Surgical treatment of symptomatic pineal cysts without hydrocephalus—meta‑analysis of the published literature

Acta Neurochirurgica (2022) 164:61–77

To examine published data and assess evidence relating to safety and efficacy of surgical management of symptomatic pineal cysts without hydrocephalus (nhSPC), we performed a systematic review of the literature and meta-analysis.

Methods Following the PRISMA guidelines, we searched Pubmed and SCOPUS for all reports with the query ‘Pineal Cyst’ AND ‘Surgery’ as of March 2021, without constraints on study design, publication year or status (PROSPERO_ CRD:42,021,242,517). Assessment of 1537 hits identified 26 reports that met inclusion and exclusion criteria.

Results All 26 input studies were either case reports or single-centre retrospective cohorts. The majority of outcome data were derived from routine physician-recorded notes. A total of 294 patients with surgically managed nhSPC were identified. Demographics: Mean age was 29 (range: 4–63) with 77% females. Mean cyst size was 15 mm (5–35). Supracerebellar infratentorial approach was adopted in 90% of cases, occipital-transtentorial in 9%, and was not reported in 1%. Most patients were managed by cyst resection (96%), and the remainder by fenestration. Mean post-operative follow-up was 35 months (0–228). Presentation: Headache was the commonest symptom (87%), followed by visual (54%), nausea/vomit (34%) and vertigo/dizziness (31%). Other symptoms included focal neurology (25%), sleep disturbance (17%), cognitive impairment (16%), loss of consciousness (11%), gait disturbance (11%), fatigue (10%), ‘psychiatric’ (2%) and seizures (1%). Mean number of symptoms reported at presentation was 3 (0–9). Outcomes: Improvement rate was 93% (to minimise reporting bias only consecutive cases from cohort studies were considered, N = 280) and was independent of presentation. Predictors of better outcomes were large cyst size (OR = 5.76; 95% CI: 1.74–19.02) and resection over fenestration (OR = 12.64; 3.07–52.01). Age predicted worse outcomes (OR = 0.95; 0.91–0.99). Overall complication rate was 17% and this was independent of any patient characteristics. Complications with long-term consequences occurred in 10 cases (3.6%): visual disturbance (3), chronic incisional pain (2), sensory disturbance (1), fatigue (1), cervicalgia (1), cerebellar stroke (1) and mortality due to myocardial infarction (1).

Conclusions Although the results support the role of surgery in the management of nhSPCs, they have to be interpreted with a great deal of caution as the current evidence is limited, consisting only of case reports and retrospective surgical series. Inherent to such studies are inhomogeneity and incompleteness of data, selection bias and bias related to assessment of outcome carried out by the treating surgeon in the majority of cases. Prospective studies with patient-reported and objective outcome assessment are needed to provide higher level of evidence.


Management of Chiari type I malformation: a retrospective analysis of a series of 91 children treated surgically

Acta Neurochirurgica (2021) 163:3065–3073

The diagnosis of Chiari I malformation, its symptomatology, and the results of its surgical management are still discussed. We report a pediatric series of CMI without associated skull base malformations or cerebellar growth anomalies operated between 2001 and 2018.

Material and methods Ninety-one children out of 146 surgically treated cases have been included in the study. Age at surgery ranged from 5 months to 17 years clinical data, and complementary examinations leading to the surgical indication have been analyzed together with the surgical outcomes. The average follow-up duration was of 4 years. The occipito-cervical decompression with duraplasty without opening the arachnoid was the procedure of election. Three quarters of patients presented with headaches, 12% with cerebellar syndrome, 13% with vertigo, 26% with nausea or vomiting, 24% with sensorimotor deficits, 11% with cranial nerve deficits, and 29% with other symptoms. Eighteen percent of patients suffered from scoliosis, 47% had an associated syrinx and 16% a ventricular dilation.

Results After the treatment, the clinical symptomatology improved in about three-quarters of the patients: headache (69.4%), nausea or vomiting (66.7%), sensorimotor deficits (55.6%), and other symptoms (78.3%). Syringomyelic cavities diminished partially in size or disappeared in 58.3% of patients, remained stable in 29.2%, and worsened in 12.5%. Only one-third of children with preoperative scoliosis benefited from the surgical treatment. No clinical signs or symptoms were found to be reliable predictors of surgical success, neither the extent of the cerebellar tonsil descent.

Conclusion Occipito-cervical decompression allows to improve the clinical condition in the majority of children with symptomatic CMI in the absence of associated cervico-spinal junction alterations, craniosynostosis, or cerebellar growth anomalies. No clinical signs or symptoms neither radiological criterion appear to be a specific finding for the surgical indication.

