Endoscopic intervention for intraventricular neurocysticercal cyst: Challenges and outcome analysis from a single institute experience.

OBJECTIVE: Endoscopic intervention is presently proposed as standard approach for the treatment of Intraventricular neurocysticercal cyst (IVNCC) as it helps to retrieve the cyst as well as CSF diversion. We present our series of 61 patients with IVNCC managed by endoscopic intervention and analyze the outcome. METHOD: A retrospective analysis of 61 patients with IVNCC managed between 1998-2019 at our institute was performed. We reviewed the clinical details of consecutive patients, management, and outcome. RESULTS: There were 61 patients with 34 males and 27 females. The mean age was 25 years. Fourth ventricular location is the most common (n = 34) followed by third ventricle(n = 14) and lateral ventricle (n = 13). Cyst retrieval could be done in 43 cases, while the cyst could not be retrieved in 18 cases due to intraventricular bleed, CSF turbidity, adhesion of cyst wall etc. Along with cyst retrieval, some patients underwent Endoscopic Third ventriculostomy, septostomy, foraminotomy for internal CSF diversion. Seven patients had a preoperative VP shunt surgery. The median follow-up was 12 months. Preoperative shunt (CI:1.33-62, P = 0.02) was associated with failure of cyst retrieval in univariate analysis as well as in multivariate regression analysis (CI: 0.02-0.94, P = 0.04). Two patients underwent shunt surgery at follow-up period due to the failure of endoscopic CSF diversion. CONCLUSION: Endoscopic management of IVNCC is a safe and effective management option, avoiding an indwelling shunt system. Endoscopic third ventriculostomy should be considered for patients with IVNCC and hydrocephalus.

Distinguishing Encephaloclastic Lesions Resulting From Primary or Secondary Pyruvate Dehydrogenase Deficiency From Other Neonatal or Infantile Cavitary Brain Lesions.

The central nervous system (CNS) is a highly complex and energy-dependent organ that is subject to a wide variety of metabolic, hypoxic-ischemic, and infectious insults that result in cystic changes. Diagnosis of metabolic defects causing extensive cystic changes is particularly challenging for the pediatric pathologist, due to the rarity of these conditions. Pyruvate dehydrogenase (PDH) deficiency is one of the most common etiologies of congenital lactic acidosis, caused by mutations in subunits of the large mitochondrial matrix complex, and characterized by periventricular cysts, although few detailed reports focusing on neuropathologic findings exist. In addition, rare defects in other mitochondrial enzymes such as short-chain enoyl-CoA hydratase (SCEH, encoded by ECHS1 gene) can cause secondary PDH deficiency and present with neonatal lactic acidosis, but neuropathological findings have never been reported. Nonmetabolic conditions can also produce CNS cystic lesions, primarily in newborns. The pathologist must therefore distinguish between these etiologically disparate conditions which can produce CNS cavitary lesions. Here, we compare and contrast the gross and microscopic findings of cysts associated with cases of PDH and SCEH deficiencies with other neonatal cystic brain diseases including periventricular leukomalacia, neonatal Alexander disease, Canavan disease, and a case of cysts associated with a vascular abnormality. Our studies show that PDH and SCEH deficiencies are not grossly or histologically distinguishable from each other and both are associated with smooth-walled cysts largely limited to the telencephalic germinal matrix. Both show an absence of prominent hemosiderin deposits, Rosenthal fibers, vacuolization of the white matter, and gliosis or axonal damage in the surrounding parenchyma. These features can help distinguish PDH/SCEH deficiency from other pediatric/neonatal cystic CNS disorders, especially those produced by hypoxic ischemic conditions. Cysts, usually bilateral, confined to the telencephalic germinal matrix should elicit metabolic and genetic testing to appropriately diagnose PDH and SCEH and distinguish them from each other.

Postoperative Cerebellar Cyst with Pseudomeningocele After Tumor Removal at the Craniovertebral Junction.

