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The objective is to describe a rare clinical case of a suprasellar cyst formed after implantation of a ventriculoperitoneal shunt. Clinical observation . A female patient, 51 years, suffered non-traumatic subarachnoid intraventricular hemorrhage, acute obstructive and subsequently normal pressure hydrocephalus requiring implantation of a ventriculoperitoneal shunt. In several years, the patient developed a suprasellar cyst manifesting through cerebral symptoms. Results . Two (2) surgeries were performed. During the 1st surgical intervention, endoscopic ventriculostomy was performed with positive outcome (full regression of the symptoms). After 6 months, cyst recurrence was confirmed, and endoscopic ventriculocystocisternostomy with replacement of a low pressure pump with intermediate pressure pump was performed. Full regression of neurological disorders was observed; magnetic resonance imaging did not show cyst recurrence 6 months after the 2 nd surgery. Conclusion . This clinical observation expands our knowledge on the diversity of mechanisms of suprasellar arachnoid cyst formation and demonstrated effectiveness of one of the techniques for prevention of cyst formation in the context of ventriculoperitoneal shunt.

Arachnoid cysts represent 1% of all intracranial lesions and appear on the arachnoid membrane (subarachnoid space). The etiology and pathogenesis of arachnoid cysts remain unclear and in their majority they are asymptomatic. Due to the developmental origin of arachnoid cysts, these formations can be identified on prenatal fetal imaging. In this report we presented a case of an enlarged suprasellar arachnoid cyst detected incidentally during a routine fetal sonographic examination at 33 weeks of gestation. The cyst remained stable in size prenatally and postnatally without compressive symptoms and signs and the patient has been set under expectant management with serial clinical evaluation and serial imaging of the cyst.

Arachnoid cysts (ACs) are malformations that account for about 1% of all intracranial lesions. The aetiology and progression of these lesions have been debated, with one possible explanation being the production of cerebro-spinal fluid (CSF) by ectopic choroid plexus (CP). To our knowledge, only seven cases of ACs incorporating CP have been reported, and we believe this to be the first reported case of a suprasellar AC containing ectopic CP. A 1-year-old boy presented with developmental delay and macrocephaly. MRI scan revealed hydrocephalus due to a suprasellar AC. An endoscopic ventriculocisternostomy was undertaken. Intra-operatively, intra-cystic, pink frond-like tissue resembling choroid plexus was identified. Histologically, the cyst wall was composed of fibrous tissue, with layered arachnoid cells, while the frond-like tissue was composed of papillary structures in keeping with normal choroid plexus tissue. We postulate that the rest of the ectopic CP may have been trapped within the double layered arachnoid fold of the diencephalic leaf of Liliequist's membrane which may drive the formation and development of certain suprasellar ACs.

Background: The ectopic posterior pituitary (EPP) is a rare condition characterized by the ectopic location of the posterior pituitary lobe associated with varying degrees of stalk anomalies. The arachnoid cysts (AC) are benign lesions of the arachnoid, which account for 1% of all intracranial space-occupying lesions. Sellar/suprasellar ACs account for approximately 1% of all ACs. This is the first case of coexistence EPP with sellar/suprasellar AC. Case Report: A 67-year-old woman presented with 6 months history of fatigue. Her medical history was positive for irregular menstruation. Her endocrine examinations indicated low free thyroxine level with low TSH level, low oestradiol with low gonadotrophin level, slightly elevated prolactin level. Her Insulin-like growth factor-1 was below the normal levels. Dynamic contrast hypophysis MRI revealed a sellar cystic lesion with a dimension of 18 × 14 × 14 mm, extending from the suprasellar cistern, traversing the diaphragma sellae and reaching the level of the floor of the 3rd ventricle, consistent with sellar/suprasellar AC. There was no wall enhancement. The optic chiasm was compressed. The precontrast T1-weighted magnetic resonance images did not demonstrate the characteristic bright spot of posterior pituitary within the sella, which was higher in position, in the region of the median eminence. The pituitary stalk was not present. Conclusion: Although speculative, we have a hypothesis to explain how the EPP and sellar/- suprasellar AC coexist in this patient. Due to the absence of stalk, CSF may enter the sella tursica from the central aperture of the diaphragma sellae through which normally the stalk passes.

In this article, we show the endoscopic views within a suprasellar arachnoid cyst.

