Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Surgical Interventions.

BACKGROUND: Chiari malformation type I (CIM) diagnoses have increased in recent years. Controversy regarding the best operative management prompted a review of the literature to offer guidance on surgical interventions. OBJECTIVE: To assess the literature to determine (1) whether posterior fossa decompression or posterior fossa decompression with duraplasty is more effective in preoperative symptom resolution; (2) whether there is benefit from cerebellar tonsillar resection/reduction; (3) the role of intraoperative neuromonitoring; (4) in patients with a syrinx, how long should a syrinx be observed for improvement before additional surgery is performed; and 5) what is the optimal duration of follow-up care after preoperative symptom resolution. METHODS: A systematic review was performed using the National Library of Medicine/PubMed and Embase databases for studies on CIM in children and adults. The most appropriate surgical interventions, the use of neuromonitoring, and clinical improvement during follow-up were reviewed for studies published between 1946 and January 23, 2021. RESULTS: A total of 80 studies met inclusion criteria. Posterior fossa decompression with or without duraplasty or cerebellar tonsil reduction all appeared to show some benefit for symptom relief and syrinx reduction. There was insufficient evidence to determine whether duraplasty or cerebellar tonsil reduction was needed for specific patient groups. There was no strong correlation between symptom relief and syringomyelia resolution. Many surgeons follow patients for 6-12 months before considering reoperation for persistent syringomyelia. No benefit or harm was seen with the use of neuromonitoring. CONCLUSION: This evidence-based clinical guidelines for the treatment of CIM provide 1 Class II and 4 Class III recommendations. In patients with CIM with or without syringomyelia, treatment options include bone decompression with or without duraplasty or cerebellar tonsil reduction. Improved syrinx resolution may potentially be seen with dural patch grafting. Symptom resolution and syrinx resolution did not correlate directly. Reoperation for a persistent syrinx was potentially beneficial if the syrinx had not improved 6 to 12 months after the initial operation. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/3-surgical-interventions .

Use of Subcutaneous Injection of Epinephrine and Triamcinolone with Tranexamic Acid Reduces Blood Loss, Transfusion Rates, and Length of Stay in Open Sagittal Craniosynostosis Repair.

OBJECTIVE: In 2017, we adopted the use of triamcinolone/epinephrine (TAC/Epi) scalp injection and later added tranexamic acid (TXA) in open sagittal synostosis surgery. We believe that this reduced blood loss and transfusion rates. METHODS: A total of 107 consecutive patients operated for sagittal synostosis aged <4 months from 2007 to 2019 were retrospectively reviewed. We collected demographics [age, sex, weight at surgery, and length of stay (LOS)], intraoperative information [estimated blood loss (EBL)], administration of packed red blood cell, transfusion of plasmalyte/albumen, operating time, baseline hemoglobin (Hb) and hematocrit (Hct), type of local anesthetic (1/4% bupivacaine vs. TAC/Epi), and use/volume of TXA. Hb, Hct, coagulation studies, and platelets at 2 hours postoperatively and postoperative day (POD) 1 were recorded. RESULTS: There were 3 groups: 1/4% bupivacaine/epinephrine (N=64), TAC/Epi (N=13), and TAC/Epi with TXA bolus/infusion intraoperatively (N=30). Groups receiving TAC/Epi or TAC/Epi with TXA had lower mean EBL ( P <0.0001), lower rate/amount of packed red blood cell transfusion ( P <0.0001), lower prothrombin time/international normalized ratio on POD 1 ( P <0.0001), higher platelets ( P <0.001), and shorter operative time ( P <0.0001). LOS was shortest for TAC/Epi with TXA ( P <0.0001). No significant differences between groups were noted on POD 1 Hb, Hct, or partial prothrombin time. Post hoc testing revealed an advantage of TAC/Epi with TXA over TAC/Epi alone for 2-hour postoperative international normalized ratio ( P =0.0249), Operating Room time ( P =0.0179), and LOS ( P =0.0049). CONCLUSIONS: Use of TAC/Epi alone reduced EBL, LOS, Operating Room time, and improved laboratory values postoperatively in open sagittal synostosis surgery. Addition of TXA further improved operative time and LOS. It is likely that lower rates of transfusion could be tolerated.

