Giant plexiform neurofibroma (GPNF) of the scalp is an extremely rare lesion reported in association with neurofibromatosis. Occipital location of GPNF is even more infrequent, especially in association with occipital dysplasia (OD). The authors report 2 pediatric cases of GPNF associated with OD. The first case had an associated meningoencephalocele, and the second had large vascular channels within the lesion and the dominant ipsilateral transverse sinus lying in the center of the calvarial defect. The authors present these 2 unusual cases with a review of literature and discuss the radiological findings, theories of etiopathogenesis of the OD, and management dilemmas.
Ravi Dadlani, Venkatraman Sadanand, Nandita Ghosal and Alangar S. Hegde
Sumit Thakar, Narayanam Anantha Sai Kiran and Alangar S. Hegde
Spinal extradural arachnoid cysts (ACs) have an infrequent predilection for the sacrum. As with their counterparts in other regions of the spine, cysts in this location are mostly asymptomatic. Common presentations in symptomatic cases include pain in the low back or perineum, radiculopathy, and sphincteric dysfunction. The authors report a hitherto undescribed presentation in which the predominant symptoms are those related to an associated holocord syrinx. This 15-year-old boy presented with fluctuating, spastic paraparesis and a dissociated sensory loss in the trunk. Admission MR imaging of the spine showed an extradural AC from S-2 to S-4 and a holocord, nonenhancing syrinx. The patient underwent S-2 laminectomy, fenestration of the cyst, and partial excision of its wall. Intradural exploration revealed a normal-looking filum terminale and the absence of any dural communication with the cyst. At a follow-up visit 6 months after surgery, his motor and sensory deficits had resolved. Follow-up MR imaging showed complete resolution of the syrinx in the absence of the sacral AC. This is the first report of a sacral extradural AC causing holocord syringomyelia. Because conventional theories of syrinx formation were not helpful in elucidating this case, a hypothesis is postulated to explain the clinicoradiological oddity.
Sumit Thakar, Yasha T. Chickabasaviah and Alangar S. Hegde
Invasive craniocerebral aspergillosis, often encountered in an immunocompromised setting, is almost uniformly fatal despite radical surgical and medical management, and is frequently a necropsy finding. The authors report a unique, self-resolving clinical course of this aggressive infection in a 10-month-old infant. The infant was brought to the emergency services in altered sensorium with a 1-week history of left-sided hemiparesis, excessive irritability, and vomiting. An MRI study of the brain revealed multiple, heterogeneously enhancing lesions in the right cerebral hemisphere with mass effect. The largest lesion in the frontotemporal cortical and subcortical regions was decompressed on an emergent basis. Histopathological findings were suggestive of invasive aspergillosis, although there was no evidence of the infection in the lungs or paranasal sinuses. Computed tomography–guided aspiration of the remaining lesions and follow-up antifungal therapy were recommended. The parents, however, requested discharge without further treatment. The child was seen at a follow-up visit 3 years later without having received any antifungal treatment. Imaging showed resolution of the infection and features of Dyke-Davidoff-Masson syndrome (cerebral hemiatrophy). This report of invasive cerebral aspergillosis resolving without medical therapy is the first of its kind. Its clinicoradiological aspects are discussed in light of previously reported cases.
Sunil V. Furtado, Sumit Thakar, Ganesh K. Murthy, Ravi Dadlani and Alangar S. Hegde
A giant spinal arachnoid cyst is an unusual cause of progressive epidural compressive syndrome. The authors describe 4 cases of a “complex” subtype of this lesion and discuss aspects of surgical management. The patients presented with progressive spastic paraparesis and were found to harbor extensive spinal extradural arachnoid cysts with multiple septations and significant paraspinal extensions. Extensive laminotomy and excision of the cyst along with its extensions were performed in all cases.
Compared with previously indexed cases of surgically managed extensive spinal extradural arachnoid cysts, the cases reported here are unique because of their complex nature. Curative treatment consists of radical excision inclusive of the paraspinal extensions as well as closure of a dural defect, if found. A laminotomy or laminoplasty should be performed to avoid postoperative instability related to the extensive exposure. Extended follow-up and instrumentation may be required in select cases.
