Application of novel non-invasive ophthalmic imaging to visualize peripapillary wrinkles, retinal folds and peripapillary hyperreflective ovoid mass-like structures associated with elevated intracranial pressure.

Background: Elevated intracranial pressure (ICP) is a serious and potentially life-threatening condition, for which clinically useful non-invasive measures have been elusive, in some cases due to their inadequate sensitivity and specificity. Our aim was to evaluate novel non-invasive ophthalmic imaging of selected pathological features seen in elevated ICP, namely peripapillary hyperreflective ovoid mass-like structures (PHOMS), peripapillary wrinkles (PPW) and retinal folds (RF) as potential biomarkers of elevated ICP. Methods: This single-center pilot study included subjects with untreated or incompletely treated high ICP. The retinas of these subjects were evaluated with averaged en-face optical coherence tomography (OCT), OCT retinal cross-sections (OCT B-scans), adaptive optics scanning light ophthalmoscopy (AOSLO), and fundus photos. Results: Seven subjects were included in the study. 6 subjects with high ICP (5 idiopathic intracranial hypertension, 1 medication induced, 30.8 ± 8.6 years, 75% female, 5 with papilledema) and 1 control (20-25 years) were included. PHOMS, PPW and RF were present in all subjects with papilledema, but neither in the high ICP subject without papilledema nor in the control subject. Averaged en-face OCT scans and AOSLO were more sensitive for PPW and RF than OCT B-scans and commercial fundus photos. Conclusion: PPW, RF and PHOMS volume have potential as non-invasive biomarkers of ICP. Novel imaging modalities may improve sensitivity. However, lack of automated image acquisition and processing limits current widespread adoption in clinical settings. Further research is needed to validate these structures as biomarkers for elevated ICP and improve clinical utility.

Unilateral Hypertrophy of the Tensor Fasciae Latae Muscle: A Case Report.

Unilateral hypertrophy of the Tensor Fasciae Latae (TFL) muscle is a rare condition often characterized by a palpable mass in the lower limbs or hip pain. Despite its rarity, several causative factors have been identified, necessitating accurate diagnosis and appropriate management. Here, we present the case of a 53-year-old patient who sought outpatient consultation for a mass in the anterolateral aspect of the right thigh. Through this case study, we aim to contribute to the limited literature on this condition by discussing our diagnostic approach, management plan, and outcomes. Upon presentation, the patient underwent a thorough physical examination, revealing a non-tender, sessile mass seemingly originating in the deep connective tissue of the thigh. A magnetic resonance image (MRI) was performed to confirm the diagnosis and assess the extent of muscle involvement. This noninvasive modality provided valuable insights into the nature and localization of the mass, providing the diagnosis and guiding subsequent management decisions. Given the benign nature of the condition and absence of associated symptoms, conservative management was favored. Physical therapy focusing on stretching and strengthening exercises was initiated to address the underlying probable causes and improve functional capacity. Close monitoring through regular follow-up appointments was also recommended to track the progression of the hypertrophy and ensure symptomatic relief. Unilateral hypertrophy of the TFL muscle is a rare entity that presents diagnostic and management challenges. Through our case study, we have highlighted the importance of a comprehensive diagnostic workup, including imaging studies, in confirming the diagnosis and guiding management decisions. Conservative approaches, such as physical therapy, can effectively manage symptoms and improve quality of life in affected individuals. Continued research and documentation of cases are essential to expand our understanding of this condition and refine treatment strategies.

Case report: Splenic inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS): a trial of immunotherapy and review of the literature.

Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) is a rare malignancy with fewer than 150 cases in the literature. IPT-like FDCS follows an indolent course with most cases definitively managed with surgical resection. We present a case of IPT-like FDCS with multiple recurrences with a trial of immunotherapy. The patient initially presented with splenic involvement requiring splenectomy, subsequently recurring in the liver requiring hepatic resections. Afterwards, there was recurrence with pelvic/small bowel involvement for which treatment was trialed with ipilimumab and nivolumab. The patient progressed despite dual immune checkpoint inhibitor therapy requiring a small bowel resection. To date, this is the first case of immunotherapy use in IPT-like FDCS. Therefore, more evidence is needed to support additional treatments in recurrent IPT-like FDCS after resection.

