Biventricular Hydrocephalus Due to Idiopatic Occlussion of Foramina of Monro

Abstract

Total congenital occlusion of the foramina of Monro in adults is extremely rare. Only 9 cases have been reported. Clinically, this manifest as a biventricular hydrocephalus and the most frequent presenting symptom is headache. The diagnosis can be confirmed by computed tomographic scan or magnetic resonance imaging. Regarding treatment, neuroendoscopy constitutes the procedure of choice. The prognosis is usually good. We describe a 35-year-old woman with a 2-month headache and sudden nausea, vomiting, and syncope. The patient was studied with computed tomograph, magnetic resonance imaging, and a ventricular infusion test, and subsequently treated with endoscopic foraminoplasty and a ventriculoperitoneal shunt. The protacted normal neurological history with late and rapid onset of symptoms speaks for a progressive restriction of the size of the foramina of Monro, which allows adaptation to the changes in cerebrospinal fluid dynamics. Regarding treatment, in almost every cases of bilateral occlusion of foramina of Monro reported to date, but in 1 case both: neuroendoscopy fenestration and the ventriculoperitoneal shunt were needed. Time is needed to allow cerebrospinal fluid dynamics and the ventricular system to normalize.

Late-onset occlusion of the Monro foramina after endoscopic third ventriculostomy in adults: Case discussion and review of the literature

“…[6] In particular, various surgical treatments have been proposed, including endoscopic approaches (septostomy and/or unilateral or bilateral foraminoplasty, with the eventual use of an intraventricular stent) and monolateral or bilateral shunt placement. [1,3,5,6,[8][9][10][11][12][13][14]16,[22][23][24][25] e first report is that of Marions, in 1986, who described two cases of true bilateral stenosis of the FM in the pre-MRI era. One patient was treated microsurgically, and in both cases, a ventriculoperitoneal shunt (VPS) was implanted.…”

“…Other patients with integral septum pellucidum underwent unilateral foraminoplasty with septostomy. [1,5,6,8,10,11,13,[22][23][24][25] Only in rare cases, such as the acute onset of hydrocephalus ascribable to a reaction to the contrast used in ventriculography [8] and in patients with bilateral obstruction due to true stenosis of the FM, [13,16] the authors preferred a VPS placement.…”

“…Some observations have been made on the imaging data and the modality to perform the stomas. In particular, while in some cases, the diagnosis was reached using ventriculography [9,12,14,25] or CT scan, [1,8,[12][13][14]16,22,24,25] in others, MRI was preferred. [1,3,5,6,[8][9][10][11]13,14,16,[22][23][24][25] Only De Bonis formulated the diagnosis of bilateral membranous occlusion of FM using an FSE and steady-state free processing (SSFP) high-resolution three-dimensional (3D) sequences MRI.…”

“…In particular, while in some cases, the diagnosis was reached using ventriculography [9,12,14,25] or CT scan, [1,8,[12][13][14]16,22,24,25] in others, MRI was preferred. [1,3,5,6,[8][9][10][11]13,14,16,[22][23][24][25] Only De Bonis formulated the diagnosis of bilateral membranous occlusion of FM using an FSE and steady-state free processing (SSFP) high-resolution three-dimensional (3D) sequences MRI. [5] Relatively to the instrumentation, Schonauer concluded that the use of the thulium laser, instead of the Fogarty balloon, could reduce the risk of unintended violation of the fornix or of the other neural structures.…”

“…Adult idiopathic occlusion of the foramen of Monro (AIOFM) is an exceeding rare condition, with few cases reported in the literature. [1,3,5,6,[8][9][10][11][12][13][14]16,[22][23][24][25] In particular, the diagnosis is established in patients with bilateral enlargement of the lateral ventricles with a small third ventricle on computed tomography (CT) scan or magnetic resonance imaging (MRI), ruling out any secondary cause of FM obstruction. [6] A recent review has classified the AIOFM into four classes, on the bases of the symmetry of the lateral ventricles enlargement (and, consequently, on the deviation of the septum) and the presence/absence of membranes at the level of the FM.…”