The patient, a 20-day-old male, was admitted due to respiratory distress that had persisted for 20 days after birth. The main clinical manifestations included gradually worsening respiratory distress and edema. The patient received treatment including mechanical ventilation and diuretics. Echocardiography indicated cardiomegaly, pulmonary hypertension, and heart failure. A comprehensive systemic examination revealed a significant blowing vascular murmur upon auscultation over the anterior fontanelle and bilateral temporal regions. Further imaging studies including cranial magnetic resonance imaging, magnetic resonance angiography, and magnetic resonance venography showed marked dilation of the superior sagittal sinus, transverse sinus, and sigmoid sinus, leading to a definitive diagnosis of dural arteriovenous fistula. After a multidisciplinary consultation, the patient underwent cerebral angiography and partial embolization of the left parietal arteriovenous fistula. Postoperatively, the patient was treated with positive inotropes, diuretics, and fluid restriction. Ultimately, the patient was weaned off the ventilator and discharged in improved condition. This article reports a case of neonatal dural arteriovenous fistula presenting with respiratory distress and discusses the multidisciplinary approach to managing this condition, which aids in early disease recognition and guides clinical decision-making.
Humans ; Male ; Infant, Newborn ; Central Nervous System Vascular Malformations/diagnosis* ; Respiratory Distress Syndrome, Newborn/etiology* ; Embolization, Therapeutic

OBJECTIVES: Digital subtraction angiography (DSA) is the current gold standard for diagnosing spinal dural arteriovenous fistulas (SDAVF). However, DSA is invasive and associated with risks such as ionizing radiation and iodine contrast allergy. Contrast-enhanced magnetic resonance angiography (CE-MRA) with high temporal/high spatial resolution allows dynamic multiphase contrast-enhanced imaging with excellent detail. This study aims to evaluate the diagnostic value of spinal CE-MRA with high temporal/high spatial resolution for SDAVF. METHODS: Clinical data were retrospectively collected from patients who underwent both conventional spinal MRI and high temporal/high spatial resolution CE-MRA at Xiangya Hospital between January 1, 2021, and January 1, 2024, and who subsequently underwent DSA or surgery within 90 days. Two experienced radiologists independently reviewed all conventional MRI and CE-MRA images. The sensitivity and specificity of conventional MRI and CE-MRA for diagnosing SDAVF were calculated against the gold standard DSA findings. Kappa statistics were used to evaluate the consistency of MRI and CE-MRA compared to DSA. The diagnostic value was further assessed by calculating the area under curve (AUC) of the receiver operating characteristic (ROC). RESULTS: A total of 60 patients were included, of whom 47 were diagnosed with SDAVF and 13 were not. Conventional MRI had 3 false negatives and 1 false positive; CE-MRA had 2 false positives and 0 false negative. The sensitivity and specificity of conventional MRI were 93.62% and 92.31%, respectively. CE-MRA demonstrated 100% sensitivity and 84.62% specificity. The main cause of false positives was the misidentification of posterior spinal arteries as feeding arteries. CE-MRA clearly displayed most feeding arteries, and the accuracy of fistula localization was 74.47% (35/47). Kappa values for conventional MRI and CE-MRA were 0.814 and 0.896, respectively (both P<0.001), indicating good agreement, with CE-MRA outperforming conventional MRI. The AUCs for diagnosing SDAVF were 0.930 for conventional MRI and 0.923 for CE-MRA (both P<0.05). CONCLUSIONS Spinal CE-MRA with high temporal/high spatial resolution is a reliable, non-invasive imaging technique with high sensitivity for diagnosing SDAVF. It can clearly visualize feeding arteries and provides valuable preoperative diagnostic and localization information to support DSA or surgical planning.
Humans ; Central Nervous System Vascular Malformations/diagnosis* ; Male ; Female ; Retrospective Studies ; Middle Aged ; Magnetic Resonance Angiography/methods* ; Angiography, Digital Subtraction/methods* ; Aged ; Adult ; Sensitivity and Specificity ; Contrast Media ; Magnetic Resonance Imaging/methods*

