Spontaneous Resolution of Asymptomatic Pediatric Suprasellar Arachnoid Cysts: Report of 2 Cases and Review of the Literature.

INTRODUCTION: Sellar/suprasellar arachnoid cysts are infrequent nonneoplastic cystic lesions that account for approximately 1% of all arachnoid cysts. While various surgical treatment options have been proposed for symptomatic lesions, treatment guidelines regarding the management of asymptomatic sellar/suprasellar arachnoid cysts remain to be elucidated. This case series aims to provide a better insight into the management and outcomes of such lesions. CASE PRESENTATION: We discuss 2 pediatric patients diagnosed with sellar/suprasellar arachnoid cysts who remained asymptomatic and demonstrated spontaneous gradual resolution of their arachnoid cysts on subsequent imaging studies. CONCLUSION: Review of the literature identified only a handful of previously reported cases of spontaneously disappearing suprasellar arachnoid cysts. There might be a role for conservative management of sellar/suprasellar arachnoid cysts in certain patients who remain asymptomatic.

Trigeminal neuropathy associated with an enlarging arachnoid cyst in Meckel's cave: case report, management strategy and review of the literature.

We describe a rare case and novel management strategy of painful trigeminal neuropathy caused by an arachnoid cyst confined to Meckel's cave. A 57-year-old female presented with several years of progressive trigeminal pain and signs of trigeminal deafferentation, including sensory loss, corneal anesthesia and mastication muscle atrophy. Medical treatment with carbamazepine provided partial and temporary pain control. Surgical treatment was eventually performed by aspiration of the arachnoid cyst through the foramen ovale using a percutaneous approach. The patient experienced relief of pain and improvement of numbness and muscle strength. To our knowledge, this is the first case description of a percutaneous drainage of a Meckel's cave arachnoid cyst.

[Left temporal arachnoid cyst and specific learning disorders associated with Pervasive Developmental Disorders - Not Otherwise Specified (PDD-NOS): contributions of an integrative neuropsychomotor, neuropsychological, psychopathological and neurosurgical approach about a case report in a child (François)]. / Kyste arachnoïdien temporal gauche et troubles spécifiques des apprentissages associés à un trouble envahissant du développement non spécifié (TED-NoS) : apports d'une approche intégrative neuro-psychomotrice neuropsychologique, psychopathologique et neurochirurgicale à propos d'une observation chez un enfant (le cas François).

Left temporal arachnoid cyst and specific learning disorders associated with pervasive developmental disorders - not otherwise specified (PDD-NOS): contributions of an integrative neuro-psychomotor, neuropsychological, psychopathological and neurosurgical approach about a case report in a child (François). With DSM-IV and DSM-IV-TR, the terminology of pervasive developmental disorders (PDD) covers two main categories of infantile disorders: disorders of "strictly" autistic nature and pervasive developmental disorders - not otherwise specified (PDD-NOS). Under the terminology of multiple complex developmental disorder (MCDD), it is proposed to classify children presenting symptoms approaching the psychotic disharmonies and usually diagnosed as PDD-NOS. Such a category of developmental disorders is now included without nosographic distinction in the autistic spectrum in the Diagnostic and Statistical Manual of mental disorders (DSM-V). CASE REPORT: We are reporting a case report of a 6-year-old boy which shows a PDD-NoS/MCDD complex symptomatology type. This child presents multiple disorders: minor neurological signs (soft signs), neuro-psychomotor disorders, developmental coordination disorder (DCD), communication, thought, and regulation of emotions disorders, attention deficit disorders (ADD); in the presence of a high verbal intellectual potential, which makes it difficult to establish a clear diagnosis. A cerebral magnetic resonance imaging (MRI) was carried out due to the presence of minor neurological signs (soft signs) and of neurodevelopmental multiple disorders. The MRI revealed a voluminous arachnoid temporo-polar left cyst with a marked mass effect on the left temporal lobe. DISCUSSION: A neurosurgical intervention allowed to observe the gradual disappearance of the specific symptomatology (in particular soft signs, neuro-psychomotor functions and autistic symptoms) secondary to the interference of the cyst's pressure with intracranial areas involving neurological and psychopathological abnormalities, underlying at the same time the reversibility of the disorders after decompression as demonstrated in some studies. There are always, with a quantitative and qualitative decrease, an emotional dysregulation, a DCD, an ADD as well as impairments in the executive functions. CONCLUSION: This clinical case underlines the necessity of an evaluation in a transdisciplinary way and to follow the developmental evolution of the child in order to focus adapted therapeutics. Furthermore, with neurodevelopmental disorders not specified, it is important to examine the presence of soft signs with standardized neuro-psychomotor assessment, and then, to propose an MRI investigation. To our knowledge, this is the first report in the literature with a school age child of an unusual association between a temporal arachnoid cyst associated with PDD-NOS/MCDD.

