Small Case Series Research Articles

Presentation and surgical management approaches of giant presacral and sacral schwannomas: a case report and systematic review and meta-analysis.

Giant sacral and presacral schwannomas are very rare conditions and their prevalence is estimated to account for only 0.3 to 3.3% of overall schwannomas. Current published literature about these tumors is limited to case reports and small case series. In this paper we systematically reviewed and analyzed the available literature on giant sacral schwannomas focusing on identifying the presenting symptoms, Klimo type, surgical approach, recurrence rate and postoperative complications. Following the PRISMA guidelines, we conducted a literature search on electronic databases to obtain the relevant studies until March 2024. We included reports of surgically managed giant sacral schwannomas; either involving tumor of at least 2 adjacent vertebra or had a maximum diameter of 5cm or more. Presenting symptoms, tumor size, morphology, surgical approach and postoperative complications were reported. A total of 102 studies with 201 unique patients were included in our analysis (mean age 45.4 ± 9.8, 49.7% females). The most common presenting symptoms were lower extremity radicular pain (19.5%), lower back pain (14.1%), lower extremity numbness or paresthesia (9%), and constipation (7.6%). Most surgeries used an open posterior approach (40.1%) or open anterior approach (33.5%). Postoperative complications occurred in (29.3%) of patients and 10% had local recurrence or progression of their tumor. On linear regression the volume of the tumor could significantly predict the estimated amount of blood loss (P = 0.03). We found that an anterior approach was far more likely to be used in patients with Klimo type III, (P < 0.001). Similarly, posterior approach was mostly used for Klimo type I and type II (P < 0.001). The management approach of giant sacral and presacral schwannomas are mainly tailored according to the tumor size and location. Due to the benign nature and the overall recurrence rate of the tumor, frequent imaging follow-up is required after safe resection with adequate clean margins.

Clinical and radiological characteristics of calcified cerebral embolism: a large case series including 242 calcified cerebral embolism.

Radiological calcified cerebral embolism (CCE) characteristics have been reported in small case series. Our aim was to describe clinical and radiological CCE characteristics in a large number of CCE and to compare characteristics between different patient groups. Characteristics of 79 stroke patients with CCE were analyzed retrospectively. Clinical characteristics included demographics, cardiovascular risk factors, stroke history, history of surgical/endovascular cardiovascular intervention, NIHSS on admission, stroke etiology, and presumed CCE source. Radiological characteristics included CCE diameter, density, number, involved cerebral artery and segment, and CCE distribution. A total of 242 CCE were analyzed in 79 patients (median age 79, 56% men). Presumed CCE source was vascular in 54%, mixed vascular/cardiac in 32%, and undetermined in 14%. Median CCE diameter was 1.55mm and median density 146HU. Multiple CCE were observed in 34% of patients. The middle cerebral artery was the most frequently (84%) involved artery. CCE predominantly involved distal segments. Single uniterritorial, multiple uniterritorial, and multiterritorial CCE were observed in 63%, 22%, and 15% of patients respectively. Patients with combined vascular/cardiac CCE source were older compared with patients with vascular CCE source (p = 0.0135). Correlation coefficient between CCE diameter and density was 0.69. Clinical characteristics were similar between the groups with single uniterritorial, multiple uniterritorial, and multiterritorial CCE, apart from diabetes (p = 0.0076). Concerning radiological characteristics, median diameter and density differed between these three groups (p = 0.0029 and p = 0.0035, respectively). CCE diameter was larger (p < 0.0001) and density higher (p < 0.0001) when CCE involved proximal arterial segments. Clinical and radiological characteristics of CCE patients and CCE are variable. CCE diameter and density and presence of diabetes history differed between CCE patients with single uniterritorial, multiple uniterritorial, and multiterritorial CCE.

Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies.

To summarize the available data on the use of immunosuppression therapies for the management of hot phases of disease and recurrent myocarditis in patients with desmoplakin cardiomyopathy (DSP-CMP). Occurrence of myocarditis episodes has been associated with worsening of outcomes in DSP-CMP. Multiple case reports and small case series have described potential benefit in using anti-inflammatory and immunosuppressive medications for the treatment of those episodes. Recently published translational data has shown a clear link between DSP variants and a propensity to inflammation. The presence of acute myocarditis episodes have been clearly demonstrated as a manifestation of DSP-CMP. These episodes are marked by myocardial inflammation and subsequent fibrosis, and, moreover, contribute to a heightened future risk of subsequent arrhythmias and heart failure. Identifying optimal strategies to prevent and/or interrupt myocardial inflammation is therefore a critical priority of patients with DSP-CMP.

