Common Peroneal Nerve and Tarsal Tunnel Release Surgery in an Adolescent Male with Hunter Syndrome: Illustrative Case.

BACKGROUND: Children with Hunter syndrome have a high prevalence of nerve compression syndromes given the buildup of glycosaminoglycans in the tendon sheaths and soft tissue structures. These are often comorbid with orthopedic conditions given joint and tendon contractures due to the same pathology. While carpal tunnel syndrome and surgical treatment has been well-reported in this population, the literature on lower extremity nerve compression syndromes and their treatment in Hunter syndrome is sparse. OBSERVATIONS: We report the case of a 13-year-old male with a history of Hunter syndrome who presented with toe-walking and tenderness over the peroneal and tarsal tunnel areas. He underwent bilateral common peroneal nerve and tarsal tunnel releases, with findings of severe nerve compression and hypertrophied soft tissue structures demonstrating fibromuscular scarring on pathology. Post-operatively, the patient's family reported subjective improvement in lower extremity mobility and plantar flexion. LESSONS: In this case, peroneal and tarsal nerve compression were diagnosed clinically and treated effectively with surgical release and postoperative ankle casting. Given the wide differential of common comorbid orthopedic conditions in Hunter syndrome and the lack of validated electrodiagnostic normative values in this population, the history and physical examination and consideration of nerve compression syndromes are tantamount for successful workup and treatment of gait abnormalities in the child with Hunter syndrome.

ApoE maintains neuronal integrity via microRNA and H3K27me3-mediated repression.

ApoE regulates neurogenesis, although how it influences genetic programs remains elusive. Cortical neurons induced from isogenic control and ApoE-/- human neural stem cells (NSCs) recapitulated key transcriptomic signatures of in vivo counterparts identified from single-cell human midbrain. Surprisingly, ApoE expression in NSC and neural progenitor cells (NPCs) is not required for differentiation. Instead, ApoE prevents the over-proliferation of non-neuronal cells during extended neuronal culture when it is not expressed. Elevated miR-199a-5p level in ApoE-/- cells lowers the EZH1 protein and the repressive H3K27me3 mark, a phenotype rescued by miR-199a-5p steric inhibitor. Reduced H3K27me3 at genes linked to extracellular matrix organization and angiogenesis in ApoE-/- NPC correlates with their aberrant expression and phenotypes in neurons. Interestingly, the ApoE coding sequence, which contains many predicted miR-199a-5p binding sites, can repress miR-199a-5p without translating into protein. This suggests that ApoE maintains neurons integrity through the target-directed miRNA degradation of miR-199a-5p, imparting the H3K27me3-mediated repression of non-neuronal genes during differentiation.

Ganglioneuroma of the Cervical Sympathetic Chain - A Rare Occurrence.

This article deals with a rare occurrence of Ganglioneuroma of the left cervical sympathetic chain in a 9-year-old girl, presenting with painless paracervical swelling noticed since 7 months. Imaging studies showed a left parapharyngeal mass extending from C1 to C6, displacing the posterior pharyngeal wall and anterolaterally displacing the contents of the carotid sheath, with no significant vascular feeders. A provisional diagnosis of? Neurogenic tumour was established and the patient underwent surgical excision. Histopathologically, the diagnosis of Ganglioneuroma was confirmed.

Surgical management of sternal tumours-a decade of experience from a tertiary care centre in India.

Tumours of the sternum can be either primary or secondary with malignancy being the most common etiology. Wide local excision of these tumours results in a midline defect which pose a unique challenge for reconstruction. As limited data on the management of these tumours exists in the literature, we hereby report 14 consecutive patients who were treated at our institute between January 2009 to December 2020. Most of them were malignant with majority of them, 11 (78%) patients, with manubrial involvement requiring partial sternectomy. Overall, the average defect size was 75 cm2. Reconstruction of the chest wall defect was done using a semi-rigid fixation: mesh and suture stabilization in 3 (21%) or suture stabilization in 7 (50%) and without mesh or suture stabilization in 3 (21%) patients. Rigid fixation with polymethyl methacrylate (PMMA) was done for one patient (7%). Pectoralis major advancement flap was most commonly used for soft tissue reconstruction with flap necrosis noted in one patient (7%). There was no peri-operative mortality and one patient required prolonged post-operative ventilation. On a median follow-up of 37.5 months, one patient (7%) had a recurrence. Sternal defects after surgical resection reconstructed with semi-rigid fixation and suture stabilization render acceptable post-operative outcomes.

