Correlation Between Ophthalmologic and Neuroradiologic Findings in Type 1 Neurofibromatosis.

BACKGROUND: Neurofibromatosis Type 1 (NF-1) is a genetic disease affecting the eye, and ocular findings such as Lisch nodules (LN) or optic pathway gliomas (OPGs) are a part of its diagnostic criteria. Recent imaging technologies such as infrared (IR) imaging and optical coherence tomography (OCT) have highlighted the visualization of choroidal focal abnormalities in these patients, even in the absence of other ocular lesions. This study aimed to establish a morphological multimodal evaluation of choroidal findings in patients with NF-1, correlating them with central nervous system (CNS) findings. METHODS: This retrospective study included 44 eyes from 22 patients with NF-1. Central 30° IR imaging was obtained, and the number and total area of detectable lesions were calculated. Both macular and optic disc scanning with OCT were performed, with and without the enhanced depth imaging technique, to assess the presence of choroidal focal hyperreflective lesions. Central macular thickness, ganglion cell layer, and outer nuclear layer thickness were assessed, as well as subfoveal choroidal thickness. The peripapillary retinal nerve fiber layer (RNFL) thickness was also assessed. Patients' magnetic resonance images (MRI) were reviewed and categorized by a neuroradiology specialist, determining the presence of OPGs and CNS hamartomas. Correlations between the ophthalmological and neuroradiological findings were established. RESULTS: Patients' mean age was 16.4 ± 7.3 years and 59.1% were women. On the MRI, 86.4% of the patients had CNS hamartomas, and 34.1% of the eyes had OPGs. LN were described in 29.5% of the eyes, whereas a total of 63.4% of the eyes presented the characteristic hyperreflective lesions in IR imaging, all of them matching the underlying choroidal lesions. A mean of 2.9 ± 3.3 lesions per eye and a median total lesion area of 1.52 mm2 were found. The presence of OPGs was correlated with a greater number (P = 0.004) and a larger area (P = 0.006) of IR lesions. For a cut-off of 3.5 lesions per eye, the sensitivity and specificity for the presence of OPGs were 75% and 80%, respectively. For a total lesion area of 2.77 mm2, the sensitivity and specificity for the presence of OPGs were 69.2% and 93.1%, respectively. Eyes with OPGs presented a significant reduction in the temporal RNFL (P = 0.018) thickness, as well as a reduction in subfoveal choroid thickness (P = 0.04). No relations were found between CNS hamartomas and ophthalmological findings. CONCLUSIONS: This study suggests that focal choroidal abnormalities are correlated with the presence of CNS lesions as OPGs in patients with NF-1, and it might be a surrogate for the need for CNS imaging in these patients.

Median Arcuate Ligament Syndrome of Diaphragm - Literature Review and Case Report.

OBJECTIVES: To report a case of median arcuate ligament syndrome (MALS) and to review current literature. METHODS: Case report and literature review using PubMed with the terms "median arcuate ligament", "Dunbar syndrome" and "MALS treatment" as major topics. The bibliography of relevant articles has been checked to identify other significant papers. RESULTS: Median arcuate ligament syndrome (MALS) or Dunbar syndrome is a rare clinical entity characterized by celiac trunk compression by median arcuate ligament and variable gastrointestinal symptoms. However, some degree of radiographic compression is observed in 10%-24% of asymptomatic patients, so MALS is a diagnosis of exclusion. Treatment options include release of median arcuate ligament (open, laparoscopic or robot-assisted) and open vascular reconstruction. Endovascular treatment is currently used only as adjuvant procedure after surgical release of median arcuate ligament. A 34-year-old woman, previously healthy, presented with a epigastric pain, mainly postprandial, for 6 months, associated to anorexia and unprovoked weight loss of 8kg over 3 months. Physical examination was normal. Other gastrointestinal pathologies were ruled out. Abdomino-pelvic computed tomography angiography revealed a focal 80% stenosis of proximal celiac trunk. An open decompression of the celiac trunk was performed. The postoperative period was uneventful and the patient was discharged 5 days later, with normal gastrointestinal transit and without abdominal pain recurrence. CONCLUSION: MALS diagnostic and therapeutic approach must be patient focused, bearing in mind the multiple clinical presentation and treatment options. Open surgical decompression of median arcuate ligament is the base of treatment.

