A rare case of pediatric ptosis: Coexistence of giant intracranial arachnoid cyst and facial asymmetry.

BACKGROUND: Intracranial arachnoid cysts are cystic congenital malformations, filled with cerebrospinal fluid (CSF) originating from the arachnoid membrane. Generally, giant arachnoid cysts present with symptoms related to increased intracranial pressure, hydrocephalus or cognitive disorders, endocrinological problems, growth retardation, seizures, headache, and nonspecific symptoms such as dizziness. They can be detected by imaging when they become symptomatic or incidentally in childhood and adulthood. Our case was referred to our clinic because of ptosis and facial asymmetry found on examination. Subsequently, a intracranial giant arachnoid cyst was found incidentally on cranial computed tomography (CT). CASE: In an 18-month-old male infant admitted with ptosis, left frontal bulging and a dystopic globe with ptosis of the left upper lid were noted. The left half of the facial region and the left nostril also appeared to be asymmetrically elongated downward relative to the right. Fundus examination revealed an optic disc coloboma in the left eye. On general physical examination, he was unable to walk. A giant fronto-temporo-parietal arachnoid cyst with the cerebral parenchyma shifted 2cm to the right of the midline was observed on cranial CT. After a cysto-peritoneal shunt was performed, the physical appearance of our patient returned to normal. CONCLUSION: Ptosis cases accompanied by abnormalities such as optic disc coloboma and facial asymmetry should be evaluated for possible midline defects and intracranial pathologies prior to eyelid surgery.

Ophthalmic manifestations of ruptured arachnoid cysts in children.

BACKGROUND: Intracranial arachnoid cysts are relatively common in the pediatric population. Rarely, they rupture, leading to acute subdural fluid collections, which can cause a sudden increase in intracranial pressure. The purpose of this study was to characterize ophthalmic sequelae in a large cohort of these patients. METHODS: The medical records of all children treated for ruptured arachnoid cysts who presented at a single tertiary pediatric hospital for initial assessment between 2009 and 2021 were reviewed retrospectively. RESULTS: Of 35 children treated for ruptured arachnoid cysts during the study period, 30 received ophthalmological examination. Papilledema was found in 57% of these children, abducens palsy in 20%, and retinal hemorrhages in 10%. Of the 30 children, 22 were seen in outpatient follow-up, of whom 5 had a best-corrected visual acuity of 20/40 or worse in one or both eyes at most recent follow-up. Cranial nerve palsies resolved in all cases without strabismus surgery. CONCLUSIONS: Given high rates of papilledema, cranial nerve palsies, and vision loss, all children with ruptured arachnoid cysts should be evaluated by pediatric ophthalmologists.

Spinal arachnoid webs in adults: Clinical and imaging features in a multicenter study.

BACKGROUND AND PURPOSE: Spinal arachnoid webs (SAWs) are rare and can present with myelopathic symptoms. This study aims to add 85 more cases of SAWs to the literature so we can continue to analyze clinical and imaging trends of SAWs to better understand this entity and its natural history. METHODS: An institutional review board-approved retrospective review of SAW cases between 2016 and 2022 within a metropolitan, multihospital network was performed, searching for MR and CT reports that included "arachnoid web." Of 108 identified reports, 85 patients had imaging and/or pathologically proven arachnoid webs. Demographic and clinical data were collected. Images were analyzed for SAW level, point of maximum kyphosis, presence of cord expansion, and signal intensity. The electronic medical record (EMR) was reviewed for age, sex, symptom presentation and duration, management, and outcomes. RESULTS: Of 85 cases, the most common presenting chief complaint was back pain and weakness. All (100%) SAWs were in the dorsal thoracic subarachnoid space, with 87% (74/85) located from thoracic (T) 2 to T6. Spinal cord expansion and signal abnormality were present in 54.1% and 23.5%, respectively. Twenty-six underwent surgical resection with 20 showing improvement of at least one symptom. CONCLUSION: We present 85 additional cases of SAWs to the existing literature. Our cases all occurred in the dorsal thoracic subarachnoid space, predominantly from T2 to T6. Patients present with symptoms like other causes of cord compression, and spinal MR studies should be evaluated routinely for the findings of SAWs. Further research could focus on understanding SAW prevalence, risk factors, and pathophysiology.

