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Ashish H. Shah, Michael E. Ivan and Ricardo J. Komotar

Meningiomas that partially or completely occlude the superior sagittal sinus may create a pseudotumor-like syndrome in certain patients. These patients may have impaired CSF absorption as a result of higher proximal venous pressure. Higher pressures after resection may encumber adequate wound healing and worsen symptoms. Here, the authors present a small series of patients with meningiomas involving the posterior third of the superior sagittal sinus, with documented high intracranial pressure prior to surgery. This paper aims to address the proposed etiology of high intracranial pressure in these patients and its associated complications, including CSF leak, wound dehiscence, pressure-related headaches, and visual complaints. In this paper, the authors propose a management plan to avoid wound complications and pseudotumor-related complications. When considering surgical intervention for patients with compromise of the posterior third of the superior sagittal sinus, careful attention must be paid to addressing potentially elevated intracranial pressure perioperatively.

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Ashish H. Shah, Anthony C. Wang and Jacques J. Morcos

Superficial arteriovenous malformations (AVMs) with favorable Spetzler-Martin grading are amenable to primary surgical resection. Careful preoperative workup including preoperative angiograms is essential to identify feeding artery aneurysms and deep venous drainage. The authors present a 37-year-old female who presented with a Spetzler-Martin Grade II right parietal superficial AVM with a 5-mm feeding artery aneurysm from the posterior cerebral artery. Given the risk of hemorrhage, the AVM was resected completely without any complications. On subsequent postoperative angiograms, the feeding artery aneurysm diminished in caliber. Feeding artery aneurysms may regress spontaneously after resection of an AVM due to flow-related changes.

The video can be found here: https://youtu.be/PpwODc9iI3g.

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Giuseppe Lanzino

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Ashish H. Shah, Angela M. Richardson, Joshua D. Burks and Ricardo J. Komotar

Recurrent treatment-refractory brain metastases can be treated with modern adjuvant therapies such as laser interstitial thermal therapy (LITT). Since previously radiated lesions may be indolent (treatment effect) or recurrent tumor, histological confirmation may be helpful. The authors present the utility of contemporaneous biopsy and LITT using intraoperative O-arm navigation in a patient who presented with multiple refractory metastases. The authors demonstrate the utility of O-arm navigation to confirm intraoperative biopsy and LITT placement. Concurrent stereotactic biopsy and LITT may be a safe and efficacious method for both the diagnosis and treatment of deep lesions that are unamenable to standard adjuvant treatment modalities.

The video can be found here: https://youtu.be/SUY-qiahMyo.

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Ashish H. Shah, Ignacio Jusué-Torres, Michael E. Ivan, Ricardo J. Komotar and Noriyuki Kasahara

In the late 19th century, Dr. William B. Coley introduced the theory that infections may aid in the treatment of malignancy. With the creation of Coley’s toxin, reports of remission during viral illnesses for systemic malignancies soon emerged. A few decades after this initial discovery, Austrian physicians performed intravascular injections of Clostridium to induce oncolysis in patients with glioblastoma. Since then, suggestions between improved survival and infectious processes have been reported in several patients with glioma, which ultimately marshaled the infamous use of intracerebral Enterobacter. These early observations of tumor regression and concomitant infection piloted a burgeoning field focusing on the use of pathogens in molecular oncology.

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Michael Ragheb, Ashish H. Shah, Sarah Jernigan, Tulay Koru-Sengul and John Ragheb

OBJECTIVE

Hydrocephalus is recognized as a common disabling pediatric disease afflicting infants and children disproportionately in the developing world, where access to neurosurgical care is limited and risk of perinatal infection is high. This surgical case series describes the Project Medishare Hydrocephalus Specialty Surgery (PMHSS) program experience treating hydrocephalus in Haiti between 2008 and 2015.

METHODS

The authors conducted a retrospective review of all cases involving children treated for hydrocephalus within the PMHSS program in Port-au-Prince, Haiti, from 2008 through 2015. All relevant epidemiological information of children treated were prospectively collected including relevant demographics, birth history, hydrocephalus etiology, head circumference, and operative notes. All appropriate associations and statistical tests were performed using univariate and multivariate logistic regression analyses.

