Congenital Arachnoid Cyst Research Articles

Giant interhemispheric arachnoid cyst – A rare case report

IHACs (interhemispheric arachnoid cysts) are a rare kind of congenital arachnoid cyst that account for5% of all occurrences. The best surgical treatment for symptomatic IHAC is still debatable, and there are no clear standards. At the time of presentation, the median age was 13 months. Macrocrania with rapidly increasing head size, seizures, infantile spasms, and developmental delay were the most common clinical manifestations.Endoscopic cyst fenestration and shunt surgery are both safe treatments for IHAC. We present an 11-month-old male infant born to non consanguineous parents who has had a steady rise in head size since 6 months of age and delayed mile stones.A magnetic resonance image of the brain revealed a massive interhemispheric arachnoid cyst, as well as corpus callosum agenesis and hypoplasia in both frontal lobes. Baby underwent endoscopic cyst fenestration and cystoperitoneal shunt.

Intradural Spinal Arachnoid Cysts in Children: A Collective Experience of 2 Centers

Pediatric intradural spinal arachnoid cysts are rare and can cause spinal cord or nerve root compression. Spinal arachnoid cysts can cause pain, motor/sensory neurological impairments, gait disturbances, spasticity, and bladder problems depending on their location. This study discusses the clinical aspects, management, surgical nuances, and postoperative clinical outcomes of symptomatic congenital intradural spinal arachnoid cysts, which are rarely seen in the pediatric population. Our study involves a retrospective evaluation of 8 pediatric patients who underwent surgery for spinal intradural arachnoid cysts at the Department of Neurosurgery, Kocaeli University School of Medicine, and the Department of Neurosurgery, Selros University School of Medicine. The demographic data of all patients, preoperative/postoperative clinical findings, surgical procedures, surgical complications, and radiological imaging were evaluated. The average age of the patients was 8.7 uated. surgicrange:1-17).The female to male ratio was 4:4. The most common complaint was weakness in the lower extremities (87.5%). Urinary problems (50%) and sensory disturbances (50%) were less frequently observed. All patients had dorsal localization of the cysts. Cyst excision was performed in 7 out of 8 patients, and cyst fenestration was performed in 1 patient. Postoperatively, 7 patients showed complete symptom resolution, while one patient had partial improvement. The success of surgical treatment depends on cyst location, neural tissue compression, and duration of symptoms. Complete removal or fenestration is determined by cyst location and accessibility. Intracystic shunts may be used in certain cases. Timely diagnosis and surgical intervention are crucial for improving neurological function in these rare cases.

Surgical outcomes of congenital arachnoid cysts and their association with neurodevelopmental disorders.

To evaluate the surgical outcomes of arachnoid cyst decompression in patients less than 16 years of age with an associated neurodevelopmental disorder (NDD). A retrospective analysis of surgically treated congenital arachnoid cyst within South Wales between 2010-2019. Surgical outcomes are measured according to clinical (COG; clinical outcome score) and radiological (NOG; neuroimaging outcome score) outcomes and the complication profiles are described. The association between arachnoid cysts and NDD both in South Wales and the literature review cohort is also discussed. The prevalence of congenital arachnoid cysts in South Wales is 0.02% and their association with neurodevelopmental disorders among patients with a congenital arachnoid cyst is high (estimated prevalence 19%). 18 (51%) in total were surgically treated in our unit; 7 with an associated NDD. All surgical modalities showed improved clinical and radiological outcomes in those with an associated neurodevelopmental disorder. 4 (15%) patients who had endoscopic treatment, and 2 (7%) of those who underwent craniotomy had complications. No complications following cystoperitoneal shunt were seen. 4 (21%) surgically treated patients were ultimately shunted following initial treatment by endoscopy or craniotomy. In a cohort of patients diagnosed with a congenital arachnoid cyst in association with a neurodevelopmental disorder, all surgical treatments show improved clinical and radiological outcomes. Cystoperitoneal shunting appears to have a favourable complication profile.

