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Magnetic resonance imaging–guided focused ultrasound thalamotomy for essential tremor in a patient with von Willebrand disease: perioperative optimization for patients with coagulopathies. Illustrative case

Caroline Folz, Andreas Seas, Fadzai Chinyengetere, Christopher Beasley, Adam Harris, Charity Oyedeji, Thomas L Ortel, Bhavya R Shah, Shivanand Lad, and Stephen C Harward

BACKGROUND

Essential tremor (ET) is one of the most common movement disorders worldwide. In medically refractory ET, deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus is the current standard of care. However, DBS carries an inherent 2% to 3% risk of hemorrhage, a risk that can be much higher in patients with concomitant coagulopathy. Magnetic resonance imaging–guided focused ultrasound (MRgFUS) thalamotomy is a surgical alternative that is highly effective in treating ET, with no reports of intracranial hemorrhage to date.

OBSERVATIONS

This is the first documented case of successful MRgFUS thalamotomy in a patient with von Willebrand disease (VWD). A 60-year-old left-handed male had medically refractory ET, VWD type 2B, and a family history of clinically significant hemorrhage after DBS. He underwent right-sided MRgFUS thalamotomy and received a perioperative course of VONVENDI (recombinant von Willebrand factor) to ensure appropriate hemostasis. Postprocedure imaging confirmed a focal lesion in the right thalamus without evidence of hemorrhage. The patient reported 90% improvement of his left-hand tremor and significant improvement in his quality of life without obvious side effects.

LESSONS

MRgFUS thalamotomy with peri- and postoperative hematological management is a promising alternative to DBS for patients with underlying coagulopathies.

Open access

Characteristics of radiation-induced brain tumors: case series and systematic review

Shumpei Onishi, Fumiyuki Yamasaki, Yasuyuki Kinoshita, Vishwa Jeet Amatya, Ushio Yonezawa, Akira Taguchi, Iori Ozono, Yugo Maeda, Novita Ikbar Khairunnisa, Yukari Go, Yukio Takeshima, and Nobutaka Horie

OBJECTIVE

Radiation therapy (RT) improves the outcome of patients with cancer but introduces the risk of radiation-induced neoplasms in cancer survivors. The most common radiation-induced brain tumors (RIBTs) are gliomas (RIGs), meningiomas (RIMs), and sarcomas (RISs). To investigate the characteristics of these RIBTs, the authors conducted a comprehensive review and analysis of their case series and relevant cases from the literature.

METHODS

Sixteen patients in the case series and 941 patients from the literature who previously underwent cranial irradiation were included in this study. The age at irradiation for primary disease was recorded, and the latency period from irradiation to the development of RIBT and the median overall survival (OS) of patients with RIBTs were analyzed using the Kaplan-Meier method. Patients were stratified by age at the time of irradiation (pediatric vs nonpediatric) and the irradiation dose (higher vs lower dose), and latency and OS were compared using the log-rank test.

RESULTS

Among patients with RIBTs, 23.4% underwent radiation at < 5 years of age, and 46.6% underwent RT in the 1st decade of life. The median ages at cranial irradiation were 8.4 (IQR 4.1–16) years in patients with RIMs, 9 (IQR 5–23) years in patients with RIGs, and 27.7 (IQR 13.8–40) years in patients with RISs. The median latency period from irradiation to the development of RIM was significantly longer than that to the development of RIG and RIS (RIM: 20 years, RIG: 9 years, RIS: 10 years; p < 0.0001). The latency period was shorter in the nonpediatric patient group with RIMs (p = 0.047). The OS was significantly longer in patients with RIMs than in those with RIGs and RISs (RIM: not reached, RIG: 11 months, RIS: 11 months; p < 0.0001). The OS of patients with RIMs and RIGs was significantly shorter in patients who received higher radiation doses (p = 0.0095 and p = 0.0026, respectively).

CONCLUSIONS

The prognosis was poor and worse for patients with RIGs and RISs than for those with RIMs, and patients with RIBTs who underwent higher-dose irradiation for primary disease had poor prognoses. Because RIBTs develop more than a decade after cranial irradiation, long-term follow-up is crucial.

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Combined neuromodulation and resection for functional cortex epilepsy: a case series

Martín A. Merenzon, Adithya Sivaraju, Aline Herlopian, Jason L. Gerrard, Imran H. Quraishi, Lawrence J. Hirsch, Dennis D. Spencer, and Eyiyemisi C. Damisah

OBJECTIVE

Medically refractory epilepsy (MRE) often requires resection of the seizure onset zone (SOZ) for effective treatment. However, when the SOZ is in functional cortex (FC), achieving complete and safe resection becomes difficult, due to the seizure network overlap with function. The authors aimed to assess the safety and outcomes of a combined approach involving partial resection combined with focal neuromodulation for FC refractory epilepsy.

