Chordoma, a rare bone tumor that occurs along the spine, has led scientists on a fascinating journey of discoveries. In this historical vignette, the authors track these discoveries in diagnosis and treatment, noting events and clinicians that played pivotal roles in our current understanding of chordoma. The study of chordoma begins in 1846 when Rudolf Virchow first observed its occurrence on a dorsum sellae; he coined the term “chordomata” 11 years later. The chordoma’s origin was greatly disputed by members of the scientific community. Eventually, Müller’s notochord hypothesis was accepted 36 years after its proposal. Chordomas were considered benign and slow growing until the early 1900s, when reported autopsy cases drew attention to their possible malignant nature. Between 1864 and 1919, the first-ever symptomatic descriptions of various forms of chordoma were reported, with the subsequent characterization of chordoma histology and the establishment of classification criteria shortly thereafter. The authors discuss the critical historical steps in diagnosis and treatment, as well as pioneering operations and treatment modalities, noting the physicians and cases responsible for advancing our understanding of chordoma.
Ronald Sahyouni, Khodayar Goshtasbi, Amin Mahmoodi, and Jefferson W. Chen
Brandon M. Lehrich, Nolan J. Brown, Shane Shahrestani, Ronald Sahyouni, and Frank P. K. Hsu
Dr. James Tait Goodrich was an internationally renowned pediatric neurosurgeon who pioneered the neurosurgical procedures for the multistage separation of craniopagus twins. As of March 2020, 59 craniopagus separations had been performed in the world, with Goodrich having performed 7 of these operations. He was the single most experienced surgeon in the field on this complex craniofacial disorder. Goodrich was a humble individual who rapidly rose through the ranks of academic neurosurgery, eventually serving as Director of the Division of Pediatric Neurosurgery at the Children’s Hospital at Montefiore Medical Center in the Bronx, New York. In this historical vignette, the authors provide context into the history of and sociocultural attitudes toward conjoined twins; the epidemiology and classification of craniopagus twins; the beginnings of surgery in craniopagus twins; Goodrich’s neurosurgical contributions toward advancing treatment for this complex craniofacial anomaly; and vignettes of Goodrich’s unique clinical cases that made mainstream news coverage.
Nolan J. Brown, Shane Shahrestani, Brian V. Lien, Seth C. Ransom, Ali R. Tafreshi, Ryan Chase Ransom, and Ronald Sahyouni
Cervical angina, or pseudoangina pectoris, is a noncardiac syndrome of chest pain that often mimics angina pectoris but is a disease of the spine. Diagnosis of cervical angina can be difficult and is often overlooked, although once identified, it can be successfully managed through conservative therapies and/or a variety of surgical interventions. Ultimately, cervical angina is an important component of the list of differential diagnoses in noncardiac chest pain. In the present study, the authors report the first comprehensive systematic review of the range of cervical and thoracic pathologies associated with cervical angina, as well as the different treatment methods used to manage this condition.
A systematic review was performed according to PRISMA guidelines and using PubMed, Web of Science, and Cochrane databases from database inception to April 29, 2020, to identify studies describing spinal pathologies related to cervical angina. The following Boolean search was performed: (“cervical” OR “thoracic”) AND (“angina” OR “chest pain”) AND (“herniation” OR “OPLL”). Variables extracted included patient demographics, cervical angina pain location, pathology and duration of symptoms, treatment and/or management method, and posttreatment pain relief.
Upon careful screening, 22 articles published between 1976 and 2020 met the study’s inclusion/exclusion criteria, including 5 case series, 12 case reports, and 5 retrospective cohort studies. These studies featured a total of 1100 patients, of which 95 met inclusion criteria (mean patient age 51.7 years, age range 24–86 years; 53.6% male). Collectively, symptom durations ranged from 1.5 days to 90 months. Cervical herniation (72.6%) accounted for the majority of cervical angina cases, and surgical interventions (84.4%) predominated over physical therapy (13.0%) and medical management strategies (9.1%). Every patient assessed at follow-up reported relief from symptoms related to cervical angina.
Cervical angina is a noncardiac syndrome of chest pain associated with a broad range of cervical and thoracic spinal pathologies, the most common of which is cervical disc herniation. Although difficult to diagnose, it can be successfully treated when identified through first-line conservative management or surgical interventions in refractory cases.
