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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.

OBJECTIVEWhile a select population of pediatric patients with Chiari malformation type I (CM-I) remain asymptomatic, some patients present with tussive headaches, neurological deficits, progressive scoliosis, and other debilitating symptoms that necessitate surgical intervention. Surgery entails a variety of strategies to restore normal CSF flow, including increasing the posterior fossa volume via bone decompression only, or bone decompression with duraplasty, with or without obex exploration. The indications for duraplasty and obex exploration following bone decompression remain controversial. The objective of this study was to describe an institutional series of pediatric patients undergoing surgery for CM-I, performed by a single neurosurgeon. For patients presenting with a syrinx, the authors compared outcomes following bone-only decompression with duraplasty only and with duraplasty including obex exploration. Clinical outcomes evaluated included resolution of syrinx, scoliosis, presenting symptoms, and surgical complications.METHODSA retrospective review was conducted of the medical records of 276 consecutive pediatric patients with CM-I operated on at a single institution between 2001 and 2015 by the senior author. Imaging findings of tonsillar descent, associated syrinx (syringomyelia or syringobulbia), basilar invagination, and clinical assessment of CM-I–attributable symptoms and scoliosis were recorded. In patients presenting with a syrinx, clinical outcomes, including syrinx resolution, symptom resolution, and impact on scoliosis progression, were compared for three surgical groups: bone-only/posterior fossa decompression (PFD), PFD with duraplasty (PFDwD), and PFD with duraplasty and obex exploration (PFDwDO).RESULTSPFD was performed in 25% of patients (69/276), PFDwD in 18% of patients (50/276), and PFDwDO in 57% of patients (157/276). The mean follow-up was 35 ± 35 months. Nearly half of the patients (132/276, 48%) had a syrinx. In patients presenting with a syrinx, PFDwDO was associated with a significantly higher likelihood of syrinx resolution relative to PFD only (HR 2.65, p = 0.028) and a significant difference in time to symptom resolution (HR 2.68, p = 0.033). Scoliosis outcomes did not differ among treatment groups (p = 0.275). Complications were not significantly higher when any duraplasty (PFDwD or PFDwDO) was performed following bone decompression (p > 0.99).CONCLUSIONSIn this series of pediatric patients with CM-I, patients presenting with a syrinx who underwent expansile duraplasty with obex exploration had a significantly greater likelihood of syrinx and symptom resolution, without increased risk of CSF-related complications, compared to those who underwent bone-only decompression.

OBJECTIVEThe treatment of atlantoaxial dislocation (AAD) and basilar invagination (BI) is challenging, especially in symptomatic patients with a history of previous surgery. Although seldom reported, posterior revision surgery to revise prior constructs can be advantageous over an anterior or combined approach. The authors describe their experience in performing posterior revision surgery using Goel’s technique.METHODSThe authors reviewed patients with AAD and BI who had undergone previous posterior surgery at the cranio-cervical junction between January 2016 and September 2017. All of these patients underwent revision surgery from a posterior approach. The Japanese Orthopaedic Association (JOA) score was used to assess clinical symptoms before and after surgery. The distance from the tip of the odontoid to Chamberlain’s line, atlantodental interval (ADI), and clivus-canal angle (CCA) were used for radiographic assessment before and after surgery.RESULTSTwelve consecutive patients were reviewed. Prior surgeries were as follows: 4 patients (4/12) with posterior osseous decompression without fusion, 7 (7/12) with reduction and fusion without decompression, and 1 (1/12) with posterior osseous decompression and reduction and fusion. With the use of Goel’s technique for revision in these cases, distraction using facet spacers afforded release of the anterior soft tissue from a posterior approach. The occiput was fixated to C2 using a cantilever technique, and autologous cancellous bone was grafted into the intraarticular joints. In all 12 patients, complete reduction of BI and AAD were achieved without injury to nerves or vessels. All patients had evidence of bony fusion on CT scans within 18 months of follow-up.CONCLUSIONSPosterior revision surgery using Goel’s technique is an effective and safe revision salvage surgery for symptomatic patients with AAD and BI.

OBJECTIVETreatment of odontoid disease from a ventral corridor has consisted of a transoral approach. More recently, the endoscopic endonasal approach (EEA) has been used to access odontoid pathology.METHODSA retrospective review was conducted of patients who underwent an EEA for odontoid pathology from 2004 to 2013. During our analysis, the mean follow-up duration was 42.6 months (range 1–80 months). Patient outcomes, complications, and postoperative swallowing function were assessed either by clinic visit or phone contact.RESULTSThirty-four patients underwent an EEA for symptomatic odontoid pathology. The most common pathology treated was basilar invagination (n = 17). Other pathologies included odontoid fractures, os odontoideum, and metastatic carcinoma. The mean patient age was 71.5 years. Thirty-one patients underwent a posterior fusion. All 34 patients experienced stability or improvement in symptoms and all had successful radiographic decompression. The overall complication rate was 76%. Nearly all of these complications were transient (86%) and the overall complication rate excluding mild transient dysphagia was only 44%. Twenty-one patients (62%) suffered from transient postoperative dysphagia: 15 cases were mild, transient subjective dysphagia (6 of whom had documented preoperative dysphagia), whereas 6 other patients required tube feedings for decreased oral intake, malnutrition, and dysphagia in the perioperative setting (5 of these patients had documented preoperative dysphagia). Sixteen patients had documented preoperative dysphagia and 6 of these had lower cranial nerve dysfunction. Postoperatively, 6 (37.5%) of 16 patients with preoperative dysphagia and 4 (67%) of 6 with lower cranial nerve dysfunction had significant dysphagia/respiratory complications. Eighteen patients had no documented preoperative dysphagia and only 2 had significant postoperative dysphagia/respiratory complications (11%). The rates of these complications in patients without preoperative dysphagia were lower than in those with any preoperative dysphagia (p = 0.07) and especially those with preexisting lower cranial neuropathies (p = 0.007). Dysphagia was also significantly more common in patients who underwent occipitocervical fixation (19/26, 73%) than in patients who underwent cervical fusion alone or no fusion (2/8, 25%; p = 0.02). All patients with perioperative dysphagia had improved at follow-up and all patients were tolerating oral diets. No patient suffered from velopalatal insufficiency. Two patients had intraoperative CSF leaks. One of these patients underwent a negative exploratory surgery for a questionable postoperative CSF leak. One patient developed infection in the resection bed requiring debridement and antibiotics. One patient died 8 days following surgery from an unknown cause. The 90-day perioperative mortality rate was 2.9%.CONCLUSIONSA completely EEA can be performed for compressive odontoid disease in all cases of neoplastic, degenerative, or invaginative atlantoaxial disease with satisfactory outcomes and low morbidity. Transient perioperative dysphagia and respiratory complications are common, usually as an exacerbation and reflection of underlying disease or occipitocervical fusion rather than the EEA, emphasizing the importance of avoiding transoral surgery.

