Object Suturectomy as a treatment for craniosynostosis was largely replaced in the late twentieth century by more extensive, but predictable, cranial remodeling procedures. Recent technical innovations, such as using the endoscope combined with postoperative orthotic reshaping, have led to a resurgence of interest in suturectomy as a safer, less invasive method. Methods A retrospective chart review was performed for all cases of sagittal synostosis treated with endoscopic sagittal suture strip craniectomy and helmet therapy between 2004 and 2008. Data collected included gestational age, genetic evaluations and syndromic status, age at operation, duration of procedure, need for blood transfusions, length of hospital stay, preoperative and postoperative head circumference percentile and cranial index, duration of helmet use, length of follow-up, complications, and revisions. Results Fifty-six patients with isolated sagittal synostosis were treated using endoscopic suturectomy and completed helmet therapy. Mean age at time of procedure was 3.24 months. Mean operative duration was 45.32 minutes. Mean hospital stay was 1.39 days. There were 2 transfusions and no deaths. The mean length of follow-up was 2.34 years. Helmet therapy was instituted for a mean of 7.47 months. Head circumference percentile increased from 61.42% to 89.27% over 2 years of follow-up. Cranial index increased from a preoperative mean of 0.69 to 0.76 over 2 years of follow-up. Reoperations for synostosis included 1 sagittal suture refusion and 2 cases in which other sutures fused. Conclusions Sagittal synostosis can be safely treated with endoscopic suturectomy and helmet therapy. Improvements in cranial volume and shape are comparable to open procedures and are enduring.

Object The primary goals of treatment in the infant with craniosynostosis are to correct the deformity and allow for adequate brain growth in as safe and effective a manner as possible. Herein, the authors present the results of treating craniosynostosis using an endoscope-assisted strip craniectomy and postoperative helmet therapy (EASC + PHT) in the hopes of providing further evidence of its role in the treatment of multiple different forms of craniosynostosis. This is a retrospective review of the patients treated with this technique at Children's Hospital Boston. Methods The electronic medical records of all children with craniosynostosis treated using this technique were reviewed retrospectively. A priori, data were collected for deformity type, patient age at surgery, number of transfusions, operative time, length of hospital stay, and anthropometric measurements. Results One hundred seventy-three patients (61 females and 112 males) were treated at our institution between July 2004 and March 2011 with EASC + PHT. The mean operative time was 46.30 minutes. Eight (4.6%) of the 173 patients received blood transfusions. The average length of hospital stay was 1.35 days, with the majority of patients being discharged the day after surgery. All complications and any patient who required additional craniofacial reconstructions are discussed. In addition, a subgroup analysis was done for patients who had undergone surgery and had longer than 1 year of follow-up. Conclusions The authors' growing database of patients supports the experiences described by others that early treatment of craniosynostosis with an EASC + PHT is a safe and efficacious technique. In addition, cost reduction due to decreased hospital stay and limitation of blood transfusions are demonstrable benefits associated with the use of this technique.

BACKGROUND Historically, symptomatic, benign intradural extramedullary (IDEM) spine tumors have been managed with surgical resection. However, minimal robust data regarding patient-reported outcomes (PROs) following treatment of symptomatic lesions exists. Moreover, there are increasing reports of radiosurgical management of these lesions without robust health-related quality of life data. OBJECTIVE To prospectively analyze PROs among patients with benign IDEM spine tumors undergoing surgical resection to define the symptomatic efficacy of surgery. METHODS Prospective, single-center observational cohort study of patients with benign IDEM spine tumors undergoing open surgical resection. Pre- and postoperative Brief Pain Index (BPI) and MD Anderson Symptom Inventory (MDASI) questionnaires were used to quantitatively assess their symptom control after surgical intervention. Matched pairs were analyzed with the Wilcoxon signed-rank test. RESULTS A total of 57 patients met inclusion criteria with both pre- and postoperative PROs. There were 35 schwannomas, 18 meningiomas, 2 neurofibromas, 1 paraganglioma, and 1 mixed schwannoma/neurofibroma. Most patients were American Spinal Injury Association Impairment (ASIA) E (93%) with high-grade spinal cord compression (77%), and underwent either a 2 or 3 level laminectomy (84%). Surgical resection resulted in statistically significant improvement in all 3 composite BPI constructs of pain-severity, pain-interference, and overall patient pain experience (P < .0001). Surgical resection resulted in statistically significant improvements in all composite scores for the MDASI core symptom severity, spine tumor, and disease interference constructs (P < .01). Three patients (5%) had postoperative complications requiring surgical interventions (2 wound revisions and 1 ventriculo-peritoneal shunt). CONCLUSION Surgical resection of IDEM spine tumors provides rapid, significant, and durable improvement in PROs.

