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CASE REPORT
Year : 2020  |  Volume : 15  |  Issue : 1  |  Page : 155-158

Spontaneous regression of pineal region arachnoid cyst: A case report and review of literature


Department of Neurosurgery, King Fahad University Hospital, Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia

Date of Submission16-Sep-2019
Date of Acceptance20-Dec-2019
Date of Web Publication25-Feb-2020

Correspondence Address:
Abdulrazaq Abdulmohsen Alojan
Department of Neurosurgery, King Fahad University Hospital, Imam Abdulrahman Bin Faisal University, Dammam
Saudi Arabia
Prof. Ahmed Ammar
Department of Neurosurgery, King Fahad University Hospital, Imam Abdulrahman Bin Faisal University, Dammam
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajns.AJNS_289_19

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  Abstract 


Arachnoid cyst is a rare benign cerebrospinal fluid-filled cyst that can develop anywhere in the brain along the arachnoid membrane and usually unaccompanied by the anomalous development of the brain structure. These cysts are usually located in the middle cranial fossa. However, they are also denoted in other regions. Arachnoid cysts are mostly asymptomatic and diagnosed incidentally. Spontaneous regression of arachnoid cysts in different anatomical regions of the brain has been reported in the literature. However, to the best of our knowledge, this is the first case reporting an unusual spontaneous regression of arachnoid cyst in the pineal region in a 3-year-old child presented to our hospital with hydrocephalus without alarming signs and was treated conservatively as the patient was stable, and the cyst showed spontaneous regression. A comprehensive review of the literature regarding spontaneous regression of arachnoid cysts has been collected and discussed in this article.

Keywords: Arachnoid cyst, benign, incidental, pineal region, spontaneous regression


How to cite this article:
Ammar A, Alojan AA, Turkistani AN, Alrayes MM. Spontaneous regression of pineal region arachnoid cyst: A case report and review of literature. Asian J Neurosurg 2020;15:155-8

How to cite this URL:
Ammar A, Alojan AA, Turkistani AN, Alrayes MM. Spontaneous regression of pineal region arachnoid cyst: A case report and review of literature. Asian J Neurosurg [serial online] 2020 [cited 2020 Apr 6];15:155-8. Available from: http://www.asianjns.org/text.asp?2020/15/1/155/279032




  Introduction Top


Arachnoid cysts are a rare benign congenital central nervous system lesions which consist of cerebrospinal fluid (CSF) and surrounded by histologically normal layers of arachnoid membrane in the absence of connection with the ventricular system.[1],[2] They account for only 1% of all intracranial space-occupying lesions and usually unaccompanied by the anomalous development of the brain structure. In general, arachnoid cysts are asymptomatic, and detection is mostly incidental.[1],[3]

Most arachnoid cyst cases are sporadic with a negative family history.[4] However, familial cases of arachnoid cysts have been reported in the medical literature, suggesting that genetic predisposition may play a role in some patients.[5] In familial cases, inheritance could be autosomal recessive.[4]

They are frequently located in the middle cranial fossa (MCF). However, they are also denoted to be seen in the suprasellar, quadrigeminal cistern, cerebral convexity, posterior fossa, and interhemispheric fissure.[1],[2],[3]

These lesions are usually located in the MCF and mostly develop in the left side of the brain with male predominance by a rate of 3/1.[1],[2]

On computed tomography (CT) scans magnetic resonance imaging (MRI), they appear extraaxial, well-circumscribed, simple cystic lesions. They are isodense to CSF on CT and isointense to CSF on all MR image sequences.[6]

Although most cases of arachnoid cysts remain limited and asymptomatic,[2] sometimes, they might manifest when they are located in the MCF and large enough to cause signs and symptoms related to intracranial mass effect or disruption of CSF dynamics.[1] Infrequently, more serious life-threatening complications may occur such as cystic rupture, intracystic bleeding, or seizure attacks, making arachnoid cysts not so benign as commonly believed.[2]

The posterior fossa region is the second most common region for those lesions, since they are often detected in the cerebellar or cerebellopontine region.[7] It is necessary to differentiate those located infratentorially from other posterior fossa lesions, such as Dandy–Walker syndrome variant, mega cisterna magna, abscesses, and cystic tumors.[8],[9]


  Case Report Top


A 3-year-old boy product of cesarean section at 38 weeks of gestation with a relatively large head sought medical advice at our hospital at the age of 4 months as a case of hydrocephalus. Prenatal ultrasound revealed a brain cyst with mild ventricular dilatation. No maternal history of oligo or polyhydramnios. No history of vomiting or signs of increased intracranial pressure, and the patient was feeding well. He was active, moving all limbs, and pupils were equally reactive bilaterally. The anterior fontanelle was flat and soft, and he was developmentally appropriate to age. Brain MRI [Figure 1] showed a well-defined cystic lesion measuring 2 cm × 2.8 cm × 3.2 cm in its maximum transverse, anterior-posterior and craniocaudal dimensions exerting mass effect over the brain stem anteriorly and causing lateral and third ventricular dilatation. Findings are suggestive of pineal region arachnoid cyst. He was planned for surgery, yet repeated MRI brain [Figure 2] showed regression of both ventricles size and the arachnoid cyst which follows the CSF signal intensity with no evidence of pathological enhancement, bleeding, and no diffusion restriction. The patient has been managed conservatively with close observation and follow-up. Since then, the patient remains asymptomatic, and he developed normally with no neurological deficits. The last MRI follow-up showed further regression of ventricular dilatation and arachnoid cyst size with no signs of intracystic bleeding or subdural collection.
Figure 1: Magnetic resonance imaging brain (a) T1 sagittal view (b) T2 coronal view, (c) T2 axial view showed a large pineal region arachnoid cyst measuring 2 cm × 2.8 cm × 3.2 cm in dimensions with secondary significant ventricular dilatation and hydrocephalus

