Punctate Midline Myelotomy: A Historical Overview and Case Series with Detailed Efficacy and Side Effect Profiles.

OBJECTIVE: To review our experience with punctate midline myelotomy (PMM) for malignant and benign visceral pain with an emphasis on detailed side-effect profiles and efficacy. METHODS: Thirteen adults (5 men) underwent microsurgical transverse-crush PMM. RESULTS: Median follow-up for the benign pain group (n = 6) was 17.5 months (10-72) and for the malignant group (n = 7) was 8 months (0.5-31). Five of seven patients in the malignant pain group obtained excellent, lasting relief. Two had initial relief followed by worsening pain with disease progression. In the benign pain group, two patients with endodermal-origin pain (gastrointestinal tract, bladder) had complete, long-lasting relief. Three patients with mesodermal-origin pain (ureter) had excellent relief for 2-3 months, followed by recurrence in two and partial (40%) recurrence in the third. One man with pre-existing cervical myelopathy underwent PMM for benign testicular-region pain from which he had long-term relief but only transient relief of coexisting low-back and leg pain. There were no motor deficits in either group, and all patients remained ambulatory and continent. The most common side effect was transient numbness of the medial leg and foot. Two patients (both with pre-existing spinal pathology) reported persistent moderate reduction of bowel, bladder, and sexual sensation. CONCLUSIONS: PMM offers substantial pain relief for carefully selected patients with intractable visceral pain. Relief from primarily endoderm-derived structures was most complete and long-lasting. Relief from mesoderm-derived structures was typically transient or incomplete. There was essentially no relief from pain of ectoderm-derived structures. Detailed preoperative counseling is important, especially for those with pre-existing neurologic deficits.

Early Effects of COVID-19 Pandemic on Neurosurgical Training in the United States: A Case Volume Analysis of 8 Programs.

OBJECTIVE: To determine the impact of the 2019 novel coronavirus disease (COVID-19) pandemic on operative case volume in 8 U.S. neurosurgical residency training programs in early 2020 and to survey these programs regarding training activities during this period. METHODS: A retrospective review was conducted of monthly operative case volumes and types for 8 residency programs for 2019 and January through April 2020. Cases were grouped as elective cranial, elective spine, and nonelective emergent cases. Programs were surveyed regarding residents' perceptions of the impact of COVID-19 on surgical training, didactics, and research participation. Data were analyzed for individual programs and pooled across programs. RESULTS: Across programs, the 2019 monthly mean ± SD case volume was 211 ± 82; 2020 mean ± SD case volumes for January, February, March, and April were 228 ± 93, 214 ± 84, 180 ± 73, and 107 ± 45. Compared with 2019, March and April 2020 mean cases declined 15% (P = 0.003) and 49% (P = 0.002), respectively. COVID-19 affected surgical case volume for all programs; 75% reported didactics negatively affected, and 90% reported COVID-19 resulted in increased research time. Several neurosurgery residents required COVID-19 testing; however, to our knowledge, only 1 resident from the participating programs tested positive. CONCLUSIONS: This study documents a significant reduction in operative volume in 8 neurosurgery residency training programs in early 2020. During this time, neurosurgery residents engaged in online didactics and research-related activities, reporting increased research productivity. Residency programs should collect data to determine the educational impact of the COVID-19 pandemic on residents' operative case volumes, identify deficiencies, and develop plans to mitigate any effects.

Woven Endobridge device for treatment of dissection-related PICA aneurysm.

Ruptured vertebrobasilar dissecting aneurysms require urgent, often challenging treatment as they have with a high re-hemorrhage rate within the first 24 hours. The patient is a 57-year-old woman who presented with severe-sudden onset headache. Further work up showed a ruptured dissecting aneurysm of the caudal loop of the posterior inferior cerebellar artery (PICA) with associated narrowing distally, in the ascending limb. The aneurysm was immediately occluded with a Woven Endobridge (WEB) device (MicroVention, Tustin, CA, USA) while flow diversion treatment of the diseased ascending limb was postponed. Follow-up angiography three months later showed complete occlusion of the aneurysm, as well as healing of the diseased distal vessel, obviating the need for further intervention. WEB embolization of a ruptured dissecting posterior circulation aneurysm provided an excellent outcome for this patient.

A Novel Plate-Based System (UNIMAX) for Posterior Instrumented Spinal Fusion.