[Arachnoid cysts of the central nervous system. Algorithms and recommendations for management]. / Quistes aracnoideos del sistema nervioso central. Algoritmos y recomendaciones generales de manejo.

The symptoms related to the presence of arachnoid cysts in the Central Nervous System depend on the size of the cyst and its growth rate, its location and, in some cases, the associated CSF dynamic disorder. Sometimes there is acute clinical presentation due to cyst rupture or acute bleeding. Although it is generally accepted that asymptomatic or paucisymptomatic cysts do not require surgical treatment, there is no consensus on the therapeutic approach of choice in symptomatic cases. The aim of this paper is to review the literature, analyzing the pros and cons of the three main surgical options (microsurgery, neuroendoscopy, and CSF shunt) based primarily on the location of the cyst. Although treatment must be always individualized, basic management recommendations may be offered.

Characteristics and management of arachnoid cyst in the pediatric headache clinic setting.

OBJECTIVE/BACKGROUND: Arachnoid cysts are generally identified incidentally on brain imaging, although they occasionally cause symptoms because of expansion or bleeding. This study aims to describe patients in whom an arachnoid cyst was identified on magnetic resonance imaging (MRI) study performed for the evaluation of headache in a pediatric headache clinic and to highlight the clinical dilemma posed by this finding. METHODS: A retrospective descriptive study design was used. The electronic database of a tertiary pediatric headache clinic was searched for all newly admitted patients with headache who underwent MRI evaluation in 2008-2013. The indications for imaging were based on clinical practice parameters recommended by the Subcommittee of the American Academy of Neurology. Clinical and imaging parameters were collected from the files. Findings were compared between patients with and without an arachnoid cyst. RESULTS: Of the 250 (31%) of 800 patients who met the inclusion criteria, 11 (4.4%) had an arachnoid cyst. Two patients had a ruptured cyst with midline shifting and a large subdural collection. Both presented with headache, vomiting, phonophobia, and photophobia. In the other 9 asymptomtic patients with an arachnoid cyst, imaging showed only a mild mass effect without midline shifting; their symptoms were considered unrelated to the cyst. The patients with a symptomatic arachnoid cyst were referred for surgery, with good outcome. CONCLUSIONS: Arachnoid cysts are found in a small percentage of brain scans performed for evaluation of headache in the setting of a hospital-based pediatric headache clinic. For the long run in these clinical settings, most of the cysts are asymptomatic. Precise anamnesis, neurologic examination, and imaging performed according to accepted practice guidelines may help clinicians determine if the headache and symptoms are caused by the cyst or if they should seek primary headache diagnosis with overlapping symptoms. The clinical distinction between symptomatic and asymptomatic patients (symptoms that are directly related to the arachnoid cyst) may be difficult. Family history of migraine may help in the diagnosis of asymptomatic patients.

Treatment options for intracranial arachnoid cysts: a retrospective study of 69 patients.