Disability or Death: A Focused Review of Informed Consent in Pediatric Neurosurgery.

The management of pediatric neurosurgical disease often requires families to choose between long-term disability and premature death. This decision-making is codified by informed consent. In practice, decision-making is heavily weighted toward intervening to prevent death, often with less consideration of the realities of long-term disability. We analyze long-term disability in pediatric neurosurgical disease from the perspectives of patients, families, and society. We then present a pragmatic framework and conversational approach for addressing informed consent discussions when the outcome is expected to be death or disability. We performed a focused review of literature regarding informed consent in pediatric neurosurgery by searching PubMed and Google Scholar with search terms including "pediatric neurosurgery," "informed consent," and "disability." The literature was focused on patients with diagnoses including spina bifida, neuro-oncology, trauma, and hydrocephalus. Patient perspective elements were physical/mental disability, lack of autonomy, and role in community/society. The family perspective involves caregiver burden, emotional toll, and financial impact. Societal considerations include the availability of public resources for disabled children, large-scale financial cost, and impacts on global health. Practical conversational steps with patients/caregivers include opening the discussion, information provision and acknowledgement of uncertainty, assessment of understanding and clarifying questions, decision-making, and decision maintenance, all while remaining sensitive to the emotional burden commensurate with these decisions. The "death or disability" paradigm represents a common challenge to informed consent in pediatric neurosurgery. Patient, family, and societal factors that inform surrogate decisions vary and sometimes conflict. Pediatric neurosurgeons must use a comprehensive approach to address the informational and relational needs of caregivers during the informed consent process.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

OBJECTIVE: The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS: The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS: A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS: Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Intrasacral Meningocele Complicating Caudal Analgesia in a 6-Year-Old Child After 3 Previous Uneventful Caudals: A Case Report.

A 6-year-old male undergoing bilateral hydrocelectomy was to receive caudal analgesia after induction of general anesthesia. After insertion of the caudal needle, cerebrospinal fluid was unexpectedly aspirated and the caudal was abandoned. The surgeon performed bilateral ilioinguinal nerve blocks just before incision. Surgery was uneventful. The patient had difficulty ambulating postoperatively, and a detailed neurologic examination revealed quadriceps weakness. A lumbosacral magnetic resonance imaging (MRI) revealed a sacral meningocele. By the next morning, quadriceps function had returned, and he was ambulating normally. The ilioinguinal block was most likely deep to the internal oblique muscle and produced femoral nerve dysfunction.

Filum Terminale Ependymoma in an Infant with Meningocele.

This report describes a case of an ependymoma found in the setting of tethered cord syndrome. We present a 3-month-old girl with prenatal diagnosis of lumbar meningocele who later underwent tethered cord release. After birth, she was neurologically intact and only found to have a skin-covered meningocele. An MRI was obtained and significant for low-lying conus terminating at L5, a focal syrinx, and Chiari II malformation. She underwent an elective meningocele repair and resection of thickened filum for tethered cord release at 3 months of age. Unexpectedly, microscopic evaluation of the filum was consistent with a small focus of ependymoma in addition to the filum tissue. Previous case reports have suggested a link between thickened filum in the setting of spinal dysraphism and myxopapillary ependymoma, but to our knowledge, this is the first report of ependymoma in the setting of tethered cord syndrome.

Alterations in cerebral ventricle size in children with congenital heart disease.