Chiari malformation type I (CM-I) is typically treated with foramen magnum decompression. However, a recent study proposed a new technique for patients with CM-I, wherein only short atlantoaxial joint fusion and distraction is applied. Posterior fusion with or without atlantoaxial distraction is a potential option for patients with CM-I associated with basilar invagination or complex anomalous bony craniovertebral junction pathology, since this procedure allows clinicians to avoid using the technically demanding transoral approach in which some or all of the odontoid tip is invisible. Distraction of the atlantoaxial joint reduces ventral cervicomedullary compression, leading to neurological improvement. Here, the authors report the case of a 50-year-old woman with CM-I plus basilar invagination whose condition immediately improved but then gradually deteriorated following atlantoaxial joint distraction and fusion. Therefore, the authors performed endonasal/transoral odontoidectomy, which resulted in prolonged neurological recovery. Short atlantoaxial fusion with distraction is a smart and ideal surgical planning concept that can result in significant neurological improvement. However, this case suggests that anterior odontoidectomy is still an essential element of the intervention strategy for patients with CM-I with complex craniovertebral junction pathology.

The bilateral sagittal split mandibular osteotomy (BSSMO), a common maxillofacial technique for expanding the oropharynx during treatment of micrognathia, is a rarely employed but useful adjunct to improve surgical access to the ventral cervical spine in children. Specifically, it provides enhanced exposure of the craniocervical junction in the context of midface hypoplasia, and of the subaxial cervical spine in children with severe kyphosis. The authors describe their technique for BSSMO and evaluate long-term outcomes in patients. The pediatric neurosurgical database at a single center was queried to identify children who underwent BSSMO as an adjunct to cervical spine surgery over a 22-year study period (1993–2015). The authors retrospectively reviewed clinical and radiographic data in all patients. The authors identified 5 children (mean age 5.3 ± 3.1 years, range 2.1–10.0 years) who underwent BSSMO during cervical spine surgery. The mean clinical follow-up was 3.0 ± 1.9 years. In 4 children, BSSMO was used to increase the size of the oropharynx and facilitate transoral resection of the odontoid and anterior decompression of the craniocervical junction. In 1 patient with subaxial kyphosis and chin-on-chest deformity, BSSMO was used to elevate the chin, improve anterior exposure of the subaxial cervical spine, and facilitate cervical corpectomy. Careful attention to neurovascular structures, including the inferior alveolar nerve, lingual nerve, and mental branch of the inferior alveolar artery, as well as minimizing tongue manipulation and compression, are critical to complication avoidance. The BSSMO is a rarely used but extremely versatile technique that significantly enhances anterior exposure of the craniocervical junction and subaxial cervical spine in children in whom adequate visualization of critical structures is not otherwise possible.

OBJECT Although anterior odontoidectomy has been widely accepted as a procedure for decompression of the craniovertebral junction (CVJ), postoperative biomechanical instability has not been well addressed. There is a paucity of data on the necessity for and choice of fixation. METHODS The authors conducted a retrospective review of consecutively treated patients with basilar invagination who underwent anterior odontoidectomy and various types of posterior fixation. Posterior fixation included 1 of 3 kinds of constructs: occipitocervical (OC) fusion with atlantoaxial (AA) fixation, OC fusion without AA fixation, or AA-only (without OC) fixation. On the basis of the use or nonuse of AA fixation, these patients were assigned to either the AA group, in which the posterior fixation surgery involved both the atlas and axis simultaneously, regardless of whether the patient underwent OC fusion, or the non-AA group, in which the OC fusion construct spared the atlas, axis, or both. Clinical outcomes and neurological function were compared. Radiological results at each time point (i.e., before and after odontoidectomy and after fixation) were assessed by calculating the triangular area causing ventral indentation of the brainstem in the CVJ. RESULTS Data obtained in 14 consecutively treated patients with basilar invagination were analyzed in this series; the mean follow-up time was 5.75 years. The mean age was 53.58 years; there were 7 males and 7 females. The AA and non-AA groups consisted of 7 patients each. The demographic data of both groups were similar. Overall, there was significant improvement in neurological function after the operation (p = 0.03), and there were no differences in the postoperative Nurick grades between the 2 groups (p = 1.00). According to radiological measurements, significant decompression of the ventral brainstem was achieved stepwise in both groups by anterior odontoidectomy and posterior fixation; the mean ventral triangular area improved from 3.00 ± 0.86 cm2 to 2.08 ± 0.51 cm2 to 1.68 ± 0.59 cm2 (before and after odontoidectomy and after fixation, respectively; p < 0.05). The decompression gained by odontoidectomy (i.e., reduction of the ventral triangular area) was similar in the AA and non-AA groups (0.66 ± 0.42 cm2 vs 1.17 ± 1.42 cm2, respectively; p = 0.38). However, the decompression achieved by posterior fixation was significantly greater in the AA group than in the non-AA group (0.64 ± 0.39 cm2 vs 0.17 ± 0.16 cm2, respectively; p = 0.01). CONCLUSIONS Anterior odontoidectomy alone provides significant decompression at the CVJ. Adjuvant posterior fixation further enhances the extent of decompression after the odontoidectomy. Moreover, posterior fixation that involves AA fixation yields significantly more decompression of the ventral brainstem than OC fusion that spares AA fixation.

