Hydrocephalus due to idiopathic stenosis of the foramina of Magendie and Luschka

Report of three cases

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✓ Idiopathic stenosis of the foramina of Magendie and Luschka is a rare cause of obstructive hydrocephalus involving the four ventricles. Like other causes of noncommunicating hydrocephalus, it can be treated with endoscopic third ventriculostomy (ETV).

Three patients who were 21, 53, and 68 years of age presented with either headaches (isolated or associated with raised intracranial pressure) or vertigo, or a combination of gait disorders, sphincter disorders, and disorders of higher functions. In each case, magnetic resonance (MR) imaging demonstrated hydrocephalus involving the four ventricles (mean transverse diameter of third ventricle 14.15 mm; mean sagittal diameter of fourth ventricle 23.13 mm; and mean ventricular volume 123.92 ml) with no signs of a Chiari Type I malformation (normal posterior fossa dimensions, no herniation of cerebellar tonsils). The diagnosis of obstruction was confirmed using ventriculography (in two patients) and/or MR flow images (in two patients). All patients presented with marked dilation of the foramen of Luschka that herniated into the cisterna pontis. All patients were treated using ETV.

No complications were observed. All three patients became asymptomatic during the weeks following the surgical procedure and remained stable at a mean follow-up interval of 36 months. Postoperative MR images demonstrated regression of the hydrocephalus (mean transverse diameter of third ventricle 7.01 mm; mean sagittal diameter of fourth ventricle 16.6 mm; and mean ventricular volume 79.95 ml), resolution of dilation of the foramen of Luschka, and good patency of the ventriculostomy (flow sequences).

These results confirm the existence of hydrocephalus caused by idiopathic fourth ventricle outflow obstruction without an associated Chiari Type I malformation, and the efficacy of ETV for this rare indication.

Article Information

Address reprint requests to: Philippe Decq, M.D., Service de Neurochirurgie, Hôpital Henri Mondor, Boulevard du Maréchal de Lattre de Tassigny, F-94010 Créteil, France. email: philippe.decq@hmn.ap-hop-paris.fr.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Preoperative T2-weighted MR images, sagittal (left) and axial (right) views, revealing dilation of the foramen of Luschka intussuscepting into the CPA as far as the cisterna pontis.

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    Case 2. Preoperative T2-weighted MR image, sagittal view. The third ventricle is dilated, with a pouchlike dilation of the floor over the premammillary recess. The cerebral aqueduct is also dilated, with a visible flow artifact confirming circulation of CSF. There is no CSF flow visible in the foramen of Magendie. There is no cerebellar herniation below the basion—opisthion line.

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    Case 3. Left: Sagittal cine-MR image demonstrating asynchronous CSF velocities measured in the cerebral aqueduct (+) and cisterna magna at the base of the skull (T). This asynchronous flow reflects obstruction of CSF flow between these two points. Upper Right: Intraoperative ventriculogram, frontal view, illustrating herniation of the foramen of Luschka, which is occluded. Lower Right: Sagittal view of the perioperative ventriculogram revealing the obstruction of the foramen of Magendie.

  • View in gallery

    Intraoperative views obtained during ETV. a: Endoscopic view of the foramen of Monro: fornix (1); choroid plexus (2); anterior septal vein (3); and thalamostriate vein (4). b: Endoscopic view of the floor of the third ventricle: anterior commissure (1); chiasma (2); infundibular recess (3); mammillary bodies (4); and, through the premammillary recess, the tip of the BA (5). c: The premammillary recess is opened using ventriculostomy forceps. d: The opening has been made and the forceps have been removed. e: After opening the premammillary recess, the endoscope must be inserted to observe the dura mater of the clivus (1) and the tip of the BA (2). Visualization of these two structures ensures efficacy of ventriculostomy and confirms the absence of the membrane of Liliequist, which must be perforated when present.

  • View in gallery

    Case 3. a: Preoperative axial T1-weighted MR image. The fourth ventricle is dilated and the foramen of Luschka herniates into the CPA. b: Preoperative sagittal T1-weighted MR image. The third ventricle is enlarged with a convex downward floor. The cerebral aqueduct and fourth ventricle are dilated. There is no Chiari Type I malformation. c: Two-year postoperative axial T1-weighted MR image. The fourth ventricle and foramen of Luschka now have normal dimensions. d: Two-year postoperative sagittal T1-weighted MR image. The ventricular cavities have a normal shape and volume.

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