Postzygotic mutations of the PIK3CA gene constitutively activate the PI3K/AKT/mTOR pathway in patients with PIK3CA-related overgrowth spectrum (PROS), causing congenital mosaic tissue overgrowth. We established primary fibroblast cells from a patient with a novel somatic frameshift mutation (c.3190_3191insA, [p.H1065fs]) in PIK3CA, in which PI3K/AKT/mTOR signaling is activated compared to control fibroblasts. We assessed the therapeutic effects of three compounds (BYL719, ARQ092, and rapamycin) on the PI3K/AKT/mTOR signaling pathway and cell growth. Notably, BYL719 is more effective at inhibiting the overactivation of all key signaling molecules in the pathway at lower concentrations in patient-derived fibroblasts, while showing no significant effect on control fibroblasts. The insertion frameshift mutation is not located within the five domains, but it is a gain-of-function PIK3CA mutation contributing to PROS development. The results further confirmed the obvious advantages of the compound in targeted therapy for PROS patients.

Introduction This study aims to summarize an algorithm for flap selection in the repair of deep lower extremity defects with bone, tendon, or joint cavity exposure. Methods The medical records of patients with deep lower extremity defects repaired using axial pattern flaps between January 2007 and December 2023 in our department were retrospectively reviewed. A three-step algorithm for axial pattern flap selection was formulated. Outcomes were evaluated based on flap survival rate, functional recovery of the affected limb, complications, and patient satisfaction. Results Ninety-six patients with a mean age of 51.2 y (range: 11-77 y) were included. The three-step algorithm involved sequential evaluation of the defect location, vascular condition of potential flaps and the size of the defect. Patients were followed up for 6 mo to 2 y. The flap survival rate was 100%, with no severe complications observed. The mean Lower Extremity Functional Scale score was 65.6 (range: 46-75). Overall, 85.4% of patients were satisfied with the outcomes. Conclusions The three-step algorithm for axial pattern flap selection—based on defect location and size, and vascular condition of potential flaps—can achieve satisfactory long-term outcomes and serves as a practical and straightforward method in clinical practice.

BACKGROUND:A successful surgery is guaranteed by the preoperative planning lineation. Existing line drawing materials often fade or vanish after sterilization. To avoid the disappearance and movement of the preoperative drawing line, the authors looked for an ideal marking material-tattoo stickers that could retain stability and precision following disinfection. METHODS:Four groups-Methylene Blue, Tattoo Sticker, Black Whiteboard Pen, and Red Marker Pen-were created for a surgical line drawing model. The 4 materials were used to make straight lines on the skin. After drying, the Black Whiteboard Pen and Red Marker Pen lines were fixed with iodine tincture. Subsequently, the marked area was sterilized thrice with iodine tincture to observe the residual rate of the marked lines. ImageJ software was used to analyze residual rates among groups. The experiments were repeated 10 times for each group. RESULTS:The residual rate was significantly higher in the Tattoo Sticker group than other groups. The differences between Methylene Blue and Tattoo Sticker groups, Black Whiteboard Pen and Tattoo Sticker groups, and Red Marker Pen and Tattoo Sticker groups were statistically significant (P<0.001). CONCLUSIONS:Tattoo stickers are ideal surgical line-drawing materials with good resistance to dissolution and stability, remaining visible even after surgical disinfection.