Basilar invagination is a challenging dilemma that neurosurgeons may face. Herein, we present a case of a 65-year-old female with a history of rheumatoid arthritis and status post a previous C4–7 ACDF who presented to our clinic with progressive weakness in her bilateral upper and lower extremities. Imaging revealed basilar invagination. She underwent an endoscopic endonasal odontoidectomy followed by an occiput–C6 fusion. We present the endonasal portion of the procedure and have highlighted the technical nuances of this approach. Our goal is to provide better insight into this surgical strategy when dealing with basilar invagination.The video can be found here: https://youtu.be/aeMbvI_zYQA

OBJECT Cranial base pathology is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze whether bisphosphonate treatment, used to improve bone strength, could also prevent the development of craniocervical junction pathology (basilar impression, basilar invagination, or platybasia) in children with OI. METHODS In this single-center retrospective study the authors analyzed the skull base morphology from lateral skull radiographs and midsagittal MR images (total of 94 images), obtained between the ages of 0 and 25 years in 39 bisphosphonate-treated OI patients. The results were compared with age-matched normative values and with findings in 70 OI patients who were not treated with bisphosphonates. In addition to cross-sectional data, longitudinal data were available from 22 patients with an average follow-up period of 7.6 years. The patients, who had OI types I, III, IV, VI, and VII, had been treated with zoledronic acid, pamidronate, or risedronate for 3.2 years on average. RESULTS Altogether 33% of the 39 bisphosphonate-treated patients had at least 1 cranial base anomaly, platybasia being the most prevalent diagnosis (28%). Logistic regression analysis suggested a higher risk of basilar impression or invagination in patients with severe OI (OR 22.04) and/or older age at initiation of bisphosphonate treatment (OR 1.45), whereas a decreased risk was associated with longer duration of treatment (OR 0.28). No significant associations between age, height, or cumulative bisphosphonate dose and the risk for cranial base anomaly were detected. In longitudinal evaluation, Kaplan-Meier curves suggested delayed development of cranial base pathology in patients treated with bisphosphonates but the differences from the untreated group were not statistically significant. CONCLUSIONS These findings indicate that cranial base pathology may develop despite bisphosphonate treatment. Early initiation of bisphosphonate treatment may delay development of craniocervical junction pathology. Careful followup of cranial base morphology is warranted, particularly in patients with severe OI.

Object The goal of the study was to report a series of consecutive patients who underwent endoscopic transnasal odontoidectomy (ETO) without resection of nasal turbinates. The techniques for this minimally invasive approach are described in detail. Methods The authors conducted a retrospective review of consecutive patients who underwent ETO for basilar invagination. All the patients had myelopathy caused by compression at the cervicomedullary junction, which required surgical decompression. Preoperative and postoperative data, including those from radiographic and clinical evaluations, were compared. Morbidity and mortality rates for the procedure are also reported in detail. Results Thirteen patients (6 men and 7 women) with a mean age of 52.7 years (range 24–72 years) were enrolled. The basilar invagination etiologies were rheumatoid arthritis (n = 5), trauma (n = 4), os odontoideum (n = 2), ankylosing spondylitis (n = 1), and postinfectious deformity (n = 1). The average follow-up duration was 51.2 months (range 0.3–105 months). One patient died 10 days after the operation as a result of meningitis caused by CSF leakage. Among the other 12 patients, the average postoperative Nurick grade (3.2) was significantly improved over that before the operation (4.1, p = 0.004). The mean (± SD) duration of postoperative intubation was 1.5 ± 2.1 days, and there was no need for perioperative tracheostomy or nasogastric tube feeding. There also was no postoperative velopharyngeal insufficiency. There were 6 (46%) intraoperative and 2 (15%) postoperative CSF leaks in the 13 patients in this series. Conclusions ETO is a viable and effective option for decompression at the ventral cervicomedullary junction. This approach is minimally invasive and causes little velopharyngeal insufficiency. The pitfall of this approach is the difficulty in repairing dural defects and subsequent CSF leakage.

Object The object of this study was to evaluate the radiographic characteristics of C-2 using multiplanar CT measurements for anchor screw placement in patients with C-1 assimilation (C1A). Insertion of a C-2 pedicle screw in the setting of C1A is relatively difficult and technically demanding, and there has been no report about the optimal sizes of the pedicles and laminae of C-2 for screw placement in C1A. Methods An institutional database was searched for all patients who had undergone cervical CT scanning and cervical spine surgery between April 2006 and December 2012. Two neurosurgeons reviewed the CT scans from 462 patients who met these criteria, looking for C1A and other anomalies of the craniocervical junction such as high-riding vertebral artery (VA), basilar invagination, and VA anomaly. The routine axial images were reloaded on a workstation, and reconstruction CT images were used to measure parameters: the minimum width of bilateral pedicles and laminae and the length of bilateral laminae of the atlas. Results Seven patients with C1A were identified, and 14 sex-matched patients without C1A were randomly selected from the same database as a control group. The mean minimum pedicle width was 5.21 mm in patients with C1A and 7.17 mm in those without. The mean minimum laminae width was 5.29 mm in patients with C1A and 6.53 mm in controls. The mean minimum pedicle and laminae widths were statistically significantly smaller in the patients with C1A (p < 0.05). Conclusions In patients with C1A, the C-2 bony structures are significantly smaller than normal, making C-2 pedicle screw or translaminar screw placement more difficult.

