Hurler syndrome is the most severe form of mucopolysaccharidosis (MPS) Type 1. Progressive neurocognitive decline in this condition can be accompanied by macrocephaly, ventriculomegaly, and/or periventricular signal changes on MRI, which often leads to a neurosurgical referral. In this case, the authors describe a 2-year-old boy with ventriculomegaly and periventricular T2 signal changes, both of which decreased following medical management of Hurler syndrome. The authors discuss the possible mechanisms for this finding and the implications for neurosurgical treatment of this condition.
Jennifer Liang and Ash Singhal
Chen-Yu Ding, Han-Pei Cai, Hong-Liang Ge, Liang-Hong Yu, Yuan-Xiang Lin, and De-Zhi Kang
The relationship between lipoprotein-associated phospholipase A2 (Lp-PLA2) and various cardiovascular and cerebrovascular diseases is inconsistent. However, the connection between Lp-PLA2 level and delayed cerebral ischemia (DCI) following aneurysmal subarachnoid hemorrhage (aSAH) remains unclear. The objective of this study was to investigate the relationships between the Lp-PLA2 levels in the early stages of aSAH and the occurrence of DCI.
The authors evaluated 114 patients with aSAH who were enrolled into a prospective observational cohort study. Serum Lp-PLA2 level at admission (D0), on the first morning (D1), and on the second morning of hospitalization (D2) were determined using commercial enzyme-linked immunosorbent assay kits. The relationship between Lp-PLA2 levels and DCI was analyzed.
Forty-three patients with aSAH (37.72%) experienced DCI. Mean serum Lp-PLA2 level decreased from 183.06 ± 61.36 μg/L at D0 (D0 vs D1, p = 0.303), to 175.32 ± 51.49 μg/L at D1 and 167.24 ± 54.10 μg/L at D2 (D0 vs D2, p = 0.040). The Lp-PLA2 level changes (D0-D1 and D0-D2) were comparable between patients with and without DCI. Multivariate model analysis revealed Lp-PLA2 level (D0) > 200 μg/L was a more significant factor of DCI compared with Lp-PLA2 (D1) and Lp-PLA2 (D2), and was a strong predictor of DCI (odds ratio [OR] 6.24, 95% confidence interval [CI] 2.05–18.94, p = 0.001) after controlling for World Federation of Neurosurgical Societies (WFNS) grade (OR 3.35, 95% CI 1.18–9.51, p = 0.023) and modified Fisher grade (OR 6.07, 95% CI 2.03–18.14, p = 0.001). WFNS grade (area under the curve [AUC] = 0.792), modified Fisher grade (AUC = 0.731), and Lp-PLA2 level (D0; AUC = 0.710) were all strong predictors of DCI. The predictive powers of WFNS grade, modified Fisher grade, and Lp-PLA2 (D0) were comparable (WFNS grade vs Lp-PLA2: p = 0.233; modified Fisher grade vs Lp-PLA2: p = 0.771). The poor-grade patients with Lp-PLA2 (D0) > 200 μg/L had significantly worse DCI survival rate than poor-grade patients with Lp-PLA2 (D0) ≤ 200 μg/L (p < 0.001).
The serum level of Lp-PLA2 was significantly elevated in patients with DCI, and decreased within the first 2 days after admission. Lp-PLA2 in the early stages of aSAH might be a novel predictive biomarker for the occurrence of DCI.
Lei Zhang, Zhiqiang Yi, Hongzhou Duan, and Liang Li
The purpose of this study was to introduce a novel autologous duraplasty procedure for the treatment of Chiari malformation Type I (CM-I).
The authors retrospectively reviewed data from patients who had been diagnosed with CM-I and had undergone suboccipital decompression and autologous duraplasty in situ or synthetic dural graft duraplasty; patients were treated in the authors' department between 2011 and 2014. All procedures were performed by the same surgeon. The 2 duraplasty methods were compared in terms of surgical factors and complications. The authors assessed the neurological outcome and MRI-documented syrinx size at the 6-month follow-up visit.
Twenty-seven patients were enrolled in this study, 13 in the duraplasty in situ group and 14 in the synthetic dural graft duraplasty group. The results showed no significant differences between the duraplasty in situ and synthetic dural graft duraplasty groups in overall operative time (4.9 hours vs 4.1 hours; p = 0.070), estimated blood loss (229 ml vs 254 ml; p = 0.159), and duration of hospital stay after the operation (13.5 days vs 12.8 days; p = 0.808). In the duraplasty in situ group, 1 case of meningitis occurred (7.7%). In the synthetic dural graft duraplasty group, the complications included 1 case of meningitis (7.1%) and 1 CSF leak (7.1%). The mean cost of hospitalization in the duraplasty in situ group (CNY 23,354) was significantly lower than that in the synthetic dural graft duraplasty group (CNY 29,385; p = 0.036).
