Positional shunt assist for slit ventricle syndrome.

PURPOSE: Hydrocephalus is one of the most common pathologies in pediatric neurosurgery. One of the causes of recurring events of headaches among shunted children is "slit ventricle syndrome" (SVS). Several potential treatments have been proposed, yet SVS often represents a treatment challenge. The goal of the current series is to present our experience with adding a positional shunt-assist (SA) (Miethke, Aesculap) for the treatment of SVS. METHODS: Clinical data was retrospectively collected from all consecutive children with SVS that were treated with SA (Miethke, Aesculap) at our center. Surgical and clinical outcomes as expressed by hospital visits, or need for additional surgery, were evaluated. RESULTS: Nine cases were included. Hydrocephalus etiology included IVH (6), postinfectious (1), and congenital syndromes (2). Average age at first shunt was 4 months. Primary shunt type was differential-pressure-valve in all. Average age at SVS onset was 4 years. Average age at SA placement was 5.5 years. There were no perioperative complications besides a single stich abscess. A 6-month follow-up period after SA was compared to a 6-month period prior to the SA: average hospital visits decreased from 2.4 to 0.6 per patient (p < 0.0002). 4/9 patients needed an LP or shunt revision before the SA surgery, while no procedure was indicated during the immediate 6-month follow-up. At the last follow-up, there was a significant reduction in the rate of ER visits compared to prior to surgery; however, the number of neurosurgical procedures did not significantly differ. CONCLUSION: Using a SA for SVS was associated with a short-term improvement of symptoms in the majority of cases, reduction in hospital visits, and reduced need for SVS-related procedures.

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.

Noninvasive intracranial pressure monitoring throughout brain compliance guiding a ventriculoperitoneal shunt replacement in hydrocephalus-case report.

INTRODUCTION: Ventriculoperitoneal (VP) shunt is the primary therapy for hydrocephalus in children; however, this technique is amenable to malfunctions, which could be detected through an assessment of clinical signs and imaging results. Furthermore, early detection can prevent patient deterioration and guide clinical and surgical treatment. CASE PRESENTATION: A 5-year-old female with a premedical history of neonatal IVH, secondary hydrocephalus, multiple VP shunts revisions, and slit ventricle syndrome was evaluated using a noninvasive intracranial pressure monitor device at the early stages of the clinical symptoms, evidencing increased intracranial pressure and poor brain compliance. Serial MRI images demonstrated a slight ventricular enlargement, leading to the use of a gravitational VP shunt, promoting progressive improvement. On the follow-up visits, we used the noninvasive ICP monitoring device to guide the shunt adjustments until symptom resolution. Furthermore, the patient has been asymptomatic for the past 3 years without requiring new shunt revisions. DISCUSSION: Slit ventricle syndrome and VP shunt dysfunctions are challenging diagnoses for the neurosurgeon. The noninvasive intracranial monitoring has allowed a closer follow-up assisting early assessment of brain compliance changes related to a patient's symptomatology. Furthermore, this technique has high sensitivity and specificity in detecting alterations in the intracranial pressure, serving as a guide for the adjustments of programmable VP shunts, which may improve the patient's quality of life. CONCLUSION: Noninvasive ICP monitoring may lead to a less invasive assessment of patients with slit ventricle syndrome and could be used as a guide for adjustments of programmable shunts.

Visual acuity loss and sixth nerve palsy as the only manifestations of slit ventricle syndrome.

The case is presented of a girl diagnosed with obstructive hydrocephalus due to pilomyxoid astrocytoma, which required a ventriculoperitoneal shunt (VPS) at the age of 5 years and 10 months. Two months later, magnetic resonance imaging of the brain did not show ventriculomegaly or other signs of increased intracranial pressure. At the age of 6 years and 2 months, a rapid onset of bilateral visual acuity loss developed and she was diagnosed with slit ventricle syndrome. Despite valve revisions of the VPS, she developed an abrupt decline of visual acuity to hand motion at 10 cm. Fundus examination revealed bilateral optic atrophy. She did not report any other systemic symptoms suggesting increased intracranial pressure, such as headache, nausea, vomiting, lethargy, irritability, or altered levels of consciousness.

Management of Slit Ventricle Syndrome: A Single-Center Case Series of 32 Surgically Treated Patients.

