Venkatakrishna Shyam Sunder,  Andronikou Savvas

Final Pr. ID: Poster #: CR-026

Chiari Type I deformity (C1) is associated with bony deformity of the skull base and herniation of cerebellar tonsils. MRI is used for diagnosis and surgery is advised for symptomatic children. We present a case series using MR imaging including CSF flow, in a variety of children with C1 to demonstrate a variety of outcomes both surgical and non-surgical: spontaneous resolution, spontaneous worsening, post-surgical improvement.

Case 1: A 6-year-old (y) girl referred for imaging with short stature and growth hormone deficiency demonstrated incidental findings of C1 without syringomyelia. No surgery (Sx) was performed, and follow-up (F/U) MRI at age 7 y demonstrated spontaneous resolution of the tonsillar ectopia and expansion of the CSF spaces at craniocervical junction (CCJ).

Case 2: A 7 y boy with headaches and staring spells underwent an MRI demonstrating 6 mm protrusion of pointed cerebellar tonsils and CSF space reduction at CCJ. No Sx was performed, and F/U imaging at age 9 y demonstrated spontaneous improvement in cerebellar tonsillar position and increased bidirectional CSF flow at CCJ.

Case 3: A 2-week-old girl underwent brain MRI demonstrating an ectopic neurohypophysis, under opercularization suggesting brain immaturity and a normal CCJ. F/U MRI at 6 y of age showed interval spontaneous development of C1 with decreased CSF spaces at CCJ.

Case 4: A 17-month-old boy underwent brain MRI for unsteady gait and poor vestibular response, which showed C1 and narrow CSF spaces at the foramen magnum and with reduced CSF flow. At age 3 y, after posterior fossa decompression, F/U MRI showed post-surgical improvement of the position of the cerebellar tonsils and increased CSF space at CCJ.

Case 5: A 8 y girl with headaches and intermittent dizziness underwent MRI demonstrating 15 mm protrusion of the pointed cerebellar tonsils and marked decrease in the CSF at CCJ. After suboccipital craniotomy, MRI at age 8 y demonstrated a post-surgically improved CSF space and improved bidirectional CSF flow, at CCJ. Read More

Authors:  Venkatakrishna Shyam Sunder , Andronikou Savvas

Keywords:  Chiari Type I deformity (C1), CSF space, CSF flow

Jaganathan Sriram,  Krishnan Venkatram,  Jayappa Sateesh,  Murphy Janice,  Phillips Paul,  Ramakrishnaiah Raghu,  Charles Glasier,  Choudhary Arabinda

Final Pr. ID: Poster #: EDU-070

Objective:
The optic nerves are covered by the meningeal sheath and the nerve is surrounded by the fluid which communicates with the CSF space intracranially. Any variations in the CSF pressure can alter the perioptic space fluid and cause pressure effects on the optic nerve head. The optic disc does not possess cells of muller which hold nerve fibers together unlike other retinal cells, and hence swells up easily with increasing CSF pressure resulting in papilledema. Conversely, a decrease in intracranial pressure can cause perioptic space to collapse. Our aim in this exhibit is to present the normal appearance of the perioptic space on MRI, normal optic nerve sheath diameter, our institutional MRI protocol for optic nerve/ perioptic space evaluation, pathologies that cause changes in the perioptic space, and how perioptic space variations can be a clue to look for pathologies.
Learning points:
Our exhibit includes:
Our institutional protocol for evaluation of the perioptic space and the optic nerve
Normal appearance of perioptic space on different MRI sequences including high resolution T2W coronal, T2W axial and BFFE sequences
Interesting case reports with altered perioptic space fluid including
1. Idiopatic intracranial hypertension
2. Shunt malfunction
3. Shunt over drainage
4. An interesting case of pseudotumor progressing to intracranial hypotension
5. Foster Kennedy syndrome
6. Intracranial hypotension
7. Differentiation of papilledema versus drusen
The importance of looking at perioptic space and how it gives clue to the underlying pathology
Associated findings to be evaluated on MRI
Discussion:
Perioptic spaces can give a clue to underlying intracranial pathologies. It is imperative for the radiologists to carefully evaluate the perioptic spaces and to look for additional findings if there is a variation. If there is dilatation of the perioptic space, it is important to look at the additional features including tortuosity of the optic nerves, protrusion of the optic disc, partial empty sella, enlarged meckel’s cave and bilateral transverse sinuses for stenosis. When there is collapse of the peri-optic space, other associated findings should be looked at including pachymeningeal enhancement, venous distention sign, cerebral edema, tonsillar ectopia, and other quantitative features like mamillopontine distance. Also, it is important to evaluate for tumors which can also result either in perioptic space distention or collapse. Read More

Authors:  Jaganathan Sriram , Krishnan Venkatram , Jayappa Sateesh , Murphy Janice , Phillips Paul , Ramakrishnaiah Raghu , Charles Glasier , Choudhary Arabinda

Keywords:  Perioptic space, distention and collapse, intracranial CSF pressure changes

