Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence).

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.

Spastic quadriplegia following intradermal use of hydrogen peroxide in the tardive curettage procedure for the treatment of a giant congenital nevus.

The possible transformation of a giant congenital melanocytic nevi (GCMN) in malignant melanoma estimated from 0.05% to 40% depend on the size of the lesions. Many are the surgical procedures proposed, including: full or partial-thickness excisions, dermabrasion, curettage in the first weeks of life and laser treatment. The curettage technique has been proposed in the literature for the treatment of GCMN in the first few weeks of life and defined as a relatively atraumatic surgery procedure without general complications. The authors report the first case in the literature of embolization due to use of subcutaneous peroxide infiltration before a tardive curettage procedure in a newborn case of GCMN resulting in spastic quadriplegia with dystonic reaction. Consequently, a lawsuit, due to this medical malpractice, has been opened.

Experiences of patients with Poland syndrome of diagnosis and care in Italy: a pilot survey.

BACKGROUND: Poland Syndrome (PS) is a rare congenital malformation involving functional and aesthetic impairments. Early diagnosis and timely therapeutic approaches play an important role in improving the quality of life of patients and kindred. This study aims to explore healthcare experiences of the diagnosis of patients affected by PS and to investigate the factors associated with diagnostic delay in Italy. RESULTS: Seventy-two patients affected by PS were asked to fill in a self- administered questionnaire on: a) diagnostic path; b) perceived quality of care received after diagnosis; c) knowledge of the rights and the socio-economic hardships related to their disease; d) evaluation of the integration of various professional skills involved in the diagnostic and therapeutic approach; e) perception of the social support provided by the Italian Association of Poland Syndrome (AISP). The average age at diagnosis was around 14 years; diagnosis was made at birth in only 31.58% of cases. Although typical symptomatology had appeared on average at an early age (4 months), only 23 patients (40.35%) received an early diagnosis (within the first year of life). Just over half of the patients (n = 30) were diagnosed in their region of origin, while 27 were diagnosed elsewhere. Furthermore, 12.28% were self-diagnoses. Among the patients who were diagnosed outside their region, 15 (88.24%) stated they had foregone some visits or treatments owing to costs and/or organizational issues. CONCLUSIONS: An analysis of the patients' experiences highlights several gaps and a lack of homogeneity in the diagnostic and therapeutic follow-up of PS patients in Italy. A specific national diagnostic and therapeutic path is essential to guarantee patients complete and appropriate health services, compliant with the ethical principles of non-discrimination, justice and empathy. Implementation of an effective information and research network and empowerment of patients' associations are necessary conditions to encourage clinical collaboration and improve the quality of life of people living with rare diseases.

Conscientious Objection to Animal Testing: A Preliminary Survey Among Italian Medical and Veterinary Students.

The use of animals for educational and research purposes is common in both veterinary and human medicine degree courses, and one that involves important ethical considerations. The aim of this study was to assess the extent of differences between the knowledge and attitudes of veterinary students and medical students on animal bioethics, on alternative strategies and on their right to conscientiously object to animal experimentation. To this end, a questionnaire was completed by 733 students (384 human medicine students (HMS) and 349 veterinary medicine students (VMS)). VMS were more aware than HMS (72.2% and 59.6%, respectively) of the existence of an Italian law on the right to conscientiously object to animal experimentation. However, very few of them had exercised this right. Many VMS (43.3%) felt that animal bioethics courses should be mandatory (only 17.4% of HMS felt the same way). More VMS than HMS (81.7% and 59.1%, respectively) expressed an interest in attending a course on alternatives to animal experimentation. The data suggest the need for appropriate educational interventions, in order to allow students to make choices based on ethical principles. Fostering close collaborations between departments of human medicine and veterinary medicine, for example, through shared study modules, could promote the development of ethical competence as a basic skill of students of both veterinary and human medicine courses.

A Survey on the Knowledge and Attitudes of Italian Medical Students toward Body Donation: Ethical and Scientific Considerations.

Post mortem body donation (PMBD) for medical training and research plays a key role in medical-surgical education. The aim of this study is to evaluate Italian medical students’ awareness and attitudes regarding this practice. A questionnaire was sent to 1781 Italian medical students (MS). A total of 472 MS responded: 406 (92.91%) had a strongly positive attitude to PMBD, while 31 (7.09%) were not in favor. The majority of subjects were Catholic (56.36%), while 185 and 21 subjects, said that they did not hold any religious beliefs, or were of other religions, respectively. Multivariate analysis showed significant associations (p-values < 0.05) between PMBD and religion, as well as perceptions of PMBD as an act of altruism, a tool for learning surgical practices, body mutilation, and an act contrary to faith. Although Italian MS believed cadaver dissection to be an important part of their education, they did not know much about it and had not received training on this altruistic choice. As future doctors, MS can play an important role in raising public awareness of the importance of PMBD for medical education and research. Specific educational programs to improve knowledge of this topic among MS are needed.