Sumit Thakar, Avinash Kurudi Siddappa, Saritha Aryan, Dilip Mohan, Narayanam Anantha Sai Kiran and Alangar S. Hegde
The mesodermal derangement in Chiari Type I malformation (CMI) has been postulated to encompass the cervical spine. The objectives of this study were to assess the cross-sectional areas (CSAs) of cervical paraspinal muscles (PSMs) in patients with CMI without syringomyelia, compare them with those in non-CMI subjects, and evaluate their correlations with various factors.
In this retrospective study, the CSAs of cervical PSMs in 25 patients were calculated on T2-weighted axial MR images and computed as ratios with respect to the corresponding vertebral body areas. These values and the cervical taper ratios were then compared with those of age- and sex-matched non-CMI subjects and analyzed with respect to demographic data and clinicoradiological factors.
Compared with the non-CMI group, the mean CSA values for the rectus capitis minor and all of the subaxial PSMs were lower in the study group, and those of the deep extensors were significantly lower (p = 0.004). The cervical taper ratio was found to be significantly higher in the study cohort (p = 0.0003). A longer duration of symptoms and a steeper cervical taper ratio were independently associated with lower CSA values for the deep extensors (p = 0.04 and p = 0.03, respectively). The presence of neck pain was associated with a lower CSA value for the deep flexors (p = 0.03).
Patients with CMI demonstrate alterations in their cervical paraspinal musculature even in the absence of coexistent syringomyelia. Their deep extensor muscles undergo significant atrophic changes that worsen with the duration of their symptoms. This could be related to a significantly steeper cervical taper ratio that their cervical cords are exposed to. Neck pain in these patients is related to atrophy of their deep flexor muscles. A steeper cervical taper ratio and alterations in the PSMs could be additional indicators for surgery in patients with CMI without syringomyelia.
Sumit Thakar, Laxminadh Sivaraju, Saritha Aryan, Dilip Mohan, Narayanam Anantha Sai Kiran and Alangar S. Hegde
The objective of this study was to assess the cross-sectional areas (CSAs) of lumbar paraspinal muscles in adults with isthmic spondylolisthesis (IS), to compare them with those in the normative population, and to evaluate their correlations with demographic factors and MRI changes in various spinal elements.
The authors conducted a retrospective study of patients who had undergone posterior lumbar interbody fusion for IS, and 2 of the authors acting as independent observers calculated the CSAs of various lumbar paraspinal muscles (psoas, erector spinae [ES], multifidus [MF]) on preoperative axial T2-weighted MR images from the L-3 to L-5 vertebral levels and computed the CSAs as ratios with respect to the corresponding vertebral body areas. These values were then compared with those in an age- and sex-matched normative population and were analyzed with respect to age, sex, duration of symptoms, grade of listhesis, and various MRI changes at the level of the listhesis (pedicle signal change, disc degeneration, and facetal arthropathy).
Compared with values in normative controls, the mean CSA value for the ES muscle was significantly higher in the study cohort of 120 patients (p = 0.002), whereas that for the MF muscle was significantly lower (p = 0.009), and more so in the patients with PSC (p = 0.002). Magnetic resonance imaging signal change in the pedicle was seen in half of the patients, all of whom demonstrated a Type 2 change. Of the variables tested in a multivariate analysis, age independently predicted lower area values for all 3 muscles (p ≤ 0.001), whereas female sex predicted a lower mean psoas area value (p < 0.001). None of the other variables significantly predicted any of the muscle area values. A decrease in the mean MF muscle area value alone was associated with a significantly increased likelihood of a PSC (p = 0.039).
Compared with normative controls, patients with IS suffer selective atrophy of their MF muscle, whereas their ES muscle undergoes a compensatory hypertrophy. Advancing age has a detrimental effect on the areas of the lumbar PSMs, whereas female sex predisposes to a decreased psoas muscle area. Multifidus muscle atrophy correlates with PSC, indicating the role of this deep stabilizer in the biomechanical stability of spondylolisthetic spines. This may be of clinical significance in targeted physiotherapy programs during the conservative management of IS.
