Indirect Carotid Cavernous Fistula with Ocular Manifestations: A Case Report.

ABSTRACT: Carotid-cavernous fistulas are rare entity with incidence of less than 1%, refers to abnormal connections between the carotid artery and cavernous sinus. Indirect types usually occur in elderly female patients and can resolve spontaneously with conservative management like external manual compression of the carotid artery. We report a case of 65-year-old female who presented with complaints of redness, proptosis, chemosis, headache and ophthalmoplegia in her right eye. Digital subtraction angiography revealed Barrow type B indirect carotid-cavernous fistulas. External manual carotid compression was done after which her symptoms improved significantly. Thus, indirect type carotid-cavernous fistulas can occur spontaneously and could be a sight threatening condition especially in elderly females but can resolve with conservative management.

Recovery of the hibernating cavernosum by penile revascularization.

Objective: Chronic ischemia-related cavernous dysfunction is considered irreversible. However, in certain patients, cavernous function appears to recover with penile revascularization. In this study, we investigated a potential cavernous dysfunction reversibility from a clinical perspective. Patients and Methods: We involved 93 young patients in the study with arterial erectile dysfunction (ED) (median age: 30 years). Erectile function tests were performed according to the standard operating procedures of the International Society of Sexual Medicine. Among the participants, 63 and 30 displayed pure arteriogenic and mixed vasculogenic (due to both arterial insufficiency and cavernous dysfunction) ED, respectively. Penile revascularization was performed by anastomosing the inferior epigastric artery to the dorsal artery. The ED treatment success was considered from a score of at least 24 on the International Index of Erectile Function-6. Results: Our results proved that penile revascularization cured 92.1% and 73.8% of the patients with pure arteriogenic and mixed vasculogenic ED, respectively (Kaplan-Meier method, log-rank test: no significant difference). The required time for curing 50% of the patients was 10.5 and 10.0 months for pure arteriogenic and mixed vasculogenic ED, respectively, indicating no recovery delay in patients with mixed vascular ED. Furthermore, the cavernous dysfunction degree did not influence cavernous function recovery. Conclusion: Penile revascularization cured ED in 73.8% of the patients with mixed vasculogenic ED. Cavernous dysfunction appears to be reversible in certain cases. Furthermore, we observed no delay in functional recovery compared to participants with healthy cavernous function. These two discoveries suggest that cavernous function recovery after penile revascularization is similar to the concept of hibernating myocardium in ischemic myocardium. Although cavernous dysfunction is considered irreversible, it could be reversed in multiple cases with blood flow restoration to the cavernous tissue.

Neuroendoscopic Surgery for Intraventricular Cavernous Malformations: a Review on Indications and Surgical Considerations.

BACKGROUND: Intraventricular cavernous malformations (IVCs) are rare vascular lesions of the central nervous system. Surgical resection remains a challenging endeavor, with conventional microsurgical techniques associated with morbidity due to direct brain tissue manipulation. Neuroendoscopic approaches offer a minimally invasive alternative, though their efficacy and safety in treating IVCs remain underexplored. METHODS: A narrative review was conducted to analyze all documented cases of IVCs treated exclusively with endoscopic transventricular approaches (ETVA). Reviews, original research papers, and case reports published from 1990 to May 2024 were included. When available, surgical videos were also reviewed. RESULTS: Seventeen patients with IVCs primarily located in the foramen of Monro were identified. The mean size of the IVCs was 17 mm (range 7-29 mm). Neuroendoscopic procedures achieved gross total resection in all cases, leading to the resolution of clinical symptoms. Except for one patient who experienced memory impairment post-surgery, no persistent neurological dysfunctions were observed. Intraoperative bleeding, a significant challenge in IVC resection, was managed with continuous warm irrigation and dedicated coagulation instruments. Additionally, 6 cases of neuroendoscopic procedures such as endoscopic third ventriculostomy (ETV) and septum pellucidotomy were reported for managing hydrocephalus (HCP). CONCLUSION: Neuroendoscopic surgery offers several advantages in treating IVCs, including minimally invasive access, precise visualization, and reduced brain tissue manipulation. Our findings support the efficacy and safety of ETVA, underscoring its potential as a valuable therapeutic strategy for selected IVCs.