The best surgical treatment of cerebral arachnoid cysts is yet to be established. Treatment options are shunting, endoscopic fenestration or microsurgical fenestration through craniotomy.Data from 69 patients with cerebral arachnoid cysts treated in our institution between 1997 and 2007 were reviewed.Cysts were located infratentorially in 20% (n = 14) and supratentorially in 80% (n = 55); of these 73% (n = 40) were in the middle cranial fossa. Mean cyst size was 61 mm (range 15-100 mm). The most common symptoms were headache (51%), dizziness (26%), cranial nerve dysfunction (23%), seizure (22%), nausea and vomiting (18%), and hemiparesis (13%). Surgery was performed in 83% (n = 57). First-line treatments were microsurgical fenestration (n = 30), endoscopic fenestration (n = 15), and cystoperitoneal/ventriculoperitoneal shunting (n = 11). More than one intervention was needed in 42% (n = 24). A particularly high rate of relapse (73%) was observed after endoscopic fenestration, following which 11 patients were admitted for reoperation. By comparison, only eight patients (28%) managed with microsurgical fenestration and four (36%) in the shunted group needed a second surgical procedure. Mean follow-up was 30 months. In the surgical series 79% (n = 45) had a good outcome.We conclude that the surgical treatment of arachnoid cysts has an overall good outcome. In our institution the best results were obtained with microsurgical decompression through craniotomy.

Hydrocephalus and arachnoid cysts.

AIM: The management of arachnoid cysts (AC) remains controversial. An additional problem derives from the management of hydrocephalus associated with an AC. In this work, we discuss existing procedures proposed in the current literature for their treatment. METHODS: We reviewed selected reports on intracranial ACs placing special interest in those about the association of hydrocephalus and ACs. We also briefly surveyed data of our patients with this association. RESULTS AND DISCUSSION: Hydrocephalus is often found in midline and posterior fossa ACs. Interhemispheric lesions may also evolve with ventriculomegaly, while middle fossa lesions rarely produce hydrocephalus. Patients' age, cyst location and size, and macrocephaly have all been related to the development of hydrocephalus. Some authors remark on the role played by hydrocephalus and hypothesize that some ACs would result from disturbed cerebrospinal fluid (CSF) dynamics. They also propose that ACs might represent a localized form of hydrocephalus. We also comment on hydrocephalus in relation to the diverse locations of ACs. Neuroendoscopic techniques have transformed previous ways of management as cystoperitoneal shunting and open fenestration. CONCLUSIONS: ACs may be pathogenetically related with hydrocephalus, and conversely, ACs may cause hydrocephalus. In some patients, aberrant CSF dynamics seems to play a major role in the development of both cyst and hydrocephalus. Hydrocephalus and ACs may be treated exclusively with neuroendoscopic procedures, although some patients will still require CSF shunting. The ideal option seems to consist of choosing the method that offers the highest success with a single procedure for treating the hydrocephalus and the AC simultaneously.

A Case of Miyazaki Syndrome Caused by Arachnoid Cyst-Peritoneal Shunt.

BACKGROUND: Miyazaki syndrome is overshunting-associated myelopathy, which is a rare complication of ventriculoperitoneal shunt. We present the first case of Miyazaki syndrome caused by cystoperitoneal (CP) shunt for an arachnoid cyst (AC) in this report. CASE DESCRIPTION: We report a case of a 42-year-old man with 12-year progressive spastic paraplegia, who underwent CP shunt for an AC at the age of 15 years. Although few findings suggested overshunting on symptoms and head computed tomography, contrast-enhanced magnetic resonance imaging revealed the engorgement of the cervical spinal epidural venous plexus compressing the spinal cord. Shunt valve replacement with a pressure-adjustable valve was performed. Postoperatively, the cervical cord compression by the enlarged spinal epidural venous plexus was completely improved, but, possibly due to delayed diagnosis and treatment, the patient's symptoms only partially improved. CONCLUSIONS: When patients with a history of any kind of shunt surgery develop myelopathy, Miyazaki syndrome should be suspected and, for early diagnosis, cervical and/or contrast-enhanced magnetic resonance imaging should be performed.