Role of neuroimaging markers on predicting of idiopathic intracranial hypertension.

BACKGROUND: The goals of neuroimaging in idiopathic intracranial hypertension (IIH) are the exclusion of mimickers and effective management of disease. In recent studies, several imaging markers have been identified as potential predictors of IIH. PURPOSE: To investigate the predictive roles of novel radiological markers as the Meckel's cave area, alongside classical radiologic markers in identifying IIH such as the empty sella. MATERIAL AND METHODS: The patients were classified according to cerebrospinal fluid (CSF) opening pressure as the IIH group and control group. The observational, case-control study included 22 patients with IIH and 22 controls. Groups were compared for presence of empty sella, Meckel's cave area, fat area of posterior neck, fat thickness of scalp, presence of transverse sinus stenosis, and ophthalmic markers, such as increase of optic nerve (ON) sheath diameter. RESULTS: In the IHH group, higher occurrences of increased ON sheath diameter, ON tortuosity, flattening of the scleral surface, and transverse sinus stenosis were observed (P < 0.001, P < 0.001, P = 0.046, and P = 0.021, respectively). Meckel's cave area and fat area of posterior neck were similar in both groups (P = 0.444 and P = 0.794). CONCLUSION: Ophthalmic markers and transverse sinus stenosis could be utilized as radiologic features supporting early and precise diagnosis of IIH. However, enlargement of Meckel's cave area and measurements of fatty area of posterior neck are not helpful for diagnosis of IIH.

A retrospective analysis of the management and surgical treatment of orbital lesions: Outcomes and rationale.

The orbital cavity is a subject of interest for various specialists, and achieving optimal outcomes requires comprehensive, multidisciplinary management. This study aims to report 10 years of experience in the preoperative, surgical, and postoperative care of patients with orbital lesions, examining their clinical, radiological, and anatomopathological features and outcomes. A retrospective review of 125 patients who underwent surgical treatment for intraorbital masses between January 2012 and December 2021 was performed. Outcome measures included postoperative diplopia, exophthalmos, decimal visual acuity, eyeball position, ocular motility, operative time, complications, and aesthetic results. A total of 107 patients were included. All cases were discussed with a neuroradiologist to determine the best therapeutic approach based on preoperative imaging. Preoperative diplopia was linked to extraconal (p = 0.03) and anterior (p = 0.001) lesions, and exophthalmos and visual acuity deterioration were associated with intraconal (p = 0.02; p = 0.03) and retrobulbar (p = 0.001; p = 0.02) lesions. Complications (11.2%) included diplopia, worsened visual acuity, postoperative blepharoptosis, and postoperative ectropion. Of the patients, 80.4% reported an "excellent" aesthetic outcome. This study underscores the importance of a multidisciplinary approach based on a thorough analysis of preoperative imaging. Periorbital approaches tailored to the lesion's three-dimensional location enables safe access to most intraorbital lesions, resulting in minimal complications and good aesthetic results.

MRI of total hip arthroplasty: technical aspects and imaging findings.

Total hip arthroplasty (THA) is the best surgical approach for treating advanced hip degeneration, providing pain relief, and improved function in most cases. In the past, MR imaging quality has been highly compromised by in-plane distortions, inadequate fat saturation, and other artifacts due to metal components of THA. Technological advancements have made pathologic conditions, which were previously hidden by periprosthetic artifacts, outstanding features due to the optimization of several sequences. To date, several short and long-term complications involving bony and soft-tissue structures may be detected through magnetic resonance imaging (MRI). The use of MRI with adapted sequences and protocols may drastically reduce artifacts thereby providing essential pre-operative elements for planning revision surgery of failed THA. This review has the purpose of conveying new insights to musculoskeletal radiologists about the techniques to suppress metal-related artifacts and the hallmark MRI findings of painful THA. CRITICAL RELEVANCE STATEMENT: Advancements in metal-suppression have given radiologists the opportunity to play an emerging role in THA management. This article provides technical and imaging insights into challenges that can be encountered in cases of THA, which may present complications and characteristic imaging findings. KEY POINTS: Imaging total hip arthroplasty requires adapted MRI protocol and awareness of the common complications. We have reported the available metal-suppression sequences for evaluating total hip arthroplasty. Many structures and conditions should be considered when dealing with painful aseptic or septic arthroplasty.