Introduction The polyaxial head pedicle screw-rod system is a commonly used spinal instrumentation technique to achieve stabilization, deformity correction, and bony fusion. We present a novel plate-based pedicle screw system (UNIMAXTM) that can be used for multi-level instrumentation with potential advantages for selected applications. Methods Bilateral titanium monoaxial pedicle screws are linked at each level by robust transversely oriented cross plates forming ring constructs capable of rigid triangulation at each level. The cross plates are then interconnected by sagittally oriented rigid plates situated medial to the screw heads. Biomechanically, the construct was tested for quasi-static torsion and fatigue in axial compression. The system is approved by the Food and Drug Administration (FDA). The system and case examples are presented showing its potential advantages. Results The quasi-static torsion, the mean for the angular displacement, torsional stiffness, and torsional ultimate strength was 2.5 degrees (SD ± 0.8), 5.3 N-m/mm (SD ± 0.7), and 21.6 N-m (SD ± 4.4). For the fatigue in axial compression, the closed ring construct failed when the applied load and bending moment were ≥ 1500 N and ≥ 60 N.m. This system maximizes the construct rigidity, allows easy extension to adjacent levels, and can be incorporated intuitively into practice. The ring construct with triangulation at each level, together with its biomechanical robustness, predicts a high pullout resistance. It requires an open posterior approach incompatible with minimally invasive techniques. Conclusion This system may have advantages over the screw-rod systems in carefully selected situations requiring extra rigidity and high pull-out strength for complex reconstructions, sagittal and/or coronal correction, patients with poor bone quality, revisions, and/or extension to adjacent levels.

Rapid high-fidelity contour shaping of titanium mesh implants for cranioplasty defects using patient-specific molds created with low-cost 3D printing: A case series.

BACKGROUND: Cranioplasty is a neurosurgical procedure to repair skull defects. Sometimes, the patients' bone flap cannot be used for various reasons. Alternatives include a custom polyether ether ketone (PEEK) implant or titanium mesh; both incur an additional cost. We present a technique that uses a 3D printer to create a patient- specific 3D model used to mold a titanium mesh preoperatively. CASE DESCRIPTION: We included three patients whose bone flap could not be used. We collected the patients' demographics, cost, and time data for implants and the 3D printer. The patients' computed tomography DICOM images were used for 3D reconstruction of the cranial defect. A 3D printer (Flashforge, CA) was used to print a custom mold of the defect, which was used to shape the titanium mesh. All patients had excellent cosmetic results with no complications. The time required to print a 3D model was ~ 6 h and 45 min for preoperative shaping of the titanium implant. The intraoperative molding (IOM) of a titanium mesh needed an average of 60 min additional operative room time which incurred $4000. The average cost for PEEK and flat titanium mesh is $12,600 and $6750. Our method resulted in $4000 and $5500 cost reduction in comparison to flat mesh with IOM and PEEK implant. CONCLUSION: 3D printing technology can create a custom model to shape a titanium mesh preoperatively for cranioplasty. It can result in excellent cosmetic results and significant cost reduction in comparison to other cranioplasty options.

Delayed recurrence of acute subdural hematoma in a patient with plasminogen activator inhibitor mutation.

BACKGROUND: Plasminogen activator inhibitor type I (PAI-1) is important for balancing the fibrinolytic effect of plasmin, and deficiency can result in increased risk of bleeding. We report a case of a patient with PAI-1 deficiency who presented with delayed spontaneous recurrence of an acute subdural hematoma (aSDH) after evacuation. CASE DESCRIPTION: A 29-year-old male presented with altered mental status (AMS) after a fall at a construction site with Glasgow Coma Scale (GCS 4T). His coagulation profile was normal, and brain computed tomography (CT) showed a left-sided aSDH. He underwent emergent evacuation of the hematoma. On postoperative day 2, he was started on heparin for venous thromboembolism (VTE) prophylaxis. His neurological examination improved and was discharged with no focal deficits. Three days later, he presented with sudden AMS (GCS 7T); CT head showed a large hematoma at the site of original surgery. The hematoma was evacuated emergently. On readmission, the family informed providers that the patient had a history of PAI-1 deficiency. Postoperatively, only mechanical VTE prophylaxis was used and the patient was started on oral TXA per hematology recommendation. The patient improved and was discharged with no focal deficit. On follow-up, he remained neurologically stable. CONCLUSION: PAI-1 deficiency should be suspected in patients with delayed posttraumatic/surgical bleeding and a normal coagulation profile. If PAI-1 deficiency is evident or suspected, then a trial of antifibrinolytic agent should be used to treat and prevent recurrence of bleeding. Furthermore, chemical VTE prophylaxis should be avoided as it increases the risk for bleeding.

