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  • Author or Editor: Juan C. Fernandez-Miranda x
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Juan C. Fernández-Miranda, Albert L. Rhoton Jr., Yukinari Kakizawa, Chanyoung Choi and Juan Álvarez-Linera

Object

The goal in this study was to examine the microsurgical and tractographic anatomy of the claustrum and its projection fibers, and to analyze the functional and surgical implications of the findings.

Methods

Fifteen formalin-fixed human brain hemispheres were dissected using the Klingler fiber dissection technique, with the aid of an operating microscope at × 6–40 magnification. Magnetic resonance imaging studies of 5 normal brains were analyzed using diffusion tensor (DT) imaging–based tractography software.

Results

Both the claustrum and external capsule have 2 parts: dorsal and ventral. The dorsal part of the external capsule is mainly composed of the claustrocortical fibers that converge into the gray matter of the dorsal claustrum. Results of the tractography studies coincided with the fiber dissection findings and showed that the claustrocortical fibers connect the claustrum with the superior frontal, precentral, postcentral, and posterior parietal cortices, and are topographically organized. The ventral part of the external capsule is formed by the uncinate and inferior occipitofrontal fascicles, which traverse the ventral part of the claustrum, connecting the orbitofrontal and prefrontal cortex with the amygdaloid, temporal, and occipital cortices. The relationship between the insular surface and the underlying fiber tracts, and between the medial lower surface of the claustrum and the lateral lenticulostriate arteries is described.

Conclusions

The combination of the fiber dissection technique and DT imaging–based tractography supports the presence of the claustrocortical system as an integrative network in humans and offers the potential to aid in understanding the diffusion of gliomas in the insula and other areas of the brain.

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Letter to the Editor

High-definition fiber tractography and language

Juan C. Fernandez-Miranda, Sudhir Pathak and Walter Schneider

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Chirag R. Patel, Eric W. Wang, Juan C. Fernandez-Miranda, Paul A. Gardner and Carl H. Snyderman

OBJECTIVE

The endoscopic endonasal approach (EEA) has been shown to be an effective means of accessing lesions of the petrous apex. Lesions that are lateral to the paraclival segment of the internal carotid artery (ICA) require lateralization of the paraclival segment of the ICA or a transpterygoid infrapetrous approach. In this study the authors studied the feasibility of adding a contralateral transmaxillary (CTM) corridor to provide greater access to the petrous apex with decreased need for manipulation of the ICA.

METHODS

Using image guidance, EEA and CTM extension were performed bilaterally on 5 cadavers. The anterior wall of the sphenoid sinus and rostrum were removed. The angle of the surgical approach from the axis of the petrous segment of the ICA was measured. Five illustrative clinical cases are presented.

RESULTS

The CTM corridor required a partial medial maxillectomy. When measured from the axis of the petrous ICA, the CTM corridor decreased the angle from 44.8° ± 2.78° to 20.1° ± 4.31°, a decrease of 24.7° ± 2.58°. Drilling through the CTM corridor allowed the drill to reach lateral aspects of the petrous apex that would have required lateralization of the ICA or would not have been accessible via EEA. The CTM corridor allowed us to achieve gross-total resection of the petrous apex region in 5 clinical cases with significant paraclival extension.

CONCLUSIONS

The CTM corridor is a feasible extension to the standard EEA to the petrous apex that offers a more lateral trajectory with improved access. This approach may reduce the risk and morbidity associated with manipulation of the paraclival ICA.

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Antonio Meola, Ayhan Comert, Fang-Cheng Yeh, Sananthan Sivakanthan and Juan C. Fernandez-Miranda

OBJECT

The dentatorubrothalamic tract (DRTT) is the major efferent cerebellar pathway arising from the dentate nucleus (DN) and decussating to the contralateral red nucleus (RN) and thalamus. Surprisingly, hemispheric cerebellar output influences bilateral limb movements. In animals, uncrossed projections from the DN to the ipsilateral RN and thalamus may explain this phenomenon. The aim of this study was to clarify the anatomy of the dentatorubrothalamic connections in humans.

METHODS

The authors applied advanced deterministic fiber tractography to a template of 488 subjects from the Human Connectome Project (Q1–Q3 release, WU-Minn HCP consortium) and validated the results with microsurgical dissection of cadaveric brains prepared according to Klingler’s method.

RESULTS

The authors identified the “classic” decussating DRTT and a corresponding nondecussating path (the nondecussating DRTT, nd-DRTT). Within each of these 2 tracts some fibers stop at the level of the RN, forming the dentatorubro tract and the nondecussating dentatorubro tract. The left nd-DRTT encompasses 21.7% of the tracts and 24.9% of the volume of the left superior cerebellar peduncle, and the right nd-DRTT encompasses 20.2% of the tracts and 28.4% of the volume of the right superior cerebellar peduncle.