Adult diffuse hepatic hemangiomatosis lesion occupying the entire abdominal and pelvic cavities: a case report.

Introduction: Adult diffuse hepatic hemangiomatosis (DHH) is an extremely rare disease. Consequently, its characteristics are poorly understood. Herein, we report a case of adult DHH involving both liver lobes but without extrahepatic involvement. To the best of our knowledge, this the largest reported adult DHH to date. Case presentation: A 51-year-old man was admitted due to abdominal distension and dyspnea. Physical examination revealed marked liver enlargement. Color Doppler, plain and contrast-enhanced computed tomography, and contrast-enhanced magnetic resonance imaging revealed a hepatic lesion sized 35.1 × 32.1 × 14.1 cm occupying nearly the entire abdominal and pelvic cavities. Diagnosis was established by liver puncture biopsy. The patient exhibited clinical signs of portal hypertension and hypersplenism, but remains free of serious DHH-related complications. He is followed up regularly, with proactive evaluation for future liver transplantation. Conclusion: This case will contribute to the current knowledge on the clinical and imaging features of this rare entity.

Synovial sarcoma of the ethmoid sinus with extension to the cavernous sinus: a case report and literature review.

Synovial sarcomas are rare soft tissue tumors primarily affecting the extremities but can occasionally manifest in unusual locations such as the ethmoid sinus, posing diagnostic challenges. We present a case of a 38-year-old male with a 7-month history of recurrent throbbing headaches, left eye pain, and facial nerve palsy, evolving into multiple stroke episodes. Radiological studies showed extension to the cavernous sinus, raising an initial suspicion of vasculitis. Histological findings of an endoscopic biopsy, however, confirmed a monophasic synovial sarcoma. The patient was referred to a specialized center for further management. Unfortunately, he developed another stroke before receiving treatment. Management included chemotherapy and definitive radiation therapy targeting the ethmoid sinus. The patient is currently receiving ongoing palliative care for symptom management. This case underscores the importance of early diagnosis and a multidisciplinary approach in managing rare and aggressive tumors such as synovial sarcoma of the ethmoid sinus.

Delayed Pontomesencephalic and Cervical Cord Venous Drainage Followed by Contralateral Carotid-Cavernous Fistula after Craniofacial Fractures: A Case Report.

A 24-year-old male was admitted with progressive cervical hypesthesia, tetraparesis, dyspnea, and a history of craniofacial fracture. Spinal magnetic resonance imaging (MRI) showed brainstem edema extending to the thoracic spine with multiple prominent perimedullary vascular structures. Cerebral digital-substraction angiography revealed Barrow type A carotid-cavernous fistula. Total occlusion with preservation of internal carotid artery flow was achieved using 1 detachable balloon and 6 coils. Postoperatively, immediate respiratory recovery, gradual extremities strength improvement, and right abducens nerve palsy were found. One month follow-up cervical MRI showed good recovery of spinal cord edema and perimedullary veins.

Circumferential exposure of the cavernous sinus and parasellar region through a combined open and endoscopic endonasal transcavernous approach, a morphometric study.

INTRODUCTION: Although recent trends currently favor the endoscopic endonasal transcavernous approach (EETA) over the pretemporal transcavernous approach (PTA) for certain cavernous sinus pathologies, dedicated assessment of the surgical exposure and maneuverability is wanting. Toward this aim, this morphometric study quantifies these variables within four cavernous sinus compartments by comparing the PTA, EETA, and a combined approach to achieve a circumferential dissection (EETA-PTA). MATERIAL: In five latex-injected specimens, exposure volumes of the EETA, PTA, and circumferential EETA-PTA approaches were quantified; the latter combined the most conservative options of both the endoscopic and open approaches. Two clinical cases illustrate the combined approach. RESULTS: EETA-PTA provided the largest volume of exposure (65.6% vs 35% PTA vs 44.6% EETA, P = 0.01) and eliminated the need to mobilize the ICA or cross cranial nerves. Although EETA and PTA approaches afforded comparable exposure volumes along the entire cavernous sinus (34.9 vs 44.6%), the EETA better exposed medial and inferior compartments (whereas the PTA exposed larger volumes in the lateral and superior compartments. The combined EETA-PTA yielded 66% of total cavernous sinus exposure volumes and eliminated the need to mobilize the ICA or cross cranial nerves. CONCLUSIONS: Our methodology aligns with strategies that use a modular concept to divide the skull base into compartments for maximal safe resection. Excluding soft tumors, the EETA is preferred for medial and inferior lesions and the PTA for superior and lateral lesions. A staged combined EETA-PTA may safely yield a 360-degree access for extensive multi-compartment lesions that span neurovascular structures within the cavernous sinus.

