bullosa is possible if precautions with anesthetic instrumentation are observed.
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ranking = 1.011275208749
keywords = epidermolysis bullosa, epidermolysis, bullosa
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4/23. esophageal stenosis in childhood: dystrophic epidermolysis bullosa without skin blistering due to collagen VII mutations.

    We report a 9-year-old girl who experienced recurrent dysphagia since infancy. Crohn's disease was suspected because she had aphthous ulcers of the mouth and anal dermatitis with hematochezia. After bougienages of esophageal stenoses and medication for inflammatory bowel disease proved unsuccessful, interdisciplinary re-examination revealed the cause of the symptoms to be an extracutaneous form of dystrophic epidermolysis bullosa, a genetic skin fragility disorder. Dystrophic epidermolysis bullosa is caused by mutations in the COL7A1 gene encoding collagen VII, a protein of the epidermal attachment complex, and typically manifests with trauma-induced skin blistering, scarring, nail dystrophy, and, in some cases, mucosal involvement. The present proband never developed skin blisters but had nail dystrophy and erosions of the oral, esophageal, and genitoanal mucosa, which healed with slight scarring. mutation analysis disclosed compound heterozygosity for recessive mutations in the COL7A1 gene. The paternal mutation 425 A-->G caused abnormal splicing resulting in a premature stop codon. The maternal mutation G2775S led to the substitution of a glycine by a serine in the triple helical domain of collagen VII. This case shows that mucosal disease and esophageal strictures in childhood are not always acquired, but can also represent a genetic defect of dermal-epidermal adhesion, even in the absence of skin blistering.
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ranking = 1.2
keywords = epidermolysis bullosa, epidermolysis, bullosa
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5/23. 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 = 0.81127520874903
keywords = epidermolysis bullosa, epidermolysis, bullosa
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6/23. A case of recessive dystrophic epidermolysis bullosa with esophageal stenosis treated with endoscopic bougienage.

    Recessive dystrophic epidermolysis bullosa (RDEB) is a rare, hereditary, chronic, non-inflammatory mechanobullous disease. Esophageal involvement is invariable in RDEB patients. A 15-year-old girl visited with protracted vesiculobullous skin eruptions since birth. On physical examination, bullae, atrophic scars, milia, crusts and erosions were noted primarily on the distal extremities. Loss of entire nails and syndactyly of the right fourth and fifth toes were seen. diagnosis of RDEB was confirmed by clinical, histopathologic, immunofluorescent and electron microscopic findings. Endoscopic and esophagographic studies revealed a stenosis and several hemorrhagic bullae and erosions. She successfully underwent bougienage under endoscopic and fluoroscopic control
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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7/23. epidermolysis bullosa acquisita with oesophageal stenosis.

    epidermolysis bullosa acquisita (EBA) is a rare acquired subepidermal blistering disorder associated with autoimmunity to type VII collagen. Although the full clinical spectrum of EBA is still being defined, it is now known that EBA has greater clinical heterogeneity than previously suggested. We describe a patient with EBA which closely approximated the severity of the recessive form of dystrophic epidermolysis bullosa.
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ranking = 0.25637604374516
keywords = epidermolysis bullosa, epidermolysis, bullosa
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8/23. Pharyngogastric colonic interposition for total oesophageal occlusion in epidermolysis bullosa.

    Epidermolysis bullosa comprises a group of rare heritable disorders, characterized by blistering of skin and other epithelial lined structures following minor trauma. In the oesophagus, trauma from food boluses leads to bullae, ulceration and scarring, with formation of strictures. Oesophageal strictures are usually managed with balloon dilatation. We describe a case of a 19-year-old woman whose oesophageal stricture did not respond to balloon dilatation. She underwent a substernal colon interposition between the pharynx and stomach. The surgery included cervical, thoracic and abdominal approaches, with involvement of three specialist surgeons. Three months after surgery, the patient reported to be swallowing with little difficulty. Because of the high risk of morbidity and mortality associated with this surgery, we recommended that the patient should be managed in a specialist centre, with availability of intensive care facilities and the possibility of providing a multidisciplinary approach during surgery.
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ranking = 0.81127520874903
keywords = epidermolysis bullosa, epidermolysis, bullosa
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9/23. 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 = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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10/23. 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 = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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