Low-frequency, therapeutic ultrasound treatment for congenital ectodermal dysplasia in toddlers.

ABSTRACT

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.