OBJECTIVE: To compare the clinical effect of microsurgery and endovascular embolization in the treatment of spinal dural arteriovenous fistula (SDAVF) by meta-analysis. METHODS: A systematic review was performed to retrieve all relevant literature about surgical treatment or endovascular embolization of SDAVF up to December 2019 through PubMed, Embase, Web of Science, Cochrane Central Register of Controlled Trials Results, CNKI, Wanfang Data, and SinoMed. The Chinese and English key words included: "SDAVF", "spinal dural arteriovenous fistula", "spinal AVM", "spinal vascular malformation and treatment". The included studies were evaluated using the Newcastle-Ottawa scale. The early failure rate, long-term recurrence, neurological recovery, and complications were evaluated and the clinical effects of the two methods in the treatment of SDAVF were compared by using RevMan 5.3 software. And a further subgroup analysis of the therapeutic effect of endovascular embolization with different embolic agents was conducted. RESULTS: A total of 46 studies involving 1 958 cases of SDAVF were included, in which 935 cases were treated by microsurgery and 1 023 cases were treated by endovascular embolization. The funnel plot demonstrated that there was no publication bias. The results of meta-analysis showed that the incidence of early surgical failure was lower than that of endovascular embolization (OR=0.20, 95%CI: 0.13-0.30, P < 0.05), and the long-term recurrence was also lower than that of endovascular embolization (OR=0.36, 95%CI: 0.22-0.58, P < 0.05). The improvement of neurological function in the surgical patients is significantly higher than that in the patients treated with endovascular embolization (OR=2.86, 95%CI: 1.36-5.99, P < 0.05). There was no significant difference in the occurrence of complications in these two groups (OR=1.52, 95%CI: 0.88-2.64, P=0.14). In the cases of endovascular embolization, the risk of treatment failure or recurrence was higher with Onyx glue than with n-butyl 2-cyanoacrylate (NBCA), and the difference was statistically significant (OR=4.70, 95%CI: 1.55-14.28, P < 0.05). CONCLUSION Although the treatment of dural arteriovenous fistulas by intravascular embolization has been widely used, the clinical effect of microsurgery is still better than that of endovascular embolization. Large scale and high-quality randomized controlled trials are required to validate the efficacy and safety of endovascular treatment in SDAVF patients.
Central Nervous System Vascular Malformations/surgery* ; Embolization, Therapeutic/methods* ; Enbucrilate/therapeutic use* ; Endovascular Procedures/methods* ; Humans ; Microsurgery/methods* ; Retrospective Studies ; Treatment Outcome

Arteriovenous Fistula/diagnostic imaging* ; Central Nervous System Vascular Malformations/diagnostic imaging* ; Embolization, Therapeutic ; Humans

Intracranial and spinal dural arteriovenous fistulas (DAVFs) are vascular pathologies of the dural membrane with arteriovenous shunts. They are abnormal communications between arteries and veins or dural venous sinuses that sit between the two sheets of the dura mater. The dura propria faces the surface of brain, and the osteal dura faces the bone. The location of the shunt points is not distributed homogeneously on the surface of the dural membrane, but there are certain areas susceptible to DAVFs. The dura mater of the olfactory groove, falx cerebri, inferior sagittal sinus, tentorium cerebelli, and falx cerebelli, and the dura mater at the level of the spinal cord are composed only of dura propria, and these areas are derived from neural crest cells. The dura mater of the cavernous sinus, transverse sinus, sigmoid sinus, and anterior condylar confluence surrounding the hypoglossal canal are composed of both dura propria and osteal dura; this group is derived from mesoderm. Although the cause of this heterogeneity has not yet been determined, there are some specific characteristics and tendencies in terms of the embryological features. The possible reasons for the segmental susceptibility to DAVFs are summarized based on the embryology of the dura mater.
Arteries ; Brain ; Cavernous Sinus ; Central Nervous System Vascular Malformations ; Colon, Sigmoid ; Dura Mater ; Embryology ; Membranes ; Mesoderm ; Neural Crest ; Pathology ; Population Characteristics ; Spinal Cord ; Veins

Direct puncture and embolization of the transverse sinus (TS) for treatment of dural arteriovenous fistula (DAVF) is typically performed with coils with or without glue. We report a case of DAVF at the left TS that was treated with Onyx embolization via direct puncture of the TS. A 75-year-old woman presented with tremor, festinating gait, and dysarthria. A left TS-DAVF with retrograde superior sagittal sinus and cortical venous reflux (Cognard type IIa+b) was identified on cerebral angiography, and both TSs were occluded with thrombi. We considered that achieving complete cure by transvenous embolization via the femoral vein or transarterial embolization via occipital feeders would be difficult. Thus, we performed a small craniotomy at the occipital bone to puncture the TS. The midportion of the TS was directly punctured with a 21-G microneedle under fluoroscopic guidance. We inserted a 5-F sheath into the TS. A microcatheter was then navigated into the affected sinus. Coils were placed through the microcatheter to support Onyx formation by reducing the pressure of shunting flow. Onyx embolization was performed with the same microcatheter. The DAVF was almost completely occluded except for the presence of minimal shunting flow to the proximal TS. After 1 week, time-of-flight magnetic resonance angiography showed complete resolution of DAVF. The patient showed resolved tremor and markedly improved mental status at 1-month follow up. Direct puncture and embolization of the TS using coils and Onyx is effective and feasible method for the treatment of DAVF when other approaches seem difficult.
Adhesives ; Aged ; Central Nervous System Vascular Malformations ; Cerebral Angiography ; Craniotomy ; Dysarthria ; Female ; Femoral Vein ; Follow-Up Studies ; Gait Disorders, Neurologic ; Humans ; Magnetic Resonance Angiography ; Methods ; Occipital Bone ; Punctures ; Superior Sagittal Sinus ; Tremor

