Transgender and Alagille Syndrome: A Rare Case of a Trans Woman with Alagille Syndrome.

Alagille syndrome is a rare autosomal dominant disorder with variable expression. Liver damage, especially cholestatic, is the most common feature of the syndrome. Transgender patients may suffer from a great distress due to the discrepancy between assigned sex at birth and unaffirmed gender identity. Gender affirmation treatment options for these patients include hormone therapy (HT) to induce secondary sexual characteristics and various surgical procedures. Estrogen-based hormonal treatments have been linked to an increased risk of liver enzyme elevation and disruption of bilirubin metabolism, especially in those with a genetic susceptibility. The case presented here is the first described Alagille syndrome transgender patient to undergo gender affirmation treatment, including (HT) and vulvo-vaginoplasty surgery.

Using Virtual Reality for Deep Inferior Epigastric Perforator Flap Preoperative Planning.

This study was designed to compare VR stereoscopical three-dimensional (3D) imaging with two-dimensional computed tomography angiography (CTA) images for evaluating the abdominal vascular anatomy before autologous breast reconstruction. Methods: This prospective case series feasibility study was conducted in two tertiary medical centers. Participants were women slated to undergo free transverse rectus abdominis muscle, unilateral or bilateral deep inferior epigastric perforator flap immediate breast reconstruction. Based on a routine CTA, a 3D VR model was generated. Before each procedure, the surgeons examined the CTA and then the VR model. Any new information provided by the VR imaging was submitted to a radiologist for confirmation before surgery. Following each procedure, the surgeons completed a questionnaire comparing the two methods. Results: Thirty women between 34 and 68 years of age were included in the study; except for one, all breast reconstructions were successful. The surgeons ranked VR higher than CTA in terms of better anatomical understanding and operative anatomical findings. In 72.4% of cases, VR models were rated having maximum similarity to reality, with no significant difference between the type of perforator anatomical course or complexity. In more than 70% of the cases, VR was considered to have contributed to determining the surgical approach. In four cases, VR imaging modified the surgical strategy, without any complications. Conclusions: VR imaging was well-accepted by the surgeons who commented on its importance and ease compared with the standard CTA presentation. Further studies are needed to determine whether VR should become an integral part of preoperative deep inferior epigastric perforator surgery planning.

A new simple, safe, and easy solution for upper lip dermabrasion.

BACKGROUND: Upper lip wrinkling is a common complaint of patients seeking perioral rejuvenation. Lately, manual dermabrasion has become more popular due to its safety, minimal cost, and favorable results. In several hospitals, the ability to efficiently sterilize sand paper has been questioned. METHODS: Between 2007 and 2010, 29 patients underwent manual dermabrasion of the skin of the upper lip using an electric cautery scratch pad during their surgeries. RESULTS: The average patient was aged 60.2 years. The average healing period was 5.8 days. Patient satisfaction from the procedure ranged from very good to excellent. No serious or long lasting complications have been encountered during our follow-up period.

Radiotherapy-induced basal cell carcinomas of the scalp: are they genetically different?