Cryoprobe-Assisted Excision of Cavernous Hemangioma of the Orbit: A Clinical Case Report.

This case report details the management of orbital cavernous hemangioma in a middle-aged female. The treatment involved a medial orbitotomy combined with an innovative application of a cryoprobe. This approach underscores the benefits of open orbitotomy, particularly when combined with cryoextraction. This combination provides improved control over bleeding and results in a substantial reduction in surgical time, offering valuable insights in the context of the contemporary trend towards endoscopic surgeries.

The oculomotor cistern and pituitary adenomas: anatomical and clinical study.

OBJECTIVE: The oculomotor cistern (OMC) is a meningeal cuff filled with CSF that contains the oculomotor nerve (cranial nerve [CN] III) at the level of the lateral wall of the cavernous sinus. Only a few studies have investigated the involvement of the OMC by pituitary adenomas (pituitary neuroendocrine tumors [PitNETs]), mainly with relatively small case series. The aim of this study was to perform a histomorphological description of the OMC and systematically analyze its involvement by PitNETs from radiological, clinical, and surgical perspectives. METHODS: Ten hemisellae from formalin-fixed specimens were studied with 3-µm sections. Digital image analysis software was used for morphological and quantitative assessments. Clinical, radiological, surgical, and histological data of patients undergoing endoscopic transsphenoidal surgery for PitNETs at the University of Brescia, Italy, between 2014 and 2021 were recorded. OMC involvement was graded as not compressed, compressed, and invaded. The same surgical team operated on all patients. RESULTS: The OMC had an elliptical shape with an average area of 3.1 mm2 and a length of 5.5 mm. No cisternal points of weakness were recognized in the histomorphological study. Of 315 patients, 246 had complete data: apoplexy and CN III palsy were documented in 6.9% and 8.5%, respectively. OMC compression and invasion were recorded in 106 (43.1%) and 23 (9.3%) patients. Significant associations between OMC involvement and PitNET dimensions (p < 0.001), Knosp grade (p < 0.001), preoperative oculomotor palsy (p < 0.001), Ki-67 percentage (p = 0.009), and recurrence/progression of residual tumor (p = 0.008) were found. A new postoperative CN III palsy was evident in 2%: transient in 4 cases, and persistent in 1 patient treated for a recurrent PitNET who experienced a local infection complication. Preoperative CN III palsy improved in 10 cases. CONCLUSIONS: Significant OMC involvement by PitNETs might be underrecognized, but it can be treated using the endoscopic transsphenoidal approach, and it affects patient outcomes.

Early and long-term outcome of surgical versus conservative management for intracranial cerebral cavernous malformation: Meta-analysis of reconstructed time-to-event data.

