Lymphatic Patterns in the Superficial Circumflex Iliac Artery Perforator Flap.

BACKGROUND: Lymphedema is a chronic condition, characterized by fluid buildup and tissue swelling and is caused by impairment of the lymphatic system. The lymph interpositional flap transfer technique, in which lymph flow is restored with a flap that includes subdermal lymphatic channels, is an option for surgical reconstruction. The superficial circumflex iliac artery perforator (SCIP) flap can be used for this purpose. This study aimed to describe and characterize the lymphatic patterns within the vascular territory of the SCIP flap. METHODS: This cross-sectional multicenter study involved 19 healthy volunteers aged ≥18 years of both sexes assessing the bilateral SCIP flap zone. Superficial lymphatic patterns were evaluated at 4-, 14-, and 24 minutes after indocyanine green (ICG) lymphography injection. Standardized procedures were implemented for all participants in both hospitals. RESULTS: The linear pattern was predominant bilaterally. The median number of lymphatic vessels and their length increased over time. Most lymphatic vessels in the SCIP flap were oriented toward the inguinal lymph node (ILN). However, the left SCIP zone lymphatic vessels were directed opposite to the ILN. CONCLUSION: The two sides SCIP zones were not significantly different. The primary direction of the bilateral lymphatic vessels was toward the ILN, although only single-side lymphatic vessels were in the opposite direction. These findings emphasize the importance of assessing lymphatic axiality and coherent lymphatic patterns prior to undertaking the SCIP as an interposition flap, to ensure effective restoration of lymphatic flow.

SurLym trial: study protocol for a multicentre pragmatic randomised controlled trial on the added value of reconstructive lymphatic surgery to decongestive lymphatic therapy for the treatment of lymphoedema.

INTRODUCTION: Lymphoedema is a chronic condition caused by lymphatic insufficiency. It leads to swelling of the limb/midline region and an increased risk of infection. Lymphoedema is often associated with mental and physical problems limiting quality of life. The first choice of treatment is a conservative treatment, consisting of exercises, skin care, lymph drainage and compression. Reconstructive lymphatic surgery is also often performed, that is, lymphovenous anastomoses, lymph node transfer or a combination. However, robust evidence on the effectiveness of reconstructive lymphatic surgery is missing. Therefore, the objective of this trial is to investigate the added value of reconstructive lymphatic surgery to the conservative treatment in patients with lymphoedema. METHODS AND ANALYSIS: A multicentre randomised controlled and pragmatic trial was started in March 2022 in three Belgian university hospitals. 90 patients with arm lymphoedema and 90 patients with leg lymphoedema will be included. All patients are randomised between conservative treatment alone (control group) or conservative treatment with reconstructive lymphatic surgery (intervention group). Assessments are performed at baseline and at 1, 3, 6, 12, 18, 24 and 36 months. The primary outcome is lymphoedema-specific quality of life at 18 months. Key secondary outcomes are limb volume and duration of wearing the compression garment at 18 months. The approach of reconstructive lymphatic surgery is based on presurgical investigations including clinical examination, lymphofluoroscopy, lymphoscintigraphy, lymph MRI or CT angiography (if needed). All patients receive conservative treatment during 36 months, which is applied by the patient's own physical therapist and by the patient self. From months 7 to 12, the hours a day of wearing the compression garment are gradually decreased. ETHICS AND DISSEMINATION: The study has been approved by the ethical committees of University Hospitals Leuven, Ghent University Hospital and CHU UCL Namur. Results will be disseminated via peer-reviewed journals and presentations. TRIAL REGISTRATION NUMBER: NCT05064176.

The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema.

Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be either caused by reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). Its main common complication is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen the lymphatic function and can be the cause of raised morbidity of the patient if not treated correctly/urgently. The term primary lymphoedema covers a group of rare conditions caused by abnormal functioning and/or development of the lymphatic system. It covers a highly heterogeneous group of conditions. An accurate diagnosis of primary lymphoedema is crucial for the implementation of an optimal treatment plan and management, as well as to reduce the risk of worsening. Patient care is diverse across Europe, and national specialised centres and networks are not available everywhere. The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) gathers the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. There are six different working groups in VASCERN, which focus on arterial diseases, hereditary haemorrhagic telangiectasia, neurovascular diseases, lymphoedema and vascular anomalies. The working group Paediatric and Primary Lymphedema (PPL WG) gathers and shares knowledge and expertise in the diagnosis and management of adults and children with primary and paediatric lymphoedema. The members of PPL WG have worked together to produce this opinion statement reflecting strategies on how to approach patients with primary and paediatric lymphoedema. The objective of this patient pathway is to improve patient care by reducing the time to diagnosis, define the best management and follow-up strategies and avoid overuse of resources. Therefore, the patient pathway describes the clinical evaluation and investigations that lead to a clinical diagnosis, the genetic testing, differential diagnosis, the management and treatment options and the patient follow up at expert and local centres. Also, the importance of the patient group participation in the PPL WG is discussed.

Preoperative breast imaging and histopathological findings in chest contouring surgery on transmen.

BACKGROUND: Chest contouring is the most common surgical procedure transmen receive. Only a few articles discuss the importance of preoperative imaging and postoperative histopathological analysis of excised breast tissue. We studied the findings of preoperative breast imaging and the results of postoperative histopathological analysis in a clinical setting. MATERIALS AND METHODS: Data from 220 patients were collected retrospectively from 2005 to 2018. Preoperative imaging modalities and their findings were recorded and classified according to the American College of Radiology Breast Imaging Reporting and Data System. The histopathological findings in breast specimens were categorized based on the World Health Organization Classification of Breast Tumors (5th edition). RESULTS: Preoperative imaging was performed in 133 (60.5%) patients. Patients in the ultrasound-only group were younger (mean age 22.8) than the other groups (mammogram (MGR) 37 years and MGR+US 35.5 years). Preoperative imaging results were normal in 131 (98.5%) patients. Two patients needed further evaluation. Histopathological results were available on 206 (93.6%) patients. The most common histopathological findings were fibrosis (67.5%), atrophy (34.3%), and chronic mastopathy (14.5%). There were no high-risk or malignant findings. CONCLUSIONS: The need for further examinations based on routine preoperative imaging was low (1.5%). Therefore, more individualized patient selection for preoperative imaging is justified. There were no high-risk or malignant findings in histopathological analysis, and the occurrence of benign findings was similar to that reported in previous studies. Despite our findings, based on current knowledge, histopathological examination of excised breast tissue can still be recommended. Therefore, future studies are needed to define clear guidelines.

Translation and validation of a Finnish version of the Facial Clinimetric Evaluation (FaCE) Scale.

BACKGROUND: The Facial Clinimetric Evaluation (FaCE) scale is a patient-reported health status instrument developed for assessing the health-related quality of life (HRQoL) in patients with facial nerve paralysis. The aim of this study was to translate and validate the FaCE scale for the Finnish-speaking population. METHODS: The FaCE scale was translated according to international guidelines. Sixty patients in an outpatient clinic prospectively completed the translated FaCE scale and generic HRQoL instrument (15D). The objective facial paralysis grading was made using the Sunnybrook and House-Brackmann scales. Repeated FaCE and 15D instruments were mailed to patients 2 weeks later. Total scores of the FaCE instrument and subscales were calculated, and floor and ceiling effects were examined. Exploratory factor analysis was made. Internal consistency, reliability, and repeatability were assessed. Convergence with 15D instrument, Sunnybrook, and House-Brackmann scales was examined. RESULTS: The total internal consistency of the FaCE scale was high (Cronbach's alpha 0.83). There were no statistically significant differences found between mean scores of the subscales in test-retest analysis (p > 0.05). Intra-class correlations coefficients were high, ranging between 0.78 and 0.92, and the correlations were statistically significant (p < 0.001). There were statistically significant correlations observed between the FaCE scale and the 15D, Sunnybrook, and House-Brackmann scores. CONCLUSION: The FaCE scale was successfully translated and validated in Finnish with good validity and reliability. We also demonstrated statistically significant correlations between the generic HRQoL15D instrument and both the Sunnybrook and House-Brackmann physician-based grading scales. The FaCE scale is now ready for use in Finnish facial paralysis patients.

