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Jonathan Roth and Shlomi Constantini

OBJECT

Tumors leading to occlusion of the sylvian aqueduct include those of pineal, thalamic, and tectal origins. These tumors cause obstructive hydrocephalus and thus necessitate a CSF diversion procedure such as an endoscopic third ventriculostomy (ETV), often coupled with an endoscopic biopsy (EBX). Lesions located posterior to the massa intermedia pose a technical challenge, as the use of a rigid endoscope for performing both an ETV and EBX is limited. The authors describe their experience using a combined rigid and flexible endoscopic procedure through a single bur hole for both procedures in patients with posterior third ventricular tumors.

METHODS

Since January 2012, patients with posterior third ventricular tumors causing hydrocephalus underwent dual ETV and EBX procedures using the combined rigid-flexible endoscopic technique. Following institutional review board approval, data from clinical, radiological, surgical, and pathological records were retrospectively collected.

RESULTS

Six patients 3.5–53 years of age were included. Lesion locations included pineal (n = 3), fourth ventricle (n = 1), aqueduct (n = 1), and tectum (n = 1). The ETV and EBX were successful in all cases. Pathologies included pilocytic astrocytoma, pineoblastoma, ependymoma Grade II, germinoma, low-grade glioneural tumor, and atypical choroid plexus papilloma. One patient experienced an immediate postoperative intraventricular hemorrhage necessitating evacuation of the clots and resection of the tumor, eventually leading to the patient's death.

CONCLUSIONS

The authors recommend using a combined rigid-flexible endoscope for endoscopic third ventriculostomy and biopsy to approach posterior third ventricular tumors (behind the massa intermedia). This technique overcomes the limitations of using a rigid endoscope by reaching 2 distant regions.

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Gyang Markus Bot, Shlomi Constantini, and Jonathan Roth

Cavum septum pellucidum (CSP) cysts are relatively rare. The most common presenting symptom is headache, which is thought to be secondary to elevated intracranial pressure. Many CSP cysts are treated surgically; conservative treatment is seldom recommended. The authors describe 3 cases of pediatric CSP cysts that were managed without surgery.

The patients ranged in age from 5 months to 8 years old. Two presented with headaches, which were associated with mild ventricular enlargement in 1 case. Over the course of 5–15 months, 2 cysts became markedly reduced in size, and in one of these 2 cases a substantial reduction in ventricle size was also observed. At last follow-up, all 3 children were asymptomatic.

The authors note that CSP cysts are often associated with headaches. In the absence of hydrocephalus, they recommend conservative management with clinical and radiological follow-up.

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Harold L. Rekate

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Leeat Granek, Shahar Shapira, Shlomi Constantini, and Jonathan Roth

OBJECTIVE

The objective of this study was to explore approaches to intraoperative decision-making in pediatric neurosurgeons when they encounter unexpected events, uncertainties, or complications while operating on children.

METHODS

Twenty-six pediatric neurosurgeons from 12 countries around the world were interviewed using a semistructured interview guide. The grounded theory method of data collection and analysis was used. Analysis involved line-by-line coding and was inductive, with codes and categories emerging from participants’ narratives.

RESULTS

When asked to discuss the strategies they used to make intraoperative decisions, neurosurgeons reported three distinct approaches that formed a philosophy of practice. This included the theme of professional practice—with the subthemes of preparing for uncertainty, doing no harm, being creative and adaptive, being systematic, and working on teams. The second theme pertained to patient and caregiver practices—with the subthemes of shared decision-making and seeing the whole patient. The third theme involved surgeon practice—with the subthemes of cultivating self-awareness and learning from experience.

CONCLUSIONS

Pediatric neurosurgeons have a structured, diverse, and well-thought-out analytical philosophy and practice regarding intraoperative decision-making that encompasses a range of approaches including the following: doing no harm, cultivating self-awareness, and seeing the whole patient; and concrete practices such as preparing in advance for uncertainty, working on teams, and learning from experience. These philosophies and practices can be structured and codified in order to teach residents how to develop intraoperative judgment techniques.

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Jonathan Roth, Ami Mayo, Hanoch Elran, Nissim Razon, and Yoram Kluger

Object. Metallic particles contained in antihuman bombs increase the number of fatalities. The ballistics of these particles depends on the explosive that is used, the distance from the explosion, the shape of the particle projected, and the biomechanics of the injured tissue. The authors present their experience with penetrating spherical bolt injuries to the brain.

Methods. The authors retrospectively reviewed clinical and radiological data obtained in eight patients with penetrating spherical bolt injuries to the cranium: four had Glasgow Coma Scale (GCS) scores less than 8 (three died, one from an unrelated injury) and four had a GCS score of 15 (all survived). Two of the latter patients suffered unique anatomical injuries attributed to the distinctive ballistics of spherical bolts: in one patient the bolt penetrated the cavernous sinus causing minimal cranial nerve injury, and in the other patient the bolt lodged in the fourth ventricle causing acute hydrocephalus without other neurological deficits.

Conclusions. Penetrating spherical bolts to the brain may be lethal. Nevertheless, they have unique ballistics that cause highly delineated anatomical damage and minor neurological deficits.

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Jonathan Roth, Marian M. Bercu, and Shlomi Constantini

Hypothalamic hamartomas (HHs) are typically located within the vicinity of the third ventricle. They can be attached to the walls of the third ventricle, within the interpeduncular cistern (third ventricle floor), and/or attached to the mammillary bodies and hypothalamus. Depending on their location, resection is performed either through the third ventricle, approaching from above, or via a frontotemporal craniotomy (pterional or frontoorbital), approaching from below. “Above” approaches typically include the transcallosal–anterior interforniceal approach, and recently, purely endoscopic approaches performed transforaminally.