Spinal Cord Stimulation for Visceral Pain: Present Approaches and Future Strategies.

INTRODUCTION: The introduction of successful neuromodulation strategies for managing chronic visceral pain lag behind what is now treatment of choice in refractory chronic back and extremity pain for many providers in the United States and Europe. Changes in public policy and monetary support to identify nonopioid treatments for chronic pain have sparked interest in alternative options. In this review, we discuss the scope of spinal cord stimulation (SCS) for visceral pain, its limitations, and the potential role for new intradural devices of the type that we are developing in our laboratories, which may be able to overcome existing challenges. METHODS: A review of the available literature relevant to this topic was performed, with particular focus on the pertinent neuroanatomy and uses of spinal cord stimulation systems in the treatment of malignant and nonmalignant gastrointestinal, genitourinary, and chronic pelvic pain. RESULTS: To date, there have been multiple off-label reports testing SCS for refractory gastrointestinal and genitourinary conditions. Though some findings have been favorable for these organs and systems, there is insufficient evidence to make this practice routine. The unique configuration and layout of the pelvic pain pathways may not be ideally treated using traditional SCS implantation techniques, and intradural stimulation may be a viable alternative. CONCLUSIONS: Despite the prevalence of visceral pain, the application of neuromodulation therapies, a standard approach for other painful conditions, has received far too little attention, despite promising outcomes from uncontrolled trials. Detailed descriptions of visceral pain pathways may offer several clues that could be used to implement devices tailored to this unique anatomy.

Intraoperative CT for Neuronavigation Guidance and Confirmation of Foramen Ovale Cannulation for Glycerol Trigeminal Rhizotomy: A Technical Report and Case Series.

Glycerol rhizotomy was originally described as an initial surgical treatment for trigeminal neuralgia after the failure of medical therapy. Here we describe its use as a salvage procedure, typically after failure of multiple other modalities including microvascular decompression, stereotactic radiosurgery, and/or other percutaneous procedures. Foramen ovale cannulation as a "salvage procedure" may be complicated by lack of cerebrospinal fluid (CSF) return despite adequate cannulation of the foramen ovale, making conventional fluoroscopic confirmation of adequate needle placement less certain. In this article, we describe the application of intraoperative CT, fused with high-resolution preoperative CT/MRI for neuronavigation to accurately cannulate the foramen ovale and Meckel's cave for glycerol rhizotomy. Intraoperative CT, again fused with high-resolution preoperative CT and MRI studies, was then used to confirm accurate trajectory through the foramen ovale and the adequate location of the needle tip in Meckel's cave before injecting glycerol. We present our initial experience with 14 patients who underwent glycerol rhizotomy by these techniques depending on intraoperative CT. It appears that intraoperative CT-guided neuronavigation provides a practical, reliable, and accurate route to the foramen ovale and aids in the confirmation of adequate needle placement even when there is a lack of CSF return. These methods may be especially useful for difficult cannulations typical in salvage procedures. In an era of feasible intraoperative guidance, with advanced stereotactic planning software allowing the fusion of intraoperative CT with high-resolution preoperative CT and MRI datasets, these techniques can be applied to foramen ovale cannulation for glycerol rhizotomy without major modification.

Pseudoanginal chest pain associated with vagal nerve stimulation: a case report.

