Subtemporal Decompression in Resistant Slit Ventricle Syndrome in Children: An Observational Study and Survival Analysis.

INTRODUCTION: Slit ventricle syndrome (SVS) remains a challenging problem in the early-shunted paediatric population. Various surgical and non-surgical treatments have been devised for this condition. However, there is currently no gold standard for its optimal management. Among various treatment modalities, subtemporal decompression (STD) is often performed as a last resort. We present our experience of STD in paediatric patients with SVS in whom initial treatment with programmable valves and anti-syphon device were not successful. METHODS: This is a single-centre retrospective observational study and survival analysis. Patients who underwent STD for SVS were included. Pre- and post-operative imaging data and clinical outcomes were collected. RESULTS: There were 20 patients (12 M, 8 F) with a mean age of 9 years (SD: 4) at first STD. 90% (n = 18) of patients had multiple shunt revisions pre-STD. At first STD, 70% (n = 14) and 30% (n = 6) of patients had unilateral or bilateral STD, respectively. STD led to a reduction in the frequency of shunt revisions in 60% (n = 12) of patients. The median time required before further STD, shunt surgery, or cranial vault surgery was 14 months. The median time before a further STD was required (either revision or contralateral side) was 89 months. At a median follow-up of 66.5 months (range: 1-159), 65% (n = 13) of patients had improvement in symptoms. CONCLUSIONS: A large proportion of patients with persistent SVS symptoms, refractory to multiple shunt revisions, benefitted from STD in combination with shunt optimization. It was also safe and well-tolerated. Therefore, in patients who have multiple failed shunts, STD may reduce the morbidity associated with further shunt revisions and can significantly improve symptomatology.

A case of recurrent flight-induced cerebrospinal fluid shunt overdrainage.

Shunted patients often complain of headaches after flights. The effect of air travel on shunt systems is unknown. We describe the case of a patient with longstanding hydrocephalus, who suffered flight-induced clinical deterioration and shunt overdrainage in two independent occasions. The patient, clinically stable for 1.5 and 5 years before each episode, reported severe headaches starting during the descent stages of the air travel. On both occasions, brain MRI imaging demonstrated pronounced ventricular size reduction. This case suggests that flight-induced shunt overdrainage can occur and should be suspected in patients with prolonged headaches and/or clinical deterioration triggered by air travel.

Effect of position on intracranial pressure and compliance: a cross-sectional study including 101 patients.

OBJECTIVE: A better understanding of the effect of position on intracranial pressure (ICP) and compliance is important for the development of treatment strategies that can restore normal cerebrospinal fluid (CSF) dynamics. There is limited knowledge on the effect of position on intracranial compliance. In this cross-sectional study the authors tested the association of pulse amplitude (PA) with position and the day/night cycle. Additionally, they describe the postural ICP and PA changes of patients with "normal" ICP dynamics. METHODS: This single-center retrospective study included patients with suspected and/or confirmed CSF dynamics abnormalities who had been examined with elective 24-hour ICP monitoring between October 2017 and September 2019. Patients had been enrolled in a short exercise battery including four positions: supine, lumbar puncture position in the left lateral decubitus position, sitting, and standing. Each position was maintained for 2 minutes, and mean ICP and PA were calculated for each position. The 24-hour day and night median ICP and PA data were also collected. Linear regression models were used to test the correlation of PA with position and day/night cycle. All linear regressions were corrected for confounders. The postural ICP monitoring results of patients without obvious ICP dynamics abnormality were summarized. RESULTS: One hundred one patients (24 males and 77 females) with a mean age of 39 ± 13years (mean ± standard deviation) were included in the study. The adjusted linear regression models demonstrated a significant association of ICP with position and day/night cycle, with upright (sitting and standing) and day ICP values lower than supine and night ICP values. The adjusted linear regression model was also significant for the association of PA with position and day/night cycle, with upright and day PA values higher than supine and night PA results. These associations were confirmed for patients with and without shunts. Patients without clear ICP dynamics abnormality had tighter control of their postural ICP changes than the other patients; however, the difference among groups was not statistically significant. CONCLUSIONS: This is the largest study investigating the effect of postural changes on intracranial compliance. The results of this study suggest that PA, as well as ICP, is significantly associated with posture, increasing in upright positions compared to that while supine. Further studies will be needed to investigate the mechanism behind this association.

Brain MRI and Ophthalmic Biomarkers of Intracranial Pressure.

