Tumoral calcinosis (TC), a calcium hydroxyapatite–based mass, is common in the extremities and hips, but has rarely been reported in the spine, and has never been reported within the spinal cord. It may occur sporadically, in familial form, or as a consequence of disorders that promote soft-tissue calcification. Gross-total resection appears to be curative, but the diagnosis of TC is rarely considered prior to surgery. In this report, the authors describe the management of the first case of intramedullary TC located at the T-5 level in a 20-month-old boy who presented with lower-extremity spasticity. Additionally, salient features of the TC diagnosis, radiological patterns, histological findings, treatment, and outcomes are discussed.
Vivek A. Mehta, Chetan Bettegowda and George I. Jallo
Tej D. Azad, Michael C. Jin, Lydia J. Bernhardt and Chetan Bettegowda
Diffuse midline glioma (DMG) is a highly malignant childhood tumor with an exceedingly poor prognosis and limited treatment options. The majority of these tumors harbor somatic mutations in genes encoding histone variants. These recurrent mutations correlate with treatment response and are forming the basis for molecularly guided clinical trials. The ability to detect these mutations, either in circulating tumor DNA (ctDNA) or cerebrospinal fluid tumor DNA (CSF-tDNA), may enable noninvasive molecular profiling and earlier prediction of treatment response. Here, the authors review ctDNA and CSF-tDNA detection methods, detail recent studies that have explored detection of ctDNA and CSF-tDNA in patients with DMG, and discuss the implications of liquid biopsies for patients with DMG.
Vivek A. Mehta, Chetan Bettegowda, George I. Jallo and Edward S. Ahn
Craniosynostosis, the premature closure of cranial sutures, has been known to exist for centuries, but modern surgical management has only emerged and evolved over the past 100 years. The success of surgery for this condition has been based on the recognition of scientific principles that dictate brain and cranial growth in early infancy and childhood. The evolution of strip craniectomies and suturectomies to extensive calvarial remodeling and endoscopic suturectomies has been driven by a growing understanding of how a prematurely fused cranial suture can affect the growth and shape of the entire skull. In this review, the authors discuss the early descriptions of craniosynostosis, describe the scientific principles upon which surgical intervention was based, and briefly summarize the eras of surgical management and their evolution to present day.
Chikezie I. Eseonu, C. Rory Goodwin, Xin Zhou, Debebe Theodros, Matthew T. Bender, Dimitrios Mathios, Chetan Bettegowda and Michael Lim
Calcium phosphate cement provides a biomaterial that can be used for calvarial reconstruction in a retrosigmoid craniectomy for microvascular decompression (MVD). This study evaluates the outcomes of postoperative CSF leak and wound infection for patients undergoing a complete cranioplasty using calcium phosphate cement versus incomplete cranioplasty using polyethylene titanium mesh following a retrosigmoid craniectomy for MVD.
The authors evaluated 211 cases involving patients who underwent first-time retrosigmoid craniectomies performed by a single attending surgeon fortrigeminal neuralgia from October 2008 to June 2014. From this patient population, 111 patients underwent calvarial reconstruction after retrosigmoid craniectomy using polyethylene titanium mesh, and 100 patients had reconstructions using calcium phosphate cement. A Pearson’s chi-square test was used to compare postoperative complications of CSF leak and wound infection in these 2 types of cranioplasties.
The polyethylene titanium mesh group included 5 patients (4.5%) with postoperative CSF leak or pseudomeningocele and 3 patients (2.7%) with wound infections. In the calcium phosphate cement group, no patients had a CSF leak, and 2 patients (2%) had wound infections. This represented a statistically significant reduction of postoperative CSF leak in patients who underwent calcium phosphate reconstructions of their calvarial defect compared with those who underwent polyethylene titanium mesh reconstructions (p = 0.03). No significant difference was seen between the 2 groups in the number of patients with postoperative wound infections.
Calcium phosphate cement provides a viable alternative biomaterial for calvarial reconstruction of retrosigmoid craniectomy defects in patients who have an MVD. The application of this material provides a biocompatible barrier that reduces the incidence of postoperative CSF leaks.