Diffusion tensor imaging and ventricle volume quantification in patients with chronic shunt-treated hydrocephalus

J Neurosurg 129:1611–1622, 2018

The object of this study was to use diffusion tensor imaging (DTI) and tract-based spatial statistics (TBSS) to characterize the long-term effects of hydrocephalus and shunting on white matter integrity and to investigate the relationship of ventricular size and alterations in white matter integrity with headache and quality-of-life outcome measures.

METHODS Patients with shunt-treated hydrocephalus and age- and sex-matched healthy controls were recruited into the study and underwent anatomical and DTI imaging on a 3-T MRI scanner. All patients were clinically stable, had undergone CSF shunt placement before 2 years of age, and had a documented history of complaints of headaches. Outcome was scored based on the Headache Disability Inventory and the Hydrocephalus Outcome Questionnaire. Fractional anisotropy (FA) and other DTI-based measures (axial, radial, and mean diffusivity; AD, RD, and MD, respectively) were extracted in the corpus callosum and internal capsule with manual region-of-interest delineation and in other regions with TBSS. Paired t-tests, corrected with a 5% false discovery rate, were used to identify regions with significant differences between patients and controls. Within the patient group, linear regression models were used to investigate the relationship between FA or ventricular volume and outcome, as well as the effect of shunt-related covariates.

RESULTS Twenty-one hydrocephalus patients and 21 matched controls completed the study, and their data were used in the final analysis. The authors found significantly lower FA for patients than for controls in 20 of the 48 regions, mostly posterior white matter structures, in periventricular as well as more distal tracts. Of these 20 regions, 17 demonstrated increased RD, while only 5 showed increased MD and 3 showed decreased AD. No areas of increased FA were observed. Higher FA in specific periventricular white matter tracts, tending toward FA in controls, was associated with increased ventricular size, as well as improved clinical outcome.

CONCLUSIONS The study shows that TBSS-based DTI is a sensitive technique for elucidating changes in white matter structures due to hydrocephalus and chronic CSF shunting and provides preliminary evidence that DTI may be a valuable tool for tailoring shunt procedures to monitor ventricular size following shunting and achieve optimal outcome, as well as for guiding the development of alternate therapies for hydrocephalus.


Pulsatile versus non-pulsatile tinnitus in idiopathic intracranial hypertension

Acta Neurochirurgica (2018) 160:2025–2029

Tinnitus is a symptom commonly associated with idiopathic intracranial hypertension (IIH) that can have a profound effect on quality of life. We aim to determine tinnitus symptom response after dural venous sinus stenting (DVSS) or CSF diversion with a shunt, in patients with both pulsatile (PT) and non-pulsatile tinnitus (NPT).

Methods: Single-centre cohort of IIH patients (2006–2016) who underwent 24-h ICP monitoring (ICPM). An un-paired t test compared ICP and pulse amplitude (PA) values in IIH patients with PT vs. NPT.

Results: We identified 59 patients with IIH (56 F:3 M), mean age 32.5 ± 9.49 years, 14 of whom suffered from tinnitus. Of these 14, seven reported PT and seven reported NPT. Patients with tinnitus had a mean 24-h ICP and PA of 9.09 ± 5.25 mmHg and 6.05 ± 1.07 mmHg respectively. All 7 patients with PT showed symptom improvement or resolution after DVSS (n = 4), secondary DVSS (n=2) or shunting (n = 1). In contrast, of the 7 with NPT, only 1 improved post intervention (DVSS), despite 2 patients having shunts and 5 having DVSS.

Conclusions: NPT and PT were equally as common in our group of IIH patients. DVSS appears to be an effective management option for IIH patients with a clear history of pulsatile tinnitus. However, non-pulsatile tinnitus was more persistent and did not respond well to either DVSS or CSF diversion.

Epilepsy, headache, and abdominal pain after shunt surgery for idiopathic normal pressure hydrocephalus: the INPH-CRasH study

J Neurosurg 128:1674–1683, 2018

Adverse events related to shunt surgery are common and might have a negative effect on outcome in patients with idiopathic normal pressure hydrocephalus (INPH). The authors’ objectives were to establish the frequencies of epilepsy, headache, and abdominal pain and determine their impact on patient quality of life (QOL), in long-term follow-up after shunt surgery for INPH.

METHODS One hundred seventy-six shunt-treated patients with INPH (mean age 74 years) and 368 age- and sexmatched controls from the population were included. The mean follow-up time after surgery was 21 months (range 6–45 months). Each participant answered a questionnaire regarding present frequency and severity of headache and abdominal pain. Confirmed diagnoses of epilepsy and all prescriptions for antiepileptic drugs (AEDs) before and after shunt surgery for INPH were gathered from national registries. Equivalent presurgical and postsurgical time periods were constructed for the controls based on the date of surgery (the division date for controls is referred to as virtual surgery). All registry data covered a mean period of 6 years (range 3–8 years) before surgery/virtual surgery and 4 years (range 2–6 years) after surgery/virtual surgery. Provoked epileptic seizures were excluded. Patient QOL was assessed with the EuroQoL 5-dimension 5-level instrument.

