Posterior fossa craniotomy for trapped fourth ventricle in shunt-treated hydrocephalic children: long-term outcome

Clinical article

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Object

Trapped fourth ventricle (TFV) is a rare late complication of postinfectious or posthemorrhagic hydrocephalus. This entity is distinct from a large fourth ventricle because TFV entails pressure in the fourth ventricle and posterior fossa due to abnormal inflow and outflow of CSF, causing significant symptoms and signs. As TFV is mostly found in children who were born prematurely and have cerebral palsy, diagnosis and treatment options are a true challenge.

Methods

Between February 1998 and February 2007, 12 children were treated for TFV in Dana Children's Hospital by posterior fossa craniotomy/craniectomy and opening of the TFV into the spinal subarachnoid space. The authors performed a retrospective analysis of relevant data, including pre- and postoperative clinical characteristics, surgical management, and outcome.

Results

Thirteen fenestrations of trapped fourth ventricles (FTFVs) were performed in 12 patients. In 6 patients with prominent arachnoid thickening, a stent was left from the opened fourth ventricle into the spinal subarachnoid space. One patient underwent a second FTFV 21 months after the initial procedure. No perioperative complications were encountered. All 12 patients (100%) showed clinical improvement after FTFV. Radiological improvement was seen in only 9 (75%) of the 12 cases. The follow-up period ranged from 2 to 9.5 years (mean 6.11 ± 2.3 years) after FTFV.

Conclusions

Fenestration of a TFV via craniotomy is a safe and effective option with a very good long-term outcome and low rate of morbidity.

Abbreviations used in this paper: CN = cranial nerve; FTFV = fenestration of trapped fourth ventricle; IVH = intraventricular hemorrhage; PHH = posthemorrhagic hydrocephalus; PIH = postinfectious hydrocephalus; TFV = trapped fourth ventricle.

Article Information

* Drs. Udayakumaran and Beni-Adani contributed equally to this work.

Address correspondence to: Liana Beni-Adani, M.D., Department of Pediatric Neurosurgery, Dana Children's Hospital, Tel Aviv Sourasky Medical Center, 6 Weizman Street, Tel Aviv, 64239, Israel. email: lianabenia@gmail.com.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Intraoperative photograph showing the site of the suboccipital area (SO) craniotomy (about 2 × 3 cm), the location of the foramen magnum (FM), and the first (C1) and second (C2) cervical vertebrae.

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    Left: Intraoperative photograph demonstrating abnormally thick arachnoidal layers in the region of the outlet of the fourth ventricle and the cisterna magna. Right: Intraoperative photograph demonstrating occlusion of the foramen of Magendie by an abnormal membranous layer, between the split tonsils and cerebellum superiorly and the brainstem (star) inferiorly (right).

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    Left: Intraoperative photograph showing the fenestration of the TFV (4th V) with a stent leading from it to the open spinal subarachnoid space (SAS). Note that the stent is anchored with nonabsorbable fine suture to the arachnoid. Right: Intraoperative photograph showing wide fenestration of the TFV without the use of a stent. Note the opening of the fourth ventricle, lateral gutters (G), cisterna magna, and opening into the spinal subarachnoid space.

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    Case 4. Preoperative head CT scans obtained in an infant who was diagnosed prenatally with IVH (fetal IVH), had a postnatal presentation of posthemorrhagic hydrocephalus, and was subsequently treated with shunt placement. Note the small and undilated fourth ventricle at this stage (right).

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    Case 4. Preoperative (left) and postoperative (right) sagittal T2-weighted MR images obtained in the same infant in Fig. 4. The preoperative image shows a dilated fourth ventricle with mass effect on the brainstem; no prepontine CSF is visible. The postoperative image (obtained 7 years after FTFV) shows the resolution of the signs of entrapment. Notice the flow void at the outlet of the fourth ventricle, CSF in front of the brainstem, and the open cisterna magna.

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    Case 6. a: Sagittal T2-weighted preoperative MR image showing a large dilated fourth ventricle with mass effect on the brainstem in a patient with multicystic hydrocephalus. The patient at this stage was treated by endoscopic fenestration of the huge third ventricle cyst and endoscopic third ventriculostomy in addition to the supratentorial shunt that was well functioning. The TFV did not diminish in size and the child continued to experience severe swallowing problems. b: Sagittal postoperative T2-weighted MR image obtained at the 7-year follow-up showing a smaller fourth ventricle with relief of mass effect on the brainstem. Note the stent from the fourth ventricle into the space below the foramen magnum (arrow). c: Axial T2-weighted MR image (obtained at the same time as panel b) showing that the fourth ventricle is still large, although there is clear CSF in front of the brainstem, and the stent lies in the middle of the large decompressed fourth ventricle.

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    Case 8. Preoperative (a and b) and postoperative (c) CT scans obtained in a child who presented acutely with bradycardia. The preoperative scans demonstrate a dilated fourth ventricle with mass effect on the brainstem (a) and small well-drained supratentorial ventricles (b). The axial postoperative image (obtained more then 3 years after FTFV) shows the smaller fourth ventricle with a stent in situ (white arrow) and resolution of the mass effect on the brainstem as represented by the space anterior to the brainstem.

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