Objetivos: Descrever um caso clínico de síndrome do ligamento arqueado do diafragma (SLA) e realizar uma revisão da literatura. Métodos: Descrição de um caso clínico e revisão da literatura com recurso ao PubMed com os termos "median arcuate ligament", "Dunbar syndrome" e "MALS treatment". A bibliografia dos artigos relevantes foi verificada para identificar outros artigos pertinentes. Resultados: A síndrome do ligamento arqueado (SLA) ou síndrome de Dunbar é uma entidade clínica rara caracterizada pela compressão do tronco celíaco associada a sintomas gastrointestinais variáveis. No entanto, algum grau de compressão radiográfica é observado em 10-24% de doentes assintomáticos, o que torna o diagnóstico de SLA de exclusão. As opções terapêuticas incluem a secção do ligamento arcuato (via convencional, laparoscópica ou robótica) e a reconstrução vascular. A abordagem endovascular é atualmente utilizada apenas como procedimento adjuvante após a secção do ligamento arqueado. Doente do sexo feminino de 34 anos de idade, previamente saudável, recorre ao médico assistente por um quadro de dor epigástrica, especialmente pós-prandial, com 6 meses de evolução, associada a anorexia e perda ponderal não provocada superior a 8kg, num período de 3 meses. O exame objetivo não relevou alterações. Outras patologias gastrointestinais foram excluídas. A angiografia por tomografia computorizada revelou uma estenose focal de 80% na porção proximal do tronco celíaco. A doente foi submetida a uma descompressão cirúrgica por via convencional. O período pós-operatório decorreu sem intercorrências, tendo alta 5 dias após a intervenção com o trânsito gastrointestinal restabelecido e sem recorrência da dor abdominal. Conclusão: A abordagem diagnóstica e terapêutica do SLA deve ser individualizada e focada no doente, tendo em conta as múltiplas apresentações clínicas e possíveis opções terapêuticas. A descompressão cirúrgica convencional do ligamento arqueado continua a ser a base do tratamento.

Median Arcuate Ligament Syndrome - Literature Review and Case Report.

INTRODUCTION: Median arcuate ligament syndrome (MALS) or Dunbar syndrome is a rare clinical entity characterized by celiac trunk compression by median arcuate ligament and variable gastrointestinal symptoms (postprandial epigastric pain, nausea, weight loss, anorexia and diarrhea). However, some degree of radiographic compression is observed in 10%-24% of asymptomatic patients. Besides the extrinsic vascular compression, MALS has a multifactorial etiology and it has been suggested as a neurogenic disease resulting in altered sensation and pain from the somatic nerves in the splanchnic plexus. MALS is a diagnosis of exclusion, so other causes must be excluded. Treatment options include release of median arcuate ligament (open, laparoscopic or robot-assisted) and open vascular reconstruction. Endovascular treatment is currently used only as adjuvant procedure after surgical approach, in refractory cases with residual stenosis of celiac trunk. OBJECTIVE: To report a case of MALS and to review current literature. METHODS: The authors report a clinical case and present a literature review using PubMed with the terms "median arcuate ligament", "Dunbar syndrome" and "MALS treatment" as major topics. The bibliography of relevant articles has been checked to identify other significant papers. RESULTS: A 34-year-old woman, previously healthy, recurred to a General Practitioner with a recurrent epigastric pain, exacerbated by ingestion, without relieving factors, in the previous 6 months. Patient also reported anorexia and unprovoked weight loss of 8Kg over 3 months. Physical examination was normal. Other gastrointestinal pathologies were ruled out. Computed Tomography Angiography (CTA) abdomen revealed a focal 80% stenosis of the celiac trunk, located 8mm from its origin in aorta and a post- -stenotic enlargement of 9mm. An open decompression of the celiac trunk was performed. Through an 8cm median supraumbilical laparotomy, supraceliac abdominal aorta was approached. The compressive band across the celiac trunk was identified and cut. Further dissection was performed until the celiac artery became completely exposed and its branches identified. The postoperative period was uneventful and the patient was discharged 5 days later, with normal gastrointestinal transit and without recurrence of the abdominal pain. 1 month later, the patient remained asymptomatic. A long-term follow-up with annual duplex scan and clinical evaluation must be done, in order to evaluate the need of a revascularization due to persistent stenosis or aneurysmal degeneration. CONCLUSION: MALS diagnostic and therapeutic approach must be patient focused, bearing in mind the multiple clinical presentation and treatment options. Open surgical decompression of median arcuate ligament is the base of therapy.