In this issue of the Journal of Neurosurgery: Spine, Professor Atul Goel submits a single-institution, singlesurgeon retrospective analysis of 65 adult and pediatric patients with “complex” Chiari malformations (CMs), that is, CM Type I associated with complex bony anomalies of the craniocervical junction and brainstem compression.4 Professor Goel is respected as a clinical pioneer and thought leader for disorders at the craniocervical junction. He asserts that the basic pathophysiological mechanism for CM with or without basilar invagination and with or without syringomyelia is C1–2 instability. This assertion challenges conventional theories. Bollo et al.1 recently thoroughly described this entity in the pediatric neurosurgical literature; the authors identified basilar invagination, CM 1.5, and a clivoaxial angle < 125° as patient risk factors necessitating reduction and occipitocervical fusion in addition to mandatory suboccipital decompression. In the present study Goel speculates that CM and syringomyelia are “Nature’s protective mechanism” of the body and compares CM to an “air bag.” This speculation, at best, is unsupported and unsubstantiated by evidence in the study and the literature and, at worst, sounds flowery and sophomoric. This literary metaphor seems out of place in a scientific journal. Professor Goel attempts to demonstrate in his series a new paradigm for treating symptomatic CM, that is, C1–2 posterior instrumented fusion alone using C-1 lateral mass screws and C-2 pars or pedicle screws—without any attempt at CM decompression and without occipitocervical fusion. Notably, in his series, there was one death (1.5%) from vertebral artery injury (out of 3 total vertebral artery injuries; approximately 5% rate of injury) while exposing entry points for the C-1 lateral mass screws. Professor Goel managed these patients with a C1–2 posterior instrumented fusion. As part of the preparation for placing the C-1 lateral mass screws, anomalous vertebral arteries needed to be “dissected” away from the C-1 facet process. Why? This seems completely unnecessary and adds significant risk to the procedure (vertebral artery injury or spasm). The C-1 arch is already fused to the occiput in more than a few examples provided as figures; therefore, fusing from the occiput (with occipital screws and plates) to C-2 would be a biomechanically equivalent construct, without the loss of additional range of motion at the craniocervical junction and without having to manipulate the vertebral arteries. Related to the above point, basilar invagination and/or platybasia are typically regarded as signs of craniocervical junction instability, and an occipitocervical construct is usually indicated. Moreover, in a subgroup of patients (Group A) in Professor Goel’s study, cervical traction was applied intraoperatively “to reduce basilar invagination and restore craniovertebral alignment.” How does a C1–2 fusion alone maintain this reduction? Again, occipitocervical fusion seems necessary to maintain this reduction. Mortality rates for CM decompression surgery with or without duraplasty range from 0% to 0.9% in large series.5,6 Vertebral artery injury rates range from 0% to 0.7% for posterior fossa decompression with and without duraplasty for the surgical treatment of CM.2 Professor Goel’s mortality rate and vertebral artery injury rate using this new surgical approach for CM seem alarmingly high, unjustified, and lie outside of published benchmarks for the surgical treatment of CM using suboccipital craniectomy plus or minus duraplasty. While the patient-reported outcomes seem very satisfactory (63 [97%] of 65) on health-related quality of life (HRQOL) outcome surveys (Japanese Orthopaedic Asso

Object Transoral microscopic odontoidectomy has been accepted as a standard procedure to treat basilar invagination over the past several decades. In recent years the emergence of new technologies, including endoscopic odontoidectomy and posterior reduction, has presented a challenge to the traditional treatment algorithm. In this article, the authors describe 1 patient with basilar invagination who was successfully treated with endoscopic transnasal odontoidectomy combined with posterior reduction. The purpose of this report is to validate the effectiveness of this treatment algorithm in selected cases and describe several operative nuances and pearls based on the authors' experience. Methods One patient with basilar invagination caused by a congenital osseous malformation underwent endoscopic transnasal odontoidectomy combined with posterior reduction in a single operative setting. The purely endoscopic transnasal odontoidectomy was first conducted with the patient supine. The favorable anatomical reduction was then achieved through a posterior approach after the patient was moved prone. Results The patient was extubated after recovery from anesthesia and allowed oral food intake the next day. No complications were noted, and the patient was discharged 4 days after the operation. Postoperative imaging demonstrated excellent decompression of the anterior cervicomedullary junction pathology. The patient was followed up for 12 months and remarkable neurological recovery was observed. Conclusions The endoscopic transnasal odontoidectomy is a better minimally invasive approach for anterior decompression and can make the posterior reduction easier because the anterior resistant force is eliminated. The subsequent posterior reduction can make decompression of the ventral side of the cervicomedullary junction more effective because the C-2 vertebral body is pushed forward. A combination of these 2 approaches has the advantages of minimally invasive access and a faster patient recovery, and thus is a valid alternative in selected cases.

Object Decompression of the foramen magnum is widely accepted as the procedure of choice for patients with Chiari malformation Type I (CM-I). This study was undertaken to determine the mechanisms responsible for neurological deterioration after foramen magnum decompression and the results of secondary interventions. Methods Between 1987 and 2010, 559 patients with CM-I presented, 107 of whom had already undergone a foramen magnum decompression, which included a syrinx shunt in 27 patients. Forty patients who were neurologically stable did not undergo another operation. Sixty-seven patients with progressive symptoms received a recommendation for surgery, which was refused by 16 patients, while 51 patients underwent a total of 61 secondary operations. Hospital and outpatient records, radiographic studies, and intraoperative images were analyzed. Additional follow-up information was obtained by telephone calls and questionnaires. Short-term results were determined after 3 and 12 months, and long-term outcomes were evaluated using Kaplan-Meier statistics. Results Sixty-one secondary operations were performed after a foramen magnum decompression. Of these 61 operations, 15 involved spinal pathologies not related to the foramen magnum (spinal group), while 46 operations were required for a foramen magnum issue (foramen magnum group). Except for occipital pain and swallowing disturbances, the clinical course was comparable in both groups. In the spinal group, 5 syrinx shunt catheters were removed because of nerve root irritations or spinal cord tethering. Eight patients underwent a total of 10 operations on their cervical spine for radiculopathies or a myelopathy. No permanent surgical morbidity occurred in this group. In the foramen magnum group, 1 patient required a ventriculoperitoneal shunt for hydrocephalus 7 months after decompression. The remaining 45 secondary interventions were foramen magnum revisions, of which 10 were combined with craniocervical fusion. Intraoperatively, arachnoid scarring with obstruction of the foramen of Magendie was the most common finding. Complication rates for foramen magnum revisions were similar to first decompressions, whereas permanent surgical morbidity was higher at 8.9%. Postoperative clinical improvements were marginal in both surgical groups. With the exception of 1 patient who underwent syrinx catheter removal and had a history of postoperative meningitis, all patients in the spinal group were able to be stabilized neurologically. Long-term results in the foramen magnum group revealed clinical stabilizations in 66% for at least 5 years. Conclusions Neurological deterioration in patients after a foramen magnum decompression for CM-I may be related to new spinal pathologies, craniocervical instability, or recurrent CSF flow obstruction at the foramen magnum. Whereas surgery for spinal pathologies is regularly followed by clinical stabilization, the rate of long-term success for foramen magnum revisions was limited to 66% for 5 years due to severe arachnoid scarring in a significant proportion of these patients. Therefore, foramen magnum revisions should be restricted to patients with progressive symptoms.