Analysis of clinical management of infected breast implants and of factors associated to successful breast pocket salvage in infections occurring after breast reconstruction.

OBJECTIVES: Considerable efforts have been devoted so far to improve salvage procedures of infected breast implants in absence of defined guidelines or validated clinical protocols. Within a cohort of prospectively recruited patients who underwent breast reconstruction, we performed a retrospective review of proven implant infections in order to describe factors contributing to management success. METHODS: We collected data in 1293 consecutive patients who underwent two stage (expander+prosthesis) breast reconstruction with at least 12 months of follow-up. Demographic data, timing of infection, type of microorganism, intent of salvage, fate of the implant, type of antibiotic treatment and follow-up were recorded in a prospective data collection on clinical records. RESULTS: Implant infections occurred in 103 of 1293 patients (8%). Among these, 73 (71%) were proven infections with confirmed microbiology. Implant pocket salvage was attempted in 43/73 (59%). patients A higher proportion of expander implant pockets were successfully saved compared to prosthetic pocket (p=0,04). Gram-positive microrganisms represented the majority of etiologic agents, with coagulase negative staphylococci prevailing over Staphylococcus aureus. No association was observed between success rate and type of infecting microorganism. A higher proportion of patients with previous or intraoperative radiotherapy or with perioperative chemotherapy underwent an attempt of implant salvage (p=0,081 and 0,0571 trend, respectively). No single antibiotic regimen was superior to the others in terms of success rate. Implant pocket salvage was higher in expanders compared to prostheses (74% vs 33% p=0,04). Higher success rates in implant pocket salvage were evident when implant replacement was preceded and followed by antibiotic treatment compared to inpatient antibiotic treatment alone (100% versus 57%, p=0,035). CONCLUSION: Patient selection in clinical practice leads to differences in patients with breast implant infection who are considered for attempts at implant salvage vs. those who are treated with implant removal. Salvage of breast implant pockets can be obtained in the majority of patients with combined one-step implant replacement surgery and antibiotic treatment. Increased efforts and protocols to recruit patients into pocket salvage management are needed.

One-Stage Immediate Breast Reconstruction: A Concise Review.

BACKGROUND: One-stage direct-to-implant immediate breast reconstruction (IBR) is performed simultaneously with breast cancer resection. We explored indications, techniques, and outcomes of IBR to determine its feasibility, safety, and effectiveness. MATERIAL AND METHODS: We reviewed the available literature on one-stage direct-to-implant IBR, with or without acellular dermal matrix (ADM), synthetic mesh, or autologous fat grafting. We analyzed the indications, preoperative work-up, surgical technique, postoperative care, outcomes, and complications. RESULTS: IBR is indicated for small-to-medium nonptotic breasts and contraindicated in patients who require or have undergone radiotherapy, due to unacceptably high complications rates. Only patients with thick, well-vascularized mastectomy flaps are IBR candidates. Expandable implants should be used for ptotic breasts, while anatomical shaped implants should be used to reconstruct small-to-medium nonptotic breasts. ADMs can be used to cover the implant during IBR and avoid muscle elevation, thereby minimizing postoperative pain. Flap necrosis, reoperation, and implant loss are more common with IBR than conventional two-staged reconstruction, but IBR has advantages such as lack of secondary surgery, faster recovery, and better quality of life. CONCLUSIONS: IBR has good outcomes and patient-satisfaction rates. With ADM use, a shift from conventional reconstruction to IBR has occurred. Drawbacks of IBR can be overcome by careful patient selection.

Breast reconstruction with anatomical implants: A review of indications and techniques based on current literature.

One important modality of breast cancer therapy is surgical treatment, which has become increasingly less mutilating over the last century. Breast reconstruction has become an integrated part of breast cancer treatment due to long-term psychosexual health factors and its importance for breast cancer survivors. Both autogenous tissue-based and implant-based reconstruction provides satisfactory reconstructive options due to better surgeon awareness of "the ideal breast size", although each has its own advantages and disadvantages. An overview of the current options in breast reconstruction is presented in this article.

Tissue expansion for breast reconstruction: Methods and techniques.