BACKGROUND: Cerebral cavernous malformations (CCMs) present challenges in clinical management due to a lack of definitive evidence from clinical trials. Surgical intervention and observational management are commonly used, yet their efficacy and long-term outcomes remain controversial. OBJECTIVE: This meta-analysis evaluates the effectiveness of surgical intervention versus conservative management in patients with symptomatic CCMs over various time frames to determine optimal treatment strategies. METHODS: A systematic review and reconstructed time-to-event meta-analysis were conducted, following PRISMA guidelines. Data from selected studies comparing surgical intervention to conservative management for CCMs were analyzed using pooled patient data from Kaplan-Meier curves. New focal neurological deficit (FND) or intracranial hemorrhage (ICH) were the outcome metrics. RESULTS: Four eligible studies, comprising 290 patients, were included. Surgical intervention showed 43 events over a mean time to FND/ICH of 6.372 years (95 % CI: 3.536-8.005), while observational management had 48 events with a significantly longer mean time of 10.992 years (95 % CI: 6.070-8.005). No significant difference was found at 2 years (p = 0.910), but at 5 and 10 years, surgical intervention had more events and shorter mean times (p < 0.0001). Sensitivity analysis for previously bleeding CCMs showed no significant difference in events (p = 0.131). CONCLUSION: This meta-analysis suggests observational management may achieve favorable long-term outcomes for symptomatic CCMs. Despite ongoing controversies, the findings highlight the need for further research, particularly randomized controlled trials, to refine treatment strategies and optimize patient care.

Stereotactic Radiosurgery for Intracranial Cavernous Malformations: International Stereotactic Radiosurgery Society, Systematic Review, Meta-Analysis, and Practice Guidelines.

OBJECTIVE: The International Stereotactic Radiosurgery Society (ISRS) aims to establish evidence-based guidelines for single-fraction stereotactic radiosurgery (SRS) in treating intracranial cavernous malformations. METHODS: We conducted a systematic review following PRISMA and MOOSE guidelines, searching electronic databases up to January 2024 to assess SRS's impact on post-treatment hemorrhage rates. Pooled risk ratios (RR) and confidence intervals were utilized to quantify this effect, along with assessments of lesion volume changes, seizure outcomes, and SRS-related adverse effects. RESULTS: Our meta-analysis included 32 studies with 2672 patients. A significant decrease in annual hemorrhage rates was observed post-treatment (RR=0.17), with rates of RR=0.29 in the first 2 years and RR=0.11 thereafter. Hemorrhage rates significantly differed before and after 2 years post-SRS (RR=0.36). Among epileptic patients, 20.2% had epilepsy pre-treatment, and 49.9% were seizure-free post-SRS, while 30.6% experienced reduced seizure frequency. Lesion volume changes showed a reduction in 46.9%, stability in 47.1%, and an increase in 6.7%. Symptomatic radiation effects affected 8% of patients. Subgroup analysis revealed symptomatic change rates of 6% at doses ≤13Gy compared to 9% at doses >13Gy. Permanent clinical deficits were rare (2%). CONCLUSION: This meta-analysis suggests SRS is an effective intervention for intracranial cavernous malformations, significantly reducing hemorrhage rates and improving seizure outcomes. ISRS practice guidelines are provided.

Nontraumatic intra-diploic arachnoid cyst communicating with sphenoid bone and in close proximity to cavernous sinus in a known case of Wilson disease: A rare entity.

Arachnoid cysts can be intra-cranial or along the spinal cord. Intracranial arachnoid cyst is a very rare finding, trauma being the leading case of it. It is extremely rare for the development of intra-diploic arachnoid cyst without a traumatic history. We present a case of an intra-diploic arachnoid cyst communicating with the greater wing of the sphenoid and in close proximity to the cavernous sinus in a known case of Wilson disease for 22 years. Due to its low incidence, there is a gap in the knowledge and discussion of this cystic lesion, its pathophysiology and management, which are discussed in this case report.

A potential treatment for erectile dysfunction: Effect of platelet-rich plasma administration on axon and collagen regeneration in cavernous nerve injury.