Synovial Chondromatosis of the Hand and Wrist: A 35-Year Retrospective Case Review.

Background: Synovial chondromatosis is a condition that rarely occurs in the hand and wrist. Recurrence and malignant transformation are two potential complications of the disease. We set out to retrospectively review 35 years of practice within our sarcoma and tertiary hand unit and identify recurrence or malignant transformation. Methods: This is a retrospective analysis of patients with synovial chondromatosis seen at our sarcoma and tertiary hand unit for the past 35 years. Incidence of recurrence or malignant transformation was noted. Where possible, patients were clinically and radiologically reviewed with range of motion, grip strength and patient evaluation measures obtained. Results: Of 12 patients presenting to the practice, seven patients were followed up and, of these, two demonstrated recurrences. The recurrence was diagnosed based on patient-reported symptoms, clinical examination and the requisition of further imaging. Of the five who could not be followed up, two demonstrated recurrence and one had malignant transformation with a low-grade chondrosarcoma on histology. Conclusions: While a rare disease, we demonstrated recurrence within our small case series. Fusion to obliterate the joint is an option in the management of recurrence. For the diagnostically challenging, genetic testing may be beneficial, though genetic rearrangements are not seen in all cases. All patients should be counselled on symptoms to watch for as a small proportion may experience recurrence and a small percentage may undergo malignant transformation. Level of Evidence: Level IV (Therapeutic).

An assessment of long-term complications following prehospital intraosseous access: A nationwide study

BackgroundThe guidelines for Advanced Life Support issued by the European Resuscitation Council recommend considering drug delivery through intraosseous access if intravenous access to the vascular bed is not feasible or unsuccessful. Emergency prehospital intraosseous cannulation may theoretically lead to an increased risk of long-term complications such as osteomyelitis, osteonecrosis, or compartment syndrome. Such complications have previously been reported in case reports or small sample case series. We systematically investigated long-term complications potentially associated with intraosseous cannulation using validated Danish health registries. MethodsData sources were the nationwide electronic Prehospital Patient Record system, the Danish National Patient Registry, and the Danish Civil Personal Registry. We investigated all patients who were subjected to prehospital intraosseous cannulation in Denmark from January 2016 through December 2019. During a follow-up period of 180 days from the index date we extracted information concerning mortality status and potential long-term complications defined as osteomyelitis, osteonecrosis, or compartment syndrome from the day of prehospital intraosseous cannulation. ResultsOf the 5,387 patients receiving intraosseous access, 375 were unidentified and lost to follow-up. Of the 5012 remaining patients, 4,775 were adults, and 237 were children. No children and “less than five” adults had long-term complications. No osteonecrosis, osteomyelitis or compartment syndrome appeared later than 175 days after an intraosseous cannulation. ConclusionsLong-term complications such as osteomyelitis, osteonecrosis, or compartment syndrome following prehospital intraosseous cannulation and drug delivery occurred in less than 0.1% of the cases. Our findings indicate that prehospital intraosseous cannulation may be safe across age groups.

Evaluating Failure Rates of Primary Quadriceps Tendon Repairs Across Different Surgical Techniques.