OBJECTIVE: In this work, the authors review recent data on the different methods and techniques of TE/implant-based reconstruction to determine the complication profiles and the advantages and disadvantages of the different techniques. This information will be valuable for surgeons performing breast reconstructions. MATERIALS AND METHODS: A thorough literature review was conducted by the authors concerning the current strategy of tissue expander (TE)/implant-based breast reconstruction following breast cancer surgery. RESULTS: Loss of the breast can strongly affect a woman's personal and social life while breast reconstruction reduces the sense of mutilation felt by women after a mastectomy, and provides psychosocial as well as aesthetic benefits. TE/implant-based reconstruction is the most common breast reconstructive strategy, constituting almost 65% of all breast reconstructions in the US. Although numerous studies have been published on various aspects of alloplastic breast reconstructions, most studies are single-center observations. No evidence-based guidelines are available as yet. Conventional TE/implant-based reconstruction can be performed as a two-stage procedure either in the immediate or delayed setting. Moreover, the adjunctive use of acellular dermal matrix further broadened the alloplastic breast reconstruction indication and also enhanced aesthetic outcomes. CONCLUSIONS: TE/implant-based reconstruction has proved to be a safe, cost-effective, and reliable technique that can be performed in women with various comorbidities. Short operative time, fast recovery, and absence of donor site morbidity are other advantages over autologous breast reconstruction.

Relationship between lunar cycle and haemorrhagic complication rate in surgery.

BACKGROUND: The aim of this study was to evaluate a possible relationship between lunar cycles and haemorrhagic complication rate in surgery. MATERIALS AND METHODS: The possible relationship between moon phases and surgical outcome was tested by evaluating the haemorrhagic complication rate for 18,760 patients who underwent surgery between January 2001 and December 2008 at the National Institute for Cancer Research in Genoa. A total of 103 lunar phases were considered using Chi-square (χ2) test analysis, and patients were allocated a surgery date. RESULTS: One hundred and sixty-seven haemorrhagic complications were observed. Three hundred and nine new moon phase days were analysed and 12 incidences of complications detected, with a 3.9% complication rate per day. In the waxing moon phase, 1184.5 d were analysed with 68 incidences of complications at a daily rate of 5.7%. In the full moon phase there was a 4.9% complication rate per day (15 incidences in 309 d), whereas in the waning moon phase, the 6% percentage rate per day resulted from 72 incidences in 1184.5 d. CONCLUSIONS: No statistically significant correlations were found between moon cycles and postoperative haemorrhagic complications (p = .50).

Autologous platelet-rich plasma (PRP) in chronic penile lichen sclerosus: the impact on tissue repair and patient quality of life.

PURPOSE: Lichen sclerosus (LS) is a chronic inflammatory skin condition that frequently involves the anogenital region. Ongoing research is focused on finding more effective treatments for tissue repair and reducing symptoms. The aim of this study is to evaluate the effectiveness of platelet-rich plasma (PRP) local injections in penile LS. METHODS: Forty-five male patients affected by penile LS underwent injections of autologous PRP in the affected skin areas. Age at diagnosis and at first treatment, number of treatments, clinical conditions (phimosis, splitting, inflammation, synechiae, meatus stenosis), symptoms (pain, burning, itching), and functional impairment were considered. Treatment efficacy was also evaluated through the Investigator's Global Assessment (IGA) on a six-point Likert scale and the Dermatology Life Quality Index (DLQI). RESULTS: The patient age at LS diagnosis was 36.20 ± 9.19 years, while the mean age at the first PRP treatment was 42.96 ± 11.32 years (p < 0.001). The number of treatments/patient ranged from 2 to 10. The follow-up was 17.60 ± 5.63 months. After PRP injections, it was observed in all patients a significant improvement in clinical conditions, with reduction/disappearance of symptoms. Topical steroid therapy, interrupted before PRP treatment, was not restarted by any patient. Only one patient underwent a later circumcision procedure. Both IGA scale and DLQI score showed a significant difference (p < 0.001) before and after PRP treatment. CONCLUSIONS: PRP treatment in penile LS seems to be helpful to regenerate scarring, reduce symptoms, and improve patient quality of life. Further studies are necessary to evaluate long-term results.

Two-Stage Tissue-Expander Breast Reconstruction: A Focus on the Surgical Technique.

OBJECTIVE: Breast cancer, the most common malignancy in women, comprises 18% of all female cancers. Mastectomy is an essential intervention to save lives, but it can destroy one's body image, causing both physical and psychological trauma. Reconstruction is an important step in restoring patient quality of life after the mutilating treatment. MATERIAL AND METHODS: Tissue expanders and implants are now commonly used in breast reconstruction. Autologous reconstruction allows a better aesthetic result; however, many patients prefer implant reconstruction due to the shorter operation time and lack of donor site morbidity. Moreover, this reconstruction strategy is safe and can be performed in patients with multiple health problems. Tissue-expander reconstruction is conventionally performed as a two-stage procedure starting immediately after mammary gland removal. RESULTS: Mastectomy is a destructive but essential intervention for women with breast cancer. Tissue expansion breast reconstruction is a safe, reliable, and efficacious procedure with considerable psychological benefits since it provides a healthy body image. CONCLUSION: This article focuses on this surgical technique and how to achieve the best reconstruction possible.