Compared with synthetic dural graft duraplasty, autologous duraplasty in situ is a safe, effective, and cost-effective procedure for the treatment of CM-I. The long-term outcome of this procedure requires investigation.
Michael Schulder, Danny Liang, and Peter W. Carmel
Object. In this article the authors report on a novel, compact device for magnetic resonance (MR) imaging that has been developed for use in a standard neurosurgical operating room.
Methods. The device includes a permanent magnet with a field strength of 0.12 tesla. The poles of the magnet are vertically aligned, with a gap of 25 cm. When not in use the magnet is stored in a shielded cage in a corner of the operating room; it is easily moved into position and attaches to a regular operating table. The magnet is raised for imaging when needed and may be lowered to allow surgery to proceed unencumbered. Surgical navigation with optical and/or magnetic probes is incorporated into the system.
Twenty-five patients have undergone removal of intracranial lesions with the aid of this device. Operations included craniotomy for tumor or other lesion in 18 patients and transsphenoidal resection of tumor in seven. The number of scans ranged from two to five per surgery (average 3.4); image quality was excellent in 45%, adequate in 43%, and poor in 12%. In four patients MR imaging revealed additional tumor that was then resected; in five others visual examination of the operative field was inconclusive but complete tumor removal was confirmed on MR imaging. In 21 patients early postoperative diagnostic MR studies corroborated the findings on the final intraoperative MR image.
Using a water-covered phantom, the accuracy of the navigational tools was assessed; 120 data points were measured. The accuracy of the magnetic probe averaged 1.3 mm and 2.1 mm in the coronal and axial planes, respectively; the optical probe accuracy was 2.1 mm and 1.8 mm in those planes.
Conclusions. This device provides high-quality intraoperative imaging and accurate surgical navigation with minimal disruption in a standard neurosurgical operating room.
Han-Jung Chen, Cheng-Loong Liang, and Kang Lu
Object. Transthoracic endoscopic T2–3 sympathectomy is currently the treatment of choice for palmar hyperhidrosis. Compensatory sweating of the face, trunk, thigh, and sole of the foot was found in more than 50% of patients who underwent this procedure. The authors conducted this study to investigate the associated intraoperative changes in plantar skin temperature and postoperative plantar sweating.
Methods. One hundred patients with palmar hyperhidrosis underwent bilateral transthoracic endoscopic T2–3 sympathectomy. There were 60 female and 40 male patients who ranged in age from 13 to 40 years (mean age 21.6 years). Characteristics studied included changes in palmar and plantar skin temperature measured intraoperatively, as well as pre- and postoperative changes in plantar sweating and sympathetic skin responses (SSRs).
In 59 patients (59%) elevation of plantar temperature was demonstrated at the end of the surgical procedure. In this group, plantar sweating was found to be exacerbated in three patients (5%); plantar sweating was improved in 52 patients (88.1%); and no change was demonstrated in four patients (6.8%). In the other group of patients in whom no temperature change occurred, increased plantar sweating was demonstrated in three patients (7.3%); plantar sweating was improved in 20 patients (48.8%); and no change was shown in 18 patients (43.9%). The difference between temperature and sweating change was significant (p = 0.001).
Compared with the presympathectomy rate, the rate of absent SSR also significantly increased after sympathectomy: from 20 to 76% after electrical stimulation and 36 to 64% after deep inspiration stimulation, respectively (p < 0.05).
Conclusions. In contrast to compensatory sweating in other parts of the body after T2–3 sympathetomy, improvement in plantar sweating was shown in 72% and worsened symptoms in 6% of patients. The intraoperative plantar skin temperature change and perioperative SSR demonstrated a correlation between these changes.
Sun Liyong, Yuhai Bao, Jiantao Liang, Mingchu Li, and Jian Ren
The posterior interhemispheric approach is a versatile approach to access lesions of the pineal region, posterior incisural space, posterior region of third ventricle, and adjacent structures. We demonstrate the case of a 26-year-old woman with symptoms of increased intracranial pressure and hydrocephalus caused by a meningioma at the posteromedial tentorial incisura. Gross-total removal of the tumor was successfully achieved via a posterior interhemispheric transtentorial approach. The patient reported an immediate and significant symptomatic improvement after surgery. The detailed operative technique and surgical nuances, including the surgical corridor, tentorium incision, tumor dissection and removal are illustrated in this video atlas.
The video can be found here: https://youtu.be/nSNyjQKl7aE.
Report of two cases
Cheng-Hong Toh, Yao-Liang Chen, Ho-Fai Wong, Kuo-Chen Wei, Shu-Hang Ng, and Yung-Liang Wan
✓ Rosai—Dorfman disease (RDD) is an idiopathic proliferation of histiocytes that affects the lymph nodes. Central nervous system involvement in the absence of nodal disease is extremely rare. On neuroimaging studies, intracranial RDD appears as solitary or multiple well-circumscribed, dura-based lesions. The authors report on two cases of RDD with locally aggressive features including dural sinus invasion, which to their knowledge has never before been described.
