Spontaneous spinal epidural hematomas (SSEH) is a rare entity with an estimated incidence of 0.1 in 100,000, defined as a an epidural hematoma without any known inciting events such as trauma or an iatrogenic procedure. Currently, there are no local data pertaining to this disease entity. This paper presents a case of a 52 year old male, without any known comorbid coagulopathies or vasculopathies, and no preceding history of trauma or surgical procedures with sudden onset right-sided radicular pain and hemiparesis with left-sided hemianesthesia with spinal MRI findings of a spinal epidural hematoma but no vascular malformations seen on spinal angiogram. He was managed non-surgically, given short-course steroids, and sent home after 7 days in the hospital. There was no noted progression of his baseline deficits nor any recurrence of another acute spinal event. Upon follow up after 6 months, there was noted resolution of weakness and with only residual mild and patchy sensory deficit on the left. Traditionally treated surgically, this case reports highlights the role of conservative management in this rare condition with good functional outcome.

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

Vascular malformations are aberrant proliferations of blood vessels that may present at birth or develop subsequently. Capillary malformations (CMs) represent the most common category of vascular malformations. They generally present as localized discoloration, including port-wine stains (PWSs) and telangiectasia. Certain PWSs, especially those affecting the V2 dermatome, may give rise to pyogenic granulomas or other epithelial or mesenchymal hamartomas.

We present a case of a 46-year-old male with a 5-year history of a solitary, progressively enlarging firm papule on an erythematous patch or “birthmark” on his left cheek. Despite infrequent manipulation, there were no reports of bleeding or any associated symptoms, including pain, pruritus, and thrills. The patient sought consultation for cosmetic reasons and wanted to have the lesion removed. Hence, an excision biopsy revealed a well-defined proliferation of mature thick and thin-walled vessels, lined by a single layer of endothelial cells, surrounded by dysmorphic and irregularly arranged vessels consistent with arteriovenous malformation with capillary malformation.

Capillary malformation (CM) is the most common form of vascular malformation. Fifty percent of port-wine stains (PWSs), which is the most common (CM) in children, are in the face area innervated by the second branch of the trigeminal nerve. Arteriovenous malformations manifest in the advanced stages of PWSs and, alongside pyogenic granuloma, represent underreported histological changes inside mature PWSs, especially in the face area innervated by the second branch of the trigeminal nerve (V2).

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*

Humans ; Pulmonary Atresia/therapy* ; Pregnancy ; Female ; China ; Consensus ; Fetal Heart ; Ultrasonography, Prenatal ; Heart Defects, Congenital/therapy* ; Ventricular Septum ; Cardiac Surgical Procedures/methods*

Congenital heart disease is the most common birth defect, affecting 1%–1.2% of live born infants. Pulmonary atresia with intact ventricular septum (PA-IVS) accounts for <1% of all total heart defects. The cause of PA-IVS has been unclear. Thus, experience for prenatal diagnosis of PA-IVS is limited in any single institution. This is the case of a 28-year-old gravida 1 para 0 who came in at 34 + 5 weeks of gestational age. Fetal two-dimensional (2D) echocardiography revealed Type II PA-IVS, higher risk for univentricular circulation postnatally. She gave birth at term by vaginal delivery, with confirmed findings through a 2D echocardiography. Prenatal diagnosis of PA-IVS allows options for the termination of pregnancy, fetal cardiac interventional therapy, early postnatal initiation of prostaglandin E1, and planned early neonatal interventional surgeries for palliation and repair. Early assessment of fetal cardiac features is useful for a better outcome.

Vascular anomalies consist of tumours or malformations made up of abnormal growth or collections of blood vessels that can result in functional or cosmetic problems. While many vascular anomalies are present at birth, some do not appear until later in life, making diagnosis more challenging. Although many vascular anomalies are benign, some are associated with serious complications and may involve multiple organ systems. This article highlights the important features of clinically significant vascular anomalies to help physicians promptly identify and refer these cases to a specialised multidisciplinary team for evaluation and management. The discussion includes the various presenting complaints of vascular anomalies in children, namely, rapidly growing birthmarks, painful lesions, seizures/neurological manifestations, bleeding diathesis, cardiac/airway abnormalities and part of an overgrowth syndrome.
Infant, Newborn ; Child ; Humans ; Vascular Malformations/pathology* ; Vascular Diseases ; Syndrome

Vascular malformations are due to abnormal development of blood and/or lymphatic vessels during embryonic life without endothelial cell proliferation. Most of the previous treatments were symptomatic methods as surgery and sclerotherapy because the pathogenic mechanism was not clearly understood. With advances in molecular biology, the pathogenesis of vascular malformations is thought to be related to inherited and/or somatic mutations that eventually activate the PI3K/ATK/mTOR, Ras/Raf/MEK/ERK pathways. Also, related studies have promoted the use of targeted inhibitors. This article provides a review of current causative genes and targeted drugs for pediatric vascular malformations, aiming to provide a basis for promoting accurate molecular diagnosis and precision targeted therapy for these diseases.
Humans ; Child ; Cell Proliferation ; Vascular Malformations/therapy*

Vascular malformations are due to abnormal development of blood and/or lymphatic vessels during embryonic life without endothelial cell proliferation. Most of the previous treatments were symptomatic methods as surgery and sclerotherapy because the pathogenic mechanism was not clearly understood. With advances in molecular biology, the pathogenesis of vascular malformations is thought to be related to inherited and/or somatic mutations that eventually activate the PI3K/ATK/mTOR, Ras/Raf/MEK/ERK pathways. Also, related studies have promoted the use of targeted inhibitors. This article provides a review of current causative genes and targeted drugs for pediatric vascular malformations, aiming to provide a basis for promoting accurate molecular diagnosis and precision targeted therapy for these diseases.
Humans ; Child ; Cell Proliferation ; Vascular Malformations/therapy*

Humans ; May-Thurner Syndrome ; Coronary Restenosis ; Constriction, Pathologic/surgery* ; Stents/adverse effects* ; Iliac Vein ; Treatment Outcome ; Retrospective Studies