Recent studies highlighted the role of platelet-rich plasma (PRP) in progenitor cell homing, migration, and nerve cell regeneration while also inhibiting fibrosis and apoptosis in cavernous nerve injury (CNI). The aim of this study was to investigate the effect of PRP administration on axon and collagen regeneration in CNI. A true experimental study using a post-test-only control group design was conducted. Twenty-five male Wistar rats (Rattus norvegicus), weighing 200-300 grams, were divided into five groups: two control groups (sham procedure and negative control), and three experimental groups receiving local PRP, intraperitoneal PRP, and a combination of local and intraperitoneal PRP. The cavernous nerve was injured with a hemostasis clamp for one minute before 200 µL of 200 PRP was injected locally, intraperitoneally, or both, depending on the group. After four weeks, the rats were euthanized, tissue segments (2 mm) from each cavernous nerve and mid-penis were collected and analyzed for collagen density, axon diameter, and number of myelinated axons. Our study found that collagen growth was slower in CNI group without PRP (sham procedure) compared to all PRP groups (local, intraperitoneal, and combination). The intraperitoneal PRP group had the highest collagen density at 5.62 µm; however, no significant difference was observed in collagen density among all groups (p=0.056). Similar axon diameter was found across the groups, with no statistically significant difference observed (p=0.856). In the number of myelinated axons, a significant difference was found among all groups with significantly more axons in local PRP and combined local and intraperitoneal PRP groups compared to others (p=0.026). In conclusion, PRP administration improved the number of myelinated axons in CNI, suggesting PRP role in CNI regeneration and the potential for an innovative approach to treating erectile dysfunction associated with CNI.

Experience in the diagnosis and treatment of non-invasive bilateral carotid cavernous sinus fistula based on CT image examination.

Carotid cavernous fistula is a rare but clinically important vascular abnormality that is challenging to diagnose and treat. The clinical data of a patient with bilateral carotid cavernous fistula diagnosed by CT images were retrospectively analyzed. Through the analysis of CT images, the patient was accurately located and the diagnosis was confirmed. CT images can provide detailed anatomical information and accurately show the location, morphology and hemodynamic characteristics of carotid cavernous fistula. Through CT image examination, we successfully diagnosed bilateral carotid cavernous fistula patients, and can provide an important reference for surgical treatment. Therefore, CT image examination can provide accurate diagnosis and surgical planning information, and provide support for the formulation of individual treatment plans for patients. The application of this method is helpful to improve the early diagnosis rate and treatment effect of carotid cavernous fistula.

Bilateral Carotid-Cavernous Fistula Following Traumatic Fall: A Case Report.

Carotid-cavernous fistulas (CCFs) are abnormal connections between the carotid arteries and the cavernous sinus, posing significant neuro-ophthalmologic risks. This report presents a rare case of bilateral post-traumatic CCFs, focusing on clinical presentation, diagnosis, and management. Symptoms mimic conjunctivitis, causing diplopia, exophthalmos, and ophthalmoplegia. Diagnosis relied on computed tomography, magnetic resonance angiography, and digital subtraction angiography. Management involved transarterial embolization with coils, achieving successful outcomes. This highlights the importance of timely intervention and comprehensive imaging to prevent complications. Teaching point: This case report details a rare instance of bilateral post-traumatic carotid-cavernous fistulas, emphasizing clinical presentation, diagnostic evaluation, and management.

Accurate brain pharmacokinetic parametric imaging using the blood input function extracted from the cavernous sinus.

Brain pharmacokinetic parametric imaging based on dynamic positron emission tomography (PET) scan is valuable in the diagnosis of brain tumor and neurodegenerative diseases. For short-axis PET system, standard blood input function (BIF) of the descending aorta is not acquirable during the dynamic brain scan. BIF extracted from the intracerebral vascular is inaccurate, making the brain parametric imaging task challenging. This study introduces a novel technique tailored for brain pharmacokinetic parameter imaging in short-axis PET in which the head BIF (hBIF) is acquired from the cavernous sinus. The proposed method optimizes the hBIF within the Patlak model via data fitting, curve correction and Patlak graphical model rewriting. The proposed method was built and evaluated using dynamic PET datasets of 67 patients acquired by uEXPLORER PET/CT, among which 64 datasets were used for data fitting and model construction, and 3 were used for method testing; using cross-validation, a total of 15 patient datasets were finally used to test the model. The performance of the new method was evaluated via visual inspection, root-mean-square error (RMSE) measurements and VOI-based accuracy analysis using linear regression and Person's correlation coefficients (PCC). Compared to directly using the cavernous sinus BIF directly for parameter imaging, the new method achieves higher accuracy in parametric analysis, including the generation of Patlak plots closer to the standard plots, better visual effects and lower RMSE values in the Ki (P = 0.0012) and V (P = 0.0042) images. VOI-based analysis shows regression lines with slopes closer to 1 (P = 0.0019 for Ki ) and smaller intercepts (P = 0.0085 for V). The proposed method is capable of achieving accurate brain pharmacokinetic parametric imaging using cavernous sinus BIF with short-axis PET scan. This may facilitate the application of this imaging technology in the clinical diagnosis of brain diseases.