Modified radical mastectomy is the mainstream surgical treatment for breast cancer in which nipple-areolar complex and fusiform skin may be excised due to cancer invasion. Latissimus Dorsi (LD) flap combined with prosthesis implantation is a common method of immediate breast reconstruction after modified radical mastectomy. In this study, we introduced the design and transfer of flap based on the mastectomy incision for better practicality and appearance. From January 2016 to December 2022, a retrospective analysis was performed in patients who received immediate breast reconstruction with LD flap and prosthesis implantation after modified radical mastectomy. The design of LD flap and its transfer were based on the incision of mastectomy and divided into two types: (1) Type I, transverse flap + rotational transfer—the incision of mastectomy was horizontal or from lower inner quadrant to upper outer quadrant, the skin paddle of LD flap was transversely oriented, and the flap was transpositioned with a 180 degree rotation of the skin paddle to cover the defect; (2) Type II, transverse flap + translational transfer—the incision of mastectomy was from upper inner quadrant to lower outer quadrant, the skin paddle of LD flap was transversely oriented, and the flap was transpositioned to the defect without rotation of the skin paddle. The length of the pedicle was adjusted to fit the flap transfer. The demographics, surgical technique, satisfaction score and complications were summarized and analyzed. 31 cases were included in the study, all being female patients with a mean age of 37.6 years. Patients were followed up for 6 to 48 months (mean, 21.2 months). Type I surgery was performed in 18 patients and type II in 13 patients. The average volume of the breast implant inserted was 195.16 ml. All flaps survived well. Seroma in donor site occurred in 5 of 18 type I patients and 3 of 13 type II patients, respectively. Mastectomy skin flap necrosis occurred in 1 type I patient and 1 type II patient. 4 patients reported shoulder weakness but recovered after rehabilitation training. In terms of satisfaction scores, the values of type I and type II group were 7.4 ± 1.4 and 8.5 ± 1.5, respectively, without statistical difference (p = 0.180). Design and transfer of LD flap based on the mastectomy incision in immediate breast reconstruction with LD flap and prosthesis implantation facilitate surgical procedure and can achieve satisfactory results. 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 .

Purpose The objective of this study is to offer a thorough overview of the clinical and pathological characteristics, potential pathogenic genes, treatment modalities, and prognoses of a cohort of Chinese individuals diagnosed with Fibro-adipose Vascular Anomaly (FAVA). This study aims to advance the comprehension of this specific medical condition. Methods A total of 15 cases of FAVA patients admitted to our center between June 2015 and December 2023 were included in this study. Clinical symptoms, laboratory tests, imaging findings, and pathological data were systematically reviewed to outline the clinical and pathological features of FAVA. Furthermore, whole-exome sequencing was conducted on lesion samples from five patients to identify potential pathogenic genes. The treatment modalities encompassed sclerotherapy and surgical interventions, with treatment outcomes assessed based on clinical symptoms, imaging characteristics, and enhancement of limb functions. Results The FAVA lesions were predominantly located in the limbs in 14 patients and in the trunk in 1 patient. The clinical manifestations included pain (9/15, 60%), muscle contracture deformity (3/15, 20%), and joint dysfunction (3/15, 20%). Coagulation function assessments indicated that 3 patients exhibited localized intravascular coagulation (LIC). Magnetic resonance imaging (MRI) findings displayed mixed vascular malformation components and fibrofatty components within the muscles, showing isointense or hyperintense signals on T1-weighted images and heterogeneous hyperintense signals on T2-weighted images. Histopathological analysis revealed activation of the PI3K-AKT-mTOR signaling pathway. Whole-exome gene sequencing of 5 cases identified 10 genes potentially linked to pathogenicity. All patients underwent ethanol sclerotherapy. Surgical intervention was performed on five patients due to muscle contracture and joint dysfunction. Some patients reported pain relief following sclerotherapy, whereas those who underwent surgery demonstrated significant enhancement of joint function. Conclusions The Chinese patients diagnosed with FAVA in this study presented characteristic clinical symptoms, imaging features, and pathological traits. The lesion tissue showed activation of the PI3K-AKT-mTOR signaling pathway, with only one patient (1/5, 20%) exhibiting a PIK3CA mutation. This finding underscores the intricate nature of the pathogenic genes and pathogenesis of FAVA. Although sclerotherapy demonstrated restricted efficacy in alleviating pain symptoms, surgical intervention was deemed necessary for addressing joint dysfunction resulting from muscle contracture.

BACKGROUND:Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue malignancy with aggressive growth. OBJECTIVE:To summarize the surgical treatment and results of DFSP based on a case series and literature review. METHODS:Seventeen DFSP cases were analyzed retrospectively at our hospital from June 2016 to December 2022, including 14 males and 3 females. All these cases were treated by wide local excision with negative margins. Postoperative follow-up was performed to observe recurrence, survival, and the effect of wound repair. RESULTS:Routine postoperative pathology confirmed the morphological subtypes of these cases, including 12 cases of classical DFSP, 3 cases of DFSP-fibrosarcoma, 1 case of myxoid, and 1 case of Bednar. The median defect size was 72 cm2 (interquartile range 20-750 cm2). All patients required split skin (47.1%) or local flap (52.9%) to reconstruct the soft tissue defect caused by tumor resection. All split-skin grafts were viable, and one flap case had marginal necrosis, which healed by dressing change. After 6-32 months of follow-up, local recurrence occurred in 1 case, and extensive intra-abdominal metastasis and death occurred in another case. CONCLUSION:Wide local excision with negative margins can achieve a favorable prognosis in classical DFSP. The use of flaps for reconstruction results in better appearance and function.

