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Pediatric intraventricular cavernous hemangioma: A neurological rarity
Pragyan Sarma1, Manish Kumar Garg1, Sujata Chaturvedi2, Ishita Pant2
1 Department of Neurosurgery, UCMS and GTB Hospital, Delhi, India
2 Department of Pathology, Institute of Human Behaviour and Allied Sciences, Delhi, India
|Date of Submission||30-Aug-2021|
|Date of Decision||09-Jan-2022|
|Date of Acceptance||26-Dec-2021|
|Date of Web Publication||30-Jan-2023|
Department of Neurosurgery, UCMS and GTB Hospital, Dilshad Garden, Delhi 110095
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Introduction: Pediatric intraventricular cavernous hemangioma is a rare clinical entity. These categories of tumors in children pose a significant challenge to the treating neurosurgeon. We hereby report a case of cavernous hemangioma located in occipital horn of the lateral ventricle in a 10-year-old child supported by radiological and pathological evidence, which according to our literature search is a very rare phenomenon.Clinical Case Report: A 10-year-old male child was admitted with symptoms of raised intracranial pressure without any other clinical localizing signs. Magnetic resonance imaging (MRI) revealed an intraventricular lesion in the left occipital horn which was intensely hyperintense on T2-weighted (T2W) with contrast enhancement. He underwent gross total excision using a minimally invasive approach. Histopathology was suggestive of cavernous hemangioma. Postoperative recovery was uneventful.Conclusion: Intraventricular hemangiomas itself are very rare tumors. In published literature, till now only 28 such cases in pediatric population are reported. Although MRI provides useful clues to diagnosis, histopathological correlation is mandatory to confirm the diagnosis.
Keywords: Cavernous hemangioma, intraventricular, pediatric
| Introduction|| |
Cavernous hemangiomas (CH; cavernomas, cavernous angiomas, cavernous malformations) are developmental malformations that are widely distributed across various locations in the brain. But these lesions are very rare to find inside the ventricles. Since the time Rudolf Finkelnburg first reported intraventricular cavernous malformation way back in 2005, till now 136 such cases in different age groups have been reported. The most common location of intraventricular CH is the lateral ventricle followed by the third ventricle. However in pediatric population till now only 41 such cases are reported. We hereby present a case of 10-year-old child who underwent gross total resection of the CH located in the occipital horn of the lateral ventricle with good outcome along with review of literature.
| Case Report|| |
A 10-year-old male patient presented with intermittent holocranial dull aching headache lasting for 2 months. She also had a history of intermittent episodes of vomiting and blurring of vision at the peak of headache. On examination, the only positive finding was bilateral florid papilledema. There was no neurological deficit. In view of the presenting symptoms, she was investigated further with magnetic resonance image (MRI) which revealed a relatively well-defined mass lesion occupying the left occipital horn. It was heterogeneous on T1-weighted (T1W) [Figure 1A] and intensely hyperintense on T2-weighted (T2W) images. There was a surrounding rim of low-signal [Figure 1B] and [C] intensity around the mass, which was not well delineated on T2W image. Lesion was enhancing homogenously with contrast with speckling and obstructive hydrocephalus. Patient was taken up for surgery and was placed in a lateral position. Under the guidance of neuronavigation, skin incision was planned and a mini craniotomy was done. Following this dura was opened lesion was approached through the superior parietal lobule. Peroperatively the lesion was soft, suckable with a lobular surface occupying the occipital horn. There were dense adhesions with ventricular wall, which were very carefully dissected. Piecemeal excision was done and attachment with the choroid plexus was coagulated. Gross total excision was achieved followed by hemostasis. Histopathology showed large hyalinized vessels filled with organized thrombus. Findings were compatible with characteristics of CH [Figure 2]. Postop computed tomography (CT) showed no residue and no evidence [Figure 3] of any operative site hematoma. Postop MRI showed [Figure 4A]-[C] gross total excision of the lesion. At the time of last follow-up, the patient was asymptomatic.
