title: A two-step multidisciplinary approach to treat recurrent esophageal strictures in children with epidermolysis bullosa dystrophica.
abstract: In children with severe generalized recessive dystrophic epidermolysis bullosa (RDEB), esophageal scarring leads to esophageal strictures with dysphagia, followed by malnutrition and delayed development. We describe a two-step multidisciplinary therapeutic approach to overcome malnutrition and growth retardation. In Step 1, under general anesthesia, orthograde balloon dilation of the esophagus is followed by gastrostomy creation using a direct puncture technique. In Step 2, further esophageal strictures are treated by retrograde dilation via the established gastrostomy; this step requires only a short sedation period. A total of 12 patients (median age 7.8 years, range 6 weeks to 17 years) underwent successful orthograde balloon dilation of esophageal strictures combined with direct puncture gastrostomy. After 12 and 24 months in 11 children, a substantial improvement of growth and nutrition was achieved (body mass index [BMI] standard deviation score [SDS] + 0.59 and + 0.61, respectively). In one child, gastrostomy was removed because of skin ulcerations after 10 days. Recurrent esophageal strictures were treated successfully in five children. The combined approach of balloon dilation and gastrostomy is technically safe in children with RDEB, and helps to promote catch-up growth and body weight. In addition, recurrent esophageal strictures can be treated successfully without general anesthesia in a retrograde manner via the established gastrostomy.
authors.affiliation: Department of General and Visceral Surgery, University Hospital Muenster, Muenster, Germany.
authors.forename: Thorsten
authors.initials: T
authors.lastname: Vowinkel
authors.affiliation: Department of General and Visceral Surgery, University Hospital Muenster, Muenster, Germany.
authors.forename: Mike
authors.initials: M
authors.lastname: Laukoetter
authors.affiliation: Department of General and Visceral Surgery, University Hospital Muenster, Muenster, Germany.
authors.forename: Rudolf
authors.initials: R
authors.lastname: Mennigen
authors.affiliation: Department of Anaesthesiology, Intensive Care, Emergency Medicine and Pain Medicine, University Medicine Greifswald, Greifswald, Germany.
authors.forename: Klaus
authors.initials: K
authors.lastname: Hahnenkamp
authors.affiliation: Department of Anaesthesiology and Intensive Care, University Hospital Muenster, Muenster, Germany.
authors.forename: Antje
authors.initials: A
authors.lastname: Gottschalk
authors.affiliation: Department of Anaesthesiology and Intensive Care, University Hospital Muenster, Muenster, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Boschin
authors.affiliation: Department of General Paediatrics, University Hospital Muenster, Muenster, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Frosch
authors.affiliation: Department of General and Visceral Surgery, University Hospital Muenster, Muenster, Germany.
authors.forename: Norbert
authors.initials: N
authors.lastname: Senninger
authors.affiliation: Department of General and Visceral Surgery, University Hospital Muenster, Muenster, Germany.
authors.forename: Dirk
authors.initials: D
authors.lastname: Tübergen
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D004106:Dilatation / Q000379:methods*; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications*; D004940:Esophageal Stenosis / Q000209:etiology / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D005774:Gastrostomy; D006801:Humans; D007223:Infant; D008297:Male; D012008:Recurrence; D016896:Treatment Outcome