Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis

Soderlund, Karl A.; Mamlouk, Mark D.; Shah, Vinil; Roland, Jarod L.; Dillon, William P.

doi:10.1007/s00247-021-05132-6

Cited by 8 publications

(3 citation statements)

References 8 publications

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“…Biomarkers have been identified, especially serum angiopoietin‐2 (Ang‐2) levels, which are elevated 21,22 . Other rare symptoms include pseudotumor cerebri, which may be due to blockage of venous return in the neck by lymphangiomatous tissue, and cerebrospinal fluid (CSF)‐lymphatic fistula causing spontaneous intracranial hypotension 23,24 . The complex and diverse nature of presenting symptoms, along with the lack of familiarity of clinicians with this rare disorder, frequently results in the misdiagnosis of KLA as pneumonia, cancer, bone marrow failure, or other vascular anomalies, sometimes leading to years of diagnostic uncertainty and delays in effective treatment.…”

Section: Clinical Presentation and Imagingmentioning

confidence: 99%

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

McDaniel

Adams

Steele³

et al. 2023

Pediatric Blood & Cancer

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Kaposiform lymphangiomatosis (KLA) is a life‐threatening rare disease that can cause substantial morbidity, mortality, and social burdens for patients and their families. Diagnosis often occurs long after initial symptoms, and there are few centers in the world with the expertise to diagnose and care for patients with the disease. KLA is a lymphatic anomaly and significant advancements have been made in understanding its pathogenesis and etiology since its first description in 2014. This review provides multidisciplinary, comprehensive, and state‐of‐the‐art information on KLA patient presentation, diagnostic imaging, pathology, organ involvement, genetics, and pathogenesis. Finally, we describe current therapeutic approaches, important areas for research, and challenges faced by patients and their families. Further insights into the pathogenesis of KLA may advance our understanding of other vascular anomalies given that similar signaling pathways may be involved.

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Section: Clinical Presentation and Imagingmentioning

confidence: 99%

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

McDaniel

Adams

Steele³

et al. 2023

Pediatric Blood & Cancer

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“…Recently, Yokoi et al [ 6 ] reported a case of a child with GDS and CSF leak into osteolytic posterior bony elements Th9-10, treated with direct surgical repair and vertebral fixation. Soderlund et al [ 4 ] presented a case of a child with KLA who developed postural headaches due to a CSF-lymphatic fistula related to a lymphatic malformation associated with the right Th10 root nerve. In this case, the surgical ligation of the CSF-lymphatic fistula was undertaken, resulting in the resolution of the headache.…”

Section: Discussionmentioning

confidence: 99%

Cerebrospinal fluid–lymphatic fistula in a child with generalized lymphatic anomaly treated with targeted blood patch — a rare case report and review of the literature

Frič,

Heier,

Züchner

et al. 2024

Childs Nerv Syst

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Spontaneous intracranial hypotension may result in debilitating postural headaches and severe neurological symptoms due to secondary cerebellar sagging. The most common cause is the cerebrospinal fluid (CSF) leak within the spinal canal. Although previously reported in only a few cases, also paraspinal lymphatic malformations causing vertebral bone destruction may occasionally result in CSF leak to these pathological formations. Here, we present a case of a 9-year-old girl with generalized lymphatic anomaly (GLA) presenting with severe postural headache. Radiological imaging revealed a typical feature of cerebellar sagging. Myelography localized the CSF leakage into vertebral bodies of C7 and Th1, which both were partly involved in pathological paravertebral masses of known lymphatic anomaly, and from there along the right C8 nerve root sleeve into the anomaly. As the C8-nerve root could not be ligated due to the risk of significant neurological injury, we attempted image-guided targeted percutaneous epidural placement of a blood patch directly into the foramen at the affected level. The procedure resulted in obliteration of the fistula and regression of cerebellar sagging, with significant relief of symptoms. Although it is an extremely rare coincidence, patients with paraspinal lymphatic malformations may develop intraspinal CSF leak into these pathological formations. The present case report suggests that besides a direct surgical obliteration of the fistula and sacrificing the nerve root, a targeted percutaneous epidural blood patch may be a possible alternative in the case of a functionally important nerve root.

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“…Although we did not experience any difficulty repositioning patients on the foam wedge, alternative means of elevating the patient's pelvis without requiring movement on the CT table, such as placing an inflatable mattress (HoverMatt; HoverTech International) under the patient's hips and inflating it transiently after the intrathecal injection of contrast, might represent further refinements to the technique. 16…”

Section: Discussionmentioning

confidence: 99%

Same-Day Bilateral Decubitus CT Myelography for Detecting CSF-Venous Fistulas in Spontaneous Intracranial Hypotension

Jones

Goadsby

2022

AJNR Am J Neuroradiol

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Lateral decubitus CT myelography is a sensitive technique for detecting CSF-venous fistulas in patients with spontaneous intracranial hypotension. It might be necessary to perform bilateral studies to locate a fistula. We report on the feasibility of obtaining diagnostic-quality bilateral decubitus CT myelography in a single session, avoiding the need to schedule separate examinations for the left and right sides on different days.ABBREVIATIONS: CTM ¼ CT myelography; CVF ¼ CSF-venous fistula; DSM ¼ digital subtraction myelography; SIH ¼ spontaneous intracranial hypotension S pontaneous intracranial hypotension (SIH) is a debilitating condition that results from leakage of CSF from the spine. In up to one-quarter of patients with SIH, the underlying cause is a CSF-venous fistula (CVF), an abnormal connection between the subarachnoid space of a nerve root sheath and adjacent veins. 1 Targeted treatment of CVFs, by injection of blood and/or fibrin sealant, 2 endovascular occlusion, or neurosurgery requires accurate localization of the fistula. Performing CT myelography (CTM) or digital subtraction myelography (DSM) with the patient in the lateral decubitus position increases the sensitivity for CVFs by exposing the nerve root sleeves on the dependent side to a higher concentration of contrast. 1,3,4 Although most CVFs occur in the thoracic spine on the right side, 5 on which side a CVF will be found cannot be known beforehand. Moreover, CVFs can rarely occur bilaterally. 6,7 A complete examination, therefore, requires bilateral decubitus myelograms. Currently most centers undertaking lateral decubitus CTM or DSM examine the right and left sides on separate days, primarily because of manufacturer-set dose constraints on the maximum intrathecal iodine dose 8,9 but also because the contrast is diluted by CSF when turning the patient to the opposite side, which leads to decreased opacification of meningeal diverticula. 8 This practice is

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Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis

Cited by 8 publications

References 8 publications

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment

Cerebrospinal fluid–lymphatic fistula in a child with generalized lymphatic anomaly treated with targeted blood patch — a rare case report and review of the literature

Same-Day Bilateral Decubitus CT Myelography for Detecting CSF-Venous Fistulas in Spontaneous Intracranial Hypotension

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