BACKGROUND: Vagal nerve stimulation (VNS) can be an effective therapy for patients with epilepsy refractory to anti-epileptic drugs or intracranial surgery. While generally well tolerated, it has been associated with laryngospasm, hoarseness, coughing, dyspnea, throat and atypical chest pain, cardiac symptoms such as bradycardia and occasionally asystole. We report on a patient receiving vagal nerve stimulation who experienced severe typical anginal chest pain during VNS firing without any evidence of cardiac ischemia or dysfunction. Thus, the pain appeared to be neuropathic from the stimulation itself rather than nociceptive secondary to an effect on heart function. CASE PRESENTATION: A 29-year-old man, with a history of intractable frontal lobe epilepsy refractory to seven anti-epileptic medications and subsequent intracranial surgery, underwent VNS implantation without complications. On beginning stimulation, he began to have intermittent chest pain that corresponded temporally to his intermittent VNS firing. The description of his pain was pathognomonic of ischemic cardiac chest pain. On initial evaluation, he displayed Levine's sign and reported crushing substernal chest pain radiating to the left arm, as well as shortness of breath walking upstairs that improved with rest. He underwent an extensive cardiac workup, including 12-lead ECG, cardiac stress test, echocardiogram, 12-day ambulatory cardiac monitoring, and continuous ECG monitoring each with and without stimulation of his device. The workup was consistently negative. Inability to resolve the pain necessitated the disabling and eventual removal of the device. CONCLUSION: To our knowledge, this is the first report of pseudoanginal chest pain associated with VNS. This occurrence prompted our review of the mechanisms of cardiac chest pain and suggests that vagal afferents may convey anginal pain separately or in parallel with known spinal cord pain mechanisms. These insights into the physiology of chest pain may be of general interest and important to surgeons implanting VNS devices who may potentially encounter such symptoms.

Cost comparison among punctate midline myelotomy, intrathecal pain pump, and spinal cord epidural stimulator.

BACKGROUND: Invasive pain procedures can be valuable tools to manage chronic pain. Here, we compared the costs of three procedures used to address chronic pain; punctate midline myelotomy (PMM), placement of a spinal cord stimulator (SCS), or placement of an intrathecal pain pump (ITPP). CASE DESCRIPTION: This retrospective chart review yielded 9 patients with chronic pain syndromes; 3 had PMM, 3 had SCS, and 3 had ITPP procedures. Variables studied included; pain type, the procedures performed, and the cost of each procedure. The Wilcoxon rank-sum and one-way analysis of variance were used to compare the three groups (P < 0.05). PMM was performed for patients with chronic nonmalignant visceral pain and SCS was utilized for failed back syndrome, while ITPP was placed in two patients with chronic visceral cancer pain and one patient with chronic somatic cancer pain. The mean length of stay was significant shorter for SCS and PMM versus ITPP (e.g., 1, 3.6 ± 0.6 and 15 ± 5.6 days). The mean procedure costs were significantly higher for SCS versus PMM and ITPP (105,234, $71,087, and $79,333); for the latter PMM and ITPP, procedural costs were not significantly different. CONCLUSION: For the three pain procedures discussed in this report, PMM is the most cost-effective as it obviates the need for efficacy trials, and there are: no implant device costs, no medication refills, no maintenance costs, and no complication management costs.

Midthoracic Punctate Midline Myelotomy for Treatment of Chronic, Intractable, Nonmalignant, Abdominal Visceral Pain: 2-Dimensional Operative Video.

Punctate midline myelotomy (PMM) is a surgical procedure that damages the ascending fibers of the postsynaptic dorsal column (PSDC) pathway to interrupt visceral pain transmission.1-3 It can offer relief to patients with chronic visceral pain conditions that are refractory to other treatments. Here, we present a surgical video of midthoracic PMM in a patient with chronic, intractable, nonmalignant visceral abdominal pain that failed over a decade of medical treatment. We choose T7-8 as the level for laminectomy in patients with pelvic or lower abdominal pain, because the postsynaptic pain fibers transmitting visceral pain sensation from the lower abdominal organs will invariably be caudal to this level. The patient developed immediate and complete relief of her visceral pain after the procedure, which was sustained through the 11-wk follow-up period to date and was able to be weaned off narcotics. Postoperatively, she remained full strength and had no impairment of light touch or proprioception of her lower extremities. Detailed physical examination showed a reduced vibratory sensation on the glabrous skin of her great toes. Regarding patient-reported sensory changes (not detected on physical examination), she reported some numbness on the insides of her feet that had resolved by 11-wk follow-up. She also reported some numbness of the vulva, but not of the vagina. However, by 11-wk follow-up, she reported this had resolved and she had normal sexual function. The only persistent sensation at 11-wk follow-up was slight tingling in her toes that was not bothersome to her.4 The patient presented in the following video consented to both the surgical procedure and the publication of her clinical history and operative video.

