Evaluation of surgical treatment strategies and outcome for cerebral arachnoid cysts in children and adults.

OBJECTIVE: The best treatment strategies for cerebral arachnoid cysts (CAC) are still up for debate. In this study, we present CAC management, outcome data, and risk factors for recurrence after surgical treatment, focusing on microscopic/endoscopic approaches as compared to minimally invasive stereotactic procedures in children and adults. METHODS: In our single-institution retrospective database, we identified all patients treated surgically for newly diagnosed CAC between 2000 and 2022. Microscopic/endoscopic surgery (ME) aimed for safe cyst wall fenestration. Stereotactic implantation of an internal shunt catheter (STX) to drain CAC into the ventricles and/or cisterns was used as an alternative procedure in patients aged ≥ 3 years. Treatment decisions in favor of ME vs. STX were made by interdisciplinary consensus. The primary study endpoint was time to CAC recurrence (TTR). Secondary endpoints were outcome metrics including clinical symptoms and MR-morphological analyses. Data analysis included subdivision of the total cohort into three distinct age groups (AG1, < 6 years; AG2, 6-18 years; AG3, ≥ 18 years). RESULTS: Sixty-two patients (median age 26.5 years, range 0-82 years) were analyzed. AG1 included 15, AG2 10, and AG3 37 patients, respectively. The main presenting symptoms were headache and vertigo. In AG1 hygromas, an increase in head circumference and thinning of cranial calvaria were most frequent. Thirty-five patients underwent ME and 27 STX, respectively; frequency did not differ between AGs. There were two (22.2%) periprocedural venous complications in infants (4- and 10-month-old) during an attempt at prepontine fenestration of a complex CAC, one with fatal outcome in a 10-month-old boy. Other complications included postoperative bleeding (2, 22.2%), CSF leaks (4, 44.4%), and meningitis (1, 11.1%). Overall, clinical improvement and significant volume reduction (p = 0.008) were seen in all other patients; this did not differ between AGs. Median follow-up for all patients was 25.4 months (range, 3.1-87.1 months). Recurrent cysts were seen in 16.1%, independent of surgical procedure used (p = 0.7). In cases of recurrence, TTR was 7.9 ± 12.7 months. Preoperative ventricular expansion (p = 0.03), paresis (p = 0.008), and age under 6 years (p = 0.03) were significant risk factors for CAC recurrence in multivariate analysis. CONCLUSIONS: In patients suffering from CAC, both ME and STX can improve clinical symptoms at low procedural risk, with equal extent of CAC volume reduction. However, in infants and young children, CAC are more often associated with severe clinical symptoms, stereotactic procedures have limited use, and microsurgery in the posterior fossa may bear the risk of severe venous bleeding.

Multiple spinal extradural arachnoid cysts presenting as compressive myelopathy in a teenager: case report and literature review with special emphasis on postoperative spine deformity in the current minimally invasive era.

INTRODUCTION: Multiple synchronous spinal extradural arachnoid cysts are extremely rare in children and can lead to cord compression and disability. Multiple spinal extradural arachnoid cysts (SEDACs) in children with immature and growing spine need to be studied separately from multiple SEDACs in adults with mature and fully grown spine because of multiple surgical limitations in children (blood loss, surgery duration, long-term spinal stability after long segment exposure, etc.). MATERIAL AND METHODS: We planned a non-systematic literature review of similar cases described in literature to analyse the pattern of presentation, management, and outcome of this surgically curable disease. RESULTS: A total of 28 cases of paediatric multiple SEDACs in the age range of 5 months to 17 years and mean age of 11.54 years were analysed. Exposure was achieved by laminectomy in 9, laminoplastic laminotomy in 13, laminectomy in first surgery followed by laminoplasty in second surgery (re-exploration) in 1, hemilaminectomy in 2, and technique "not reported" in 3 children. Complete/near-complete recovery was seen in 27 (96.42%) children. Postoperative spine deformity was reported in 7 (25%) children and was progressive in 2 children during serial follow-up. CONCLUSIONS: Symptomatic multiple synchronous SEDACs in children is a rare surgically curable condition. Sincere attempt to find out a dural defect in all cysts is a very important step in multiple SEDAC surgery. Total cyst wall excision with closure of all dural defects is the gold standard treatment for symptomatic cases. Laminoplasty is preferred for excision of multiple SEDACs in children to prevent postoperative spine deformity during long-term follow-up. Long serial follow-up for postoperative spine deformity is necessary.