BACKGROUND: The treatment of tinea capitis using radiotherapy was introduced at the beginning of the twentieth century. In Israel, between 1949 and 1960, approximately 17,000 children underwent radiotherapy treatments for tinea capitis (actual numbers are probably higher due to irradiation in countries of origin as a prerequisite for immigration). Skin cancer presents a major problem for patients who underwent irradiation for the treatment of tinea capitis [aggressive biological behavior, multiple basal cell carcinomas (BCCs), up to 40 lesions in a single patient, with no predisposing condition such as Gorlin's or Bazex's syndromes]. There are ample data in the literature concerning the molecular changes in ultraviolet (UV) radiation-induced BCCs. However, similar data regarding ionizing radiation-induced BCCs are scarce. One work found higher rates of p53 and PTCH (both are tumor suppressor genes whose alterations are associated with BCC formation and frequency, but not biological behavior) abnormalities in post ionizing radiation BCCs. The absence of documented differences in gene expression that would account for a different biological behavior of radiotherapy-related BCCs, coupled with the aggressive and recurrent nature of these lesions, has propelled us to examine these differences by comparing gene expression in BCCs of the scalps of patients who were previously irradiated for tinea capitis in their childhood and of the scalps of patients who were not. METHODS: Tissue samples of excised scalp BCCs from seven previously irradiated patients (five male, two female) and seven not previously irradiated patients (six male, one female) were frozen upon excision and genetically analyzed using DNA microarray chips. RESULTS: No correlation was found between previous ionizing irradiation and gene expression. CONCLUSIONS: The negative results of this study, coupled with the observation of aggressive biological behavior of BCCs in previously irradiated patients merit further attention. Other explanations for the aggressive biological behavior of radiotherapy-induced BCCs come to mind. One such explanation could be that the difference between the groups lies not in the tumor itself, but in the host, who is more susceptible to the local destruction caused by the tumor due to changes in the surrounding tissue (e.g., impaired blood supply due to radiation, structural damage in seemingly healthy skin). This hypothesis will be the focus of further research. LEVEL OF EVIDENCE II: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

3D Mohs surgery: a new concept.

Although Mohs surgery is considered a skin-sparing technique, when dealing with aggressive skin tumor that penetrates the deep tissues, the Mohs surgeon usually sacrifices uninvolved skin. We present our technique of 3D Mohs as a new concept for skin-sparing surgery. After raising a skin flap above the residual tumor, Mohs resection was performed on the deep tissues horizontally and simultaneously on the inner plan of the flap vertically. When "clear" borders were achieved, the skin flap was sutured back into place. The results show that the defect was significantly smaller, and the hair on the Mohs-treated vertical flap continue to grow, thus contributing to a more aesthetic outcome. We conclude that careful use of the 3D Mohs technique as we describe spares the healthy uninvolved skin and offers better aesthetic and functional result.

Evading a surgical pitfall: mastopexy--augmentation made simple.

Many women have a dwindled ptotic breast. The surgical solution for these two concurring problems has two separate procedures: augmentation and mastopexy. Combining these two procedure into one surgery is considered unpredictable and avoided by many physicians. This study presents a revised mastopexy-augmentation technique found to be safer and more simple, enabling these two procedures to be performed together. A retrospective review of 60 patients who underwent surgery by a single surgeon is presented. The presented method has yielded a relatively low reoperation rate of 10% and a high satisfaction rate.

Silicone nipple shields: an innovative postoperative dressing technique after nipple reconstruction.

BACKGROUND: The newly reconstructed nipple is extremely sensitive to mechanical pressure and shearing forces, which can cause flap necrosis and sloughing of the skin, eventually promoting infection. Current available dressing solutions are cumbersome, inefficient, displeasing, or otherwise not readily obtainable. METHODS: In this study, 10 patients with newly reconstructed nipples were instructed to use breastfeeding nipple shields as the sole means of nipple dressing after the reconstruction procedure. RESULTS: No complications were observed overall. Patients reported full adherence to the postoperative dressing regimen as well as ease of use, availability, low costs, and pleasing aesthetic appearance under garments. DISCUSSION: Silicone breastfeeding nipple shields offer an efficient, affable, cheap, widely available, and aesthetically pleasing form of postoperative dressing for reconstructed nipples. Their use may enhance patient compliance with the dressing regimen and lower the postoperative complication rate.

Silicone-based scar therapy: a review of the literature.

Hypertrophic and keloid scars still are among the banes of plastic surgery. In the treatment arsenal at the disposal of the plastic surgeon, topical silicone therapy usually is considered the first line of treatment or as an adjuvant to other treatment methods. Yet, knowledge concerning its mechanisms of action, clinical efficacy, and possible adverse effects is rather obscure and sometimes conflicting. This review briefly summarizes the existing literature regarding the silicone elastomer's mechanism of action on scars, the clinical trials regarding its efficacy, a description of some controversial points and contradicting evidence, and possible adverse effects of this treatment method. Topical silicone therapy probably will continue to be the preferred first-line treatment for hypertrophic scars due to its availability, price, ease of application, lack of serious adverse effects, and relative efficacy. Hopefully, future randomized clinical trials will help to clarify its exact clinical efficacy and appropriate treatment protocols to optimize treatment results.