Shane Shahrestani, Brandon M. Lehrich, Ali R. Tafreshi, Nolan J. Brown, Brian V. Lien, Seth Ransom, Ryan C. Ransom, Alexander M. Ballatori, Andy Ton, Xiao T. Chen, and Ronald Sahyouni
Frailty is a clinical state of increased vulnerability due to age-associated decline and has been well established as a perioperative risk factor. Geriatric patients have a higher risk of frailty, higher incidence of brain cancer, and increased postoperative complication rates compared to nongeriatric patients. Yet, literature describing the effects of frailty on short- and long-term complications in geriatric patients is limited. In this study, the authors evaluate the effects of frailty in geriatric patients receiving cranial neurosurgery for a primary CNS neoplasm.
The authors conducted a retrospective cohort study of geriatric patients receiving cranial neurosurgery for a primary CNS neoplasm between 2010 and 2017 by using the Nationwide Readmission Database. Demographics and frailty were queried at primary admission, and readmissions were analyzed at 30-, 90-, and 180-day intervals. Complications of interest included infection, anemia, infarction, kidney injury, CSF leak, urinary tract infection, and mortality. Nearest-neighbor propensity score matching for demographics was implemented to identify nonfrail control patients with similar diagnoses and procedures. The analysis used Welch two-sample t-tests for continuous variables and chi-square test with odds ratios.
A total of 6713 frail patients and 6629 nonfrail patients were identified at primary admission. At primary admission, frail geriatric patients undergoing cranial neurosurgery had increased odds of developing acute posthemorrhagic anemia (OR 1.56, 95% CI 1.23–1.98; p = 0.00020); acute infection (OR 3.16, 95% CI 1.70–6.36; p = 0.00022); acute kidney injury (OR 1.32, 95% CI 1.07–1.62; p = 0.0088); urinary tract infection prior to discharge (OR 1.97, 95% CI 1.71–2.29; p < 0.0001); acute postoperative cerebral infarction (OR 1.57, 95% CI 1.17–2.11; p = 0.0026); and mortality (OR 1.64, 95% CI 1.22–2.24; p = 0.0012) compared to nonfrail geriatric patients receiving the same procedure. In addition, frail patients had a significantly increased inpatient length of stay (p < 0.0001) and all-payer hospital cost (p < 0.0001) compared to nonfrail patients at the time of primary admission. However, no significant difference was found between frail and nonfrail patients with regard to rates of infection, thromboembolism, CSF leak, dural tear, cerebral infarction, acute kidney injury, and mortality at all readmission time points.
Frailty may significantly increase the risks of short-term acute complications in geriatric patients receiving cranial neurosurgery for a primary CNS neoplasm. Long-term analysis revealed no significant difference in complications between frail and nonfrail patients. Further research is warranted to understand the effects and timeline of frailty in geriatric patients.
Nolan J. Brown, Elliot H. Choi, Julian L. Gendreau, Vera Ong, Alexander Himstead, Brian V. Lien, Shane Shahrestani, Seth C. Ransom, Katelynn Tran, Ali R. Tafreshi, Ronald Sahyouni, Alvin Chan, and Michael Y. Oh
Tranexamic acid (TXA) is an antifibrinolytic agent associated with reduced blood loss and mortality in a wide range of procedures, including spine surgery, traumatic brain injury, and craniosynostosis. Despite this wide use, the safety and efficacy of TXA in spine surgery has been considered controversial due to a relative scarcity of literature and lack of statistical power in reported studies. However, if TXA can be shown to reduce blood loss in laminectomy with fusion and posterior instrumentation, more surgeons may include it in their armamentarium. The authors aimed to conduct an up-to-date systematic review and meta-analysis of the efficacy of TXA in reducing blood loss in laminectomy and fusion with posterior instrumentation.
A systematic review and meta-analysis, abiding by PRISMA guidelines, was performed by searching the databases of PubMed, Web of Science, and Cochrane. These platforms were queried for all studies reporting the use of TXA in laminectomy and fusion with posterior instrumentation. Variables retrieved included patient demographics, surgical indications, involved spinal levels, type of laminectomy performed, TXA administration dose, TXA route of administration, operative duration, blood loss, blood transfusion rate, postoperative hemoglobin level, and perioperative complications. Heterogeneity across studies was evaluated using a chi-square test, Cochran’s Q test, and I2 test performed with R statistical programming software.