Clinical profile and treatment outcomes of idiopathic intracranial hypertension: a multicenter study from Korea.

BACKGROUND: Currently, there is a relative lack of detailed reports regarding clinical presentation and outcome of idiopathic intracranial hypertension in Asians. This study aims to describe the clinical features and treatment outcomes of Korean patients with idiopathic intracranial hypertension. METHODS: We prospectively recruited patients with idiopathic intracranial hypertension from one hospital and retrospectively analyzed the medical records of 11 hospitals in Korea. We collected data regarding preceding medical conditions or suspected medication exposure, headache phenotypes, other associated symptoms, detailed neuroimaging findings, treatments, and outcomes after 1-2 and 3-6 months of treatment. RESULTS: Fifty-nine (83.1% women) patients were included. The mean body mass index was 29.11 (standard deviation, 5.87) kg/m2; only 27 patients (45.8%) had a body mass index of ≥ 30 kg/m2. Fifty-one (86.4%) patients experienced headaches, patterns of which included chronic migraine (15/51 [29.4%]), episodic migraine (8/51 [15.7%]), probable migraine (4/51 [7.8%]), chronic tension-type headache (3/51 [5.9%]), episodic tension-type headache (2/51 [3.9%]), probable tension-type headache (2/51 [3.9%]), and unclassified (17/51 [33.3%]). Medication overuse headache was diagnosed in 4/51 (7.8%) patients. After 3-6 months of treatment, the intracranial pressure normalized in 8/32 (25.0%), improved in 17/32 (53.1%), no changed in 7/32 (21.9%), and worsened in none. Over the same period, headaches remitted or significantly improved by more than 50% in 24/39 patients (61.5%), improved less than 50% in 9/39 (23.1%), and persisted or worsened in 6/39 (15.4%) patients. CONCLUSION: Our findings suggest that the features of Asian patients with idiopathic intracranial hypertension may be atypical (i.e., less likely obese, less female predominance). A wide spectrum of headache phenotypes was observed. Medical treatment resulted in overall favorable short-term outcomes; however, the headaches did not improve in a small proportion of patients.

Tumor-like conditions that mimic liver tumors.

Non-neoplastic tumor-like conditions of the liver can appear similar to hepatic neoplasms. In many cases, a biopsy is required to confirm the pathology. However, several tumor-like conditions can be correctly diagnosed or suggested prospectively, thus saving patients from unnecessary anxiety and expense. In this image-focused review, we present the ultrasound, computed tomography, magnetic resonance imaging, and positron emission tomography scan features of eight such entities. Clues that indicate the correct pathology are discussed, and the usual clinical setting is described. Many of these lesions are treated differently from true neoplasms, and the current treatment plan is discussed in many of the cases presented. After reviewing this article, the reader will have a better understanding of these lesions and the situations in which they should be included in the differential diagnosis.

Characterisation and Visual Outcomes of Fulminant Idiopathic Intracranial Hypertension: A Narrative Review.

Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative review of fulminant IIH with the aim of better characterising fulminant IIH presentation and visual outcomes. SCOPUS and PubMed were searched for papers referencing IIH, benign intracranial hypertension, or pseudotumour cerebri. Abstracts were screened for rapid degradation in vision. All studies were required to meet both the modified Dandy and fulminant IIH criteria. Thirty-six studies met the inclusion criteria. Demographics, treatments, and visual outcome data were collected. Case studies made up 69% of the studies and 31% were case series. In total, 72 patients with fulminant IIH were reported, of which 23.6% were paediatric and 96% were female. Surgical intervention occurred in 85% of patients. Anaemia was present in 11% of patients and 85.7% of paediatric patients had a sixth cranial nerve palsy. In conclusion, we propose the following practice guidelines to assist in diagnosing and treating fulminant IIH patients: 1) patients who present with optic disc oedema require urgent visual field testing to evaluate for vision loss; 2) a paediatric patient presenting with a sixth cranial nerve palsy should have a comprehensive eye examination; 3) fulminant IIH can occur in patients with a normal body mass index; and 4) anaemia should be tested for in the setting of fulminant IIH. As little is known about the optimal treatment mechanisms for this presentation, multi-institutional and international collaborations will be a critical step for future research.