CONCLUSIONS

The connections of the DN with the RN and thalamus are bilateral, not ipsilateral only. This affords a potential anatomical substrate for bilateral limb motor effects originating in a single cerebellar hemisphere under physiological conditions, and for bilateral limb motor impairment in hemispheric cerebellar lesions such as ischemic stroke and hemorrhage, and after resection of hemispheric tumors and arteriovenous malformations. Furthermore, when a lesion is located on the course of the dentatorubrothalamic system, a careful preoperative tractographic analysis of the relationship of the DRTT, nd-DRTT, and the lesion should be performed in order to tailor the surgical approach properly and spare all bundles.

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Srinivas Chivukula, Maria Koutourousiou, Carl H. Snyderman, Juan C. Fernandez-Miranda, Paul A. Gardner and Elizabeth C. Tyler-Kabara

Object

The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies.

Methods

A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed.

Results

A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eighty-five patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up.

Conclusions

Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population.

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Maria Koutourousiou, Paul A. Gardner, Juan C. Fernandez-Miranda, Elizabeth C. Tyler-Kabara, Eric W. Wang and Carl H. Snyderman

Object

The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging and controversial management dilemmas in neurosurgery. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for both pediatric and adult craniopharyngiomas. The object of the present study was to present the results of EES and analyze outcome in both the pediatric and the adult age groups.

Methods

The authors retrospectively reviewed the records of patients with craniopharyngioma who had undergone EES in the period from June 1999 to April 2011.

Results

Sixty-four patients, 47 adults and 17 children, were eligible for this study. Forty-seven patients had presented with primary craniopharyngiomas and 17 with recurrent tumors. The mean age in the adult group was 51 years (range 28–82 years); in the pediatric group, 9 years (range 4–18 years). Overall, the gross-total resection rate was 37.5% (24 patients); near-total resection (> 95% of tumor removed) was 34.4% (22 patients); subtotal resection (≥ 80% of tumor removed) 21.9% (14 patients); and partial resection (< 80% of tumor removed) 6.2% (4 patients). In 9 patients, EES had been combined with radiation therapy (with radiosurgery in 6 cases) as the initial treatment. Among the 40 patients (62.5%) who had presented with pituitary insufficiency, pituitary function remained unchanged in 19 (47.5%), improved or normalized in 8 (20%), and worsened in 13 (32.5%). In the 24 patients who had presented with normal pituitary function, new pituitary deficit occurred in 14 (58.3%). Nineteen patients (29.7%) suffered from diabetes insipidus at presentation, and the condition developed in 21 patients (46.7%) after treatment. Forty-four patients (68.8%) had presented with impaired vision. In 38 (86.4%) of them, vision improved or even normalized after surgery; in 5, it remained unchanged; and in 1, it temporarily worsened. One patient without preoperative visual problems showed temporary visual deterioration after treatment. Permanent visual deterioration occurred in no one after surgery. The mean follow-up was 38 months (range 1–135 months). Tumor recurrence after EES was discovered in 22 patients (34.4%) and was treated with repeat surgery (6 patients), radiosurgery (1 patient), combined repeat surgery and radiation therapy (8 patients), interferon (1 patient), or observation (6 patients). Surgical complications included 15 cases (23.4%) with CSF leakage that was treated with surgical reexploration (13 patients) and/or lumbar drain placement (9 patients). This leak rate was decreased to 10.6% in recent years after the introduction of the vascularized nasoseptal flap. Five cases (7.8%) of meningitis were found and treated with antibiotics without further complications. Postoperative hydrocephalus occurred in 7 patients (12.7%) and was treated with ventriculoperitoneal shunt placement. Five patients experienced transient cranial nerve palsies. There was no operative mortality.

Conclusions

With the goal of gross-total or maximum possible safe resection, EES can be used for the treatment of every craniopharyngioma, regardless of its location, size, and extension (excluding purely intraventricular tumors), and can provide acceptable results comparable to those for traditional craniotomies. Endoscopic endonasal surgery is not limited to adults and actually shows higher resection rates in the pediatric population.

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David T. Fernandes Cabral, Georgios A. Zenonos, Juan C. Fernandez-Miranda, Eric W. Wang and Paul A. Gardner

OBJECTIVE

Iatrogenic tumor seeding after open surgery for chordoma has been well described in the literature. The incidence and particularities related to endoscopic endonasal surgery (EES) have not been defined.

METHODS

The authors retrospectively reviewed their experience with EES for clival chordoma, focusing on cases with iatrogenic seeding. The clinical, radiographic, pathological, and molecular characterization data were reviewed.

