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

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

Cerebral Hemorrhage ; pathology ; physiopathology ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; pathology ; physiopathology ; Hemodynamics ; physiology ; Humans ; Infant ; Male ; Risk Factors ; Stroke ; pathology ; physiopathology ; Vascular Malformations ; pathology ; physiopathology

Syringomyelia is a disorder in which a cavity has formed within the spinal cord. Idiopathic syringomyelia is not associated with identifiable causes such as Chari type 1 malformation, spinal cord tumor, vascular malformation, tethered cord, arachnoiditis, hydrocephalus, or previous spinal surgery. The main neurologic symptoms of idiopathic syringomyelia are toe-walking, constipation, incontinence, abnormal reflexes, and lower extremity weakness. Patients may present with various symptoms such as scoliosis, cutaneous markers, pain in the lower extremities or back, or may be asymptomatic. Herein, we report a young child with idiopathic syringomyelia presenting with subtle neck pain. A 23-month-old boy visited the neurologic clinic after 3 months of right occipital area neck pain. He had no history of trauma or central nervous system infection, and neurologic examination results were normal except for right posterior neck hyperesthesia. Brain and spinal magnetic resonance imaging showed an ovoid intramedullary cystic lesion (9.7×5.0×4.7 mm) at C6/7 of the spinal cord. There was no evidence of Chiari malformation or other lesions that can be primary pathologies of syringomyelia. Electromyogram/nerve conduction velocity results were normal. The subject was diagnosed as idiopathic syringomyelia. His symptoms and neurologic/radiologic indications showed no change at a 1-year follow-up. Idiopathic syringomyelia symptoms are varied and may be overlooked by physicians. Pediatricians may consider syringomyelia if patients complain about persistent sensory abnormality. All patients who present with syringomyelia should undergo detailed neuroimaging of the entire neuraxis to elucidate the proximate cause of the lesion.
Arachnoid ; Arachnoiditis ; Brain ; Central Nervous System Infections ; Child ; Constipation ; Follow-Up Studies ; Humans ; Hydrocephalus ; Hyperesthesia ; Infant ; Lower Extremity ; Magnetic Resonance Imaging ; Male* ; Neck ; Neck Pain ; Neuroimaging ; Neurologic Examination ; Neurologic Manifestations ; Pathology ; Reflex, Abnormal ; Scoliosis ; Spinal Cord ; Spinal Cord Neoplasms ; Syringomyelia ; Vascular Malformations

Intracranial hemorrhage is common and is caused by diverse pathology, including trauma, hypertension, cerebral amyloid angiopathy, hemorrhagic conversion of ischemic infarction, cerebral aneurysms, cerebral arteriovenous malformations, dural arteriovenous fistula, vasculitis, and venous sinus thrombosis, among other causes. Neuroimaging is essential for the treating physician to identify the cause of hemorrhage and to understand the location and severity of hemorrhage, the risk of impending cerebral injury, and to guide often emergent patient treatment. We review CT and MRI evaluation of intracranial hemorrhage with the goal of providing a broad overview of the diverse causes and varied appearances of intracranial hemorrhage.
Arteriovenous Malformations ; Central Nervous System Vascular Malformations ; Cerebral Amyloid Angiopathy ; Cerebral Infarction ; Craniocerebral Trauma ; Hematoma, Subdural ; Hemorrhage ; Humans ; Hypertension ; Intracranial Aneurysm ; Intracranial Hemorrhages* ; Magnetic Resonance Imaging ; Neuroimaging ; Pathology ; Sinus Thrombosis, Intracranial ; Subarachnoid Hemorrhage ; Vasculitis

Aorta, Thoracic ; pathology ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; diagnosis ; mortality ; Humans ; Infant ; Infant, Newborn ; Male ; Vascular Malformations ; diagnosis ; mortality

OBJECTIVETo investigate the safe and effective treatment for painful venous malformation (VM) in limbs.

METHOD(1) 97 cases with painful VM underwent MRI to detect the location of VM, as well as its size and structure, its relationship with the surrounding tissue. Statistical analysis was also performed. (2) The embolic agent (ethanol) was first injected to embolize the draining vessels of VM, then the Polidocanol plus Methotrexate (MTX) was followed to keep the embolization effect on VM. The therapeutic effect was observed and analyzed.

RESULTSFrom January 2010 to January 2012, 97 patients with painful VM were treated. A Spearman correlation analysis showed no significant correlation between symptoms of pain and lesion growth, volume, or MRI grades (P > 0.05). The lesions in the muscle space are more likely to have the symptoms of pain (P < 0.01), followed by the lesions in the muscle, then the lesions in the joint and subcutaneous tissue. The pain relieve percentage was 95.9% (93/97) after one time embolic sclerotherapy. No severe complication, such as distant embolization, nerve damage, or muscle atrophy happened. No pain reoccurrence happened after 0.5-1.5 years of follow-up period.