BACKGROUND: Cerebellar cyst formation after surgery is uncommon, and few cases of this condition have been previously reported. These cases had an intraparenchymal cyst in the cerebellar hemisphere that required surgical fenestration of the cyst. We herein present a rare case of a postoperative cerebellar cyst with pseudomeningocele and magnetic resonance images indicating a fistula between the cyst and pseudomeningocele. CASE DESCRIPTION: A patient presented with an intraparenchymal cyst and surrounding edema in the cerebellar hemisphere that developed after a C1 laminectomy and a small suboccipital craniectomy for the removal of an accessory nerve neurinoma at the craniovertebral junction. Fast imaging employing steady-state acquisition images identified the fistula connecting the cyst and extradural cerebrospinal fluid retention. Conservative management with administration of dexamethasone induced spontaneous regression of the cyst, and no recurrence had occurred by the 1-year follow-up. CONCLUSIONS: Watertight dural closure is important for the prevention of this rare complication after posterior fossa surgery. However, an arachnoid tear on the cerebellar fissure and adjacent dural defect are necessary antecedents for this rare condition. High-resolution fast imaging employing steady-state acquisition images could provide additional information for the etiology of postoperative cerebellar cyst.

Hemorrhagic and Cystic Brain Metastases Are Associated With an Increased Risk of Leptomeningeal Dissemination After Surgical Resection and Adjuvant Stereotactic Radiosurgery.

BACKGROUND: Brain metastases (BM) treated with surgical resection and focal postoperative radiotherapy have been associated with an increased risk of subsequent leptomeningeal dissemination (LMD). BMs with hemorrhagic and/or cystic features contain less solid components and may therefore be at higher risk for tumor spillage during resection. OBJECTIVE: To investigate the association between hemorrhagic and cystic BMs treated with surgical resection and stereotactic radiosurgery and the risk of LMD. METHODS: One hundred thirty-four consecutive patients with a single resected BM treated with adjuvant stereotactic radiosurgery from 2008 to 2016 were identified. Intracranial outcomes including LMD were calculated using the cumulative incidence model with death as a competing risk. Univariable analysis and multivariable analysis were assessed using the Fine & Gray model. Overall survival was analyzed using the Kaplan-Meier method. RESULTS: Median imaging follow-up was 14.2 mo (range 2.5-132 mo). Hemorrhagic and cystic features were present in 46 (34%) and 32 (24%) patients, respectively. The overall 12- and 24-mo cumulative incidence of LMD with death as a competing risk was 11.0 and 22.4%, respectively. On multivariable analysis, hemorrhagic features (hazard ratio [HR] 2.34, P = .015), cystic features (HR 2.34, P = .013), breast histology (HR 3.23, P = .016), and number of brain metastases >1 (HR 2.09, P = .032) were independently associated with increased risk of LMD. CONCLUSION: Hemorrhagic and cystic features were independently associated with increased risk for postoperative LMD. Patients with BMs containing these intralesion features may benefit from alternative treatment strategies to mitigate this risk.

Evolution of cerebellar tonsillar ischemia to cerebellar tonsillar cysts in the Chiari I malformation: radiological, surgical, and histological evidence.

BACKGROUND: Rare reports in the literature have documented the presence of a cyst at the tip of the cerebellar tonsil in patients with Chiari I malformation. The current study aimed to better elucidate these cysts and identify their etiology via a histological and radiologically analysis. PATIENTS AND METHODS: We prospectively reviewed all new Chiari I malformation (CM-I) patients who presented to our clinic during a 2-year period. This was a pediatric cohort made up of 340 children aged 3 to 18 years. Specifically, all patients were screened for ischemic or cystic lesions of the herniated cerebellar tonsils on MRI. RESULTS: Out of 340 patients, 2.9 % were found to have signal changes on MRI consistent with ischemia or cysts in the cerebellar tonsils. Of the 340 patients, 20 % underwent posterior fossa decompression. Of the 67 patients, cerebellar tonsillar ischemia was observed in 10.4 % and cerebellar tonsillar cysts were seen in 6 %. Four of the seven operative patients with cerebellar tonsillar ischemia and concomitant syringomyelia and three of these patients with tonsillar cysts concomitant syringomyelia and cerebellar tonsillar cysts underwent subpial dissection of some cerebellar tonsil to ensure that CSF egress from the fourth ventricle to the cervical subarachnoid space, and this transected tissue was also sent for histological analysis. Three of the four patients found to have intraoperative tonsillar cysts were noted to have tonsillar ischemic changes on preoperative imaging in this same region. For both ischemic and cystic cerebellar tonsils, histologically, the tissue demonstrated loss of Purkinje cells with concomitant Bergmann gliosis. The ischemic and cystic tissues were virtually the same, histologically. CONCLUSIONS: Based on our findings, cerebellar tonsillar ischemia and cysts in patients with CM-I can often be seen radiologically. Histologically, these ischemic and cystic tissues are the same. Moreover, we document patients where ischemic lesions progressed to cysts, radiologically. Taken together, cerebellar tonsillar ischemia and cysts are on a continuum and represent chronic compression of this herniated part of the cerebellum.