BACKGROUND AND OBJECTIVE:Arteriovenous malformations (AVMs) in soft tissues are uncommon congenital vascular malformations, which are challenging to treat due to the high flow, risk of uncontrollable bleeding, and infiltrative growth. Surgical resection and interventional embolization are the main treatment for AVMs. The authors aim to summarize an individualized treatment strategy for treating AVMs in soft tissues to achieve the optimal outcome. METHODS:The medical records of patients with soft tissue AVMs who were treated in our center from January of 2006 to December of 2023 were reviewed retrospectively. Treatment included surgical resection, interventional embolization, combinational therapy, bleomycin A5 injection, and copper wire retention. The treatment option for each patient was based on the Schobinger classification, the location, depth, and size of the lesion. The patients were followed up for 3 months to 5 years. The indications, precautions, complications, and outcomes of above treatment were analyzed, and the treatment strategy was hence summarized. RESULTS:A total of 68 patients were included in the study, including 37 male patients and 31 female patients with a mean age of 24.7 years (range: 3-62). Patients were followed up for 3 months to 5 years. Nine Schobinger stage I, 43 stage II, 14 stage III, and 2 stage IV case were included. Lesions consisted of 5 Yakes type I, 33 type II, 11 type III, and 16 type IV. Three patients could not be identified with Yakes classification due to lack of arteriographic data. Surgery alone was performed in 35 cases, including simple surgical resection with primary closure/local flap transfer (n=18), surgical resection with expanded flap reconstruction (n=7), and surgical resection followed by skin grafting (n=10). Interventional embolization alone was performed in 11 cases, including 7 cases through an intra-arterial approach and 4 cases through a direct puncture of the nidus. Ten cases underwent combinational treatment which consisted of a preoperative embolization and a subsequent surgery within 48 hours. Five cases underwent bleomycin A5 injection and 7 cases underwent copper wire retention therapy. Control (42, 61.8%) and improvement (13, 19.1%) was achieved in 68 patients (55, 80.9%). During the follow-up, 6 patients reported recurrence and received another surgery. Complications of surgical treatment included partial flap necrosis (n=5) and incision dehiscence (n=3) which healed after dress changing. No skin necrosis or ectopic embolism occurred after embolization. Sixty-six patients were very satisfied with the appearance and 2 patients were basically satisfied with the appearance. CONCLUSIONS:The individualized therapy considering Schobinger classification, site, depth, and size of AVMs can achieve satisfactory results. Surgery and embolization are still the mainstay treatment methods for arteriovenous malformations.

