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Open access

Enlarged tumefactive perivascular, or Virchow-Robin, spaces and hydrocephalus: do we need to treat? Illustrative cases

Belal Neyazi, Vanessa Magdalena Swiatek, Klaus-Peter Stein, Karl Hartmann, Ali Rashidi, Seraphine Zubel, Amir Amini, and I. Erol Sandalcioglu

BACKGROUND

Perivascular spaces (PVSs) are spaces in brain parenchyma filled with interstitial fluid surrounding small cerebral vessels. Massive enlargements of PVSs are referred to as “giant tumefactive perivascular spaces” (GTPVSs), which can be classified into three types depending on their localization. These lesions are rare, predominantly asymptomatic, and often initially misinterpreted as cystic tumor formations. However, there are several reported cases in which GTPVSs have induced neurological symptoms because of their size, mass effect, and location, ultimately leading to obstructive hydrocephalus necessitating neurosurgical intervention. Presented here are three diverse clinical presentations of GTPVS.

OBSERVATIONS

Here, the authors observed an asymptomatic case of type 1 GTPVS and two symptomatic cases of type 3 GTPVS, one causing local mass effect and the other hydrocephalus.

LESSONS

GTPVSs are mostly asymptomatic lesions. Patients without symptoms should be closely monitored, and biopsy is discouraged. Hydrocephalus resulting from GTPVS necessitates surgical intervention. In these cases, third ventriculostomy, shunt implantation, or direct cyst fenestration are surgical options. For patients presenting with symptoms from localized mass effect, a thorough evaluation for potential neurosurgical intervention is imperative. Follow-up in type 3 GTPVS is recommended, particularly in untreated cases. Given the infrequency of GTPVS, definitive guidelines for neurosurgical treatment and subsequent follow-up remain elusive.

Open access

BRAF-mutated suprasellar glioblastoma mimicking craniopharyngioma: illustrative case

Bryan Zheng, Belinda Shao, Jennifer Mingrino, Jonathan Poggi, Richard S Dowd, Douglas C Anthony, John E Donahue, and Curtis E Doberstein

BACKGROUND

Suprasellar masses commonly include craniopharyngiomas and pituitary adenomas. Suprasellar glioblastoma is exceedingly rare with only a few prior case reports in the literature. Suprasellar glioblastoma can mimic craniopharyngioma or other more common suprasellar etiologies preoperatively.

OBSERVATIONS

A 65-year-old male with no significant history presented to the emergency department with a subacute decline in mental status. Work-up revealed a large suprasellar mass with extension to the right inferior medial frontal lobe and right lateral ventricle, associated with significant vasogenic edema. The patient underwent an interhemispheric transcallosal approach subtotal resection of the interventricular portion of the mass. Pathological analysis revealed glioblastoma, MGMT partially methylated, with a BRAF V600E mutation.

LESSONS

Malignant glioblastomas can mimic benign suprasellar masses and should remain on the differential for a diverse set of brain masses with a broad range of radiological and clinical features. For complex cases accessible from the ventricle where the pituitary complex cannot be confidently preserved via a transsphenoidal approach, an interhemispheric approach is also a practical initial surgical option. In addition to providing diagnostic value, molecular profiling may also reveal therapeutically significant gene alterations such as BRAF mutations.

Open access

Approaches to ventriculoperitoneal shunt scalp erosion: countersinking into the calvarium. Illustrative case

Denise Brunozzi, Melissa A LoPresti, Jennifer L McGrath, and Tord D Alden

BACKGROUND

Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors.

OBSERVATIONS

The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing.

LESSONS

Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.

Open access

Can we build better? Challenges with geospatial and financial accessibility in the Caribbean. Illustrative case

Ellianne J dos Santos Rubio, Chrystal Calderon, Annegien Boeykens, and Kee B Park

BACKGROUND

Within the Caribbean, Curaçao provides a neurosurgical hub to other Dutch Caribbean islands. At times, the inefficiency of neurosurgical referrals leads to unsatisfactory patient outcomes in true emergency cases.