Angiomatous lesion and delayed cyst formation after gamma knife surgery for intracranial meningioma: case report and review of literatures.

Gamma Knife has become a major therapeutic method for intracranial meningiomas, vascular malformations and schwannomas with exact effect. In recent years an increasing number of delayed complications after Gamma Knife surgery have been reported, such as secondary tumors, cystic changes or cyst formation. But angiomatous lesion and delayed cyst formation after Gamma Knife for intracranial lesion has rarely been reported. Here we report the first case of angiomatous lesion and delayed cyst formation following Gamma Knife for intracranial meningioma and discuss its pathogenesis.

Levodopa-responsive hemiparkinsonism secondary to cystic expansion from a coiled cerebral aneurysm.

An 80-year-old woman with longstanding hemifacial spasm had a 1 cm × 1.5 cm internal carotid artery terminus aneurysm treated with endovascularly delivered bare metal coils. Follow-up imaging revealed an expansile perianeurysmal cyst that coincided with development of contralateral dopa-responsive hemiparkinsonism. This is the first report of perianeurysmal cyst expansion causing levodopa-responsive hemiparkinsonism.

Cerebral cyst formation following stereotactic ablative irradiation for non-nasopharyngeal head and neck malignancies: imaging findings and relevant dosimetric parameters.

OBJECTIVE: To describe the clinical characteristics, imaging findings and relevant dosimetric parameters of cases presenting with cerebral cyst formation (CCF) after single or oligo-fractionated stereotactic radiotherapy (SRT) for non-nasopharyngeal head and neck malignancies (HNMs). METHODS: We identified four cases with the follow-up duration of 5.7-9.1 years from SRT. The irradiated sites included the middle ear in one case and the ethmoid sinus in three cases, two of the latter possessed brain invasion. The chronological changes in MR images and the dose-volume histogram of the adjacent brain tissue were evaluated. RESULTS: CCF with or without multiple septi presented with a latency of 29-86 months (median, 45.5 months), which was preceded by either non-specific parenchymal enhancement or typical radiation necrosis. In three cases, CCF adjacent to the frontal base resultantly caused mass effect, and two of these three cases required surgical intervention at 38 and 54 months, respectively, after SRT for alleviation of symptoms. The relation of the irradiated brain volumes to the biological equivalent dose based on the linear-quadratic (LQ) and LQ-cubic models was represented as a threshold. CONCLUSION: When contemplating SRT for HNM cases, caution should be exercised to the dose-volume relation-ship of the adjacent brain tissue, especially the frontal base, as well as other critical structures, and long-term vigilant follow-up is also mandatory. ADVANCES IN KNOWLEDGE: CCF can occur as an unusual consequence of late brain injury with variable but mostly long latency following SRT for non-nasopharyngeal HNMs adjacent to the brain, even superficial parts that were previously irradiated via conventional radiotherapy.

[Reversible porencephalic cyst related to shunt dysfunction]. / Cavidad porencefálica reversible secundaria a disfunción valvular.