OBJECTIVEThe current treatment of chordomas is associated with significant morbidity, high rates of local recurrence, and the potential for metastases. Stereotactic radiosurgery (SRS) as a primary treatment could reduce the need for en bloc resection to achieve wide or marginal margins. Spinal SRS outcomes support the exploration of SRS’s role in the durable control of these conventionally radioresistant tumors. The goal of the study was to evaluate outcomes of patients with primary chordomas treated with spinal SRS alone or in combination with surgery.METHODSClinical records were reviewed for outcomes of patients with primary chordomas of the mobile spine and sacrum who underwent single-fraction SRS between 2006 and 2017. Radiographic local recurrence-free survival (LRFS), overall survival (OS), symptom response, and toxicity were assessed in relation to the extent of surgery.RESULTSIn total, 35 patients with de novo chordomas of the mobile spine (n = 17) and sacrum (n = 18) received SRS and had a median post-SRS follow-up duration of 38.8 months (range 2.0–122.9 months). The median planning target volume dose was a 24-Gy single fraction (range 18–24 Gy). Overall, 12 patients (34%) underwent definitive SRS and 23 patients (66%) underwent surgery and either neoadjuvant or postoperative adjuvant SRS. Definitive SRS was selectively used to treat both sacral (n = 7) and mobile spine (n = 5) chordomas. Surgical strategies for the mobile spine were either intralesional, gross-total resection (n = 5) or separation surgery (n = 7) and for the sacrum en bloc sacrectomy (n = 11). The 3- and 5-year LRFS rates were 86.2% and 80.5%, respectively. Among 32 patients (91%) receiving 24-Gy radiation doses, the 3- and 5-year LRFS rates were 96.3% and 89.9%, respectively. The 3- and 5-year OS rates were 90.0% and 84.3%, respectively. The symptom response rate to treatment was 88% for pain and radiculopathy. The extent or type of surgery was not associated with LRFS, OS, or symptom response rates (p > 0.05), but en bloc resection was associated with higher surgical toxicity, as measured using the Common Terminology Criteria for Adverse Events (version 5.0) classification tool, than epidural decompression and curettage/intralesional resection (p = 0.03). The long-term rate of toxicity ≥ grade 2 was 31%, including 20% grade 3 tissue necrosis, recurrent laryngeal nerve palsy, myelopathy, fracture, and secondary malignancy.CONCLUSIONSHigh-dose spinal SRS offers the chance for durable radiological control and effective symptom relief with acceptable toxicity in patients with primary chordomas as either a definitive or adjuvant therapy.

Background:Leukoencephalopathy, Calcification, and Cyst (LCC) is a syndrome describing the rare concurrence of these three unusual radiographic findings. Here, we describe the neurosurgical management in a patient afflicted with LCC and review the existing literature on surgical indications and outcomes.Case Description:A 24-year-old man presented with symptoms of progressive headache, gait imbalance and horizontal diplopia. Magnetic resonance imaging (MRI) showed radiographic findings typically associated with LCC, including a large pontine cyst with significant mass effect. The patient's symptoms resolved after open surgical cyst drainage. However, he suffered cyst re-accumulation 3 months after the initial procedure and ultimately underwent placement of a ventriculo-cysto-peritoneal shunt. At the 3-year follow-up, the patient remained symptom free with continued cyst decompression.Conclusion:Our case report suggests that ventriculo-cysto-peritoneal shunting appeared an effective strategy in LCC patients in whom the cyst fenestration failed. We present this case report in the context of the first systematic review of literature on neurosurgical management strategies for patients afflicted with LCC.

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