From Pseudotumor Cerebri to Neurobrucellosis: A Journey With Several Lessons.

We present a case admitted for evaluation of suspected idiopathic intracranial hypertension (IIH) with an unusual but important departure from the expected algorithm. A 31-year-old lady came with a two-week duration of a mild headache and one-week duration of double vision with no previously documented fever or any comorbidities. Clinically, she had papilledema and bilateral abducens palsy with no signs of meningeal irritation. MRI brain radiology was consistent with IIH. Her CSF study showed pleocytosis with elevated protein levels and normal glucose. Serology was positive for Brucella melitensis at low titers but CSF culture grew Brucella melitensis, confirming the diagnosis of neurobrucellosis. Her headache and abducens palsy improved over the first two weeks, and the papilledema resolved over two months with antibiotics. This clinical mimic is important for physicians (including neurophysicians) and Infectious Disease specialists. The radiological mimic comes from chinked (small) ventricles, unlike most meningeal diseases which can present with papilledema and abducens palsy including tuberculosis, cryptococcosis, and leptomeningeal carcinomatosis. A CSF study is mandatory in the workup of IIH despite massive improvements in imaging.

A Rare Inflammatory Myofibroblastic Tumor of the Spleen: A Case Report.

BACKGROUND/AIM: Inflammatory myofibroblastic tumors (IMTs) are rare, solid, potentially malignant lesions of uncertain etiology. Histologically, IMTs exhibit a combination of lymphocytes and inflammatory cells within a fibroblastic myxoid layer. The diagnosis of IMTs poses a challenge for various medical specialties, including surgeons, pathologists, and oncologists, due to their non-specific clinical presentation. Furthermore, radiologists face difficulties in interpreting computed tomography (CT) or magnetic resonance imaging (MRI) results, which often yield polymorphic and inconclusive findings. Ultimately, histopathologists play a crucial role in reaching a definitive diagnosis based on the tumor's histological characteristics. They are detected in every system of the human body, most commonly in the lungs. Here, we report an uncommon occurrence of IMT in the spleen of a patient with nonspecific abdominal pain. CASE REPORT: A 56-year-old Caucasian female presented to Konstantopouleio General Hospital of Nea Ionia, Athens, Greece, with abdominal pain and discomfort. The patient had no significant medical history and normal laboratory tests. An abdominal CT revealed a large mass in the spleen. A splenectomy was performed. Histopathological analysis of the tumor revealed IMTS. CONCLUSION: Splenic IMT is a rare benign tumor with moderate malignant potential. It lacks a distinct clinical presentation and is typically identified either incidentally or during the examination of abdominal pain.

Neurofilament light chain is elevated in patients with newly diagnosed idiopathic intracranial hypertension: A prospective study.

BACKGROUND: Idiopathic intracranial hypertension is a secondary headache disorder potentially causing visual loss. Neurofilament light chain is a candidate, prognostic biomarker, but further studies of neuronal biomarkers are needed. Our objective was to investigate neurofilament light chain in cerebrospinal fluid (cNfL) and plasma (pNfL), amyloid-beta 42 (Aß-42), total-tau and phosphorylated-tau in cerebrospinal fluid in new-onset idiopathic intracranial hypertension. METHODS: Prospective case-control study including new-onset idiopathic intracranial hypertension and age, sex and BMI matched controls. Biomarkers were compared between patients and controls and related to papilledema, visual fields and opening pressure. RESULTS: We included 37 patients and 35 controls. Patients had higher age-adjusted cNfL (1.4 vs. 0.6 pg/mL, p-adjusted < 0.001), pNfL (0.5 vs. 0.3 pg/mL, p-adjusted < 0.001) and total-tau/Aß-42 (0.12 vs. 0.11, p-adjusted = 0.039). Significant, positive linear correlations were found between cNfL, pNfL, total-tau/Aß-42 and opening pressure. Patients with severe papilledema had elevated cNfL compared to mild-moderate papilledema (median cNfL: 4.3 pg/mL (3.7) versus 1.0 pg/mL (1.4), p-adjusted = 0.009). cNFL was inversely associated with perimetric mean deviation (r = -0.47, p-adjusted < 0.001). CONCLUSIONS: cNfL, pNfL and total-tau/Aß-42 were elevated in new-onset idiopathic intracranial hypertension. cNfL was associated with severity of papilledema and visual field defects at diagnosis. This indicates early axonal damage. Neurofilament light chain is a candidate biomarker for disease severity.

