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Alexander G. Weil, John Ragheb, Toba N. Niazi and Sanjiv Bhatia

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Alexander G. Weil, Andrew L. Middleton, Toba N. Niazi, John Ragheb and Sanjiv Bhatia

OBJECT

Operations on tumors of the posteromedial temporal (PMT) region, that is, on those arising from the posterior parahippocampal, fusiform, and lingual gyri, are challenging to perform because of the deep-seated location of these tumors between critical cisternal neurovascular structures and the adjacent temporal and occipital cortexes. Traditional surgical approaches require temporal or occipital transgression, retraction, or venous sacrifice. These approaches may result in unintended complications that should be avoided. To avoid these complications, the supracerebellar-transtentorial (SCTT) approach to this region has been used as an effective alternative treatment in adult patients. The SCTT approach uses a sitting position that offers a direct route to the posterior fusiform and lingual gyri of the temporal lobe. The authors report the feasibility, safety, and efficacy of this approach, using a modified lateral park-bench position in a small cohort of pediatric patients.

METHODS

The authors carried out a retrospective case review of 5 consecutive patients undergoing a paramedian SCTT approach between 2009 and 2014 at the authors' institution.

RESULTS

The SCTT approach in the park-bench position was used in 3 boys and 2 girls with a mean age of 7.8 years (range 13 months to 16 years). All patients presented with a seizure disorder related to a tumor in a PMT region involving the parahippocampal and fusiform gyri of the left (n = 3) or right (n = 2) temporal lobe. No procedure-related complications were observed. Gross-total resection and control of seizures were achieved in all cases. Tumor classes and types included 1 Grade II astrocytoma, 1 pleomorphic xanthoastrocytoma, 1 ganglioglioma, and 2 glioneural tumors. None of the tumors had recurred by the mean follow-up of 22 months (range 1–48 months). Outcomes of epileptic seizures were excellent, with seizure symptoms in all 5 patients scoring in Engel Class IA.

CONCLUSIONS

The SCTT approach represents a viable option when resecting tumors in this region, providing a reasonable working corridor and low morbidity. The authors' experience in a cohort of pediatric patients demonstrates that complete resection of the lesions in this location is feasible and is safe when involving an approach that involves using a park-bench lateral positioning.

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Anna Lo Presti, Alexander G. Weil, Aria Fallah, Eric C. Peterson, Toba N. Niazi and Sanjiv Bhatia

Sickle cell disease (SCD) is an autosomal recessive hematological disorder, characterized by sickling of the red blood cells in response to a hypoxic stress and vaso-occlusive crises. It is associated with moyamoya-like changes on cerebral angiographic imaging in 43% of patients. Cerebral aneurysms, arteriovenous malformations, and dural arteriovenous fistulas (AVFs) have been described in association with SCD and moyamoya disease. However, the description of a pial AVF (pAVF) in a patient with SCD and/or moyamoya formation has not yet been reported. The authors present the case of a 15-year-old boy with SCD-associated moyamoya disease harboring a pAVF who developed a de novo venous aneurysm 8 months after undergoing indirect superficial temporal artery-middle cerebral artery (MCA) bypass that was complicated by bilateral ischemia of the MCA territory. The pAVF was successfully treated with transarterial embolization using Onyx. The authors describe the possible pathophysiological mechanisms and management strategies for this rare occurrence.

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Walter J. Jermakowicz, Alexander G. Weil, Artyom Vlasenko, Sanjiv Bhatia and Toba N. Niazi

Cognard Type V dural arteriovenous fistulas (dAVFs) are a unique type of cranial vascular malformation characterized by congestion of the perimedullary venous system that may lead to devastating spinal cord pathology if left untreated. The authors present the first known case of a pediatric patient diagnosed with a Type V dAVF. A 14-year-old girl presented with a 3-week history of slowly progressive unilateral leg weakness that quickly progressed to bilateral leg paralysis, sphincter dysfunction, and complete sensory loss the day of her presentation. MRI revealed an extensive T2 signal change in the cervical spine and tortuous perimedullary veins along the entire length of the cord. An emergency cranial angiogram showed a Type V dAVF fed by the posterior meningeal artery with drainage into the perimedullary veins of the cervical spine. The fistula was not amenable to embolization because vascular access was difficult; therefore, the patient underwent urgent suboccipital craniotomy and ligation of the arterialized venous drainage from the fistula. The patient’s clinical course immediately reversed; she had a complete recovery over the course of a year, and she remains asymptomatic at the 2-year follow-up. This report adds to a growing body of evidence that describes the diverse and unpredictable nature of Type V dAVFs and highlights the need to obtain a cranial angiogram in pediatric patients with unexplained myelopathy and cervical cord T2 signal change on MRI.

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Han Yan, Taylor J. Abel, Naif M. Alotaibi, Melanie Anderson, Toba N. Niazi, Alexander G. Weil, Aria Fallah, John H. Phillips, Christopher R. Forrest, Abhaya V. Kulkarni, James M. Drake and George M. Ibrahim

OBJECTIVE

In this systematic review and meta-analysis the authors aimed to directly compare open surgical and endoscope-assisted techniques for the treatment of sagittal craniosynostosis, focusing on the outcomes of blood loss, transfusion rate, length of stay, operating time, complication rate, cost, and cosmetic outcome.

METHODS

A literature search was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Relevant articles were identified from 3 electronic databases (MEDLINE, EMBASE, and CENTRAL [Cochrane Central Register of Controlled Trials]) from their inception to August 2017. The quality of methodology and bias risk were assessed using the Effective Public Health Practice Project Quality Assessment Tool for Quantitative Studies. Effect estimates between groups were calculated as standardized mean differences with 95% CIs. Random and fixed effects models were used to estimate the overall effect.