Validation of the BODY-Q Chest module in Finnish trans men undergoing chest wall masculinization.

BACKGROUND AND OBJECTIVE: The aim of gender affirmation surgery is to ease gender dysphoria. In transgender men, chest wall masculinization is the most common gender affirmation surgery. The BODY-Q Chest module is currently the only instrument developed to measure health-related quality of life (HRQL) in men undergoing chest wall surgery. Linguistic validation and cultural adaption to Finnish were performed previously. The study aims to validate the BODY-Q Chest module in transgender men who have undergone surgical chest wall masculinization. METHODS: All transgender patients who underwent chest wall masculinization at Helsinki University Hospital between 2005 and 2018 were invited to the study. The BODY-Q Chest module comprises two scales-chest and nipple. Data were obtained using the BODY-Q Chest module, the 15D questionnaire, and specifically targeted items designed by the authors. The statistical analyses were conducted to exclude selection bias, evaluate validity of the instrument, and compare it to other instruments. RESULTS: Of the 220 patients invited, 123 participated in the survey (response rate 56%). Ceiling effects were observed with 18.9% and 20.5% scoring maximum points. Cronbach's alpha was 0.92 and 0.88 for the chest and nipple scales, respectively. In exploratory factor analysis, both scales loaded to one factor confirming unidimensionality. Correlation with the generic 15D questionnaire was low. CONCLUSIONS: The BODY-Q Chest module provides valid scores with sufficient consistency and reliability when measuring HRQL in transgender men undergoing chest wall masculinization. Moreover, it offers specificity that existing or generic instruments cannot provide. Ceiling effect was expected due to the postoperative status of participants.

Paediatric lymphoedema: An audit of patients seen by the paediatric and primary lymphoedema group of vascular European Reference Network (VASCERN).

Little is known about the overall prevalence of lymphoedema in children and the types of paediatric lymphoedema seen by specialist centres. Therefore, this study was aimed to provide a profile of children with primary or secondary lymphoedema seen by the expert centres of the paediatric and primary lymphoedema working group (PPL-WG) of VASCERN and to compare the profile between the different countries. A retrospective review of all children (aged up to 18 years) seen for the first time by the expert centres over one year (2019) was carried out. Lymphoedema-, patient- and genetics-related data was collected and described for the whole group and compared between the different European countries/UK. In 2019, a total of 181 new children were seen by eight expert centres. For primary lymphoedema, the phenotype was based on the St George's classification of lymphatic anomalies. The percentages diagnosed according to each category were: 7.2% for syndromic lymphoedema, 2.8% for systemic/visceral involvement, 30.4% for congenital, 35.9% for late-onset lymphoedema and 19.3% for vascular/lymphatic malformations. 4.4% had secondary lymphoedema. Nearly 10% of all children had had at least one episode of cellulitis. The median delay from onset of symptoms to being seen by an expert centre was 2.4 years. In 44.4% of the children with primary lymphoedema a genetic test was performed, of which 35.8% resulted in a molecular diagnosis. Across the different centres, there was a wide variety in distribution of the different categories of paediatric lymphoedema diagnosed and the frequency of genetic testing. In conclusion, this paper has demonstrated that there is a large delay between the onset of paediatric lymphoedema and the first visit in the expert centres and that an episode of cellulitis is a relatively common complication. Diagnostic variation across the centres may reflect different referral criteria. Access to genetic testing was limited in some centres. It is recommended that these issues are addressed in the future work of the PPL-WG to improve the referral to the expert centres and the consistency in service provision for paediatric lymphoedema in Europe.

Phase 1 Lymfactin® Study: 24-month Efficacy and Safety Results of Combined Adenoviral VEGF-C and Lymph Node Transfer Treatment for Upper Extremity Lymphedema.