Multiple saccular aneurysms of the extracranial and intracranial internal carotid artery associated with convexobasia and arachnoid cyst in a 6-year-old boy: a case report.

INTRODUCTION: We report an extremely rare case of multiple saccular aneurysms of the extracranial and intracranial internal carotid artery associated with convexobasia of the left temporal region and arachnoid cyst in a 6-year-old boy. CASE REPORT: A 6-year-old male patient was admitted to the hospital with chief complaint of ptosis and engorgement of the left eyelid for 50 days. A 4 x 10-cm bony protuberance of the left temporal region with hemangiectasia was observed to beat with pulse without vascular murmur. computed tomographic angiography (CTA) showed two saccular aneurysms of the left internal carotid artery. The first one which was about 3 cm in length and 2 cm in maximum diameter was located in the middle of the petrous portion (including parts of C2 and C3). The second one which was about the same size was located from the middle of the cavernous portion to the upper end of the left internal carotid artery. Computed tomography showed an arachnoid cyst in the left temporal region and abnormality of the left temporal bone. According to the specific circumstances of this patient, we believe that surgical treatment is rather risky and the loss might outweigh the gain. Therefore, we suggested to the parents that the child should be followed up for the coming years for monitoring and possible treatment. DISCUSSION: Multiple saccular aneurysms of extracranial and intracranial internal carotid artery in children are extremely rare. According to this case, the child had congenital bony protuberance of the left temporal region with hemangiectasia, which made this case particularly specific. Besides all those discovered abnormalities, this child was a healthy boy. Due to the difficulty and high risk of possible surgical treatment, we suggested that no further treatment be performed at the moment and the child should be followed up for a long period of time. Worthy examinations such as CT, CTA, and MRI are recommended for reviewing the development of aneurysms and CNS of this boy. Further treatment might be performed in the future. We welcome all forms of discussion about this case and similar cases around the globe.

A woman with abdominal pain and headache.

We describe a case of a 27-year-old woman who came to the Emergency Department presenting severe abdominal pain. She was evaluated by a gynaecologist and submitted to pelvic ecography without finding relevant alterations. In the successive hours, she presented severe headache in occipital region and in the posterior neck, poorly responsive to analgesic drugs. A cerebral CT scan was performed and was normal, and the patient came to our Department of Neurology. The neurological examination on admission was normal. This woman had a 7 years of long-lasting history of headache, diagnosed in another hospital as migraine without aura, and she referred a recent and progressive worsening of both the frequency and the severity of the headache. In the suspect of subarachnoidal haemorrhage, a lumbar puncture was performed, and was negative for bleeding, showing only a mild increase in the number of cells (12 leucocytes). Following the lumbar puncture, the patient presented a dramatic improvement of the headache. In the successive days, she lamented sellar hypoesthesia and, when asked, she referred a recent history of urinary and faecal retention. She was, therefore, submitted to an NMR of the lumbar and sacral medulla with evidence of a voluminous extradural formation in the sacral region suggestive for extradural sacral meningeal cyst. She was finally dispatched to the Department of Neurosurgery for surgical asportation of the cyst.

Treatment option for arachnoid cysts.

