Overview of complications associated with epidermolysis bullosa: A multicenter retrospective clinical analysis of 152 cases.

BACKGROUND/PURPOSE: Epidermolysis bullosa (EB) is a rare disease of skin and mucosa which may causes surgical complications. We review these in a large patient cohort from Saudi Arabia. METHODS: A retrospective study was conducted at 21 centers between 2003 and 2020. Demographic data and information on EB type [Simplex (EBA), Dystrophic (DEB) and Junctional (JEB)]. The dataset included clinical features, operations, surgical complications, and treatment. RESULTS: There were 152 (63 male) children with EB [EBS n = 93 (61.2%); DEB n = 30 (19.7%); JEB n = 25 (16.4%), and Kindler syndrome n = 4, (2.6%)]. Children with JEB and DEB tended to have a higher frequency of skin and musculoskeletal system complications (skin cancer, pseudosyndactyly and recurrent skin infection). Esophageal strictures were mostly seen in DEB (n = 19, 63%) and to a lesser extent in EBS (n = 20, 21%) and JEB (n = 4, 16%). Pyloric atresia was uncommon (n = 4) and limited to those with JEB. Percutaneous gastrostomy for feeding support was used in all types. Ankyloglossia was common but often recurred (76%) after division. Circumcision was usually safe and complication-free in male children except in those with severe JEB. Phimosis was reported in 10% of uncircumcised patients. CONCLUSIONS: Our series showed that surgeons play a key role in the management of some complications associated with EB. It is also important to be aware of the particular sub-type as this can predict the natural history and likely response to treatment. LEVEL OF EVIDENCE: 2.

The Outcome of Pediatric Esophageal Strictures Managed with Endoscopic Balloon Dilation in Saudi Arabia.

Background: Esophageal strictures can affect nutrition of infants and children impairing their weight gain. To our knowledge, this is the first article evaluating and comparing between the two methods of dilation in terms of outcome and one of few, if any, to assess both weight and height to evaluate the nutritional outcomes following dilation. To determine the safety, efficacy, and long-term effects of endoscopic dilation in managing pediatric esophageal strictures by assessing the clinical and nutritional outcomes. Methods: A retrospective study of 137 patients with esophageal strictures who underwent either endoscopic balloon dilatation or Savary dilatation, or both. Outcome parameters measured include the number of dilatations, nutritional status, and if symptoms had been relieved. Results: The most frequent cause of esophageal strictures was post-tracheoesophageal fistula repair (n = 51, 37.2%), and the majority were lower third strictures (n = 47, 34.3%). However, 8 cases (5.8%) had failed the dilation procedure. Savary dilatation had the highest number of complications. Overall, success rate was 79.6%. Higher success rate was for cases dilated by endoscopic balloon dilation (EBD) (n = 47, 90.4%). There was a statistically significant correlation between the success rate and the method of dilatation (P = .042). Statistically significant increment of weight was recorded for lower strictures (P = .001). Conclusion: EBD was associated with the highest success rate. Endoscopic dilatations are safe and efficient in managing pediatric esophageal strictures with improvement in both clinical and nutritional outcomes.