INTRODUCTION: The surgical treatment of hydrocephalus is one of the most commonly procedures of modern pediatric neurosurgical practice, and cerebrospinal fluid shunts are the main means of treatment of hydrocephalus, being independent of site of obstruction and ventricular shunt complications are frequent, especially in children. The most common complications are shunt obstructions, mechanical disconnections, overdrainage and infection. CASE REPORT: A 7.5-year-old girl with ventriculoperitoneal shunt presented with intermittent headache. A head computed tomography scan revealed a cyst located in right temporal lobe. After ventriculoperitoneal shunt revision, a gradual resolution of the cyst was demonstrated on computed tomography. CONCLUSIONS: Cerebrospinal fluid porencephaly is a rare postoperative complication of a ventricular shunt that results from dysfunction of the distal catheter, especially in children with taut ventricles.

TITLE: Cavidad porencefalica reversible secundaria a disfuncion valvular.Introduccion. El tratamiento quirurgico de la hidrocefalia es uno de los procedimientos quirurgicos mas habituales en la neurocirugia pediatrica, y las derivaciones ventriculoperitoneales constituyen una herramienta fundamental en el tratamiento de la hidrocefalia tanto infantil como del adulto. Las complicaciones de las valvulas son relativamente frecuentes, sobre todo en la poblacion pediatrica, y, entre estas, las mas habituales incluyen: las obstrucciones, las desconexiones, el hiperdrenaje y las infecciones. Caso clinico. Niña de 7,5 años, portadora de una valvula ventriculoperitoneal, que presentaba cefalea intermitente. Un estudio con tomografia computarizada demostro una lesion quistica temporal derecha. Tras la revision valvular, la tomografia computarizada evidencio la reduccion del quiste. Conclusiones. La formacion de una cavidad porencefalica es una complicacion poco frecuente. Se relaciona con problemas en el cateter distal en pacientes con ventriculos dilatados y de las que existen escasas referencias en la bibliografia.

Intraparenchymal cyst development after deep brain stimulator placement.

Following deep brain stimulation (DBS) surgery, a variety of potential mechanical or functional complications ranging from perioperative events to hardware malfunction may occur. We present 2 patients who developed a unique complication of cyst formation at the tip of the DBS electrode in the absence of infection. One patient had a unilateral ventral intermediate lead placement for essential tremor, and the other had bilateral subthalamic nucleus (STN) placement for Parkinson's disease. After a period of symptom control, at 3 and 8 months after surgery, respectively, both patients developed new neurological deficits and were found to have a cyst at the left DBS lead tip. The right lead in the patient with the bilateral STN implant was without issue. Both affected leads were removed and the problematic symptoms regressed quickly over several days, though the lesion effect on the patients' initial tremor symptoms lasted for months. Bacteriological cultures of the removed electrodes and wounds were negative. We report a rare complication of DBS and show that simply removing the involved lead results in cyst resolution.

Sustained improvements in plasma ACTH and clinical status in a patient with Nelson's syndrome treated with pasireotide LAR, a multireceptor somatostatin analog.

CONTEXT: Nelson's syndrome refers to aggressive pituitary corticotroph adenoma growth after bilateral adrenalectomy for treatment of Cushing's disease (CD). Pasireotide, a novel somatostatin analog, has been effective in treating CD. Here, the first case report of a patient with Nelson's syndrome treated with pasireotide is presented. CASE PRESENTATION: A 55-year-old female was diagnosed with CD in 1973 at age 15 years and underwent bilateral adrenalectomy 1 year later. She subsequently developed Nelson's syndrome and underwent multiple surgeries and radiotherapy for adenoma growth. After presentation with ocular pain, third cranial nerve palsy, and a finding of suprasellar tumor enlargement with hemorrhage, she began pasireotide long-acting release 60 mg/28 days im. At baseline, fasting plasma ACTH was 42 710 pg/mL (normal, 5-27 pg/mL), and fasting plasma glucose was 98 mg/dL. After 1 month, ACTH declined to 4272 pg/mL, and it has remained stable over 19 months of follow-up. Hyperpigmentation progressively improved. Magnetic resonance imaging scans show reduction in the suprasellar component. Fasting plasma glucose increased to 124 mg/dL, and the patient underwent diabetes management. EVIDENCE ACQUISITION AND SYNTHESIS: In this clinical case seminar, the current understanding of the treatment of Nelson's syndrome and the use of pasireotide in CD are summarized. CONCLUSION: A case of Nelson's syndrome with clinically significant and dramatic biochemical and clinical responses to pasireotide administration is reported. Hyperglycemia was noted after pasireotide administration. Pasireotide may represent a useful tool in the medical management of Nelson's syndrome. Further study of the potential benefits and risks of pasireotide in this population is necessary.