Cavernous Malformations of the Central Nervous System: A Comprehensive Review of Pathophysiology, Diagnosis, and Management.

Cavernous malformations (CMs) of the central nervous system (CNS) are vascular anomalies characterized by clusters of dilated, thin-walled blood vessels prone to leakage and hemorrhage. These malformations can occur throughout the CNS, including the brain and spinal cord, and present with a wide range of clinical manifestations, from asymptomatic cases to severe neurological deficits. Advances in neuroimaging, particularly magnetic resonance imaging (MRI), have greatly improved the diagnosis and understanding of CMs, enabling more precise differentiation from other vascular lesions. The management of CMs has evolved alongside advancements in surgical and radiosurgical techniques, offering various therapeutic options depending on the lesion's characteristics and patient symptoms. While conservative management is often appropriate for asymptomatic or minimally symptomatic lesions, surgical resection or stereotactic radiosurgery may be indicated in cases with recurrent hemorrhage or significant neurological impairment. This comprehensive review explores the pathophysiology, clinical presentation, diagnosis, and management of CMs, highlighting current evidence-based practices and emerging therapeutic approaches. The review also addresses the genetic and molecular underpinnings of CMs, particularly in hereditary cases, and discusses potential future directions in research and treatment. By synthesizing the latest knowledge in the field, this review aims to enhance clinical decision-making and promote further investigation into the optimal management of CMs in the CNS.

Cavernous Angioma: A Rare Cause of Multiple Cranial Nerve Palsies.

Acute onset of neurological deficit is highly suggestive of stroke; in such cases, computed tomography (CT) brain is the initial choice of investigation. While CT brain can differentiate between hemorrhagic and ischemic infarct, more often than not, it is unable to detect the underlying etiology of intracerebral hemorrhage. In these situations, magnetic resonance imaging (MRI) brain is crucial in determining the exact etiology and helps us tailor the specific management best suited for our patient. The case under discussion is of a 48-year-old male who presented with multiple cranial nerve palsies and ipsilateral cerebellar involvement in whom CT brain revealed a hemorrhage involving left hemipons and left middle cerebellar peduncle while an MRI brain revealed an unexpected cavernous angioma which changed the management and prognosis of the patient, proving its superiority over CT brain.

Abdominal Pain Mimicking a Neurological Disorder: A Case Report of Spinal Cavernous Malformation in a Pediatric Patient.

We present a case of a 13-year-old boy with abdominal pain initially misdiagnosed as gastrointestinal in origin. Despite initial outpatient management, his symptoms rapidly deteriorated, revealing a central-medullary cavernous malformation causing spinal cord compression. This case underscores the importance of a comprehensive pediatric examination and highlights new treatment approaches for spinal cavernous malformations.

Intramedullary cavernous malformation: A case report.

Cavernous malformations are rare vascular anomalies of the central nervous system, occurring in the spinal cord in just 5% of cases. Despite being documented in the literature, intramedullary cavernous malformations are exceedingly rare and often challenging to distinguish from other intramedullary lesions. We report a case of a 42-year-old patient with back pain, right-sided dysesthesias, and impaired proprioception in the distal limbs for approximately 3 months. Magnetic resonance imaging, crucial for differential diagnosis, identified intramedullary cavernous malformations at T11-12. Several conditions can hide the real cause of back pain; however, magnetic resonance imaging can reveal common conditions (such as discal hernia) and rare findings like cavernous malformations. Magnetic resonance imaging remains the study of choice for diagnosing and characterizing intramedullary cavernous malformations.