Timothy Y. Kim, Christopher M. Jackson, Yuanxuan Xia, Leila A. Mashouf, Kisha K. Patel, Eileen S. Kim, Alice L. Hung, Adela Wu, Tomas Garzon-Muvdi, Matthew T. Bender, Chetan Bettegowda, John Y. K. Lee and Michael Lim
Trigeminal neuralgia (TN) is a neuropathic pain disorder characterized by severe, lancinating facial pain that is commonly treated with neuropathic medication, percutaneous rhizotomy, and/or microvascular decompression (MVD). Patients who are not found to have distinct arterial compression during MVD present a management challenge. In 2013, the authors reported on a small case series of such patients in whom glycerin was injected intraoperatively into the cisternal segment of the trigeminal nerve. The objective of the authors’ present study was to report their updated experience with this technique to further validate this novel approach.
The authors performed a retrospective analysis of data obtained in patients in whom glycerin was directly injected into the inferior third of the cisternal portion of the trigeminal nerve. Seventy-four patients, including 14 patients from the authors’ prior study, were identified, and demographic information, intraoperative findings, postoperative course, and complications were recorded. Fisher’s exact test, unpaired t-tests, and Kaplan-Meier survival curves using Mantel log-rank test were used to compare the 74 patients with a cohort of 476 patients who received standard MVD by the same surgeon.
The 74 patients who underwent MVD and glycerin injection had an average follow-up of 19.1 ± 18.0 months, and the male/female ratio was 1:2.9. In 33 patients (44.6%), a previous intervention for TN had failed. On average, patients had an improvement in the Barrow Neurological Institute Pain Intensity score from 4.1 ± 0.4 before surgery to 2.1 ± 1.2 after surgery. Pain improvement after the surgery was documented in 95.9% of patients. Thirteen patients (17.6%) developed burning pain following surgery. Five patients developed complications (6.7%), including incisional infection, facial palsy, CSF leak, and hearing deficit, all of which were minor.
Intraoperative injection of glycerin into the trigeminal nerve is a generally safe and potentially effective treatment for TN when no distinct site of arterial compression is identified during surgery or when decompression of the nerve is deemed to be inadequate.
Chetan Bettegowda, Owoicho Adogwa, Vivek Mehta, Kaisorn L. Chaichana, Jon Weingart, Benjamin S. Carson, George I. Jallo and Edward S. Ahn
Choroid plexus tumors (CPTs) are rare intracranial neoplasms that constitute approximately 2%–5% of all pediatric brain tumors. Most of these tumors present with severe hydrocephalus. The optimal perioperative management and oncological care remain a matter of debate. The authors present the epidemiological and clinical features of CPTs from a 20-year single-institutional experience.
A total of 39 consecutive patients with pathologically proven CPTs (31 choroid plexus papillomas [CPPs] and 8 choroid plexus carcinomas [CPCs]) were included in this series. Patient demographics, clinical presentation, comorbidities, indications for surgery, radiological studies, tumor location, and all operative variables were reviewed for each case. Multivariate regression analysis was performed to identify independent predictors of tumor recurrence and survival.
The overall mean age (± SD) was 13.13 ± 19.59 years (15.27 ± 21.10 years in the CPP group and 3.66 ± 3.59 years in the CPC group). Hydrocephalus was noted at presentation in 34% of patients. The most common presenting symptoms were headache (32%) and nausea/vomiting (26%). Gross-total resection (GTR) was achieved in 86% of CPPs and in 71% of CPCs (p = 0.57). There was 100% survival in patients with CPPs observed at the 5- and 10-year follow-up and 71% survival in patients with CPCs at the 5-year follow-up. In a multivariate regression analysis, a diagnosis of papilloma, preoperative vision changes, or hydrocephalus; right ventricle tumor location; and GTR were all independently associated with a decreased likelihood of tumor recurrence at last follow-up.
The authors' study suggests that patients with CPCs are more likely to experience local recurrence and metastasis; hence, GTR with chemotherapy and radiotherapy, particularly for CPCs, is pivotal in preventing recurrence and prolonging survival. While GTR was important for local control following resection of CPPs, it had a minimal effect on prolonging survival in this patient cohort.
