Explore the vast range of titles available.

In patients with epidermolysis bullosa (EB), surgery may be required to remove squamous cell carcinoma (SCC) of the hands or to correct pseudo-syndactyly. Dermal substitutes may represent a suitable tool to promote the healing of surgical wounds in EB. We review our experience with a collagen-elastin dermal matrix to promote surgical wound healing due to hand surgery to correct pseudo-syndactyly or SCC resection in patients affected by EB. Overall, 12 patients (mean age, 29±11 years, range 13-51; four females) with EB were considered, with a total of 25 different interventions (16 SCC removals; three amputations for recurrent infiltrating SCC; six pseudo-syndactyly corrections). All patients received a collagen-elastin dermal matrix sheet. Neodermis was achieved in all patients, and no adverse events potentially associated with the graft occurred. No SCC recurrence was reported at sites operated for either SCC resection or amputation. The opening of the first or second commissure appears to be maintained for over 1 year after the procedure, preserving digital function. We believe that the application of a dermal substitute, together with a close dressing protocol, may represent a suitable strategy for obtaining physiological tissue regeneration and maintaining proper hand function in patients with EB.

Capillary malformations (MCs), also referred to as port-wine stains (PWSs), or port-wine birthmarks (PWBs) represent one of the most common congenital vascular birthmarks. They consist of ectatic postcapillary venules within the papillary and superficial reticular dermis. The pathogenesis is due to a somatic activating mutation in guanine nucleotide–binding protein G(q) subunit alpha which mosaically occurs in endothelial cells (ECs) during embryogenesis. As a true congenital vascular malformation, PWSs are always present at birth, appearing as a flat pink to red macula mostly arising in the head and neck unilaterally. Although lightening during the first few months of life is possible, these lesions generally stay stable or thicken and darken over time. Consequent functional impairment, tissue thickening, development of blebs, and psychological burden may be relevant, so early laser therapy should often be considered to deter progression. What we present here is a unique cohort of 10 children presented to the Vascular Birthmarks Foundation (VBF) in Latham, NY, between 2016 and 2021, who were followed over time for cutaneous vascular anomalies present at birth. The clinical features were strongly suggestive of PWSs. However, all 10 of these children showed a significant spontaneous regression of the lesions during the observational period. In four cases, there was complete resolution. According to the international literature, capillary malformations persist throughout life and typically grow and darken if early laser treatment is not initiated. Such spontaneous improvement is extremely rare after the first 6 months of life. In the present case series, we photographically documented this previously unreported circumstance. Level of evidence: Level V, risk/prognostic study.

The effect of multiple general anesthesia (mGA) procedures administered in early life is a critical theme and has led the Food and Drug Administration (FDA) to issue an alert. This systematic review seeks to explore the potential effects on neurodevelopment of mGA on patients under 4 years. The Medline, Embase and Web of Science databases were searched for publications up to 31 March 2021. The databases were searched for publications regarding “children multiple general anesthesia OR pediatric multiple general anesthesia”. Case reports, animal studies and expert opinions were excluded. Systematic reviews were not included, but they were screened to identify any possible additional information. A total of 3156 studies were identified. After removing the duplicates, screening the remaining records and analyzing the systematic reviews’ bibliography, 10 studies were considered suitable for inclusion. Comprehensively, a total cohort of 264.759 unexposed children and 11.027 exposed children were assessed for neurodevelopmental outcomes. Only one paper did not find any statistically significant difference between exposed and unexposed children in terms of neurodevelopmental alterations. Controlled studies on mGA administered before 4 years of age support that there might be a greater risk of neurodevelopmental delay in children receiving mGA, warranting the need for careful risk/benefit considerations.

Background It is well known that benign tracheal stenosis represents an obstacle to open surgery, and that its treatment could be challenging. Two endoscopic techniques have so far been adopted to restore tracheal patency: balloon dilatation (BA) through laryngoscopy, and tracheal stenting (ST) with rigid bronchoscopy. The main objective of this study was to compare the efficacy of BA and ST to treat benign tracheal stenosis not eligible for surgery. We also compared the rate of adverse events in the two treatment groups. Methods A retrospective, observational cohort study was carried out at the University Hospital of Modena (Italy) from November 2012 to November 2017 in two separate departments. Patients were considered to be “stabilized” (primary outcome) if they did not report significant respiratory symptoms, or restenosis in the long‐term (2 years) following the endoscopic procedure. Results Sixty‐six patients were included in the study (33 in the BA and 33 in the ST group, respectively). Unadjusted Kaplan–Meier estimates showed a greater therapeutic effect of ST compared to BA at 2 years (hazard ratio = 3.9 95%CI [1.5–9.8], p = .01). After adjusting for confounders, stratified analyses showed that this effect was significant in patients with complex stenosis, idiopathic etiology, and degree of stenosis >70%. Compared with BA, ST showed a higher rate of adverse events (p = .01). Conclusions Compared to BA, ST seems to be more effective in achieving stabilization of tracheal patency in complex benign tracheal stenosis, although burdened with a significantly higher number of adverse effects. These findings warrant future prospective study for confirmation. Level of evidence: 3.