Modified McKenzie procedure for the treatment of fixed painful torticollis.

Spasmodic torticollis is characterized by involuntary movements of the neck muscles. In this video, the authors present the case of a 48-year-old man with painful right-sided rotational torticollis with contributions from both the suboccipital and the left sternocleidomastoid (SCM) muscles. He underwent a suboccipital craniectomy and C1-2 laminectomy with selective denervation of bilateral suboccipital and left-sided SCM muscles (modified McKenzie procedure). At the 2-week follow-up, he showed significant improvement and was able to rotate his neck about 70° toward the midline. Surgical treatment of spasmodic torticollis focuses on interrupting the motor pathway responsible for head turning. The modified McKenzie procedure is valuable, especially when other therapies fail. The video can be found here: https://youtu.be/TK-WybKnGJM.

Punctate Midline Myelotomy for Chronic, Intractable, Non-malignant Visceral Pain: A Case Report.

Punctate midline myelotomy (PMM) has a strong anatomic and functional basis for its role in the treatment of visceral pain. The procedure derived from advances in the understanding of the postsynaptic dorsal column (PSDC) pathway and the converging laboratory and clinical evidence that this spinal cord pathway plays a dominant role in conveying visceral pain to higher levels of the nervous system. The result of PMM is a concise, effective interruption of the PSDC pathway with minimal to no side effects. While considerable evidence now documents that PMM has good efficacy and safety in treating malignant visceral pain, there is little experience describing its application to chronic severe refractory visceral pain of benign origin.  We present the case of a patient with a 13-year history of severe non-malignant chronic abdominal visceral pain who obtained complete pain relief from a PMM at the T7 level. Intraoperative somatosensory evoked potential (SSEP) monitoring did not show changes after making the PMM lesion. As of six-months postoperative follow-up, the benefit shows no sign of fading, all pain medications have been discontinued, and there has been no impairment of motor function, bowel or bladder function, sexual function, gait or station. Upon detailed questioning, the patient endorsed only mild subjective reduced sensation of the inner aspects of her feet that was not bothersome to her. On detailed testing, position sense was preserved throughout; the Romberg test was negative, and the only finding was reduced vibratory sensation over the great toe pads. We cautiously suggest that the PMM operation may allow relief from severe, intractable, benign visceral pain syndromes for which effective treatments are otherwise elusive. The procedure warrants further study for such conditions.

Multiple Recurrences of Trigeminal Neuralgia Caused by Deformation of the Trigeminal Nerve.

Microvascular decompression (MVD) is frequently used for the treatment of trigeminal neuralgia (TN). We present a case of TN with multiple recurrences despite different interventions. A 50-year-old patient presented with a five-year history of left TN. He initially had an MVD with Teflon padding that eliminated his pain for a year. When pain recurred, he went on to stereotactic radiosurgery, which did not help. He then had two percutaneous retrogasserian glycerol injections, the first one relieved pain for a year and the second only six months. After the second recurrence, we repeated the magnetic resonance imaging (MRI) of the brain, and it showed kinking of the nerve with the padding in place. We decided to re-explore the nerve based on the MRI findings. Intraoperatively, we observed the Teflon padding had become adherent to the petrous bone which caused deformation of the nerve. We did adhesiolysis with debulking of the padding, following which the nerve appeared more relaxed. Postoperatively, the patient had immediate resolution of his pain. At eight-month follow-up, the patient remained pain-free. Multiple factors can be involved in recurrence of TN after MVD. In this case, the size of the padding, continued distortion from the offending artery in addition to scarring from radiosurgery may have contributed to the deformation of the nerve and the recurrence of symptoms. A new MRI can be beneficial when the neuralgia symptoms recur in delayed fashion after successful MVD. Also, the use of a more compact padding material, like Gore-Tex, may cause less deformation of the nerve.

Punctate Midline Myelotomy Reduces Pain Responses in a Rat Model of Lumbar Spine Pain: Evidence that the Postsynaptic Dorsal Column Pathway Conveys Pain from the Axial Spine.