BACKGROUND: Lymphedema is a common problem after breast cancer treatment. Lymfactin® is a prolymphangiogenic growth factor vector inducing the expression of human vascular endothelial growth factor C (VEGF-C). It promotes growth and repair of lymphatic vessels. METHODS: Lymfactin® was combined with microvascular lymph node transfer surgery (VLNT) to study the safety and efficacy of the treatment in breast cancer-related upper limb lymphedema (BCRL) patients. This is a continuation study with a 3 year efficacy and 5 year safety follow-up. RESULTS: Fifteen patients were recruited in the study between June 2016 and February 2018. Three patients received a lower dose (1 × 1010 viral particles (vp)), and 12 patients received a higher dose (1 × 1011 vp) of Lymfactin®, respectively. In the higher dose group, the reduction of excess arm volume was on average 46% after the 12 month follow-up, and the transport index was improved in 7/12 patients. At baseline, removal of the compression garment for 7 days resulted in significant arm swelling (105.7±161.0 ml, p=0.0253). However, at 12 months, there was less and not significant swelling after removal of the garment (84.4±143.0 ml, p=0.0682). Lymphedema Quality of Life Inventory (LQOLI or LyQLI) questionnaire showed significant and sustained improvement of quality of life. CONCLUSIONS: During 24 months' of follow-up, the results indicate that Lymfactin® is well tolerated. The most promising findings were a 46% reduction in excess arm volume and a nonsignificant volume increase after garment removal at 12 months, suggesting that there is potential for the reduction of lymphedema.

Delay in the initiation of adjuvant chemotherapy in patients with breast cancer with mastectomy with or without immediate breast reconstruction.

BACKGROUND: Patients with breast cancer undergoing mastectomy should be offered the option of immediate breast reconstruction (IBR). The aim of this retrospective study was to assess whether there is a delay in the initiation of adjuvant chemotherapy in patients undergoing mastectomy with or without IBR. METHOD: The study included patients aged 70 years or younger with clinically node-negative breast cancer who underwent unilateral mastectomy with IBR (IBR group) or mastectomy alone (no-IBR group) followed by adjuvant chemotherapy at the Helsinki University Hospital between January 2012 to July 2018. RESULTS: A total of 645 patients were included; 186 in the IBR group and 459 in the no-IBR group. Sixty-six (35.5 per cent) patients in the IBR group and 102 (22.2 per cent) patients in the no-IBR group received their first chemotherapy cycle later than 6 weeks after surgery (P < 0.001). The respective numbers for later than 8 weeks were 17 (9.1 per cent) and 14 (3.1 per cent) (P = 0.001). Among all 645 patients, postoperative complications were a significant risk factor for a delay in the initiation of chemotherapy. Sixty-seven (39.9 per cent) patients with and 101 (21.2 per cent) patients without complications had a delay in chemotherapy (P < 0.001). The delay in chemotherapy was due to complications in 39 (59.1 per cent) in the IBR group and in 28 (27.5 per cent) in the no-IBR group (P < 0.001). CONCLUSION: Patients undergoing mastectomy alone were more likely to receive adjuvant chemotherapy within 6 weeks after surgery compared with the IBR patients. IBR significantly increased the risk of postoperative complications in comparison with mastectomy alone. The complications, in turn, were a significant risk factor for delay in adjuvant chemotherapy.

Long-term Results of Microvascular Lymph Node Transfer: Correlation of Preoperative Factors and Operation Outcome.

Our objective was to analyze whether a correlation could be observed between preoperative factors and microvascular lymph node transfer outcome after long-term follow-up. METHODS: We included 67 patients in this retrospective case series. The incidence of cellulitis, the difference of arm circumference, the use of the compression garments both preoperatively and postoperatively, and subjective symptoms, such as pain, were analyzed. Volumetry and lymphoscintigraphy results were also analyzed in a subgroup of patients. We correlated preoperative factors with postoperative results. RESULTS: After 70 ± 17 months of follow-up, 42% of the patients were able to discontinue the use of compression garments. The subjective pain symptoms were reduced in 75% of the patients. The incidence of cellulitis was reduced from preoperative 0.20 ± 0.55/y to postoperative 0.02 ± 0.08/y. As a novel finding, the patients with preoperative cellulitis were more likely to continue the use of the compression garments. CONCLUSIONS: The surgery is beneficial to most studied lymphedema patients, although it is not the cure for all patients. The incidence of cellulitis was reduced, and further, the presence of preoperative cellulitis seems to affect the outcome of the operation.

