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Non-endoscopic percutaneous gastrostomy placement in children with recessive dystrophic epidermolysis bullosa

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Abstract

Recessive dystrophic epidermolysis bullosa (RDEB) is associated with high nutritional demands, esophageal strictures and dysphagia. About one quarter of the patients require gastrostomy tube placement to maintain adequate nutritional status. To minimize trauma to the skin and pharyngoesophageal mucosa caused by commonly used gastrostomy tube insertion techniques, we used a non-endoscopic, percutaneous, image-guided approach. This approach combines the use of ultrasound for mapping of the liver and spleen, water-soluble contrast enema to visualize the colon, and gastric insufflation to define the stomach. The gastrostomy tube is replaced by a low-profile button gastrostomy tube 10–12 weeks postoperatively. The five female patients reported in this series ranged in age from 6 to 9 years. They all tolerated the procedure well and no perioperative complications were encountered. All were able to tolerate feedings on postoperative day 1 and all underwent successful replacement of gastrostomy tubes by low-profile button tubes. Our experience suggests that a non-endoscopic, image-guided approach to gastrostomy tube placement offers a safe, effective, and minimally traumatic alternative to more commonly used approaches. It minimizes the risk of procedure-related morbidity and leads to overall improvement in the quality of life. As such, we strongly recommend that it be included in the treatment armamentarium for patients with epidermolysis bullosa and nutritional failure.

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Authors and Affiliations

  1. Department of Surgery, Division of General and Thoracic Pediatric Surgery, Cincinnati Children’s Hospital Epidermolysis Bullosa Center, 3333 Burnet Avenue, MLC 3018, Cincinnati, OH, 45229-3039, USA

    Wolfgang Stehr & Richard G. Azizkhan

  2. Department of Pediatrics, Division of Gastroenterology, Cincinnati Children’s Hospital Epidermolysis Bullosa Center, Cincinnati, OH, USA

    Michael K. Farrell

  3. Department of Pediatrics, Division of Pediatric Dermatology, Cincinnati Children’s Hospital Epidermolysis Bullosa Center, Cincinnati, OH, USA

    Anne W. Lucky

  4. Department of Radiology, Cincinnati Children’s Hospital Epidermolysis Bullosa Center, Cincinnati, OH, USA

    Neil D. Johnson & John M. Racadio

Authors
  1. Wolfgang Stehr
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  2. Michael K. Farrell
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  3. Anne W. Lucky
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  4. Neil D. Johnson
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  5. John M. Racadio
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  6. Richard G. Azizkhan
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Corresponding author

Correspondence to Richard G. Azizkhan.

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Stehr, W., Farrell, M.K., Lucky, A.W. et al. Non-endoscopic percutaneous gastrostomy placement in children with recessive dystrophic epidermolysis bullosa. Pediatr Surg Int 24, 349–354 (2008). https://doi.org/10.1007/s00383-007-2100-x

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  • DOI: https://doi.org/10.1007/s00383-007-2100-x

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