PURPOSE: Children with tetralogy of Fallot (TOF) and superior cavopulmonary anastomoses (SCPA) can have chronically elevated central venous pressure (CVP), which has been postulated to put patients at risk for cerebral ventriculomegaly. We aimed to examine cerebral ventricle size in children with these congenital heart lesions before and after surgery to determine how changes in CVP affect ventricle size. METHODS: We reviewed the records of patients who underwent SCPA or TOF repair between 2006 and 2015. Patients with pre- or post-operative cranial imaging were included. Frontal-occipital (FO) horn ratios were calculated as measures of cerebral ventricle volume. Reported normal mean FO ratio is 0.37 ± 0.03. Patient characteristics including occipito-fronto circumference (OFC) and available CVP measurements were recorded. CVP, FO ratios, and OFC percentiles were compared using paired and unpaired t tests and Wilcoxon matched pairs signed-rank test as appropriate. RESULTS: We reviewed 44 patients who underwent SCPA and 31 patients who underwent TOF repair who had cranial imaging studies available. In the 22 patients who underwent SCPA and had pre- and post-operative imaging, mean FO ratios significantly increased from 0.37 ± 0.03 to 0.40 ± 0.04 (P < 0.001). In contrast, in the seven patients with TOF with pre- and post-operative imaging, FO ratio was elevated at baseline and remains so after surgical repair, 0.43 ± 0.08 to 0.42 ± 0.08 (P = 0.65). Similar patterns were noted with OFC percentiles, which were significantly increased as compared to baseline after SCPA (P < 0.001) but were not significantly changed after TOF repair (P = 0.58). Finally, when available, preoperative and postoperative CVP measurements of all patients were examined, CVP increased in patients who underwent SCPA, from 6.5 ± 2 mmHg preoperatively to 9.1 ± 2.3 mmHg postoperatively (P < 0.001), while CVP remained statistically unchanged in patients who underwent TOF repair, 12.9 ± 3.3 mmHg preoperatively to 14.4 ± 3.1 mmHg postoperatively (P = 0.2). CONCLUSION: Cerebral ventriculomegaly was observed in patients with SCPA and TOF, and the observed changes in FO ratio and OFC may be related, at least in part, to CVP.

Pediatric Pontine Cavernous Malformations: The Presigmoid, Posterior Petrosal Approach.

BACKGROUND: Brainstem cavernous malformations (cavernomas) in children have a high risk of hemorrhage and neurological deterioration. This risk is magnified if the child has a genetic predisposition for cavernoma formation. The surgical management is challenging and carries a significant risk of morbidity. OBJECTIVE: To describe the feasibility of a posterior petrosal approach to brainstem cavernomas in a pediatric population. METHODS: A single institution operative experience with this technique was reviewed; 2 cases were identified and are technically described here with supportive figures and illustrations, as well as a focused literature review. RESULTS: Two pediatric cases with multiple symptomatic hemorrhages from large expanding pontine cavernomas were identified. Both cavernomas were resected through a presigmoid posterior petrosal approach. While this approach is well described in the adult literature for ventral brainstem lesions, its description for the treatment of pontine cavernomas in the pediatric populations is scarce. CONCLUSION: This study demonstrates the utility and feasibility of the posterior petrosal approach in two pediatric patients at different points in cranial base development.

Parent/guardian knowledge regarding implanted shunt type, setting, and symptoms of malfunction/infection.

OBJECTIVE Patients with shunts often interact with providers distant from their primary hospital, making it important that the parent(s)/guardian(s) is well versed in the type of shunt implanted and symptoms of malfunction/infection. This is particularly important with magnetic-sensitive programmable valves, as the use of MRI becomes more prevalent. METHODS Over a 6-month period, primary caregivers of 148 consecutive patients who received shunts were prospectively administered questionnaires at clinic visits. Caregivers were asked to do the following: 1) identify shunt valve name, type, and setting if applicable; 2) list symptoms of shunt malfunction/infection; and 3) indicate whether they had access to references regarding shunt type/setting, booklets from the Hydrocephalus Association, and quick reference cards with symptoms of shunt malfunction/infection. One cohort of caregivers (n = 75) was asked to carry informational cards with shunt valve/setting information (group I); this cohort was compared with another subgroup of caregivers (n = 73) not carrying cards (group II). RESULTS The mean (± SD) age of patients at implantation/revision was 3.71 ± 4.91 years, and the age at follow-up was 6.12 ± 5.4 years. The average time from surgery to administration of the questionnaire was 2.38 ± 3.22 years. There were 86 new shunt insertions and 62 revisions. One hundred twenty-eight caregivers (87%) could identify the type of valve (programmable vs nonprogrammable). On the other hand, only 72 caregivers (49%) could identify the valve name. Fifty-four of 73 (74%) caregivers of patients who had shunts with programmable valves could correctly identify the valve setting. One hundred caregivers (68%) had a copy of the Hydrocephalus Association booklet, and 103 (70%) had quick reference cards. Eighty caregivers (54%) had references on shunt type/setting. Most caregivers (127 [86%]) could name ≥ 3 signs/symptoms of shunt malfunction, with vomiting (61%), headache (49%), and sleeps more/lethargic (35%) most frequently reported. Caregivers of patients in group I were more likely to have cards with symptoms of shunt infection or malfunction (p = 0.015); have information cards regarding shunt type/setting (p < 0.001); and correctly identify valve type (p = 0.001), name (p < 0.001), and setting if programmable (p = 0.0016). There were no differences in ability to list symptoms of shunt malfunction or infection (p = 0.8812) or in access to Hydrocephalus Association booklets (p = 0.1288). There were no significant demographic differences between the groups, except that group I patients had a shorter time from surgery to last follow-up (1.66 vs 3.17 years; p = 0.0001). CONCLUSIONS Education regarding the care of patients with shunts by providing written cards with shunt type/setting and access to reference materials seems to be effective. Developing plans for guided instruction with assessment in the clinic setting of a caregiver's knowledge is important for patient safety.