Atypically located arachnoid cyst in a five-year-old cat.

Arachnoid cysts are cystic lesions that occur in spinal or intracranial locations in the leptomeningeal space. Four intracranial cases have been described in cats, three of which were diagnosed by imaging techniques alone. We now report the clinical, gross and histopathological findings in a 5-year-old, male-neutered European Shorthair cat that presented with chronic, asymmetrical encephalopathy. Using magnetic resonance imaging, a focal, fluid-filled cavity that did not show contrast enhancement was identified in the left temporal and piriform lobes. Necropsy confirmed the presence of a cystic, meningeal cavity filled with clear, serous fluid. Histologically, the cyst had an irregular, hypereosinophilic surface and single psammoma bodies with moderate perivascular oedema in the adjacent neuroparenchyma. Immunohistochemical evidence of meningeal tissue surrounding the cyst confirmed the diagnosis of an arachnoid cyst, which should be considered as a differential diagnosis of intracranial, fluid-filled cavities.

Spinal intradural arachnoid cyst associated with diastematomyelia in an adult: a case report and review of literature.

We report a case of a spinal intradural arachnoid cyst in a thirty year old female with a previously undiagnosed and untreated diastematomyelia. The cyst was located exactly between the two hemi-cords of the split cord malformation. The patient was treated successfully by laminoplasty and total removal of the cyst. Additionally, sectioning of the filium terminale was done in the same sitting by a separate incision. This case posed a diagnostic dilemma. Though the clinical findings favoured an extramedullary lesion, the possibility of cystic degeneration in the spinal cord due to the tethered cord syndrome could not be completely negated. Spinal intradural arachnoid cysts have been reported in association with various neural tube defects, mainly in the paediatric age group. However, such a lesion associated with diastematomyelia in an adult, to the best of our knowledge, has not been reported previously.

Tinnitus Triggered by a Cerebellopontine Arachnoid Cyst in an Adolescent.

BACKGROUND Tinnitus can be a symptom of a wide range of disorders. The identification and treatment of the underlying condition is essential for management of tinnitus in children. Tinnitus can occur with medical conditions other than sensorineural hearing loss. Cerebellopontine arachnoid cysts are rare and can cause tinnitus and hearing loss in adults. Tinnitus caused by an arachnoid cyst has not been reported in an adolescent. We report clinical and radiological features of a teenager with bothersome tinnitus caused by an arachnoid cyst. CASE REPORT A 14-year-old girl with unilateral tinnitus for 10 months presented to the Pediatric Otolaryngology Clinic. The loudness and duration of tinnitus had progressed gradually. Turning the head to the right induced right otalgia and tinnitus. The patient denied hearing loss, vertigo, exposure to loud noise, feeling of fullness in ear, otorrhea, facial weakness, numbness, dysphagia, changes in smell or taste, and problems with the jaw or temporomandibular joint. The focused neurological examination and head and neck examination were within normal limits. The patient had normal hearing on audiometry. T1-weighted, T2-spin-echo, T2-FLAIR, and diffusion-weighted magnetic resonance imaging sequences were obtained, revealing a right cerebellopontine angle arachnoid cyst. After arachnoid cyst marsupialization, the patient's tinnitus and otalgia resolved. CONCLUSIONS This case highlights the importance of suspecting identifiable nonauditory system disorders as causes of tinnitus in children. Thorough analysis of clinical findings and timely use of imaging is critical to prevent delay in diagnosis and treatment of children with bothersome tinnitus caused by rare medical conditions.