PURPOSE:The objective of this study is to offer a thorough overview of the clinical and pathological characteristics, potential pathogenic genes, treatment modalities, and prognoses of a cohort of Chinese individuals diagnosed with Fibro-adipose Vascular Anomaly (FAVA). This study aims to advance the comprehension of this specific medical condition. METHODS:A total of 15 cases of FAVA patients admitted to our center between June 2015 and December 2023 were included in this study. Clinical symptoms, laboratory tests, imaging findings, and pathological data were systematically reviewed to outline the clinical and pathological features of FAVA. Furthermore, whole-exome sequencing was conducted on lesion samples from five patients to identify potential pathogenic genes. The treatment modalities encompassed sclerotherapy and surgical interventions, with treatment outcomes assessed based on clinical symptoms, imaging characteristics, and enhancement of limb functions. RESULTS:The FAVA lesions were predominantly located in the limbs in 14 patients and in the trunk in 1 patient. The clinical manifestations included pain (9/15, 60%), muscle contracture deformity (3/15, 20%), and joint dysfunction (3/15, 20%). Coagulation function assessments indicated that 3 patients exhibited localized intravascular coagulation (LIC). Magnetic resonance imaging (MRI) findings displayed mixed vascular malformation components and fibrofatty components within the muscles, showing isointense or hyperintense signals on T1-weighted images and heterogeneous hyperintense signals on T2-weighted images. Histopathological analysis revealed activation of the PI3K-AKT-mTOR signaling pathway. Whole-exome gene sequencing of 5 cases identified 10 genes potentially linked to pathogenicity. All patients underwent ethanol sclerotherapy. Surgical intervention was performed on five patients due to muscle contracture and joint dysfunction. Some patients reported pain relief following sclerotherapy, whereas those who underwent surgery demonstrated significant enhancement of joint function. CONCLUSIONS:The Chinese patients diagnosed with FAVA in this study presented characteristic clinical symptoms, imaging features, and pathological traits. The lesion tissue showed activation of the PI3K-AKT-mTOR signaling pathway, with only one patient (1/5, 20%) exhibiting a PIK3CA mutation. This finding underscores the intricate nature of the pathogenic genes and pathogenesis of FAVA. Although sclerotherapy demonstrated restricted efficacy in alleviating pain symptoms, surgical intervention was deemed necessary for addressing joint dysfunction resulting from muscle contracture.

The peroneal artery perforator flap is widely used to repair deep defects in the distal lower leg and ankle. However, the success of flap transplantation depends on the accurate location of the peroneal artery perforators, which can be a challenge due to potential vascular damage and anatomical variations. This study utilizes digital subtraction angiography and high-frequency ultrasound to clarify the anatomical features of the peroneal artery and its perforators and accurately locate these perforators, thereby improving preoperative design and clinical outcomes. Peroneal artery perforator sequential flaps were employed to repair the wounds and donor sites, with the second donor site sutured directly. A total of 36 peroneal artery perforators were identified in seven patients, with an average of 5.14 perforators per patient. The majority of these perforators (47.22%) were concentrated in the middle segment of the lower leg. All flaps underwent tension-free primary closure and survived successfully, presenting a smooth appearance, a fine texture, and a color similar to that of the surrounding skin. Only a linear scar was left in the secondary donor site, which did not affect the overall appearance of the limb. This technique can accurately localize peroneal artery perforators, optimize the design of peroneal artery perforator sequential flaps, and facilitate the success of the surgery and postoperative esthetic recovery.

Achieving rapid hemostasis, infection control, and tissue regeneration remains a central challenge in acute wound management, particularly under emergency and battlefield conditions. Current hemostatic materials often suffer from delayed coagulation, low bioactivity, and potential immunogenicity, limiting their use in critical care. Here, we present a chemically engineered charged silk-based hydrogel (SAMA) that integrates ultrafast gelation, intrinsic antibacterial activity, and programmable biological function. SAMA is synthesized via dual side-chain modification of silk fibroin with methacrylate (MA) for photo-crosslinking and dimethylamino groups (DMA) for surface charge tuning. The hydrogels enable photo-triggered crosslinking within 8 s under 405 nm light, while offering programmable surface charge and aqueous stability. The positively charged SAMA-5.0(+) hydrogel markedly enhances coagulation and platelet activation, as evidenced by reduced blood loss, shortened clotting time, and elevated CD62P+/PAC-1+ platelet populations. The hydrogels exhibit over 95 % antibacterial efficacy against Staphylococcus aureus and Escherichia coli, while maintaining excellent cytocompatibility, hemocompatibility, and promoting favorable responses in endothelial and immune cells. Mechanistically, SAMA(+) induces macrophage polarization toward the anti-inflammatory M2 phenotype, suppressing IL-6-mediated inflammation and facilitating angiogenesis and collagen remodeling. Controlled release assays demonstrate first-order kinetics for both the model protein (BSA) and ropivacaine, enabling sustained and timely delivery under neutral and mildly acidic wound environments. These findings position charged SAMA hydrogels as a clinically translatable solution for acute wound care, integrating rapid sealing, antimicrobial protection, immune regulation, and tissue repair into a low-cost platform suitable for wound and emergency applications.