RESULTS Epilepsy was more common in shunt-treated patients with INPH than in controls (4.5% vs 1.1%, respectively; p = 0.023), as was treatment with AEDs (14.8% vs 7.3%, respectively; p = 0.010). No difference was found between the populations before surgery/virtual surgery (epilepsy, 2.3% [INPH] vs 1.1% [control], p = 0.280; AED treatment, 8.5% [INPH] vs 5.4% [control], p = 0.235). New-onset epilepsy and new AED treatment after surgery/virtual surgery were more common in INPH (epilepsy, 2.3% [INPH] vs 0.0% [control], p = 0.011; AED, 8.5% [INPH] vs 3.3% [control], p = 0.015). At follow-up, more patients with INPH than controls experienced headache several times per month or more often (36.1% vs 11.6%, respectively; p < 0.001). Patients with INPH and unilateral headache had more right-sided headaches than controls (p = 0.038). Postural headache was experienced by 16% (n = 27 of 169) of the patients with INPH. Twenty percent (n = 35) of the patients with INPH had persistent abdominal pain. Headache was not correlated to lower QOL. The study was underpowered to draw conclusions regarding QOL in patients with INPH who had epilepsy and abdominal pain, but the finding of no net difference in mean QOL indicates that no correlation between them existed.

CONCLUSIONS Epilepsy, headache, and abdominal pain are common in long-term follow-up in patients after shunt surgery for INPH and are more common among patients with INPH than in the general population. All adverse events, including mild and moderate ones, should be considered during postoperative follow-ups and in the development of new methods for shunt placement.


Surgical Decompression of Arachnoid Cysts Leads to Improved Quality of Life

Suprasellar Arachnoid Cysts- Toward a New Simple Classification Based on Prognosis and Treatment Modality

Neurosurgery 78:613–625, 2016

There is no previous prospective study on the outcome of surgical decompression of intracranial arachnoid cysts (AC).

OBJECTIVE: To investigate if surgical fenestration for AC leads to change in patients’ health-related quality of life.

METHODS: Prospective study including 76 adult patients operated for AC. Patients responded to Short Form-36 and Glasgow Benefit Inventory quality of life questionnaires, and to visual analogue scales, assessing headache and dizziness pre- and postoperatively. Patient scores were compared with those of a large sample of healthy individuals.

RESULTS: Preoperatively, 84.2% of the patients experienced headache and 70.1% dizziness. Mean pre- versus postoperative Visual Analogue Scale scores for headache and dizziness dropped from 45.6 to 25.7 and from 35.2 to 12.2, respectively. Preoperative Short Form-36 scores were significantly below age norms in all subscales, but improved after surgery into normal range in 7 out of 8 subscales for middle-aged and older patients. Younger patients’ scores remained lower than age norm in 6 out of 8 subscales. A significant postoperative improvement was seen in 3 out of 4 Glasgow Benefit Inventory subscales. Improvement in headache and/or dizziness, but not preoperative cyst size or reduction in cyst volume, correlated with improvement in 6 out of 8 Short Form-36 subscales and 3 out of 4 Glasgow Benefit Inventory subscales. Only 1 patient experienced a severe complication causing permanent invalidity.

CONCLUSION: Surgery for AC can be performed with a fairly low risk of complications and yields significant improvement in quality of life correlated to postoperative improvement in headache and dizziness. These findings may justify a more liberal approach to surgical treatment for AC.

Headache in Patients With Pituitary Lesions

Intraoperative high-field MRI for transsphenoidal reoperations of nonfunctioning pituitary adenoma

Neurosurgery 78:316–323, 2016

Headache is a presenting feature in 37% to 70% of patients with pituitary tumor. Other pituitary lesions may also present with headache, and together these lesions account for about 20% of all primary brain lesions. Although pituitary lesions have been associated with headache, the exact nature of the relationship remains undefined. It is not always clear whether the presenting headache is an unrelated primary headache, a lesion-induced aggravation of a preexisting primary headache, or a separate secondary headache related to the lesion.

OBJECTIVE: To characterize headache in patients referred to a multidisciplinary neuroendocrine clinic with suspected pituitary lesions and to assess changes in headache in those who underwent surgery.

METHODS: We used a self-administered survey of headache characteristics completed by patients upon presentation and after any pituitary surgical procedure.

