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M. Yashar S. Kalani, Ali M. Elhadi, Wyatt Ramey, Peter Nakaji, Felipe C. Albuquerque, Cameron G. McDougall, Joseph M. Zabramski, and Robert F. Spetzler

Object

Aneurysms are relatively rare in the pediatric population and tend to include a greater proportion of large and giant lesions. A subset of these large and giant aneurysms are not amenable to direct surgical clipping and require complex treatment strategies and revascularization techniques. There are limited data available on the management of these lesions in the pediatric population. This study was undertaken to evaluate the outcome of treatment of large and giant aneurysms that required microsurgical revascularization and vessel sacrifice in this population.

Methods

The authors retrospectively identified all cases in which pediatric patients (age < 18 years) with aneurysms were treated using cerebral revascularization in combination with other treatment modalities at their institution between 1989 and 2013.

Results

The authors identified 27 consecutive patients (19 male and 8 female) with 29 aneurysms. The mean age of the patients at the time of treatment was 11.5 years (median 13 years, range 1–17 years). Five patients presented with subarachnoid hemorrhage, 11 with symptoms related to mass effect, 2 with stroke, and 3 with seizures; in 6 cases, the aneurysms were incidental findings. Aneurysms were located along the internal carotid artery (n = 7), posterior cerebral artery (PCA) (n = 2), anterior cerebral artery (n = 2), middle cerebral artery (MCA) (n = 14), basilar artery (n = 2), vertebral artery (n = 1), and at the vertebrobasilar junction (n = 1). Thirteen were giant aneurysms (45%). The majority of the aneurysms were fusiform (n = 19, 66%), followed by saccular (n = 10, 34%). Three cases were previously treated using microsurgery (n = 2) or an endovascular procedure (n = 1). A total of 28 revascularization procedures were performed, including superficial temporal artery (STA) to MCA (n = 6), STA to PCA (n = 1), occipital artery to PCA (n = 1), extracranial-intracranial (EC-IC) bypass using radial artery graft (n = 3), EC-IC using a saphenous vein graft (n = 7), STA onlay (n = 3), end-to-end anastomosis (n = 1), and in situ bypasses (n = 6). Perioperative stroke occurred in 4 patients, but only one remained dependent (Glasgow Outcome Scale [GOS] score 3). At a mean clinical follow-up of 46 months (median 14 months, range 1–232 months), 26 patients had a good outcome (GOS score 4 or 5). There were no deaths. Five patients had documented occlusion of the bypass graft. The majority of aneurysms (n = 24) were obliterated at last follow-up. There was a single case of a residual aneurysm and one case of recurrence. Angiographic follow-up was unavailable in 3 cases.

Conclusions

Cerebral revascularization remains an essential tool in the treatment of complex cerebral aneurysms in children.

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Rami O. Almefty, Tammy L. Tyree, David J. Fusco, Stephen W. Coons, and Peter Nakaji

Histiocytic sarcoma is a rare malignancy with only 10 reports confirmed primarily involving the CNS. The diagnosis is dependent on the finding of malignant cells with histiocytic morphology and immunophenotype. The authors report a case of pathologically proven HS of the CNS. A 16-year-old boy presented with headaches, emesis, and altered sensorium. Noncontrast head CT scanning demonstrated a left parietal mass consistent with a tumor. Surgery was undertaken. Intraoperative findings revealed green-yellow exudates consistent with an abscess. Cultures were obtained and broad-spectrum antibiotics were started. The patient subsequently underwent multiple surgical procedures, including drainage and debulking of abscesses and hemicraniectomy. Two months after initial presentation, the patient's diagnosis of histiocytic sarcoma was confirmed. Pathological examination demonstrated necrotizing inflammation with preponderant neutrophil infiltration, variably atypical mononuclear and multinucleate histiocytes, and numerous mitoses. Additional immunohistochemistry studies confirmed immunoreactivity for CD68, CD45, CD45RO, and CD15 and were negative for CD3, CD20, melanoma cocktail, CD30, CD1a, CD34, HMB-45, and melan-A. Once the diagnosis of histiocytic sarcoma was confirmed, antibiotics were stopped and radiation therapy was undertaken. Despite treatment, the patient's neurological status continued to decline and the patient died 126 days after initial presentation.

