Fatal intratumoral hemorrhage in tectal plate glioblastoma multiforme following ventriculoperitoneal shunt.

Ventriculo-peritoneal (V-P) shunting is an accepted procedure before a direct approach to large tumors producing obstructive hydrocephalus. However, it has been associated with some complications. Intratumoral hemorrhage is an uncommon but fatal complication of V-P shunt insertion. The exact mechanism of this complication is unknown, but several mechanisms have been proposed including rapid lowering of intracranial pressure following V-P shunt placement or cerebrospinal fluid (CSF) drainage and sudden decrease in CSF volume and pressure after V-P shunting. We report an 8-year-old girl who presented with severe headache, double vision, and bilateral papilledema. Brain magnetic resonance imaging with and without gadolinium administration revealed a large tectal plate tumor compressing the aqueduct which caused hydrocephalus. Due to progressive decline in consciousness level, an emergency CSF diversion was planned and she underwent left side posterior parietal V-P shunt placement. Eight hours after V-P shunt insertion, she suddenly developed tonic-clonic seizures and collapsed into deep coma with dilated pupils and stopped breathing and died 1-day later. Histological examination of the autopsy specimen showed the tumor was a glioblastoma multiforme with massive intratumoral hemorrhage. The possible pathophysiological mechanisms of this fatal complication are discussed here and the pertinent literature is reviewed. It seems that endoscopic third ventriculostomy is the procedure of choice for CSF diversion in patients with tectal plate gliomas and decreases the occurrence of intratumoral hemorrhage.

Introduction

Intracranial tumors are a well-recognized, but uncommon cause of intracranial hemorrhage. The associated hemorrhage is commonly intratumoral and occurs in 6-10% of all brain tumors.[12] It most commonly occurs in glioblastoma multiforme (GBM), metastatic tumors, and pituitary adenomas.[1234]

Tectal plate tumors are a subgroup of brain stem tumors that represent about 10% of brain stem gliomas in children.[56] The majority of tectal plate tumors are benign gliomas and only a small percentage is malignant.[567] GBM of the tectal plate is extremely rare and only two previous cases have been reported.[58] Intratumoral hemorrhage in a tectal plate glioma is also extremely unusual.[7] Intratumoral hemorrhage in GBM occurs spontaneously, but it has been reported to develop following cerebrospinal fluid (CSF) diversion. It can occur immediately or in a delayed fashion following ventriculo-peritoneal (V-P) shunt insertion.[9] Intratumoral hemorrhage following CSF diversion is a fatal and serious complication of shunt procedure and most commonly occurs in the posterior fossa tumors.[1249101112] However, intratumoral hemorrhage in thalamic and pineal region GBMs have been reported after CSF diversion.[9]

We report the first case of fatal intratumoral hemorrhage in tectal plate GBM following V-P shunt insertion in a pediatric patient. The pertinent literature on this topic is reviewed.

Case Report

An 8-year-old previously healthy girl was admitted after presenting with vomiting, severe headache and double vision over the course of 48 h. She had 2 months history of progressively increasing headaches. On examination, she was drowsy and had bilateral papilledema.

Noncontrast computed tomography (CT) scan of the brain revealed obstructive hydrocephalous with a hypodense mass at tentorial hiatus with a single area of hyperdensity. Brain magnetic resonance imaging (MRI) with and without gadolinium administration revealed a large tectal plate tumor compressing the aqueduct, which caused hydrocephalus. The tumor was hypointense on T1-weighted images with an area of hyperintensity compatible with intratumoral hemorrhage and was hyperintense on T2-weighted images and enhanced peripherally [Figure 1a–d].

Figure 1

(a) Axial T1-weighted magnetic resonance imaging of the brain showing a hypointense mass with a single area of hyperintensity in the posterior third ventricle region as well as hydrocephalus, (b) sagittal T1-weighted magnetic resonance imaging of the brain showing a large hypointense mass with area of hyperintensity in the tectal plate region (c) axial contrast-enhanced T1-weighted magnetic resonance imaging of the brain showing a rim enhancing mass in the posterior third ventricle region and accompanying hydrocephalus, (d) sagittal contrast-enhanced T1-weighted magnetic resonance imaging of the brain showing a large rim enhancing mass in the tectal plate region

She underwent medical treatment with steroids and was admitted for tumor resection. Due to progressive decline in consciousness level, an emergency CSF diversion was planned and she underwent left side posterior parietal V-P shunt placement. Intraoperatively, the CSF was clear and was under high pressure. Minimal CSF was allowed to drain. Postoperatively, she recovered rapidly and was very well in general condition. She was fully conscious and talked with her family. Eight hours after V-P shunt insertion, she suddenly developed tonic-clonic seizures and collapsed into deep coma with dilated pupils and stopped breathing. She was immediately intubated and resuscitated. Urgent brain CT scan showed massive intratumoral hemorrhage and upward herniation with V-P shunt well placed in the left frontal horn [Figure 2]. Because of poor neurological state and absent brainstem reflexes, decompressive surgery was not contemplated and she died 1-day later. Histological examination of the autopsy specimen showed the tumor was a GBM with massive intratumoral hemorrhage.

