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*

Dural arteriovenous fistula (D-AVF) at the foramen magnum is an extremely rare disease entity. It produces venous hypertension, and can lead to progressive cervical myelopathy thereafter. On the other hand, the venous hypertension may lead to formation of a venous varix, and it can rarely result in an abrupt onset of subarachnoid hemorrhage (SAH) when the venous varix is ruptured. The diagnosis of D-AVF at the foramen magnum as a cause of SAH may be difficult due to its low incidence. Furthermore, when the D-AVF is fed solely by the ascending pharyngeal artery (APA), it may be missed if the external carotid angiography is not performed. The outcome could be fatal if the fistula is unrecognized. Herein, we report on a rare case of SAH caused by ruptured venous varix due to D-AVF at the foramen magnum fed solely by the APA. A review of relevant literatures is provided, and the treatment modalities and outcomes are also discussed.
Angiography ; Arteries* ; Central Nervous System Vascular Malformations* ; Diagnosis ; Fistula ; Foramen Magnum* ; Hand ; Hypertension ; Incidence ; Rare Diseases ; Spinal Cord Diseases ; Subarachnoid Hemorrhage* ; Varicose Veins*

Persistent primitive trigeminal artery (PPTA) is the most common carotid-basilar anastomosis; on the other hand, persistent primitive otic artery (PPOA) is extremely rare. PPTA is often misdiagnosed as PPOA on cerebral angiography. We present a case of PPTA that mimicked PPOA on cerebral angiography. We further describe the utility of brain computed tomography angiography for differential diagnosis of PPTA from PPOA, together with a review of previous literature.
Angiography ; Arteries* ; Brain ; Central Nervous System Vascular Malformations ; Cerebral Angiography* ; Diagnosis, Differential ; Hand

PURPOSE: To report a case of cavernous sinus dural arteriovenous fistula following Tolosa-Hunt syndrome. CASE SUMMARY: A 64-year-old female with a history of hypertension, presented with blepharoptosis and periorbital pain in the right eye and diplopia. Her right pupil was dilated. She had right exotropia and right hypertropia with inability to elevate, depress, and adduct the right eye. Magnetic resonance imaging including angiography, revealed hyperintensities in the right cavernous sinus consistent with inflammation and no vascular abnormalities. Three days after oral corticosteroid therapy, the pain disappeared. A presumptive diagnosis was Tolosa-Hunt syndrome presenting as a form of complete oculomotor nerve palsy. Two months later, she experienced severe pain in the right periocular area, even though blepharoptosis was resolved and ocular motility was improved. A rapid response to high-dose intravenous corticosteroids was consistent with recurrence of Tolosa-Hunt syndrome. Three months later, she had normal ocular motility, but developed a conjunctival injection, pulsatile orbital bruits, and exophthalmos in the right eye. Cerebral angiography showed a cavernous sinus dural arteriovenous fistula. She received a transvenous coil embolization and her symptoms markedly improved over 2 months. CONCLUSIONS: Tolosa-Hunt syndrome, a granulomatous inflammation in the cavernous sinus may be followed by cavernous sinus dural arteriovenous fistula and should be considered during follow-up.
Adrenal Cortex Hormones ; Angiography ; Blepharoptosis ; Cavernous Sinus* ; Central Nervous System Vascular Malformations* ; Cerebral Angiography ; Diagnosis ; Diplopia ; Embolization, Therapeutic ; Exophthalmos ; Exotropia ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Inflammation ; Magnetic Resonance Imaging ; Middle Aged ; Oculomotor Nerve Diseases ; Orbit ; Pupil ; Recurrence ; Strabismus ; Tolosa-Hunt Syndrome*

Intracranial dural arteriovenous fistula (dAVF) usually results in various problems in the brain. But it can be presented as a myelopathy, which may make early diagnosis and management to be difficult. We recently experienced a case of cervical myelopathy caused by intracranial dAVF. A 60-year-old man presented with a 3-year history of gait disturbance due to a progressive weakness of both legs. Neurological examination revealed spastic paraparesis (grade IV) and Babinski sign on both sides. Magnetic resonance imaging showed serpentine vascular signal voids at C2-T1 on T2-weighted image with increased signal intensity and swelling of spinal cord at C1-C4. We performed a brain computed tomography angiography and found intracranial dAVF with multiple arteriovenous shunts. Venous drainages were noted at tentorial veins and cervical perimedullary veins. After Onyx embolization, the patient showed gradual improvement in motor power and gait disturbance. The venous drainage pattern is a well-known prognostic factor of dAVF. In our case, the intracranial dAVF drained to spinal perimedullary vein, which seemed to result in the ischemic myelopathy. Although it is rare condition, it sometimes can cause serious complications. Therefore, we should keep in mind the possibility of intracranial dAVF when a patient presents myelopathy.
Angiography ; Arteriovenous Fistula ; Brain ; Central Nervous System Vascular Malformations* ; Drainage ; Early Diagnosis ; Gait ; Humans ; Ischemia ; Leg ; Magnetic Resonance Imaging ; Middle Aged ; Neurologic Examination ; Paraparesis, Spastic ; Reflex, Babinski ; Spinal Cord ; Spinal Cord Diseases* ; Spinal Cord Ischemia ; Veins