RESULTS

Among the 401 children treated within PMHSS, postinfectious hydrocephalus (PIH) accounted for 39.4% (n = 158) of cases based on clinical, radiographic, and endoscopic findings. The majority of children with hydrocephalus in Haiti were male (54.8%, n = 197), born in the rainy season (59.7%, n = 233), and born in a coastal/inland location (43.3%, n = 61). The most common surgical intervention was endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) (45.7%, n = 175). Multivariate logistic regression analysis yielded coastal birth location (OR 3.76, 95% CI 1.16–12.18) as a statistically significant predictor of PIH. Increasing head circumference (adjusted OR 1.06, 95% CI 0.99–1.13) demonstrated a slight trend toward significance with the incidence of PIH.

CONCLUSIONS

This information will provide the foundation for future clinical and public health studies to better understand hydrocephalus in Haiti. The 39.4% prevalence of PIH falls within observed rates in Africa as does the apparently higher prevalence for those born during the rainy season. Although PIH was the most frequent etiology seen in almost all birth locations, the potential relationship with geography noted in this series will be the focus of further research in an effort to understand the link between climate and PIH in Haiti. The ultimate goal will be to develop an appropriate public health strategy to reduce the burden of PIH on the children of Haiti.

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Manish Kuchakulla, Ashish H. Shah, Valerie Armstrong, Sarah Jernigan, Sanjiv Bhatia and Toba N. Niazi

OBJECTIVE

Carotid body tumors (CBTs), extraadrenal paragangliomas, are extremely rare neoplasms in children that often require multimodal surgical treatment, including preoperative anesthesia workup, embolization, and resection. With only a few cases reported in the pediatric literature, treatment paradigms and surgical morbidity are loosely defined, especially when carotid artery infiltration is noted. Here, the authors report two cases of pediatric CBT and provide the results of a systematic review of the literature.

METHODS

The study was divided into two sections. First, the authors conducted a retrospective review of our series of pediatric CBT patients and screened for patients with evidence of a CBT over the last 10 years (2007–2017) at a single tertiary referral pediatric hospital. Second, they conducted a systematic review, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, of all reported cases of pediatric CBTs to determine the characteristics (tumor size, vascularity, symptomatology), treatment paradigms, and complications.

RESULTS

In the systematic review (n = 21 patients [includes 19 cases found in the literature and 2 from the authors’ series]), the mean age at diagnosis was 11.8 years. The most common presenting symptoms were palpable neck mass (62%), cranial nerve palsies (33%), cough or dysphagia (14%), and neck pain (19%). Metastasis occurred only in 5% of patients, and 19% of cases were recurrent lesions. Only 10% of patients presented with elevated catecholamines and associated sympathetic involvement. Preoperative embolization was utilized in 24% of patients (external carotid artery in 4 and external carotid artery and vertebral artery in 1). Cranial nerve palsies (cranial nerve VII [n = 1], IX [n = 1], X [n = 4], XI [n = 1], and XII [n = 3]) were the most common cause of surgical morbidity (33% of cases). The patients in the authors’ illustrative cases underwent preoperative embolization and balloon test occlusion followed by resection, and both patients suffered from transient Horner’s syndrome after embolization.

CONCLUSIONS

Surgical management of CBTs requires an extensive preoperative workup, anesthesia, and multimodal surgical management. Due to a potentially high rate of surgical morbidity and vascularity, balloon test occlusion with embolization may be necessary in select patients prior to resection. Careful thorough preoperative counseling is vital to preparing families for the intensive management of these children.

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Noam Alperin, James Ryan Loftus, Ahmet M. Bagci, Sang H. Lee, Carlos J. Oliu, Ashish H. Shah and Barth A. Green

OBJECTIVE

This study identifies quantitative imaging-based measures in patients with Chiari malformation Type I (CM-I) that are associated with positive outcomes after suboccipital decompression with duraplasty.

METHODS

Fifteen patients in whom CM-I was newly diagnosed underwent MRI preoperatively and 3 months postoperatively. More than 20 previously described morphological and physiological parameters were derived to assess quantitatively the impact of surgery. Postsurgical clinical outcomes were assessed in 2 ways, based on resolution of the patient's chief complaint and using a modified Chicago Chiari Outcome Scale (CCOS). Statistical analyses were performed to identify measures that were different between the unfavorable- and favorable-outcome cohorts. Multivariate analysis was used to identify the strongest predictors of outcome.

RESULTS

The strongest physiological parameter predictive of outcome was the preoperative maximal cord displacement in the upper cervical region during the cardiac cycle, which was significantly larger in the favorable-outcome subcohorts for both outcome types (p < 0.05). Several hydrodynamic measures revealed significantly larger preoperative-to-postoperative changes in the favorable-outcome subcohort. Predictor sets for the chief-complaint classification included the cord displacement, percent venous drainage through the jugular veins, and normalized cerebral blood flow with 93.3% accuracy. Maximal cord displacement combined with intracranial volume change predicted outcome based on the modified CCOS classification with similar accuracy.