RESULTS: One hundred thirty-three participants completed the preoperative questionnaire (response rate of 99%). The overall prevalence of headache was 63%. Compared to patients without headache, the group with headache was more likely to be female (P = .001), younger (P = .001), and to have had a prior headache diagnosis (P < .001). Seventy-two percent of patients reported headache localized to the anterior region of the head. Fifty-one patients with headache underwent transsphenoidal pituitary surgery. Headache was not associated with increased odds of having surgery (odds ratio, 0.90). At 3 months, 81% of surgically treated patients with headache who completed the postoperative questionnaire (21/26) reported improvement or resolution of headaches. No patient who completed the postoperative questionnaire (44/84) reported new or worsened headache.

CONCLUSION: Frequent, disabling headaches are common in patients with pituitary lesions referred for neuroendocrine consultation, especially in younger females with a preexisting headache disorder. Surgery in this group was associated with headache improvement or resolution in the majority and was not found to cause or worsen headaches. Suggestions for revision of the International Classification of Headache Disorders diagnostic criteria pertaining to pituitary disorders are supported by these findings.

Long-term outcome and prognostic factors after C2 ganglion decompression in 68 consecutive patients with intractable occipital neuralgia

Long-term outcome and prognostic factors after C2 ganglion decompression in 68 consecutive patients with intractable occipital neuralgia

Acta Neurochir (2015) 157:85–92

Occipital neuralgia is a rare cause of severe headache characterized by paroxysmal shooting or stabbing pain in the distribution of the greater occipital or lesser occipital nerve. In cases of intractable occipital neuralgia, a definite cause has not been uncovered, so various types of treatment have been applied. The aim of this study is to evaluate the prognostic factors, safety, and long-term clinical efficacy of second cervical (C2) ganglion decompression for intractable occipital neuralgia.

Methods Retrospective analysis was performed in 68 patients with medically refractory occipital neuralgia who underwent C2 ganglion decompression. Factors based on patients’ demography, pre- and postoperative headache severity/characteristics, medication use, and postoperative complications were investigated. Therapeutic success was defined as pain relief by at least 50 % without ongoing medication.

Results The visual analog scale (VAS) score was significantly reduced between the preoperative and most recent follow-up period. One year later, excellent or good results were achieved in 57 patients (83.9 %), but poor in 11 patients (16.1 %). The long-term outcome after 5 years was only slightly less than the 1-year outcome; 47 of the 68 patients (69.1 %) obtained therapeutic success. Longer duration of headache (over 13 years; p=0.029) and presence of retro-orbital/frontal radiation (p=0.040) were significantly associated with poor prognosis.

Conclusions In the current study, C2 ganglion decompression provided durable, adequate pain relief with minimal complications in patients suffering from intractable occipital neuralgia. Due to the minimally invasive and nondestructive nature of this surgical procedure, C2 ganglion decompression is recommended as an initial surgical treatment option for intractable occipital neuralgia before attempting occipital nerve stimulation. However, further study is required to manage the pain recurrence associated with longstanding nerve injury.

Endoscopic endonasal resection of Rathke cleft cysts: clinical outcomes and surgical nuances

J Neurosurg 112:1333–1339, 2010. DOI: 10.3171/2009.10.JNS09348

Rathke cleft cysts (RCCs) are benign lesions that can be diagnosed as an incidental finding associated with headaches, pituitary dysfunction, or vision deterioration. Typically, they occur in a sellar or suprasellar location. The aim of this study was to review the clinical presentation and outcomes associated with endoscopic endonasal resection of these lesions.
Methods: The authors retrospectively reviewed a series of 35 patients with a diagnosis of RCC after endoscopic endonasal resection at the University of Pittsburgh between January 1998 and July 2008.
Results: All 35 patients underwent a purely endoscopic endonasal approach (EEA). The average patient age was 34 years (range 12–67 years), and the average follow-up was 19 months (range 1–60 months). Clinical follow-up data were available for 32 patients, and radiographic follow-up data were accessible for 33 patients. All of the patients underwent complete removal of the cyst contents, and according to radiography studies 2 patients had a recurrence, neither of which required reoperation. The mean cyst volume was 1052.7 mm3 (range 114–6044 mm3). Headache was a presenting symptom in 26 (81.2%) of 32 patients, with 25 (96.1%) of 26 having postoperative improvement in their headaches. Fifteen (57.7%) of the 26 patients had complete pain resolution, and 10 (38.5%) had a > 50% reduction in their pain scores. Six (18.8%) of 32 patients initially presented with pituitary dysfunction, although 2 (33.3%) had postoperative improvement. Three (9.4%) of 32 patients had temporary pituitary dysfunction postoperatively, although there was no permanent pituitary dysfunction. Neither were there any intraoperative complications, postoperative CSF leaks, or new neurological deficits. The average hospital stay was 1.8 days (range 1–5 days).
Conclusions: The EEA is a safe and effective approach in the treatment of RCCs. None of the patients in this study experienced any worsening of their preoperative symptoms or pituitary function, and 96% of the patients who had presented with headache experienced complete or significant pain relief following treatment.
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