Loss of mechanosensitive sclerostin may accelerate cranial bone growth and regeneration.

OBJECTIVE: Cranial defects can result from trauma, infection, congenital malformations, and iatrogenic causes and represent a surgical challenge. The current standard of care is cranioplasty, with either autologous or allogeneic material. In either case, the intrinsic vascularity of the surrounding tissues allows for bone healing. The objective of this study was to determine if mechanotransductive gene manipulation would yield non-weight-bearing bone regeneration in a critical size calvarial defect in mice. METHODS: A mouse model of Sost deletion in Sost knockout (KO) mice was created in which the osteocytes do not express sclerostin. A critical size calvarial defect (4 mm in diameter) was surgically created in the parietal bone in 8-week-old wild-type (n = 8) and Sost KO (n = 8) male mice. The defects were left undisturbed (no implant or scaffold) to simulate a traumatic calvariectomy model. Eight weeks later, the animals were examined at necropsy by planimetry, histological analysis of new bone growth, and micro-CT scanning of bone thickness. RESULTS: Defects created in wild-type mice did not fill with bone over the study period of 2 months. Genetic downregulation of sclerostin yielded animals that were able to regenerate 40% of the initial critical size defect area 8 weeks after surgery. A thin layer of bone covered a significant portion of the original defect in all Sost KO animals. A statistically significant increase in bone volume (p < 0.05) was measured in Sost KO mice using radiodensitometric analysis. Immunohistochemical analysis also confirmed that this bone regeneration occurred through the Wnt pathway and originated from the edge of the defect; BMP signaling did not appear to be affected by sclerostin. CONCLUSIONS: Mechanical loading is an important mechanism of bone formation in the cranial skeleton and is poorly understood. This is partially due to the fact that it is difficult to load bone in the craniomaxillofacial skeleton. This study suggests that modulation of the Wnt pathway, as is able to be done with monoclonal antibodies, is a potentially efficacious method for bone regeneration that requires further study.

Risk of deep venous thrombosis in elective neurosurgical procedures: a prospective, Doppler ultrasound-based study in children 12 years of age or younger.