Subdural hemorrhage in middle cranial fossa arachnoid cysts: a report of two cases at two ends of the spectrum.

Arachnoid cysts are usually asymptomatic, benign lesions commonly occurring in the middle cranial fossa. However, the cysts may rupture in rare cases causing intracystic or subdural hemorrhages with significant mass effect. We report two cases of middle cranial fossa arachnoid cyst with subdural hemorrhage with very different clinical course. The first case presented with significant mass effect with cerebral herniation and had significant neurological morbidity post-surgery. The second case had minimal symptoms and was managed conservatively with offer of elective surgery. The report underscores the importance of prompt diagnosis and appropriate surgical intervention in managing arachnoid cysts with hemorrhage, highlighting the potential for diverse clinical presentations and outcomes.

Long-term outcomes following surgical treatment of spinal arachnoid cysts: a population-based consecutive cohort study.

BACKGROUND CONTEXT: Spinal arachnoid cysts (SACs) are rare, cerebrospinal fluid-filled sacs lined by an arachnoid membrane in the spinal canal. Symptoms can develop due to pressure on the spinal cord or adjacent spinal nerves by the cyst itself or by interrupted flow of cerebrospinal fluid. If noninvasive management fails or neurological deterioration occurs, surgical treatment is recommended. However, data is lacking on long-term outcomes after surgery. PURPOSE: To determine long-term outcomes in patients surgically treated for SACs. STUDY DESIGN: Population-based cohort-study. PATIENT SAMPLE: All consecutive patients treated for either intra- or extradural SACs with surgery between 2005 and 2020 at the author's institution were included. OUTCOME MEASURES: American Spinal Injury Association Impairment Scale (AIS) and modified Japanese Orthopedic Association score (mJOA). METHODS: Data was primarily extracted from electronic patient medical notes. Telephone interviews were performed to assess long-term postoperative outcomes. All analyses were conducted using the statistical software program R version 4.0.5. Statistical significance was set at p<.05. RESULTS: Thirty-four patients were included. Cyst excision was performed in 11 (32%) cases, and fenestration in the remaining 23 (68%). The median follow-up time was 8.0 years. Surgery resulted in a significant long-term improvement in both AIS (p=.012) and mJOA (p=.005). Sensory deficit was the symptom that most often improved (81%), followed by pain (74%) and motor function (64%). AIS deteriorated in two patients, of which one case was attributed to a surgical complication. Local cyst recurrence requiring reoperation was seen in 4 (12%) cases, all of them following cyst fenestration. One patient (3%) required reoperation for progression of the cyst progression at a different level. CONCLUSION: This study reports outcomes of surgically treated SACs with the longest follow-up time to date. Microsurgical cyst excision or fenestration were safe treatment options, and the neurological improvements seen in the immediate postoperative phase were maintained at long-term follow-up.

An unusual cause of chronic neuropathic pain: report of a case of multiple intradural spinal arachnoid cysts and review of the literature.

Spinal arachnoid cysts (SACs) arise either intra- or extradurally and are usually solitary, while cases of multiple SACs have been scarcely reported in the literature. Herein, we report on a rare case of multiple and recurring intradural spinal arachnoid cysts (SACs) causing severe spinal cord compression and neuropathic radicular pain, in a 35-year-old female with a 10-year follow-up. Two separate attempts at surgery were performed but only provided temporary relief since the cysts recurred and new ones formed along the entirety of the spinal cord. Finally, a conservative approach with physiotherapy and a combination of analgesic medications was pursued.

[Giant arachnoid cyst with hydrocephalus and secondary chronic intracranial hypertension]. / Quiste aracnoideo gigante con hidrocefalia e hipertensión intracraneal crónica secundaria.

TITLE: Quiste aracnoideo gigante con hidrocefalia e hipertensión intracraneal crónica secundaria.

Treatment of symptomatic sacral arachnoid cysts in the pediatric population.