Endoscopic cyst fenestration in the treatment of uniloculated hydrocephalus in children.

OBJECT: The treatment of uniloculated hydrocephalus is a difficult problem in pediatric neurosurgery. Definitive treatment is surgical, yet the approach remains controversial. This study evaluates the role of endoscopic cyst fenestration (ECF) in the management of this disease. METHODS: Thirty-one pediatric patients with uniloculated hydrocephalus who underwent endoscopic surgery, performed by the author, between May 1999 and December 2010 constitute the patient group for this study. The patients included 17 boys and 14 girls, with ages ranging from 5 months to 5 years (mean 22.9 months). Patients with multiloculated hydrocephalus were not included. The patients' charts were reviewed for demographic data, radiological findings, information regarding morbidity, improvement of hydrocephalus, incidence of recurrence, shunt dependency, and the need for shunt revision. RESULTS: Neuroepithelial cysts were the most common cause (17 cases), followed by postoperative gliosis due to previous shunt infection (9 cases), intraventricular hemorrhage (3 cases), and meningitis (2 cases). Multiplanar MRI was reliable in making the diagnosis and is indicated if CT shows disproportionate hydrocephalus. Surgical treatment included ECF (31 cases), endoscopic revision of malfunctioning preexisting shunts (9 cases), endoscopic third ventriculostomy (4 cases), and placement of a new shunt (3 cases). Endoscopic cyst fenestration was easily performed in all the cases, with devascularization of the cyst wall by coagulation to prevent recurrence. Improvement of hydrocephalus was observed in 26 cases (83.9%). Among the group of patients without prior shunts (22 cases), 3 patients (13.6%) required repeat ECF and 3 patients (13.6%) required placement of a shunt (new shunt placement). In the 9 patients with preexisting shunts, endoscopy reduced the mean rate of shunt revision from 2.7 revisions per year before fenestration to 0.25 per year after fenestration. Four of these 9 patients had multiple shunts, which could be converted to a single shunt; however, repeat ECF was necessary in all 9 patients. With a mean follow-up duration of 4.3 years, none of the patients with a prior shunt was able to become shunt-independent, whereas 86.4% of patients without a prior shunt were able to avoid shunt placement. Endoscopic complications were reversible (unilateral subdural effusion in 5 cases, minor arterial bleeding in 2 cases, CSF leakage in 1 case), and there was no death (0%). CONCLUSIONS: Endoscopic cyst fenestration is recommended in the treatment of uniloculated hydrocephalus because it is effective, simple, minimally invasive, and associated with low morbidity and mortality rates. The fact that all previously shunt-treated patients needed repeat ECF and that none of these patients was able to become shunt-independent makes it clear that uniloculated hydrocephalus due to postoperative gliosis induced by previous shunt infection carries the worst prognosis.

Delayed complications in patients surviving at least 3 years after stereotactic radiosurgery for brain metastases.

