Cases reported "Laryngostenosis"

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1/4. Epidermolysis bullosa associated with laryngeal stenosis.

    A boy with epidermolysis bullosa developed progressive laryngeal obstruction after age six months, with tracheotomy necessary at age three years. By eight years, the larynx was completely occluded by web and scar. Stenosis recurred repeatedly over the next seven years, three to six months after each corrective procedure, but laryngeal patency has been better-maintained since age 15. Laryngeal stenosis, presumably secondary to epithelial detachment, although a rare complication of epidermolysis bullosa, can be serious and life-threatening.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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2/4. Acute laryngeal obstruction in junctional epidermolysis bullosa.

    A boy with junctional epidermolysis bullosa died from acute laryngeal obstruction at the age of 29 months, having been hoarse since early infancy. Post mortem studies showed gross narrowing of the laryngeal airway by cystic dilatations of the ducts of the seromucinous glands, and replacement of the laryngeal epithelium by oedematous granulation tissue. Laryngeal involvement appears to occur not infrequently in junctional epidermolysis bullosa, and contributes to its considerable mortality.
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ranking = 2.69121779451
keywords = epidermolysis bullosa, epidermolysis, bullosa
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3/4. Epidermolysis bullosa letalis in the larynx causing acute respiratory failure: a case presentation and review of the literature.

    The case history is presented of a 29-month-old boy with the characteristic skin lesions of epidermolysis bullosa letalis, requiring steroid therapy. After an episode of gastrointestinal haemorrhage, probably caused by the steroids, the child experienced progressive stridor and respiratory failure and died 10 days later. The postmortem findings in the larynx were of marked mucosal inflammation and cystic swelling of the serumucinous glands producing severe narrowing of the supraglottic airway. The laryngeal cysts were thus entirely different in nature to the cutaneous bullae.
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ranking = 0.551463700915
keywords = epidermolysis bullosa, epidermolysis, bullosa
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4/4. 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 = 0.551463700915
keywords = epidermolysis bullosa, epidermolysis, bullosa
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