Proposal of the TBN Classification of Thoracic Anomalies and Treatment Algorithm for Poland Syndrome.

BACKGROUND: Poland syndrome is a congenital deformity characterized by unilateral anomalies of pectoralis muscles, breast, nipple, axillary fold, subcutaneous tissue, ribs, and upper limb. The thoracic anomaly, which is the pathognomonic malformation of Poland syndrome, presents a wide phenotype variability and has been classified by different authors. However, these classifications do not include all the possible phenotypes of Poland syndrome. The aim of this study is to propose a simple classification of the whole spectrum of thoracic anomalies and a treatment algorithm that could have a practical value for determining the surgical approach. METHODS: Since 2008, 100 patients have been evaluated by the same plastic surgical team at San Martino Hospital-IST and Istituto Gaslini of Genoa, Italy, using the thorax, breast, nipple-areola complex (TBN) classification. Thoracic anomalies were classified as follows: thorax (T), from T1 (muscle defect only) to T4 (complex deformity with rib and sternal involvement); breast (B), in B1 (hypoplasia) or B2 (amastia); and nipple-areola complex (N), from N1 (dislocation <2 cm) to N3 (athelia). RESULTS: The most frequent thoracic anomalies were T1 (47 percent) and N2 (74 percent), whereas in female patients, B1 was more frequent than B2. The surgical approach to breast and pectoral reconstruction was based not only on the patient's age and sex, but also on the type of anomaly according to the TBN classification. In particular, a two-step approach with tissue expanders was required in N2 and N3 cases, whereas in N1 patients a single step was sufficient. CONCLUSION: The TBN classification can be a useful tool for surgical decision-making according to each specific thoracic anomaly. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, IV.

Implant-Based Breast Reconstruction Using a Polyester Mesh (Surgimesh-PET): A Retrospective Single-Center Study.

BACKGROUND: Although the introduction of acellular dermal matrices is considered one of the most important advancements in alloplastic breast reconstruction, costs and local policy limit their use in Italy. The purpose of this study was to assess short-term outcomes following Surgimesh-PET-based breast reconstruction. METHODS: A single-center, retrospective, case-control study was performed from January 1, 2012, to December 31, 2013, by enrolling 206 breast reconstructions performed in 196 patients after oncologic (n = 200) or prophylactic (n = 6) mastectomy. Group A included 63 patients who underwent 70 immediate Surgimesh-PET-assisted breast reconstructions, and group B included 133 patients who underwent 136 standard breast reconstructions. RESULTS: No significant differences between groups occurred for early postoperative complications (p = 0.610), major complications that required surgical revision (p = 0.887), volume (p = 0.498) or width of the prosthesis (p = 0.201), skin-sparing mastectomy (p = 0.315), or axillary surgery (p = 0.265). Multivariate logistic regression showed that prior radiotherapy was the only significant variable for early postoperative complications in both whole series (p = 0.011) and group B (p = 0.046), whereas body mass index greater than 25 was an independent predictor in group A (p = 0.041). Prior radiotherapy was the only variable that reached statistical significance in the multivariate model for major complications in the whole series (p = 0.005). CONCLUSIONS: Short-term outcomes of Surgimesh-PET-based alloplastic breast reconstruction are promising. Further studies are needed to evaluate the long-term results of this surgical approach. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Body Image Disorders and Surgical Timing in Patients Affected by Poland Syndrome: Data Analysis of 58 Case Studies.

BACKGROUND: Poland syndrome is a congenital anomaly of pectoralis muscles, breast, chest, and upper arm. Several studies have reported that patients affected by chest wall deformities often experience body image disorders and decreased quality of life. Cosmetic corrective surgery is generally postponed until physical development is achieved, and latissimus dorsi flap surgery is usually suggested. This study aims to propose a new surgical timing for these patients. METHODS: Patients affected by Poland syndrome (n = 58) and control patients (n = 50) were included in the study, and their body-related psychopathology was evaluated through the Body Uneasiness Test, a valuable multidimensional tool for the clinical assessment of body uneasiness. The Global Severity Index and several subscales were considered. Age and surgical status were taken into account. RESULTS: Significant differences were detected between Poland syndrome patients and controls with respect to all Body Uneasiness Test subscales. Among subjects aged younger than 20 years, the group of patients not operated on was the most affected, whereas the group of peers after surgery showed scores similar to those detected in the control group. CONCLUSIONS: Surgical planning for patients with Poland syndrome should start in the period of growth to allow proper body image stabilization. Current surgical options allow for reduction of the use of invasive interventions such as the latissimus dorsi flap, in favor of less invasive surgical techniques such as expanders, implants, and autologous fat grafting. This new surgical timing will help to ameliorate problems with physical and mental development. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Interaction Between Breast Cancer Cells and Adipose Tissue Cells Derived from Fat Grafting.