OBJECTIVE: Slit ventricle syndrome (SVS) is an iatrogenic disease occurring in patients with ventriculoperitoneal shunt. This article reports the management modalities and results in a case series from a single center. METHODS: We reviewed a series 48 hospitalized patients with severe SVS whom we managed in a 10-year period. Thirty-seven patients harboring programmable valves (P-valves) first underwent attempts at valve reprogramming. This treatment produced no effect in 21 patients, who therefore required surgical treatment. Surgery was also required by 11 patients without P-valve. Accordingly, 32 patients had to be operatively treated by shunt externalization followed by valve replacement or endoscopic third ventriculostomy basing on intracranial pressure and ventricular size. The new valve was either ProGav Mietke (Aesculap) or Medos Codman (Integra), each equipped with its own antisiphon system. In selected cases, a programmable antisiphon system (ProSa Mietke) was used. RESULTS: Surgical mortality was 3% and major morbidity accounted for 6%. Complete resolution was obtained in 55% of cases, improvement in 32%, and no effect or worsening in 13%. Only 1 patient became shunt free after endoscopic third ventriculostomy. Medos and ProGrav provided comparable outcomes, whereas ProSa was determinant in selected cases. Pediatric age, uncomplicated shunt courses, and short SVS histories were significantly favorable indicators. CONCLUSIONS: SVS management remains problematic. However, this study individuated factors that may improve the outcome, such as wider use of P-valves to treat hydrocephalus, timely diagnosis of overdrainage, and earlier and more aggressive indications to manage SVS.

The Expanding Role of Posterior Vault Distraction Osteogenesis in Idiopathic Intracranial Hypertension and Slit Ventricle Syndrome.

ABSTRACT: The purpose of this study was to compare perioperative safety and efficacy of posterior vault distraction osteogenesis (PVDO) in patients with primary nonsynostotic cephalo-cranial disproportion, namely slit ventricle syndrome and idiopathic intracranial hypertension (IIH), to a cohort of patients with craniosynostosis (CS). A retrospective review of patients undergoing PVDO from 2009 to 2019 at our institution was performed. Craniosynostosis patients were matched by sex and age at PVDO to the nonsynostotic cohort. Operative details, perioperative outcomes, and distraction patterns were analyzed with appropriate statistics. Nine patients met inclusion criteria for the non-CS cohort. Six patients (67%) underwent PVDO for slit ventricle and the remaining 3 patients (33%) underwent PVDO for IIH. The majority of CS patients were syndromic (n = 6, 67%) and had multisuture synostosis (n = 7, 78%). The non-CS cohort underwent PVDO at a median 56.1 months old [Q1 41.1, Q3 86.6] versus the CS cohort at 55.7 months [Q1 39.6, Q3 76.0] (P = 0.931). Total hospital length of stay was longer in the non-CS patients (median days 5 [Q1 4, Q3 6] versus 3 [Q1 3, Q3 4], P = 0.021). Non-CS patients with ventriculoperitoneal shunts had significantly less shunt operations for ICP concerns post-PVDO (median rate: 1.74/year [Q1 1.30, Q3 3.00] versus median: 0.18/year [Q1 0.0, Q3 0.7]; P = 0.046). In this pilot study using PVDO to treat slit ventricle syndrome and IIH, safety appears similar to PVDO in the synostotic setting. The cohort lacks adequate follow-up to assess long term efficacy, although short-midterm follow-up demonstrates promising results with less need for shunt revision and symptomatic relief. Future studies are warranted to identify the preferred surgical approach in these complex patients.

Novel Endoscopic Technique for Inserting a Sheath into a Slit Ventricle: The Water-Slide Technique.