Quadriceps tendon ruptures occur infrequently in the general population. Biomechanical data suggest advantages with the use of suture anchor fixation for major tendon repair. Clinical studies of quadriceps tendon repair have been limited to small case series. To evaluate clinical failure following primary quadriceps tendon repair with transosseous tunnel or suture anchor repair. Cohort study; Level of evidence, 3. The Military Health System Data Repository was queried to identify all adult patients who underwent primary quadriceps tendon repair in the Military Health System between 2014 and 2018. Patients were excluded if they had incomplete records, polytrauma, open injury, prior ipsilateral total knee arthroplasty did not undergo quadriceps tendon repair, or underwent revision repair. Follow-up was obtained by manual chart review of both orthopaedic and nonorthopaedic records, recording any evidence of ongoing knee issues. Univariate analysis was performed to determine associations between potential risk factors and repair failure. Multicollinearity was assessed between potential risk factors, and candidate variables were included in multivariate logistic regression models to determine independent risk factors for repair failure. Following application of inclusion/exclusion criteria, 245 knees in 234 patients were included. Mean age of the cohort was 52 years. Patients were predominantly male (223/234; 95%) and military retirees (143/234; 61%). Transosseous tunnel repair was the most frequently employed surgical technique (147/245; 60%), followed by suture anchor repair (78/245; 32%). Repair failure requiring revision surgery occurred in 11% of knees (27/245). Surgical-site infection following index surgery was associated with eventual rerupture (P = .02). There was no difference in failure rate between transosseous tunnel repair and suture anchor repair (12.2% vs 9.0%; P = .51). Among knees undergoing suture anchor repair, no difference in failure was found between knot-tying and knotless suture anchor fixation (P = .73). We observed no difference in failure of primary quadriceps tendon repair between transosseous tunnel and suture anchor repair types or between the 2 main suture anchor implant subtypes (knotless vs knot-tying anchors). A greater than previously reported rerupture rate was observed, indicating the need for continued investigation into optimal surgical techniques.

Vulvar Epidermolytic Hyperkeratosis: A Comprehensive Systematic Review of Case Reports and Series

Background: Vulvar epidermolytic hyperkeratosis (EHK) is an exceedingly rare dermatological condition, often presenting as solitary or multiple lesions in the vulvar region. Due to its clinical resemblance to other vulvar disorders, such as condyloma acuminatum, Bowenoid papulosis, and squamous cell carcinoma, vulvar EHK poses significant diagnostic challenges. While individual case reports and small case series have documented instances of vulvar EHK, comprehensive studies systematically consolidating the clinical, histopathological, and therapeutic aspects of this condition remain lacking. Objectives: To address this gap, this systematic review consolidates all available case reports and case series on vulvar EHK. The review aims to provide a comprehensive analysis of clinical presentations, histopathological features, diagnostic challenges, treatment approaches, and patient outcomes. Methods: We conducted a systematic review following the PRISMA guidelines. We searched multiple databases (PubMed, Web of Science, Scopus) for studies published up to 30 September 2024. Only case reports and case series with histopathologically confirmed vulvar EHK were included, as no higher-level studies (e.g., randomized controlled trials or cohort studies) were available due to the rarity of this condition. Exclusion criteria were male cases, oral EHK or other unrelated conditions, and literature reviews. We extracted and analyzed data on: patient demographics, time to diagnosis, anatomical distribution, clinical presentation, associated symptoms, histopathological features, patient history, risk factors, HPV status, treatment, and outcomes. Risk of bias was assessed using the CARE checklist and JBI Checklist for Case Series. Additionally, original clinical and histopathological images from our department were included to enhance the review. Results: A total of 19 studies, encompassing 30 cases of histopathologically confirmed vulvar EHK, were identified. Most cases presented with hyperkeratotic plaques and papules localized on the labia majora. Histopathological analysis consistently revealed hyperkeratosis, acanthosis, and vacuolar degeneration in the granular and spinous layers. Misdiagnosis was common, with lesions frequently mistaken for condyloma acuminatum or other vulvar neoplasms. Conservative management, including observation and topical therapies, was associated with disease stability in asymptomatic cases, while surgical excision demonstrated complete remission in all cases where it was employed. The rarity of vulvar EHK and reliance on case reports and series limit the generalizability of findings. Conclusions: Vulvar EHK is often misdiagnosed due to its similarity to malignancies and sexually transmitted infections. This review, the first of its kind, highlights the importance of prompt histopathological diagnosis to avoid the psychological impact of a cancer or sexually transmitted disease diagnosis and unnecessary, distressing, or aggressive treatments. Further research is needed to explore the role of HPV in vulvar EHK and to establish standardized diagnostic and treatment guidelines.