In patients with epidermolysis bullosa (EB), surgery may be required to remove squamous cell carcinoma (SCC) of the hands or to correct pseudo-syndactyly. Dermal substitutes may represent a suitable tool to promote the healing of surgical wounds in EB. We review our experience with a collagen-elastin dermal matrix to promote surgical wound healing due to hand surgery to correct pseudo-syndactyly or SCC resection in patients affected by EB. Overall, 12 patients (mean age, 29±11 years, range 13-51; four females) with EB were considered, with a total of 25 different interventions (16 SCC removals; 3 amputations for recurrent infiltrating SCC; 6 pseudo-syndactyly corrections). All patients received a collagen-elastin dermal matrix sheet. Neodermis was achieved in all patients, and no adverse events potentially associated with the graft occurred. No SCC recurrence was reported at sites operated for either SCC resection or amputation. The opening of the first or second commissure appears to be maintained for over 1 year after the procedure, preserving digital function. We believe that the application of a dermal substitute, together with a close dressing protocol, may represent a suitable strategy for obtaining physiological tissue regeneration and maintaining proper hand function in patients with EB.

In patients with Epidermolysis Bullosa (EB), surgery may be required to remove Squamous Cell Carcinoma (SCC) of the hands or to correct pseudo-syndactyly. Dermal substitutes may represent a suitable tool to promote the healing of surgical wounds in EB. We review our experience with a collagen-elastin dermal matrix to promote surgical wound healing due to hand surgery to correct pseudo-syndactyly or SCC resection in patients affected by EB. Overall, 12 patients (mean age, 29±11 years, range 13-51; four females) with EB were considered, with a total of 25 different interventions (16 SCC removals; three amputations for recurrent infiltrating SCC; six pseudo-syndactyly corrections). All patients received a collagen-elastin dermal matrix sheet. Neodermis was achieved in all patients, and no adverse events potentially associated with the graft occurred. No SCC recurrence was reported at sites operated for either SCC resection or amputation. The opening of the first or second commissure appears to be maintained for over 1 year after the procedure, preserving digital function. We believe that the application of a dermal substitute, together with a close dressing protocol, may represent a suitable strategy for obtaining physiological tissue regeneration and maintaining proper hand function in patients with EB.

Background Recessive dystrophic epidermolysis bullosa (RDEB) is a rare and severe mucocutaneous fragility disorder due to mutations in the COL7A1 gene encoding collagen VII, the major constituent of anchoring fibrils essential for epithelial adhesion. RDEB is characterized by unremitting blistering, chronic painful wounds and fibrotic scarring that results in hand and foot pseudosyndactyly, microstomia, and esophageal strictures. RDEB complications include nutritional compromise, chronic anemia, failure to thrive, delayed puberty, osteoporosis, and renal involvement. In addition, early onset cutaneous squamous cell carcinomas (cSCC) represent the first cause of premature death. Despite recent progress in wound care, disease management still relies on symptomatic and preventive measures. No clinical practice guidelines specifically focused on the care of RDEB are currently available. The present multidisciplinary consensus recommendations were generated following a modified Delphi method with the aim to provide healthcare professionals with practical statements on RDEB management from birth to adulthood. Results Ten experts from six Italian EB reference centers developed 86 statements based on existing clinical practice guidelines and consensus recommendations for EB, literature data, and personal expertise. A multidisciplinary group of 30 members, representative of all major specialties relevant to RDEB management, participated to the anonymous online voting process. All statements reached consensus (> 75% agreement) at first voting round. Statements are divided into four major areas: (1) diagnosis, (2) neonatal age and infancy, (3) from childhood to adulthood, and (4) transversal age-independent issues, each of the last three comprising multiple domains of care. In particular, the section on patient care from childhood to adults deals with measures for management of wounds, gastrointestinal, eye and renal involvement, nutritional compromise, anemia, hand and foot deformities, cSCC, delayed puberty and osteoporosis, sexuality, pregnancy and delivery. Transversal issues comprise: pain and itch management, patient care in the operating theatre, physiotherapy and occupational therapy, therapeutic patient education and psychosocial support. Conclusions The proposed practical and synthetic recommendations cover all major issues in the management of patients with RDEB from birth to adulthood. They can represent a useful tool to support hospital healthcare personnel as well as primary care physicians in the complex multidisciplinary management of RDEB.