Sound Evidence - October 2008 - (Page 58) Sound Evidence Low-frequency, Therapeutic Ultrasound Treatment for Congenital Ectodermal Dysplasia in Toddlers Deborah Caswell, RN, MNS, NP-C, UCLA-Gonda (Goldschmied) Vascular/Wound Healing Center, UCLA Division of Vascular Surgery, David Geffen School of Medicine, UCLA Center for Health Sciences, Los Angeles, Calif; and Brianna M. McNulty, UCLA Division of Vascular Surgery, Los Angeles, California Hay-Wells Syndrome is a rare genetic disorder characterized by ankyloblepharon, ectodermal dysplasia, and cleft palate. Recalcitrant scalp wounds with secondary infections are common. This case series describes the use of acoustic pressure wound therapy in 3-year-old fraternal twins (male and female) with HWS-associated scalp wounds. Present since infancy, the wounds were severe and extensive at presentation to the authors’ wound clinic. Previous management consisted of standard topical treatments, including foam; oxidized, regenerated-cellulose/collagen with silver; calcium alginate; silver sulfadiazine cream; and biologic tissue matrix. Following admission to the authors’ wound clinic, acoustic pressure wound therapy was administered one to three times weekly for 3 to 10 minutes for 7 months in addition to standard topical treatments to provide nonsurgical debridement and reduce wound bioburden without inflicting additional pain. Substantial improvements occurred during the first 5 weeks of consistent treatment. When treatments became sporadic due to health and family issues, wound deterioration occurred. After 7 months, wound sizes decreased by 31.3% in the boy and 1.1% in the girl, 70% of the wound surface in both children was covered with granulation tissue, and no clinical signs of infection were evident. The treatments were well tolerated. So far, the twins each received a total of 37 treatments. Consistent, long-term acoustic pressure wound therapy improved the status of severe, recalcitrant, Hay-Wells Syndrome-associated scalp wounds. KEYWORDS: acoustic pressure wound therapy, AEC Syndrome, ectodermal dysplasia, Hay-Wells Syndrome, wounds Ostomy Wound Management 2008;54(10):58–61 ay-Wells syndrome (HWS) is a rare, genetic disorder that belongs to a group of disorders known as ectodermal dysplasias.1-3 Characterized by ankyloblepharon (fused eyelids), ectodermal dysplasia, and cleft palate (with or without cleft lip), HWS is also known as AEC syndrome. This H autosomal dominant disorder is associated with partial or complete hair loss, absent or dystrophic nails, abnormal dentition, anhidrosis, blocked tear ducts, webbed fingers and toes, superfluous nipples, and ear deformities.2-4 Newborns with HWS typically have large areas of denuded skin, including erosions of the Support for Sound Evidence is provided by an educational grant from Celleration, Inc, Eden Prairie, Minn, to HMP Communications/Ostomy Wound Management. Celleration support to authors included medical writing and/or statistical support only; study design, patient selection, data collection, and metrics to measure wound healing are specific to the author. The opinions and statements herein are also specific to the author and are not necessarily those of Celleration, Inc., OWM, or HMP Communications. Please note: these articles are subject to peer review. The opinions herein may not be consistent with the labeling for MIST Therapy Systems. Patients are selected for educational benefit. Visit www.celleration.com for the full package insert. Results may vary. 58 OstomyWound Management http://www.celleration.com
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