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Endoscopic Treatment Strategy for a Disproportionately Large Neurologia medico-chirurgica Advance Publication Date: June 12, 2020 ORIGINAL ARTICLE doi: 10.2176/nmc.oa.2019-0299 NMC Endoscopic Treatment Strategy for a Disproportionately Large Communicating Fourth Ventricle: Case Series and Literature Review Neurol Med Chir (Tokyo) Teppei KAWABATA,1 Kazuhito TAKEUCHI,1 Yuichi NAGATA,1 Takayuki ISHIKAWA,2 Jungsu CHOO,3 and Toshihiko WAKABAYASHI1 0470-8105 1Department of Neurosurgery, Graduate School of Medicine, Nagoya University, Nagoya, Aichi, Japan 1349-8029 2Department of Neurosurgery, Ichinomiya Municipal Hospital, Ichinomiya, Aichi, Japan 3Department of Neurosurgery, Chukyo Hospital, Nagoya, Aichi, Japan The Japan Neurosurgical Society Abstract An isolated fourth ventricle (IFV) is characterized by fourth ventricular dilation due to obstruction of its 10.2176/nmc.oa.2019-0299 inlet and outlet. A disproportionately large communicating fourth ventricle (DLCFV) is a rare subtype of IFV, characterized by dilation of the fourth ventricle, regardless of the size of the lateral ventricles, with nmc-oa-2019-0299 no apparent obstruction of the cerebral aqueduct. To our knowledge, this is the first case series describing endoscopic diagnosis and treatment strategy for DLCFV. We retrospectively reviewed six cases of DLCFV in which endoscopic surgery was performed at our institution and affiliated facilities between June 2013 XX and March 2017. DLCFV was diagnosed using radiographic imaging and intraoperative endoscopy. We also conducted a PubMed search and included only original studies related to DLCFV treatment written in English in our review of the literature. Endoscopic third ventriculostomy (ETV) was performed in all patients. Additional endoscope-assisted placement of a fourth ventriculoperitoneal (VP) shunt was performed in two patients who could not be managed with ETV alone because of severe adhesion of the XX interpeduncular cistern due to subarachnoid hemorrhage (SAH). The patients’ symptoms and the size of the fourth ventricle improved with surgical treatment, without complications. Endoscopic surgery for DLCFV appears to be a safe and effective treatment. Based on our treatment strategy, ETV is the first-line treatment for DLCFV. Endoscope-assisted placement of the fourth VP shunt can be treatment for severe adhesion of the interpeduncular cistern. XX2020 Key words: endoscopic third ventriculostomy, hydrocephalus, isolated fourth ventricle, ventriculo- XX peritoneal shunt XX Introduction by apparent patency of the aqueduct.6) Since the 24December2019 first report of DLCFV in 1980,4) a limited number A disproportionately large communicating fourth of cases have been reported. The standard treatment ventricle (DLCFV) is a rare subtype of an isolated for DLCFV has not been determined, although several fourth ventricle (IFV) that is a type of obstructive procedures to treat occlusive hydrocephalus are © 2020 The Japan Neurosurgical Society hydrocephalus, although IFV occurring after infec- well established. Treatments for occlusive hydro- tions or shunt placement has been previously cephalus include ventricular drainage, ventriculo- 1–5) 2020 reported and discussed. IFV develops as a marked peritoneal (VP) shunt, lowering of the opening dilation of the fourth ventricle due to the obstruc- pressure of the adjustable valve, endoscopic third tion of its outlet and the aqueduct. In contrast, ventriculostomy (ETV), aqueductoplasty with or DLCFV, although a subtype of IFV, is characterized without stent placement, interventriculostomy, septostomy, and foraminoplasty.6–10) Some of the reported cases of DLCFV developed after ventricular Received December 24, 2019; Accepted March 31, 2020 drainage or VP shunt placement for hydrocephalus.11) Copyright© 2020 by The Japan Neurosurgical Society This DLCFV differs from other occlusive hydrocephalus work is licensed under a Creative Commons Attribution- in this respect. The root cause of DLCFV is obstruc- NonCommercial-NoDerivatives International License. tion of the outlet of the fourth ventricle, but the 31March2020 1 Neurologia medico-chirurgica Advance Publication Date: June 12, 2020 2 T. Kawabata et al. pathophysiology has not been well described so far. “human species” filter were performed. Only orig- Endoscopic treatment could be the first-line treat- inal studies related to DLCFV treatment written in ment for DLCFV because DLCFV is one of the types English were included. We excluded studies that of obstructive hydrocephalus. In addition, endoscopic could not definitively diagnose DLCFV and that did findings could help clarify the pathophysiology of not document treatment for the condition. Hence, DLCFV. One study showed that ETV was effective a recently published case series was excluded from