Hemorrhagic colloid cyst: Case report and review of the literature.

Colloid cysts are cystic lesions located at the anterior part of third ventricle, close to foramen of Monro and contain colloid material. Hemorrhage in a colloid cyst is very rare. On literature review, we found only six cases of hemorrgic colloid cyst; among them, four were diagnosed at postmortem examination. We report a rare case of a hemorrhagic colloid cyst in a young lady who presented with sudden onset severe headache and vomiting. Computed tomography (CT) scan showed isodense lesion located at foramen of Monro with hyperdense areas suggestive of hemorrhage with foraminal obstruction. The lesion was not enhancing on contrast. Magnetic resonance imaging (MRI) brain revealed a well-defined cystic mass lesion located at the foramen of Monro which was hyperintense on T1 and hypointense on T2-weighted images. Excision of the colloid cyst was performed which revealed hemorrhagic clot instead of colloid material. Histopathological examination revealed a colloid cyst with hemorrhage. We believe that this is the first such reported case of successful clinical outcome following early diagnosis and excision of a hemorrhagic colloid cyst in an adult.

Introduction

Colloid cysts of third ventricle are rare intracranial lesions. They comprise about 0.5% to 1 % of all intracranial tumors.[1] The clinical presentation is disparate, varying from incidentally found cysts to sudden death due to acute hydrocephalus. A sudden increase in the size of the cyst due to hemorrhage has been rarely reported. The cysts usually consist of a thin walled capsule with grayish translucent gelatinous amorphous material containing necrotic leucocytes or cholesterol. The cyst wall is composed of cuboidal, columnar, or pseudostratified epithelial lining with interspersed mucosal goblet cells. Rarely, a colloid cyst wall is thickened, vascular, and consists of organized hematoma of varying ages. We describe a case of hemorrhage in a colloid cyst with good outcome in a young female. We highlight difficulties in excision of lesion and review the literature of this rare pathology.

Case Report

A 20-year-old lady presented with a 6 months history of progressive headache and memory disturbances. Few days prior to presentation, she had three episodes of generalized tonic clonic seizures. She also had gait disturbances and severe giddiness. Higher mental function examination revealed impaired recent memory. Fundoscopic examination showed bilateral papilloedema. Cranial nerve examination, motor and sensory examination was normal. Plantar reflexes were extensor on both sides and Romberg's sign was positive. Computed tomography (CT) scan of the brain showed isodense lesion located at foramen of Monro with hyperdense areas suggestive of hemorrhage with foraminal obstruction. The lesion was not enhancing on contrast [Figure 1]. Magnetic resonance imaging (MRI) brain revealed obstructive hydrocephalus due to a large lesion in the third ventricle which was homogenously hyperintense on T1-weighted images and homogenously hypointense on T2-weighted images [Figure 2].

Figure 1

CT brain (axial view). (a) Isodense lesion located at foramen of Monro with hyperdense areas suggestive of hemorrhage with foraminal obstruction. (b) No evidence of enhancement on contrast.

Figure 2

MRI brain (axial view). (a) Homogenously hyperintense lesion at foramen of Monro in T1-weighted sequence. (b) Lesion appears uniformly hyperintense on T2-weighted sequence

Right lateral ventricle was entered via right frontoparietal craniotomy and an interhemispheric transcallosal approach. A grayish red, well-defined, soft to firm lesion was reached through the interforniceal approach. Needle aspiration was attempted but was unsuccessful. The surface of the cyst was vascular, so it was coagulated. The cyst wall was thickened and its separation from surrounding tissue was difficult. On opening the cyst, there was no colloid material, but a brownish yellow organized clot was found which was removed piecemeal. The clot was easily separated from the inner cyst wall. After almost complete evacuation of cyst content, the thick wall was held with biopsy forceps with one hand and with the other hand it was dissected from the surrounding parenchymal tissue. A complete excision of the cyst was ensured. Both internal cerebral veins were seen and found intact. Pathological examination confirmed hemorrhage within the colloid cyst. Microscopic examination also showed blood clots containing hemosiderin laden macrophages and many rhomboid cholesterol crystals and chronic inflammatory cells [Figure 3]. Post-operatively she had no seizures. Her memory function completely improved over a 3 week period.

Figure 3

Photomicrograph. (a) Pseudostratified columnar epithelial cells with occasional ciliated and goblet cells with a thin capsule of fibrous connective tissue suggestive of colloid cyst presence of red blood cells, hemosiderin laden macrophages, rhomboid cholesterol crystals, and chronic inflammatory cells suggestive of hemorrhage in colloid cyst (H and E staining, Magnification 10×). (b) showing cuboidal-to-pseudostratified columnar epithelial cells (H and E staining, Magnification 40×).

Discussion

Colloid cysts are benign thin walled cystic lesions located at the anterior part of third ventricle, close to foramen of Monro and contain colloid material.[2] These are rare cystic lesions with incidence of 1% per million person-years in epidemiological studies and about 1 per 8500 persons in post-mortem series which suggest that a small percentage become symptomatic.[345678]

Although colloid cysts are congenital tumors, their presentation in childhood is rare. They are usually symptomatic in patients aged 20-50 years. They consist of about 0.5-1% of primary brain tumor and 15-20% of intraventricular masses.[1] The origin of these cysts continues to be uncertain. Diencephalic ependymoma, invagination of neuroepithelium of the ventricle, or the respiratory epithelium of endodermal origin are the other etiological possibilities.