CONCLUSIONS

Tested physiological measures were stronger predictors of outcome than the morphological measures in patients with CM-I. Maximal cord displacement and intracranial volume change during the cardiac cycle together with a measure that reflects the cerebral venous drainage pathway emerged as likely predictors of decompression outcome in patients with CM-I.

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Ashish H. Shah, Karthik Madhavan, Deborah Heros, Daniel M. S. Raper, J. Bryan Iorgulescu, Brian E. Lally and Ricardo J. Komotar

Object

The discovery of incidental low-grade gliomas (LGGs) on MR imaging is rare, and currently there is no existing protocol for management of these lesions. Various studies have approached the dilemma of managing patients with incidental LGGs. While some advocate surgery and radiotherapy, others reserve surgery until there is radiological evidence of growth. For neurosurgeons and radiologists, determining the course of action after routine brain imaging poses not only a medical but also an ethical dilemma. The authors conducted a systematic review of case reports and case series in hopes of enhancing the current understanding of the management options for these rare lesions.

Methods

A PubMed search was performed to include all relevant MR imaging studies in which management of suspected incidental LGG was reported. Comparisons were made between the surgical treatment arm and the active surveillance arm in terms of outcome, mode of discovery, reasons for treatment, and histology.

Results

Nine studies with 72 patients were included in this study (56 in the surgical arm and 16 in the active surveillance arm). Within the surgical arm, 49% remained deficit free after treatment, 25% showed evidence of tumor progression, 13% underwent a second treatment, and 7% died. The active surveillance group resulted in no unanticipated adverse events, with serial imaging revealing no tumor growth in all cases. Lesion regression was reported in 31% of this group. The surgical arm's mortality rate was 7% compared with 0% in the active surveillance arm.

Conclusions

Treatment decisions for incidental LGG should be individualized based on presenting symptoms and radiological evidence of growth. The asymptomatic patient may be monitored safely with serial MR imaging and occasionally PET scanning before treatment is initiated. In patients presenting with nonspecific symptoms or concurrent symptomatic lesions, treatment may be initiated earlier to reduce potential morbidity. All treatment decisions must be tempered by patient factors and expectations of anticipated benefit.

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Ashish H. Shah, George M. Ibrahim, Jun Sasaki, John Ragheb, Sanjiv Bhatia and Toba N. Niazi

OBJECTIVE

Although endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has gained increasing prominence in the management of hydrocephalus caused by intraventricular hemorrhage of prematurity, the rates of long-term shunt independence remain low. Furthermore, limited evidence is available to identify infants who might benefit from the procedure. The authors tested the hypothesis that elevated venous pressure that results from comorbid cardiac disease might predispose patients to ETV/CPC failure and shunt dependence.

METHODS

A retrospective analysis was performed on a consecutive series of 48 infants with hydrocephalus who underwent ETV/CPC and also underwent preoperative echocardiography between 2007 and 2014. Comorbid cardiac abnormalities that are known to result in elevated right heart pressure were reviewed. Associations between ETV/CPC success and the presence of pulmonary hypertension, right ventricular hypertrophy, left-to-right shunting, ventricular septal defect, or patent ductus arteriosus were determined using multivariate logistic regression analysis.

RESULTS

Of the 48 children who met the inclusion criteria, ETV/CPC failed in 31 (65%). In univariate analysis, no single echocardiogram abnormality was associated with shunt failure, but the presence of 2 or more concurrent echocardiogram abnormalities was associated with ETV/CPC failure (17 [85%] of 20 vs 14 [50%] of 28, respectively; p = 0.018). In multivariate logistic regression analysis, when the authors adjusted for the child’s ETV success score, the presence of 2 abnormalities remained independently associated with poor outcome (2 or more echocardiogram abnormalities, OR 0.13, 95% CI 0.01–0.7, p = 0.032; ETV success score, OR 1.1, 95% CI 1–1.2, p = 0.05).

CONCLUSIONS

In this study, cardiac abnormalities were inversely associated with the success of ETV/CPC in infants with hydrocephalus of prematurity. ETV/CPC might not be as efficacious in patients with significant cardiac anomalies. These results provide a basis for future efforts to stratify surgical candidacy for ETV/CPC on the basis of comorbid abnormalities. Proper cardiac physiological pressure monitoring might help elucidate the relationship between cardiac abnormalities and hydrocephalus.