A total of 7 articles were included in the qualitative study, while 6 articles featuring 411 patients underwent statistical analysis. The most common route of administration for TXA was intravenous with 15 mg/kg administered preoperatively. After the beginning of surgery, TXA administration patterns were varied among studies. Blood transfusions were increased in non-TXA cohorts compared to TXA cohorts. Patients administered TXA demonstrated a significant reduction in blood loss (mean difference −218.44 mL; 95% CI −379.34 to −57.53; p = 0.018). TXA administration was not associated with statistically significant reductions in operative durations. There were no adverse events reported in either the TXA or non-TXA patient cohorts.
TXA can significantly reduce perioperative blood loss in cervical, thoracic, and lumbar laminectomy and fusion procedures, while demonstrating a minimal complication profile.
Ali O. Jamshidi, André Beer-Furlan, Daniel M. Prevedello, Ronald Sahyouni, Mohamed A. Elzoghby, Mina G. Safain, Ricardo L. Carrau, John A. Jane Jr., and Edward R. Laws
The endoscopic endonasal approach has been proposed as a primary surgical strategy for select craniopharyngiomas. However, those tumors that arise from the sella have not been classified with the other craniopharyngioma subtypes in terms of surgical nuances, intraoperative findings, and postoperative outcomes. The authors describe their experience with a select subtype of craniopharyngioma arising within the sella subjacent to the diaphragma sellae and refer to these tumors as type 0.
After obtaining IRB approval, three institutions retrospectively reviewed their data collected from 2005 to 2017. Patients eligible for inclusion in the study were those who had tumors that originated from the sella inferior to the diaphragma sellae. Demographic, clinical, radiological, surgical, and follow-up data were examined and analyzed.
Twenty-eight patients (average age 19.3 years, range 3–60 years) were included in this study. Sixteen patients (57%) were younger than 18 years of age. All patients had characteristic imaging features of an expanded sella. Seventy-five percent of the patients presented with some form of visual field deficit (89% had radiographic optic apparatus compression) and 39% with hypopituitarism. The average maximal tumor diameter in the axial, coronal, or sagittal plane was 3.1 cm. Gross-total resection was achieved in 82% of the patients. Twenty-one percent of patients experienced an iatrogenic complication, and there were only two cases (7%) of postoperative cerebrospinal fluid (CSF) leakage. Only two patients (7%) required the use of a nasoseptal flap as part of their original reconstruction. Pathology was uniformly the adamantinomatous subtype. Postoperative objective visual outcomes were improved in 71% of the patients with visual symptoms or visual field deficits on presentation and stable in 24%. Mean follow-up was 45.1 months (range 3–120 months) with an 18% recurrence rate at a mean of 44.4 months (range 10–84 months). One patient was lost to follow-up. Thirty-six percent of patients received postoperative radiation to treat recurrence or residual tumor. Endocrine data are also presented.
Craniopharyngiomas that originate within the sella below the diaphragma sellae are a select subtype characterized by 1) an enlarged sella, 2) an intact diaphragma sellae at surgery, and 3) an adamantinomatous pathology. These tumors can be treated transnasally without the absolute need for neurovascular flap reconstruction, as there is a low risk of CSF leakage.
Matthew A. Liu, Julian L. Gendreau, Joshua J. Loya, Nolan J. Brown, Amber Keith, Ronald Sahyouni, Mickey E. Abraham, David Gonda, and Michael L. Levy
Chordomas are rare malignant neoplasms that develop from the primitive notochord with < 5% of the tumors occurring in pediatric patients younger than the age of 20. Of these pediatric chordomas, those affecting the craniocervical junction (C1–C2) are even more rare; therefore, parameters for surgical management of these pediatric tumors are not well characterized.
In this case, a 3-year-old male was found to have a clival chordoma on imaging with extension to the craniocervical junction resulting in spinal cord compression. Endoscopic-assisted transoral transclival approach for clival tumor resection was performed first. As a second stage, the patient underwent a left-sided far lateral craniotomy and cervical laminectomy for resection of the skull base chordoma and instrumented fusion of the occiput to C3. He made excellent improvements in strength and dexterity during rehab and was discharged after 3 weeks.
In pediatric patients with chordoma with extension to the craniocervical junction and spinal cord compression, decompression with additional occipito-cervical fusion appears to offer a good clinical outcome. Fusion performed as a separate surgery before or at the same time as the initial tumor resection surgery may lead to better outcomes.