OBJECTIVE: We retrospectively assessed the efficacy of stereotactic radiosurgery (SRS) for dural arteriovenous fistulas (DAVFs) involving the transverse-sigmoid sinus and analyzed the angiographic and clinical results with our 8-year experience.METHODS: Nine patients with intracranial DAVFs involving the transverse-sigmoid sinus underwent SRS using a Gamma Knife® (Elekta Inc., Atlanta, GA, USA) between 2009 and 2016. Five patients underwent SRS for residual DAVFs after embolization and four patients were treated with SRS alone. The median target volume was 1.9 cm3 (range, 0.8–14.2) and the median radiation dose of the target was 17 Gy (range, 16–20). The median follow-up period was 37 months (range, 7–81).RESULTS: Pulsating tinnitus (33%) was the most common symptom. DAVFs were completely obliterated in four patients (44%) and subtotally obliterated in five (56%). Six patients (67%) showed complete recovery of symptoms or signs, and three (33%) showed incomplete recovery. One patient experienced a recurrent seizure. Adverse radiation effects after SRS occurred in one patient (11%). The total obliteration rates after SRS were 16.7%, 37.5%, and 68.7% at 1, 2, and 3 years, respectively. The median interval from SRS to total obliteration of the fistula was 31 months (range, 12–38). The rates at which the symptoms started to improve were 40% at 1 month and 80% at 2 months after SRS. Symptoms started to improve at a median of 5 weeks after SRS (range, 3–21).CONCLUSION: SRS with or without embolization is a safe and effective treatment to relieve symptoms and obliterate DAVFs on the transverse-sigmoid sinus.
Central Nervous System Vascular Malformations ; Fistula ; Follow-Up Studies ; Humans ; Radiation Effects ; Radiosurgery ; Retrospective Studies ; Seizures ; Tinnitus ; Transverse Sinuses

No abstract available.
Abducens Nerve Diseases ; Abducens Nerve ; Central Nervous System Vascular Malformations ; Drainage

Superficial siderosis (SS) of the central nervous system is a rare disease, which is caused by the accumulation of iron from the hemoglobin in the superficial layer of the brain, spinal cord, and central parts of cranial nerves. The etiology of SS is the accumulation of hemosiderin in the subarachnoid space due to chronic or repeated hemorrhage resulting in progressive and irreversible neurological dysfunction. The cause of the disease is aneurysm, trauma, tumor, and vascular malformation. In most cases, the cause of bleeding is unknown. Clinical features include sensorineural hearing loss, cerebellar ataxia, and myelopathy. Until now, magnetic resonance imaging (MRI) has only been diagnosed and there is no standardized treatment. We will investigate clinical features and MRI findings of SS disease in the central nervous system using 2 patient cases.
Aneurysm ; Brain ; Central Nervous System ; Cerebellar Ataxia ; Cranial Nerves ; Dizziness ; Hearing Loss, Sensorineural ; Hemorrhage ; Hemosiderin ; Humans ; Iron ; Magnetic Resonance Imaging ; Rare Diseases ; Siderosis ; Spinal Cord ; Spinal Cord Diseases ; Subarachnoid Space ; Vascular Malformations ; Vertigo

OBJECTIVE: Bilateral cavernous sinus dural arteriovenous fistula (CSdAVF) is very rare, even in Asian countries. The research intended to present clinical and radiologic outcomes of treating such fistulas through endovascular embolization. MATERIALS AND METHODS: Data was obtained from 220 consecutive patients, with CSdAVF, who were treated from January 2004 to December 2015. Bilateral CSdAVF was identified in 17 patients (7.7%). The clinical and radiologic outcomes of the fistulas were assessed with an emphasis on the technical aspects of treatment. RESULTS: At the time of treatment, 7 and 10 patients presented with bilateral and unilateral symptoms, respectively. In the former cases, 4 patients had progressed from unilateral to bilateral symptoms. Bilateral fistulas were treated with a single-stage transvenous embolization (TVE) in 15 patients, via bilateral inferior petrosal sinuses (IPS) (n = 9) and unilateral IPS (n = 6). In the other 2 patients with one-sided dominance of shunting, only dominant fistula was treated. Two untreated lesions were found on follow-up to have spontaneously resolved after treatment of the dominant contralateral fistula. Of the 34 CSdAVF lesions, complete occlusion was achieved in 32 lesions after TVE. Seven patients (41.2%) developed worsening of cranial nerve palsy after TVE. During the follow-up period, 4 patients obtained complete recovery, whereas the other 3 remained with deficits. CONCLUSION: With adjustments of endovascular procedures to accommodate distinct anatomical configurations, endovascular treatment for bilateral CSdAVF can achieve excellent angiographic occlusion results. However, aggravation of symptoms after TVE may occur frequently in bilateral CSdAVF. In the patients with one-sided dominance of shunt, treatment of only dominant fistula might be an alternative option.
Arteriovenous Fistula ; Asian Continental Ancestry Group ; Cavernous Sinus* ; Central Nervous System Vascular Malformations* ; Cranial Nerve Diseases ; Endovascular Procedures ; Fistula ; Follow-Up Studies* ; Humans