Pigmented lesions clinic for early detection of melanoma: preliminary results.

BACKGROUND: Early detection of malignant melanoma of the skin is the most important factor in patient survival. Naked-eye diagnostic sensitivity and specificity are low. Patients with multiple nevi are at high risk to develop melanomas and the clinical follow-up of such patients is difficult, resulting in missed melanomas on the one hand and unnecessary biopsies on the other. OBJECTIVES: To describe the set-up of a special clinic aimed at early detection of melanoma and follow-up of high risk patients and preliminary results from 20 months of operation. METHODS: We established a pigmented lesions clinic based on a digital photography studio enabling documentation and comparison over time of full body photography and dermoscopy. RESULTS: In the first 20 months of work, 895 patients were seen, 206 of them for follow-up visits. A total of 29,254 photos were taken. Altogether, 236 lesions were suspicious (either clinically or dermoscopically) and the patients were advised to excise them. Seven melanomas were found in this initial examination (which did not include long-term follow-up). CONCLUSIONS: With multimode photographic cutaneous surveillance, early detection of melanoma in high risk patients has been reported. Our clinic utilizes the same techniques and diagnostic algorithm as other leading clinics throughout the world, thus enabling us to deliver better follow-up for those patients.

Burns in Israel: demographic, etiologic and clinical trends, 1997-2003.

BACKGROUND: Burns are a major public health problem, with long hospitalization stay in both intensive care units and general wards. In Israel about 5% of all hospitalized injuries are burn injuries. There are no long-term epidemiological studies on burn injuries in adults in Israel. OBJECTIVES: To identify risk factors for burn injuries and provide a starting point for the establishment of an effective prevention plan. METHODS: We analyzed the demographic, etiologic and clinical data of 5000 burn patients admitted to the five major hospitals with burn units in Israel during a 7 year period (1997-2003). Data were obtained from the records of the Israeli National Trauma Registry. The differences between various groups were evaluated using the chi-square test. RESULTS: Male gender was twice as frequent as female gender in burn patients (68.0% vs. 31.9%), and non-Jewish ethnicity was more common when considering their proportion in the total population (62.3% vs. 36.8%). Second and third-degree burns with body surface areas less than 10% constituted the largest group (around 50%). The largest age group was 0-1 years, constituting 22.2% of the cases. Inhalation injury was uncommon (1.9%). The most common etiologies were hot liquids (45.8%) and open fire (27.5%). Children less than 10 years old were burnt mainly by hot liquids while the main cause of burns for adults > 20 years old was an open flame. The majority of burns occurred at home (58%); around 15% were work related. The mean duration of hospitalization was 13.7 days (SD 17.7); 15.5% were in an intensive care unit with a mean duration of 12.1 days (SD 17.1). Surgical procedures became more common during the period of the study (from 13.4% in 1998 to 26.59% in 2002, average 19.8%). The mortality rate was 4.4%. We found a strong correlation between burn degree and total body surface area and mortality (0.25% mortality for 2nd to 3rd-degree burns with less than 10% TBSA, 5.4% for 2nd to 3rd-degree burns with 20-39% TBSA, and 96.6% for burns > 90% TBSA). The worst prognosis was for those over the age of 70 (mortality rate 35.3%) and the best prognosis was for the 0-1 year group (survival rate 99.6%). CONCLUSIONS: The groups at highest risk were children 0-1 years old, males and non-Jews (the incidence rate among non-Jews was 1.5 times higher than their share in the general population). Those with the highest mortality rate were victims of burns > 90% TBSA and patients older than 70. Most burns occurred at home.

Burnout and compassion fatigue: prevalence and associations among Israeli burn clinicians.