Phase 1 LymfactinⓇ Study: Short-term Safety of Combined Adenoviral VEGF-C and Lymph Node Transfer Treatment for Upper Extremity Lymphedema.

OBJECTIVE: To study the safety and tolerability of LymfactinⓇ treatment combined with microvascular lymph node transfer surgery in patients with upper limb lymphedema. BACKGROUND: Upper limb lymphedema is a common clinical challenge after breast cancer surgery and/or radiotherapy. LymfactinⓇ is an adenovirus type 5-based gene therapy involving expression of human vascular endothelial growth factor C (VEGF-C) in the damaged tissue. It aims to correct deficient lymphatic flow by promoting the growth and repair of lymphatic vessels. METHODS: In Phase I, LymfactinⓇ was combined with microvascular lymph node transfer surgery to study the safety and tolerability of LymfactinⓇ and the biodistribution of the viral vector in patients with upper limb lymphedema. RESULTS: Fifteen patients with breast cancer-associated secondary lymphedema of the upper arm were recruited between December 2016 and February 2018. Three patients received a lower dose (1 × 1010) and 12 a higher dose (1 × 1011) of viral particles, respectively. No dose-limiting toxicities were observed, and the study was completed with the pre-determined maximum dose. Commonly reported adverse events during the 12-month follow-up were common cold, fever, gastroenteritis, pain in the operation area, headache, muscle ache and elevated liver enzymes. Serious adverse events consisted of two erysipelas infections in the lymphedema arm (requiring hospitalization) and one hematoma of the flap donor site. CONCLUSIONS: After 12 months' follow-up, results indicate that LymfactinⓇ is well tolerated. The study continues with a 36-months efficacy and 5 years safety follow-up of the patients. The oncological safety aspects of LymfactinⓇ will require a longer follow-up period.

The Surgical Techniques and Outcomes of Secondary Phalloplasty After Metoidioplasty in Transgender Men: An International, Multi-Center Case Series.

INTRODUCTION: Some transgender men express the wish to undergo genital gender-affirming surgery. Metoidioplasty and phalloplasty are procedures that are performed to construct a neophallus. Genital gender-affirming surgery contributes to physical well-being, but dissatisfaction with the surgical results may occur. Disadvantages of metoidioplasty are the relatively small neophallus, the inability to have penetrative sex, and often difficulty with voiding while standing. Therefore, some transgender men opt to undergo a secondary phalloplasty after metoidioplasty. Literature on secondary phalloplasty is scarce. AIM: Explore the reasons for secondary phalloplasty, describe the surgical techniques, and report on the clinical outcomes. METHODS: Transgender men who underwent secondary phalloplasty after metoidioplasty were retrospectively identified in 8 gender surgery clinics (Amsterdam, Belgrade, Bordeaux, Austin, Ghent, Helsinki, Miami, and Montreal). Preoperative consultation, patient motivation for secondary phalloplasty, surgical technique, perioperative characteristics, complications, and clinical outcomes were recorded. MAIN OUTCOME MEASURE: The main outcome measures were surgical techniques, patient motivation, and outcomes of secondary phalloplasty after metoidioplasty in transgender men. RESULTS: Eighty-three patients were identified. The median follow-up was 7.5 years (range 0.8-39). Indicated reasons to undergo secondary phalloplasty were to have a larger phallus (n = 32; 38.6%), to be able to have penetrative sexual intercourse (n = 25; 30.1%), have had metoidioplasty performed as a first step toward phalloplasty (n = 17; 20.5%), and to void while standing (n = 15; 18.1%). Each center had preferential techniques for phalloplasty. A wide variety of surgical techniques were used to perform secondary phalloplasty. Intraoperative complications (revision of microvascular anastomosis) occurred in 3 patients (5.5%) undergoing free flap phalloplasty. Total flap failure occurred in 1 patient (1.2%). Urethral fistulas occurred in 23 patients (30.3%) and strictures in 27 patients (35.6%). CLINICAL IMPLICATIONS: A secondary phalloplasty is a suitable option for patients who previously underwent metoidioplasty. STRENGTHS & LIMITATIONS: This is the first study to report on secondary phalloplasty in collaboration with 8 specialized gender clinics. The main limitation was the retrospective design. CONCLUSION: In high-volume centers specialized in gender affirming surgery, a secondary phalloplasty in transgender men can be performed after metoidioplasty with complication rates similar to primary phalloplasty. Al-Tamimi M, Pigot GL, van der Sluis WB, et al. The Surgical Techniques and Outcomes of Secondary Phalloplasty After Metoidioplasty in Transgender Men: An International, Multi-Center Case Series. J Sex Med 2019;16:1849-1859.