BACKGROUND: The efficacy and safety of endoscopic procedures for slit ventricle syndrome (SVS) have been presented in recent studies. However, inserting a sheath into a target ventricle is essential in the beginning of endoscopic procedures for SVS, and this maneuver is challenging owing to the quite narrow ventricular space. We report a novel flexible endoscopic technique, the water-slide technique, that can effectively guide a sheath into a slit ventricle without the use of other adjunctive devices, such as a neuronavigation system and an ultrasound machine. METHODS: Ten endoscopic procedures using the water-slide technique were performed in 9 patients with SVS. All patients had undergone ventriculoperitoneal shunt placement. Using the technique, the sheath was first placed on the flexible endoscope. The endoscope was then inserted into the lateral ventricle along the tract around the pre-existing ventricular catheter with irrigation of artificial cerebrospinal fluid via the working channel of the endoscope. After the endoscope reached the ventricle, the sheath was inserted into the ventricle along the endoscope. The endoscope was then used as a stylet. The endoscopic procedure was performed afterward. Continuous irrigation of artificial cerebrospinal fluid via the working channel is important when using this technique. RESULTS: In all cases, the sheath was successfully introduced to the slit ventricle without the use of adjunctive devices. There were no postoperative complications correlated with the maneuver. CONCLUSIONS: This novel endoscopic technique is effective and safe for inserting a sheath into a target ventricle in the management of SVS.

Predictors of fast and ultrafast shunt failure in pediatric hydrocephalus: a Hydrocephalus Clinical Research Network study.

OBJECTIVE: The primary objective of this study was to use the prospective Hydrocephalus Clinical Research Network (HCRN) registry to determine clinical predictors of fast time to shunt failure (≤ 30 days from last revision) and ultrafast time to failure (≤ 7 days from last revision). METHODS: Revisions (including those due to infection) to permanent shunt placements that occurred between April 2008 and November 2017 for patients whose entire shunt experience was recorded in the registry were analyzed. All registry data provided at the time of initial shunt placement and subsequent revision were reviewed. Key variables analyzed included etiology of hydrocephalus, age at time of initial shunt placement, presence of slit ventricles on imaging at revision, whether the ventricles were enlarged at the time of revision, and presence of prior fast failure events. Univariable and multivariable analyses were performed to find key predictors of fast and ultrafast failure events. RESULTS: A cohort of 1030 patients with initial shunt insertions experienced a total of 1995 revisions. Of the 1978 revision events with complete records, 1216 (61.5%) shunts remained functional for more than 1 year, and 762 (38.5%) failed within 1 year of the procedure date. Of those that failed within 1 year, 423 (55.5%) failed slowly (31-365 days) and 339 (44.5%) failed fast (≤ 30 days). Of the fast failures, 131 (38.6%) were ultrafast (≤ 7 days). In the multivariable analysis specified a priori, etiology of hydrocephalus (p = 0.005) and previous failure history (p = 0.011) were independently associated with fast failure. Age at time of procedure (p = 0.042) and etiology of hydrocephalus (p = 0.004) were independently associated with ultrafast failure. These relationships in both a priori models were supported by the data-driven multivariable models as well. CONCLUSIONS: Neither the presence of slit ventricle syndrome nor ventricular enlargement at the time of shunt failure appears to be a significant predictor of repeated, rapid shunt revisions. Age at the time of procedure, etiology of hydrocephalus, and the history of previous failure events seem to be important predictors of fast and ultrafast shunt failure. Further work is required to understand the mechanisms of these risk factors as well as mitigation strategies.

Aesthetic Cranial Vault Expansion in a Child With Slit Ventricle Syndrome and Eumorphic Face.

Ventricular shunting procedures represent the classical surgical treatment for hydrocephalus. Slit ventricle syndrome (SVS) with craniocerebral disproportion (CCD) and secondary craniosynostosis (SCS) is a well-known but uncommon complication following cerebrospinal fluid (CSF) shunting in children. Its general management includes shunt upgrade or revision, placement of anti-siphon devices, lumbo-peritoneal shunting, and endoscopic third ventriculostomy. Cranial expansion is generally considered a major procedure and is indicated when less invasive treatments fail. In these cases, SVS and associated SCS have been usually managed through anterior cranial vault expansion. This procedure aims to decrease the risk of further shunt revisions and to improve neurologic symptoms, but it takes the disadvantage of considerable iatrogenic alterations of the facial proportions. The authors report a case of a 6-year old boy with SVS and SCS, who maintained eumorphic face and was treated by an innovative post-coronal vault expansion ensuring a wide volume increase while avoiding any change of facial conformation.

Slit Ventricle as a Neurosurgical Emergency: Case Report and Review of Literature.