Thin flap sulcus‐deepening trochleoplasty in patellar instability yields good functional outcomes without progressive cartilage deterioration in the short‐term follow‐up—A retrospective single‐surgeon cohort study

AbstractPurposeSulcus‐deepening trochleoplasty (TP) effectively treats patellofemoral (PF) instability (PFI) caused by high‐grade trochlear dysplasia (TD), but current evidence is based on small case series. We hypothesised, that TP would result in significant functional improvements and a low re‐dislocation rate but would not accelerate the progression of PF cartilage deterioration.MethodsWe retrospectively reviewed all TP cases performed by a single surgeon between 2015 and 2021. Inclusion criteria were postoperative Magnetic resonance imaging (MRI) &gt;6 and &gt;12 months and a clinical follow‐up &gt;12 months. Patients with simultaneous cartilage repair, open physes or incomplete records were excluded. Clinical outcomes were assessed using pre‐ and postoperative scores, postoperative Banff Patellofemoral Instability Instrument (BPII) 2.0 and Knee Injury and Osteoarthritis Outcome Score (KOOS), re‐dislocation rate and patient satisfaction. PF cartilage was evaluated via Area Measurement and Depth &amp; Underlying Structures (AMADEUS) scores preoperatively, at 6 months and at the final follow‐up.ResultsWe included 113 patients (25.8 ± 8.3 years) with high‐grade TD (Dejour B–D; mean lateral inclination angle: −2.9 ± 9.1°), 85% of whom had advanced cartilage lesions. All underwent TP, lateral retinacular lengthening (LRL) and medial patellofemoral ligament reconstruction (MPFL‐R). After 34.8 ± 20.9 months, function, pain levels and Tegner scores improved significantly (p &lt; 0.001). KOOS dimensions were: symptoms 79.9 ± 13.5, pain 86.4 ± 12.1, activity 91.9 ± 8.3, sports 71.7 ± 22.2 and quality‐of‐life 58.1 ± 23.8. BPII 2.0 was 64.3 ± 31.4. Preoperative AMADEUS scores (55.2 ± 17.4) remained stable at 6 months (p = 0.343) but improved to 58.4 ± 16.0 at 28.6 (12–89) months (p = 0.004). Complication and re‐dislocation rates were 5.3% and 1.8%, with 93% patient satisfaction.ConclusionSulcus‐deepening TP with MPFL‐R and LRL yields good to excellent short‐term results without progressive chondral deterioration, enabling patients to return to their prior or higher activity levels despite advanced preoperative chondral lesions. TP can be considered a safe, joint‐preserving technique for PF stabilisation.Level of EvidenceLevel III, retrospective cohort study.

Psychometric evaluation of the Autism Symptom Dimensions Questionnaire.

To replicate and extend previous psychometric findings for the Autism Symptom Dimensions Questionnaire (ASDQ). Using a cross-sectional design in two samples, including a total of 3366 children and adolescents (aged 2-17 years; 1399 with autism spectrum disorder) and a small case series, factor structure, measurement invariance, reliability, construct validity, screening and diagnostic efficiency, and detection of reliable change were examined for the ASDQ. Strong psychometric properties were observed, including replicable factor structure, strong measurement invariance, adequate-to-excellent scale and conditional reliability, strong convergent and discriminant validity, and good screening efficiency. Importantly, two crucial measurement aspects required for a good treatment outcome measure were found, that is, good test-retest stability and the potential to detect reliable change. As expected for an informant-reported questionnaire, diagnostic efficiency was weak. The ASDQ showed evidence of favorable and well-replicated psychometric properties in two samples. Good screening but weak diagnostic efficiency, coupled with good test-retest stability, suggest that optimal clinical use is in screening and in detailed clinical characterization beyond what is offered by current informant-reported measures, with the potential for monitoring response to intervention. Despite relative brevity, the ASDQ provides good coverage of broad and specific aspects of the autism behavioral phenotype, which is consistent with the criteria of the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition.