Facial muscle reanimation by transcutaneous electrical stimulation for peripheral facial nerve palsy.

Reanimation of paralysed facial muscles by electrical stimulation has been studied extensively in animal models, but human studies in this field are largely lacking. Twenty-four subjects with a peripheral facial nerve palsy with a median duration of three years were enrolled. We studied activations of four facial muscles with electrical stimulation using surface electrodes. In subjects whose voluntary movement was severely impaired or completely absent, the electrical stimulation produced a movement that was greater in amplitude compared with the voluntary effort in 10 out of 18 subjects in the frontalis muscle, in 5 out of 14 subjects in the zygomaticus major muscle, and in 3 out of 8 subjects in the orbicularis oris muscle. The electrical stimulation produced a stronger blink in 8 subjects out of 22 compared with their spontaneous blinks. The stimulation could produce a better movement even in cases where the muscles were clinically completely paretic, sometimes also in palsies that were several years old, provided that the muscle was not totally denervated. Restoring the function of paralysed facial muscles by electrical stimulation has potential as a therapeutic option in cases where the muscle is clinically paretic but has reinnervation.

An International Collaborative Standardizing Patient-Centered Outcome Measures in Pediatric Facial Palsy.

IMPORTANCE: Standardization of outcome measurement using a patient-centered approach in pediatric facial palsy may help aid the advancement of clinical care in this population. OBJECTIVE: To develop a standardized outcome measurement set for pediatric patients with facial palsy through an international multidisciplinary group of health care professionals, researchers, and patients and patient representatives. DESIGN, SETTING, AND PARTICIPANTS: A working group of health care experts and patient representatives (n = 21), along with external reviewers, participated in the study. Seven teleconferences were conducted over a 9-month period between December 3, 2016, and September 23, 2017, under the guidance of the International Consortium for Health Outcomes Measurement, each followed with a 2-round Delphi process to develop consensus. This process defined the scope, outcome domains, measurement tools, time points for measurements, and case-mix variables deemed essential to a standardized outcome measurement set. Each teleconference was informed by a comprehensive review of literature and through communication with patient advisory groups. Literature review of PubMed was conducted for research published between January 1, 1981, and November 30, 2016. MAIN OUTCOMES AND MEASURES: The study aim was to develop the outcomes and measures relevant to children with facial palsy as opposed to studying the effect of a particular intervention. RESULTS: The 21 members of the working group included pediatric facial palsy experts from 9 countries. The literature review identified 1628 papers, of which 395 (24.3%) were screened and 83 (5.1%) were included for qualitative evaluation. A standard set of outcome measurements was designed by the working group to allow the recording of outcomes after all forms of surgical and nonsurgical facial palsy treatments among pediatric patients of all ages. Unilateral or bilateral, congenital or acquired, permanent or temporary, and single-territory or multiterritory facial palsy can be evaluated using this standard set. Functional, appearance, psychosocial, and administrative outcomes were selected for inclusion. Clinimetric and psychometric outcome measurement tools (clinician-, patient-, and patient proxy-reported) and time points for measuring patient outcomes were established. Eighty-six independent reviews of the standard set were completed, and 34 (85%) of the 40 patients and patient representatives and 44 (96%) of the 46 health care professionals who participated in the reviews agreed that the standard set would capture the outcomes that matter most to children with facial palsy. CONCLUSIONS AND RELEVANCE: This international collaborative study produced a free standardized set of outcome measures for evaluating the quality of care provided to pediatric patients with facial palsy, allowing benchmarking of clinicians, comparison of treatment pathways, and introduction of value-based reimbursement strategies in the field of pediatric facial palsy. LEVEL OF EVIDENCE: NA.

