ABSTRACT
Individuals with epidermolysis bullosa (EB) may present with a broad spectrum of growth impairment and multiorgan disorders, with compromised nutritional status and quality of life. The provision of nutrients through a gastrostomy tube may minimize EB-related malnourishment but may also result in skin injuries and infections. In this systematic review we consider the current evidence about the effectiveness of gastrostomy in restoring nutritional status and improving quality of life in patients with EB. Seven studies (n = 146) met selection criteria and patients ranged in age from 6 weeks to 33 years of age. Although it is not a risk-free procedure, the placement of a gastrostomy tube is a feasible and safe alternative to provide nutritional support and to improve the quality of life of patients.
Subject(s)
Epidermolysis Bullosa Dystrophica/surgery , Gastrostomy , Nutritional Status , Quality of Life , Adolescent , Adult , Child , Child, Preschool , Enteral Nutrition/methods , Epidemiologic Methods , Epidermolysis Bullosa Dystrophica/psychology , Female , Humans , Infant , Male , Malnutrition/prevention & control , Malnutrition/psychology , Young AdultABSTRACT
Epidermolysis bullosa (EB) is characterized by extreme fragility of the skin and mucosae. Anesthetic and surgical techniques have to be adapted to those children and routine practice may not be adequate. Urological problems are relatively common, but surgical techniques adapted to those children have not been well debated and only low evidence is available to this moment. Herein we discuss the specifics of anesthetic and surgical techniques chosen to treat a six year old EB male presenting with symptomatic phimosis.
Subject(s)
Anesthesia, General/methods , Epidermolysis Bullosa Dystrophica/surgery , Phimosis/surgery , Urologic Surgical Procedures, Male/methods , Child , Epidermolysis Bullosa Dystrophica/complications , Humans , Male , Phimosis/etiology , Surgical Fixation Devices , Treatment Outcome , Urologic Surgical Procedures, Male/instrumentationABSTRACT
Epidermolysis bullosa (EB) is characterized by extreme fragility of the skin and mucosae. Anesthetic and surgical techniques have to be adapted to those children and routine practice may not be adequate. Urological problems are relatively common, but surgical techniques adapted to those children have not been well debated and only low evidence is available to this moment. Herein we discuss the specifics of anesthetic and surgical techniques chosen to treat a six year old EB male presenting with symptomatic phimosis.
Subject(s)
Child , Humans , Male , Anesthesia, General/methods , Epidermolysis Bullosa Dystrophica/surgery , Phimosis/surgery , Urologic Surgical Procedures, Male/methods , Epidermolysis Bullosa Dystrophica/complications , Phimosis/etiology , Surgical Fixation Devices , Treatment Outcome , Urologic Surgical Procedures, Male/instrumentationABSTRACT
A epidermólise bolhosa é uma doença hereditária que causa alterações em proteínas estruturais da pele e consequente fragilidade da epiderme. Manifesta-se por surgimento de flictenas por todo o corpo e deformidades funcionais de membros, especialmente nas mãos, sendo que as formas mais características são pseudossindactilia e contraturas. Neste trabalho, descrevemos o caso de um paciente de 12 anos com deformidades nas mãos e flictenas pelo corpo que foi submetido à cirurgia da mão para recuperação da movimentação funcional (AU)
Epidermolysis bullosa is a hereditary disease that causes changes in structural proteins of the skin and consequent fragility of the epidermis. It is manifested by the appearance of blisters all over the body and functional deformities of limbs, especially the hands, and the most characteristic forms are pseudosyndactyly and contractures. In this paper, we describe the case of a 12-year-old patient with deformities in his hands and blisters over the body who underwent hand surgery for recovery of functional movement (AU)