Endoscopic Treatment of Middle Cranial Fossa Arachnoid Cysts in Children: Surgical Results of 65 Cases

Congenital arachnoid cysts in the middle fossa accounts for 50% of all intracranial arachnoid cysts. Several management options have been described; however, no single technique has been universally adopted. We describe a series of pediatric patients with middle cranial fossa arachnoid cyst that were treated through an innovative minimally invasive endoscopic technique, along with their clinical aspects and surgical outcomes. Data from 65 patients operated between 2012 and 2018 were retrospectively analyzed. Follow-up ranged from 12 to 96 months. Clinical presentation and surgical outcomes were collected. Endoscopic cystocisternostomy was performed in all patients using a mini endoscope. There were 41 male and 24 female patients, with a mean age of 5.3 years at the time of treatment. Except for 4 patients, all presented with 1 or more symptoms, and the most frequent were intracranial hypertension signs. All patients were treated with the same surgical technique, with an efficacy of 81.5%. Using a mini endoscope allow us to perform multiple fenestrations along the arachnoid and deeply inspect the basal cisterns, achieving a much wider communication between the cyst and subarachnoid space with a high success rate and minimal invasiveness.

Chronic Subdural Hematoma Associated with Congenital Arachnoid Cysts: Management Dilemmas

Chronic subdural hematoma (CSDH) is one of the commonest diseases encountered by a neurosurgeon in daily practice. It is however rarely seen in young patients. Congenital arachnoid cysts have been implicated in both traumatic and spontaneous CSDH in young individuals. Optimum treatment strategies to address the CSDH and arachnoid cyst are not very well described. We report a young gentleman who was treated for a CSDH with arachnoid cysts, two months after a mild head injury. The patient was operated with a simple burr hole drainage of hematoma with a drain. He was discharged with no further need to address the arachnoid cyst. CSDH associated with arachnoid cysts can be treated with simple burr hole drainage. Craniotomy, fenestration and cerebrospinal fluid (CSF) diversion should be reserved only as secondary procedures.

Magnetic resonance imaging pattern of congenital brain anomalies in the neurosurgery department of a teaching hospital in Nigeria: An initial experience

Context: Congenital brain anomalies are of diverse types however few are seen in clinical practice. They are associated with high morbidity and mortality; prompt diagnosis and management aid in mitigating some of their adverse effects. Magnetic resonance imaging (MRI) is ideal for imaging these conditions, especially in pediatric cases due to its lack of ionizing radiation and excellent soft-tissue delineation. Not much literature exists for Nigeria due to challenges with statistics and record keeping. Aim: The aim of this study is to document the common congenital brain anomalies encountered using MRI in our environment. Settings and Design: A 5-year retrospective study of all pediatric brain MRI conducted and reviewed in Lagos University Teaching Hospital. 73 pediatric brain MRI scans were reviewed. Materials and Methods: Morphologic evaluation of all pediatric brain MRI conducted in the last 5 years (March 2012–February 2016) was reviewed, retrospectively, by three independent radiologists. Clinical presentations were also documented. Statistical Analysis Used: Descriptive statistics was done using SPSS: PASW Statistics for Windows, Version 18.0 Results: Seventy-three pediatric brain MRI scans were conducted in the last 5 years with congenital brain anomalies seen in 19 (26.0%) of the cases. Their ages ranged from 3 months to 17 years with a mean age of 6.7 ± 6.1 years. There were 9 (47.4%) males and 10 (52.6%) females. The common anomalies are congenital hydrocephalus 7 (35%), of which aqueductal stenosis was 6 (32%), arteriovenous malformations 3 (16%), cerebral atrophy 3 (16%), and arachnoid cysts 2 (11%). Predominant clinical features were delayed developmental milestones, macrocephaly, seizures, headaches, and vomiting. Conclusion: The common congenital brain anomalies in our environment are congenital hydrocephalus, aqueductal stenosis, arteriovenous malformations, cerebral atrophy and arachnoid cysts. MRI is useful in evaluating these anomalies; early diagnosis and prompt intervention can be offered to mitigate adverse effects.