|Figure 1: (A) T1W image showing heterointensity of the lesion. (B) T2W showing intense hyperintensity of the lesion. (C) Contrast image showing enhancement|
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|Figure 2: Microphotograph showing proliferation of variably sized, dilated, and thin-walled vessels lined by a single layer of flat endothelial cells (HE 100×)|
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|Figure 4: (A) T1W. (B) T2W. (C) T1W contrast showing gross total excision with postoperative changes|
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| Discussion|| |
In contrast to arteriovenous malformations, CH lacks definite feeding artery and draining veins and hence is often angiographically occult. They are benign lesions with a slow growth curve and are not prone to large bleeds. They can be diagnosed across a wide array of locations in the central nervous system (CNS) but these lesions are commonly not seen inside the ventricles. In terms of presenting features, there is as such no significant difference between parenchymal and intraventricular lesions with symptoms of raised intracranial pressure being the most common symptom. CH may also present as focal deficits, seizures, or following an episode of bleeding. However, taking into consideration the rare location, natural course and symptoms of these intraventricular lesions, they have not been fully evaluated in details and there is no established algorithm for management. Shirvani et al. performed a detailed review of 136 cases and found that approximately one-fourth of the cases belonged to pediatric population. Intraventricular lesions cause a higher incidence of mass effects that may be explained by the pressure over the critical structures inside the ventricles. Also, these lesions in contrast to parenchymal lesions, are prone to grow at an increased rate in view of the lack of brain tissue around it and thereby being offered no mechanical resistance. Intralesional bleeding can occur depending upon the size of the lesion but CH causing intraventricular hemorrhage (IVH) is very rare. Kivelev et al. analyzed 89 cases of intraventricular cavernomas and found that lesions in the lateral or fourth ventricle in addition to mass effect over critical structures may present with hydrocephalus requiring temporary external ventricular drain and sometimes permanent cerebrospinal fluid diversion (CSF) diversion. In literature, there is no evidence of the definite utility of ventriculoperitoneal shunts before definite surgery. The author recommends that in absence of neurological deterioration due to hydrocephalus per se, it is not advisable to perform preop shunting as there are complications of the procedure itself. Besides any degree of bleeding can cause shunt blockade. In our case, however, though symptoms of hydrocephalus were very prominent, the patient did not require any temporary or permanent CSF diversion. Kivelev et al. also reported that seizures are less common in intraventricular lesions as true perilesional intracerebral gliosis and hemosiderosis does not occur with these lesions.
On imaging, these lesions have a very characteristic appearance. CT scan shows foci of high-density areas, scanty perilesional edema, patchy calcification, evidence of recent bleeding episodes, and minimal to absent contrast enhancement. MRI usually shows mixed signal intensities. High-signal intensities signify methemoglobin presence and low intensities point toward intralesional calcification and fibrosis. Sometimes an area of low-signal intensity may be observed in the boundaries due to the paramagnetic effects of hemosiderin. CH usually shows heterogenous contrast enhancement. Nonetheless in pediatric populations these findings can be confused with central neurocytoma, choroid plexus papilloma, anaplastic astrocytoma, and ependymoma. In our case, initially the most probable diagnosis considered initially was central neurocytoma but later biopsy was suggestive of CH.
Irrespective of the imaging findings, surgical resection is the treatment of choice. Location of the tumor, size of the lesion, presenting symptoms, and relation of the lesion to critical vital structure determine the surgical approach. The different approaches for intraventricular CH are summarized in [Table 1]. While deciding upon the microsurgical approach, it is always advisable to consider the pros and cons of individual approaches and have a detailed discussion with the patient before surgery. Neuroendoscopic resection is a viable alternative to microsurgery., Unlike microsurgery, avoidance of dissection and retraction of normal brain and injury to superficial and bridging veins in the brain surface are advantages of endoscopy. However, large size and firm consistency of the tumor, absence of hydrocephalus, inferior resolution compared to microscope, inability to obtain three-dimensional view, and limited maneuverability of instruments are disadvantages of endoscopic resection.
|Table 1: Proposed approaches concerning different locations of cavernoma|
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In comparison to most of the other similar cases reported in the literature, in our case the lesion was of bigger size and despite filing up the ventricular cavity and effacing the walls of the ventricle it was surprising that the patient had no symptoms of mass effect and only presented with symptoms of hydrocephalus. One of the possible explanations for it is that the ventricular cavity and brain in particular in children has more capacity of plasticity and adaptability compared to adults. Also despite preoperative hydrocephalus, no form of CSF diversion was done prior to surgery. Following tumor excision, symptoms of hydrocephalus also resolved which proves that the normal CSF pathways can be restored with meticulous tumor excision and good hemostasis. The excision was carried out with the help of a syringe port through a very small incision under Neuro navigation guidance.
| Conclusion|| |
Intraventricular CH is rare findings in pediatric population. These lesions can attain large sizes with minimal symptoms complication surgery. Microsurgical excision is the gold standard of treatment but endoscopic excision is a viable alternative. Tumor characteristics, experience of the treating doctor, and pitfalls of each approach should decide the approach undertaken in individual cases. Future randomized trials and long-term follow-up of microsurgery and endoscopic resection are required to define a definite management algorithm.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]