Acute health care environments can be stressful settings with clinicians experiencing deleterious effects of burnout and compassion fatigue affecting their mental health. Subsequently, the quality of patient care and outcomes may be threatened if clinicians experience burnout or compassion fatigue. Therefore, the aim of this descriptive, cross-sectional study was to evaluate the prevalence of burnout and compassion fatigue among burn clinicians in Israel. Fifty-five clinicians from Burns, Plastics and Reconstruction Surgery and Intensive Care completed four validated surveys to assess burnout (Maslach Burnout Inventory), depression (PRIME-MD), health-related quality of life (SF-8), and compassion fatigue (Professional Quality of Life version 5). Burn clinicians were compared with Plastics and Reconstruction Surgery and Intensive Care clinicians. This study identified a high prevalence of burnout (38.2%) among Intensive Care, Plastics and Reconstruction and Burns clinicians, with Burns clinicians having a greatly increased prevalence of burnout compared to Intensive Care clinicians (OR =24.3, P=0.017). Additional factors contributing to compassion fatigue were those without children (P=0.016), divorced (P=0.035), of a younger age (P=0.019), and a registered nurse (P=0.05). Burnout increased clinicians' risk of adverse professional and personal outcomes and correlated with less free time (P<0.001), increased risk of experiencing work-home disputes (P=0.05), increased depression (P=0.001) and decreased career satisfaction (P=0.01). Burnout was also associated with higher physical (mean difference =3.8, P<0.001) and lower mental (mean difference =-3.5, P<0.001) Quality of Life scores. Caring for burn survivors can lead to burnout, compassion fatigue, and vicarious trauma. Identifying strategies to abate these issues is essential to ensure improved clinicial environments and patient outcomes.

A Literature Review of Burns in Reconstructed Breasts After Mastectomy.

OBJECTIVE: The goal of this literature review is to review and combine case studies of accidental burns to the breast, following mastectomy and immediate breast reconstruction with autologous tissue, implants, or tissue expanders. METHODS: The authors searched PubMed and Cochrane Library and reviewed cases of burns of reconstructed breasts after mastectomy from July 1985 to May 2015. Only studies in the English language were included in their search. They also report 5 new cases of burns in patients with burns to the breast, which were either reconstructed with implants or tissue expanders at Sheba Medical Center (Ramat Gan, Israel). RESULTS: The authors found 21 publications regarding burns after breast reconstruction dating from 1985 to 2014, which equaled 59 cases of reported burns in the 21 included publications. The most common causes of burns were due to heat conduction (37/59) followed by solar radiation (19/59) and heat convection (3/59). The majority of the cases were treated by a conservative approach. The 5 new cases added were all due to thermal radiation (5/5). Two of these cases were treated conservatively (2/5), and 3 underwent surgery (3/5). CONCLUSION: The removal of thermoregulatory capabilities of the skin and the thickness of the remaining tissue in the mastectomy procedure are key to understanding the cause of burns to reconstructed breasts.

Validation of the Hebrew version of the Burn Specific Health Scale-Brief questionnaire.

BACKGROUND: The Burns Specific Health Scale-Brief (BSHS-B) questionnaire is a suitable measurement tool for the assessment of general, physical, mental, and social health aspects of the burn survivor. AIM: To translate, culturally adapt and validate the BSHS-B to Hebrew (BSHS-H), and to investigate its psychometric properties. METHODS: Eighty-six Hebrew speaking burn survivors filled out the BSHS-B and SF-36 questionnaires. Ten of them (11.63%) completed a retest. The psychometric properties of the scale were evaluated. Internal consistency, criterion validity, and construct validity were assessed using interclass correlation coefficient, Cronbach's alpha statistic, Spearman rank test, and Mann-Whitney U test respectively. RESULTS: BSHS-H Cronbach's alpha coefficient was 0.97. Test-retest interclass coefficients were between 0.81 and 0.98. BSHS-H was able to discriminate between facial burns, hand burns and burns >10% body surface area (p<0.05). BSHS-H and SF-36 were positively correlated (r(2)=0.667, p<0.01). CONCLUSIONS: BSHS-H is a reliable and valid instrument for use in the Israeli burn survivor population. The translation and cross-cultural adaptation of this disease specific scale allows future comparative international studies.