A 60-year-old woman presented with progressive dizziness and vertigo that had lasted for 1 week. Cranial computerized tomography and magnetic resonance imaging revealed an extraaxial homogeneous lobulated enhancing mass involving the right occipital lobe and the right cerebellar hemisphere. Invasion of the right transverse sinus was identified on a cerebral digital subtraction angiogram. A 59-year-old man with no prior medical illness experienced progressive weakness of both upper extremities and a partial complex seizure. Magnetic resonance imaging of his brain revealed a well-circumscribed enhancing mass in the left frontal lobe with extension to the right frontal lobe and invasion of the superior sagittal sinus. Both patients underwent resection of their brain masses. Pathological studies identified the disease as RDD in both patients.
Jinqian Liang, Ran Ding, Sooyong Chua, Zheng Li, and Jianxiong Shen
The safety of spinal fusion has been poorly studied in children with surgically corrected congenital cardiac malformations (CCMs). The objective of this study was to evaluate the safety of spinal fusion in patients with CCMs following cardiac surgery.
A retrospective study was conducted on 32 patients with scoliosis who received surgical treatment for their CCMs (CCM group). Sixty-four age- and sex-matched patients with scoliosis and normal hearts who received spinal fusion served as the control group. These 2 groups were compared for demographic distribution, blood loss, transfusion requirements, and incidence of postoperative complications.
The ages, curve pattern distributions, and number of levels fused were similar between the 2 groups before spinal fusion. Overall, a total of 7 patients in the CCM group (21.9%) and 5 (7.8%) in the control group had documented postoperative complications. The perioperative allogenic blood transfusion rate and mean red blood cell transfusion requirement in the CCM group were significantly higher than those found in patients in the control group (68.7% vs 28.1%, respectively, p = 0.000; and 2.68 ± 2.76 units/patient vs 0.76 ± 1.07 units/patient, respectively, p = 0.011). In the CCM group, a preoperative major curve magnitude ≥ 80° was the most accurate indicator of an increased risk for a major complication (p = 0.019), whereas no statistically significant correlation was noted between postoperative complications and age, type of congenital heart disease, operative duration, and estimated blood loss during the operation and transfusion.
Spinal fusion subsequent to prior cardiac surgery is relatively safe and effective in correcting the spinal deformity for patients with scoliosis and surgically corrected CCMs. A preoperative major curve magnitude ≥ 80° may be a risk factor in predicting postoperative complications in scoliotic patients with surgically corrected CCMs.
Xiaofeng Deng, Liang Wu, Chenlong Yang, and Yulun Xu
Neuropathic arthropathy (Charcot joint) caused by syringomyelia is rare and commonly misdiagnosed. Few cases have been reported by neurosurgeons. The aims of this study were to analyze the clinical and imaging presentations of neuropathic arthropathy and to discuss the effect of surgical management of the primary neurological deficits on neuropathic arthropathy.
The authors retrospectively reviewed clinical and imaging data of 12 patients with neuropathic arthropathy caused by syringomyelia who were referred to the department of neurosurgery between January 2003 and September 2012. Radiographs revealed destruction, dislocation, disorganization, and increased density or debris in the joints. Magnetic resonance imaging showed a syrinx of the spinal cord in all patients, with Chiari malformation in 11 patients and tethered spinal cord in 1 patient. Neurosurgical operations were performed in 5 of 12 patients, including posterior fossa decompression in 4 patients and syrinx-subarachnoidal shunt placement in 1 patient. Surgical management of the neuropathic joints was not performed in any of the patients. All patients were followed up, with a mean duration of 39 months.
Sixteen joints were involved, including 10 elbows, 3 shoulders, 2 interphalangeal joints, and 1 wrist. The side of the syrinx on cervical axial MRI was consistent with the side of the affected limb in every patient. Five patients who underwent neurosurgical treatments stated improvement in neurological dysfunctions and no deterioration in symptoms related to neuropathic arthropathy. In the 7 patients without neurosurgical treatments, 5 reported aggravation of neuropathic arthropathy manifestations, with deterioration of neurological symptoms in 4 of the 5 patients. The condition of the other 2 patients remained stable.
The elbow is the most frequently involved joint in neuropathic arthropathy caused by syringomyelia, followed by the shoulder. The authors speculate that the side of the syrinx determines the side of the neuropathic arthropathy. A detailed medical history and a careful physical examination are crucial for differentiating neuropathic arthropathy from other joint lesions. This study suggests that early management of the primary neurological condition may play an important role in preventing the development of neuropathic arthropathy and avoiding disease progression.