BACKGROUND: Symptomatic slit ventricle is one of the most challenging complications of shunt surgery in children. Clinical signs and symptoms may appear with a wide range of intracranial pressure (ICP) values. We report the case of a 10-year-old girl, who did not present the classic clinical features of extremely elevated ICP, which was proven by multiple invasive ICP recordings, performed during shunt revisions. CASE DESCRIPTION: At the age of 6 months, the patient presented squeal for many hours, accompanied with sunset eyes, bulging anterior fontanel, and dilated ventricles of all 4 ventricles on computed tomography scan. Acute ventriculoperitoneal shunt insertion was performed with adjustable valve. During the following 9 years, she was regularly seen and medically treated for intermittent headache, with nausea and vomiting. From 9 years of age, she was hospitalized for severe (10/10 on the visual analog scale), unbearable headache, agitation, and screaming on multiple occasions. Altogether, we had to revise the shunt system 5 times throughout 1 year. Radiologic imaging always showed narrow ventricles. Ophthalmologic examination of the fundus never revealed signs of raised ICP. Perioperative monitoring of the ICP with intraparenchymal sensor showed unexpected high values of 40-45 mm Hg. However, repetitive shunt revisions were successful only temporarily because the symptoms always returned. Only bilateral shunting of the ventricular system was able to eliminate the symptoms permanently. CONCLUSIONS: Images of slit ventricle can be associated either with low or extremely high ICP needing urgent surgical consideration, including ICP monitoring. Bilateral shunt insertion can be effective treatment for slit ventricle syndrome.

Slit Ventricle Syndrome Leads to 10-Year History of Repetitive Transient Central Herniation Masquerading as Seizures: Hydrocephalus Case Report.

BACKGROUND: Slit-ventricle syndrome (SVS) is a recognized complication of ventricular shunt malfunction, resulting in cyclical symptoms without ventricular dilatation. We present a case of SVS with transient, repetitive, and progressive signs of brainstem herniation evidenced by pupillary dilatation, posturing, and unresponsiveness, with diffuse voltage attenuation on electroencephalogram (EEG). CASE DESCRIPTION: A 32-year-old female presented with a history of hydrocephalus and ventriculoperitoneal shunt placement at 9 months of age. She began experiencing significant headaches in college, later accompanied by stereotypical 5- to 25-minute episodes of unresponsiveness, posturing and pupillary dilatation, and failing anticonvulsant therapy. No neurosurgical evaluation was sought because of small ventricles on brain imaging. Episodes became progressively more frequent over a 10-year period, eventually occurring daily. On presentation, 5 clinical events were captured on EEG over 12 hours of monitoring. With each episode, she became unresponsive and hypertensive, with fixed, dilated pupils and flexor posturing. Between events, she was awake and alert, without confusion or postictal state. She had papilledema and limited extraocular movements, with normal pupils and vital signs. Computed tomography scanning showed small ventricles. A shunt tap revealed no flow. With each episode onset, an EEG revealed an abrupt background rhythm slowing to 2-3 Hz delta range without epileptiform discharges. Between events, EEGs displayed normal waveform activity. Emergent ventriculoperitoneal shunt revision resulted in no further episodes in a 4-year follow-up period. CONCLUSIONS: SVS can lead to severe intermittent brainstem herniation syndrome in the setting of shunt malfunction. Seizure diagnosis should be reserved for cases with proven functional shunt and EEG confirmation of epileptiform activity.

The Total Calvarial Transsutural Distraction Osteogenesis for 26 Children with Slit Ventricle, Craniosynostosis, or Microcephaly After Shunt Operation.