Imaging diagnosis and clinical outcomes of eight horses with sinus gas expansion

SummaryBackgroundSinus gas expansion is a rare condition in equine patients, resulting in progressive deformity of the facial bones. The condition is sparsely documented in veterinary literature. Similar lesions are categorised as sinus pneumocele or pneumosinus dilatans in human patients, based on the presence or absence of sinus wall erosion.ObjectivesTo describe imaging findings in horses with gas expansion of the paranasal sinuses.Study designMulticentric retrospective descriptive case series.MethodsHorses with a computed tomographic (CT) description of sinus gas expansion were included. Computed tomography changes were described, and lesions were categorised as sinus pneumocele or pneumosinus dilatans. Signalment, history and clinical signs were recorded, as well as radiological signs, histology and follow‐up information, when available.ResultsEight horses met the inclusion criteria, including seven diagnosed with sinus pneumocele and one with pneumosinus dilatans. Clinical signs were variable, depending on the affected bones and sinuses. The conchofrontal sinus was the most frequently affected. Irregular sinus wall erosion, marginal separation of the bone diploe and abnormal septation were consistent imaging findings in horses with sinus pneumocele. Active bone remodelling was confirmed by histology in one patient. Based on CT, the sinus mucosa was thickened adjacent to the sinus wall erosion in 5/7 patients, with hazy dystrophic mineralisation in three horses. One horse had a follow‐up CT at 26 months, showing progression of the lesion. The horse with pneumosinus dilatans had minor facial bone deformity and severe gas expansion of all left‐sided paranasal sinuses, largely encroaching on the right paranasal sinuses, without bone erosion.ConclusionDiagnostic imaging and more particularly CT is valuable for identifying and characterising abnormal gas expansion of the paranasal sinuses in horses. Based on this small case series, the condition is slowly progressive and inconsistently associated with clinical signs. Its pathophysiology remains unclear, warranting further exploration.

The Potential Elimination of Blood Transfusions in Lumbar Spine Fusion Surgery: Clinical Case Series of 620 Consecutive Minimally Invasive TLIF Surgeries and Review of the Literature

Background Lumbar spine fusion is one of the most common surgical procedures performed in the operative management of adult degenerative spine disorders. The dual incision, mini-open TLIF surgery uses a minimally invasive surgical technique that decompresses neural elements through placement of an anterior interbody disc spacer and posterior stabilization with pedicle screws. It is well established that this approach results in significantly less muscle dissection than the open technique used in conventional TLIF, however a quantitative assessment of the need for blood transfusions during this procedure has been limited to relatively small case series. The aim of this study was to assess the incidence of blood transfusion amongst a large group of patients undergoing a mini-open dual incision TLIF procedure over an extended amount of time. A retrospective study was completed of 620 consecutive patients surgically treated with the Minimally Invasive Transforaminal Lumbar Interbody Fusion (MI-TLIF) procedure, the largest such study completed by a single surgeon at multiple institutions from 2004 to 2020. Methods The study sample comprises 620 patients who underwent a mini-open TLIF procedure through a dual incision technique for treatment of lumbar degenerative diseases from January 2004 to October 2020. No patients who underwent the procedure were excluded from the series. Surgical and medical record review was conducted to determine whether patients required blood transfusions during their hospitalization. No patients were lost to three-month follow up. Results The study group consisted of 620 patients, 332 women and 288 men. Ages ranged from 20 to 88 years old with a mean age of 54 years. No patients in the series required a blood transfusion during their surgery, hospitalization, or 3-month postoperative period. Conclusions The dual incision MIS Mini-open transforaminal lumbar interbody fusion allows for decompression of neural elements, pedicle screw instrumentation, and placement of an anterior interbody cage while potentially eliminating the need for blood transfusions. While previous studies have shown the potential for reduced blood loss with the mini-open technique compared to the open technique, this study adds a significant volume of data-driven evidence to support this conclusion. As such, this study represents the largest single-surgeon case series to show consistent absence of blood transfusions with a mini-open TLIF technique over sixteen years.

Microdissection of Distinct Morphological Regions Within Uveal Melanomas Identifies Novel Drug Targets.

Background/Objectives: Uveal melanomas (UMs) are rare but often deadly malignancies that urgently require viable treatment options. UMs often exhibit tumour heterogeneity, with macroscopic and microscopic differences in morphology between different regions of the same tumour. However, to date, the clinical significance of this and how it may help guide personalised therapy have not been realised. Methods: Using targeted DNA and RNA sequencing of a small case series of large, high-risk primary UMs, we explored whether morphologically distinct regions of the same tumour were associated with distinct molecular profiles. Results: In four of the seven tumours analysed, we detected different sets of genetic variants following the separate analysis of microdissected melanotic and amelanotic regions of the same tumour. These included a MET exon 14 skipping RNA transcript that predicts sensitivity to crizotinib and variants in other genes that are important in active clinical trials for patients with UM and advanced solid tumours. The integration of TCGA data also identified recurrent mutational events in genes that were not previously implicated in UM development (FANCA, SLX4, BRCA2, and ATRX). Conclusions: Our findings show that the molecular analysis of spatially separated and morphologically distinct regions of the same tumour may yield additional, therapeutically relevant genetic variants in uveal melanomas and have implications for the future molecular testing of UMs to identify targeted therapies.