PURPOSE: Little is known about delayed complications after stereotactic radiosurgery in long-surviving patients with brain metastases. We studied the actual incidence and predictors of delayed complications. PATIENTS AND METHODS: This was an institutional review board-approved, retrospective cohort study that used our database. Among our consecutive series of 2000 patients with brain metastases who underwent Gamma Knife radiosurgery (GKRS) from 1991-2008, 167 patients (8.4%, 89 women, 78 men, mean age 62 years [range, 19-88 years]) who survived at least 3 years after GKRS were studied. RESULTS: Among the 167 patients, 17 (10.2%, 18 lesions) experienced delayed complications (mass lesions with or without cyst in 8, cyst alone in 8, edema in 2) occurring 24.0-121.0 months (median, 57.5 months) after GKRS. The actuarial incidences of delayed complications estimated by competing risk analysis were 4.2% and 21.2% at the 60th month and 120th month, respectively, after GKRS. Among various pre-GKRS clinical factors, univariate analysis demonstrated tumor volume-related factors: largest tumor volume (hazard ratio [HR], 1.091; 95% confidence interval [CI], 1.018-1.154; P=.0174) and tumor volume≤10 cc vs >10 cc (HR, 4.343; 95% CI, 1.444-12.14; P=.0108) to be the only significant predictors of delayed complications. Univariate analysis revealed no correlations between delayed complications and radiosurgical parameters (ie, radiosurgical doses, conformity and gradient indexes, and brain volumes receiving >5 Gy and >12 Gy). After GKRS, an area of prolonged enhancement at the irradiated lesion was shown to be a possible risk factor for the development of delayed complications (HR, 8.751; 95% CI, 1.785-157.9; P=.0037). Neurosurgical interventions were performed in 13 patients (14 lesions) and mass removal for 6 lesions and Ommaya reservoir placement for the other 8. The results were favorable. CONCLUSIONS: Long-term follow-up is crucial for patients with brain metastases treated with GKRS because the risk of complications long after treatment is not insignificant. However, even when delayed complications occur, favorable outcomes can be expected with timely neurosurgical intervention.

Prevalence of choroid plexus cysts in term and near-term infants with congenital heart disease.

OBJECTIVE: We retrospectively studied term and near-term infants to determine if there is an increased prevalence of choroid plexus cysts associated with congenital heart disease. MATERIALS AND METHODS: We reviewed 231 consecutive cranial ultrasound examinations from January 2005 through December 2008 on infants between 37-42 weeks of gestation (115 infants with and 116 without congenital heart disease). After exclusion for intracranial hemorrhage or anomalies excluding hydronephrosis, studies on 87 infants with and 99 without congenital heart disease were scored by a single pediatric radiologist blinded to clinical data for the presence of choroid plexus cysts. All studies used a multifrequency 6-8-MHz phased-array transducer. Results were analyzed using Pearson's chi-square test. RESULTS: Of 87 infants with congenital heart disease (53/87 [61%] cyanotic, 34/87 [39%] acyanotic), 26% (23/87) had choroid plexus cysts versus 12% (12/99) without heart disease (χ(2) = 4.48, p < 0.05). The rate of choroid plexus cysts in the cyanotic group (13/54 or 24%) and acyanotic group (10/33 or 30%) (χ(2) = 0.050, p > 0.05) were similar. Among 28 patients who underwent renal ultrasound, 50% (10/20) with congenital heart disease had choroid plexus cysts compared with 12.5% (1/8) of those without (χ(2) = 3.36, p < 0.10). CONCLUSION: We documented an increased prevalence of choroid plexus cysts in infants with congenital heart disease but no difference according to type. Our data revealed a possible association of choroid plexus cysts, hydronephrosis, and congenital heart disease. We suggest ultrasound evaluation of the kidneys and heart in infants with choroid plexus cysts.

Endoscopic transsphenoidal, transclival resection of an enterogenous cyst located ventral to the brainstem: case report.

BACKGROUND AND IMPORTANCE: Enterogenous cysts are rare tumors found most commonly in the spine, but they have also been reported intracranially. Cases of enterogenous cysts located within the posterior fossa have traditionally been resected via difficult craniotomies that require prolonged retraction and risk injury to cranial nerves. We describe a method for resection of an enterogenous cyst located anterior to the brainstem via the endoscopic transsphenoidal approach. CLINICAL PRESENTATION: A 37-year-old man was found to have a 2-cm mass anterior to the brainstem during routine screening after a trauma. The mass was located within the prepontine cistern, enhanced with gadolinium contrast, and showed no restrictive diffusion. This lesion was most consistent with an enterogenous cyst. A minimally invasive endoscopic endonasal transsphenoidal transclival approach was performed for gross total resection of the tumor. CONCLUSION: We discuss the endoscopic transsphenoidal approach used for the resection of an enterogenous cyst in the posterior fossa anterior to the brainstem. The transsphenoidal approach provides direct access to lesions in this location using a minimally invasive technique while avoiding excessive brain retraction or injury to cranial nerves. In addition, we provide an updated review of the literature for enterogenous cysts located within the posterior fossa.