Gastrostomy for infants with severe epidermolysis bullosa simplex in neonatal intensive care.

Orphanet Journal of Rare Diseases
M MarroC Chiaverini

Abstract

Severe epidermolysis bullosa simplex (EBS sev) is a rare genodermatosis characterized by congenital generalized blistering and mucosal involvement. Increased needs and decreased intake quickly lead to nutritional imbalance. Enteral nutrition support is proposed, but classical nasogastric tubes are not well tolerated in these patients and gastrostomy is preferred. To report the experience with EBS sev in neonatal units of French reference centers for gastrostomy. In this retrospective multicentric study, we included all patients with EBS sev who had gastrostomy placement before age 9 months during neonatal care hospitalization. Nine infants (5 males/4 females) with severe skin and mucosal involvement were included. A gastrostomy was decided, at an early age (mean 3.7 months, range 1.4 to 8 months) in infants with mean weight 4426 g (range 3500 to 6000 g). Techniques used were endoscopy with the pull technique for 5 infants and surgery under general anesthesia for 4. Main complications were local but resolved after treatment. All infants gained weight after gastrostomy. The mean withdrawal time (n = 7) for the gastrostomy was 35.8 months (range 10.5 months to 6.5 years). Seven children had persistent oral disorders. Gastrostomy i...Continue Reading

References

Feb 1, 2012·Pediatric Dermatology·Lesley HaynesAnna E Martinez
May 6, 2014·Journal of Pediatric Gastroenterology and Nutrition·Kamlesh PatelDakshesh Parikh
Dec 1, 2015·Gastrointestinal Endoscopy Clinics of North America·Maireade E McSweeney, C Jason Smithers
Jul 14, 2018·Current Opinion in Pediatrics·Andrea Volpe, Georgina Malakounides

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