Surgical management of hand deformity in Epidermolysis Bullosa: our experience in a case series of pediatric patients

Gestione chirurgica della deformità della mano in Epidermolisi Bullosa: la nostra esperienza in una serie di casi di pazienti pediatrici

Mario Lando 1, Giulia Bernante 2, Alessandro Portoghese 2, Camilla Reggiani 3, Barbara Ferrari 3, Chiara Fiorentini 3, Raimondo Feminó 4, Cristina Magnoni 3

1 Department of Muscle-Skeletal Surgery Hand and Microsurgery, Department University Hospital Modena, Modena, Italy; 2 Division of Plastic Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Modena, Italy; 3 Division of Regenerative and Oncological Dermatological Surgery, Modena University Hospital, Modena, Italy; 4 Department of General and Specialist Surgeries, Anesthesia and Intensive Care Unit 2,University Hospital Policlinico of Modena, Modena, Italy

DOI 10.53239/2784-9651-2023-19

In this study we present 2 patients for a total of 2 surgically treated hands. Data were collected between 2017 and 2023 at Azienda Universitaria Policlinico di Modena (Modena, Italy). The postoperative follow-up period ranged between 12 months and 18 months, with an average duration of 15 months. The procedure performed on these patients involved a first web release for the thumb and/or pseudosyndactyly release for the remaining digits and/or the surgical treatment of camptodactyly with Malek cutaneous approach. Acellular dermal substitute (Matriderm®) was used to cover the remaining commissures, digits, and the remainder of the hand. Postoperative rehabilitation ensued. The patient’s medical history, physical examination findings, and treatment are described. The outcome of surgical release and rehabilitation are discussed. Results: Long-term results are encouraging, demonstrating maintenance of functions.

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