Punctate midline myelotomy (PMM) has been successfully applied clinically in humans for the relief of intractable visceral pain. The operation is thought to work by interrupting the postsynaptic dorsal column pathway (PSDC) of the spinal cord. In fact, PMM was developed specifically for that purpose after it was demonstrated in rats that the PSDC conveyed about 90% of the visceral pain information to the thalamus. The application of PMM also to the problem of severe intractable back or spine pain was never tested, and it has never been established whether the PSDC pathway relates only to visceral pain or whether there may be a broader involvement with pain affecting structures of embryological midline origin, perhaps including the spine. Retrospective analyses of decades of results from various attempted myelotomy procedures in man for the relief of pain are consistent with the notion that the common element crucial to the successful midline or visceral pain relief was the interruption--even incomplete--of the PSDC pathway. Herein, we present evidence from a rat model of lumbar facet pain that interruption of the PSDC significantly reduces pain responses. The implications for the possible treatment of severe intractable spine pain in man are discussed.

Increasing time to postoperative stereotactic radiation therapy for patients with resected brain metastases: investigating clinical outcomes and identifying predictors associated with time to initiation.

We sought to determine the impact of time to initiation (TTI) of post-operative radiosurgery on clinical outcomes for patients with resected brain metastases and to identify predictors associated with TTI. All patients with resected brain metastases treated with postoperative SRS or fractionated stereotactic radiation therapy (fSRT) from 2012 to 2016 at a single institution were reviewed. TTI was defined as the interval from resection to first day of radiosurgery. Receiver operating characteristic (ROC) curves were used to identify an optimal threshold for TTI with respect to local failure (LF). Survival outcomes were estimated using the Kaplan-Meier method and analyzed using the log-rank test and Cox proportional hazards models. Logistic regression models were used to identify factors associated with ROC-determined TTI covariates. A total of 79 resected lesions from 73 patients were evaluated. An ROC curve of LF and TTI identified an optimal threshold for TTI of 30.5 days, with an area under the curve of 0.637. TTI > 30 days was associated with an increased hazard of LF (HR 4.525, CI 1.239-16.527) but was not significantly associated with survival (HR 1.002, CI 0.547-1.823) or distant brain failure (DBF, HR 1.943, CI 0.989-3.816). Fifteen patients (20.5%) required post-operative inpatient rehabilitation. Post-operative rehabilitation was associated with TTI > 30 days (OR 1.48, CI 1.142-1.922). In our study of resected brain metastases, longer time to initiation of post-operative radiosurgery was associated with increased local failure. Ideally, post-op SRS should be initiated within 30 days of resection if feasible.

Fungal brain abscess caused by "Black Mold" (Cladophialophora bantiana) - A case report of successful treatment with an emphasis on how fungal brain abscess may be different from bacterial brain abscess.

BACKGROUND: Central nervous system infection with Cladophialophora bantiana (Black Mold) is rare. It carries a high mortality rate, that is more than 70%, despite multimodal therapy. CASE DESCRIPTION: We present a rare case of "black mold" fungal brain abscess that was successfully treated with good patient outcome. The case is unusual because there were two fungal brain abscesses located bilaterally symmetrically in the mesial frontal lobes, and the response to different treatment strategies was well documented by over 25 magnetic resonance imaging (MRI) scans. Initial attempts to treat these lesions by repeated surgical excision and systemic amphotericin B was followed by continued growth rather than resolution. We realized that the application of treatment principles learned from bacterial brain abscess may not transpose intuitively to the treatment of fungal brain abscess. Therefore, a new treatment strategy was adopted that avoided further attempts at resection in favor of long-term oral voriconazole and repeated intracavitary aspiration and instillation of amphotericin B on an outpatient basis. Without further resection, the lesions stabilized and the aspirates eventually sterilized, however, the enhancing capsule never resolved on MRI scans. All treatment was stopped after 1 year. The apparently sterilized lesions have been followed for an additional 3 years without further growth, and the patient remains functionally, intellectually, and behaviorally normal. CONCLUSION: We conclude that, in the case of fungal abscess, it may be preferable to sterilize the lesion in situ rather than attempting to achieve resolution on imaging studies by repeated surgical resection of the capsule which can be counterproductive. This strategy accepts that the capsule may be important to the patient's immune defense against the fungus. Helping that defense barrier with intracapsular and systemic antifungal agents, rather than capsular removal, may be the better strategy for patients in whom early aggressive resection has failed. The basis for the apparent differences in the response of fungal versus bacterial brain abscess to surgical resection is discussed in the light of pathological findings from this and other cases.