OBJECTIVE The risk of venous thromboembolism (VTE) from deep venous thrombosis (DVT) is significant in neurosurgical patients. VTE is considered a leading cause of preventable hospital deaths and preventing DVT is a closely monitored quality metric, often tied to accreditation, hospital ratings, and reimbursement. Adult protocols include prophylaxis with anticoagulant medications. Children's hospitals may adopt adult protocols, although the incidence of DVT and the risk or efficacy of treatment is not well defined. The incidence of DVT in children is likely less than in adults, although there is very little prospectively collected information. Most consider the risk of DVT to be extremely low in children 12 years of age or younger. However, this consideration is based on tradition and retrospective reviews of trauma databases. In this study, the authors prospectively evaluated pediatric patients undergoing a variety of elective neurosurgical procedures and performed Doppler ultrasound studies before and after surgery. METHODS A total of 100 patients were prospectively enrolled in this study. All of the patients were between the ages of 1 month and 12 years and were undergoing elective neurosurgical procedures. The 91 patients who completed the protocol received a bilateral lower-extremity Doppler ultrasound examination within 48 hours prior to surgery. Patients did not receive either medical or mechanical DVT prophylaxis during or after surgery. The ultrasound examination was repeated within 72 hours after surgery. An independent, board-certified radiologist evaluated all sonograms. We prospectively collected data, including potential risk factors, details of surgery, and details of the clinical course. All patients were followed clinically for at least 1 year. RESULTS There was no clinical or ultrasound evidence of DVT or VTE in any of the 91 patients. There was no clinical evidence of VTE in the 9 patients who did not complete the protocol. CONCLUSIONS In this prospective study, no DVTs were found in 91 patients evaluated by ultrasound and 9 patients followed clinically. While the study is underpowered to give a definitive incidence, the data suggest that the risk of DVT and VTE is very low in children undergoing elective neurosurgical procedures. Prophylactic protocols designed for adults may not apply to pediatric patients. Clinical trial registration no.: NCT02037607 (clinicaltrials.gov).

Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review.

BACKGROUND: Chordomas are rare, slow-growing malignant neoplasms derived from remnants of the embryological notochord. Pediatric cases comprise only 5% of all chordomas, but more than half of the reported pediatric chordomas are intracranial. For patients of all ages, intracranial chordomas typically present with symptoms such as headaches and progressive neurological deficits occurring over several weeks to many years as they compress or invade local structures. There are only reports of these tumors presenting acutely with intracranial hemorrhage in adult patients. CASE DESCRIPTION: A 10-year-old boy presented with acute onset of headache, emesis, and diplopia. Head computed tomography and magnetic resonance imaging of brain were suspicious for a hemorrhagic mass located in the left petroclival region, compressing the ventral pons. The mass was surgically resected and demonstrated acute intratumoral hemorrhage. Pathologic examination was consistent with chordoma. CONCLUSION: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors' knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus.

Spring-assisted cranial vault expansion in the setting of multisutural craniosynostosis and anomalous venous drainage: case report.

Patients with multisutural craniosynostosis can develop anomalous venous connections between the intracranial sinuses and cutaneous venous system through enlarged emissary veins. Cranial vault remodeling in this subset of patients carries the risk of massive intraoperative blood loss and/or occlusion of collateral draining veins leading to intracranial venous hypertension and raised intracranial pressure, increasing the morbidity of cranial expansion. The authors report the use of spring-mediated expansion as a technique for cranial reconstruction in which the collateral intracranial venous drainage system can be preserved. A patient with bilateral lambdoid, sagittal, and unicoronal synostosis presented for cranial reconstruction. A tracheostomy and ventriculoperitoneal shunt were placed prior to intervention. At the time of reconstruction, a Luckenschadel skull abnormality and Chiari malformation Type I were present. A preoperative CT venogram demonstrated large collateral superficial occipital veins, small bilateral internal jugular veins, and hypoplastic jugular foramina. Collateral flow from the transverse and sigmoid sinuses through large occipital emissary veins was seen. Spring-mediated cranial vault expansion was performed with care to preserve the large collateral veins at the occipital midline. Four springs were placed at each lambdoid and the posterior and anterior sagittal sutures following 1-cm strip suturectomies. Removal of the springs was performed 2 months postoperatively. Cranial vault expansion was performed without disturbing the aberrant intracranial/extracranial venous collateral system. Estimated blood loss was 150 ml. A CT scan obtained 3 months postoperatively showed resolution of the Luckenschadel deformity and a 40% volumetric increase in the skull compared with the preoperative CT. Patients with anomalous venous drainage patterns and multisutural synostosis can undergo spring-mediated cranial vault expansion while preserving the major emissary veins draining the intracranial sinuses. Risks of blood loss, intracranial venous hypertension, and increased intracranial pressure may be decreased compared with traditional techniques of repair.