Classification of pediatric headache cases referred to a neurology department.

BACKGROUND: Headaches are very common in children. The patients often have mild symptoms, but on occasion may have severe, secondary headaches. The present study aimed to clarify the details of children with headaches seen at the outpatient clinic of a pediatric neurological department. METHODS: The present, retrospective observational study was conducted at a tertiary pediatric hospital in Japan and enrolled children referred to the neurology department outpatient clinic for headache between April 2018 and March 2021. RESULTS: In total, 113 cases of headache were examined; of these, 99 (87.6%) were primary headaches, one case (0.9%) was a secondary headache, and 13 (11.5%) were unclassified or unspecified. There were 46 cases (40.7%) of tension-type headache (TTH), both confirmed and suspected, 30 cases (26.5%) of migraine, and 23 cases (20.4%) of a combination of the TTH and migraine. One case of secondary headache was attributed to an infection. Arachnoid cysts were found in seven patients (7.8%). Acute drug treatments were administered to 93 patients (82.3%), with acetaminophen being the most common drug, followed by ibuprofen. Prophylactic drug treatments were administered to 39 patients (34.5%), with goreisan (a Chinese herbal medicine containing Alisma orientale, Poria cocos, Polyporus umbellatus, Atractylodes lancea, and Cinnamomum cassia) being the most common (41%). CONCLUSIONS: Few cases of secondary headache and none of emergency headache were diagnosed. The prevalence of arachnoid cysts was higher than in the general pediatric population, suggesting that arachnoid cysts might be associated with headache.

Clinical variety and prognosis of intracranial arachnoid cysts in children.

Arachnoid cysts (AC) occur in different intracranial locations. Management and prognosis depend on the clinical presentation and treatment guidelines do not exist. With this study, we want to demonstrate the clinical variety of arachnoid cysts in children and place a focus on outcome factors in operated cases. This retrospective study of a consecutive single unit series of children, who underwent AC surgery between January 2010 and September 2019, provides demographic, clinical, imaging data, and information about surgical treatment and outcome. Overall, 63 patients (71.4 male) underwent surgery. Mean age was 50 months (0-191). Mean follow-up was 40 months (0-121). Eighty-one percent of patients presented with symptoms/signs of raised ICP. Focal neurological deficits were present in 15.9%, headache in 11.1% of children. Galassi cysts represented the predominant type (30.2%), followed by suprasellar (14.3%), quadrigeminal (12.7%), retrocerebellar, CPA and midline (each 11.1%), and hemispheric cysts (7.9%). Endoscopic and microsurgical fenestrations were performed in 27% and 58.7%, stent or shunt insertion in 6.3%/57.9% of the cases. In 33.3% of the cases one and in 12.7%, a second reintervention became necessary. Reoperation rate was significantly higher in children < 1 year (p = 0.003). Cyst volume decreased in 85.7%. Seventy percent of the patients were symptom free, 5% suffered from headache, and 22% from developmental disorders. All focal neurological symptoms resolved. Complication rate and outcome are depending on age and cyst location. Recurrence and revision rates are significantly higher in young infants (p = 0.003). Midline cysts with CCA are associated with developmental disorders.

[Symptomatic arachnoid cyst in an elderly patient who developed motor aphasia].

The patient was an 83-year-old woman with a history of breast cancer, distal right radial edge bone fracture, and cervical spine symptoms who had been diagnosed with an arachnoid cyst 9 years previously. She was examined by a nearby doctor, because of an approximately 1-year history of reduced verbal output; she also begun experiencing difficulties with walking. However, she was diagnosed with aging, a history of cervical spondylosis, and the effects of past fractures.At the time of this consultation, she was conscious and lucid, with mild right-sided hemiparesis, was unable to write, and had mild motor aphasia. Head magnetic resonance imaging revealed an arachnoid cyst (longer axis: 10 cm) in the left frontal lobe that did not take up contrast media. There was also a midline shift. The cause of the right hemiparesis and motor aphasia was probably compression of the left frontal lobe by the arachnoid cyst.We performed excision of the cyst wall by craniotomy and placed a cyst-peritoneal shunt under general anesthesia. At approximately one week after surgery, the patient was able to write and her motor aphasia improved. She was discharged 20 days after the operation.It is rare for an arachnoid cyst to increase in size after childhood. In the present case, surgical treatment led to a good outcome in an elderly patient with a symptomatic arachnoid cyst. Arachnoid cysts rarely increase in size. These cysts may become symptomatic in elderly people after lying clinically dormant for a long time.