OBJECTIVE: To evaluate the utility of brain MRI and ophthalmic biomarkers for the prediction of intracranial hypertension, we have studied the association between six biomarkers and 24-hour intracranial pressure (ICP) monitoring results in 45 patients. METHODS: This single-centre observational study includes patients who underwent 24-hour ICP monitoring, brain MRI (within three months) and ophthalmic assessment (during ICP monitoring). Six biomarkers were investigated: pituitary gland shape, vertical tortuosity of the optic nerve, distension of the optic nerve sheath, optic disc protrusion (MRI), papilloedema (slit lamp biomicroscopy) and spontaneous venous pulsations (SVP, infrared video recordings). RESULTS: Forty-five patients (mean age 39±14SD, 38 females) met the inclusion criteria. All 6 biomarkers had a significant association with 24-hour ICP. Concave pituitary gland was observed with moderately elevated median ICP. Protrusion of the optic disc (MRI), papilloedema and absence of SVP were associated with the highest median ICP values. Twenty patients had raised ICP (median 24-hour ICP>5.96 mmHg, cut-off obtained through Youden index calculation). Patients with all normal biomarkers had normal median ICP in 94% (St.Err.=6%) of the cases. All the patients with 3 or more abnormal biomarkers had intracranial hypertension. The combination of at least one abnormal biomarker in MRI and ophthalmic assessments was highly suggestive of intracranial hypertension (AUC 0.94, 95% CI 0.93-0.94) CONCLUSIONS: Brain MRI and ophthalmic biomarkers can non-invasively guide the management of patients with suspected CSF dynamics abnormalities. Patients with multiple abnormal biomarkers (≥3) or a combination of abnormal MRI and ophthalmic biomarkers are likely to have intracranial hypertension and should be managed promptly.

Ultra-low-pressure hydrocephalic state in NPH: benefits of therapeutic siphoning with adjustable antigravity valves.

BACKGROUND: Idiopathic normal-pressure hydrocephalus (NPH) is a condition of the elderly treated by ventriculoperitoneal shunt (VP) insertion. A subset of NPH patients respond only temporarily to shunt insertion despite low valve opening pressure. This study aims to describe our experience of patients who benefit from further CSF drainage by adding adjustable antigravity valves and draining CSF at ultra-low pressure. METHODS: Single-centre retrospective case series of patients undergoing shunt valve revision from an adjustable differential pressure valve with fixed antigravity unit to a system incorporating an adjustable gravitational valve (Miethke proSA). Patients were screened from a database of NPH patients undergoing CSF diversion over 10 consecutive years (April 2008-April 2018). Clinical records were retrospectively reviewed for interventions and clinical outcomes. RESULTS: Nineteen (10F:9M) patients underwent elective VP shunt revision to a system incorporating an adjustable gravitational valve. Mean age 77.1 ± 7.1 years (mean ± SD). Eleven patients (58%) showed significant improvement in walking speed following shunt revision. Fourteen patients/carers (74%) reported subjective improvements in symptoms following shunt revision. CONCLUSIONS: Patients presenting symptoms relapse following VP shunting may represent a group of patients with ultra-low-pressure hydrocephalus, for whom further CSF drainage may lead to an improvement in symptoms. These cases may benefit from shunt revision with an adjustable gravitational valve, adjustment of which can lead to controlled siphoning of CSF and drain CSF despite ultra-low CSF pressure.

Paradoxical response of intracranial pressure to shunt valve setting adjustments.

BACKGROUND: The hydrodynamics of cerebrospinal fluid shunts have been described in vitro; however, knowledge on the response of intracranial pressure (ICP) to valve settings adjustments in vivo is limited. This study describes the effect of adjusting the shunt valve setting on ICP in a cohort of patients with complex symptom management. METHOD: Single-centre retrospective observational study. Patients who underwent ICP-guided valve setting adjustments during 24-h continuous ICP monitoring, between 2014 and 2019, were included. Patients with suspected shunt malfunction were excluded. Median night ICP before and after the valve adjustments were compared (Δ night ICP). The responses of ICP to valve adjustment were divided into 3 different groups as follows: expected, paradoxical and no response. The frequency of the paradoxical response and its potential predicting factors were investigated. RESULTS: Fifty-one patients (37 females, 14 males, mean age 38 years) receiving 94 valve setting adjustments met the study inclusion criteria. Patients' underlying conditions were most commonly hydrocephalus (47%) or idiopathic intracranial hypertension (43%). The response of ICP to valve setting adjustments was classified as 'expected' in 54 cases (57%), 'paradoxical' in 17 cases (18%) and 'no effect' (Δ night ICP < 1 mmHg) in 23 cases (24%). There was a significant correlation between the Δ night ICP and the magnitude of valve setting change in both the investigated valves (Miethke ProGAV, p = 0.01 and Medtronic Strata, p = 0.02). CONCLUSIONS: Paradoxical ICP changes can occur after shunt valve setting adjustments. This observation should be taken into account when performing ICP-guided valve adjustments and is highly relevant for the future development of "smart" shunt systems.