METHODS

The authors performed a retrospective analysis of individuals diagnosed with MRE who underwent surgical intervention from January 2015 to December 2022. Patients whose SOZ was located in FC and were treated with resection combined with simultaneous implantation of a focal neuromodulation device (responsive neurostimulation [RNS] device) with more than 12 months of follow-up data were included. All patients underwent a standard epilepsy preoperative assessment including intracranial electroencephalography and extraoperative stimulation mapping. Resections were performed under general anesthesia, followed by the concurrent implantation of an RNS device.

RESULTS

Seven patients (4 males, median age 32.3 years, all right-handed) were included. The median interval from seizure onset to surgery was 17.4 years. The epileptogenic network included sensorimotor areas (cases 2, 3, and 6), visual cortex (case 1), language areas (cases 4 and 7), and the insula (case 5). The median follow-up was 3 years (range 1–5.8 years). No significant changes in neuropsychological tests were reported. One permanent nondisabling planned neurological deficit (left inferior quadrantanopia) was observed. Six patients had stimulation activated at a median of 4.7 months after resection. All patients achieved good seizure outcomes (5 with Engel class I and 2 with Engel class II outcomes).

CONCLUSIONS

Maximal safe resection combined with focal neuromodulation presents a promising alternative to stand-alone resections for MRE epileptogenic zones overlapping with functional brain. This combined approach prioritizes the preservation of function while improving seizure outcomes.

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Endoscopic endonasal resection of Rathke cleft cysts: a single-institution analysis of 148 consecutive patients

Hanna N. Algattas, Zachary C. Gersey, David Fernandes Cabral, Ali A. Alattar, Hussein Abdallah, Nallammai Muthiah, Anamil Khiyami, Neha Mehrotra, Tiba Abdulwahid, Eric W. Wang, Carl H. Snyderman, Georgios A. Zenonos, Pouneh K. Fazeli, and Paul A. Gardner

OBJECTIVE

The traditional treatment of sellar Rathke cleft cysts (RCCs) generally involves transsellar drainage; however, suprasellar RCCs present unique challenges to appropriate management and technical complexity. Reports on overall outcomes for the endoscopic endonasal approach (EEA) for this pathology are limited. The EEA for RCCs allows three surgical techniques: marsupialization, fenestration, and fenestration with cyst wall resection.

METHODS

The authors performed a retrospective review of consecutive patients with RCCs that had been treated via an EEA at a single institution between January 2004 and May 2021. Marsupialization entailed the removal of cyst contents while maintaining a drainage pathway into the sphenoid sinus. Fenestration involved the removal of cyst contents, followed by separation from the sphenoid sinus, often with a free mucosal graft or vascularized nasoseptal flap. Cyst wall resection, either partial or complete, was added to select cases.

RESULTS

A total of 148 patients underwent an EEA for RCC. Marsupialization or fenestration was performed in 88 cases (59.5%) and cyst wall resection in 60 (40.5%). Cysts were classified as having a purely sellar origin (43.2%), sellar origin with suprasellar extension (37.8%), and purely suprasellar origin (18.9%). Radiological recurrence was demonstrated in 22 cases (14.9%) at an average 39.7 months’ follow-up (median 45 months, range 0.5–99 months), including 13 symptomatic cases (8.8%). Cases with cyst wall resection had no significantly different rate of recurrence (11.7% vs 15.9%, p = 0.48) or postoperative permanent anterior pituitary dysfunction (21.6% vs 12.5%, p = 0.29) compared to those of fenestrated and marsupialized cases. There was no significant difference in postoperative permanent posterior pituitary dysfunction based on technique, although such dysfunction tended to worsen with cyst wall resection (13.6% vs 4.0%, p = 0.09).

Based on cyst location, purely suprasellar cysts were more likely to have a radiological recurrence (28.6%) than sellar cysts with suprasellar extension (12.5%) and purely sellar cysts (9.4%; p = 0.008). Most notably, of the 28 purely suprasellar cysts, selective cyst wall resection significantly improved the long-term (10-year) recurrence risk compared to fenestration alone (17.4% vs 80.0%, p = 0.0005) without any significant added risk of endocrinopathy.

CONCLUSIONS

Endoscopic endonasal marsupialization or fenestration of sellar RCCs may be the ideal treatment strategy, whereas purely suprasellar cysts benefit from partial cyst wall resection to prevent recurrence. Selective cyst wall resection reduced long-term recurrence rates without significantly increasing rates of hypopituitarism.