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies (CLOVES) constitute a rare overgrowth disorder resulting from a mosaic function-acquiring mutation in the PIK3CA gene. Targeted drugs for the PI3K-AKT signaling pathway remain under clinical trial and surgery is commonly used to meet both aesthetic and functional requirements for CLOVES patients. We report here the course and experience of a male patient treated at our institution for up to 13 years. The course of treatment consisted of nine anhydrous ethanol sclerotherapy procedures and two segmental trunk mass resections. After undergoing sequential treatment, the patient experienced improved thoracic deformity and scoliosis, enabling him to grow and develop normally.

Background: Burns and chronic ulcers may cause severe skin loss, leading to critical health issues like shock, infection, sepsis, and multiple organ failure. Effective healing of full-thickness wounds may be challenging, with traditional methods facing limitations due to tissue shortage, infection, and lack of structural support. Methods: This study explored the combined use of gene transfection and dermal substitutes to improve wound healing. We used the DGTM (genes: DNP63A, GRHL2, TFAP2A, and MYC) factors to transfect adipose-derived stem cells (ADSCs), inducing their differentiation into keratinocytes. These transfected ADSCs were then incorporated into Pelnac® dermal substitutes to enhance vascularization and cellular proliferation for better healing outcomes. Results: Gene transfer using DGTM factors successfully induced keratinocyte differentiation in ADSCs. The application of these differentiated cells with Pelnac® dermal substitute to dermal wounds in mice resulted in the formation of skin tissue with a normal epidermal layer and proper collagen organization. This method alleviates the tediousness of the multiple transfection steps in previous protocols and the safety issues caused by using viral transfection reagents directly on the wound. Additionally, the inclusion of dermal substitutes addressed the lack of collagen and elastic fibers, promoting the formation of tissue resembling healthy skin rather than scar tissue. Conclusion: Integrating DGTM factor-transfected ADSCs with dermal substitutes represents a novel strategy for enhancing the healing of full-thickness wounds. Further research and clinical trials are warranted to optimize and validate this innovative approach for broader clinical applications.

Background: It is necessary to precisely locate the branches for better result of flap transplantation for the high variability of perforating branches of posterior tibial artery (PTA). In the research, digital subtraction angiography (DSA) and high-frequency ultrasound are jointly used to study the distribution and exact location of PTA perforating branches to guide clinical practice. Methods: From January 2020 to December 2022, 16 patients underwent DSA examination and 5 of them accepted further high-frequency ultrasound examination. The authors analyzed the distribution, number, location, direction, and lengths of PTA branches and used PTA perforator flaps to repair middle and inferior lower leg defects according to the above imaging findings. The donor site was repaired with skin grafts or a sequential PTA perforator flap. Results: A total of 81 branches of PTA were identified in 16 patients. Most of the perforating branches were in the middle and distal thirds of tibia. There was a significant correlation between the length of perforating branches and their perforating sites. Cluster analysis showed that larger branches appeared most frequently in the range of 15 to 20 cm from the tip of the inner ankle. 5 cases of PTA perforator flaps survived well. Conclusion: DSA can clearly show the distribution of PTA and its branches, especially the dominant ones. The imaging findings can effectively guide PTA perforator flap design and harvesting, and thus improve the result of flap transplantation.

Fibro-adipose vascular anomaly (FAVA) is a rare and complex vascular malformation associated with persistent pain, limb contracture, and even restriction of activity. However, the pathophysiology of FAVA remains unclear. Although FAVA is a benign vascular malformation, it is highly misdiagnosed and often thus undergoing repeated surgical resection and interventional sclerotherapy, resulting in worsening of symptoms and irreversible dysfunction. Therefore, aggressive diagnosis and treatment are essential. There are several different treatment options for FAVA, including surgical resection, sclerotherapy, cryoablation, drug therapy, and physical therapy. This article reviews the clinical manifestations, pathological features, pathogenesis, and treatment methods of FAVA.