Background and Importance: Suprasellar arachnoid cysts are insidious and progressive ventricular cysts that cause hydrocephalus, ataxia, optic, and endocrinological symptoms. These cysts can be successfully treated via an endoscopic ventriculocystocisterostomy (VCC). Clinical Presentation: A three-year-old patient presented with emesis and bobble-head doll syndrome with a suprasellar arachnoid cyst. Through our video, we present our neuronavigation-assisted endoscopic VCC without cautery and the relevant neuroanatomy. Conclusion: Neuroendoscopic fenestration is a preferred approach over resection or shunting for a suprasellar arachnoid cyst. In addition, VCC has decreased recurrence and increased radiologic improvement over a ventriculocystostomy (VC).

Background Case 1: Mum was a 30 years old multiparous woman and known smoker. Antenatal ultrasound scan showed an intracranial arachnoid cyst measuring 14 × 13 mm, that continued to grow in subsequent scans. Baby was born at 37+5 weeks and day 2 cranial ultrasound scan showed a large cystic lesion on the suprasellar region measuring 17 × 17 mm. MRI on day 6 of life showed a large suprasellar arachnoid cyst 18 × 22 mm in size which was causing significant mass effect displacing and compressing the midbrain and basal ganglia. As the baby was clinically asymptomatic and progressing and developing appropriately, the neurosurgical team opted for a conservative management with a multidisciplinary approach with involvement of ophthalmology, endocrinology, and local general paediatricians for close monitoring of growth and development as well as regular blood tests (Renal function and pituitary function). He remains well until the present and is currently 15 months old. Case 2: Mother was 34 years old smoker and multiparous. Antenatal ultrasound scan showed a left sided 25 × 33 mm intracranial arachnoid cyst. Postnatal cranial ultrasound performed on day 3 of life showed a large arachnoid cyst 37 × 47 mm in size on the left posterior area of the brain. At the Neurosurgical follow up clinic MRI showed the cyst was stable in size with mild mass effect noticed. The baby remains under close follow up and monitoring by the neurosurgical team, with conservative management until the present at age of 16 months. Objectives Methods Results Review & Discussion: Arachnoid cysts are benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane. They are most commonly developmental anomalies, however some are acquired and they represent 1% of all space occupying lesions in childhood. Arachnoid cysts in the antenatal period can be found in isolation or with other intracranial abnormalities associated. The antenatal diagnosis should be confirmed by neonatal neuroimaging; cranial ultrasonography is the first line imaging modality and should happen within the first 24 hrs of age. Brain MRI is the gold standard investigation and should happen within the first 2 weeks of life. Antenatally diagnosed arachnoid cysts may remain clinically silent in postnatal period or even regress spontaneously; but they usually have a tendency to grow rapidly in the perinatal period. There is no clear guidance on the most appropriate management strategy for these cases. Management include different types of neurosurgical procedures, but endoscopic ventriculocystocisternostomy seems to be the overall preferred approach. Conclusions Arachnoid cysts are rare and can remain silent during childhood before presenting with clinical manifestations. Rapid enlargement of incidentally diagnosed antenatal arachnoid cysts can occur in perinatal period. Serial evaluation by imaging in the perinatal period is necessary to demonstrate the rapid cyst enlargement and the need for appropriate intervention. Neurosurgical approach is variable and dependent on clinical features and the department’s experience or preference, but the cysts which occur in isolation without any other intracranial anomalies and with normal karyotype usually lead to a good outcome.

Suprasellar arachnoid cysts represent a rare occurrence in the pediatric population and usually cause symptoms related to mass effect and can occasionally cause endocrine dysfunctions. The association between SAC and the syndrome of inappropriate antidiuretic hormone (SIADH) in the pediatric population has rarely been described previously. In most cases, SIADH is temporary and resolves by treating the underlying cause. The first-line treatment consists of fluid restriction in asymptomatic children. Oral urea and demeclocycline are other effective treatment options. Vaptans are a new class of medication for the management of SIADH. These agents are a nonpeptide vasopressin V2 receptor antagonist that selectively antagonizes the antidiuretic effect of AVP, resulting in excretion of diluted urine or “aquaresis.” Their efficacy has been shown in adult patients with euvolemic or hypervolemic hyponatremia. However, evidence is lacking in pediatric patients with SIADH. We report the case of a 9-year-old female child with a SAC, who underwent endoscopic fenestration at the age of 2 years. After surgery she developed chronic hyponatremia due to SIADH. Hyponatremia was refractory to treatment with fluid restriction, oral sodium, and urea. In order to normalize serum sodium levels, tolvaptan treatment was started on a compassionate-use basis; 24–48 h later serum sodium levels returned to normal. To date, tolvaptan has been used regularly for 6 years with no side effects occurring during the treatment period. This is the first case of a child with chronic SIADH secondary to SAC successfully treated with tolvaptan. Further studies are needed to demonstrate its usefulness on a broader case series.