Cystic Neutrophilic Granulomatous Mastitis: Imaging Features With Histopathologic Correlation.

Cystic neutrophilic granulomatous mastitis (CNGM) is a rare type of granulomatous lobular mastitis (GLM) with a distinct histologic pattern characterized on histopathology by clear lipid vacuoles lined by peripheral neutrophils ("suppurative lipogranulomas"), often containing gram-positive bacilli and strongly associated with Corynebacterial infection (in particular, Corynebacterium kroppenstedtii). Cystic neutrophilic granulomatous mastitis has a distinct histopathologic appearance, but the imaging appearance is less well described and has been limited to case reports and small case series published primarily in pathology literature. Mammographic findings of CNGM include focal asymmetry, skin thickening, and irregular or oval masses. Sonographic findings of CNGM include irregular mass, complex collection/abscess, dilated ducts with intraductal debris, axillary lymphadenopathy, and skin thickening with subcutaneous edema. The imaging features of CNGM are nonspecific, and biopsy is required. Identifying a causative organism, when possible, requires a Gram stain, microbiological culture, and, potentially, molecular analysis. Although therapeutic options exist for CNGM, including antibiotics, steroids, and surgical intervention, there is no current consensus on optimal treatment.

Dexamethasone palmitate for children with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) has a wide range of clinical presentations and is sometimes life-threatening. It is often treated with systemic corticosteroids and etoposide, but no optimal treatment has been identified. Dexamethasone palmitate (DP) contains a combination of dexamethasone and a lipid emulsion and is selectively taken up by activated macrophages. Recently, a small case series reported the efficacy of dexamethasone palmitate (DP) in HLH. Here, we present the results of a nationwide survey in Japan detailing 14 cases of EBV-HLH treated with DP in children. One week after DP initiation, fever, cytopenia, and splenomegaly resolved in 77%, 38%, and 77% of patients (10, 5, and 10 of 13 patients, 1 missing). A 50% or greater reduction in ferritin levels was observed in 62% of patients (8 of 13 patients, 1 missing). In addition, the attending physician judged DP to be effective or partially effective in 12/14 (86%) patients. DP-related adverse events were uncommon, with only two infectious events reported. Thus, DP can be a therapeutic option for EBV-HLH.

The Natural History of Portal Venous System Aneurysms.

Portal venous system aneurysms (PVA) are increasingly diagnosed on cross-sectional computed tomography (CT) imaging. However, the natural history of these aneurysms is poorly understood and reports are limited to small case series. Terms relevant to PVA were searched in radiology reports (2010-2022) with PVA presence confirmed by manual review. PVA were defined as a diameter greater than 1.5 cm in patients without cirrhosis and 1.9 cm in those with cirrhosis. Aneurysm growth was defined as greater than 20% increase in size while aneurysm regression as greater than 20% decrease in size. Patient demographics, comorbid conditions, and PVA outcomes were abstracted. Univariate statistics were used to compare groups. Thirty-eight aneurysms with radiographic follow up were identified in 35 patients, involving the portal vein (n=18, 47.4%), splenic vein (n=10, 26.3%), superior mesenteric vein (n=3, 7.9%), and portal confluence (n=7, 18.4%). While 12 (31.6%) were idiopathic, the remaining 26 (68.4%) were associated with portal hypertension (n=20, 52.6%) and prior liver transplant (n=4, 10.5%). The median growth was 0.2 cm (-2.6-2.4 cm) over median follow up over 5.0 years (0.3-16.6). Five (13.2%) PVA regressed and were largely idiopathic (80.0%, p=0.03). Thirteen (34.2%) PVA grew and were associated with portal hypertension (n=11, 84.6%, p=0.003) and thrombosis (n=6, 46.2%, p=0.05). Nine (23.7%) PVA thrombosed, predominantly in males (n=7, 77.8%). The median growth was 1.0 cm (-0.7-1.9 cm). Three patients (33.3%) were symptomatic from PVA thrombosis including abdominal pain (n=2, 22.2%), intestinal ischemia (n=1, 11.1%), and variceal bleeding (n=2, 22.2%). Four (44.4%) patients were treated with anticoagulation. No aneurysms ruptured. Of the 58 PVA initially identified with and without radiographic follow up, 5 (8.6%) underwent intervention with a median diameter of 4.0 cm (3.4-5 cm). Intervention included vein ligation (n=1, 20.0%), aneurysmorrhaphy (n=1, 20.0%) and aneurysmectomy (n=3, 60.0%). There was one case of aneurysm recurrence 20 years following resection and one post-operative mortality. Two-thirds of PVA, including size greater than 3 cm, remain stable on surveillance. While annual surveillance is initially recommended to confirm PVA stability, interval imaging can be subsequently extended given low growth rates. Over 20% of PVA thrombosed but none ruptured. Although we did not observe any cases of rupture, the devastating consequences of rupture necessitate consideration of surgical intervention for large symptomatic PVA.