Object Chiari malformation Type I (CM-I) is a congenital anomaly often treated by decompressive surgery. Patients who fail to respond to standard surgical management often have complex anomalies of the craniovertebral junction and brainstem compression, requiring reduction and occipitocervical fusion. The authors hypothesized that a subgroup of “complex” patients defined by specific radiographic risk factors may have a higher rate of requiring occipitocervical fusion. Methods A retrospective review was conducted of clinical and radiographic data in pediatric patients undergoing surgery for CM-I between 1995 and 2010. The following radiographic criteria were identified: scoliosis, syringomyelia, CM Type 1.5, medullary kinking, basilar invagination, tonsillar descent, craniocervical angulation (clivoaxial angle [CXA] < 125°), and ventral brainstem compression (pB–C2 ≥ 9 mm). A multivariate Cox regression analysis was used to determine the independent association between occipitocervical fusion and each variable. Results Of the 206 patients who underwent CM decompression with or without occipitocervical fusion during the study period, 101 had preoperative imaging available for review and formed the study population. Mean age at surgery was 9.1 years, and mean follow-up was 2.3 years. Eighty-two patients underwent suboccipital decompression alone (mean age 8.7 years). Nineteen patients underwent occipitocervical fusion (mean age 11.1 years), either as part of the initial surgical procedure or in a delayed fashion. Factors demonstrating a significantly increased risk of requiring fusion were basilar invagination (HR 9.8, 95% CI 2.2–44.2), CM 1.5 (HR 14.7, 95% CI 1.8–122.5), and CXA < 125° (HR 3.9, 95% CI 1.2–12.6). Conclusions Patients presenting with basilar invagination, CM 1.5, and CXA < 125° are at increased risk of requiring an occipitocervical fusion procedure either as an adjunct to initial surgical decompression or in a delayed fashion. Patients and their families should be counseled in regard to these findings as part of a preoperative CM evaluation.

Object The treatment of basilar invagination in the pediatric age group is dependent on the possibility of preoperative reduction. Reducible lesions obviate a ventral decompression and require a dorsal-only approach for stabilization with or without a suboccipital decompression. Methods The authors describe a technique of intraoperative reduction of basilar invagination with the use of general anesthesia and neuromuscular blockade in the presence of crown halo traction. Using the O-arm device, a 3D CT scan is generated in the sagittal plane to demonstrate the reduction intraoperatively. This technique was successful in 6 pediatric patients with basilar invagination. Results The average age of the patients was 10.8 years, and they were followed for a mean period of 8.5 months. The patients had mild basilar invagination or partial reduction in extension on preoperative MR imaging. Intraoperative reduction was demonstrated in all patients by using the reported technique with intraoperative CT. All patients underwent occipitocervical fusion, and all but one underwent a suboccipital decompression. There were no complications related to the operation, and all but one reported improvement of symptoms on the last postoperative visit. Conclusions Intraoperative reduction performed using neuromuscular blockade and intraoperative traction is an effective method for further reduction of basilar invagination in the pediatric age group. This is the first reported application of intraoperative CT imaging performed using the O-arm device in craniocervical surgery in which successful reduction is demonstrated in detail.

The management of intradiploic CSF collection is controversial. Although it is a benign lesion, even then delay in diagnosis and treatment may lead to significant morbidity. The authors report a very rare case of giant posttraumatic intradiploic pseudomeningocele involving the occipital bone, occipital condyles, and clivus. The pathogenesis and management of intradiploic CSF collection are discussed. This 16-year-old boy presented with a history of enlarging swelling in the suboccipital region associated with headache, lower cranial nerve palsy, and features of high cervical compressive myelopathy. Investigations revealed a giant intradiploic lesion involving the occipital bone, condyles, and clivus associated with secondary basilar invagination, hydrocephalus, and syringomyelia. Intrathecal contrast administration did not reveal communication of intradiploic space with the subarachnoid space. A large occipital craniotomy was performed. A linear fracture and dural defect in the midline was identified, which was closed with fascial graft after removing the inner table of the skull. Cranioplasty was performed using the expanded calvarial bone. Ventriculoperitoneal shunt insertion was performed for hydrocephalus, and the patient improved remarkably. Posttraumatic intradiploic CSF collection, although a benign condition, may present with severe complications if treatment is delayed. Early diagnosis and treatment is essential. The authors suggest that this condition should be treated early, as for growing skull fractures.

Object The authors' experience with treatment of 8 patients with “vertical mobile and reducible” atlantoaxial dislocation is reviewed. The probable pathogenesis, radiological and clinical features, and management issues in such cases are discussed. Methods Between January 2006 and March 2008, 8 patients who presented with vertical mobile and reducible atlantoaxial dislocations were treated at the Department of Neurosurgery at King Edward Memorial Hospital in Mumbai, India. The vertical atlantoaxial dislocation/basilar invagination reduced completely on extension of the neck, with no need of any cervical traction. According to the extent of superior migration of the odontoid process, and measurements based on the vertical atlantoaxial instability index, the dislocation was graded as mild, moderate, or severe. All patients were treated using the C-1 lateral mass and C-2 pars plate and screw method of fixation. Results The study group was composed of 5 male and 3 female patients (mean age 24 years, age range 8–54 years). All patients presented with the physical features of short neck, torticollis, pain in the nape of the neck, and varying degrees of quadriparesis. In 6 patients there was a history of trauma prior to the onset of major neurological symptoms. The dislocation was mild in 3 cases, moderate in 1, and severe in 4. All patients had clinical neurological improvement following surgery. The follow-up duration ranged from 4 to 30 months (mean 18 months). Conclusions Vertical mobile and reducible atlantoaxial dislocation is a discrete clinical entity. Abnormal inclination and incompetence of the facet joint appears to be the primary causative factor that resulted in vertical dislocation or basilar invagination. Posterior fixation in the reduced dislocation position forms the basis of treatment.