The Impact of a Centralized Plastic Surgery Research Infrastructure on Scholarly Productivity and Output.

Although research and innovation is a key within the field of plastic and reconstructive surgery, the impact of team structure, interpersonal dynamics, and/or standardized infrastructure on scholarly output has been infrequently studied. In this work, we present the formation and implementation of a novel plastic surgery research program that aims to unite previously disparate clinical and translational research efforts at our institution to facilitate critical inquiry. From July 2022 to June 2023, our department launched a pilot research program based on three pillars: (1) formalization of a research curriculum (monthly research meetings for agenda setting and discussion for project honing, formal research leadership for meeting facilitation and workflow regulation), (2) development of a centralized database to compile ongoing research (Google Drive repository to house all ongoing research documents, facilitate real-time editing, and provide resources/templates for assisting in the research process), and (3) bolstering of a core research identity built on mentorship and collaboration (more frequent interactions to shift previously siloed faculty-student mentorship into a robust milieu of intercollaboration). During the first year, we saw an increased number of publications and presentations, as well as robust participation and contribution from faculty, residents, and medical students. Future directions will focus on addressing resource limitation, such as project idea availability and funding, to sustain the success and growth of this novel research infrastructure.

Study of pathogenic T-helper cell subsets in Asian Indian patients with Takayasu arteritis.

The relapses and refractory disease are a challenge in the management of patients with Takayasu arteritis (TAK). We quantified pathogenic CD4 + memory T helper cells bearing surface markers CD161 and/or p-glycoprotein (MDR1) in patients with TAK. Peripheral blood mononuclear cells of 21 patients with TAK and 16 age-matched controls were stained with anti-CD3, anti-CD4, anti-CD45RA, anti-CD161 and anti-p-glycoprotein antibodies and subjected to flow cytometry by FACS ARIAIII. Eighteen patients underwent follow-up immunophenotyping. Intracellular staining for interleukin-17 and interferon-γ was performed for 18 patients and 11 controls. Surgical arterial biopsies of 6 TAK and 5 non-inflammatory controls were subjected to immunohistochemistry with anti-CD161 and anti-p-glycoprotein. At baseline the frequency of MDR1 + CD4 + and CD161 + MDR1 + CD4 + memory T cells was higher in TAK than controls (p = 0.002 and 0.01, respectively). After stimulation, the frequency of IFN-y + CD161 + cells was higher in TAK than controls (p = 0.028). Modal fluorescence intensity of CD161 + MDR1 + CD45RA - CD4 + cells was higher in active as compared with stable disease (p = 0.041). At 6 months, MDR1 + and CD161 + MDR1 + memory CD4 + T cells decreased significantly only in patients who had complete/partial response to treatment (p = 0.047 and 0.02, respectively). To conclude, MDR1 + and MDR1 + CD161 + CD4 + memory T-helper cells are increased in patients with TAK. These cells decreased only in patients with response to treatment during subsequent follow-up.

Examining Racial and Gender Diversity in the Plastic Surgery Pipeline: Where is the Leak?

Background: There is limited information about minority representation throughout the plastic and reconstructive surgery (PRS) pipeline. The aim of this study was to examine trends in representation among minorities at different stages of the PRS training pathway, starting with potential candidates in high school through practicing physicians. Methods: The PRS pipeline was defined as high school; college; medical school applicants, matriculants, and graduates; PRS residency applicants, matriculants, and active residents; and PRS practicing physicians. Racial data for each stage were obtained from the US Census and Association of American Medical Colleges. The proportion of races at each stage were divided by their US population counterpart proportions to produce representation quotients (RQs). Medians and interquartile ranges (IQRs) are reported. Mann-Whitney U tests compared RQ values within identities between successive stages. Results: Black students had high representation in high school (RQ = 1.26 [IQR: 1.21-1.29]) but had significant, stepwise decreases in representation in subsequent stages. A similar trend was observed for Hispanic individuals, who had their highest representation in high school (1.43 [1.37-1.50]), followed by significant decreases in RQ at nearly every subsequent stage up to and including practicing physicians (0.30 [0.28-0.31). Asian individuals were overrepresented at every stage (high school RQ: 1.01 [1.00-1.03]; practicing physician RQ: 2.30 [2.27-2.32]). White individuals were underrepresented before residency but had an RQ that approximated 1 in subsequent stages. Conclusions: Racial minorities experienced decreases in representation at each successive stage in the PRS pipeline following high school. Ongoing diversity efforts should focus on premedical recruitment and professional support for minority students.