P24.08: Prenatal sonographic diagnosis of congenital arachnoid cyst: clinical outcomes in a tertiary care centre

To investigate the prenatal ultrasonographic(US) findings which are associated to abnormality and neonatal outcome of 21 fetuses with congenital arachnoid cysts(CAC) diagnosed antenatally. A retrospective study of all cases which were prenatally and postnatally diagnosed as CACs was performed in patients referred to the Severance Hospital from November 2005 to December 2016. 17 CAC fetuses were identified by US at the median gestational age of 30 weeks (range 24-38). Of the 17 probable CAC, 11 (64.7%) cases were diagnosed with CAC and 6 cases were confirmed with other diagnosis based on MRI finding. 4 cases were misdiagnosed as other different disease based on prenatal US. 3 (75%) of the 4 misdiagnosed cases were confirmed postnatally as posterior fossa CAC and 1 case was confirmed as intraventricular arachnoid cyst. In terms of location, 7 (46.7%) had CAC in posterior fossa. Of the 15 confirmed CAC, only one case presented agenesis of the corpus callosum and two cases presented secundum atrial septal defect and ventricular septal defect. None of the 15 cases was confirmed as chromosomal abnormality. For 6 cases, conservative management was done, while other 9 babies, fenestration of CAC was performed. In 4 of the 9 cases, surgery was performed in the second week of life (day 6 to 12). Because of enlarged CAC with aggravated symptoms, 4 needed subsequent cystoperitoneal shunt. Depending on the location of CAC, it can be diagnosed incorrectly as other disease with similar US finding. According to our study and previous literature, the prognosis of CAC depends on the presence of the corpus callosum or other congenital malformations, and the progression of ventriculomegaly. Therefore, if CAC is suspected, it is necessary to conduct the detailed US screening for congenital malformation. Also, there is a need for proper consultation for parents with regard to the possibility of surgery after delivery.

Pediatric interhemispheric arachnoid cyst: An institutional experience.

Background:Interhemispheric arachnoid cysts (IHACs) are a rare type of congenital arachnoid cyst accounting for <5% of all cases. The optimum surgical management of symptomatic IHAC is still controversial, and there are no clear guidelines.Materials and Methods:Retrospective analysis of six pediatric patients of IHAC operated from 2012 to 2015 at our institute. There was definitive sex predisposition with all patients being males. Endoscopic cystoventriculostomy, cystocisternostomy, and cystoperitoneal shunt surgeries were performed in three patients each.Results:The median age at presentation was 13 months. The most common clinical presentations were macrocrania with rapidly increasing head size, seizures, infantile spasms, and developmental delay. The mean duration of follow-up was 24.16 months (range: 3–36 months). Cyst size decreased in all the patients on follow-up imaging. Head size stabilized in all the patients. None of the patients required a second surgical procedure till the last follow-up.Conclusions:Both endoscopic cyst fenestration and shunt surgery are safe and effective in management of IHAC.

Prenatal Diagnosis of Ambient Cistern Cyst

Ambient cistern is an expansion of the subarachnoid space of the brain located between the posterior end of the corpus callosum, thalamus and the superior surface of the cerebellum. It represents the extension of the superior cistern to the midbrain, connecting it to the interpeduncular cistern. Ambient cistern cyst accounts for 20 % of all congenital arachnoid cysts (Sinha S, Brown JI. Childs Nerv Syst 2004; 20: 100 – 103). It is rarely detected prenatally, mostly during the third trimester of gestation.

Tension Arachnoid Cyst Causing Uncal Herniation in a 60 Year Old: A Rare Presentation

Arachnoid cysts are congenital benign cysts accounting for approximately 1% of all intracranial mass lesions. Uncal herniation due to arachnoid cyst is a rare mode of presentation. It is hypothesized that only tension arachnoid cyst could cause the life-threatening condition that results from a progressive deterioration and worsening of a simple and usually congenital arachnoid cyst, associated with the formation of a "ball valve" at the point of an opening on the cyst wall. To-date only one case of an arachnoid cyst causing Uncal herniation has been reported to the best of our knowledge. We present a rare case of uncal herniation in a 60-year-old lady caused by a giant left temporal arachnoid cyst. She presented to us in emergency room after experiencing headaches since last one week followed by vomiting, seizures, and altered state of consciousness. She was operated immediately and marsupialization of the arachnoid cyst was performed. She showed good recovery. Although arachnoid cyst is a benign slowly growing pathology, it can lead to Uncal herniation as a "tension" arachnoid cyst, possible due to "ball-valve" mechanism. Elective treatment should be considered to prevent progressive significant enlargement of cyst.