Post-COVID-19 Pulmonary Inflammatory Pseudotumors Treated With Steroid Taper.

The aftermath of COVID-19 continues to unveil an array of pulmonary complications, extending beyond the acute phase of the viral infection. Among these emerging sequelae, we present the case of a 58-year-old individual who developed pulmonary inflammatory pseudotumors (PIPs) following recovery from COVID-19. PIPs are exceedingly rare benign lesions that can pose a diagnostic challenge due to their clinical and radiological resemblance to malignant neoplasms. Histologically, PIPs are characterized by a proliferation of myofibroblastic spindle cells accompanied by inflammatory infiltrates, including lymphocytes, plasma cells, and histiocytes. As our understanding of post-COVID-19 complications evolves, this case serves as the first exploration into the complex interplay between COVID-19 infections and the subsequent development of inflammatory pseudotumors. In this report, an investigation is performed into the clinical presentation, diagnostic challenges, and successful management of post-COVID-19 PIPs with a focus on the pivotal role of corticosteroid therapy in mitigating the inflammatory response associated with this unique post-viral entity and resolution of the masses.

Circumferential Pannus at C1-C2 Causing Spinal Cord Compression.

Periodontoid pannus formation is a pathologic condition caused by a multitude of different etiologies, however, it is most commonly due to rheumatoid arthritis. In these cases, the pannus is typically located in the retro-odontoid space ventral to the spinal cord, leading to progressive neural compression. We describe in this report, a patient who presented with progressive high cervical myelopathy, who on imaging revealed both a retro-odontoid pannus and a posterior C1-C2 mass causing severe circumferential compression of the spinal cord. The patient was successfully treated with a C1-C2 laminectomy and occipitocervical fusion. Periodontoid pannus is a common entity; however, the presence of a C1-C2 posterior pannus is a unique finding. To our knowledge, circumferential pannus at C1-C2 causing neural compression is a clinical entity that has not been previously reported.

Lateral micro-orbitotomy as the technique of choice for orbital pseudotumor in IgG4-RD. Case report and review of the literature.

Immunoglobulin G4-related disease (IgG4-RD) is characterized by a systemic fibroinflammatory infiltrate that often involves the orbit in addition to other tissues. Thus it has to be considered in the differential diagnosis of orbital tumors. We report the clinical case of a 64-year-old woman who presented with right mydriasis, progressive proptosis and paralysis of the third cranial nerve of 1 year of evolution. Cranial MRI identified an intraconal lesion of the right orbit, located between the external and inferior rectus muscles and the optic nerve, and she was scheduled for surgery by transcranial approach with lateral micro-orbitomy. A satisfactory macroscopic excision was achieved with no remarkable complications and a definitive deferred histological result of pseudotumor by IgG4-RD. Follow-up for 24 months showed no tumor recurrence, and the patient clinically improved from ophthalmoplegia. This case highlights the efficacy of lateral orbitotomy in the etiologic diagnosis and successful therapeutic outcome of complex orbital lesions associated with IgG4-RD pseudotumor.

A case report of IgG4-related hepatic inflammatory pseudotumor in a 3-year old boy.