With the advent of CT/MRI, the numbers of asymptomatic colloid cysts have increased. The natural history is varied and most of the patients have an insidious course. These epithelium lined cysts are troublesome because of their location; they can create serious morbidity and occasionally mortality due to acute obstructive hydrocephalus, and very rarely due to intracystic hemorrhage.[91011] Treatment of asymptomatic and insidiously diagnosed cyst is debatable. Most neurosurgeons advise intervention to prevent life-threatening complications, even though a long-term follow-up study of 68 subjects with asymptomatic colloid cysts suggests that only a few (8%) subjects go on to develop symptoms at 10 years.[12]

Memory deficit, headache, and cognitive disturbances are most usual symptoms. Dementia, gait disturbances, and urinary incontinence are other modes of presentation. These symptoms are mostly explained by associated hydrocephalus caused by CSF flow obstruction at the level of foramen of Monro. This non-communicating hydrocephalus is the most widely accepted explanation for the cases with acute death. It is not clear why colloid cysts should give rise to an acute and complete obstruction. A sudden change in the pressure/volume compliance in the cranial cavity may result in change in the size and location of the cyst. This might be the reason for sudden bilateral blockade of foramen Monro and sudden neurological deterioration. Although hemorrhagic changes of colloid cysts can give rise to an acute increase of cyst dimension resulting in an acute and complete obstruction of CSF circulation, the cause of hemorrhage is not known. In our case, besides headache and vomiting, no life-threatening clinical symptoms were observed. It implies that it is not necessary that hemorrhage will increase in size of cyst and that will induce hydrocephalus and deterioration in clinical condition.

Hemorrhagic colloid cysts are rare. On literature review, we found only six cases of a hemorrhagic colloid cyst; among them four were diagnosed at post-mortem examination.[11314151617]

Beems et al described the first clinically diagnosed hemorrhagic colloid cyst with a disastrous clinical course.[1] They reported a 35-year-female patient, a diagnosed case of a colloid cyst, who had sudden deterioration one day prior to surgery because of acute hemorrhage in a colloid cyst. In spite of emergency ventriculostomy and endoscopic subtotal excision of cyst, outcome remained relatively poor. The authors concluded that the possibility of hemorrhagic changes in a colloid cyst should be weighted in treatment decisions. Farooq et al reported a rare case of hemorrhage in a colloid cyst in 9-year-old girl who presented with headache, nausea, and sudden deterioration in mental status.[13] She underwent emergency ventriculostomy, craniotomy, and resection of cyst. She had an excellent recovery. Besides these two cases, four post-mortem proven cases of the hemorrhagic colloid cyst have been published [Table 1].[14151617]

Table 1

Reported cases of post-mortem-proven hemorrhagic colloid cyst

The optimal surgical management of colloid cysts continues to be a matter of debate. There are various approaches for the management of symptomatic colloid cysts. The operative procedures usually consist of open microsurgical excision techniques.[910181920] Open microsurgical excision was traditionally considered the “gold standard” treatment. However, operative approaches are not without risks. A transcallosal approach avoids the potential risks of a cortical incision, but still it is associated with occasionally serious morbidity. The reported complications of this approach include cortical venous infarct, forniceal damage, and injury to the deep venous system, subdural hematoma, disconnection syndromes, ventriculitis, and meningitis.[9192122]

Less invasive procedures like stereotactic aspiration or stereotactic-guided surgeries as well as an endoscopic approach have also been described.[21232425] The later has an advantage of excellent visualization and minimally invasive corridor through the naturally dilated ventricular system. The quest for less invasive approaches to manage these lesions led to the introduction of aspiration techniques for decompression of the cyst.

The minimally invasive approach to deal with this lesion includes free-hand aspiration which was first described in 1957 by Gutierrez-Lara et al.[26] Accuracy of this procedure was enhanced by the help of the sterotaxy device, and it was used first by Bosch and co-workers.[27] Powell et al are credited with the first successful endoscopic aspiration of the colloid cysts.[28] Auer et al used a neodymium:yttrium-aluminum-garnet laser to endoscopically coagulate the cyst wall.[29] Lewis and Kohler have compared craniotomy and endoscopic procedures for colloid cyst resection and have found shorter operative times and hospital stay in patients undergoing endoscopic surgery.[18] However, Kondziolka and colleagues found that cysts which are hypodense or isodense on plain CT may only be amenable to successful aspiration.[25] Endoscopic transventricular resection strikes a fine balance between the morbidity of open surgery and the low efficacy of needle aspiration. Moreover, leaving the mucin-secreting wall behind after aspiration increases the possible risk of recurrence.[23]

A hemorrhagic colloid cyst can create a management problem as it cannot be considered similar to a simple colloid cyst. A thickened cyst wall and the content of the cyst preclude needle aspiration. Manipulation of well-defined solid mass without decompression is always at the cost of damage to surrounding vital structures. The solid adherent clot cannot be sucked out and piecemeal removal is inevitable. We feel that even though endoscope may have several advantages over the microscope, an open microsurgical technique should be considered the best treatment option for a hemorrhagic colloid cyst.