RESULTS

Of 316 screened records, 10 met the inclusion criteria, of which 3 were included in the meta-analysis. These studies reported on 303 patients treated endoscopically and 385 patients treated with open surgery. Endoscopic surgery was associated with lower estimated blood loss (p < 0.001), shorter length of stay (p < 0.001), and shorter operating time (p < 0.001). From the literature review of the 10 studies, transfusion rates for endoscopic procedures were consistently lower, with significant differences in 4 of 6 studies; the cost was lower, with differences ranging from $11,603 to $31,744 in 3 of 3 studies; and the cosmetic outcomes were equivocal (p > 0.05) in 3 of 3 studies. Finally, endoscopic techniques demonstrated complication rates similar to or lower than those of open surgery in 8 of 8 studies.

CONCLUSIONS

Endoscopic procedures are associated with lower estimated blood loss, operating time, and days in hospital. Future long-term prospective registries may establish advantages with respect to complications and cost, with equivalent cosmetic outcomes. Larger studies evaluating patient- or parent-reported satisfaction and optimal timing of intervention as well as heterogeneity in outcomes are indicated.

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Han Yan, Taylor J. Abel, Naif M. Alotaibi, Melanie Anderson, Toba N. Niazi, Alexander G. Weil, Aria Fallah, John H. Phillips, Christopher R. Forrest, Abhaya V. Kulkarni, James M. Drake and George M. Ibrahim

OBJECTIVE

Despite increasing adoption of endoscopic techniques for repair of nonsagittal single-suture craniosynostosis, the efficacy and safety of the procedure relative to established open approaches are unknown. In this systematic review the authors aimed to directly compare open surgical and endoscope-assisted techniques for the treatment of metopic, unilateral coronal, and lambdoid craniosynostosis, with an emphasis on quantitative reported outcomes.

METHODS

A literature search was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Relevant articles were identified from 3 electronic databases (MEDLINE, EMBASE, and CENTRAL [Cochrane Central Register of Controlled Trials]) from their inception to August 2017. The quality of methodology and bias risk were assessed using the Effective Public Health Practice Project (EPHPP) Quality Assessment Tool for Quantitative Studies.

RESULTS

Of 316 screened records, 7 studies were included in a qualitative synthesis of the evidence, of which none were eligible for meta-analysis. These reported on 111 unique patients with metopic, 65 with unilateral coronal, and 12 with lambdoid craniosynostosis. For all suture types, 100 (53%) children underwent endoscope-assisted craniosynostosis surgery and 32 (47%) patients underwent open repair. These studies all suggest that blood loss, transfusion rate, operating time, and length of hospital stay were superior for endoscopically treated children. Although potentially comparable or better cosmetic outcomes are reported, the paucity of evidence and considerable variability in outcomes preclude meaningful conclusions.

CONCLUSIONS

Limited data comparing open and endoscopic treatments for metopic, unilateral coronal, and lambdoid synostosis suggest a benefit for endoscopic techniques with respect to blood loss, transfusion, length of stay, and operating time. This report highlights shortcomings in evidence and gaps in knowledge regarding endoscopic repair of nonsagittal single-suture craniosynostosis, emphasizing the need for further matched-control studies.

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Alexander G. Weil, Aria Fallah, Shelly Wang, George M. Ibrahim, Lior M. Elkaim, Prasanna Jayakar, Ian Miller, Sanjiv Bhatia, Toba N. Niazi and John Ragheb

OBJECTIVE

Although hemispherectomy is an effective treatment for children with intractable hemispheric epilepsy syndromes, as many as 40% of patients eventually develop seizure recurrence. The causes of seizure recurrence in these patients are incompletely understood. The authors sought to evaluate the efficacy of hemispherectomy at their center and determine whether contralateral MRI abnormalities can predict seizure recurrence.

METHODS

A retrospective review of consecutive hemispherectomies performed at Miami Children’s Hospital between January 2000 and June 2014 was performed. Time-to-event analysis was performed. The “event” was defined as any seizures following resective epilepsy surgery (not including seizures in the first postoperative week and auras). Several preoperative variables were analyzed to determine their suitability to predict seizure recurrence following surgery.

RESULTS

Sixty-nine patients (44 boys) with a mean age of 8.2 ± 5.9 years (range 0.1–20.8 years) underwent 72 hemispherectomies; 67 of these were functional hemispherectomies, while another 5 were completion of a previous functional hemispherectomy (2 completions of functional hemispherectomies, 3 anatomical hemispherectomies). The duration of epilepsy was 5.8 ± 5.5 years with 66 cases (91.7%) having daily seizures. Etiology included stroke (n = 28), malformation of cortical development (n = 11), hemimegalencephaly (n = 11), encephalitis (n = 13), and other (n = 7). Engel class I outcome was achieved in 59 (86%) and 56 (81%) patients at 1 and 2 years of follow-up, respectively. The mean time to seizure recurrence was 33.5 ± 31.1 months. In univariate analyses, the absence of contralateral abnormalities on MRI (HR 4.09, 95% CI 1.41–11.89, p = 0.009) was associated with a longer duration of seizure freedom. The presence of contralateral MRI abnormalities was associated with contralateral ictal seizures on preoperative scalp EEG (p = 0.002). Fifteen patients experienced 20 complications (20/72, 27.8%), including the development of hydrocephalus necessitating CSF diversion in 9 cases (13%), hygroma in 1, hemispheric edema in 1, aseptic meningitis in 2, postoperative hemorrhage in 2, infection in 2, ischemic stroke in 2, and blood transfusion–contracted hepatitis C in 1 case.

CONCLUSIONS

Patients with bihemispheric abnormalities, as evidenced by contralateral MRI abnormalities, have a higher risk of earlier seizure recurrence following functional hemispherectomy.