Evaluation of Non-Coverage Cost Effective Technique for Management of Epidermolysis Bullosa Mitten Hands in Developing Countries | ||||
| The Egyptian Journal of Plastic and Reconstructive Surgery | ||||
| Article 8, Volume 45, Issue 1, January 2021, Page 41-47 PDF (20.41 MB) | ||||
| Document Type: Research article | ||||
| DOI: 10.21608/ejprs.2021.165800 | ||||
 View on SCiNiTO | ||||
| Authors | ||||
Adel H. Amr; Omar Mahmoud  ; Mohamed A. Ghanem
| ||||
| The Department of Plastic, Reconstruction, Maxillofacial Surgeries and Burn Management, Faculty of Medicine, Ain Shams University, Cairo, Egypt | ||||
| Abstract | ||||
| Background: Epidermolysis bullosa (EB) is a group of rare genetic disorders known by susceptibility of the skin epidermis and mucosa that separate from underlying tissues and cause increase in skin fragility, blistering, and scarring in response to minor traumatic injuries. Many methods were described in the literatures for soft tissue coverage after the release of mitten hands which include cultured keratinocytes; using epithelium raised from cocoon, to split skin grafts or full-thickness skin grafts; and cellular allograft dermal matrix. In this study, we described a simple and efficient noncoverage technique, as it eliminates the donor site complications when using STSG or FTSG, and also minimizes cost management by avoid using cellular allograft dermal matrix, specially in developing countries, where cost effectiveness due to financial issues should be considered. Aim of this Study: Is to describe and evaluate the noncoverage technique based on the Birmingham Epidermolysis Bullosa Severity Score Sheet (BEBSS) in patients suffering from epidermolysis bullosa mitten hands. Patients and Methods: We operated upon nine patients suffering from epidermolysis bullosa mitten hands by using the non-coverage technique to obtain full release of all fingers and web spaces. Evaluation and scoring of preoperative and postoperative hand scars were based on the 'Birmingham Epidermolysis Bullosa Severity Score Sheet. Results: Showed major improvement in the mean of postoperative BEBSS with lower rate of recurrence and postoperative complications. Hand function was remarkably improved within 2 months which was demonstrated subjectively by the capability of grasping and pinching objects such as cups, spoons and pens using unilateral hand. All patients and care givers were satisfied with the improvement in function and the ability to perform more tasks than preoperatively. Conclusion: Managing soft tissue defects after release of EB mitten hands can be left with no coverage and with appropriate dressing for self-re-epithelialization can be ideal method especially in developing countries where allografts are relatively expensive and not available. | ||||
| Keywords | ||||
| Non-Coverage; Cost; Epidermolysis Bullosa; Developing Countries | ||||
| References | ||||
|
Tian F., Li B. and Tian L.J.: Treatment of severe hand deformities caused by epidermolysis bullosa. Orthopedics, 2011. 2- Fine J.D., Bruckner-Tuderman L., Eady R.A., et al.: Inherited epidermolysis bullosa: Updated recommendations on diagnosis and classification. J. Am. Acad. Dermatol., 2014. 3- Luria S., Radwan S., Zinger G., et al.: Hand surgery for dystrophic epidermolysis bullosa. J. Pediatr. Orthop., 2014. 4- Pfendner E.G. and Lucky A.W.: Dystrophic epidermolysis bullosa. NCBI bookshelf. A service of the National Library of Medicine [updated 2018 Sep 13]. National Institutes of Health; Seattle (WA), 2006. 5- Eisenberg Llewelyn M.D.: Surgical management of hands in children with recessive dystrophic epidermolysis bullosa: Use of allogeneic composite cultured skin grafts. Br. J. Plast. Surg., 1998. 6- Fivenson D.P., Scherschun L. and Cohen L.V.: Apligraf in the treatment of severe mitten deformity associated with recessive dystrophic epidermolysis bullosa. Plast. Reconstr. Surg., 2003. Egypt, J. Plast. Reconstr. Surg., January 2021 47 7- Bernardis C. and Box R.: Surgery of the hand in recessive dystrophic epidermolysis bullosa. Dermatologic Clinics. Apr., 2010. 8- Wong A., Ward R., Hadley N., et al.: A scoring system for epidermolysis bullosa. Br. J. Dermatol., 2007. 9- Ladd A.L., Kibele A. and Gibbons S.: Surgical treatment and postoperative splinting of recessive dystrophic epidermolysis bullosa. J. Hand Surg., 1996. 10- Eisenberg Llewelyn M.D.: Surgical management of hands in children with recessive dystrophic epidermolysis bullosa: Use of allogeneic composite cultured skin grafts. Br. J. Plast. Surg., 1998. 11- Jutkiewicz J., Noszczyk B.H. and Wrobel M.: The use of Biobrane for hand surgery in Epidermolysis bullosa. Journal of Plastic, Reconstructive & Aesthetic Surgery. Aug., 2010. 12- Özkaya Ö., Örerog˘lu A.R. and Akan M.: The use of acellular dermal matrix in treatment of mitten hand in epidermolysis bullosa patients. Journal of Hand and Microsurgery. Jun., 2013. 13- Zhou X., Zhang Y., Zhao M., Jian Y., Huang J., Luo X., Yang J. and Sun D.: Surgical management of hand deformities in patients with recessive dystrophic epidermolysis bullosa. Journal of Plastic Surgery and Hand Surgery. Jan., 2020. | ||||
|
Statistics Article View: 191 PDF Download: 157 |
||||
