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Fluoroscopically guided dilation of esophageal strictures in patients with dystrophic epidermolysis bullosa: long-term results.

[dystrophic epidermolysis bullosa]

The purpose of this study was to investigate the immediate and long-term outcomes after fluoroscopically guided balloon dilation of esophageal strictures in a series of patients with dystrophic epidermolysis bullosa (DEB).Between 2005 and 2011, the medical records of all patients with DEB treated with fluoroscopically guided balloon dilation of esophageal strictures were included in the study and retrospectively analyzed. The indication for treatment was dysphagia attributed to at least one radiologically verified esophageal stricture. The primary endpoints of the study included procedural technical success, clinical improvement assessed with a 0-4 dysphagia score, and major complication rate. Secondary endpoints were patient survival and reintervention rates.Nineteen consecutively registered patients with DEB (age range, 10-51 years; mean, 30 ± 12.2 years) and dysphagia due to esophageal strictures were treated with fluoroscopically guided balloon dilation. In total, 90 procedures and 121 dilations were performed to manage 28 lesions. Balloon diameter ranged from 8 to 18 mm. The mean follow-up time was 47.51 ± 16.64 months (range, 17-73 months). The technical success rate was 96.7% (87/90). There were no major complications. The mean reintervention rate was 1.19 dilations per patient per year, and the postprocedural dysphagia score (0.72 [95% CI, 0.56-0.87]) was significantly lower than baseline (2.50 [95% CI 2.35-2.65]) (p < 0.001).Repeated fluoroscopically guided balloon dilation is safe and effective for the management of dysphagia caused by esophageal strictures in DEB. Use of this technique was associated with marked clinical improvement in dysphagia and satisfactory long-term reintervention rates with no major complications.

Diseases presenting "long-term outcomes" symptom

  • acute rheumatic fever
  • alpha-thalassemia
  • aromatase deficiency
  • cholangiocarcinoma
  • classical phenylketonuria
  • congenital adrenal hyperplasia
  • congenital diaphragmatic hernia
  • cushing syndrome
  • dystrophic epidermolysis bullosa
  • esophageal squamous cell carcinoma
  • hirschsprung disease
  • homocystinuria without methylmalonic aciduria
  • kabuki syndrome
  • lamellar ichthyosis
  • omenn syndrome
  • phenylketonuria
  • proteus syndrome
  • trochlear dysplasia
  • von hippel-lindau disease

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