OBJECTIVE:The thoracodorsal artery perforator flap has not been widely used in clinical practice partly due to a lack of imaging evidence. The authors aim to investigate the types of thoracic dorsal artery perforators through angiography and provide our experiences in the utilization of thoracic dorsal artery perforator flaps to repair adjacent wounds. METHODS:This study was divided into two parts. The first part was an angiography of the thoracodorsal artery in 12 patients based on DSA and CTA images from January 2015 to December 2023. The branches and perforators of the thoracodorsal artery were observed and classified. The second part study, between January 2019 and December 2023, illustrated the clinical application of thoracodorsal artery perforator flap in repairing neighboring wounds in 10 cases where a small area of muscular vascular pedicle was preserved with oblique branch of thoracodorsal artery trunk dissected to extend the pedicle. The donor area of the flap was closed primarily. The patients were followed up to evaluate the functional recovery of the shoulder joint after operation. RESULTS:The perforators of thoracodorsal artery were classified into 2 types based on the number and diameter: type I, the dominant perforating type (1-2 dominant perforators plus varying numbers of tiny perforators); and type II, the capillary perforating type (a larger number of tiny perforators without dominant perforators). The diameter of the dominant perforator is >0.5 mm, while the diameter of the capillary perforators is <0.5 mm. In the first part of the study, there were 8 cases of dominant perforating type and 4 cases of capillary perforating type. In the second part of the study, 10 cases of thoracodorsal artery perforator flaps were applied to repair the chest wall (3 cases), the back (1 case), the axilla (2 cases), the shoulder (2 cases) and the upper arm (2 cases). All the flaps fully survived with no complications, such as hematoma and seroma in the donor area. The function of the shoulder joint was not affected in these patients. CONCLUSIONS:The angiography showed that thoracodorsal artery perforators could be categorized into 2 types, namely the dominant perforators and capillary perforators. The thoracodorsal artery perforator flap with a small area of muscular vascular pedicle can be harvested with blood supply from both types of perforators. With reliable blood supply, flexible selection of tissue volume and minimal muscle injury, it becomes a good choice for repairing wounds on the thoracic-dorsal-shoulder area.

The reconstruction of defects in the lower extremity with bone or tendon exposure presents a significant challenge in clinical practice. Among the various surgical options available, the peroneal artery perforator flap (PAPF) stands out as a superior choice for repairing such defects. However, due to potential damage and variations in the peroneal artery’s branches and perforators, identifying these anatomic features accurately is crucial for optimizing flap design and transplantation. This study reports the successful experience of reconstructing soft tissue defects of the lower extremity using PAPF. Preoperative digital subtraction angiography (DSA) was used to visualize the peroneal artery and its branches. High-frequency ultrasound was also employed to accurately locate perforating branches. This approach enables visualization, customization, and precision in flap surgery, assisting in flap design and transplantation.

BACKGROUND:Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia and consumptive coagulopathy associated with vascular tumors, such as Kaposiform hemangioendothelioma (KHE). The pathogenesis of KMP remains unclear and its treatment is challenging. In this study, we tried to establish an animal model of KMP, which may facilitate the research on the etiology and new treatment. METHODS:A fresh sample of KHE from a one-month-old female infant with KMP was scissored into pieces and transplanted subcutaneously into the back of the nude mice. Blood routine examination was performed before the transplantation and 2, 4, 8, 12, and 16 weeks after the transplantation. Transplanted tumors were harvested 2, 4, 8, 12, and 16 weeks after the transplantation. H-E staining, immunohistochemistry staining of cluster of differentiation 31 (CD31) and alpha-smooth muscle actin (α-SMA), and ultrastructural observation were performed on the plugs. RESULTS:Blood test showed a significant decrease in the number of platelets 2 weeks after transplantation. The number of platelets showed an overall trend of recovery from 2 weeks despite a slight decrease at 12 weeks after transplantation. There was no significant difference in the platelet count at 16 weeks after transplantation compared with the original state. H-E staining showed abundant irregular blood sinuses in the transplanted tumors with plenty of blood cells 2 weeks after the transplantation. 4, 8, and 12 weeks after transplantation, the density of blood sinuses decreased progressively. 16 weeks after transplantation, the plugs involuted into fibrous tissue. Immunohistochemistry staining showed the positive expression of CD31 in the endothelial cells and α-SMA in the perivascular cells. Ultrastructural observation also showed the features of KHE and progressive evolution of the tumors. CONCLUSIONS:We successfully established an experimental model of KMP by the xenograft of KHE in nude mice, which manifested profound thrombocytopenia and typical pathological structure.