OBJECTIVE: Symptomatic sacral arachnoid cysts are extremely rare in pediatric patients, resulting in a lack of consensus regarding optimal treatment measures. The current study evaluated the clinical symptoms and surgical indications, techniques, and outcomes of pediatric patients who underwent treatment for sacral arachnoid cysts with the aim of developing recommendations for follow-up and treatment. METHODS: This retrospective study included pediatric patients who underwent surgical treatment for sacral arachnoid cysts between January 2000 and December 2020 at the Department of Pediatric Neurosurgery, Acibadem University Faculty of Medicine. RESULTS: Thirteen patients were included in the study, 9 of whom were girls and 4 were boys. Five patients presented with urinary incontinence, 2 of whom also exhibited constipation. Other chief complaints included recurrent urinary tract infections (UTIs) and low-back pain (n = 4 patients each). Urological evaluation was performed in all patients, followed by urodynamic examination in those with urinary symptoms. Spinal MRI showed extra- and intradural sacral cysts in 12 patients and 1 patient, respectively. The latter patient exhibited recurrence during follow-up and underwent reintervention. Samples from the excised cyst walls were sent for pathological examination. Five patients with urinary incontinence, 2 with constipation, 4 with recurrent UTIs, and 3 with low-back pain exhibited resolution of symptoms after treatment. However, 1 patient with low-back pain did not show any improvement in symptoms. No postoperative complications were observed in the current study. The patients were followed-up regularly after surgery, and the mean follow-up duration was 4 years. CONCLUSIONS: Sacral arachnoid cysts in pediatric patients may be associated with urinary system dysfunction and low-back pain. Surgery is the treatment of choice for symptomatic patients and those with enlarged cysts with radiological evidence of compression, and the morbidity and mortality rates associated with surgery are low.

How to locate the fistula orifice of spinal extradural arachnoid cyst: Surgical experience and clinical outcome.

BACKGROUND: In clinical practice, spinal extradural arachnoid cysts (SEAC) are relatively rare. The key to the treatment of SEAC is recognize and close the dural defects (fistula orifice), but there is currently no convenient method to locate and identify the fistula. We propose a method for predicting the location of lumbar/thoracolumbar SEAC fistula based on surgical experience, subsequently closing the fistula through posterior unilateral interlaminar fenestration. Evaluating its surgical efficacy and investigated its effect on patient prognosis. METHODS: A stepped approach based on clinical experience is proposed. A retrospective analysis was performed on 6 patients diagnosed with thoracolumbar SEAC disease and treated with posterior unilateral interlaminar fenestration through the position by pre-estimated fistula orifice in our hospital's Department of Neurosurgery between January 2017 and January 2022. RESULTS: All patients who received this treatment experienced significantly lower postoperative VAS pain scores and ODI index compared to their corresponding preoperative values (P < 0.01). During the ongoing follow-up after surgery, no unstable vertebral column, adverse effects, or complications were reported. CONCLUSIONS: The use of posterior unilateral interlaminar fenestration for the treatment of large SEAC in the adult lumbar/thoracolumbar segment can reduce spinal cord manipulation and enhance spine stability. The disease can be treated by sealing the fistula orifice through a small fenestra, the position of which is assessed before surgery. This surgical method reduces trauma and improves the prognosis of patients with large SEAC.

Controversies in the Treatment of Arachnoid Cysts with Special Emphasis on Temporal Arachnoid Cysts.

Intracranial arachnoid cysts (ACs) are benign lesions. The incidence in children is 2.6%. ACs are often diagnosed incidentally. Because of the broad use of CT and MR imaging, the frequency of AC diagnosis has increased. In addition, prenatal diagnosis of ACs is becoming more common. This places clinicians in a difficult situation with regard to the optimal treatment, since the presenting symptoms are often vague and operative management includes not negligible risks. It is generally accepted that conservative management is indicated in cases with small and asymptomatic cysts. In contrast, patients with definite signs of raised intracranial pressure should be treated. There are however clinical situations in whom the decision about the preferred treatment is difficult to make. Unspecific symptoms such as headaches and neurocognitive or attention deficits can be challenging to evaluate, whether they are related to the presence of the AC or not. The treatment techniques intent to establish a communication between the cyst and the normal cerebrospinal spaces or consist of a diversion of the cyst fluid by a shunt system. Which surgical method (open craniotomy for cyst fenestration, endoscopic fenestration, or shunting) is preferred differs between neurosurgical centers or the pediatric neurosurgeon in charge. Each treatment option has a unique profile of advantages and disadvantages which should be considered when discussing treatment with the patients or their caregivers.