Congenital intradiploic arachnoid cyst presenting as painless proptosis

A 12-year-old girl presented with several years of progressive painless proptosis and orbital fullness. On imaging, she was found to have marked expansion and remodeling centered in the right greater wing of the sphenoid bone with an adjacent middle cranial fossa arachnoid cyst. A clinical diagnosis of intradiploic arachnoid cyst was made. This entity was benign, and other ophthalmic and neurologic signs or symptoms were absent. Our patient was observed without surgical intervention. To our knowledge, this is the first such case reported in a child. Although this cyst has been described in the occipital and frontal bones, this is the first description of occurrence in the sphenoid bone.

Two novel mutations in theSpastingene of Chinese patients with hereditary spastic paraplegia

Hereditary spastic paraplegia (HSP) is a clinically and genetically heterogeneous group of neurodegenerative diseases. Mutations in the spastin (SPG4) gene are responsible for approximately 40% of autosomal dominant HSP (AD-HSP) and 6.5-18% of sporadic cases. Spastin mutations were screened in 11 AD-HSP families and 11 sporadic cases by direct sequencing and MLPA assay. Novel mutations were detected in 100 healthy controls by PCR-RFLP. We identified seven different spastin mutations in five probands and one sporadic patient. Two of seven mutations were novel. The c.458delT was a pathogenic mutation, but the effect of c.1724 G>T remained unknown. This study allowed us to estimate the frequency of the SPG4 mutations in Chinese at 45% (5/11) in families with AD-HSP and 9% (1/11) in sporadic cases. In addition, our data showed p.T614I was not associated with congenital arachnoid cysts.

Congenital spinal intradural arachnoid cyst associated with intrathoracic meningocele in a child

Congenital spinal intradural arachnoid cyst associated with intrathoracic meningocele is very rare. We report a case in a 9-year-old Chinese boy who presented with a two-week history of progressive paraparesis and gait ataxia. Magnetic resonance imaging revealed that a dorsal intradural extramedullary cystic lesion extended from T1 to T5 and compressed the spinal cord. A left lateral intrathoracic meningocele pouch was found incidentally at the level of T1. The arachnoid cyst as well as meningocele was removed and the spinal cord compression was relieved. Arachnoid cyst was confirmed by histological examination. The patient recovered well postoperatively. This is the second report of such a case in the world according to the available literature. The take-home message for our case is that the surgical approach should be individualized, depending on the size and location.

Quadrigeminal Arachnoid Cysts in a Kitten and a Dog

Two quadrigeminal arachnoid cysts with different pathogenesis are described in 2 different species. A 10-week-old male Persian kitten with a progressively decreasing level of consciousness died spontaneously. At necropsy, mild internal hydrocephalus, caudal cerebellar coning, and cerebellar herniation through the foramen magnum were associated with a congenital quadrigeminal arachnoid cyst compressing the rostral cerebellum and shifting the entire cerebellum caudally. In contrast, a possibly acquired quadrigeminal cyst was observed in a 2-year-old male neutered Yorkshire Terrier in association with necrotizing encephalitis. Quadrigeminal arachnoid cysts have been rarely reported in dogs and humans.

Symptomatic prepontine cistern flattening after an arachnoid cyst shunting:: a report of 2 cases

Background Controversy still exists about the best management modality for congenital arachnoid cyst. Shunting procedures have been one of the treatment options offered to patients with this condition. Although arachnoid cysts shunting have been associated with a variety of clinical conditions, its relationship with flattened prepontine cistern has not been described. Case Description We describe two children with congenital arachnoid cyst managed with cystoperitoneal shunt, both of whom were later discovered to have developed flattened prepontine cistern and imaging findings of overcrowding of the posterior cranial fossa. Conclusion These cases demonstrate that flattening of the prepontine cisterns and acquired posterior cranial fossa overcrowding may be a potential fall out in patients with cystoperitoneal shunt due to an arachnoid cyst.

Adult Traumatic Leptomeningeal Cyst -Case Report-

A 60-year-old man presented with a traumatic leptomeningeal cyst manifesting as local tenderness in the right parietal region and local headache 2 years after head injury. Magnetic resonance imaging showed a small arachnoid cyst under bone defect. Dural and bone plasty were performed. Intraoperative examination found small and round defects of the dura and bone. Progressive headache was relieved after the surgery. This rare case of adult posttraumatic leptomeningeal cyst occurred within an unusually short period after trauma, and was associated with a small and round bone defect and small dural defect usually characteristic of congenital arachnoid cyst.