OBJECTIVE: The objective of this study is to establish which treatment is the best operative intervention for arachnoid cyst. MATERIALS AND METHODS: We reviewed a series of 209 cases with arachnoid cysts focusing on the effectiveness and safety. The cysts were treated with several surgical procedures including open surgery for fenestration, endoscopic fenestration, or cystoperitoneal shunting. RESULTS: Follow-up imaging studies showed that 176 out of 209 arachnoid cysts (84.2%) reduced in size during a mean postoperative follow-up period of 6.9 years (range, 1 to 14 years). Although a cystoperitoneal shunt was the best method for early achieving an obliteration of the sylvian cyst (89%), it had the danger of shunt dependency (42%) in addition to four early complications. Although endoscopic fenestration tended to be less effective in reducing the size of a sylvian cyst, it was safe and particularly effective in completely obliterating a suprasellar, quadrigeminal, and prepontine cyst. CONCLUSION: Although the shunt for arachnoid cyst can get the more rapid good radiological outcome, the shunt-related complication and dependency would be hazardous. We suggest that endoscopic or reduced open procedures offer the advantage of avoiding a large craniotomy or the known complications of a cystoperitoneal shunt in treatment of arachnoid cysts. We could get the nearly same surgical outcome without shunt complications with endoscopic or open procedures.

Management of intracranial arachnoid cysts: institutional experience with initial 32 cases and review of the literature.

Surgical indications and modalities in treatment of intracranial arachnoid cysts still remain controversial owing to limited understanding of the pathophysiologic mechanisms and natural history of this pathology. Current literature favours endoscopic interventions for arachnoid cysts. We retrospectively reviewed 32 intracranial arachnoid cysts managed over 11-year period in our institution. Post-therapeutic results were clinically and radiologically assessed. Supratentorial location of cysts was noted in 75% cases (n=24) while 25% cysts were located infratentorially (n=8). The mean cyst size was 54mm in largest dimension (range 10-100mm; median 50mm). Hemiparesis was noted in 37% cases, raised intracranial pressure and seizures in 34% cases each, while cranial nerve dysfunction was noted in 16% cases. Seventy five percent cases were surgically managed: excision and marsupialization was done in 53% cases (n=17), stereotactic aspiration in 12.5% cases (n=4), endoscopic fenestration in 6.25% cases (n=2) and cystoperitoneal shunting initially in 1 case (3%) and after recurrence of primarily excised cysts in 2 cases. Conservative treatment with regular clinical and imaging control was done in 25% cases (n=8). The mean follow-up was 72 months (range: 12-108 months). Good outcome was noted in 72% cases, 16% cases remained unchanged while only one case with giant suprasellar cyst worsened. With excision and marsupialization, 65% of cysts reduced in size (n=11/17), 17% cysts resolved completely (n=3/17). The overall recurrence rate was 29%. Surgery excision and marsupialization of symptomatic cases provided good results.

Does subjective improvement in adults with intracranial arachnoid cysts justify surgical treatment?

OBJECTIVE Subjective improvement of patients who have undergone surgery for intracranial arachnoid cysts has justified surgical treatment. The current study aimed to evaluate the outcome of surgical treatment for arachnoid cysts using standardized interviews and assessments of neuropsychological function and balance. The relationship between arachnoid cyst location, postoperative improvement, and arachnoid cyst volume was also examined. METHODS The authors performed a prospective, population-based study. One hundred nine patients underwent neurological, neuropsychological, and physiotherapeutic examinations. The arachnoid cysts were considered symptomatic in 75 patients, 53 of whom agreed to undergo surgery. In 32 patients, results of the differential diagnosis revealed that the symptoms were due to a different underlying condition and were unrelated to an arachnoid cyst. Neuropsychological testing included target reaction time, Grooved Pegboard, Rey Auditory Verbal Learning, Rey Osterrieth complex figure, and Stroop tests. Balance tests included the extended Falls Efficacy Scale, Romberg, and sharpened Romberg with open and closed eyes. The tests were repeated 5 months postoperatively. Cyst volume was pre- and postoperatively measured using OsiriX software. RESULTS Patients who underwent surgery did not have results on balance and neuropsychological tests that were different from patients who declined or had symptoms unrelated to the arachnoid cyst. Patients with a temporal arachnoid cyst performed within the normal range on the neuropsychological tests. Seventy-seven percent of the patients who underwent surgery reported improvement, yet there were no differences in test results before and after surgery. Arachnoid cysts in the temporal region and posterior fossa did not influence the preoperative results of neuropsychological and motor tests. The arachnoid cyst volume decreased postoperatively (p < 0.0001), but there was no relationship between volume reduction and clinical improvement. CONCLUSIONS The results of this study speak against objectively verifiable improvement following surgical treatment in adults with intracranial arachnoid cysts.

