Background: Poland syndrome (OMIM: 173800) is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, breast, shoulder, arm, and hand. The extent and severity of the abnormalities vary among affected individuals. Main body: The aim of this work is to provide recommendations for the diagnosis and management of people affected by Poland syndrome based on evidence from literature and experience of health professionals from different medical backgrounds who have followed for several years affected subjects. The literature search was performed in the second half of 2019. Original papers, meta-analyses, reviews, books and guidelines were reviewed and final recommendations were reached by consensus. Conclusion: Being Poland syndrome a rare syndrome most recommendations here presented are good clinical practice based on the consensus of the participant experts.
Syndactyly release may require skin grafting to fill the skin defects, which might lead to complications or poor cosmetic outcomes. A simple graftless technique for syndactyly release with a hyaluronic acid (HA) scaffold used to cover the bare areas is described. Between 2008 and 2011, release of 26 webs in 23 patients was performed. All skin defects were covered with Hyalomatrix(®) PA. One patient was excluded due to early post-operative infection that required HA scaffold removal before its integration. Web creep, secondary deformities, scar quality, and patient and parental satisfaction were assessed. Mean follow-up of the group of 22 patients was 24 months. There were no secondary deformities and minimal degree of web creep. All patients had close to normal pigmentation and good pliability at the sites of scaffold application. The results confirm the use of a HA scaffold as a promising alternative to skin grafting in syndactyly release surgery.
Hand injuries after high-pressure injection are a medical emergency. These events occur frequently in workers during industrial cleaning, painting, and lubrication, and may have devastating consequences, leading to eventual amputation and poor functional outcomes. The authors have investigated the evolution, management, and outcome. Medical records of occupational medicine units and hand surgery units were collected in order to spot the high-pressure gear accident cases. Records were analyzed by dividing the subjects into two groups: those treated within 6 h and after 6 h of the trauma. A follow-up was carried out at least 1 year after treatment; the post-treatment outcomes were assessed. Of the 71 (100%) subjects, 26 (37%) were treated ≤6 h and 45 (63%) >6 h. A total of 28% (n = 20) underwent amputation. In 61% of cases, accidents had occurred in the iron and steel sector. High viscosity materials with a delayed treatment beyond 6 h seemed to determine compartmental syndrome and following amputation. A significantly better outcome was reported among subjects treated ≤6 h compared to those treated >6 h, 20% (n = 7) versus 26% (n = 9), respectively. Early management of this type of injury is crucial. The results of this study may contribute to providing guidelines to occupational physicians in order to best manage this type of emergency.
The two etiologies differ in terms of patient's clinical features, morphology defect and axis involvement. This data may be helpful to the clinician during the patient's diagnostic workup by indicating the necessity for genetic testing and for determining the anomaly's recurrence risk.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.