Molecular and entomological surveillance of malaria vectors in urban and rural communities of Benguela Province, Angola.

BACKGROUND: Malaria is a major public health problem in Angola, with Anopheles gambiae sensu lato (s.l.) and An. funestus s.l. being the primary vectors. This study aimed to clarify the information gaps concerning local Anopheles mosquito populations. Our objectives were to assess their abundance, geographical dispersion, and blood-feeding patterns. We also investigated their insecticide resistance. Molecular methods were used to identify sibling species, determine the origin of blood meals, measure Plasmodium falciparum infection rates, and detect the presence of knockdown resistance (kdr) mutations. METHODS: Adult mosquitoes were collected indoors using CDC light traps from nine randomly selected households at two sentinel sites with distinct ecological characteristics. The samples were collected from 1 February to 30 June 2022. Anopheles mosquitoes were morphologically identified and subjected to molecular identification. Unfed Anopheles females were tested for the presence of P. falciparum DNA in head and thorax, and engorged females were screened for the source of the blood meals. Additionally, members of An. gambiae complex were genotyped for the presence of the L1014F and L1014S kdr mutations. RESULTS: In total, 2226 adult mosquitoes were collected, including 733 Anopheles females. Molecular identification revealed the presence of Anopheles coluzzii, An. gambiae senso stricto (s.s.), An. arabiensis, and An. funestus s.s. Notably, there was the first record of An. coluzzii/An. gambiae s.s. hybrid and An. vaneedeni in Benguela Province. Plasmodium falciparum infection rates for An. coluzzii at the urban sentinel site and An. funestus s.s. at the rural site were 23.1% and 5.7%, respectively. The L1014F kdr mutation was discovered in both resistant and susceptible An. coluzzii mosquitoes, while the L1014S mutation was detected in An. gambiae s.s. for the first time in Benguela Province. No kdr mutations were found in An. arabiensis. CONCLUSIONS: This study provides valuable insights into the molecular characteristics of malaria vectors from the province of Benguela, emphasising the need for continuous surveillance of local Anopheles populations regarding the establishment of both kdr mutations for tailoring vector control interventions.

Exploring the use of ChatGPT in predicting anterior circulation stroke functional outcomes after mechanical thrombectomy: a pilot study.

BACKGROUND: Accurate prediction of functional outcomes is crucial in stroke management, but this remains challenging. OBJECTIVE: To evaluate the performance of the generative language model ChatGPT in predicting the functional outcome of patients with acute ischemic stroke (AIS) 3 months after mechanical thrombectomy (MT) in order to assess whether ChatGPT can used to be accurately predict the modified Rankin Scale (mRS) score at 3 months post-thrombectomy. METHODS: We conducted a retrospective analysis of clinical, neuroimaging, and procedure-related data from 163 patients with AIS undergoing MT. The agreement between ChatGPT's exact and dichotomized predictions and actual mRS scores was assessed using Cohen's κ. The added value of ChatGPT was measured by evaluating the agreement of predicted dichotomized outcomes using an existing validated score, the MT-DRAGON. RESULTS: ChatGPT demonstrated fair (κ=0.354, 95% CI 0.260 to 0.448) and good (κ=0.727, 95% CI 0.620 to 0.833) agreement with the true exact and dichotomized mRS scores at 3 months, respectively, outperforming MT-DRAGON in overall and subgroup predictions. ChatGPT agreement was higher for patients with shorter last-time-seen-well-to-door delay, distal occlusions, and better modified Thrombolysis in Cerebral Infarction scores. CONCLUSIONS: ChatGPT adequately predicted short-term functional outcomes in post-thrombectomy patients with AIS and was better than the existing risk score. Integrating AI models into clinical practice holds promise for patient care, yet refining these models is crucial for enhanced accuracy in stroke management.

