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

Abstract

Spontaneous intracranial hypotension is an uncommon etiology of secondary headaches in children. We report a unique case of a girl with kaposiform lymphangiomatosis who developed postural headaches and imaging features of spontaneous intracranial hypotension without a spinal extradural collection. The girl underwent dynamic computed tomography myelography which revealed a cerebrospinal fluid (CSF)-lymphatic fistula related to a lymphatic malformation associated with the right T10 nerve. She underwent surgical ligation of the CSF-lymphatic fistula, resulting in resolution of the headaches. Spinal CSF-lymphatic fistulas are rare and have previously been reported in two patients with Gorham-Stout disease. The current report suggests that patients with systemic lymphatic anomalies who develop postural headaches should undergo evaluation for spontaneous intracranial hypotension and a CSF-lymphatic fistula. If discovered, surgical ligation is a potential treatment.

Keywords: Cerebrospinal fluid; Cerebrospinal fluid leak; Cerebrospinal fluid-lymphatic fistula; Child; Kaposiform lymphangiomatosis; Lymphatic anomaly; Spontaneous intracranial hypotension.

Fig. 1

Kaposiform lymphangiomatosis at 7 months old. Coronal fat-suppressed T2-weighted MR image shows multiple T2 hyperintense lesions (arrows) in the neck, mediastinum, liver and spleen. These lesions did not enhance after contrast administration (not shown) and were compatible with lymphatic malformations

Fig. 2

Spontaneous intracranial hypotension at 9 years old. Midline sagittal fluid-attenuated inversion recovery (FLAIR) MR image of the brain shows typical features of spontaneous intracranial hypotension, including brain sagging with flattening of the pons (single white arrow), tonsillar ectopia (black arrow), dural venous sinus engorgement (double white arrows) and pituitary engorgement (arrowhead)

Fig. 3

Syrinx secondary to spontaneous intracranial hypotension. Midline sagittal T2-weighted MR image of the spine shows a cervical cord syrinx (arrows) secondary to long-standing cerebellar tonsillar herniation. No extradural spinal collection was identified. Multiple T2 hyperintense lesions in the cervical and upper thoracic spine are consistent with osseous involvement of kaposiform lymphangiomatosis (arrowheads at C2 and C3)

Fig. 4

Cerebrospinal fluid-lymphatic fistula detected on dynamic computed tomography (CT) myelogram. a, b Axial (a) and coronal (b) dynamic CT myelographic images show rapid egress of intrathecal contrast material from a meningeal diverticulum into a lymphatic structure in the right T10–T11 neural foramen and paraspinal soft tissues (arrows). c A coronal reformatted dynamic CT myelographic image demonstrates an opacified thoracic duct (arrows) to the level of its drainage into the left innominate vein, resembling a lymphangiogram

Fig. 5

Paraspinal lymphatic malformation. An axial fat-suppressed T2-weighted image of the spine shows a right T10–T11 paraspinal hyperintense lesion (arrow) consistent with a lymphatic malformation in the context of other known system lymphatic malformations. There is an additional linear structure anteriorly (arrowhead) that likely represents communication with the thoracic duct, as this opacified on the computed tomography myelogram

Fig. 6

Surgical ligation of cerebrospinal fluid (CSF)-lymphatic fistula. a An intraoperative photo shows the CSF-lymphatic fistula involving the nerve root (arrow) elevated with a right-angle forceps exposed through a T10–T11 foraminotomy. b This nerve root and fistula were completely divided (double arrow) to resolve the CSF leak

Fig. 7

Improved spontaneous intracranial hypotension after surgery. A midline sagittal T2-weighted image of the brain performed 1 week after ligation of the cerebrospinal fluid-lymphatic fistula demonstrates improved imaging features of spontaneous intracranial hypotension characterized by slightly diminished tonsillar ectopia (single arrow), normal triangular morphology of the transverse sinus (double arrows) and near normalization of the pontomesencephalic angle (arrowhead)