Symptomatic intracranial hypertension in an adult patient with spinal muscular atrophy and arachnoid cysts receiving nusinersen.

 In patients with spinal muscular atrophy (SMA) headache after intrathecal administration of nusinersen is usually attributed to post-lumbar puncture syndrome. However, lumbar puncture opening pressure (LOP) has also been reported to be increased in children with SMA, both before and after treatment with nusinersen, although symptoms associated with increased LOP were not observed. We report to our knowledge the first case of symptomatic intracranial hypertension in an adult SMA patient. This 21-year-old man suffered from headache and vomiting followed by visual disturbances after the 12th injection of nusinersen. Bilateral papilledema was recognized ophthalmologically. MRI of the head showed signs of intracranial hypertension and additionally arachnoid cysts but not hydrocephalus. Symptoms resolved after 8 weeks of treatment with repeated lumbar punctures and acetazolamide. This case raises the possibility of intracranial hypertension as a complication of nusinersen therapy although arachnoid cysts represent another risk factor for intracranial hypertension. We recommend that patients suffering from headache after nusinersen injections should not only be questioned and examined for symptoms suggestive of post-lumbar puncture syndrome, but also intracranial hypertension.

Comparison of Surgical Techniques for Intracranial Arachnoid Cysts: A Volumetric Analysis.

AIM: To compare the volumetric changes of intracranial arachnoid cysts (IACs) in different surgical techniques. MATERIAL AND METHODS: Sixty-six patients who underwent IAC surgery in our department between 2010 and 2020 were studied retrospectively. Based on the surgical technique, clinical and volumetric changes, postoperative complications, recurrence rates, and length of hospital stay were statistically compared. RESULTS: Microsurgical fenestration (MF) was performed on 32 (48.5%) patients, endoscopic fenestration (EF) on 17 patients, cystoperitoneal shunt (CPS) on 11 patients, and EF + CPS in six patients. The mean IAC volume change rate was 68.54 mL, and the mean cyst volume change rate was 40.68%. The MF technique produces a significantly greater mean cyst volume change than the EF technique. The mean volume change in sylvian IAC is 4.8 times greater than in posterior fossa IAC, a significant difference. The mean cyst volume change is four times greater in patients with skull deformity than in patients with balance loss, and this difference is statistically significant. In patients with cranial deformity, the mean cyst volume change is 2.6 times greater than in patients with neurological dysfunction. This difference is also statistically significant. The volume of IAC decreased more in patients with postoperative complications, with a significant difference between the postoperative complication and the change in IAC volume. CONCLUSION: MF can achieve better volumetric reduction in IAC, particularly in patients with sylvian arachnoid cysts. However, more volumetric reduction increases the risk of postoperative complications.

Scoliosis due to scar contracture caused by infection after cyst-peritoneal shunt: a case report and literature review.

BACKGROUND: Cyst-peritoneal (CP) shunt is one of the most common methods for the treatment of intracranial arachnoid cysts (ACs). Infection is a common postoperative complication. We report a patient with scoliosis due to scar contracture caused by infection after CP shunt. CASE DESCRIPTION: A 12-year-old boy underwent CP shunt surgery for the left frontoparietotemporal AC when he was 2 years old. At the age of 7 years, he underwent a shunt catheter removal procedure because of the infection caused by the fistula leading from the subcutaneous tunnel to the body surface. However, contracture of the subcutaneous scar from fistula infection caused scoliosis and limited range of motion of the right arm. At the age of 12, the patient received scar lysis and his symptoms improved. CONCLUSION: We presented the first case of scoliosis due to scar contracture caused by infection after CP shunt. In this case, timely release of scar tissue can effectively correct scoliosis and limb movement limitation.

Factors that Influence Subdural Hemorrhage Secondary to Intracranial Arachnoid Cysts in Children.