Umesh Srikantha, Kiran S. Khanapure, Aniruddha T. Jagannatha, Krishna C. Joshi, Ravi G. Varma and Alangar S. Hegde
Minimally invasive techniques are being increasingly used to treat disorders of the cervical spine. They have a potential to reduce the postoperative neck discomfort subsequent to extensive muscle dissection associated with conventional atlantoaxial fusion procedures. The aim of this paper was to elaborate on the technique and results of minimally invasive atlantoaxial fusion.
Minimally invasive atlantoaxial fusion was done initially in 4 fresh-frozen cadavers and subsequently in 5 clinical cases. Clinical cases included patients with reducible atlantoaxial instability and undisplaced or minimally displaced odontoid fractures. The surgical technique is illustrated in detail.
Among the cadaveric specimens, all C-1 lateral mass screws were in the correct position and 2 of the 8 C-2 screws had a vertebral canal breach. Among clinical cases, all C-1 lateral mass screws were in the correct position. Only one C-2 screw had a Grade 2 vertebral canal breach, which was clinically insignificant. None of the patients experienced neurological worsening or implant-related complications at follow-up. Evidence of rib graft fusion or C1–2 joint fusion was successfully demonstrated in 4 cases, and flexion-extension radiographs done at follow-up did not show mobility in any case.
Minimally invasive atlantoaxial fusion is a safe and effective alternative to the conventional approach in selected cases. Larger series with direct comparison to the conventional approach will be required to demonstrate clinical benefit presumed to be associated with a minimally invasive approach.
Sumit Thakar, Niranjana Rajagopal, Subramaniyan Mani, Maya Shyam, Saritha Aryan, Arun S. Rao, Rakshith Srinivasa, Dilip Mohan and Alangar S. Hegde
The utility of telemedicine (TM) in neurosurgery is underexplored, with most of the studies relating to teletrauma or telestroke programs. In this study, the authors evaluate the cost-effectiveness of TM consultations for follow-up care of a large population of patients who underwent neurosurgical procedures.
A decision-analytical model was used to assess the cost-effectiveness of TM for elective post–neurosurgical care patients from a predominantly nonurban cohort in West Bengal, India. The model compared TM care via a nodal center in West Bengal to routine, in-person, per-episode care at the provider site in Bangalore, India. Cost and effectiveness data relating to 1200 patients were collected for a 52-month period. The effectiveness of TM care was calculated using efficiency in terms of the percentage of successful TM consultations, as well as patient-perceived utility values for overall experience of the type of health care access that they received. Incremental cost-effectiveness ratio (ICER) analysis was done using the 4-quadrant charting of the cost-effectiveness plane. One-way sensitivity and tornado analyses were performed to identify thresholds where the care strategy would change.
The overall utility for the 3 TM scenarios was found to be higher (89%) than for the utility of routine care (80%). TM was found to be more cost-effective (Indian rupee [INR] 2630 per patient) compared to routine care (INR 6848 per patient). The TM strategy “dominates” that of routine care by being more effective and less expensive (ICER value of -39,400 INR/unit of effectiveness). Sensitivity analysis revealed that cost-effectiveness of TM was most sensitive to changes in the number of TM patients, utility and success rate of TM, and travel distance to the TM center.
TM care dominates the in-person care strategy by providing more effective and less expensive follow-up care for a remote post–neurosurgical care population in India. In the authors’ setting, this benefit of TM is sustainable even if half the TM consultations turn out to be unsuccessful. The viability of TM as a cost-effective care protocol is attributed to a combination of factors, like an adequate patient volume utilizing TM, patient utility, success rate of TM, and the patient travel distance.
Sumit Thakar, Dilip Mohan, Sunil V. Furtado, Narayanam Anantha Sai Kiran, Ravi Dadlani, Saritha Aryan, Arun S. Rao and Alangar S. Hegde
The objective of this study was to assess the cross-sectional areas (CSAs) of the superficial, deep flexor (DF), and deep extensor (DE) paraspinal muscles in patients with cervical spondylotic myelopathy (CSM), and to evaluate their correlations with functional status and sagittal spinal alignment changes following central corpectomy with fusion and plating.
In this retrospective study of 67 patients who underwent central corpectomy with fusion and plating for CSM, the CSAs of the paraspinal muscles were calculated on the preoperative T2-weighted axial MR images and computed as ratios with respect to the corresponding vertebral body areas (VBAs) and as flexor/extensor CSA ratios. These ratios were then compared with those in the normative population and analyzed with respect to various clinicoradiological factors, including pain status, Nurick grade, and segmental angle change at follow-up (SACF).