Cerebral cavernous malformations (CMs) are vascular malformations of the central nervous system, which can be detected in the absence of any clinical symptoms. Nodules and cysts with mixed signal intensity and a peripheral hemosiderin rim are considered brain magnetic resonance imaging (MRI) findings typical of CMs. A 48-year-old man was admitted to our hospital because of abnormal MRI findings without significant neurological symptoms. A cyst with an internal fluid-fluid level was found in the left basal ganglia on the initial brain MRI. We decided to observe the natural course of the asymptomatic lesion with serial MRI follow-up. On MRI at the 5-month follow-up, the cystic mass was enlarged and showed findings consistent with those of cystic CM. Surgical resection was performed and the pathological diagnosis was CM. Our experience suggests that the initial presentation of a CM can be a pure cyst and neurosurgeons should consider the likelihood of CMs in cases of cystic cerebral lesions with intracystic hemorrhage.
Basal Ganglia ; Brain ; Central Nervous System ; Diagnosis ; Follow-Up Studies ; Hemangioma, Cavernous, Central Nervous System* ; Hemorrhage ; Hemosiderin ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Vascular Malformations

Spinal dural arteriovenous fistula (SDAVF) is the most common spinal vascular malformation, however it is still rare and underdiagnosed. Magnetic resonance imaging findings such as spinal cord edema and dilated and tortuous perimedullary veins play a pivotal role in the confirmation of the diagnosis. However, spinal angiography remains the gold standard in the diagnosis of SDAVF. Classic angiographic findings of SDAVF are early filling of radicular veins, delayed venous return, and an extensive network of dilated perimedullary venous plexus. A series of angiograms of SDAVF at different locations along the spinal column, and mimics of serpentine perimedullary venous plexus on MR images, are demonstrated. Thorough knowledge of SDAVF aids correct diagnosis and prevents irreversible complications.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Central Nervous System Vascular Malformations/*diagnosis/epidemiology/etiology ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Spinal Cord Diseases/diagnosis ; Spine/radiography

Diagnosis of traumatic arteriovenous fistula (AVF) is usually delayed because it takes time to be enlarged enough to emerge radiologically or because symptoms occur a few months after the trauma. A 56-year-old woman presented with a newly developed tinnitus immediately after a head trauma. Pulsatile high-frequency tinnitus was heard also by examiner and recorded using a transcanal microphone. Angiography revealed an intracranial dural AVF fed by the middle meningeal artery, draining the superior sagittal sinus on the affected side. After percutaneous transarterial embolization, tinnitus successfully disappeared. The clinical presentation, radiologic and angiographic features, and management are discussed. To our knowledge, this is the first reported case of pulsatile tinnitus caused by AVF developed immediately after a trauma. We emphasize that precise physical examination, laboratory tests, and appropriate radiographic imaging are essential for accurate diagnosis and treatment when a patient presents with pulsatile tinnitus, especially after a head trauma.
Angiography ; Arteriovenous Fistula ; Central Nervous System Vascular Malformations* ; Craniocerebral Trauma* ; Diagnosis ; Female ; Golf* ; Head* ; Humans ; Meningeal Arteries ; Middle Aged ; Physical Examination ; Superior Sagittal Sinus ; Tinnitus*

Dural ateriovenous fistula (DAVF) at the craniocervical junction is rare. We report a patient presenting with brainstem dysfunction as an uncommon onset. Brainstem lesion was suggested by magnetic resonance image study. Angiogram revealed a DAVF at a high cervical segment supplied by the meningeal branch of the right vertebral artery, with ascending and descending venous drainage. Complete obliteration of the fistula was achieved via transarterial Onyx embolization. Clinical cure was achieved in the follow-up period; meanwhile, imaging abnormalities of this case disappeared. Accordingly, we hypothesize that a brainstem lesion of this case was caused by craniocervical DAVF, which induced venous hypertension. Thus, venous drainage patterns should be paid attention to because they are important for diagnosis and theraputic strategy.
Brain Stem* ; Central Nervous System Vascular Malformations ; Diagnosis ; Drainage ; Estrogens, Conjugated (USP)* ; Fistula* ; Follow-Up Studies ; Humans ; Hyperemia ; Hypertension ; Vertebral Artery