A 19-year-old woman reported progressively worsening involuntary head movements since the age of 7. She had visited multiple hospitals and been labelled as having a functional disorder. Detailed evaluation identified a large supra-sellar arachnoid cyst with hydrocephalus. We made a diagnosis of bobble-head doll syndrome, which is only very rarely associated with suprasellar arachnoid cyst. Her symptoms improved rapidly following endoscopic third ventriculostomy with cyst fenestration and gradual decompression of the ventricles. Clearly, not all suppressible movements should be labelled as psychogenic.

A male 3-year-old child was referred to our service due to increasing head size and mild headache. On physical examination, head circumference was 55 cm (bigger than 97th percentile), the child was conscious, alert and oriented to person, place, time and event, his pupils were isochoric, the light reflex was bilaterally positive, eye movements showed a slight palsy on the abduction of the left eye, and a partial vision loss on the left eye was detected. Gait was also impaired. MRI presented a cystic lesion in the suprasellar region suggesting arachnoid cyst, associated with hydrocephalus. An endoscopic ventricular approach was performed through a straight incision over Kocher’s point. The cyst was easily identified, coagulated with cautery and incised with endoscopic scissors. A ball-valve mechanism was well visualized. Prepontine cistern was also sharply opened and the whole cyst wall was coagulated. The patient was discharged on the second postoperative day showing a clinical improvement. Late head CT presented also a radiologic improvement.

Objective Suprasellar arachnoid cysts are a rare but important pediatric neurosurgical pathology with unknown ideal management. They have been previously managed with techniques including open craniotomy with microsurgical fenestration, cystoperitoneal shunting, endoscopic ventriculocystostomy, and endoscopic ventriculocystocisternostomy (VCC), without a consistent consensus on the best surgical approach. We present an overview of the literature on surgical management of suprasellar arachnoid cysts. Methods A literature search following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted for all articles evaluating treatment modalities for suprasellar arachnoid cysts, using PubMed, OVID, and Web of Science. Results Twenty-five articles on management of suprasellar arachnoid cysts in children were identified. Few published studies exist that examine different types of surgical management across a single institution. The majority of studies reported best clinical outcomes in patients treated with endoscopic approaches when compared with microsurgical fenestration or cystoperitoneal shunting, reporting lower rates of infection, shunt dependence, and need for revision in addition to better resolution of clinical symptoms. Furthermore, most studies argue that VCC is superior to ventriculocystostomy, offering better long-term improvement of clinical symptoms and lower rates of failure. Conclusions This study examines the current literature on suprasellar arachnoid cyst surgical management to conclude that an endoscopic approach in comparison with other approaches has the best outcomes. Of the endoscopic options available, VCC provides patients with the best long-term resolution of symptoms and the lowest need for revision. These findings should be further investigated with larger multicenter studies to further compare different surgical techniques and outcomes.

A 22-year-old man was admitted with gradually aggravating stereotypic head movement with hypomania. Brain magnetic resonance imaging showed a large suprasellar arachnoid cyst extending into the third ventricle, with obstructive hydrocephalus, characteristic of bobble-head doll syndrome. Endoscopic fenestration of the suprasellar arachnoid cyst was performed. Stereotypic head movement stopped immediately after surgery and hypomanic symptoms gradually improved within a month. During 4 years of follow-up observation without medication, neuropsychiatric symptoms did not relapse. We report our experience of surgically treating stereotypy and hypomania in a case of bobble-head doll syndrome and discuss the possible neuropsychiatric mechanisms of this rare disease.