A rare association between a dermoid cyst and arachnoid cyst of the cerebellopontine angle: a case report.

Supratentorial arachnoid cysts are usually asymptomatic and may be discovered by chance at autopsy; however, infratentorial arachnoid cysts, which correspond to liquid forms enclosed by an arachnoid sheet but whose pathogenesis is unknown, might cause symptoms. They don't need to be treated if they're asymptomatic and were discovered by chance. A variety of neurological symptoms can be present depending on their localization. Intracranial dermoid cysts are uncommon tumours that develop from ectopic epithelial cells in the brain. They are benign, slow-growing, and rarely rupture. The association between the two diseases is extremely rare and when it is present may suggest the existence of a common factor. We present a unique case of a young girl who developed headache and ataxia as a result of an intracranial infratentorial dermoid cyst and an arachnoid cyst of the cerebellopontine angle. Complete removal of the dermoid cyst and drainage of the cyst leads to a full recovery. Dermoid and arachnoid cyst are two pathologies with a possible common embryogenic factor, early surgery can give a better outcome in the long term.

Arachnoid Cysts of the Internal Auditory Canal: Multiplanar Magnetic Resonance Imaging With Audio-Vestibular Correlates.

OBJECTIVES/HYPOTHESIS: To investigate prevalence, radiological characteristics, and functional correlates of arachnoid cysts (AC) of the internal auditory canal (IAC) region, including associations of nerve compression with auditory/vestibular symptoms and asymmetrical audiogram or vestibular testing. STUDY DESIGN: Retrospective study. METHODS: T2-weighted magnetic resonance imaging (MRI) studies of IACs were retrospectively analyzed from 1247 patients with asymmetric auditory or vestibular symptoms. Patients with radiological findings of AC of the IAC were identified. Multiplanar analysis was used to analyze cyst position in the IAC and assess nerve displacement or compression. Size, position, and presence of nerve compression were correlated with symptoms. RESULTS: Twenty-four patients had a cyst in the middle or fundus in the IAC. Diameter (P = .04) and position (P = .002) of AC were associated with symptoms. Sagittal analyses identified displacement versus compression (P = .003) more reliably than axial imaging. Symptom laterality was associated with the site of radiological abnormality. Vestibular nerve compression was associated with vertigo (P = .0001), and cochlear nerve compression was associated with auditory symptoms (P < .0001). CONCLUSIONS: In a retrospective series of patients undergoing MRI of IACs for asymmetric auditory or vestibular impairment, clinical symptom profile correlated with blinded assessment of IAC lesions. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2323-2331, 2021.

Arachnoid Web Fenestration: Diagnostic and Surgical Nuances.