OBJECTIVE: Among shunt complications, the postshunt slit ventricle (PSSV) and the postshunt craniosynostosis (PSCS) may be managed by shunt valve upgrade and/or cranial expansion surgery. Here, we analyzed 26 children with PSSV, PSCS, or microcephaly who received simple generalized cranial expansion (ie, total calvarial transsutural distraction osteogenesis [TC-TSuDO]). METHODS: Among 254 children with shunt surgery, 26 children received TC-TSuDO. These 26 children included 14 with PSSV, 4 with PSCS, and 8 with both PSSV and PSCS. The mean age of patients who underwent shunt procedures was 8.2 ± 10.6 months, and the mean time interval from shunt surgery to TC-TSuDO was 26.8 ± 29.5 months. The mean age of children at the time of TC-TSuDO was 33.3 ± 30.2 months. We analyzed head circumferences, lumbar puncture pressures, development status, operative factors, and postoperative complications. RESULTS: The mean preoperative head circumference was -2.1 ± 1.9, which increased to -1.4 ± 2.1 (P < 0.001) postsurgically. The mean preoperative lumbar puncture pressure was 26.2 ± 10.7 cm H2O which decreased to 11.9 ± 3.5 cm H2O (P < 0.001) after surgery. The mean operation time was 138 ± 66 minutes. The mean intensive care unit stay was 0.27 ± 0.53 days. There were no mortalities but 2 patients suffered from distractor malfunction and 1 patient showed wound discharge. CONCLUSIONS: We suggest that postshunt complications such as PSSV or PSCS, especially those that are accompanied by increased intracranial pressure or postshunt microcephaly, may be managed for patients with TC-TSuDO, which has been shown to be safe, simple, and effective.

Shunt Testing In Vivo: Observational Study of Problems with Ventricular Catheter.

Most shunt obstructions happen at the inlet of the ventricular catheter. Three hundred six infusion studies from 2007 to 2011 were classified as having a typical pattern of either proximal occlusion or patency. We describe different patterns of shunt ventricular obstruction.Solid block: Cerebrospinal fluid (CSF) aspiration was impossible. Baseline pressure was without pulse waveform (respiratory waveform may be visible). A quick increase of pressure to a level compatible with the shunt's setting was recorded in response to infusion. Distal occlusion of the shunt via transcutaneous compression resulted in a rapid increase in pressure to levels above 50 mmHg. This pattern was attributed to a solid ventricular block.Slit ventricles: At baseline, a pattern similar to that of the solid block was observed. After compression, the pressure increases, the pulse waveform appears, and the intracranial pressure is often stabilized at 25-40 mmHg. It is probable that previously slit ventricles were opened during the test.Partial block: In a partial block of the ventricular catheter by an in-growing choroid plexus, the pulse waveform at baseline was observed and CSF aspiration was possible. During infusion, the pressure increased, but the pulse amplitude disappeared. During the increase in the pressure in the shunt prechamber, the connection with the ventricles is disturbed by repositioning of the plexus.Infusion study via the shunt prechamber is able to visualize ventricular obstruction of the hydrocephalus shunt.

Shunt dependency syndrome after cystoperitoneal shunting of arachnoid cysts.

PURPOSE: The goal of this study was to investigate the mechanisms, diagnosis, and treatment of shunt dependency syndrome in patients with temporal lobe arachnoid cysts who were initially treated with cystoperitoneal (CP) shunting. METHODS: Thirteen patients with temporal lobe arachnoid cysts who had initially been treated with CP shunt placement and had developed shunt dependency syndrome were treated by the senior author at Tiantan Hospital between April 2010 and January 2012. The clinical manifestations, neuroimaging findings, intracranial pressure (ICP) data, treatment methods, and therapeutic results were reviewed retrospectively. RESULTS: The study included ten males and three females. The mean age at the time of development of shunt dependency syndrome was 12.3 years (range 5.5-24 years). In most patients, neuroimaging findings showed a collapsed cyst (the cyst appeared almost unchanged in only one patient) and normal or small ventricles (only one patient had enlarged ventricles). Three patients underwent simple replacement of the shunt, four underwent ventriculoperitoneal shunt placement, and the other six underwent lumboperitoneal shunt placement. All patients experienced resolution of their symptoms postoperatively. The mean duration of follow-up was 20 months. CONCLUSIONS: Shunt dependency syndrome is a rare but serious complication of shunting an arachnoid cyst. This condition is similar to the slit ventricle syndrome, but also has some differences. ICP monitoring may confirm the diagnosis when there are no significant radiological findings. Achievement of a shunt-free state might be the ultimate goal for all shunted patients.

ETV as a last resort.