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Fluoroscopic sniff test as a diagnostic tool for phrenic nerve injury in patients with traumatic brachial plexus injury

Pasin Watanapokasin, Yuwarat Monteerarat, Mathee Ongsiriporn, Roongsak Limthongthang, Torpon Vathana, and Panai Laohaprasitiporn

OBJECTIVE

Traumatic brachial plexus injury (BPI) is a high-morbidity condition with an escalating incidence. One of the treatment options is neurotization using the ipsilateral phrenic nerve. Therefore, diagnosis of nerve dysfunction is a crucial step in preoperative planning. This study aimed to assess the accuracy and reliability of the fluoroscopic sniff test for preoperative diagnosis of phrenic nerve injury in patients with traumatic BPI.

METHODS

The study was conducted from June 2019 to August 2023 at a tertiary care hospital. A preoperative fluoroscopic sniff test was performed. During brachial plexus surgery, direct phrenic nerve stimulation was conducted as a gold standard of phrenic nerve function. Two nonoperating orthopedic surgeons interpreted the accuracy and reliability of the test.

RESULTS

Seventy-four patients with traumatic BPI (66 males and 8 females) with a median age of 26 years were enrolled. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of the fluoroscopic sniff test were 90.9% (95% CI 75.7%−98.1%), 100% (95% CI 91.4%−100%), 100% (95% CI 88.4%−100%), 93.2% (95% CI 82.3%−97.6%), and 95.9% (95% CI 88.6%−99.2%), respectively. Interobserver reliability showed excellent agreement (κ = 1, p < 0.001).

CONCLUSIONS

The fluoroscopic sniff test was proven to be an accurate, reliable, and simple tool to evaluate phrenic nerve function in patients with traumatic BPI. Preoperative testing should be performed to reduce operative time to identify the phrenic nerve as a donor for nerve transfer surgery in cases in which no function is detected from the fluoroscopic sniff test.

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Fractionated radiotherapy after gross-total resection of spinal chordoma: a systematic review of survival outcomes using individualized patient data

Julian L. Gendreau, Kritika Gowda, Foad Kazemi, Melanie Horowitz, Moshe Shalom, Cathleen C. Kuo, Yusuf Mehkri, Michael Yan, Kristin J. Redmond, Daniel Lubelski, and Debraj Mukherjee

OBJECTIVE

Spinal chordoma treatment guidelines recommend resection. However, in patients in whom gross-total resection (GTR) is achieved, the benefits of radiation therapy (RT) are unclear. Therefore, the authors performed a systematic review to determine if RT is associated with postoperative progression-free survival (PFS) or overall survival (OS) after achieving GTR of spinal chordoma.

METHODS

The PubMed database was searched for studies including individualized data of patients undergoing GTR with or without RT for spinal chordoma. Patients < 18 years of age or those who underwent stereotactic body RT were excluded. Qualitative assessment was performed using Newcastle-Ottawa Scale guidelines. Log-rank tests for time-to-event data and a Cox proportional-hazards model were generated for a multivariable statistical model.

RESULTS

Complete data of 132 patients were retrieved, with 37 (28%) patients receiving adjuvant RT and 95 (72%) not receiving adjuvant RT. The mean follow-up was not statistically significantly different between those undergoing RT and not undergoing RT (54.02 months and 65.43 months, respectively). Patients were more likely not to undergo RT if their disease was located in the sacrum versus the mobile spine (p < 0.001). When controlling for age ≥ 65 years, male sex, disease location, and treatment year ≥ 2010, patients undergoing RT had similar PFS and OS when compared with those not undergoing RT on multivariable survival analysis (HR 0.935 [95% CI 0.703–2.340], p = 0.844 and HR 2.078 [95% CI 0.848–5.090], p = 0.110, respectively). However, age ≥ 65 years was associated with poorer OS in adjusted analyses (HR 2.761 [95% CI 1.185–6.432], p = 0.018) relative to patients < 65 years of age.

CONCLUSIONS

After achieving GTR of spinal chordoma, the utility of RT on PFS and OS remains unclear. Age ≥ 65 years appears to be associated with OS in spinal chordoma patients. Additional multicenter prospective studies are needed to determine the utility of RT in this patient population.

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Importance of the lumbar paraspinal muscles on the maintenance of global sagittal alignment after lumbar pedicle subtraction osteotomy

Thomas Caffard, Artine Arzani, Krizia Amoroso, Erika Chiapparelli, Samuel J. Medina, Lukas Schönnagel, Jiaqi Zhu, Bruno Verna, Kyle Finos, Isaac Nathoo, Soji Tani, Gaston Camino-Willhuber, Ali E. Guven, Arman Zadeh, Ek Tsoon Tan, John A. Carrino, Jennifer Shue, Oliver Dobrindt, Timo Zippelius, David Dalton, Andrew A. Sama, Federico P. Girardi, Frank P. Cammisa, and Alexander P. Hughes

OBJECTIVE

There are limited data about the influence of the lumbar paraspinal muscles on the maintenance of sagittal alignment after pedicle subtraction osteotomy (PSO) and the risk factors for sagittal realignment failure. The authors aimed to investigate the influence of preoperative lumbar paraspinal muscle quality on the postoperative maintenance of sagittal alignment after lumbar PSO.