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

Final Pr. ID: Poster #: EDU-078

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

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

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

Kuehne Alexander,  Chen Danling,  Hwang James,  Ehrlich Lauren,  Lisse Sean

Final Pr. ID: Poster #: CR-046

Meningoceles in the cranium occur when an osseous defect in the skull base allows for the protrusion or herniation of the meninges into the extracranial compartment. Meningoceles vary in severity and may be asymptomatic or complicated by meningitis, brain abscess, pneumocephalus, or cerebrospinal fluid leak. Meningoceles can be congenital, traumatic, iatrogenic (such as in sinus or craniofacial surgery), spontaneous (due to increased intracranial pressure) or idiopathic. The most common sites of meningoceles in the skull base are the cribriform plate, sphenoid sinus, perisellar region, and tegmen tympani or mastoideum. Accurate and timely detection is essential for avoiding complications of meningoceles. Localization of the site of the meningoceles and assessment of their size and composition is accomplished through a variety of imaging techniques, such as with CT or MR myelography. Surgical repair of meningoceles may be accomplished through both open and endoscopic approaches.
A 2-year-old male patient with a past medical history of febrile seizures and postinfectious hydrocephalus requiring ventriculoperitoneal shunt placement and multiple shunt revisions initially presented with emesis and lethargy concerning for shunt malfunction. MRI showed a fluid-signal intensity located adjacent to the petrous portion of the right temporal bone, extending caudally to the right upper neck, which raised concern for cerebrospinal fluid (CSF) leak. Lumbar puncture was notable for an elevated CSF opening pressure of 40 cm H2O. CT myelogram confirmed a small (7mm) meningocele protruding through an osseous defect in the right jugular foramen, which completely opacified with intrathecally-injected contrast. Delayed images obtained following two hours further demonstrated subtle contrast enhancement surrounding the right jugular vein in the upper neck, raising concern for a slow CSF leak from the meningocele. Due to elevated CSF opening pressure, shunt malfunction was theorized to be the cause of the patient’s emesis and lethargy. Shunt was revised and patient was subsequently able to be discharged. Repeat follow up imaging demonstrated stabile size of patient’s meningocele. Due to small size, and resolution of patient’s symptoms following shunt revision, patient’s meningocele will be followed with recurrent MR imaging. Read More

Authors:  Kuehne Alexander , Chen Danling , Hwang James , Ehrlich Lauren , Lisse Sean

Keywords:  CSF, MRI, Neuroradiology

Hoodeshenas Safa,  Averill Lauren,  Mody Tejal,  Johnson Craig

Final Pr. ID: Poster #: EDU-079

CSF shunts play a vital role in diverting excess cerebrospinal fluid to other body compartments, thereby preventing potentially life-threatening complications. This presentation aims to provide a comprehensive overview of CSF shunt systems and their evaluation using multimodal imaging techniques.
We will briefly review the various types of CSF shunts and their essential components. The discussion will also cover imaging protocols and MRI safety considerations for patients with shunts, with a focus on what clinicians need to see in imaging reports.
Additionally, special emphasis will be placed on shunt-related complications, such as shunt malfunctions (e.g., disconnections, migration, leakage and overdrainage); over-shunting myelopathy (Miyazaki syndrome); slit ventricle syndrome; trapped ventricle; infections (ventriculitis, meningitis); and distal catheter complications (e.g., peritoneal CSF pseudocysts with and without infection, peritonitis, pleural effusions). Read More

Authors:  Hoodeshenas Safa , Averill Lauren , Mody Tejal , Johnson Craig

Keywords:  Shunts, CSF, Hydrocephalus

Tierradentro-garcia Luis,  Martinez Mesha

Final Pr. ID: Poster #: EDU-063

Background:

Connective tissue disease can affect multiple systems in children. In the central nervous system, these entities can present as cerebrospinal fluid (CSF) disorders, such as CSF leaks, due to dysregulation of collagen and/or other extracellular matrix components. Most patients present with chronic, daily, unremitting headaches that usually improve when lying down. Imaging findings can show classic signs of intracranial hypotension, signs of idiopathic intracranial hypertension, or both. The latter, termed “mixed CSF pressure disorders”, a newer entity described in the literature, can present a diagnostic dilemma as patients can present with atypical symptoms in combination with a clinical history of hypermobility. Conventional CT myelography can confirm capacious thecal sacs, multiple CSF leaks, prominence of perimedullary veins, or multiple dural diverticula. The senior author, a pediatric neurointerventionalist, treated the presented cases.

Education goals:

1) To present common heritable (e.g. COL11A2, COL5A2, ADAMTS2, ZNF469, FBN1, FLNA, TNXB) connective tissue diseases that can manifest with CSF disorders in children.
2) To highlight the main findings on conventional CT myelography to detect CSF leaks.
3) To correlate CT myelography findings with brain and spinal MRI in children with CSF disorders.
4) To discuss the options for management from a neurointerventional perspective. Read More

Authors:  Tierradentro-garcia Luis , Martinez Mesha

Keywords:  CSF, Interventional