for a patient with DLCFV.6) We routinely performed this review because it did not mention the charac- endoscopic surgery for DLCFV based on the first teristics or provide the images of each patient.12) case report of the usefulness of ETV in the patient with DLCFV. Here, we present the first case series Results of endoscopic diagnosis and surgical treatment of patients with DLCFV. We describe the endoscopic Six patients with DLCFV were identified during the surgery and treatment strategy for DLCFV and review study period. A summary of the patient character- the literature. istics is shown in Table 1. The mean duration of the follow-up period was 44.7 months (range, 32–60). Materials and Methods The mean age of the patients was 38.5 years (range, 2–67), and three of them were women. Three patients In this retrospective study, all six patients with had no particular medical history other than hydro- DLCFV who underwent endoscopic surgeries at cephalus, although a medical history of hydroceph- Nagoya University and affiliated facilities between alus resulted from subarachnoid hemorrhage (SAH) June 2013 and March 2017 were included. Written in two patients and from intraventricular hemorrhage informed consent was obtained from all individual (IVH) in one patient. Four patients received ventric- participants included in the study. We diagnosed ular drainage or VP shunt as the initial treatment DLCFV using radiographic imaging and intraoper- for hydrocephalus, but the fourth ventricular dila- ative endoscopy that revealed an enlargement of tation did not resolve. the entire cerebral ventricular system with a partic- All patients had patency of the aqueduct based ularly marked dilatation of the fourth ventricle and on the intraoperative endoscopic observation. All patency of the aqueduct. Hence, patients whose patients underwent ETV. Two patients, who had a radiographic imaging showed equal enlargement of severe adhesion of the interpeduncular cistern due the entire cerebral ventricular system were excluded. to SAH, underwent an additional endoscope-assisted Moreover, patients who did not undergo surgery placement of a fourth VP shunt to treat the hydro- were excluded from this study. cephalus. Using endoscopy, we could accurately According to our strategy, ETV is the first-line place the ventricular catheter in the fourth ventricle treatment for DLCFV. The operative method of ETV through the cerebral aqueduct. All patient symptoms was standard under the direct view of the flexible improved after ETV or additional endoscope-assisted endoscope: we fenestrated the floor of the third placement of a fourth VP shunt, and all procedures ventricle at the tuber cinereum between both were complication-free. The size of the fourth mammillary bodies and the infundibular recess with ventricle improved to varying degrees via surgical a balloon catheter. Endoscope-assisted placement treatment in all patients. of a fourth VP shunt could be an additional treat- ment in patients with a severe adhesion of the Illustrated cases interpeduncular cistern. The operative method of Case 1 and Case 4 are described in detail, as they endoscopic-assisted placement of a fourth VP shunt are representative of the six patients we treated for is as follows: first, we insert a ventricular catheter DLCFV. Case 1 had no particular medical history into the lateral ventricle. Second, we place the related to hydrocephalus, whereas Case 4 had a ventricular catheter sequentially into the foramen history of intracranial hemorrhage. of Monro, the cerebral aqueduct, and finally, the fourth ventricle, while investigating the structures Case 1 under the direct view of the flexible endoscope.11) A 43-year-old man presented with nausea and ataxic gait disturbance. The findings of a systemic Literature review examination and the patient’s medical history were For this study, PubMed searches for the terms normal. Computed tomography (CT) revealed that “disproportionately large communicating fourth the cerebral ventricles were enlarged; a ventricular ventricle,” “isolated fourth ventricle,” and “fourth drainage tube had already been placed in a previous ventricular outlet obstruction,” combined with a hospital (Figs. 1A and 1D). He was referred to us Neurologia medico-chirurgica Advance Publication Date: June 12, 2020 Table 1 Patient characteristics from six endoscopically treated cases of disproportionately large communicating fourth ventricle Case Age Operative Adhesion of the Follow-up Etiology Aqueduct Treatment Outcome number (years)/sex history basilar cistern (months) 1 43, M Idiopathic Ventricular Patent (−) ETV Improvement 52 drainage 2 67, F Idiopathic None Patent (−) ETV Improvement 40 3 46, F Idiopathic None Patent (−) ETV Improvement 32 4 39, F SAH VP shunt Patent (+) ETV, placement of
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