The mean CSA values for all muscle groups and the DF/DE ratio were significantly lower in the study cohort compared with an age- and sex-matched normative study group (p < 0.001). Among various independent variables tested in a multivariate regression analysis, increasing age and female sex significantly predicted a lower total extensor CSA/VBA ratio (p < 0.001), while a longer duration of symptoms significantly predicted a greater total flexor/total extensor CSA ratio (p = 0.02). In patients undergoing single-level corpectomy, graft subsidence had a positive correlation with SACF in all patients (p < 0.05), irrespective of the preoperative segmental angle and curvature, while in patients undergoing 2-level corpectomy, graft subsidence demonstrated such a correlation only in the subgroup with lordotic curvatures (p = 0.02). Among the muscle area ratios, the DF/DE ratio demonstrated a negative correlation with SACF in the subgroup with preoperative straight or kyphotic segmental angles (p = 0.04 in the single corpectomy group, p = 0.01 in the 2-level corpectomy group). There was no correlation of any of the muscle ratios with change in Nurick grade.
Patients with CSM demonstrate significant atrophy in all the flexor and extensor paraspinal muscles, and also suffer a reduction in the protective effect of a strong DF/DE CSA ratio. Worsening of this ratio significantly correlates with greater segmental kyphotic change in some patients. A physiological mechanism based on DF dysfunction is discussed to elucidate these findings that have implications in preventive physiotherapy and rehabilitation of patients with CSM. Considering that the influence of a muscle ratio was significant only in patients with hypolordosis, a subgroup that is known to have facetal ligament laxity, it may also be postulated that ligamentous support supersedes the influence of paraspinal muscles on postoperative sagittal alignment in CSM.
Sumit Thakar, Laxminadh Sivaraju, Kuruthukulangara S. Jacob, Aditya Atal Arun, Saritha Aryan, Dilip Mohan, Narayanam Anantha Sai Kiran and Alangar S. Hegde
Although various predictors of postoperative outcome have been previously identified in patients with Chiari malformation Type I (CMI) with syringomyelia, there is no known algorithm for predicting a multifactorial outcome measure in this widely studied disorder. Using one of the largest preoperative variable arrays used so far in CMI research, the authors attempted to generate a formula for predicting postoperative outcome.
Data from the clinical records of 82 symptomatic adult patients with CMI and altered hindbrain CSF flow who were managed with foramen magnum decompression, C-1 laminectomy, and duraplasty over an 8-year period were collected and analyzed. Various preoperative clinical and radiological variables in the 57 patients who formed the study cohort were assessed in a bivariate analysis to determine their ability to predict clinical outcome (as measured on the Chicago Chiari Outcome Scale [CCOS]) and the resolution of syrinx at the last follow-up. The variables that were significant in the bivariate analysis were further analyzed in a multiple linear regression analysis. Different regression models were tested, and the model with the best prediction of CCOS was identified and internally validated in a subcohort of 25 patients.
There was no correlation between CCOS score and syrinx resolution (p = 0.24) at a mean ± SD follow-up of 40.29 ± 10.36 months. Multiple linear regression analysis revealed that the presence of gait instability, obex position, and the M-line–fourth ventricle vertex (FVV) distance correlated with CCOS score, while the presence of motor deficits was associated with poor syrinx resolution (p ≤ 0.05). The algorithm generated from the regression model demonstrated good diagnostic accuracy (area under curve 0.81), with a score of more than 128 points demonstrating 100% specificity for clinical improvement (CCOS score of 11 or greater). The model had excellent reliability (κ = 0.85) and was validated with fair accuracy in the validation cohort (area under the curve 0.75).
The presence of gait imbalance and motor deficits independently predict worse clinical and radiological outcomes, respectively, after decompressive surgery for CMI with altered hindbrain CSF flow. Caudal displacement of the obex and a shorter M-line–FVV distance correlated with good CCOS scores, indicating that patients with a greater degree of hindbrain pathology respond better to surgery. The proposed points-based algorithm has good predictive value for postoperative multifactorial outcome in these patients.