Introduction: Intracranial arachnoid cysts are rare in adults. We report a large suprasellar arachnoid cyst involving a third ventricle in a 19 year old male. Case report: A 19 year old male with mild cognitive impairment was admitted after an episode of loss of consciousness (LOC). A type 1 suprasellar arachnoid cyst (SAC-1) was diagnosed on CT (CT) and magnetic resonance (MR) imaging. Endoscopic fenestration was performed. At surgery, the cyst wall was noted to be markedly thickened with prominent vascularity. Histopathology showed thickened and densely fibrotic meninges, an unusual feature for an arachnoid cyst. 36 months after surgery, the patient remains asymptomatic. Keywords: Arachnoid cysts, Suprasellar, Intracranial, Adult, Hydrocephalus

Background and Importance: Arachnoid cysts are benign nontumoral lesions that are usually found incidentally in brain imaging. Suprasellar Arachnoid Cysts (SSACs) are rare. They can cause hydrocephalus and increased intracranial pressure, lower cranial nerve impairment and endocrine disorder. Sometimes arachnoid cysts become complicated by subdural hematoma/hygroma. Case Presentation: We describe a patient with a suprasellar arachnoid cyst that was complicated with symptomatic subdural effusion and increased intracranial pressure after minor trauma. Conclusion: Although these cysts are rarely complicated with subdural hematoma/hygroma, and intracystic hemorrhage, the probability of subsequent subdural hygroma and increased intracranial pressure should not be underestimated.

Background Arachnoid cysts are uncommon lesions of the sellar region, and only rarely require decompressive surgery to treat symptoms. Pituitary abscesses are another rare lesion, and are an uncommon complication of pituitary surgery. A previously healthy 45 year old woman presented with a new finding of bitemporal hemianopsia. Magnetic resonance imaging (MRI) showed a cystic sellar lesion with suprasellar extension and compression of the optic chiasm. Endoscopic transphenoidal surgery was performed and the lesion was found to be an arachnoid cyst. She was well immediately after the operation, but 1 week later presented with headaches, fever and worsening visual acuity. MRI showed a homogenous collection in the sellar region that was compressing the chiasm. The patient was treated with antibiotics, and a second transphenoidal operation was performed, with frank pus found in the pituitary fossa. Cultures of the fluid found during the operation grew Escherichia coli and Staphylococcus lugdunensis. Her symptoms resolved after the second operation, and formal visual fields and a pituitary hormone panel were normal at this time. We report on a rare case of a pituitary abscess complicating surgery for a sellar arachnoid cyst, and discuss management of these conditions.

Bobblehead-Doll Syndrome A 5-year-old girl presented with a 2-year history of excessive head nodding. MRI of the head revealed a suprasellar arachnoid cyst and ventriculomegaly. A video shows the child displaying movements typical of the bobblehead-doll syndrome.

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.

Objective To investigate the feasibility and reliability of virtual endoscopy (VE) as a rapid, low-cost, and interactive tool for the diagnosis and surgical planning of suprasellar arachnoid cysts (SACs). Methods Eighteen patients with SACs treated with endoscopic ventriculocystostomy were recruited, and 18 endoscopic patients treated with third ventriculostomy were randomly selected as a VE reconstruction control group. After loading their DICOM data into free 3D Slicer software, VE reconstruction was independently performed by 3 blinded clinicians and the time required for each reconstruction was recorded. Another 3 blinded senior neurosurgeons interactively graded the visibility of VE by watching video recordings of the endoscopic procedures. Based on the visibility scores, receiver operating characteristic curve analysis was used to investigate the reliability of VE to diagnose SACs, and Bland-Altman plots were used to assess the reliability of VE for surgical planning. In addition, the intraclass correlation coefficient was calculated to estimate the consistency among the results of 3 reconstruction performers. Results All 3 independent reconstructing performers successfully completed VE simulation for all cases, and the average reconstruction time was 10.2 ± 9.7 minutes. The area under the receiver operating characteristic curve of the cyst visibility score was 0.96, implying its diagnostic value for SACs. The Bland-Altman plot indicated good agreement between VE and intraoperative viewings, suggesting the anatomic accuracy of the VE for surgical planning. In addition, the intraclass correlation coefficient was 0.81, which revealed excellent interperformer consistency of our simulation method. Conclusions This study substantiated the feasibility and reliability of VE as a rapid, low-cost, and interactive modality for diagnosis and surgical planning of SACs.