Multiple Keratoacanthomas Associated with Genetic Syndromes: Narrative Review and Proposal of a Diagnostic Algorithm.

Keratoacanthoma (KA) is a relatively common, fast-growing epithelial tumour, with characteristic behaviour and clinical variability. Although it appears as a solitary lesion in a majority of cases, multiple KAs do occur, secondary to skin exposure to ultraviolet radiation, chemical carcinogens or certain medications, but may also be associated with various genetic syndromes. Thus, multiple KAs may serve as an early clinical alarm sign. Prompt diagnosis of the underlying cause and identification of the mechanism of development are critical for the secondary prevention of associated organ disorders or neoplasias, the improvement of patient quality of life and familial counselling. Although researchin this field has seen important progress in the last few years, there are still many pathogenic processes that have not been elucidated. Additionally, the literature on this topic is limited to individual case reports and small case series, making it difficult for clinicians to parse available data and select the essential information. Therefore, this work aims to review current knowledge, summarizing existing studies, with focus on multiple KAs associated with genetic syndromes, and proposes a diagnostic algorithm for these rare cases to help guide clinicians in their practice. Lastly, we aim to highlight the main gaps in understanding the underlying mechanisms and suggest further research avenues.

Visualization of caudothalamic groove at expert fetal neurosonography.

To describe the sonographic features of the caudothalamic groove in the third trimester of pregnancy in a group of structurally normal fetuses and to report a small series of cases with abnormal appearance of the caudothalamic groove at antenatal cranial ultrasound. This was an observational study conducted at two fetal medicine referral units in Italy. A non-consecutive cohort of pregnant women with a singleton non-anomalous pregnancy were recruited prospectively and underwent three-dimensional (3D) ultrasound assessment of the fetal brain at 28-32 weeks' gestation. At offline analysis, the ultrasound volumes were adjusted in the multiplanar mode, according to a standardized methodology, until the caudothalamic groove was visible in the parasagittal plane. To evaluate interobserver agreement, two operators were asked independently to indicate if the caudothalamic groove was visible unilaterally or bilaterally on each volume and Cohen's kappa (κ) coefficient was calculated. The digital archives of the two centers were also searched retrospectively to retrieve cases with abnormal findings at the level of the caudothalamic groove on antenatal cranial ultrasound that were confirmed postnatally. A total of 180 non-consecutive cases were included. At offline analysis of the 3D ultrasound volumes, the caudothalamic groove was identified in the parasagittal plane by both operators at least unilaterally in 176 (97.8%) cases and bilaterally in 174 (96.7%) cases. The κ-coefficient for the agreement between the two independent operators in recognizing the caudothalamic groove was 0.89 and 0.83 for one and both hemispheres, respectively. The retrospective search of our archives yielded five cases with an abnormal appearance of the caudothalamic groove at antenatal cranial ultrasound, including two cases of hemorrhage and three cases of cyst. The caudothalamic groove is consistently seen in normal fetuses on multiplanar neurosonography in the third trimester, and abnormal findings in this region may be detected antenatally. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Use of omentum during robotic-assisted reconstructive urological surgery: a systematic review of the current literature.