Telocytes (TCs) are a distinctive cell entity of the stromal microenvironment of multiple tumors; to date, their existence in infantile hemangioma (IH) remains almost unexplored. This study was therefore undertaken to characterize the immunophenotype, location, morphology, and ultrastructure of telocytes in the IH by means of immunohistochemistry, immunofluorescence confocal microscopy, and transmission electron microscopy. Telocytes were initially identified by CD34, PDGFR-alpha, Vimentin, and AQP-1 immunostaining. Analyzing the spatial relationship among telocytes, stem cells, endothelial cells, pericytes in the IH with AQP-1/CD31, AQP-1/Glut-1, AQP-1/alpha-SMA, AQP-1/CD146 and AQP-1/CD133 double immunofluorescence. TCs were immunonegative for CD31, Glut-1, CD146, alpha-SMA, CD133, and C-kit in the IH. The ultrastructural examination confirmed the presence of TCs, namely stromal cells with characteristic cytoplasmic processes (i.e. telopodes) forming labyrinthine networks around microvessels and releasing extracellular vesicles. Our study provides evidence that telocytes are present and PDGFR-alpha and AQP-1 are specific antigenic markers in the IH.

Objective Localized intravascular coagulation (LIC) is a unique phenomenon associated with venous malformations (VMs). This study investigated the changes in LIC related indexes following sclerotherapy of VMs and its correlation with therapeutic efficacy. Methods From August 2019 to September 2023, patients who met the following criteria were enrolled into this study: (1) individuals with venous malformations accompanied by LIC; (2) those who had undergone a minimum of three sclerotherapy sessions; (3) blood coagulation function tests were conducted prior to each treatment; and (4) magnetic resonance imaging (MRI) scans were conducted both before and after three times of treatment. Those who fail to meet with the previous inclusion criteria were excluded. The coagulation profile prior to each sclerotherapy treatments was assessed. The therapeutic outcomes were evaluated at the end of treatment. Results A total of 21 patients were enrolled into the study, with the age ranging from 4 to 61 years old. Elevated D-dimer levels, with or without a decrease in fibrinogen (FIB) and an increase in fibrin degradation products (FDP) are clinical features of VMs with LIC. The coagulation indexes tend to settle down with several sclerotherapy treatments. There were 5 cases in the significantly improved group, 13 cases in the improved group, and 3 cases in the no improved group when it came to efficacy assessment. The better therapeutic efficacy is more pronounced the improvement of LIC. Conclusions LIC gradually returns to normal during sclerotherapy, and the degree of recovery is directly related to the treatment’s efficacy. One useful metric to evaluate the effectiveness of sclerotherapy for VMs is the improvement of LIC.

BackgroundIntramuscular venous malformations (IMVMs) can cause pain and contracture deformity, leading to dysfunction of limbs. Ethanol sclerotherapy is one of the main treatments for IMVMs. This study aims to evaluate the efficacy and the complications associated with intravascular ethanol sclerotherapy for IMVMs and to provide a comprehensive summary of clinical experiences for future reference.MethodsA retrospective analysis was conducted on a cohort of 118 patients diagnosed with IMVMs who were treated with ethanol sclerotherapy in our center between 2006 and 2021. The plastic surgeons utilized a standardized collection pro forma to record the clinical data. Furthermore, a follow-up period ranging from 6 months to 5 years was implemented to assess the relief of symptoms, the change of lesion size, and the recovery of functional outcomes. In addition, an analysis of long-term complications was conducted.ResultsThe clinical symptoms of the patients in this group included pain, swelling, and limited movement. On average, 5.61 mL (range 2–14 mL) of ethanol was used during the sclerotherapy procedure. The intraoperative and early postoperative complications were successfully relieved by means of timely intervention, as observed during the follow-up period. Based on the MRI results, the sizes of the lesions in 19% of the cases were significantly decreased, while a slight decrease was observed in 39% of the cases. During the follow-up period, it was found that only eight out of the 118 patients included in this study experienced long-term complications related to sclerotherapy.ConclusionsAlthough ethanol sclerotherapy has proven to be an effective first-line treatment for IMVMs, it is associated with a variety of adverse reactions and short- and long-term complications. Surgeons are required to perform operations prudently and provide timely medical intervention for postoperative complications.