METHODS

Patients who underwent lumbar PSO with preoperative lumbar MRI and pre- and postoperative whole-spine radiography in the standing position were included. Spinopelvic measurements included pelvic incidence, sacral slope, pelvic tilt, L1–S1 lordosis, T4–12 thoracic kyphosis, spinosacral angle, C7–S1 sagittal vertical axis (SVA), T1 pelvic angle, and mismatch between pelvic incidence and L1–S1 lordosis. Validated custom software was used to calculate the percent fat infiltration (FI) of the psoas major, as well as the erector spinae and multifidus (MF). A multivariable linear mixed model was applied to further examine the association between MF FI and the postoperative progression of SVA over time, accounting for repeated measures over time that were adjusted for age, sex, BMI, and length of follow-up.

RESULTS

Seventy-seven patients were recruited. The authors’ results demonstrated significant correlations between MF FI and the maintenance of corrected sagittal alignment after PSO. After adjustment for the aforementioned parameters, the model showed that the MF FI was significantly associated with the postoperative progression of positive SVA over time. A 1% increase from the preoperatively assessed total MF FI was correlated with an increase of 0.92 mm in SVA postoperatively (95% CI 0.42–1.41, p < 0.0001).

CONCLUSIONS

This study included a large patient cohort with midterm follow-up after PSO and emphasized the importance of the lumbar paraspinal muscles in the maintenance of sagittal alignment correction. Surgeons should assess the quality of the MF preoperatively in patients undergoing PSO to identify patients with severe FI, as they may be at higher risk for sagittal decompensation.

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Influence of the sagittal spinal profile type on the long-term outcome of patients with symptomatic lumbar spinal stenosis after microsurgical decompression

Anton Früh, Tarik Alp Sargut, Vincent Prinz, Nils Hecht, Jörg Franke, Malte Dinkelbach, Florian Pöhlmann, Peter Vajkoczy, and Simon Bayerl

OBJECTIVE

Microsurgical decompression for patients with symptomatic lumbar spinal stenosis (LSS) has demonstrated long-term improvement concerning pain and function. Nonetheless, a considerable proportion of these patients do not experience satisfactory alleviation of symptoms. Previous studies have not found a direct influence of single sagittal parameters on patient outcomes. However, recent research indicates that a composite of parameters, presented in specific sagittal profile types (SPTs) that were defined by Roussouly and colleagues, may affect these outcomes. This study aims to investigate the impact of SPT on long-term outcomes of patients with LSS following microsurgical decompression.

METHODS

This study is a prospective clinical observation. Patients with symptomatic LSS, who underwent microsurgical treatment and had long-term follow-up data for at least 36 months, were included. Patients with spinal deformity, fractures, or significant instability were excluded. Outcomes were measured using the numeric rating scale for pain, 36-Item Short Form Health Survey for quality of life, walking distance, Oswestry Disability Index, Roland-Morris Disability Questionnaire, and Odom’s criteria. SPT was determined in blinded fashion by using preoperative long standing radiographs.

RESULTS

The initial population of this observational study consisted of 128 patients, with long-term results available for 87 individuals, including 24 patients with SPT1, 20 with SPT2, 27 with SPT3, and 16 with SPT4. The average age was 70 years, with a slight male majority (56.3%) and a mean BMI of 27.9 kg/m2. After a median follow-up of 48 months, all groups showed significant improvement in walking distance, leg pain, and disability. Overall, 75% reported satisfaction with the surgery. However, patients with SPT1, which is characterized by low sacral slope and specific spinal curvatures, experienced significantly less improvement in back pain (p = 0.018) and related disability (p = 0.030), and lower satisfaction compared to other SPT groups (p = 0.008).

CONCLUSIONS

The sagittal spinal type is influencing the long-term outcome of patients suffering from symptomatic LSS. Patients with a combination of a flat sacral slope and a low overall lumbar lordosis with a high lordosis in the lower lumbar spine (i.e., spinal SPT1) showed worse outcome concerning back pain and had decreased satisfaction with surgery than comparable subjects from other SPT groups. Consequently, the authors recommend the assessment of sagittal spinal types in patients diagnosed with symptomatic spinal stenosis prior to decompression surgery. Inclusion of SPT in the preoperative consultation process can provide valuable insights, potentially guiding practitioners to more tailored patient counseling.

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Letter to the Editor. Electroencephalography for monitoring brain function and predicting prognosis in patients with TBI

Huisheng Yuan

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Letter to the Editor. Ventriculogallbladder shunts as treatment alternative for infants with necrotizing enterocolitis

Hector E. James