Alpha Defensin-1 Level Correlates with Periprosthetic Infection Severity following Implant-based Breast Reconstruction.

Background: Accurate diagnosis of periprosthetic infections following breast reconstructions is paramount to reduce morbidity. Alpha defensin-1 (AD-1) is an antimicrobial peptide released by neutrophils. This study evaluates the relationship between quantitative AD-1 levels and infection severity in patients with suspected periprosthetic infection. Methods: Retrospective review was conducted of patients with prior breast implant reconstruction undergoing surgery for either suspected infection or prosthesis exchange and revision. The AD-1 level in periprosthetic fluid was sent for quantitative analysis. Association between AD-1 levels with outcomes, management, systemic markers of infection, and overall infection severity was evaluated. Results: Thirty-eight breasts were included. Infected breasts had higher AD-1 levels (3.91 versus 0.14, P < 0.01), greater odds of erythema [odds ratio (OR) 2.98 (1.53-5.82), P = 0.01], purulence [OR 2.84 (1.51-5.35), P = 0.01], fever [OR 1.84 (1.15-2.93), P = 0.01], threatened implant exposure [OR 2.97 (1.48-5.95), P < 0.01], and true implant exposure [OR 1.79 (1.04-3.08), P = 0.04]. Increasing AD-1 was an independent risk factor for washout (P < 0.01), and explant [OR 2.48 (1.47-4.2), P < 0.01]. AD-1 positively correlated with white blood cell count (ß = 1.81 cells/µL, P < 0.01), and serum lactate (ß = 0.19 meq/L, P < 0.04). Increasing AD-1 level was an independent predictor of infection severity (χ² = 22.77, P < 0.01). Conclusions: AD-1 levels correlate with infection severity, highlighting its potential both when clinical examination is ambiguous and when treatment response is being monitored. Although further evaluation is warranted, AD-1 may demonstrate utility in novel breast implant salvage algorithms.

Perspectives of Patients With Relapsed and Refractory Acute Myeloid Leukemia and High-Risk Myelodysplastic Syndrome on Patient-Clinician Communication About Prognosis and the Future.

Background: Patients with acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (MDS) experience unpredictable disease trajectories and high prognostic uncertainty, which serve as barriers to patient-clinician communication about prognosis and their values and preferences for the future in the event of worsening health. Little is known about patients' day-to-day lived experiences and how this shapes their willingness to engage in such conversations. Objectives: To explore participant perspectives on living with their illness and patient-clinician communication about prognosis and the future. Design: This is a qualitative study using semi-structured interviews. Setting/Subjects: Patients with relapsed and refractory (R/R) AML and high-risk MDS from a northeastern U.S. cancer center. Data Collection: Interviews were transcribed verbatim and thematic analysis was used to generate findings. Results: Of the 14 participants, the mean age was 66 years, 79% were men, 93% were White, married, and had AML. The overarching theme that describes the experience was "Taking One Day at a Time" in a Fog of Uncertainty. Uncertainty was a universal perception related to the challenges for clinicians to predict prognosis. To cope with uncertainty, most participants tried to focus on the present and maintain normality in everyday life. Participants valued encouragement and positivity in patient-clinician communication, however, the majority were not ready to discuss prognosis and the future in the event of worsening health. Of note, 7 of 14 participants died within three months after the interview. Conclusions: These data describe a unique perspective of patients with R/R AML and high-risk MDS that clinicians could use to enhance communication strategies.