Object The authors investigated the changes in the bone architecture and the characteristics of the neck and craniovertebral region in selected cases of basilar invagination. The reversal in these changes that occurred after decompression and fixation are analyzed. The implications of such an analysis in understanding the pathogenesis of a number of features that are characteristically associated with basilar invagination are evaluated. Methods One hundred and seventy selected patients with basilar invagination who underwent atlantoaxial joint distraction-fixation surgery at the authors' institution between 1999 and April 2008 were reviewed. The study was prospective after June 2006. A variety of parameters were used for radiological and physical assessments. The evaluation was done on the basis of pre- and postoperative imaging studies and clinical photographs. In the 41 prospective cases, additional direct physical measurements of the neck were performed. Results Prior to surgery there were several physical changes such as reduced neck length, torticollis, exaggerated lordosis of the cervical spine, and reduced craniospinal angulation. Other findings included reduced discspace height, significant posterior cervical osteophyte formation, assimilation of atlas (72%), single-level (29%) or multiple-level (3%) cervical fusions, and an increase in the spinal subarachnoid space both above and below the level of maximum neural compression at the tip of the odontoid process. After surgical decompression of the region, there was remarkable recovery in craniovertebral alignments, and an increase in neck length (maximum up to 42 mm) was obvious on physical and radiological examination in 85% of patients. The disc-space height increased and there was a reversal of altered cervical lordosis, craniospinal angulation (maximum up to 36°), and torticollis. Conclusions It appears that a number of physical spinal changes characteristically associated with basilar invagination such as a short neck, exaggerated neck lordosis, torticollis, cervical spondylotic changes and fusions are potentially reversible after decompression and stabilization of the craniovertebral junction.

Osteogenesis imperfecta (OI) is a disorder of bone development caused by a genetic dysfunction of collagen synthesis. Basilar invagination (BI) is an uncommon but serious complication of OI. Brainstem decompression in OI is undertaken in certain circumstances. Transoral-transpalatopharyngeal ventral decompression with posterior occipitocervical fusion has become the treatment of choice when required. This technical note outlines a novel endoscopic transnasal approach for ventral decompression. The literature is reviewed and a strategy for the management of BI in patients with OI is outlined.

Object Basilar impression (BI) secondary to osteogenesis imperfecta (OI) is a rare but debilitating condition that is often progressive unless it is halted. More recently, ventral decompression surgery has been advocated for this condition. This study is a retrospective review of the 21-year experience of ventral decompression surgery and dorsal occipitocervical fixation in patients with BI secondary to OI and is the largest patient series reported to date. Methods Twenty patients treated between 1982 and 2003 by the senior author at the authors' institution were included in this study. All patients underwent ventral decompression surgery followed by dorsal craniocervical stabilization. Patients were followed up for a median of 10 years. Results There were no intraoperative or perioperative deaths. Postoperatively, 16 of 20 (80%) patients showed objective improvement or maintained their good preoperative level of function. After surgery, of the 15 patients admitted with Karnofsky Performance Scale (KPS) scores of 70% or less, 11 improved, two remained unchanged, one patient's condition deteriorated, and one patient died of an unrelated cause. Of five patients admitted with a KPS score of 80% or greater, no patient's condition deteriorated in the short- and midterm period, but one patient had recurrence 15 years after surgery. At the end of follow-up, 25% of the patients had recurrence of brainstem compression symptoms or had died, and 15% showed no improvement after surgery. All of the remaining patients (60%) had sustained a long-term benefit from surgery. Conclusions Aggressive ventral decompression surgery and dorsal stabilization for patients with BI secondary to OI can not only halt disease progression but can also produce a good and sustainable long-term functional outcome, even in those patients who present as severely symptomatic. Patients who presented early with minor symptoms had good long-term outcomes.

✓ The upward odontoid displacement observed in basilar invagination (BI) is generally associated with a horizontal clivus and craniocervical kyphosis, conditions that exert ventral compression at the spinomedullary junction. Ventral brainstem decompression by reduction or elimination of the odontoid invagination is part of the desired treatment. The authors describe a case of BI in an adult, who was effectively treated with the easy and safe reduction of odontoid invagination via cervical traction. Normalization of kyphosis at the craniovertebral junction and the vertical position of both a previously horizontal clivus and the cerebellar tentorium demonstrated that these conditions were not part of the original malformation but instead were caused by a reducible craniovertebral instability.

Object. Instability of the occipitocervical junction may result from degenerative disease, infection, tumor, and trauma. Surgical stabilization involving screw fixation and rigid implants has been found to be biomechanically superior to wire-based implants. To evaluate the long-term results in a large and diverse patient population, the authors prospectively studied a consecutive group of 69 patients. Methods. All patients underwent occipitocervical fusion in which rigid posterior instrumentation included either plates or rods and screws. Patients ranged in age from 11 to 90 years (mean 51.4 years); there were 34 female and 35 male patients. The mean follow-up duration was 37 months (range 6–66 months). Fifty-seven (83%) of the 69 patients had long-standing occipitocervical anomalies, whereas the remainder presented with acute instability. Basilar invagination was present in 20 patients. Results. Correction of a severe cervical kyphotic deformity was accomplished in six patients. There were no fatalities or medical complications associated with the procedures. During the follow-up period, 87% of the patients exhibited improvement in their myelopathic symptoms; in 13% the symptoms were unchanged. Complications were minimal. Stability was demonstrated on flexion/extension studies in all cases. There were no treatment-related deaths, although four patients died within the follow-up period, all due to progression of metastatic disease. Conclusions. The authors found that rigid internal fixation of the occipitocervical complex was safe, effective, and technically possible for spine surgeons familiar with occipital bone anatomy and lateral mass fixation.

✓Symptomatic irreducible basilar invagination has traditionally been approached through a transoral–transpharyngeal route with resection of the anterior portion of C-1 and the odontoid. Modification of this exposure with either a Le Fort osteotomy or a transmandibular osteotomy and circumglossal approach has increased the access to pathological conditions in this region. These traditional routes all require traversing the oral cavity and accepting the associated potential complications. The authors have developed a novel surgical approach, an endoscopic transcervical odontoidectomy, which allows access for resection of the odontoid and for brainstem and spinal cord decompression without traversing the oral cavity. In this paper they describe the technique and its advantages and present three cases in which patients underwent the endoscopic transcervical odontoidectomy for basilar invagination.Three consecutive patients (age range 42–74 years) who had irreducible basilar invagination underwent the endoscopic transcervical odontoidectomy. All were symptomatic and had neck pain and myelopathy. All were evaluated preoperatively and postoperatively with computed tomography and magnetic resonance imaging. In all cases the procedure resulted in complete decompression. There were no serious complications. No patient required prolonged intubation, tracheostomy, or enteral tube feeding. One patient had an intraoperative cerebrospinal fluid leak, which had no postoperative sequelae.The authors present an alternative surgical approach for treating ventral compression of the brainstem and spinal cord. The technique is safe and effective for decompression and provides a surgical route that can be added to the armamentarium of treatments for pathological conditions in this region.