The arachnoid cyst is a lesion commonly encountered in neurosurgery, especially in pediatric pathology. We are presenting the case of an adult patient with a suprasellar arachnoid cyst with giant perimesencephalic and mesial temporal extension discovered incidentally, where there is a discrepancy between the spectacular neuroimaging and the non-specific symptomatology. Some of the physiopathological mechanisms which led to the evolution of the cyst will also be presented.

Keyhole approaches have lately sparked strong interest because these approaches address skull base pathologies as reported by Eroglu et al. (World Neurosurg, 2019); Hickmann, Gaida, and Reisch (Acta Neurochir (Wien) 159:881–887, 2017); Jallo and Bognar (Neurosurgery, 2006); and Poblete et al. (J Neurosurg 122:1274–1282, 2015), minimizing brain retraction and improving cosmetic results. We describe the step-by-step surgical technique to drain a suprasellar arachnoid cyst by a supraorbital approach. The eyebrow incision is a direct route to expose the supraorbital corridor and even if it is smaller than a pterional approach, it permits to open the cisterns and to visualize neurovascular structures. The arachnoid cyst could be safely drained and a T-tube is placed. This technique represents a suitable option for suprasellar arachnoid cyst, avoiding more extended and invasive approaches.

A 9-year-old boy with precocious puberty was presented with severe headache and vomiting, despite having a functioning ventriculoperitoneal shunt, from the age of one year for hydrocephalus. Magnetic resonance imaging revealed a tense suprasellar arachnoid cyst indenting the third ventricle. Decompression of cyst improved his headache and the features of precocious puberty at follow-up. His hormonal levels were not abnormal on gross neuroendocrine evaluation. It was concluded that suprasellar arachnoid cyst may manifest with features similar to raised intracranial pressure despite having a functioning shunt for associated hydrocephalus. Precocious puberty in such children may manifest without gross endocrinological abnormalities.

Suprasellar cysts arachnoid cysts (SSAC) are relatively uncommon making up less than 2% of all arachnoid cysts. Suprasellar arachnoid cyst causing precocious puberty is infrequent. The pathogenesis remains unknown in the majority of patients with precocious puberty. Endoscopic ventriculocystocisternostomy procedure is an effective and minimally invasive method in the treatment of these cysts. This report describes a 7-year old male who had a huge intracranial arachnoid cyst with initial symptoms and signs of sexual precocity. Magnetic resonance image (MRI) of the brain revealed the presence of large suprasellar cystic lesion. Case Study Badri et al.; BJMMR, 7(9): 795-800, 2015; Article no.BJMMR.2015.390 796

Paciente do sexo feminino, 36 anos, que apresentava dor de cabeca e visao embacada por 2 semanas. Seu historico medico foi positivo para menstruacao irregular e hipotireoidismo. O teste de campo visual revelou defeitos nos quadrantes superiores bilaterais, e os exames de sangue indicaram pequena elevacao dos niveis de prolactina (24,4; variacao de 4,8 a 23,3), cortisol reduzido no periodo da manha (3,8; variacao 4 a 22) e niveis do hormonio do crescimento reduzidos (

Suprasellar arachnoid cysts (SACs) are benign congenital collections of cerebrospinal fluid (CSF), accounting for approximately 9% of all arachnoid cysts [1–4]. These cysts progressively enlarge from an abnormality in the interpeduncular cistern or in the membrane of Liliequist. They can be classified as communicating cysts; that is, cystic dilatations of the interpeduncular cistern, and as non-communicating intra-arachnoid cysts of the diencephalic portion of the membrane of Liliequist [5]. In communicating cysts, the basilar artery bifurcation is located inside the cyst with no overlying membrane, whereas in a non-communicating cyst, the basilar artery and its branches are pushed posteriorly against the brainstem, and the cyst can be observed through a transparent membrane (diencephalic portion of the membrane of Liliequist). A new recent classification divides SACs into three types: SAC-1, SAC-2, and SAC-3. SAC-1 may arise from an expansion of the diencephalic portion of the membrane of Liliequist. SAC-2 shows a dilatation of the interpeduncular cistern and corresponds to a defect of the mesencephalic portion of the membrane of Liliequist. SAC-3 is an asymmetrical form that expands to other subarachnoid spaces [6]. However, even on a high-definition intraoperative view it can be difficult to distinguish these types of SACs, because the diencephalic portion of the membrane of Liliequist is very thin and transparent. Due to this difficulty this classification is not applied to the intraoperative images shown here.