Examining national burn care policies--is the Israeli burn care alignment based on national data?

The treatment of burn victims constitutes a considerable challenge both to the clinician in regard to mundane treatment and to health systems in regard to structural organization. The state of Israel is in dire need of competent burn care capabilities for political, geographical, and demographic reasons. Israel currently has five designated burn units but no burn center. A review of the recent literature suggests that larger burn centers can convey lower mortality rates and better functional outcomes for severe burn patients in comparison to smaller burn units. The objective of this study is to assess Israel's burn care alignment needs and capabilities based on Israel's burn patient and burn unit data. In addition, the authors aim to compare the burn care alignment capabilities with those of the country's European and American counterparts. Data of all the burn patients hospitalized in Israel's level 1 trauma centers' burn units between the years 1998 and 2005 according to the Israeli Trauma Registry were analyzed. Simultaneously, data regarding the setup and arrangement of each burn unit were obtained from each burn unit director via phone. Between the years 1998 and 2005, 974 adult patients with burns of the second degree or higher spanning 20% TBSA and more were hospitalized in the five hospitals that operate a functional specialized burn unit. The average hospitalization period was 32.4 days while the mortality rate was 21.1%. Currently, Israel's five burn units report possessing 27 burn beds and 14 burn intensive care unit beds. Due to the continuous risk for terror attacks and military campaigns and due to Israel's inability to refer excess burn patients to neighboring countries, Israel desperately needs efficient burn care capabilities. Israel currently trails both the United States and Europe in regard to burn beds and burn centers per population. The annual quantity and severity of burn patients in Israel largely exceeds the amount needed to justify an establishment of a burn center by the current American Burn Association guidelines, while the literature provides vast amount of evidence proving burn centers' efficacy in improving outcome, shortening hospitalization periods, and reducing costs. Taking all these elements into consideration, it might be prudent to establish a national burn center in Israel to promote burn care standards and disaster planning up to international standards.

Third generation cellular multimedia teleconsultations in plastic surgery.

We conducted a study to test whether new third generation (3G) mobile phones could be integrated into service as a working tool between plastic surgeons. During an eight-month period, 58 multimedia consultations were performed involving 57 patients. The majority of the consultations were for trauma or wounds. All consultations comprised a digital photograph taken with the integrated camera and sent via the Multimedia Messaging Service (MMS). In 86% of the cases the residents reported that multimedia information contributed to their ability to independently handle similar cases in future. Satisfaction scores were high among all participants. We believe that a multimedia consultation in a hospital setting adds information to an ordinary telephone call, thus decreasing medico-legal risks. We recommend it for routine use.

Color flow mapping: a non-invasive tool for characterizing and differentiating between uveal melanomas and choroidal metastases.

The objective of this study was to characterize and differentiate vascular patterns of choroidal melanomas and choroidal metastases by color flow mapping (CFM). We conducted a retrospective chart study on CFM findings in 18 patients with choroidal melanomas and 10 patients with choroidal metastases. We evaluated the possibilities of applying CFM to identify hypo- versus hyper-vascularity within the tumor, a central 'dominant vessel', and Doppler signals in the tumor's center and/or periphery. CFM demonstrated hypervascular patterns in 33% melanomas and 100% metastases (p<0.0001). CFM identified a central dominant vessel in 94% melanomas and 0/10 metastases (p<0.0001). Vascularity occupied the center of 11/18 melanomas and 0/10 metastases (p<0.0001), the periphery of 2/18 melanomas and 9/10 metastases (p<0.0001), and equally occupied the tumor's center and periphery of 5/18 melanomas and 1/10 metastases (p<0.0001). Based on our findings, we conclude that CFM was capable of non-invasively demonstrating different and distinct vascular patterns in malignant choroidal melanomas and choroidal metastases.