Figure 2

Postoperative noncontrast computed tomography scan of the brain showing massive intratumoral hemorrhage, intraventricular hemorrhage, and upward herniation with the ventriculo-peritoneal shunt in its correct position

Discussion

Table 1 summarizes 18 cases of intratumoral hemorrhage after V-P shunting reported in the literature. The management of hydrocephalous in children with tectal plate tumors is controversial. In most cases with indolent tectal plate tumor or hamartoma, V-P shunt or endoscopic third ventriculostomy (ETV) is the only necessary procedure. However, in some patients with large enhancing tumors or tumors with intratumoral hemorrhage, V-P shunt or ETV followed by tumor resection is mandatory.[567]

Table 1

Reported cases of intratumoral hemorrhage after ventriculo-peritoneal shunt placement

Precraniotomy V-P shunt insertion is performed in many neurosurgical centers for treatment of hydrocephalus secondary to the posterior third ventricle and posterior fossa tumors. The precraniotomy V-P shunt has some advantages and disadvantages. The advantages include improvement in the general condition of the patient following decreasing intracranial pressure (ICP), rapid normalization of raised ICP, lowering the risk of infection associated with continuous external ventricular drainage, and prevention of postoperative ICP elevation and CSF leak.[2] However, a number of complications have been associated with V-P shunting including shunt malfunction, infection, metastatic tumor dissemination, upward herniation, and intracranial or intratumoral hemorrhage.[121013]

According to Epstein and Murali[10] patients with posterior fossa tumors in close proximity to incisural notch are at the greatest risk of intratumoral hemorrhage and upward herniation after V-P shunting procedure. It is thought that upward herniation is due to pressure difference between the middle and posterior fossa that occurs as a result of rapid emptying of ventricles following shunting procedure.[10]

Given the very rare occurrence of intratumoral hemorrhage following V-P shunt or CSF diversion in comparison to many shunt procedure performed for posterior fossa tumors with hydrocephalus, it seems that, there may be some underlying cause that eventually results in intratumoral hemorrhage. The exact mechanism of intratumoral hemorrhage after V-P shunt insertion is unknown, but several mechanisms have been proposed:[1234911121314]

Rapid lowering of ICP following V-P shunt placement or CSF drainage leads to increased transmural pressure across the tumor blood vessels and consequently causes intratumoral bleeding

Sudden decrease in CSF volume and pressure after V-P shunt results in disturbance between the various intracranial compartments leading to an increase in the cerebral blood flow and vascular congestion. Because the intracranial volume is constant, any rapid decrease in CSF volume is compensated with increase in the cerebral blood volume reciprocally. Vascular congestion leads to intratumoral hemorrhage.

The pathogenesis of intratumoral hemorrhage in GBM include:

Ischemic necrosis and rupture of the newly formed thin-walled, tortuous vessels of tumor that traverse the necrotic areas

Invasion of the wall of adjacent large vessels by tumor cells

Thrombosis of the draining veins of the tumor lead to tumor necrosis and direct breakdown of the tumor vessels and subsequent hemorrhage.[79]

In our case, intratumoral hemorrhage was detected before V-P shunt insertion. Intratumoral hemorrhage causes raised intratumoral pressure and this event in a patient with obstructive hydrocephalus exacerbates the pressure difference between intracranial and intatumoral components as well as between supratentorial and infratentorial compartments. After CSF diversion, the sudden decrease in CSF volume and pressure from supratentorial compartment leads to upward herniation and intratumoral hemorrhage.

Endoscopic third ventriculostomy is the procedure of choice for CSF diversion in patients with tectal plate gliomas and decreases the occurrence of intratumoral hemorrhage. The best way to avoid intratumoral hemorrhage is to plan definitive surgery, if possible.[6]

Nowadays, most authors recommend against precraniotomy V-P shunting in patients with posterior fossa tumors based on the view that few cases would need permanent shunt postoperatively and ETV supplanted the V-P shunt.[2610]

Tectal plate gliomas are usually benign lesions with indolent course and good prognosis, but occurrence of GBM in this place is very rare. GBM usually occurs in frontal lobes. There were two previously reported similar cases of GBM in tectal plate. Nemer et al.[8] reported a 45-year-old female with this tumor and associated hydrocephalus. They performed magnetic resonance spectroscopy (MRS) which showed an elevated choline/N-acetylaspartate ratio and an increase in lactate suggesting an aggressive neoplasm. They noted that MRS is a useful method to differentiate benign versus malignant lesions of this location. After placing V-P shunt to relieve hydrocephalus, suboccipital craniotomy was done for debulking surgery, which confirmed the diagnosis of GBM pathologically. Chemotherapy and/or radiotherapy were denied by the patient and there is no information about survival of this case. Chaddad Neto et al.[5] also reported a case of a 22-year-old woman with biopsy-proven tectal glioblastoma (grade IV WHO astrocytoma). After placing V-P shunt, the tumor was resected partially by a supracerebellar-infratentorial mid-line approach. The surgery was followed by radiotherapy and chemotherapy and at 6 months follow-up the patient's clinical condition was reported to be satisfactory. In tectal plate glioblastoma, CT scan may detect tumor-associated hydrocephalus, but CT scan may miss some tumors. MRI is superior to CT scan for tectal tumors. According to their experience, if it is possible the tumor should be removed as much as possible and follow-up treatments with radiotherapy and chemotherapy (e.g. temozolamide) is mandatory to prolong survival.

Conclusion

Glioblastoma of the tectal plate is very rare in pediatric patients. Intratumoral hemorrhage in the tectal plate glioma following V-P shunt insertion is rare but is a fatal complication.