A two-step multidisciplinary approach to treat recurrent esophageal strictures in children with epidermolysis bullosa dystrophica.

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.

Anesthesia for balloon dilatation of esophageal strictures in children with epidermolysis bullosa dystrophica: from intubation to sedation.

PURPOSE OF REVIEW: Dystrophic epidermolysis bullosa is a rare disease mainly affecting small children. They often have to undergo different surgical procedures, for example balloon dilatation following esophageal strictures to maintain oral intake and prevent malnutrition. RECENT FINDINGS: So far, no specific treatment is available for patients with any forms of epidermolysis bullosa. However, more and more minimal invasive surgical techniques are used to manage symptoms of the disease, for example endoscopic balloon dilatation for severe esophageal strictures. SUMMARY: In this article implications for the anesthetic management of children with dystrophic epidermolysis bullosa are described. General anesthesia and sedation techniques are feasible if specific prerequisites are fulfilled. The team providing anesthesia and endoscopy must be familiar with the nature and disabilities of this disease. Coordinated care of the involved disciplines is crucial for all planned procedures regarding perioperative management.

Ultrasonic technology: a new treatment option for anal condylomata acuminata.

PURPOSE: Treatment options for anal and perianal warts caused by human papillomavirus include topical application of cytotoxic substances or immunomodulators and ablative procedures. The objective of this prospective study was the evaluation of the ultrasound-driven Harmonic Scalpel (Ethicon Endo-Surgery, Norderstedt, Germany) for resection of anal and perianal condylomata acuminata. METHODS: Eight men and three women (age range, 26-72 years) with anal and perianal condylomata acuminata were treated by a Harmonic Scalpel blade operating at a vibration frequency of 55.5 kHz and within a temperature range of 65 degrees C to 120 degrees C. Nine patients were treatment naïve, and two patients had recurrent disease. Follow-up ranged from 4 to 26 months. RESULTS: Seven patients had perianal condylomata, two patients had exclusively intra-anal, and two patients had perianal and intra-anal warts. All condylomata were excised in a single-step procedure with complete clearing without injury of subepidermal layers. We observed no intraoperative or postoperative complications. No recurrences occurred during follow-up. CONCLUSIONS: The Harmonic Scalpel has been an effective and safe method for the treatment of anal perianal human papillomavirus condylomata without recurrent warts. Subepidermal skin levels remained uninjured; thus, no complications or unsatisfactory cosmesis occurred.

Guideline based structured documentation: the final goal?

Structured documentation of medical procedures facilitates information retrieval for research and therapy and may help to improve patient care. Most medical documents until today however consist mainly of unstructured narrative text. Here we present an application for endoscopy which is not only fully integrated into a comprehensive clinical information system, but which also supports various degrees of structuring examination reports. The application is used routinely in a German University hospital since summer 2000. We present the first unstructured version which permits storage of a free text report together with selected examination images. The next step added improved structure to the document using a catalogue of index terms. The practical advantages of selective patient retrieval are described. Today we use a version which supports fully structured, guideline based documentation of endoscopy reports in order to automatically generate essential classification codes and the narrative examination report All versions have advantages and disadvantages and we conclude that guideline based documentation may not be suitable for all endoscopy cases.