The Helsinki Face Transplantation: Surgical aspects and 1-year outcome.

BACKGROUND: Since 2005, at least 38 facial transplantations have been performed worldwide. We herein describe the surgical technique and 1-year clinical outcome in Finland's first face transplant case. METHODS: A 34-year-old male who had a severe facial deformity following ballistic trauma in 1999 underwent facial transplantation at the Helsinki University Hospital on 8th February 2016. Three-dimensional (3D) technology was used to manufacture donor and recipient patient-specific osteotomy guides and a donor face mask. The facial transplant consisted of a Le Fort II maxilla, central mandible, lower ⅔ of the midface muscles, facial and neck skin, oral mucosa, anterior tongue and floor of mouth muscles, facial nerve (three bilateral branches), and bilateral hypoglossal and buccal nerves. RESULTS: At 1-year follow-up, there have thus far been no clinical or histological signs of rejection. The patient has a good aesthetic outcome with symmetrical restoration of the mobile central part of the face, with recovery of pain and light touch sensation to almost the entire facial skin and intraoral mucosa. Electromyography at 1 year has confirmed symmetrical muscle activity in the floor of the mouth and facial musculature, and the patient is able to produce spontaneous smile. Successful social and psychological outcome has also been observed. Postoperative complications requiring intervention included early (nasopalatinal fistula, submandibular sialocele, temporomandibular joint pain and transient type 2 diabetes) and late (intraoral wound and fungal infection, renal impairment and hypertension) complications. CONCLUSION: At 1 year, we report an overall good functional outcome in Finland's first face transplant.

Increasing amount of amyloid are associated with the severity of clinical features in hereditary gelsolin (AGel) amyloidosis.

BACKGROUND: Patients with hereditary gelsolin (AGel) amyloidosis (HGA) present with hanging skin (cutis laxa) and bilateral cranial neuropathy, and require symptomatic plastic surgery. Our clinical observation of tissue fragility prompted us to design a prospective study. METHODS: Twenty-nine patients with HGA undergoing surgery were interviewed and clinically examined. The height and thickness of skin folds in standard anatomical localizations were measured. The presence and distribution of amyloid in skin samples were analyzed using Congo red staining and immunohistochemistry using antibodies against gelsolin amyloid (AGel) subunit. RESULTS: The measured skin folds stretched more in patients with HGA (e.g. skin over olecranon, p < 0.001). The skin folds were thinner in patients with HGA (e.g. forehead skin, p < 0.001). The skin and subcutaneous fat were abnormally fragile during surgery. The total amount of AGel amyloid, and its presence in the deep layers of the skin and subcutaneous fat correlated with the measurements of skin folds, age and extent of cranial neuropathy. CONCLUSIONS: The AGel amyloid in the skin and subcutis, together with morphologic changes in the dermal stroma and skin adnexa contribute to the atrophied and fragile structure of HGA skin. This is the first study to demonstrate the correlation between AGel amyloid accumulation and clinical disease severity.

Gelsolin amyloid angiopathy causes severe disruption of the arterial wall.

Hereditary gelsolin amyloidosis (HGA) is a dominantly inherited systemic disease reported worldwide. HGA is characterized by ophthalmological, neurological, and dermatological manifestations. AGel amyloid accumulates at basal lamina of epithelial and muscle cells, thus amyloid angiopathy is encountered in nearly every organ. HGA patients have cardiovascular, hemorrhagic, and potentially vascularly induced neurological problems. To clarify pathomechanisms of AGel angiopathy, we performed histological, immunohistochemical, and electron microscopic analyses on facial temporal artery branches from 8 HGA patients and 13 control subjects. We demonstrate major pathological changes in arteries: disruption of the tunica media, disorganization of vascular smooth muscle cells, and accumulation of AGel fibrils in arterial walls, where they associate with the lamina elastica interna, which becomes fragmented and diminished. We also provide evidence of abnormal accumulation and localization of collagen types I and III and an increase of collagen type I degradation product in the tunica media. Vascular smooth muscle cells appear to be morphologically and semi-quantitatively normal, only their basal lamina is often thickened. In conclusion, angiopathy in HGA results in severe disruption of arterial walls, characterized by prominent AGel deposition, collagen derangement and severe elastolysis, and it may be responsible for several, particularly hemorrhagic, disease manifestations in HGA.