OBJECTIVE: This study aimed to investigate factors that influence subdural haemorrhage (SDH) secondary to intracranial arachnoid cysts (IACs) in children. METHODS: Data of children with unruptured IACs (IAC group) and those with SDH secondary to IACs (IAC-SDH group) were analyzed. Nine factors, sex, age, birth type (vaginal or caesarean), symptoms, side (left, right, or midline), location (temporal or nontemporal), image type (I, II, or III), volume, and maximal diameter, were selected. IACs were classified as types I, II, and III according to their morphological changes observed on computed tomography images. RESULTS: There were 117 boys (74.5%) and 40 girls (25.5%); 144 (91.7%) patients comprised the IAC group and 13 (8.3%) comprised the IAC-SDH group. There were 85 (53.8%) IACs on the left side, 53 (33.5%) on the right side, 20 (12.7%) in the midline region, and 91 (58.0%) in the temporal region. The univariate analysis showed significant differences in age, birth type, symptoms, cyst location, cyst volume, and cyst maximal diameter (P < 0.05) between the 2 groups. Logistic regression using the synthetic minority oversampling technique model showed that image type III and birth type were independent factors that influenced SDH secondary to IACs (ß0 = 4.143; ß for image type = -3.979; ß for birth type = -2.542) and that the representative area under the receiver-operating characteristic curve value was 0.948 (95% confidence interval, 0.898-0.997). CONCLUSIONS: IACs are more common in boys than in girls. They can be divided into 3 groups according to their morphological changes on computed tomography images. Image type III and caesarean delivery were independent factors that influenced SDH secondary to IACs.

Symptomatic secondary spinal arachnoid cysts: a systematic review.

BACKGROUND CONTEXT: Secondary spinal arachnoid cysts have rarely been reported but present significant challenges for management. These cysts could be anteriorly located with long rostral-caudal extensions and many are related to arachnoiditis, leading to difficult-to-treat disorders. Thus far, due to the scarcity of reports, the features of the disease and the optimal therapeutic strategies remain unclear. PURPOSE: To investigate clinical features and the optimal treatment modalities of secondary spinal arachnoid cysts compared with primary spinal arachnoid cysts. STUDY DESIGN: Systematic review. PATIENT SAMPLE: Systematic review identified 103 secondary cases from 80 studies and reports. OUTCOME MEASURES: Condition of symptom relief and duration of treatment response were analyzed. METHODS: An electronic literature search of the PubMed database was conducted for studies on secondary spinal arachnoid cysts between 1990 and 2022. Non-English publications, nonhuman studies, reports of a primary cyst, studies not including case details, and studies of nonsymptomatic cases were excluded. RESULTS: This systematic review included 103 secondary cases. The most commonly reported etiologies were iatrogenic factors, trauma, and subarachnoid hemorrhage, accounting for 88 intradural extramedullary, 11 extradural, one intradural/extradural, one interdural, and one intramedullary spinal arachnoid cyst after a median duration of 30, 12, and 9 months, respectively. Extradural cysts were more prone to occur at dorsal locations and affect thoracic segments (mean cyst length: 3.4 segments). Intradural cysts showed a relatively higher ventral/dorsal ratio (1:1.09, 1.75:1, and 3.50:1 for cysts occurring from iatrogenic factors, trauma, and subarachnoid hemorrhage, respectively) and thoracic distribution, with a mean cyst length of 4.3 segments (5.1 for ventral and 3.5 for dorsal cysts). For intradural cysts, recurrence risk was lower after surgical resection than after fenestration/marsupialization (12-month recurrence risk: 21.43% vs 50.72%, log-rank test: p=.0248, Gehan-Breslow-Wilcoxon test: p=.0126). In cases treated with shunting, one recurrence (1/8 cases) was noted after external shunting and two recurrences (2/5 cases) after internal shunting at a median follow up of 12 months. CONCLUSIONS: Secondary spinal arachnoid cysts, particularly intradural cysts, are rarer and more challenging to treat than primary spinal cysts. Although fenestration/marsupialization is the commonly adopted treatment, the recurrence rate is high. For unresectable cysts, shunting procedures, particularly shunting into a body cavity (eg, pleural or peritoneal cavity) away from the subarachnoid space, could be a therapeutic alternative besides fenestration/marupialization, yet its efficacy requires confirmation by more data.

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.