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Figure 2: Magnetic resonance imaging brain (a) T1 sagittal view, (b) T2 coronal view, (c) T2 axial view showed a significant reduction in size (3 years) after follow-up, measuring 1.8 cm × 2 cm × 2 cm in dimensions with reduction of ventricular size

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  Discussion Top


The rupture of arachnoid cysts is a very uncommon sequela of the arachnoid cysts formation.[2] Two mechanisms have been proposed that explain such phenomena. The first mechanism is due to mild head injury that can tear the wall of the arachnoid cyst, causing a flap valve mechanism leading to communication with subarachnoid space. The second proposed mechanism even in patients with no known trauma, the cyst spontaneously resolves into the subdural space following a sudden transient intracranial pressure increase during a Valsalva maneuver.[1],[10]

Most patients present with symptoms of high ICP such as nausea, vomiting, headache, seizure, and rarely diplopia from VI nerve palsy.[11] Parsch et al. in 1997 reported subdural hemorrhage in 2.43% and subdural hygroma in 0.46% of patients with arachnoid cyst noted on MRI.[12] In addition, intracystic bleeding may occur inside the arachnoid cyst as reported by Hong in 2008.[13]

MRI is the modality of choice in the detection of arachnoid cysts because of its ability to demonstrate the exact location, extent, and relationship of the arachnoid cyst to the adjacent brain or spinal cord.[14]

Most arachnoid cysts are found incidentally and can be managed conservatively; however, prophylactic surgical treatment is usually not recommended except in symptomatic candidate where surgery is considered or in the coexistence of mass effect or midline shift. In addition, surgical intervention is considered in cases of epilepsy, severe headache, or neurological deficit proved to be caused by a tense arachnoid cyst. Different neurosurgical options include craniotomy and excision of the cyst, endoscopic fenestration, and cystoperitoneal shunt are the procedures performed in symptomatic arachnoid cyst depending upon the size, location, and clinical presentation. Recent advances in neurosurgical techniques in neuroendoscopy continue to favor fenestration over shunt insertion as a method of choice for initial cyst decompression.[15] It is very important to reduce the risk of recurrence of the arachnoid cyst in which the outer and inner walls must be opened, fenestrated in several points, if removal of the cyst wall is difficult. However, cystoperitoneal shunt is the preferred procedure in patients with raised intracranial pressure,[16] and craniotomy for cyst excision is adopted to treat well-circumscribed cysts that have been also reported. While in the context of cyst rupture, cyst either decreases or disappears after subdural evacuation.[17]

Spontaneous regression of arachnoid cysts in different anatomical regions of the brain has been reported in the literature. A literature-based review was performed using the PubMed search engine of the National Library of Medicine and National Institutes of Health. The following keywords were searched: “arachnoid cyst,” “spontaneous regression,” “spontaneous resolution,” and “spontaneous reduction.” The relevant literature were carefully studied, and the results were summarized in [Table 1]. Twenty-two cases were reviewed and included; different parameters were studied and analyzed, and the results were as follows: the male-to-female ratio was 16:5 with male predominance, the median age was 12 years of age, most cysts were located at the MCF (six cases), and the median duration of the cyst to resolve was 3.5 years. Most of the cases were asymptomatic upon the time of diagnosis. The reason for the spontaneous resolution of the cyst remains unclear, where different cases reported certain incidents which have been followed by cyst resolution; those incidents include head trauma,[33] cesarean delivery,[35] and suppurative meningitis.[19]
Table 1: Literature-based review summarizing the spontaneous regression of arachnoid cyst

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According to the case reported by Bristol et al.,[26] arachnoid cysts have also been associated with other intracranial tumors, where the cyst has completely resolved initially without any history of trauma or surgical intervention; interestingly, the cyst returned and became larger than it was. This observation, where the cyst return after it disappears, is also reported by Arunkumar et al., in which his patient had a midline posterior fossa arachnoid cyst that decreased in size initially, but then the cyst enlarged with worsening in the patient's neurological status.[28]


  Conclusion Top


Spontaneous regression of arachnoid cysts has been reported in different cases and different anatomical regions. Yet, this is the first case describes a spontaneous regression of arachnoid cyst in the pineal region. Although different mechanisms have been suggested in the literature, in our case, we are in favor of the flap contrary one valve mechanism which allows one-way flow of the CSF to get out of the cyst and gradually decreases the cyst internal pressure leading into cyst regression with time. Incidental arachnoid cysts in the context of asymptomatic presentation should be followed with serial MRI and close clinical follow-up, thus surgical management should be deferred as long as the arachnoid cysts remain asymptomatic. This fact should be emphasized in the management's plan of incidental and asymptomatic arachnoid cysts.[37]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms, including intraoperative images, patients imaging scans, and clinical data to be published in this journal. The authors certify that this article has gained an Institutional Review Board certificate from the IRB committee at Imam Abdulrahman Bin Faisal University, Dammam, Saudi Arabia.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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