Single Oral Z-Plasty: A Clinical Validation of a Technique for Palatal Lengthening in Primary and Secondary Cleft Palate Repair.

BACKGROUND: In cleft palate repair, palate length is associated with improved speech outcomes. Although direct closure offers poor palatal lengthening, use of two opposing Z-plasties may reorient palatal musculature and lengthen the velum. The authors previously described a novel overlapping intravelar veloplasty to achieve longitudinal closure of the nasal mucosa with a single oral Z-plasty (1ZP), lengthening the palate in cadaver studies. This study aims to corroborate this finding in clinical cases. METHODS: A retrospective comparative study of patients with a cleft palate was conducted. Patients underwent cleft palate closure with 1ZP or intravelar veloplasty with straight-line closure. Preoperative and postoperative measurements of the palate along four dimensions were recorded. Analysis was conducted on preoperative and postoperative measurements within and between groups using the Mann-Whitney-Wilcoxon or chi-square test. RESULTS: Eighty-five patients were included (1ZP, n = 65; straight-line closure, n = 20). 1ZP increased soft palate length (SPL) by 33% ( P < 0.001) and total palate length (TPL) by 10% ( P < 0.001). Primary 1ZP increased SPL by 33% ( P < 0.001) and TPL by 10% ( P < 0.001). Secondary 1ZP increased SPL by 28% ( P < 0.001) and TPL by 8% ( P < 0.001). When comparing between primary and secondary 1ZP, 1ZP was equal with regard to percentage lengthening in SPL ( P > 0.9) and TPL ( P > 0.3). When compared with straight-line closure, 1ZP showed superior percentage lengthening in SPL ( P < 0.001) and TPL ( P = 0.038). CONCLUSIONS: 1ZP results in a statistically significant increase in palate length in both primary and secondary cleft palate repair. This technique provides an effective alternative in patients for whom 2ZP is not feasible. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Delay in prior authorization of biologic therapy: Another possible cause of healthcare disparity in IBD patients.

BACKGROUND: Biologics, a mainstay in inflammatory bowel disease (IBD) treatment, typically require prior authorization from insurance companies. Multiple studies show that African Americans are less likely to be prescribed biologics. The prior authorization process may perpetuate disparities in healthcare. This study evaluated the approval time for biologics in IBD. METHODS: A chart review of IBD patients seen in a university gastroenterology clinic over 5 years was performed. Patient gender, race, IBD subtype, biologic use, and insurance type were recorded. Insurance type was classified as private or public (Medicaid or Medicare). Biologic agents evaluated included infliximab, adalimumab, vedolizumab and ustekinumab. Length of time to approval (TTA) and length of time to first infusion or administration (TFI) were recorded. Analysis was performed using t-testing, Fisher's exact testing, and ANOVA with significance set at p<0.05. The study was IRB approved. RESULTS: 458 charts were analyzed. 66 patients were being treated with a biologic. 42 had private insurance, 16 Medicaid and 8 Medicare. 37 patients had ulcerative colitis, 27 Crohn's disease, and 2 indeterminate colitis. There were 38 men and 28 women. 32 patients were white, 26 African American, 1 Asian, 5 other, and 2 declined identification. Average TTA was 30.5 days (range 1-145) and average TFI was 45.3 days (range 2-166). African Americans were more often on public insurance compared to whites (p=0.0001). Crohn's disease compared to ulcerative colitis patients were more often on public insurance (p=0.017). Significantly more private compared to public insurance patients were on infliximab (p=0.001). Medicaid and Medicare patients had significantly longer mean TTAs than private insurance patients (49.1 and 52.7 vs 19.4 days, p=0.007). African Americans had significantly longer mean TTA compared to whites (45.9 vs 24.8 days, p=0.044). Crohn's disease compared to ulcerative colitis patients had significantly longer mean TTA (39.7 vs 21.8 days, p=0.050). DISCUSSION: This study shows that prior authorization for biologic therapy was longer for African Americans. Patients on public insurance also tend to have a longer TTA, and more African Americans were on public insurance compared to White patients in this study which may explain the difference in biologic access for African Americans.

Spontaneous Reossification Following Craniectomy in a Pediatric Patient.