RESULTS

Among 173 EESs performed for clival chordomas at the authors’ institution between April 2003 and May 2016, 2 cases complicated by iatrogenic seeding (incidence 1.15%) were identified. The first case was a 10-year-old boy, who presented 21 months after an EES for a multiply recurrent clival chordoma with a recurrence along the left inferior turbinate, distinct from a right petrous apex recurrence. Both appeared as a T2-hypertintense, T1-isointense, and heterogeneously enhancing lesion on MRI. Resection of the inferior turbinate recurrence and debulking of the petrous recurrence were both performed via a purely endoscopic endonasal approach. Unfortunately, the child died 2 years later due progression of disease at the primary site, but with no sign of progression at the seeded site. The second patient was a 79-year-old man with an MRI-incompatible pacemaker who presented 19 months after EES for his clival chordoma with a mass involving the floor of the left nasal cavity that was causing an oro-antral fistula. On CT imaging, this appeared as a homogeneously contrast-enhancing mass eroding the hard palate inferiorly, the nasal septum superiorly, and the nasal process of the maxilla, with extension into the subcutaneous tissue. This was also treated endoscopically (combined transnasal-transoral approach) with resection of the mass, and repair of the fistula by using a palatal and left lateral wall rotational flap. Adjuvant hypofractionated stereotactic CyberKnife radiotherapy was administered using 35 Gy in 5 fractions. No recurrence was appreciated endoscopically or on imaging at the patient’s last follow-up, 12 months after this last procedure. In both cases, pathological investigation of the original tumors revealed a fairly aggressive biology with 1p36 deletions, and high Ki-67 levels (10%–15%, and > 20%, respectively). The procedures were performed by a team of right-handed surgeons (otolaryngology and neurosurgery), using a 4-handed technique (in which the endoscope and suction are typically passed through the right nostril, and other instruments are passed through the left nostril without visualization).

CONCLUSIONS

Although uncommon, iatrogenic seeding occurs during EES for clival chordomas, probably because of decreased visualization during tumor removal combined with mucosal trauma and exposure of subepithelial elements (either inadvertently or because of mucosal flaps). In addition, tumors with more aggressive biology (1p36 deletions, elevated Ki-67, or both) are probably at a higher risk and require increased vigilance on surveillance imaging and endoscopy. Further prospective studies are warranted to evaluate the authors’ proposed strategies for decreasing the incidence of iatrogenic seeding after EES for chordomas.

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Shannon Fraser, Paul A. Gardner, Maria Koutourousiou, Mark Kubik, Juan C. Fernandez-Miranda, Carl H. Snyderman and Eric W. Wang

OBJECTIVE

The aim in this paper was to determine risk factors for the development of a postoperative CSF leak after an endoscopic endonasal approach (EEA) for resection of skull base tumors.

METHODS

A retrospective review of patients who underwent EEA for the resection of intradural pathology between January 1997 and June 2012 was performed. Basic demographic data were collected, along with patient body mass index (BMI), tumor pathology, reconstruction technique, lumbar drainage, and outcomes.

RESULTS

Of the 615 patients studied, 103 developed a postoperative CSF leak (16.7%). Sex and perioperative lumbar drainage did not affect CSF leakage rates. Posterior fossa tumors had the highest rate of CSF leakage (32.6%), followed by anterior skull base lesions (21.0%) and sellar/suprasellar lesions (9.9%) (p < 0.0001). There was a higher leakage rate for overweight and obese patients (BMI > 25 kg/m2) than for those with a healthy-weight BMI (18.7% vs 11.5%; p = 0.04). Patients in whom a pedicled vascularized flap was used for reconstruction had a lower leakage rate than those in whom a free graft was used (13.5% vs 27.8%; p = 0.0015). In patients with a BMI > 25 kg/m2, the use of a pedicled flap reduced the rate of CSF leakage from 29.5% to 15.0% (p = 0.001); in patients of normal weight, this reduction did not reach statistical significance (21.9% [pedicled flap] vs 9.2% [free graft]; p = 0.09).

CONCLUSIONS

Preoperative BMI > 25 kg/m2 and tumor location in the posterior fossa were associated with higher rates of postoperative CSF leak. Use of a pedicled vascularized flap may be associated with reduced risk of a CSF leak, particularly in overweight patients.

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Danilo Silva, Moshe Attia and Theodore H. Schwartz

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Juan C. Fernandez-Miranda, Johnathan A. Engh, Sudhir K. Pathak, Ricky Madhok, Fernando E. Boada, Walter Schneider and Amin B. Kassam

The authors have applied high-definition fiber tracking (HDFT) to the resection of an intraparenchymal dermoid cyst by using a minimally invasive endoscopic port. The lesion was located within the mesial frontal lobe, septal area, hypothalamus, and suprasellar recess. Using high-dimensional (256 directions) diffusion imaging, more than 250,000 fiber tracts were imaged before and after surgery. Trajectory planning using HDFT in a computer model was used to facilitate cannulation of the cyst with the endoscopic port. Analysis of the proposed initial surgical route was overlaid onto the fiber tracts and was predicted to produce substantial disruption to prefrontal projection fibers (anterior limb of the internal capsule) and the cingulum. Adjustment of the cannulation entry point 1 cm medially was predicted to cross the corpus callosum instead of the anterior limb of the internal capsule or the cingulum. Following cyst resection performed using endoscopic port surgery, postoperative imaging demonstrated accurate cannulation of the lesion, with improved quantitative signal from both the anterior limb of the internal capsule and the cingulum. The observed fiber preservation from the cingulum and the anterior limb of the internal capsule, with minor injury to the corpus callosum, was in close agreement with preoperative trajectory modeling. Comparison of pre- and postoperative HDFT data facilitated quantification of the benefits and costs of the surgical trajectory. Future studies will help to determine whether HDFT combined with endoscopic port surgery facilitates anatomical and functional preservation in such challenging cases.