OBSERVATIONS

This article reports an illustrative case of a patient in need of emergency neurosurgical care, who was referred to a tertiary health institution in Curaçao. This case highlights the challenges of timely neurosurgical referrals within the Dutch Caribbean.

LESSONS

Highlighting this case may provide a foundation for further discussions that may improve neurosurgical care and access. Limiting long-distance surgical referrals in the acute care setting will aid in saving lives.

Open access

Endoscopic placement of a triventricular stent for complex hydrocephalus and isolated fourth ventricle: illustrative case

V. Jane Horak, Beste Gulsuna, Melissa A LoPresti, and Michael DeCuypere

BACKGROUND

Hydrocephalus is commonly encountered in pediatric neurosurgery. The etiology is diverse, and complexity in management increases in patients with loculated or trapped ventricles. The authors sought to examine a treatment option of endoscopic placement of a triventricular stent in a pediatric patient with complex hydrocephalus and a trapped fourth ventricle.

OBSERVATIONS

In this case, the authors present the treatment of complex hydrocephalus with a trapped fourth ventricle in a pediatric patient using endoscopic placement of a triventricular aqueductal stent. The patient had a complex neurosurgical history, which included over 15 surgeries for shunted hydrocephalus. This case highlights the unique approach used, and the authors discuss surgical nuances of the technique, as well as learning points.

LESSONS

Complex hydrocephalus can be difficult to manage because patients often have multiple catheters, loculated or trapped ventricles, and extensive surgical histories. Endoscopic placement of a triventricular stent can decrease shunt system complexity, restore normal cerebrospinal fluid pathway circulation across the cerebral aqueduct, and promote communication between the ventricles. The authors’ treatment modality resulted in the successful resolution of the trapped fourth ventricle and symptomatic improvement in hydrocephalus.

Open access

Symptomatic obstructive hydrocephalus caused by choroid plexus hyperplasia in a pediatric patient: illustrative case

Ana Sofia Alvarez, John P McGinnis, Rajan Patel, and Howard L Weiner

BACKGROUND

Choroid plexus hyperplasia has been described as a rare cause of communicating hydrocephalus due to cerebrospinal fluid (CSF) overproduction. However, this is the first report of symptomatic obstructive hydrocephalus caused by mechanical obstruction of the aqueduct by a hyperplastic choroid plexus.

OBSERVATIONS

A 4-year-old male presented with headaches and intermittent emesis. Magnetic resonance imaging (MRI) of the brain showed abnormal enlargement of the choroid plexus in the lateral ventricles with extension into the third ventricle, resulting in obstruction of the aqueduct of Sylvius, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy (ETV) was chosen as the surgical treatment. During the procedure, high pressure flow of clear CSF was noted. Normal intraventricular anatomy was identified, and no cyst or tumor was found. The postoperative MRI showed a patent third ventriculostomy, without complication, and a significant decrease in supratentorial ventricular size. The patient was discharged 3 days after surgery with a complete resolution of symptoms.

LESSONS

Choroid plexus hyperplasia has the potential to cause obstructive hydrocephalus, and it can be effectively treated with ETV. Our hypothesis is that the change in pressure caused by the procedure may have led to an uncorking of the aqueduct by the hyperplastic choroid plexus, contributing to the observed improvement.

Open access

Removal of a flanged ventricular catheter: illustrative case

M. Benjamin Larkin, Tyler T. Lazaro, Howard L. Weiner, and William E. Whitehead

BACKGROUND

Flanged ventricular catheters were created in the 1970s to decrease shunt failure by preventing the holes at the catheter tip from contacting the choroid plexus. However, the flanges on the catheter frequently scarred within and tether to the choroid plexus, resulting in higher rates of intraventricular hemorrhage when removed. Today, flanged catheters are rarely encountered.