INTRODUCTION: The field of neuroendoscopy is rapidly expanding with increasing indications for endoscopic third ventriculostomy (ETV). DISCUSSION: As a treatment for hydrocephalus, ETV has the advantage of providing a more physiological cerebrospinal fluid diversion without shunt hardware which reduces the risk of recurrent infection and malfunction. The success rate of ETV has been increasing with decreasing morbidity and mortality. CONCLUSION: Originally, ETV was indicated for cases of obstructive hydrocephalus, however the indications are expanding. To highlight this, we present a small series of cases were ETV is not traditionally indicated and was a treatment of last choice.

Endoscopic management of complex hydrocephalus.

BACKGROUND: The management of complex hydrocephalus is challenging. There is no consensus of the best treatment for isolated fourth ventricles, arachnoid cysts, and multiloculated hydrocephalus, although the avoidance of multiple shunts is desirable. We reviewed our experience with the use of endoscopic techniques to simplify complex multicompartmental hydrocephalus to determine its efficacy and safety. METHODS: We performed a retrospective study of 114 consecutive adults and children undergoing endoscopic management of hydrocephalus by a single surgeon. The type of hydrocephalus and endoscopic procedure performed were recorded. The management algorithm for simplification of complex hydrocephalus is reviewed. All patients were followed up in the office or by telephone. RESULTS: A total of 143 endoscopic procedures were performed on 114 patients with a mean age of 4.4 years (4 weeks to 32 years). The endoscopic procedures performed included cyst fenestration, septum pellucidotomy, endoscopic third ventriculostomy, aqueductal plasty with and without stent, and removal of cystercicotic cysts. Twenty-two (19.3%) patients had planned staged endoscopic procedures. Mean follow up was 65 months (range 33-122 months). Eighty-two (72%) patients were reduced to 1 shunt, shunt independence was achieved in 32 (28%) patients, and only 8 (11%) patients required shunt revision in the follow-up period. CONCLUSIONS: Endoscopic simplification of complex hydrocephalus enables dependence on only a single shunt in the majority of patients, and a significant proportion achieve shunt independence. Endoscopic management should be considered before the placement of a second shunt, and some cases require staged endoscopic procedures to adequately communicate multicompartmental hydrocephalus.

Slit ventricle syndrome: a case report of intermittent intracranial hypertension.

Slit ventricle syndrome is a rare condition whereby brain compliance is reduced and can be associated with intermittent intracranial hypertension. A 19-year-old male with a ventriculoperitoneal shunt for congenital hydrocephalus presented with a 1-day history of headache and drowsiness-symptoms from which he suffered in many recurrent episodes over the past 5 years. The improvement of headaches without surgical intervention led to the diagnosis of migraine. During this hospitalization, episodes of intermittent intracranial hypertension were documented along with the remission and relapse of the symptoms. While the patient's intracranial pressure was within normal range in over 90% of his monitoring period, which postponed shunting, replacement of his ventriculoperitoneal shut eventually resolved his symptoms. Slit ventricle syndrome with reduced ventricular compliances should be considered in patients with clinical evidence of intermittent intracranial hypertension and small ventricular size. The authors advocate shunt replacement as an appropriate treatment for this condition.

Development of intracranial hypertension after surgical management of intracranial arachnoid cyst: report of three cases and review of the literature.

OBJECTIVE: To describe three cases of delayed development of intracranial hypertension (IH) after surgical treatment of intracranial arachnoid cyst, including the pathogenesis of IH and a review of the literature. METHODS: A retrospective and prospective analysis of three male patients (two 18 years old and one 45 years old) was performed. All patients underwent surgical intervention for symptomatic intracranial arachnoid cyst in the form of fenestration of the cyst and a cystoperitoneal shunt. RESULTS: All three patients presented at a later stage with new-onset headaches after the initial management of arachnoid cyst. Magnetic resonance imaging and magnetic resonance venography ruled out any intracranial vascular pathology. Lumbar puncture and intracranial pressure monitoring showed increased intracranial pressure suggestive of idiopathic IH. To manage IH, intracranial pressure monitoring, cystoperitoneal shunt, ventriculoperitoneal shunt, and lumboperitoneal shunt were performed. CONCLUSIONS: The pathogenesis of delayed development of IH in this clinical setting is not clearly elucidated. When intracranial arachnoid cysts are treated, the possibility of future development of IH should be borne in mind. Delayed presentation with headaches in patients after treatment of intracranial arachnoid cysts should raise the possibility of IH.