Debebe Theodros, C. Rory Goodwin, Matthew T. Bender, Xin Zhou, Tomas Garzon-Muvdi, Rafael De la Garza-Ramos, Nancy Abu-Bonsrah, Dimitrios Mathios, Ari M. Blitz, Alessandro Olivi, Benjamin Carson, Chetan Bettegowda and Michael Lim
Trigeminal neuralgia (TN) is characterized by intermittent, paroxysmal, and lancinating pain along the distribution of the trigeminal nerve. Microvascular decompression (MVD) directly addresses compression of the trigeminal nerve. The purpose of this study was to determine whether patients undergoing MVD as their first surgical intervention experience greater pain control than patients who undergo subsequent MVD.
A retrospective review of patient records from 1998 to 2015 identified a total of 942 patients with TN and 500 patients who underwent MVD. After excluding several cases, 306 patients underwent MVD as their first surgical intervention and 175 patients underwent subsequent MVD. Demographics and clinicopathological data and outcomes were obtained for analysis.
In patients who underwent subsequent MVD, surgical intervention was performed at an older age (55.22 vs 49.98 years old, p < 0.0001) and the duration of symptoms was greater (7.22 vs 4.45 years, p < 0.0001) than for patients in whom MVD was their first surgical intervention. Patients who underwent initial MVD had improved pain relief and no improvement in pain rates compared with those who had subsequent MVD (95.8% and 4.2% vs 90.3% and 9.7%, respectively, p = 0.0041).
Patients who underwent initial MVD had significantly lower rates of facial numbness in the pre- and postoperative periods compared with patients who underwent subsequent MVD (p < 0.0001). The number of complications in both groups was similar (p = 0.4572).
The results demonstrate that patients who underwent other procedures prior to MVD had less pain relief and a higher incidence of facial numbness despite rates of complications similar to patients who underwent MVD as their first surgical intervention.
Daniel M. Sciubba, Rafael De la Garza Ramos, C. Rory Goodwin, Nancy Abu-Bonsrah, Ali Bydon, Timothy F. Witham, Chetan Bettegowda, Ziya L. Gokaslan and Jean-Paul Wolinsky
The goal of this study was to investigate the local recurrence rate and long-term survival after resection of spinal sarcomas.
A retrospective review of patients who underwent resection of primary or metastatic spinal sarcomas between 1997 and 2015 was performed. Tumors were classified according to the Enneking classification, and resection was categorized as Enneking appropriate (EA) if the specimen margins matched the Enneking recommendation, and as Enneking inappropriate (EI) if they did not match the recommendation. The primary outcome measure for all tumors was overall survival; local recurrence was also an outcome measure for primary sarcomas. The association between clinical, surgical, and molecular (tumor biomarker) factors and outcomes was also investigated.
A total of 60 patients with spinal sarcoma were included in this study (28 men and 32 women; median age 38 years). There were 52 primary (86.7%) and 8 metastatic sarcomas (13.3%). Thirty-nine tumors (65.0%) were classified as high-grade, and resection was considered EA in 61.7% of all cases (n = 37). The local recurrence rate was 10 of 52 (19.2%) for primary sarcomas; 36.8% for EI resection and 9.1% for EA resection (p = 0.010). Twenty-eight patients (46.7%) died during the follow-up period, and median survival was 26 months. Overall median survival was longer for patients with EA resection (undefined) compared with EI resection (13 months, p < 0.001). After multivariate analysis, EA resection significantly decreased the hazard of local recurrence (HR 0.24, 95% CI 0.06–0.93; p = 0.039). Age 40 years or older (HR 4.23, 95% CI 1.73–10.31; p = 0.002), previous radiation (HR 3.44, 95% CI 1.37–8.63; p = 0.008), and high-grade sarcomas (HR 3.17, 95% CI 1.09–9.23; p = 0.034) were associated with a significantly increased hazard of death, whereas EA resection was associated with a significantly decreased hazard of death (HR 0.22, 95% CI 0.09–0.52; p = 0.001).
The findings in the present study suggest that EA resection may be the strongest independent prognostic factor for improved survival in patients with spinal sarcoma. Additionally, patients who underwent EA resection had lower local recurrence rates. Patients 40 years or older, those with a history of previous radiation, and those with high-grade tumors had an increased hazard of mortality in this study.