This case represents a rare confirmed example of CNS histiocytic sarcoma. A profound inflammatory infiltrate seen on pathology and green exudates seen intraoperatively make the condition difficult to distinguish from an abscess. Immunohistochemistry showing a histiocytic origin and negative for myeloid, dendritic, or other lymphoid markers is essential for the diagnosis. Further research is needed to establish consensus on treatment.

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Carl H. Snyderman, Paul A. Gardner, and Juan C. Fernandez-Miranda

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Timothy Uschold, Adib A. Abla, David Fusco, Ruth E. Bristol, and Peter Nakaji

Object

The heterogeneous clinical manifestations and operative characteristics of pathological entities in the pineal region represent a significant challenge in terms of patient selection and surgical approach. Traditional surgical options have included endoscopic transventricular resection; open supratentorial microsurgical approaches through the midline, choroidal fissure, lateral ventricle, and tentorium; and supracerebellar infratentorial (SCIT) approaches through the posterior fossa. The object of the current study was to review the preoperative characteristics and outcomes for a cohort of patients treated purely via the novel endoscopically controlled SCIT approach.

Methods

A single-institution series of 9 consecutive patients (4 male and 5 female patients [10 total cases]; mean age 21 years, range 6–37 years) treated via the endoscopically controlled SCIT approach for a pathological entity in the pineal region was retrospectively reviewed. The mean follow-up time was 13.2 months.

Results

The endoscopically controlled SCIT approach was successfully used to approach a variety of pineal lesions, including pineal cysts (6 patients), epidermoid tumor, WHO Grade II astrocytoma (initial biopsy and recurrence), and malignant mixed germ cell tumor (1 patient each). Gross-total resection and/or adequate cyst fenestration was achieved in 8 cases. Biopsy with conservative debulking was performed for the single case of low-grade astrocytoma and again at the time of recurrence.

The mean preoperative tumor and cyst volumes were 9.9 ± 4.4 and 3.7 ± 3.2 cm3, respectively. The mean operating times were 212 ± 71 minutes for tumor cases and 177 ± 72 minutes for cysts. Estimated blood loss was less than 150 ml for all cases. A single case (pineal cyst) was converted to an open microsurgical approach to enhance visualization. There were no operative complications, as well as no documented CSF leaks, additional CSF diversion procedures, or air emboli. Seven patients underwent concomitant third ventriculostomy into the quadrigeminal cistern. At the time of the last follow-up evaluation, all patients had a stable or improved modified Rankin Scale score.

Conclusions

The endoscopically controlled SCIT approach may be used for the biopsy and resection of appropriately selected solid tumors of the pineal region, in addition to the fenestration and/or resection of pineal cysts. Preoperative considerations include patient presentation, anticipated disease and vascularity, degree of local venous anatomical distortion, and selection of optimal paramedian trajectory.

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M. Yashar S. Kalani, Maziyar A. Kalani, Samuel Kalb, Felipe C. Albuquerque, Cameron G. McDougall, Peter Nakaji, Robert F. Spetzler, Randall W. Porter, and Iman Feiz-Erfan

Object

Craniofacial approaches provide excellent exposure to lesions in the anterior and middle cranial fossae. The authors review their experience with craniofacial approaches for resection of large juvenile nasopharyngeal angiofibromas.

Methods

Between 1992 and 2009, 22 patients (all male, mean age 15 years, range 9–27 years) underwent 30 procedures. These cases were reviewed retrospectively.

Results

Gross-total resection of 17 (77%) of the 22 lesions was achieved. The average duration of hospitalization was 8.2 days (range 3–20 days). The rate of recurrence and/or progression was 4 (18%) of 22, with recurrences occurring a mean of 21 months after the first resection. All patients underwent preoperative embolization. Nine patients (41%) developed complications, the most common of which was CSF leakage (23%). The average follow-up was 27.7 months (range 2–144 months). The surgery-related mortality rate was 0%. Based on their mean preoperative (90) and postoperative (90) Karnofsky Performance Scale scores, 100% of patients improved or remained the same.

Conclusions

The authors' experience shows that craniofacial approaches provide an excellent avenue for the resection of large juvenile nasopharyngeal angiofibromas, with acceptable rates of morbidity and no deaths.