Temporomandibular joint arterial variability.

The study aimed to investigate temporomandibular joint (TMJ) arterial variability. In this prospective study, the vasculature variability was studied using a 3D volume rendering CT angiography including random patients at two hospitals. A 16-quadrant (A1-D4) evaluation grid was developed using the Frankfurt plan as main reference. For each quadrant, the number of arterial ramus or branches was scored as clearly visible (2), partially visible (1), or not visible (0). A total of 50 patients were enrolled (mean age of 62.9 ± 16.0); 21 (42%) were men, and 29 (58%) were women. The authors observed bilaterally higher arterial density in the posterior aspect of the ascending ramus of the mandible (p < 0.0001), corresponding to quadrants B2 (5.92 ± 2.27 and 6.14 ± 2.56), B3 (9.76 ± 2.97 and 11.18 ± 2.86) and B4 (7.38 ± 2.78 and 8.10 ± 2.42). A strong correlation was found between the number of vessels and the variability of the region (r = 0.87, p = 0.00001). No differences were observed between men and women. Within the limitations of the study, arterial variability was observed in the TMJ territory. The posterior zone of the condyle and ramus is the most vascularized area, with great variability, representing an increased risk for surgical bleeding. Therefore, this knowledge seems to be particularly relevant for surgeons dedicated to TMJ and other facial surgery or facial/cerebral radiologic interventions. The authors encourage future studies to include larger samples and to identify thoroughly the arterial branches in this area.

Bilateral Episcleral Corkscrew Vessels: Expedition into the Unknown - Case Report.

We aim to report a case of a middle-aged woman with bilateral idiopathic elevated episcleral venous pressure (IEEVP) and its difficulties in the diagnostic approach. Particularly in this case, the atypical feature of ocular hypertension without glaucomatous optic nerve damage may be misleading. We present a 66-year-old woman with longstanding bilateral "red eyes." Clinical findings included bilateral episcleral vessel engorgement and tortuosity and raised intraocular pressure with open iridocorneal angles. Despite ocular hypertension, glaucomatous neuropathy was absent and confirmed by normal structural (optical coherence tomography) and functional (standard automated perimetry) tests. The systemic workup was unremarkable. Magnetic resonance angiography showed bilateral dilated superior ophthalmic veins. Cerebral digital subtraction angiography was requested, and no carotid-cavernous fistula (or other significant vascular findings) was identified. The diagnosis of IEEVP was assumed. In conclusion, our case highlights the systematic investigation necessary in cases of bilateral episcleral vessel engorgement and tortuosity and the possible differential diagnosis to be considered to rule out life-threatening causes of elevated episcleral venous pressure. It is important for clinicians to be aware of IEEVP even in patients with atypical features that despite significative ocular hypertension had no glaucomatous damage.

Tocilizumab as a Useful Tool for Thyroid Eye Disease in Pediatric Population: A Case Report.

Thyroid eye disease (TED) characterizes by inflammation and remodeling of orbital tissues. Although the majority of pediatric TED is mild, some children present progressive and severe disease. The approach to severe disease in this group of patients, especially when noncortico-responsive, is challenging. The purpose of this work was to describe the effective use of tocilizumab as second-line therapy in steroid-resistant pediatric TED. A 13-year-old female with a history of Graves' disease presented with right eye proptosis for at least 8 months associated with mild pain on eye movements and ocular surface complaints. The ophthalmologic evaluation revealed Hertel exophthalmometry readings of 22 mm on the right eye (OD) and 19 mm on the left (OS). The remaining ophthalmic examination was unremarkable. Intravenous methylprednisolone pulses of 500 mg were initiated without any improvement after 4 treatments. Following multidisciplinary team discussion, therapy was switched to monthly tocilizumab injections at 4 mg/kg. Significant reduction of proptosis and resolution of pain and ocular surface complaints were noted immediately after the treatment switch. Exophthalmometry readings after the end of treatment that included 4 tocilizumab injections were 20 mm on OD and 19 mm on OS. No side effects were reported during the entire follow-up. Six months after treatment cessation, the patient remains stable, without any signs of orbitopathy relapse and no ophthalmologic complaints. This is the second case report showing the effectiveness of tocilizumab in pediatric TED and the first one showing its efficacy when steroids fail. Our results support the potential safety and efficacy of this immunosuppressor in children with TED.