✓The authors describe a previously unreported clinical sign that may indicate the onset of significant compression of the medulla oblongata in cases of craniovertebral junction abnormalities. This 17-year-old boy presented with mild bilateral leg weakness. Imaging studies revealed severe basilar invagination and a marked Chiari malformation. While awaiting surgery, his tongue developed an involuntary constant protrusion–intrusion repetitive motion. The onset of this so-named “trombone tongue” sign was followed shortly afterward by rapidly progressive spastic tetraparesis. After the authors performed a transmaxillary clivectomy, foramen magnum decompression, and occipitocervical fusion, they noted that the abnormal tongue motion promptly resolved and the tetraparesis gradually improved. The authors discuss their current understanding of the central control of tongue movements and present a hypothesis on the pathogenesis of trombone tongue based on the neuroanatomical basis of another abnormal tongue movement sign, lingual myoclonus.

The reduction of basilar invagination associated with Chiari I malformations in pediatric patients is often possible. Eleven children ranging in age from 1.5 to 17 years underwent a novel treatment method involving decompression, manual reduction, and posterior instrumentation-augmented fusion. Outcomes were evaluated retrospectively.After decompression of the lesion, manual craniocervical distraction and extension were performed, followed by posterior occipitocervical fusion involving rigid internal fixation. All patients were symptomatic at presentation. Intraoperative monitoring included somatosensory evoked potentials, fluoroscopy, and direct intradural demonstration of the craniocervical junction. No new neurological deficits occurred immediately after surgery. Perioperatively, symptoms in seven patients improved significantly and in four they remained unchanged. Three patients required subsequent transoral resections. During long-term follow up (mean 39.4 months, range 3-92 months) symptoms improved markedly in nine, remained unchanged in one, and progressively worsened in one patient.In selected cases, manual cervical distraction and extension, posterior fixation, and fusion appear to provide a safe, effective standalone treatment for basilar invagination associated with Chiari malformation in children.

Object. Various approaches have been described for resection of the dens of the axis, each of which has potential advantages and disadvantages. Anterior approaches such as the transoral route or its modifications are the most commonly used for resection of this structure. The transcondylar approach, however, which allows the surgeon to view the craniovertebral junction (CVJ) from a lateral perspective, has been introduced by Al-Mefty, et al., as an alternative approach. In this report, the authors describe the surgical technique of the extreme lateral—transatlas approach and their clinical experiences. Methods. The authors first examined the surgical approach to the dens from a lateral perspective in five cadaveric heads. They found that removal of the lateral mass of the atlas provided adequate exposure for resection of the dens. Following this cadaveric study, the extreme lateral—transatlas approach was successfully performed at the authors' institution over a 1-year period (September 1998–August 1999) in five patients with basilar invagination due to congenital anomaly of the CVJ and rheumatoid arthritis. Furthermore, during the same procedure, unilateral occipitocervical fusion was performed following resection of the dens. In all cases complete resection of the dens was achieved using the extreme—lateral transatlas approach. This procedure provides a sterile operative field and the ability to perform occipitocervical fusion immediately following the resection. No postoperative complications or craniocervical instability were observed. The mean follow-up period was 17.2 months (range 13–24 months). Conclusions. The extreme lateral—transatlas approach for resection of the dens was found to be safe and effective. Knowledge of the anatomy of this region, especially of the V3 segment of the vertebral artery, is essential for the success of this procedure.

Object Because transoral decompression of the cervicomedullary junction is compromised by a narrow surgical corridor, the adequacy of decompression/resection may be difficult to determine. This is problematic as spinal hardware may obscure postoperative radiological assessment, or the patient may require reoperation. The authors report three patients in whom high-field intraoperative magnetic resonance (MR) images were acquired at various stages during the transoral resection of C-2 lesions causing craniocervical junction compression. Methods In all three patients the lesions involved the cervicomedullary junction: one case each of plasmacytoma and metastatic breast carcinoma involving the odontoid process and C-2 vertebral body, and one case of basilar invagination with a Chiari type I malformation. All three patients presented with progressive myelopathy. Surgery-planning MR imaging studies, performed after the induction of anesthesia, demonstrated the lesion and its relationship to the planned surgical corridor. Transoral exposure was achieved through placement of a Crockard retractor system. In one case the soft palate was divided. Interdissection MR imaging revealed that adequate decompression had been achieved in all cases. In the two patients with carcinoma, posterior instrumentation was placed to achieve spinal stabilization. Planned suboccipital decompression and fixation was averted in the third case because MR imaging demonstrated that excellent decompression had been achieved. Conclusions Intraoperatively acquired MR images were instrumental in determining the adequacy of surgical decompression. In one patient the MR images changed the planned surgical procedure. Importantly, the acquisition of intraoperative MR images did not adversely affect operative time or neurosurgical techniques, including the instrumentation procedure.

OBJECT The transoral approach is the gold standard for ventral decompression of the brainstem caused by craniovertebral junction (CVJ) pathology. This approach is often associated with significant morbidity, related to swallowing and respiratory complications. The endoscopic endonasal approach was introduced to reduce the rate of these complications. However, the exact role of this approach in the treatment of CVJ pathology is not well defined. METHODS A comprehensive literature search was performed to identify series of patients with pathology of the CVJ treated via the endoscopie endonasal approach. Data on patient characteristics, indications for treatment, complications, and outcome were obtained and analyzed. RESULTS Twelve studies involving 72 patients were included. The most common indications for treatment were rheumatoid pannus (38.9%) and basilar invagination (29.2%). Cerebrospinal fluid leak was found in 18% of cases intraoperatively and 4.2% of cases postoperatively. One case of meningitis complicated by sepsis and death represents the procedure-related mortality of 1.4%. Of the patients without preoperative swallowing impairment, 95% returned to oral feeding on the 3rd postoperative day. Ninety-three percent of patients experienced improvement in neurological symptoms after the procedure. CONCLUSIONS The endonasal endoscopie approach is effective for the treatment of neural compression caused by CVJ pathology. It offers advantages such as lower rates of postoperative dysphagia and respiratory complications when compared with the more traditional transoral approach. However, these 2 approaches should be seen as complementary rather than alternatives. Patient-related factors as well as the surgeon’s expertise must be considered when making treatment decisions.