The authors present a combined open and endoscopic approach for resection of HHs located within the third ventricle. They used this approach in 2 young girls with relatively small lateral and third ventricles. Following an interhemispheric, transcallosal approach and exposure of the right foramen of Monro, an endoscope was inserted through the foramen, which enabled safe resection of the HH.

The main advantage of the combined approach is when the lateral and third ventricles are relatively small, making a purely endoscopic approach more challenging and possibly riskier.

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Joel S. Katz, Sarah S. Milla, Graham C. Wiggins, Orrin Devinsky, Howard L. Weiner, and Jonathan Roth

Object

Tuberous sclerosis complex (TSC) can manifest with 3 principal intracranial pathological entities: cortical tubers, subependymal nodules (SENs), and subependymal giant cell astrocytomas (SEGAs). The authors analyzed the location and growth of intraventricular lesions in a large cohort of patients with TSC.

Methods

After institutional review board protocol approval, the authors retrospectively reviewed brain MRI scans of TSC patients for whom at least 1 electronically stored cranial MRI study was available. Collected data included location, size, and growth over time of all intraventricular lesions.

Results

The authors reviewed 560 scans in 103 patients, who harbored 496 intraventricular lesions. Of the 496 lesions, 157 lesions were located along the caudate-thalamic groove (CTG) in 88 patients. Twenty SEGAs were operated on. The remaining 339 lesions were distributed along the lateral ventricle, always in contact with the course of the caudate nucleus, and were presumed to be SENs. Twenty-two patients with more than 4 years of follow-up had 34 lesions along the CTG, of which 23 were stable in size and 11 grew. All other intraventricular lesions were stable. Seven-Tesla MRI showed the intimate association of SENs and the caudate nucleus in 1 patient.

Conclusions

Intraventricular lesions in TSC patients are located throughout the lateral ventricular wall. Their location exclusively follows the course of the caudate nucleus. Only lesions along the CTG showed the potential to grow, and these were then identified as SEGAs. The remaining lesions were SENs. Understanding why these lesions develop in relation to the caudate nucleus may offer insights into therapy.

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Donato Pacione, Francine Blei, Orrin Devinsky, Howard L. Weiner, and Jonathan Roth

Object

Surgery is increasingly used to treat children with refractory epilepsy. Before surgery, the authors routinely evaluated the coagulation profile to identify coagulation abnormalities not established by personal and family history, physical examination, and routine screening tests.

Methods

Thirty-nine consecutive children undergoing testing prior to epilepsy surgery were prospectively evaluated. The authors evaluated a detailed hematological history and an elaborative hematological panel including complete blood count, hepatic panel, anticoagulant levels, coagulation profile (prothrombin time, partial thromboplastin time, international normalized ratio, fibrinogen, thrombin time, von Willebrand antigen, ristocetin cofactor, factor VIII, and individual factor assays when indicated) and platelet aggregation studies (in the presence of adenosine diphosphate, epinephrine, collagen, and ristocetin). Patient variables included tuberous sclerosis complex (TSC), age at epilepsy onset, age at surgery, seizure frequency, number and type of antiepileptic drugs, recent or present ketogenic diet, and use of selective serotonin reuptake inhibitors.

Results

Ten children (25.6%) had either coagulation or platelet function abnormalities. Abnormal coagulation was identified in 5 children, and abnormal platelet function was discovered in 6. A diagnosis of TSC was associated with a platelet function abnormality (p = 0.012), whereas children without TSC had a higher rate of coagulopathy (p = 0.041). None of the other characteristics reached statistical significance. In 2 patients (5.1%) with TSC and platelet aggregation abnormalities, the authors noted normal standard screening laboratory studies and an uneventful detailed personal and family history. One of these 2 patients developed a significant intraoperative bleeding complication.

Conclusions

A preoperative screening with standard laboratory studies and detailed history may not be adequate to fully examine underlying coagulation abnormalities in children with refractory epilepsy. Platelet aggregation studies should be considered in patients with TSC.

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Mark M. Souweidane, Peter F. Morgenstern, Sungkwon Kang, Apostolos John Tsiouris, and Jonathan Roth

Object

Fenestration of the floor of the third ventricle is vital to the success of endoscopic third ventriculostomy (ETV) in treating patients with noncommunicating hydrocephalus. A generous prepontine interval (PPI) is generally accepted as one anatomical feature that may affect the safety and functionality of ETV. Whether a diminished PPI influences the safety or success of ETV, however, has not been adequately assessed.

Methods

A review was conducted on the last 100 ETV procedures performed by the first author (M.M.S.). From archived preoperative MR imaging studies, the PPI was measured between the dorsum sellae and the basilar artery. For any patient with an interval of ≤1 mm, the technical and functional success of the procedure was recorded. Technical success was defined when a surgically created fenestration was accomplished without patient morbidity. Functional success was defined as the patient not needing any additional CSF diversionary procedure within 3 months after ETV.

Results

In the entire cohort, the PPI ranged from 0 to 9.5 mm (mean 3.2 mm). There were 15 procedures performed in patients with a PPI of ≤1 mm. In all 15 procedures, a fenestration of the tuber cinereum was accomplished without vascular injury or patient morbidity. The ETV was successful in 11 patients (73.3%). All 4 failures occurred in children who had surgery during infancy (mean age 11 months).

Conclusions

Patients with an obliterated or reduced PPI can safely undergo ETV. The functional success rate appears equivalent to historical controls. Most failures in this series may be attributed to other patient characteristics, namely young age at the time of ETV.

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Ori Barzilai, Jonathan Roth, Akiva Korn, and Shlomi Constantini