CONCLUSIONSThe treatment of embolic scleratherapy is minimal invasive, safe and effective for painful VM with stable results.

Ethanol ; therapeutic use ; Extremities ; blood supply ; Humans ; Methotrexate ; therapeutic use ; Pain ; etiology ; Pain Management ; methods ; Polyethylene Glycols ; therapeutic use ; Sclerosing Solutions ; therapeutic use ; Sclerotherapy ; methods ; Statistics, Nonparametric ; Vascular Malformations ; complications ; pathology ; therapy ; Veins ; abnormalities

PURPOSE: Magnetic resonance imaging (MRI) has been used to screen and follow-up spinal dural arteriovenous fistulae (SDAVF). The purpose of this study was to evaluate the association between MRI findings and neurologic function in SDAVF. This study also investigated clinical features and treatment results of SDAVF. MATERIALS AND METHODS: A total of 15 consecutive patients who underwent embolization or surgery for SDAVF were included. We treated seven (60%) patients with embolization and six (40%) with surgery. We analysed clinical features, MRI findings, treatment results, and neurologic function. Neurologic function was measured by the Aminoff-Logue disability scale (ALS). RESULTS: Patients with longer levels of intramedullary high signal intensity in preoperative T2-weighted images (T2WI) exhibited worse pre- and postoperative ALS scores (r=0.557, p=0.031; r=0.530, p=0.042, Pearson correlation). Preoperative ALS score was significantly correlated with postoperative ALS score (r=0.908, p=0.000, Pearson correlation). The number of levels showing intramedullary high signal intensity in T2WI decreased significantly postoperatively (5.2+/-3.1 vs. 1.0+/-1.4, p=0.001, Wilcoxon ranked test). CONCLUSION: The number of involved levels of high signal intensity in preoperative T2WI is useful for predicting pre- and postoperative neurologic function in SDAVF.
Adult ; Aged ; Angiography ; Arteriovenous Fistula/*pathology/radiography/*surgery ; Central Nervous System Vascular Malformations/*pathology/radiography/*surgery ; Embolization, Therapeutic/*methods ; Female ; Humans ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Postoperative Period ; Predictive Value of Tests ; Prognosis ; Retrospective Studies ; Severity of Illness Index ; Spinal Cord/abnormalities/*blood supply/pathology/surgery ; Treatment Outcome

PURPOSE: Magnetic resonance imaging (MRI) has been used to screen and follow-up spinal dural arteriovenous fistulae (SDAVF). The purpose of this study was to evaluate the association between MRI findings and neurologic function in SDAVF. This study also investigated clinical features and treatment results of SDAVF. MATERIALS AND METHODS: A total of 15 consecutive patients who underwent embolization or surgery for SDAVF were included. We treated seven (60%) patients with embolization and six (40%) with surgery. We analysed clinical features, MRI findings, treatment results, and neurologic function. Neurologic function was measured by the Aminoff-Logue disability scale (ALS). RESULTS: Patients with longer levels of intramedullary high signal intensity in preoperative T2-weighted images (T2WI) exhibited worse pre- and postoperative ALS scores (r=0.557, p=0.031; r=0.530, p=0.042, Pearson correlation). Preoperative ALS score was significantly correlated with postoperative ALS score (r=0.908, p=0.000, Pearson correlation). The number of levels showing intramedullary high signal intensity in T2WI decreased significantly postoperatively (5.2+/-3.1 vs. 1.0+/-1.4, p=0.001, Wilcoxon ranked test). CONCLUSION: The number of involved levels of high signal intensity in preoperative T2WI is useful for predicting pre- and postoperative neurologic function in SDAVF.
Adult ; Aged ; Angiography ; Arteriovenous Fistula/*pathology/radiography/*surgery ; Central Nervous System Vascular Malformations/*pathology/radiography/*surgery ; Embolization, Therapeutic/*methods ; Female ; Humans ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Postoperative Period ; Predictive Value of Tests ; Prognosis ; Retrospective Studies ; Severity of Illness Index ; Spinal Cord/abnormalities/*blood supply/pathology/surgery ; Treatment Outcome

Adolescent ; Adult ; Anticoagulants ; therapeutic use ; Blood Coagulation Disorders ; diagnosis ; drug therapy ; Female ; Humans ; Male ; Middle Aged ; Vascular Malformations ; diagnosis ; drug therapy ; Veins ; pathology ; Young Adult