Intracranial hypotension is a condition caused by the loss of cerebrospinal fluid through either a dural defect, ruptured meningeal diverticulum, or CSF-venous fistula. In the past decade, this condition has been increasingly diagnosed in individuals suffering from orthostatic headaches. In adults, CSF leaks are commonly caused by dural defects due to a degenerative disc osteophyte violating the ventral dura. In the pediatric population, spontaneous leaks may occur in individuals with underlying connective tissue disease, or from venous/lymphatic malformations which approximate the spinal subarachnoid space. Most commonly, a CSF leak results in an “orthostatic headache” characterized by head pain which worsens upon sitting or standing and improves when lying flat. In addition to headache, patients can experience vestibulocochlear symptoms, changes in vision, forgetfulness, or personality changes. CSF leak localization usually requires dynamic myelography, performed using either digital subtraction or CT techniques. However, specific tailored MRI protocols can aid in the detection and characterization of leaks. Treatment options include percutaneous injection of autologous blood or fibrin sealant into the epidural space, embolization of draining veins, or primary surgical repair. The goal of this exhibit is to provide an illustrative review of the various pathologies that can cause CSF leak in the pediatric population. A review of anatomy and pathophysiology followed by a case-based presentation including congenital, iatrogenic, traumatic, and idiopathic causes will be presented. Both noninvasive and invasive imaging protocols will be discussed with a focus on minimizing radiation dose in the pediatric population. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Jordan Gregory, Hampton Erica, Stence Nicholas, Milla Sarah, Callen Andrew

Keywords: CSF Leak, Intracranial hypotension, CSF-venous fistula

Generalized Lymphatic Anomaly (GLA) is a rare multisystem congenital disorder originating from the abnormal development of the lymphatic system which occur under the spectrum of Complex Lymphatic Anomaly (CLA). In addition to GLA, other CLAs include Kaposiform Lymphatic Anomaly (KLA) and Gorham-Stout Disease (GSD). Lymphatic malformations (LM) associated with GLA are usually apparent at birth or by two years of age. GLA can affect almost any organ of the body but is most commonly associated with lymphatic abnormalities in the skin, abdominal/thoracic viscera and bone. Multisite soft tissue LM can occur in all CLAs, with macrocystic lymphatic malformations being most common in GLA. These lesions can be found in the mediastinum, retroperitoneum, and subcutaneous tissue. Abdominal viscera involved include the spleen and liver. The frequency of focal splenic lesions is higher with GLA and KLA in comparison to GSD. On MRI, the lesions exhibit marked T2 hyperintensity with no discernable enhancement. In patients with larger splenic lesions, areas of T1 hyperintensity have been documented. Liver lesions in GLA have a similar appearance to the previously described splenic lesions. Nakamura et al. found that more than 30 focal splenic lesions and/or focal splenic lesions with maximum diameters greater than >10 mm were observed only in patients with GLA. On contrast enhanced CT, the lesions are generally well-circumscribed and hypodense. Within the thorax, mediastinal LMs can be seen in GLA but are more common in KLA and GSD. Chylous effusions can occur in all of the CLAs, although it has been reported that effusions in GLA were more likely to be associated with mediastinal involvement. Osseus involvement is common in patients with GLA. GLA has a predilection for the appendicular skeleton, in which the ribs are most affected although cranial, vertebral and lower extremity lesions have been reported. The lesions are usually non-contiguous with medullary destruction and sparing of the cortex. This is in contrast to GSD where cortical destruction, progressive osteolysis, contiguous lesions, and soft tissue infiltration are more common. Educational Goals: 1) Illustrate the most common imaging characteristics by each organ system affected by GLA and how to differentiate GLA from other CLAs. 2) Raise awareness of the optimal imaging evaluation in patients with GLA. 3) Outline an approach to multidisciplinary management of patients with GLA through a vascular anomalies center. Read More

Meeting name: SPR 2023 Annual Meeting & Postgraduate Course , 2023

Authors: Jordan Gregory, Zavaletta Vaz, Malone Ladonna, Katz Danielle, Nakano Taizo, Kulungowski Ann, Annam Aparna

Keywords: Generalized Lymphatic Anomaly, Lymphatic Malformations, Complex Lymphatic Anomaly