Arachnoid web (AW) is a rare phenomenon that has only been described in small case reports and case series,1 most commonly presenting with upper motor neuron signs and subtle radiographic findings, such as the classically described "scalpel sign."2 In this report, we demonstrate the use of imaging and operative techniques that have not been previously shown in the literature as a video for AW. These include high-definition magnetic resonance imaging (MRI) sequences for preoperative diagnosis, use of intraoperative ultrasonography for identification of adhesions, and operative technique for AW fenestration (Video 1). The patient consented to this manuscript. A 64-year-old female patient developed progressive difficulty with balance and ambulation that particularly worsened over the last 4 months associated with tingling and numbness in the bilateral lower extremities. Physical examination revealed spastic gait and upper motor neuron signs in the lower extremities along with left foot drop. MRI revealed a chronic noncontrast-enhancing intramedullary lesion, along with a spinal cord indentation at the level T6 with an associated fiber between the cord and the posterior dura. Surgical intervention was performed with the use of intraoperative fluoroscopy and ultrasound for real-time identification of the surgical site and the AW. Under the microscope, the dura was incised while preserving the arachnoid. The AW was carefully dissected, leaving the portions that were tethered onto the cord. Two weeks postoperatively, the patient's gait was markedly improved, with resolved neurologic function in the lower extremities. Follow-up MRI at 3 months demonstrated resolved medullary syrinx and normalization of the spinal cord contour.

Neuro-Ophthalmologic Monitoring in the Management of Increased Intracranial Pressure From Leaking Arachnoid Cysts.

BACKGROUND: Intracranial arachnoid cysts are common incidental imaging findings. They may rarely rupture, leading to the development of subdural hygromas and high intracranial pressure (ICP). Neurosurgical intervention has been advocated in the past, but recent evidence indicates that most cases resolve spontaneously. The role of neuro-ophthalmologic monitoring in identifying the few cases that have persisting vision-threatening papilledema that justifies intervention has not been emphasized. METHODS: Retrospective review of 4 cases of leaking arachnoid cysts drawn from the files of the University of Michigan Medical Center (Michigan Medicine) between 2007 and 2018. RESULTS: In 1 case, surgery was avoidable as papilledema resolved over time despite lingering imaging features of mass effect. In 3 cases, papilledema persisted with the threat of permanent vision loss, prompting neurosurgical intervention. In one of those cases, the fluid collection was thinly but extensively spread across both hemispheres without brain shift; yet, papilledema was pronounced. Emergent evacuation led to rapid resolution of papilledema and encephalopathy, but with residual optic nerve damage. CONCLUSIONS: Because constitutional symptoms and even imaging are not always reliable indicators of high ICP in leaking arachnoid cysts, neuro-ophthalmologic monitoring of papilledema is valuable in identifying the cases when neurosurgical intervention is necessary.

Suprasellar arachnoid cysts: systematic analysis of 247 cases with long-term follow-up.

At present, limited data exists to discuss the characteristics of suprasellar arachnoid cysts (SACs). The aim of this study is to elucidate the relationship between characteristics of cysts and outcomes, quantitatively analyze improvement in hydrocephalus, and evaluate the risk factors for the prognosis of SACs treated by endoscope. From June 2002 to 2017 December, 247 cases of SACs treated by endoscope in Beijing Tiantan Hospital were included in this study. The severity of hydrocephalus was evaluated by Evans' index (EI). The results showed that the slit-valve and the transparent/thin membrane were noted in 86.2% and 76.5% of overall patients, respectively, and the distribution differences among age-groups were statistically significant (p < 0.01). After a mean follow-up duration of 73.1 months, 18 patients underwent a reoperation. Ventriculocystostomy (VC) (hazard ratio (HR), 3.37; 95% confidence interval (CI), 1.2-9.47; p = 0.024) and history of treatment (HR, 3.98; 95% CI, 1.31-12.31; p = 0.015) were adverse factors for reoperation rate. MRI at 1-year follow-up revealed mean decreases of 78.4% and 9.13% in cyst size and EI. No paraventricular edema was an adverse factor associated with the improvement in hydrocephalus (HR, 11.22; 95% CI, 5.43-23.18; p < 0.01). These results indicated that ventriculocystocisternostomy (VCC) and no history of treatment is favorable factors for prognosis of SACs treated by endoscope. If feasible, VCC is the optimal choice for SACs. Slit-valve phenomenon and transparent/thin membrane are correlated with age but did not influence the outcomes of endoscopic fenestration. The mechanism for the expansion of cysts may be different between child and adult patients. Paraventricular edema is a favorable factor for the improvement in hydrocephalus after endoscopic surgery.