Radiographic and clinical outcome of syringomyelia in patients treated for tethered cord syndrome without other significant imaging abnormalities.

OBJECT: The surgical management of patients with symptoms of tethered cord syndrome (TCS) who lack significant radiographic abnormalities is controversial. One potential MRI marker for TCS is a spinal cord syrinx or syringomyelia. Alternatively, a syrinx may be a benign and incidental finding. In this report the authors evaluated a highly selected cohort of patients with symptoms of TCS with minimal radiographic abnormalities other than syringomyelia. They analyzed clinical and radiographic outcomes after tethered cord release (TCR). METHODS: A retrospective review of data from 16 children who met the study inclusion criteria was performed. All patients had been surgically treated at Riley Hospital for Children in Indianapolis, Indiana, between 2006 and 2011. All children had clinical symptoms of TCS as well as available pre- and postoperative MRI data. RESULTS: The most common presentation (12 [75%] of 16 patients) was urinary dysfunction, defined as symptoms of urgency or incontinence with abnormal urodynamic studies. Clinical follow-up data were available in 11 of these 12 patients. All 11 had improvement in symptoms at an average follow-up of 17 months. Seven (87.5%) of 8 patients presenting with back or leg pain had improvement. Three patients had progressive scoliosis; 2 had stabilization of the curve or mild improvement, and 1 patient had worsening deformity. Radiographic follow-up data were obtained an average of 14.5 months after surgery. Twelve patients (75%) had stable syringomyelia after TCR. Four patients showed improvement, with 2 having complete radiographic resolution. CONCLUSIONS: Highly selected patients with symptoms of TCS did very well clinically. Patients with abnormal urodynamic studies, pain, and gait disturbances showed a high rate of symptomatic improvement. However, a smaller percentage of patients had radiographic improvement of the syrinx. Therefore, the authors suggest that the decision to perform TCR should be based on clinical symptoms in this population. Symptomatic improvement was not necessarily related to radiographic resolution of the syrinx.

Cyst-ventricle stent as primary or salvage treatment for posterior fossa arachnoid cysts.

OBJECT: The optimal treatment of symptomatic posterior fossa arachnoid cysts is controversial. Current options include open or endoscopic resection, fenestration, or cyst-peritoneal shunt placement. There are potential drawbacks with all options. Previous authors have described stenting a cyst into the ventricular system for supratentorial lesions. The current authors have used a similar strategy for posterior fossa cysts. METHODS: The authors performed a retrospective review of 79 consecutive patients (1993-2010) with surgically treated intracranial arachnoid cysts. RESULTS: The authors identified 3 patients who underwent placement of a stent from a posterior fossa arachnoid cyst to a supratentorial ventricle. In 2 patients the stent construct consisted of a catheter placed into a posterior fossa arachnoid cyst and connecting to a lateral ventricle catheter. Both patients underwent stent placement as a salvage procedure after failure of open surgical fenestration. In the third patient a single-catheter cyst-ventricle stent was stereotactically placed. All 3 patients improved clinically. Two patients remained asymptomatic, with radiographic stability in a follow-up period of 1 and 5 years, respectively. The third patient experienced initial symptom resolution with a demonstrable reduction of intracystic pressure. However, he developed recurrent headaches after 2 years. CONCLUSIONS: Posterior fossa cyst-ventricle stenting offers the benefits of ease of surgical technique and a low morbidity rate. It may also potentially reduce the incidence of shunt-related headaches by equalizing the pressure between the posterior fossa and the supratentorial compartments. While fenestration is considered the first-line therapy for most symptomatic arachnoid cysts, the authors consider cyst-ventricle stenting to be a valuable additional strategy in treating these rare and often difficult lesions.

Circumferential fixation with craniofacial miniplates for a cervical spine injury in a child.