Spontaneous reossification following a cranial defect is described by only a few case reports. A 6-month-old male with epidural hematoma underwent decompressive craniotomy, subsequently complicated by scalp abscess requiring removal of the bone flap. On serial outpatient follow-up, the patient demonstrated near-complete resolution of cranial defect over the course of 18 months, thus deferring the need for future cranioplasty. Prior articles have identified this occurrence in children and young adults; however, the present case is the first to report of this phenomenon in an infant less than 1 year of age. A brief review of the literature is provided with the proposed physiologic underpinning for the spontaneous reossification observed. While prior studies propose that recranialization is mediated by contact with the dura mater and pericranium, new investigations suggest that calvarial bone repair is also mediated by stem cells from the suture mesenchyme.

The Impact of Venous Reconstruction on Finger Replantation Success Rates Based on Level of Injury.

PURPOSE: The requirement for anatomic venous reconstruction in digit replantation is an ongoing area of research. In this study, we evaluated our institutional experience to study whether replantation success is affected by the presence or absence of vein repair, stratified by the level of injury. METHODS: A retrospective review was performed at an urban, level-1 trauma center of all single-digit replantations performed in adults from 2012 to 2021. Patient demographics, injury mechanism, level of injury, whether a vein was repaired, and replant survival were recorded. RESULTS: Sixty-seven single replanted digits were included. Patients were, on average, 38 years old, and 94% were men. The most common mechanism of injury was a sharp laceration (81%). The overall survival rates for all replantations were 68.7% (46/67) and 60% (12/20) for distal finger replantation. Patients with digital replantations at Tamai zone III or more proximal exhibited a 1.8 times increase in survival rates when one vein was repaired versus zero veins (84.4% vs 46.7%). Patients with digital replantations at Tamai zones I and II exhibited similar survival rates. CONCLUSIONS: Replantations at or proximal to the middle phalanx should be repaired with at least one artery and vein to maximize the chance for success. However, for distal finger replantations, artery-only replantation is a viable option when vein anastomosis is not achievable. TYPO OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

A Cost-effective, 3D Printed Simulation Model Facilitates Learning of Bilobed and Banner Flaps for Mohs Nasal Reconstruction: A Pilot Study.

SUMMARY: Flap design for Mohs reconstruction is a complex 3-dimensional decision-making process. Simulation offers trainees the chance to practice techniques safely, prior to opportunities in the operating room. To aide in teaching, we developed a high-fidelity, cost-effective model of the face using three-dimensional (3D) printing to simulate flap reconstruction following Mohs surgery. We describe the design of this model and its impact on the comfort and proficiency of trainees.

Topical Micro-Emulsion of 5-Fluorouracil by a Twin Screw Processor-Based Novel Continuous Manufacturing Process for the Treatment of Skin Cancer: Preparation and In Vitro and In Vivo Evaluations.

5-Fluorouracil (5-FU), a BCS class III drug, has low oral bioavailability and is cytotoxic in nature causing severe systemic side effects when administered through the intravenous route. Topical drug delivery could potentially mitigate the systemic side-effects. Microemulsions (MEs) would be an apt solution due to enhanced partitioning of the drug to the skin. However, conventional methods for preparing MEs are inefficient since they are not continuous and are very tedious and time-consuming processes hence revealing the need for the development of continuous manufacturing technology. In our study, 5-FU MEs were prepared using a continuous manufacturing Twin Screw Process (TSP) and its efficiency in the treatment of skin cancer was evaluated. Water-in-oil MEs were prepared using isopropyl myristate as the oil phase and Aerosol OT and Tween 80 as the surfactants. The average particle size was observed to be 178 nm. Transmission electron microscopy was employed to confirm the size and shape of the MEs. FTIR study proved no physical or chemical interaction between the excipients and the drug. In vitro drug release using vertical diffusion cells and ex vivo skin permeation studies showed that the drug was released sustainably and permeated across the skin, respectively. In in vitro cytotoxicity studies, 5-FU MEs were accessed in HaCat and A431 cell lines to determine percentage cell viability and IC50. Skin irritation and histopathological examination implied that the 5-FU MEs did not cause any significant irritation to the skin. In vivo pharmacodynamics studies in rats suggested that the optimised formulation was effective in treating squamous cell carcinoma (SCC). Therefore, 5-FU MEs efficiently overcame the various drawbacks faced during oral and intravenous drug delivery. Also, TSP proved to be a technique that overcomes the various problems associated with the conventional methods of preparing MEs.