Peripetrosal arachnoid cysts.

PURPOSE OF REVIEW: The present review summarizes the current theories on arachnoid cyst formation, the common presentations of cysts surrounding or eroding the temporal bone from the middle and posterior cranial fossae, the diagnostic strategies and the management considerations and options. RECENT FINDINGS: Arachnoid cysts are most common in the middle cranial fossa and rarely present in association with the petrous apex. They are frequently found incidentally on imaging studies performed in the workup for unrelated symptoms. When they do cause symptoms, these are usually nonspecific. Thus it is important to establish the relationship between the two. Peripetrosal arachnoid cysts may cause cranial nerve deficits in addition to symptoms related to intracranial hypertension. Small asymptomatic cysts are managed conservatively with serial imaging. Large symptomatic cysts are often managed surgically with shunting, open excision or open or neuroendoscopic fenestration or marsupialization. The management of large asymptomatic cysts depends on the patient and cyst characteristics. SUMMARY: Peripetrosal arachnoid cysts are often incidental findings. Careful selection of surgical candidates is of utmost importance. Multiple surgical options with similar success rates are available. The rates and profile of their complications may differ. Overall, approximately 70% of patients experience improvement in their symptoms with surgery.

A patient with myasthenia gravis and a large arachnoid cyst - report of a case.

Myasthenia gravis is a chronic autoimmune disease characterized by weakening of voluntary muscles during the day and a marked restitution of function during the night and after rest. The symptoms may worsen over days or weeks, sometimes even in a few hours, and are usually well controlled by appropriate therapy. Arachnoid cysts are congenital or acquired deformities of the arachnoid membrane and are usually too small to cause distinct clinical symptomatology. We describe a case of a 76-year-old myasthenia gravis patient with an arachnoid cyst. To the best of our knowledge this is the first reported case of these two comorbidities together.

Long-term endocrine outcome of suprasellar arachnoid cysts.

OBJECTIVE Due to their distinct location, suprasellar arachnoid cysts are known to cause a wide variety of problems, such as hydrocephalus, endocrine symptoms, and visual abnormalities. The long-term outcome of these cysts has not been elucidated. To find out the long-term outcome of suprasellar arachnoid cysts, a retrospective review of the patients was performed. The neurological and endocrine symptoms were thoroughly reviewed. METHODS Forty-five patients with suprasellar arachnoid cysts, with an average follow-up duration of 9.7 years, were enrolled in the study. A comprehensive review was performed of the results of follow-up regarding not only neurological symptoms but also endocrine status. The outcomes of 8 patients who did not undergo operations and were asymptomatic or had symptoms unrelated to the cyst were included in the series. RESULTS Surgery was most effective for the symptoms related to hydrocephalus (improvement in 32 of 32), but endocrine symptoms persisted after surgery (4 of 4) and required further medical management. More surprisingly, a fairly large number of patients (14 of 40; 1 was excluded because no pre- or postoperative endocrine evaluation was available) who had not shown endocrine symptoms at the time of the initial diagnosis and treatment later developed endocrine abnormalities such as precocious puberty and growth hormone deficiency. The patients with endocrine symptoms detected during the follow-up included those in both the operated (n = 12 of 32) and nonoperated (n = 2 of 8) groups who had been stable during follow-up since the initial diagnosis. CONCLUSIONS This study implies that patients with suprasellar arachnoid cysts can develop late endocrine problems during follow-up, even if other symptoms related to the cyst have been successfully treated. Hence, patients with these cysts need long-term follow-up for not only neurological symptoms but also endocrine abnormalities.