Evolution of Head and Neck Microvascular Reconstructive Strategy at an Academic Centre: An 18-Year Review.

Background A remarkable development through the evolution of free flap techniques has led to the modern reconstructive head and neck surgery. This study aimed to review experiences from head and neck free-flap reconstructions performed at our institution over an 18-year period. Methods Between 1995 and 2012, 594 free-flap operations were performed on 541 head and neck patients at the Department of Plastic Surgery, Helsinki University Hospital, Finland. We retrospectively recorded hospital chart data regarding patient demographics, tumor characteristics, surgical treatment, and outcome. Results The mean age of patients increased from 53 years (1995-2000) to 56 (2007-2012), while the gender distribution remained constant (60% males). The most commonly used flap type between 1995 and 2000 was radial forearm flap (50%), while during the periods of 2001 through 2006 and 2007 through 2012 the anterolateral thigh flap was the most common method (42 and 36%, respectively). The number of different flap types and flap combinations increased during these periods (15 flap types during 1995 through 2000; 17 flap types during 2001 through 2006; and 24 flap types during 2007 through 2012). Despite a wider range of flap options used during the period 2001 through 2012, the overall flap survival rate (97%) remained constant. The prevalence of surgical complications decreased from 26% (2001 through 2006) to 21% (2007 through 2012). Conclusions During the study period, the number of flap types increased and reconstructions became more individualized. The overall surgical complication rate decreased although the mean age of patients increased.

Progressive cranial nerve involvement and grading of facial paralysis in gelsolin amyloidosis.

INTRODUCTION: Hereditary gelsolin amyloidosis (GA) is a rare condition caused by the gelsolin gene mutation. The diagnostic triad includes corneal lattice dystrophy (type 2), progressive bilateral facial paralysis, and cutis laxa. Detailed information on facial paralysis in GA and the extent of cranial nerve injury is lacking. METHODS: 29 GA patients undergoing facial corrective surgery were interviewed, examined, and studied electroneurophysiologically. RESULTS: All showed dysfunction of facial (VII) and trigeminal (V) nerves, two-thirds of oculomotor (III) and hypoglossal (XII) nerves, and half of vestibulocochlear (acoustic) (VIII) nerve. Clinical involvement of frontal, zygomatic, and buccal facial nerve branches was seen in 97%, 83%, and 52% of patients, respectively. Electromyography showed marked motor unit potential loss in facial musculature. CONCLUSIONS: Cranial nerve involvement in GA is more widespread than previously described, and correlates with age, severity of facial paralysis, and electromyographic findings. We describe a grading method for bilateral facial paralysis in GA, which is essential for evaluation of disease progression and the need for treatment.

Sentinel lymph node biopsy in cN0 squamous cell carcinoma of the lip: A retrospective study.

BACKGROUND: The purpose of this study was to assess the use of sentinel lymph node biopsy (SLNB) in clinically lymph node-negative (cN0) squamous cell carcinoma (SCC) of the lip deemed high risk for occult nodal metastasis. METHODS: Twenty-six patients with cT1 to T2 cN0 with SCC of the lip underwent SLNB at a tertiary referral center between January 2001 and March 2012. Initial staging methods were clinical examination only (65.4%), ultrasound (23.1%), or CT (11.5%). Operations were performed with the patients under local anesthesia with sedation (50%) or general anesthesia (50%). RESULTS: The mean follow-up time was 53 months. Three patients (11.5%) had a positive sentinel node and were upstaged. One SLNB-related complication was observed. Regional recurrence occurred in 2 patients (7.7%). The relationship between regional status and both tumor diameter and tumor thickness was statistically significant (p < .05). CONCLUSION: SLNB can be a viable staging technique in SCC of the lip. Tumor diameter of ≥ 20 mm and increasing tumor thickness seem to delineate higher risk for regional disease in our study. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1375-E1380, 2016.