An overview on the principles of management of haemoptysis.

Haemoptysis is a frequently encountered presentation in thoracic surgery practice. Most of the patients present with chronic haemoptysis while 5% of them will present with life-threatening acute haemoptysis. Emergency surgery used to be the first-line management in acute life-threatening haemoptysis which resulted in significant morbidity and mortality. With advancements in interventional procedures, most of these acute presentations are now being managed conservatively by interventionists. In a country like India with a high incidence of tuberculosis and other infectious diseases of the lungs, haemoptysis is even more common. While interventional procedures help to tide over the crisis and earn valuable time to stabilise a haemorrhaging patient, surgical resection is the definitive management most of the time. This review will endeavour to establish the definition, aetiology, emergency, and definitive management of a patient who presents with haemoptysis.

Primary idiopathic chylopericardium managed by uniportal VATS.

Chylopericardium is very rarely encountered in clinical practice. The common causes are post cardiac or thoracic surgery and neoplasms of the mediastinum. Most of the time, no cause is attributed and it is labelled as primary idiopathic chylopericardium. Conservative management is usually not successful and definitive surgery is required. The recommended surgery is creation of a pericardio-pleural window and thoracic duct ligation. We demonstrate that this procedure can be easily accomplished by uniportal video-assisted thoracic surgery (U-VATS).

Advance care planning, serious illness communication, and conversations to facilitate coping for patients with gastrointestinal malignancies: a narrative review.

BACKGROUND AND OBJECTIVE: Patients with advanced gastrointestinal (GI) malignancies are at high-risk for disease-related complications, treatment-related toxicity, unplanned hospitalizations, poor psychological outcomes, and short life-expectancies. Advance care planning (ACP) and serious illness communication (SIC) are two forms of communication that can help patients with GI malignancies explore the future, especially in the event of worsening health. While there are some limitations to traditional ACP, SIC that focuses on what matters most to patients with GI malignancies in the future (future-focused SIC), has the potential to improve future medical decision-making, help patients cognitively and emotionally process and accept their illness over time, help them feel heard and understood, allow them to positively cope with their disease, and may also help their caregivers in a variety of ways. METHODS: Narrative review using PubMed and Google Scholar to search for relevant literature published between 2010-2022. KEY CONTENT AND FINDINGS: We present several key studies that highlight the complex, heterogenous nature of ACP and SIC research and its mixed outcomes for patients with GI malignancies. We also offer suggestions on how to optimize future-focused SIC research in this patient population. In the second half of this article, we suggest a practical approach to conducting future-focused SIC for patients with GI malignancies which includes a communication framework based on the literature and expert-opinion. We also provide practical tips on how to normalize these conversations and how to help patients use these conversations for future medical decision-making. CONCLUSIONS: Future-focused SIC has the potential to benefit patients with advanced GI malignancies in a variety of ways. Optimizing research outcome measures that highlight the patient experience with this communication is crucial to move this area of research forward.

Lateral Canthotomy Revisited: A Refined Surgical Approach for Orbital Access.

The transconjunctival incision is a common and effective approach for establishing surgical exposure to the orbital floor. When access to the lateral orbit is also required, this incision may be extended by an accompanying lateral canthotomy, which releases the tarsal plates from the conjunctiva. Although this approach broadens surgical access through a simple extension, it is often remarked for unpredictable healing patterns and negative aesthetic sequelae, such as rounding of the lateral canthal angle. Traditionally, lateral canthotomy is performed by a transverse incision through a natural skin crease of the lateral palpebral fissure. Herein, we discuss our experience with a less common approach to lateral canthotomy, in which only the inferior crus of the lateral canthal tendon is divided. This approach limits manipulation of delicate orbital anatomy and aims to minimize unsightly scarring while still affording excellent visualization of the lateral orbit and orbital floor.