Chudley-McCullough Syndrome: A Recognizable Clinical Entity Characterized by Deafness and Typical Brain Malformations.

Chudley-McCullough syndrome, a rare autosomal recessive disorder due to pathogenic variants in the GPSM2 (G-protein signaling modulator 2) gene, is characterized by early-onset sensorineural deafness and a typical combination of brain malformations, including ventriculomegaly, (partial) agenesis of the corpus callosum, cerebellar dysplasia, arachnoid cysts, frontal subcortical heterotopia, and midline polymicrogyria. When hearing loss is managed early, most patients have minor or no impairment of motor and cognitive development, despite the presence of brain malformations. We report 2 cases of Chudley-McCullough syndrome, one presenting with congenital deafness and normal development except for speech delay and one presenting prenatally with ventriculomegaly and an atypical postnatal course characterized by epileptic spasms, deafness, and moderate intellectual disability. These highlight the challenges faced by clinicians when predicting prognosis based on pre- or postnatal imaging of brain malformations. We have also reviewed the phenotype and genotype of previous published cases to better understand Chudley-McCullough syndrome.

Compresión medular secundaria a quiste aracnoideo intradural de etiología traumática / Spinal cord compression secondary to traumatic intradural arachnoid cyst

Los quistes aracnoideos espinales son entidades raras y casi siempre tienen una etiología congénita o espontánea. Los casos secundarios son muy poco frecuentes y muchas veces constituyen una causa de deterioro neurológico tras varios meses o años de un traumatismo espinal. Presentamos el caso de un varón diagnosticado de quiste aracnoideo espinal de etiología traumática, agrupamos los casos descritos hasta el momento en la literatura y revisamos las distintas opciones quirúrgicas relacionándolas con las teorías fisiopatológicas que tratan de explicar la génesis de estas lesiones

Spinal arachnoid cysts are rare entities, most of which have a congenital or spontaneous etiology. Secondary cases are infrequent and can constitute a cause of neurological deterioration several months or years after spinal trauma. We present the case of a male patient with traumatic spinal arachnoid cyst, together with a review of the cases published to date. Finally, we explain the different surgical options for this pathology relating them to the physiopathological theories that try to explain the origin of these lesions

A Case of Arachnoid Cyst Presenting with Cognitive Impairment and Hypomania Symptoms. / Bilissel Bozukluk ve Hipomani Belirtileriyle Basvuran Bir Araknoid Kist Olgusu.

Arachnoid cysts are benign congenital malformations of the arachnoid which account for approximately 1.4% of the intracranial lesions. Although it is usually asymptomatic, it may be accompanied by headache, hydrocephalus and seizure. Psychiatric disorders associated with arachnoid cysts are rare. In this article, we present a giant arachnoid cyst with hypomania symptoms and marked cognitive impairment. A 44-year-old female patient was admitted to our outpatient clinic with a 4-year history of headache, nervousness and attention problems. Magnetic resonance imaging revealed a giant arachnoid cyst with a size of 5.5x10.5x12.5 cm was found in the left hemisphere of the patient. Considering the patient's irritability, increase in the amount of speech, flight of ideas, sleep disturbance and attention disorders, the diagnosis of hypomania was made. The neuropsychological tests showed that the speed of information processing, mental flexibility and attention functions decreased, and executive functions were impaired. The patient was consulted to the neurosurgery department. But no surgical treatment was offered. Drug therapy for hypomanic symptoms and cognitive impairment was planned, but could not be started since the patient did not attend to the follow-up exams. Albeit the lack of followup constitutes a limitation for our report, we believe that the size of the cyst, significant impairment of cognitive functions and the presence of hypomania symptoms might contribute significantly to the literature. Other cases with arachnoid cyst displaying cognitive impairment were summarized in our article.