Invasive cerebrospinal fluid cysts and cephaloceles of the petrous apex.

OBJECTIVE: To describe the presentation, diagnostic evaluation, and surgical management of petrous apex cerebrospinal fluid (CSF) cysts and cephaloceles. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Six patients with symptomatic CSF cysts or cephaloceles. INTERVENTION(S): All patients underwent operative intervention. MAIN OUTCOME MEASURE(S): Presentation, imaging characteristics, operative findings, surgical approach, resolution of symptoms, and complications. RESULTS: Six patients presented with various neurotologic symptoms including vertigo, otalgia, diplopia, meningitis, hearing loss, and retroorbital headaches. Four lesions were centered within the anterior petrous apex and were classified as a cephalocele originating from Meckel's cave. The remaining two lesions were arachnoid cysts that involved the posterior petrous apex. Cysts and cephaloceles both demonstrated bone erosion on computed tomography and were hyperintense on T2-weighted magnetic resonance imaging and isointense or hypointense on T1-weighted magnetic resonance imaging. A variety of surgical approaches was used to treat these lesions. Preoperative symptoms were improved in five of six cases. One patient developed a postoperative CSF leak that resolved with conservative measures. CONCLUSION: Petrous apex CSF cysts and cephaloceles may present with a variety of neurotologic symptoms. Imaging often helps narrow the differential diagnosis, but these lesions can still be confused with other erosive skull base lesions such as cholesterol granulomas, epidermoids, or tumors. Optimal treatment of symptomatic posterior petrous apex CSF cysts is marsupialization via a posterior fossa approach (i.e., retrosigmoid or retrolabyrinthine). A middle fossa approach with obliteration of the anterior petrous apex may be used to treat symptomatic CSF cephaloceles arising from Meckel's cave.

Brucellar brain abscess and bilateral arachnoid cysts, unilaterally complicated by subdural haematoma.

Arachnoid cysts are benign developmental cysts that occur along the cerebrospinal axis. Brucellar abscesses in the brain are relatively uncommon, with only a few cases reported in the literature. We report here a patient with a brucellar brain abscess and bilateral arachnoid cysts (one complicated with subdural haemorrhage), who was successfully managed with craniotomy and antibiotics.

Coexistence of central precocious puberty and intraventricular arachnoid cyst: a brief literature update

Central precocious puberty (CPP) is a rare disease. The mean annual incidence in girls is 0.8-1.1/100,000 and in boys 0-0.1/100,000. Intracranial arachnoid cysts (ICACs) are usually congenital and represent 1% of intracranial masses in newborns. Intraventricular location is rare. The objective of this work is to carry out a literature updated review of the coexistence of CPP and intraventricular arachnoid cyst (IVAC). ICACs are usually asymptomatic but can present with CPP in 10-40% of patients. IVACs represents only 0.3-1.4% of ICACs, and most seemed originate from the velum interpositum cistern. CPP in girls is usually idiopathic, while in 30-70% of boys are due to an intracranial lesion. Therefore, the coexistence of PPC and IVAC is very rare in boys and exceptional in girls. The exact mechanism of a cyst´s influence on the hypothalamic-pituitary axis is not completely understood. Theories include increased ventricular volume, associated mass effect on the hypothalamus, and direct compression of portions of the hypothalamic-pituitary axis. Analysis of LH peaks after GnRH testing is the gold standard for the diagnosis of CPP. Brain MRI should be part of the assessment in boys and also in girls since clinical features, including age and sex, are not helpful in predicting those with underlying brain pathology. In cases of CPP with IVAC, surgery does not have any effect on the course of pubertal development. The indication for surgery is the onset of neurological symptoms. The medical treatment selected, safe and effective, is GnRH analog depot preparations. In conclusion, there seems to be a consensus for the diagnosis and management of the coexistence of CPP and IVAC, but the etiopathogenesis is not yet well recognized (AU)