Vivek A. Mehta, Chetan Bettegowda, Sebastian A. Ahmadi, Petra Berenberg, Ulrich-Wilhelm Thomale, Ernst-Johannes Haberl, George I. Jallo and Edward S. Ahn
Symptom response to spinal cord untethering, and the impact of duraplasty and scoliosis on retethering, are poorly understood in tethering after myelomeningocele (MMC) repair. In this retrospective study, the authors examined the outcomes of children who developed first-time spinal cord tethering following MMC repair. The response of symptoms to untethering and the role of duraplasty and scoliosis in retethering are explored.
The authors performed a review of 54 children with first-time symptomatic spinal cord tethering following MMC repair to determine the impact of untethering on symptoms, the impact of dural repair type on retethering, and the role of scoliosis on the prevalence and time to retethering.
The average patient age was 10.3 ± 4.9 years, and 44% were males. The most common presenting symptoms of tethered cord syndrome were urinary (87%), motor (80%), gait (78%), and sensory (61%) dysfunction. The average postoperative time to symptom improvement was 2.02 months for sensory symptoms, 3.21 months for pain, 3.50 months for urinary symptoms, and 4.48 months for motor symptoms, with sensory improvement occurring significantly earlier than motor improvement (p = 0.02). At last follow-up (an average of 47 months), motor symptoms were improved in 26%, maintained in 62%, and worsened in 11%; for sensory symptoms, these rates were 26%, 71%, and 3%, respectively; for pain, 28%, 65%, and 7%, respectively; and for urinary symptoms, 17%, 76%, and 7%, respectively. There was no difference in symptom response with type of dural repair (primary closure vs duraplasty). Symptomatic retethering occurred in 17 (31%) of 54 patients, but duration of symptoms, age at surgery, and type of dural repair were not associated with retethering. Scoliosis was not associated with an increased prevalence of retethering, but was associated with significantly earlier retethering (32.5 vs 61.1 months; p = 0.042) in patients who underwent additional untethering operations.
Symptomatic retethering is a common event after MMC repair. In the authors' experience, sensory improvements occur sooner than motor improvements following initial untethering. Symptom response rates were not altered by type of dural closure. Scoliosis was associated with significantly earlier retethering and should be kept in mind when caring for individuals who have had previous MMC repair.
Daniel M. Sciubba, Mohamed Macki, Mohamad Bydon, Niccole M. Germscheid, Jean-Paul Wolinsky, Stefano Boriani, Chetan Bettegowda, Dean Chou, Alessandro Luzzati, Jeremy J. Reynolds, Zsolt Szövérfi, Patti Zadnik, Laurence D. Rhines, Ziya L. Gokaslan, Charles G. Fisher and Peter Paul Varga
Clinical outcomes in patients with primary spinal osteochondromas are limited to small series and sporadic case reports. The authors present data on the first long-term investigation of spinal osteochondroma cases.
An international, multicenter ambispective study on primary spinal osteochondroma was performed. Patients were included if they were diagnosed with an osteochondroma of the spine and received surgical treatment between October 1996 and June 2012 with at least 1 follow-up. Perioperative prognostic variables, including patient age, tumor size, spinal level, and resection, were analyzed in reference to long-term local recurrence and survival. Tumor resections were compared using Enneking appropriate (EA) or Enneking inappropriate surgical margins.
Osteochondromas were diagnosed in 27 patients at an average age of 37 years. Twenty-two lesions were found in the mobile spine (cervical, thoracic, or lumbar) and 5 in the fixed spine (sacrum). Twenty-three cases (88%) were benign tumors (Enneking tumor Stages 1–3), whereas 3 (12%) exhibited malignant changes (Enneking tumor Stages IA–IIB). Sixteen patients (62%) underwent en bloc treatment—that is, wide or marginal resection—and 10 (38%) underwent intralesional resection. Twenty-four operations (92%) followed EA margins. No one received adjuvant therapy. Two patients (8%) experienced recurrences: one in the fixed spine and one in the mobile spine. Both recurrences occurred in latent Stage 1 tumors following en bloc resection. No osteochondroma-related deaths were observed.
In the present study, most patients underwent en bloc resection and were treated as EA cases. Both recurrences occurred in the Stage 1 tumor cohort. Therefore, although benign in character, osteochondromas still require careful management and thorough follow-up.