Dissection Flap Fenestration with a Transjugular Intrahepatic Portosystemic Shunt Needle: An Adjuvant Technique in Endovascular Treatment of Post-Dissection Thoraco-Abdominal Aortic Aneurysms.

INTRODUCTION: In chronic aortic dissection complicated by aneurysmal degeneration, the absence of spontaneous tears between the true and false lumen at visceral artery level may limit treatment by fenestrated/branched endovascular aneurysm repair (F/BEVAR). The creation of new fenestrations may be required to allow access to the visceral vessels. TECHNIQUE: In this video, the endovascular treatment of a 70 year old white man with chronic type B aortic dissection complicated by Crawford type II thoraco-abdominal aortic aneurysmal degeneration is presented. The right renal artery had a false lumen origin without nearby visible re-entry tears. He underwent dissection flap fenestration at visceral vessel level using a transjugular intrahepatic portosystemic shunt (TIPS) needle and subsequent dilation with a high pressure balloon. A Zenith TX2 dissection endovascular graft was deployed proximally and extended distally with a Zenith dissection endovascular stent until the fenestration level was reached. In a second stage, a F/BEVAR was performed, with fenestration to the left renal artery and branches to right renal artery, superior mesenteric artery, and coeliac trunk. One year follow up computed tomography angiography showed visceral branch patency and a reduction of the aneurysm sac. DISCUSSION: The chronic dissection flap may be thick and fibrotic, creating a technical challenge for endovascular fenestration. The off label use of a TIPS needle in this procedure created a new fenestration at the desired level and allowed definitive post-dissection treatment of the thoraco-abdominal aneurysm.

Orbital Apex Syndrome due to Orbital Mucormycosis after Teeth Infection: A Successful Case Report.

Rhino-orbital-cerebral mucormycosis is a severe and rapidly progressive fungal infection associated with low survival rates. Early diagnosis and proper management are mandatory. We report the case of a 20-year-old female with diabetic ketoacidosis and teeth infection, who presented with sudden orbital apex syndrome. Several surgical procedures were necessary to provide a definite histopathological diagnosis of the fungal infection. Ultimately, given the progressive aggravation of the infection and the ineffectiveness of antifungal and antibiotic agents, an orbital exenteration was performed. A mycobacteriological examination revealed a polymicrobial culture with Mucorales. One year after the initial presentation, the patient is alive and with a good general health condition.

Moyamoya Syndrome after Radiation Therapy: a Clinical Report.

Moyamoya syndrome (MMS) is a rare, chronic and progressive vasculopathy with a characteristic angiographic pattern and well-recognized predisposing conditions, such as cranial therapeutic radiation. We report the case of a 36-year-old Caucasian female with a history of craniopharyngioma treated with whole-brain radiotherapy 20 years previously. She was admitted to the emergency department with disorientation and imperceptible speech lasting for 1 hour. Upon examination, she had slight motor aphasia, without sensory or motor deficits. However, the neurological deficits worsened on standing position. The computed tomography (CT) angiogram and transcranial Doppler ultrasonography revealed occlusion of the distal portion of the left internal carotid artery (ICA). Mechanical thrombectomy was attempted without success. Head CT was repeated, revealing left periventricular acute ischaemic stroke. The cerebral angiography showed total occlusion of the left ICA with an exuberant network of transdural collateral vessels, confirming MMS. The patient completed a functional rehabilitation program with progressive improvement of deficits and maintained a multidisciplinary follow-up. MMS is a serious late complication from cranial radiation therapy and a well-described risk factor for ischaemic stroke in younger patients. Therefore, early detection and prompt treatment are mandatory, as the consequences can be disastrous, such as cognitive and neurologic decline due to repeated strokes. LEARNING POINTS: Moyamoya syndrome should be considered in younger patients presenting with acute neurologic deficits, particularly those treated with cranial radiotherapy.The treatment of acute stroke in patients with moyamoya syndrome should include intravenous hydration and avoidance of hypotension.The benefit of antiplatelet agents is limited and equivocal for patients with moyamoya syndrome and oral anticoagulants are not recommended for long-term treatment.