Neuronavigation-guided endoscopic decompression of superior orbital fissure fracture: case report and literature review.

OBJECTIVE: We present a rare case of an isolated superior orbital fissure fracture resulting from blunt injury to the face and presenting with selective cranial nerve deficits surgically treated with a neuroendoscopic approach. The anatomy of the superior orbital fissure is detailed, and the peculiarities of the surgical approach described. METHOD: A review of the existing literature reveals this is the first reported case of a neuronavigation-assisted endoscopic approach used in the extraction of a superior orbital fracture fragment with good outcome. Current guidelines for an endoscopic approach in orbital trauma are reviewed, and pertinent literature is discussed. CONCLUSION: Neuronavigation-assisted decompression should be considered as an effective means of removing superior orbital fissure fractures.

Coexistent intraventricular abnormalities in periventricular giant arachnoid cysts.

OBJECT: Arachnoid cysts are congenital lesions that arise during development by splitting of the arachnoid membrane. Large cysts can be adjacent to CSF pathways causing a marked midline shift and hydrocephalus. The association between a large arachnoid cyst and hydrocephalus has been commonly described as being due to a mass effect, but these previous reports have not focused closely on any associated intraventricular abnormalities. METHODS: Seven patients who were previously treated with a cystoperitoneal shunt presented with shunt failure, hydrocephalus, and/or cyst expansion. All of these patients had giant arachnoid cysts extending to the periventricular region from the original site, which was the sylvian fissure in 4 patients, and the suprasellar cistern, quadrigeminal cistern, and interhemispheric fissure in 1 patient each. Endoscopic exploration of the ventricular system and cyst fenestration was then performed in all patients. RESULTS: The endoscopic findings were obstruction of the cerebral aqueduct by a membrane not related to the cyst in 5 patients, occlusion of the foramen of Monro in 6, septum pellucidum hypoplasia in 2, and occlusion of the cerebral aqueduct by a quadrigeminal arachnoid cyst in 1. Endoscopic procedures performed were septum pellucidum fenestration and/or foraminoplasty in 5 patients, aqueductoplasty in 2, endoscopic third ventriculostomy in 5, fenestration of the lamina terminalis in 1, and direct cystocisternostomy in 1. After the endoscopic procedure, signs and symptoms of increased intracranial pressure and hydrocephalus improved in all patients, with a reduction in size of the cyst and the ventricle. CONCLUSIONS: Ventricular abnormalities contributing to hydrocephalus may be associated with arachnoid cysts. These abnormalities may more likely reflect a common origin than a casual relation. Foramen of Monro stenosis and cerebral aqueduct occlusion associated with an arachnoid cyst can be more frequent than has been previously believed. In cases of periventricular giant arachnoid cysts, endoscopic exploration is a good alternative for examining the ventricular system and identifying and treating CSF obstructions caused by and/or related to arachnoid cysts.

Apnea and macrocephaly-cutis marmorata telangiectatica congenita.

The authors report a case of an infant girl with macrocephaly-cutis marmorata telangiectatica congenita (Macrocephaly-CMTC). This patient presented with developmental delay, mild subcostal retractions, and occasional apneic spells. An MRI demonstrated mild to moderate lateral ventricle hydrocephalus, left hemi-megalencephaly, and left cerebellar tonsillar herniation with full occlusion of the cisterna magna. Her foramen magnum was narrowed, measuring 17.5mm in transverse diameter. This value was significantly below the 50th percentile for age, which is 23.5mm. Together, these findings were suggestive of cervicomedullary cord compression, concerning for sudden death. The patient underwent posterior fossa decompression by suboccipital craniectomy and cervical laminectomy. Initially due to hypertrophy and paralysis of the left true and false vocal cords, endotracheal intubation was not achieved, requiring tracheostomy tube placement. To our knowledge this is the first report of apnea in a patient diagnosed with M-CMTC, likely due to cervicomedullary cord compression and perhaps exacerbated by unilateral laryngeal hypertrophy. M-CMTC is a newly-described hemi-hypertrophy syndrome affecting the neurodevelopment of affected children. The authors emphasize airway obstruction secondary to unilateral hypertrophy of the vocal cords in addition to brainstem compromise as a consideration for the etiology of apnea in M-CMTC patients presenting with signs and symptoms of cervicomedullary cord compression.