OBSERVATIONS

The authors describe an illustrative case of a 7-year-old girl recently adopted from another country with a history of myelomeningocele and shunted hydrocephalus. She had been treated with a flanged catheter at birth. She presented with a shunt infection, which required removal of the flanged catheter tethered to the choroid.

LESSONS

The authors illustrate the safe removal of a posterior-entry flanged ventricular catheter tethered to the choroid plexus using monopolar flexible electrocautery. The removal was monitored with a flexible endoscope inserted from an ipsilateral anterior burr hole and was followed by an endoscopic third ventriculostomy.

Open access

Brain metastasis and intracranial leptomeningeal metastasis from malignant peripheral nerve sheath tumors: illustrative cases

Masasuke Ohno, Shoichi Haimoto, Satoshi Tsukushi, Waki Hosoda, Fumiharu Ohka, and Ryuta Saito

BACKGROUND

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft-tissue tumors. Intracranial metastasis from MPNSTs is quite rare.

OBSERVATIONS

The authors report on a 73-year-old male whose MPNST metastasized to the brain and a 32-year-old male with leptomeningeal metastasis from MPNST and review 41 cases of MPNST that developed intracranial metastasis, as reported in the literature.

LESSONS

Brain metastasis and leptomeningeal metastasis of MPNSTs show different clinical courses and require pathology-specific treatment.

Open access

Severe hyperglycorrhachia and status epilepticus after endoscopic aqueductoplasty: illustrative case

Anand A. Dharia, Ahmad Masri, Jay F. Rilinger, and Christian B. Kaufman

BACKGROUND

While hypoglycorrhachia is observed and managed frequently, there are few reports in the literature of clinically significant hyperglycorrhachia after neurosurgery. Understanding the effects and management of severe hyperglycorrhachia is important to the neurosurgeon and neurocritical care teams who care for patients in these rare scenarios.

OBSERVATIONS

The authors present the case of a 3-month-old male with congenital hydrocephalus who faced profound hyperglycorrhachia and status epilepticus after an endoscopic aqueductoplasty using an irrigant composed of lactated Ringer’s solution with dextrose 5% in water. A multidisciplinary approach was developed to monitor and treat the patient’s seizures and cerebrospinal fluid (CSF) osmolytes.

LESSONS

This case provides several learning opportunities for understanding CSF physiology, pathogenesis of common brain injuries related to osmotic shifts and inflammatory states, as well as clinical management of hyperglycorrhachia. It also reiterates the significance of meticulous intraoperative assessment to avoid preventable medical errors.

Open access

Endoscopic third ventriculostomy and biopsy of a tectal lesion using flexible neuroendoscopy and urological cup forceps: illustrative case

Meredith Yang, Daniel Wolfson, Melissa A. LoPresti, Emma Poland, Sandi Lam, and Michael DeCuypere

BACKGROUND

Tectal region tumors often clinically present as obstructive hydrocephalus due to mass effect on the outflow of the third ventricle and cerebral aqueduct. Pathology in this region varies; thus, biopsy can be of great value in the management decision making. Appropriate instrumentation remains an area of interest to further advance flexible neuroendoscopic techniques and applications.

OBSERVATIONS

The authors report an illustrative case using flexible neuroendoscopy through a single burr hole for simultaneous endoscopic third ventriculostomy (ETV) and tectal tumor biopsy using urological cup forceps in a 13-year-old boy who had presented with obstructive hydrocephalus.

LESSONS

The authors demonstrate the feasibility of simultaneous ETV and tectal lesion biopsy via flexible neuroendoscopy to address obstructive hydrocephalus and obtain a tissue biopsy in a single-site procedure. They found that the use of flexible cup forceps designed for uroscopy is an important complement to flexible neuroendoscopy. Given the evolving applications of flexible neuroendoscopy, this has implications for instrumentation adaptation and future design.