Unstable pediatric cervical spine injuries present significant challenges in terms of fixation. Given the smaller cervical vertebral bodies in the preschool-aged population, commercially available pediatric cervical fixation instrumentation may be unsuitable because of the inappropriately large size of the screws and plates. The authors describe a 2-year-old girl who sustained an unstable C6-7 distraction injury during a motor vehicle accident. Because of the small size of her vertebral bodies, standard cervical spine instrumentation was not feasible, and posterior wiring alone was believed to be insufficient because of the complete distraction of all 3 spinal columns. The patient was taken to the operating room where craniofacial plates with an inherent locking mechanism were used to circumferentially stabilize the cervical spine. Follow-up examination 6 months postoperatively demonstrated stable cervical spine alignment and fusion with no evidence of the failure of either the anterior or posterior hardware. The use of craniofacial miniplates with an intrinsic locking mechanism represents a superior alternative for both anterior and posterior cervical fixations when spinal instrumentation is needed in the pediatric age group.

Spinal congenital dermal sinuses: a 30-year experience.

OBJECTIVE: Dermal sinus tracts are an uncommon form of spinal dysraphism often presenting in childhood with skin findings, neurologic deficit, or infection. We reviewed our surgical experience, examining presenting symptomatology, operative findings, and patient outcomes. METHODS: A retrospective analysis of operated dermal sinus tract cases by the senior author (A.H.M.) from 1970 to present was made. RESULTS: Twenty-eight patients were identified; 17 female and 11 male. Five cervical, 4 thoracic, 9 lumbar, and 9 lumbosacral tracts were explored. Sixteen patients presented at <1 year of age, and 12 were >1 year. Reasons for referral included cutaneous findings (15), neurologic deficit (8), foot abnormalities (4), infection (3), pain (2), and scoliosis (1). Our initial examination revealed cutaneous findings (eg, sinus ostea, pigmentation changes, erythema, skin tags, subcutaneous masses) in 27 patients and neurologic deficit in 19. Age-related differences were apparent. Patients >1 year were more likely to have neurologic deficit (92%) as compared with those <1 year (50%). Bifid spinous processes were noted at dural tract entry in 17 patients. Twenty-two tethered cords, 14 inclusion tumors, and 6 patients with evidence of arachnoiditis were found intraoperatively. Mean follow-up was 33 months. Eleven (39%) remained neurologically intact, 12 (43%) improved, 2 (7%) were unchanged, and 3 (11%) were worse with 2 having decreased perianal sensation and 1 slightly worsened motor function postoperatively. CONCLUSIONS: Although most patients were referred for cutaneous stigmata evaluation, >50% had neurologic deficit, intradural tumors, or tethered cords. Skin findings identification should initiate prompt radiologic evaluation and neurosurgical intervention with intradural exploration. Timely intervention may preserve or improve neurologic function in these patients.

Long-term outcomes during treatment of chronic pain with intrathecal clonidine or clonidine/opioid combinations.

To evaluate the effectiveness of intrathecal clonidine or clonidine/opioid admixture for the treatment of chronic pain states, a retrospective chart audit of 15 patients seen by the Pain Medicine and Neurosurgical Services was performed. Subjects included 9 men and 6 women aged 26-86 years. Diagnoses included complex regional pain syndrome, neuropathic pain, and cancer pain. All patients received a trial of single-shot and/or short-term infusion of clonidine. Those reporting a significant reduction in pain, or at least 50% reduction in their visual analog scale (VAS), received long-term therapy. Intrathecal clonidine as a single-shot dose, infusion, or as intrathecal polytherapy did not improve VAS scales from pre-treatment values in 5 patients. Ten patients reported significant pain relief or >50% decrease in VAS scores with the initial trial and received long-term therapy. Two received clonidine alone for 7-11 months before the therapy failed; others failed after just a few days. Seven of eight initially responded to clonidine alone (75-950 microg/day) before failing and requiring a second drug. Three received hydromorphone (200-8000 microg/day) and four morphine (0.15-15 mg/day) with clonidine. Four patients then failed 2-drug therapy (duration 6-21 months). Two continue with intrathecal clonidine/hydromorphone (duration 19-29 months) and 1 with clonidine/morphine (duration 21 months). After initiation of intrathecal clonidine, one patient reported good relief with clonidine/morphine until his death 5 months later. In this population, intrathecal clonidine was of limited utility for most patients. It may be of benefit for subset(s) of patients, but in our experience, duration of relief is typically <18 months.