Due to its biological properties, the omentum is a very useful tool in the hands of reconstructive urologists. The purpose of this systematic review is to present all existing evidence regarding the use of omentum during different robotic-assisted reconstructive urological surgeries. In accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Guidelines, three databases (PubMed, Scopus and Cochrane) were systematically screened. The following search string was used: (omentum OR omental) AND robotic. Retrospective studies and case-series were included, while case-reports were not included. 13 studies met all eligibility criteria and were included in final qualitative synthesis. Seven studies reported robotic vesicovaginal fistula repair, two studies reported robotic vesicouterine or vesicocervical fistula repair, one study reported robotic rectovesical fistula repair, one study reported robotic rectourethral fistula repair, one study reported ureterolysis with omental wrap and one study reported robotic repair of long ureteral strictures with omental wrap and autologous onlay flap or graft ureteroplasty. Recurrence rates ranged from 0% to 6.7%. The Grade I-II complications according to Clavien-Dindo Classification ranged from 0 to 40%, while no Grade III-IV were reported. Robotic repair with the use of omentum is potentially a feasible, safe and efficient approach for complicated urological surgeries, such as vesicovaginal, vesicouterine, vaginocervical, rectovesical and rectourethral fistulas, idiopathic retroperitoneal fibrosis and long ureteral strictures. Because the evidence is based on small case series, further publications are needed to enhance confidence in omental harvesting and render it a routine component of reconstructive Urology.

Risk Factor of Cutaneous Symptoms Associated with Enfortumab Vedotin for Urothelial Carcinoma

In 2021, Enfortumab Vedotin (EV) was approved for the treatment of locally advanced and metastatic urothelial carcinoma (UC) in Japan. Some patients require withdrawal or dose reduction due to cutaneous symptoms associated with EV treatment. Currently, the only reported associations of EV with cutaneous symptoms are pre-treatment performance status (PS) and body weight. However, no report has focused on the association between hematological factors and cutaneous symptoms. We retrospectively examined the occurrence of cutaneous symptoms and associated factors in patients who had been treated with EV for UC at Nagasaki University Hospital between February 2022 and June 2023. We evaluated the six patients with bladder cancer and three cases of upper urinary tract cancer. The median age was 73 [57-85] years. Seven patients had cutaneous reactions with EV treatment, and the median date of onset of the cutaneous symptoms was seven [4 -28] days. PS was better inthe group with skinsymptoms compared with the group without (median : 1.0 vs 3.0), and in those with a higher incidence of eosinophilia or cutaneous symptoms on the previous regimen (71. 4% vs 0%). We suggested that the presence of eosinophilia or cutaneous symptoms at the time of pre-EV regimen administration may be a predictor of the appearance of cutaneous symptoms at the time of EV treatment. The limitation is that it is a single-center, small-case series, which does not allow for a rigorous statistical study.

Fusion Free Median Corpectomy - Long-Term Consequence of Foregoing Fusion and Fixation: A Serendipitous Observation.

Anterior cervical corpectomy and fusion (ACCF) involves placement of a graft/implant to ensure fusion and stabilization along with neural decompression. We share our experience with a subset of ACCF patients in whom graft/implant could not be placed post decompression for varying reasons but had a favorable long-term outcome. The necessity for routine fusion after corpectomy is critically analyzed, and the feasibility of an alternative surgical option without graft/implant is discussed. A retrospective observational study of long-term outcomes of five patients with cervical compressive myelopathy who underwent neural decompression alone without an in situ graft/implant was done. All the patients underwent the modified cervical corpectomy technique, involving a smaller wedge size (12 × 15 mm) corpectomy. Pre- and postoperative clinical, radiological, electrophysiological, and operative findings were meticulously evaluated and analyzed. This retrospective observational study reviewed records of 124 patients who underwent ACCF between 1998 and 2012. The surgical procedure involved a wedge median cervical corpectomy. In seven patients, graft/implant could not be placed or had to be removed for various reasons. Long-term follow-up was available for five of these patients who were included in the study. In two patients, the graft could not be placed intraoperatively due to hemodynamic complications. In one patient, the graft had to be removed due to postoperative infection, and in two patients, the graft had to be removed due to graft extrusion. Four patients showed marked improvement, and one patient showed minimal improvement on long-term follow-up in clinical, radiological, and electrophysiological parameters. This small case series suggests that a modified cervical corpectomy technique, involving a smaller wedge size (12 × 15 mm), may be considered in selected cases without radicular symptoms. This approach helps to provide neural decompression without compromising stability and avoids graft-related complications. While promising, further research, including prospective randomized-controlled trials, is essential to validate the biomechanical properties and clinical outcomes of this modified technique compared to traditional ACCF.