Background: Hepatic Inflammatory Pseudotumor (IPT) is an infrequent condition often masquerading as a malignant tumor, resulting in misdiagnosis and unnecessary surgical resection. The emerging concept of IgG4-related diseases (IgG4-RD) has gained widespread recognition, encompassing entities like IgG4-related hepatic IPT. Clinically and radiologically, corticosteroids and immunosuppressive therapies have proven effective in managing this condition. Case Presentation: A 3-year-old Chinese boy presented to the clinic with an 11-month history of anemia, fever of unknown origin, and a tender hepatic mass. Blood examinations revealed chronic anemia (Hb: 6.4 g/L, MCV: 68.6 fl, MCH: 19.5 pg, reticulocytes: 1.7%) accompanied by an inflammatory reaction and an elevated serum IgG4 level (1542.2 mg/L). Abdominal contrast-enhanced computed tomography unveiled a 7.6 cm low-density mass in the right lateral lobe, while magnetic resonance imaging demonstrated slight hypointensity on T1-weighted images and slight hyperintensity on T2-weighted images, prompting suspicion of hepatic malignancy. A subsequent liver biopsy revealed a mass characterized by fibrous stroma and dense lymphoplasmacytic infiltration. Immunohistochemical analysis confirmed the presence of IgG4-positive plasma cells, leading to the diagnosis of IgG4-related hepatic IPT. Swift resolution occurred upon initiation of corticosteroid and mycophenolate mofetil therapies. Conclusion: This study underscores the diagnostic approach to hepatic IPT, utilizing histopathology, immunostaining, imaging, serology, organ involvement, and therapeutic response. Early histological examination plays a pivotal role in clinical guidance, averting misdiagnosis as a liver tumor and unnecessary surgical interventions.

Intracranial inflammatory pseudotumour related to IgG4: A very rare case.

Background: Intracranial inflammatory pseudotumours (IPT) are rare entities that frequently lead to misdiagnosis with malignant lesions. The identification of these lesions is difficult, but important to avoid inadvertent iatrogenicity and to adjust therapeutic protocols. Case Presentation: We report the case of a 30-year-old man who presented a single tonic-clonic seizure. Brain imaging showed a right frontal lesion with intra and extra axial components. Facing the radiologic presentation, a brain tumor was suspected, thus the patient underwent surgery. Pathological exam concluded to a plasma cell granuloma. A whole-body CT-scan showed only a thoracic aortitis. Complete blood work studies came back negative. The patient was also tested for an array of antibodies among which antinuclear antibodies were positive (blood level superior to 1/100). CSF evaluation revealed clear fluid with normal glucose concentration, normal protein levels and lymphocytic pleocytosis. Finally, IgG-4 plasma levels were elevated which led to the diagnosis of an IgG4-RD. The patient was put under prednisolone with a favorable outcome. Conclusion: IPT have several etiologies, among which IgG4 related disease may be one of the less known as only 2 cases have previously been reported. Herein, we report a new case of a young man who presented for seizures related to an intracranial lesion of an IgG4 related disease. The challenge is to suspect such conditions to avoid unnecessary surgeries.

A Case Report of Inflammatory Myofibroblastic Tumor: A Rare Benign Lung Tumor.

Inflammatory myofibroblastic tumors (IMTs) of the lung are a rare type of mesenchymal tumors that tend to occur more in the lungs of children. They are extremely rare in adults. IMTs require extensive pulmonary resection because they are commonly locally invasive. The key to preventing recurrence is complete resection, and the prognosis is excellent after surgery. We report a case of a patient with an inflammatory pseudotumor of the lung. The patient is a 27-year-old female who presented with a dry cough. A chest radiograph and computed tomography showed a lesion in the left main bronchus and near-total left lung collapse. As surgery was necessary to establish the diagnosis, left pneumonectomy was performed followed by a histological examination of the surgical specimen which confirmed inflammatory pseudotumor.

Hepatic pseudotumor: A diagnostic challenge.

Hepatic pseudotumors are rare lesions of unknown origin, characterized by the proliferation of fibrous connective tissue and inflammatory cell infiltrates. They mimic malignant lesions clinically, and radiologically, given their non-specific clinical and imaging features. The pathophysiology of hepatic pseudotumor is incompletely understood and there are no standardized criteria for diagnosis. Pseudotumors have been reported to develop in various organs in the body with the lung and liver being the most common site. Hepatic pseudotumors develop in patients with underlying triggers of liver inflammation and injury, including infections, autoimmune liver diseases, bile duct injury, or surgery. Hepatic pseudotumors respond well to conservative treatment with antibiotics, and steroids and some may regress spontaneously, thus avoiding unnecessary resection. This condition is rewarding to treat. It is important to recognize pseudotumor as a distinct clinical entity and include it in the differential of liver masses with atypical imaging features.