An evaluation of efficacy of the auto-dissemination technique as a tool for Aedes aegypti control in Madeira, Portugal.

BACKGROUND: The frequency and intensity of arboviral epidemics is steadily increasing and posing an intractable public health burden. Current vector control methods are proving ineffectual and despite progress in the development of high technology approaches, there is an urgent need for the development of tools for immediate implementation. Several studies suggest that the auto-dissemination of pyriproxyfen (PPF) is a promising new approach to larviciding although there is little detail on the conditions under which it is optimally effective. Here, we evaluate the efficacy of the approach in urban and rural sites in Madeira, Portugal. RESULTS: Auto-dissemination of PPF through adapted Biogents Sentinel traps (BGSTs) resulted in a modest but consistent impact on both juvenile and adult mosquito populations, but with considerable spatial heterogeneity. This heterogeneity was related to the distance from the BGST dissemination station as well as the local density of adult mosquitoes. There was evidence that the impact of PPF was cumulative over time both locally and with gradual spatial expansion. CONCLUSIONS: The density of adult mosquitoes and the spatial distribution of dissemination devices are key factors in mediating efficacy. In addition, urban topography may affect the efficiency of auto-dissemination by impeding adult mosquito dispersal. Further studies in a range of urban landscapes are necessary to guide optimal strategies for the implementation of this potentially efficacious and cost-effective approach to larviciding.

Percepção dos médicos sobre reuniões entre níveis de atenção em uma rede municipal de saúde de Pernambuco, Brasil / Doctors' perception of meetings between levels of care in a municipal health network in Pernambuco, Brazil

O estudo analisa a percepção de médicos da atenção primária(AP)e especializada (AE) sobre a intervenção Reuniões Conjuntas entre Níveis de Atenção em uma rede municipal de saúde de Pernambuco, Brasil. Estudo avaliativo realizado por meio de entrevistas com 86 médicos. Utilizou-se análise univariada e bivariada dos dados. Os resultados demonstraram que79,2%dos profissionais da APe 20,8%da AE referiram ter conhecimento sobre o componente Linha de Cuidado em Diabetes, enquanto73,5% AP e26,5%AEsobre Discussão Conjunta de Casos em Saúde Mental. O principal motivo para utilização da linha de cuidado para profissionais da AP foi dar bom atendimento ao paciente (43,3%), enquanto os da AE referiram padronização da atenção e acompanhamento ao paciente (43,8%). Dificuldades referidas: deficiências dos serviços, realização do apoio diagnóstico e seguimento do tratamento. Verificaram-se percentuais mais elevados quanto à confiança e colaboração na perspectiva dos profissionais da AP. Entretanto, pouco mais de um terço dos médicos afirmaram conhecer o profissional do outro nível de atenção. Os resultados demonstram que a intervenção foi percebida como fundamental para melhoria da articulação e cooperação entre AP e AE com fomento ao desenvolvimento da percepção da forma de atuação e implicação dos profissionais nesse processo.

The studyanalyzes the perception of doctors in primary(PC)and secondary care(SC)about the intervention Joint Meetings between Health Care Levels in a municipal health care network of Pernambuco, Brazil. Evaluative studycarried out through interviews with 86 doctors. Univariate and bivariate data analysis was performed. Results demonstrated that 79.2% of PC professionals and 20.8% of SC reported knowledge about Diabetes Shared Care Guidelinescomponent, while 73.5% PC and 26.5% SC about Joint Discussions of Clinical Cases in Mental Health. According to PC professionals, the main reason to using the care guidelines was to provide good patient care (43.3%) whilst standardization of care and patient follow-up (43.8%) were reported by the SC professionals. As difficulties: deficiencies in services, exams and continuity of treatment.Higher percentages related to trust and collaboration in the perspective of PC professionals were achieved. However, near a third of doctors referred to know the professional at the other level of care. The results demonstrate that the intervention was perceived as essential to improving articulation and cooperation between PC and SC towards a perception development of the performance way and professionals' involvement in this process.