A new case of VACTERL association with congenital arachnoid cyst

Neonatal Intensive Care Unit, Departman of Pediatrics, Republic of Turkey Ministry of Health Ankara Diskapi Children’s and Research Hospital, Diskapi and Department of Pediatrics, Division of Clinical Genetics, Hacettepe University Faculty of Medicine, Sihhiye, Ankara, Turkey Correspondence to Dr Sevim Balci, Kibris sok. 17/8 Asaci Ayranci 06690 Ankara, Turkey E-mail: s.balci@hacettepe.edu.tr; eseresin@superonline.com

Asymptomatic Huge Congenital Arachnoid Cyst Successfully Treated by Endoscopic Surgery -Case Report-

A female neonate was the first child of a 30-year-old mother, with unremarkable medical history. Prenatal ultrasonography performed at 36 weeks of gestation suggested intracranial mass lesion. The baby was delivered by cesarean section at 41 weeks of gestation because of bradycardia and asphyxia. The birth weight, height, and head circumference were within the normal ranges with soft fontanels. Congenital anomaly was not observed with normal neurological findings. She was referred to our department at age 5 months. Physical examination revealed normal developmental milestones and intact endocrinological function without macrocephaly. Cerebral magnetic resonance (MR) imaging revealed a unilocular huge cyst appearing as homogeneously hypointense on T(1)- and hyperintense on T(2)-weighted images, and extending into the basal, suprasellar, ambient, quadrigeminal, interpeduncular, prepontine, right cerebellopontine angle, and premedullary cisterns. The pituitary stalk was markedly stretched and displaced ventrally, and the brainstem was displaced dorsally by the cyst. No other brain anomalies, dysgenesis of the corpus callosum, or ventriculomegaly were recognized. Neuroendoscopic cystocisternostomy was performed to form a communication between the cyst cavity and premedullary cistern. Pressurized watery fluid was released on puncturing the cyst wall which consisted of transparent membrane. Surveillance MR imaging at 2 and 9 months after the surgery revealed remarkable regression without regrowth of the cyst. She remained in good condition and showed normal development during the follow up for 1 year 9 months. Less invasive prophylactic surgery using the neuroendoscope may be beneficial for carefully selected cases of asymptomatic neonatal arachnoid cysts.

Prenatal Diagnosis of Arachnoid Cysts

Arachnoid cysts are a rare central nervous system malformation, representing only 1% of all intracranial masses in newborns. Primary (congenital) arachnoid cysts are benign accumulation of clear fluid between the dura and the brain substance throughout the cerebrospinal axis in relation to the arachnoid membrane and do not communicate with the subarachnoid space. Secondary (acquired) arachnoid cysts result from hemorrhage, trauma, and infection and usually communicate with the subarachnoid space. The common locations of arachnoid cysts are the surface of the brain at the level of main brain fissures, such as sylvian, rolandic and interhemispheric fissures, sella turcica, the anterior cranial fossa, and the middle cranial fossa. Arachnoid cysts may be associated with ventriculomegaly and dysgenesis of corpus callosum. Prenatal ultrasound and magnetic resonance imaging have led to the increased diagnosis of fetal arachnoid cysts. This article provides a thorough review of fetal arachnoid cysts, including prenatal diagnosis, differential diagnosis and associated chromosomal abnormalities, as well as comprehensive illustrations of perinatal imaging findings of fetal arachnoid cysts. Prenatal diagnosis of intracranial hypoechoic lesions should include a differential diagnosis of arachnoid cysts and prompt genetic investigations.

A new SPG4 mutation in a variant form of spastic paraplegia with congenital arachnoid cysts.

The clinical and genetic findings are described for 16 patients from a large Italian family with a variant form of hereditary spastic paraplegia and congenital arachnoid cysts inherited as an autosomal dominant trait. A molecular study has revealed a novel missense mutation, T614I, in exon 17 of SPG4, which may play a role in both focal cortical dysgenesis and neurodegeneration of the motor neurons in the corticospinal tract.