BACKGROUND: Adipose tissue transplantation has the benefit of providing both regenerative and aesthetic outcomes in breast cancer treatment. However, the transplanted tissue can stimulate the growth of residual cancer cells. OBJECTIVES: The aim of this study is to identify the interactions between adipose tissue cell subpopulations and human cancer cell lines. METHODS: Intact adipose tissue from lipofilling procedures as well as fibroblasts derived from adipose tissue, were cocultured in the presence of MDA-MB-231, MCF-7 e ZR-75-1 breast cancer cell lines. The influence on cancer cell lines of fibroblasts, induced to differentiate into specific adipocytes, was also assayed. RESULTS: All cancer cell lines displayed a significant increase in proliferation rate when cocultured in the presence of either intact adipose tissue or induced adipocytes. To a lesser extent, uninduced fibroblasts stimulate breast cancer cell proliferation. CONCLUSIONS: Recent studies have shown that the microenvironment surrounding breast cancer cells may stimulate growth and promote progression of residual cancer cells when surgery is performed on the main tumor mass. Accordingly, the graft of adipose tissue could potentially promote or accelerate the development of a subclinical tumor or support its locoregional recurrence. Our data suggest that adipocytes have a remarkable influence on the proliferation of cancer cell lines. The oncological safety of the lipofilling procedure outcome is still debated; thus, further studies and consistent follow-up examination are needed.

Which is the Ideal Breast Size?: Some Social Clues for Plastic Surgeons.

To provide plastic surgeons with more detailed information as to factors affecting the perception of female attractiveness, the present study was aimed to investigate whether the interaction effect of breast and body size on ratings of female attractiveness is moderated by sociodemographic variables and whether ratings of shapeliness diverge from those of attractiveness.A community sample of 958 Italian participants rated the attractiveness and the shapeliness of 15 stimuli (5 breast sizes × 3 body sizes) in which frontal, 3/4, and profile views of the head and torso of a faceless woman were jointly shown.Bigger breast sizes obtained the highest attractiveness ratings, but the breast-by-body size interaction was also significant. Evidence was found of a moderator role of sex, marital status, and age. When the effects of breast and body size and their interaction had been ruled out, sex differences were at best very slight and limited to very specific combinations of breast and body sizes. Ratings of attractiveness and shapeliness were highly correlated and did not significantly differ.Results suggest that to address women's psychological needs, concerns, and expectations about their appearance, plastic surgeons should not simply focus on breast size but should carefully consider the 'big picture': the body in its entirety.

Fibrin gels loaded with cisplatin and cisplatin-hyaluronate complexes tested in a subcutaneous human melanoma model.

Fibrin gels are attractive biomaterials for local delivery of a variety of agents, from drugs to proteins. Similarly, polymer-anticancer-drug conjugates and nanoparticles are emerging as potential candidates for cancer treatment. Combining these different approaches, we have studied the efficacy of fibrin gels loaded with cisplatin (DDP) and a complex of DDP with hyaluronate (DDP-HA) for tumor growth inhibition in a melanoma model. Loaded gels prepared at relatively high fibrinogen concentration (22 mg/ml) showed good in vitro antiproliferative activities, prolonged release of the anticancer drug, and a long persistence (10-15 days) in vivo when implanted subcutaneously (sc) in immunodeficient mice. Gels loaded with DDP or DDP-HA containing 1/3 or even 1/6 of their systemic dose (6 mg/kg) and positioned under the tumor mass in mice bearing a sc human SK-Mel-28 tumor showed an antitumor activity better than that of the original parent compound given intraperitoneally (ip). Moreover, in an additional experiment in vivo, fibrin gels loaded with N-trimethyl chitosan-based nanoparticles containing a DDP-HA complex were assayed, resulting in a further 8 % improvement of anticancer activity, with lesser adverse systemic toxic effects. Taken together, these results suggest that the combination of fibrin gels and drugs complexed with suitable macromolecules holds great promise for loco-regional anticancer therapy of melanoma and other surgically removable cancer types.