The transoral approach is considered the gold-standard surgical route for performing anterior odontoidectomy and ventral decompression of the craniovertebral junction for pathological conditions that result in symptomatic cervicomedullary compression, including basilar invagination, rheumatoid pannus, platybasia with retroflexed odontoid processes, and neoplasms. Extended modifications to increase the operative corridor and exposure include the transmaxillary, extended “open-door” maxillotomy, transpalatal, and transmandibular approaches. With the advent of extended endoscopic endonasal skull base techniques, there has been increased interest in the last decade in the endoscopic endonasal transclival transodontoid approach to the craniovertebral junction. The endonasal route represents an attractive minimally invasive surgical alternative, especially in cases of irreducible basilar invagination in which the pathology is situated well above the palatine line. Angled endoscopes and instrumentation can also be used for lower-lying pathology. By avoiding the oral cavity and subsequently using a transoral retractor, the endonasal route has the advantages of avoiding complications related to tongue swelling, tracheal swelling, prolonged intubation, velopharyngeal insufficiency, dysphagia, and dysphonia. Postoperative recovery is quicker, and hospital stays are shorter. In this report, the authors describe and illustrate their method of purely endoscopic endonasal transclival odonotoidectomy for anterior decompression of the craniovertebral junction and describe various operative pearls and nuances of the technique for avoiding complications.

Object The goal of this study was to compare the indications, benefits, and complications between the endoscopic endonasal approach (EEA) and the microscopic transoral approach to perform an odontoidectomy. Transoral approaches have been standard for odontoidectomy procedures; however, the potential benefits of the EEA might be demonstrated to be a more innocuous technique. The authors present their experience with 12 consecutive cases that required odontoidectomy and posterior instrumentation. Methods Twelve consecutive cases of craniovertebral junction instability with or without basilar invagination were diagnosed at the National Institute of Neurology and Neurosurgery in Mexico City, Mexico, between January 2009 and January 2013. The EEA was used for 5 cases in which the odontoid process was above the nasopalatine line, and was compared with 7 cases in which the odontoid process was beneath the nasopalatine line; these were treated using the transoral microscopic approach (TMA). Odontoidectomy was performed after occipital-cervical or cervical posterior augmentation with lateral mass and translaminar screws. One case was previously fused (Oc–C4 fusion). The senior author performed all surgeries. American Spinal Injury Association scores were documented before surgical treatment and after at least 6 months of follow-up. Results Neurological improvement after odontoidectomy was similar for both groups. From the transoral group, 2 patients had postoperative dysphonia, 1 patient presented with dysphagia, and 1 patient had intraoperative CSF leakage. The endoscopic procedure required longer surgical time, less time to extubation and oral feeding, a shorter hospital stay, and no complications in this series. Conclusions Endoscopic endonasal odontoidectomy is a feasible, safe, and well-tolerated procedure. In this small series there was no difference in the outcome between the EEA and the TMA; however, fewer complications were documented with the endonasal technique.

Object In this study the authors share their experience using intraoperative spinal navigation and imaging for endoscopic transnasal approaches to the odontoid in 5 patients undergoing C1–2 surgery for basilar invagination at Stanford Hospital and Clinics from 2010 to 2013. Methods Of these 5 patients undergoing C1–2 surgery for basilar invagination, 4 underwent a 2-tiered anterior C1–2 resection with posterior occipitocervical fusion during a first stage surgery, followed by endoscopic endonasal odontoidectomy in a separate setting. Intraoperative stereotactic navigation was performed using a surgical navigation system in all cases. Navigation accuracy, characterized as target registration error, ranged between 0.8 mm and 2 mm, with an average of 1.2 mm. Intraoperative imaging using a CT scanner was also performed in 2 patients. Results Endoscopic decompression of the brainstem was achieved in all patients, and no intraoperative complications were encountered. All patients were extubated within 24 hours after surgery and were able to swallow within 48 hours. After appropriate initial reconstruction of the defect at the craniocervical junction, no postoperative CSF leakage, arterial injury, or need for reoperation was encountered; 1 patient developed mild postoperative velopharyngeal insufficiency that resolved by the 6-month follow-up evaluation. There were no deaths and no patients required tracheostomy placement. The average inpatient stay after surgery varied between 72 and 96 hours, without extended intensive care unit stays for any patient. Conclusions Technologies such as intraoperative CT scanning and merged MRI/CT can provide the surgeon with detailed, virtual real-time information about the extent of complex endoscopic vertebral segment resection and brainstem decompression and lessens the prospect of revision or secondary procedures in this challenging surgical corridor. Moreover, patients experience limited morbidity and can tolerate early oral intake after transnasal endoscopic odontoidectomy. Essential to the successful undertaking of these endoscopic adventures is 1) an understanding of the endoscopic nasal, skull base, and neurovascular anatomy; 2) advanced and extended-length instrumentation including navigation; and 3) a team approach between experienced rhinologists and spine surgeons comfortable with endoscopic skull base techniques

Chiari malformation is a developmental disorder that is often associated with other abnormalities of the cerebrospinal axis. Despite widespread recognition of this association, there is relatively little information on the treatment of these coexisting disorders in the setting of cerebellar tonsillar ectopia. In an effort to improve the care provided to pediatric patients with Chiari malformations the authors reviewed their management practices over the last 20 years. Specifically, they recorded presenting symptoms, radiological studies, comorbidities, and management (surgical and nonsurgical) of 112 patients (all < 20 years of age) with Chiari malformation without myelodysplasia. They found an associated syrinx in 29% of patients, basilar invagination in 17%, and scoliosis in 14%. The basal angle varied from 120 to 190° and Boogard's angle varied from 120 to 220°; both angles were larger than those measured in normal controls. The vertical height of the posterior fossa was shortened and the volume decreased as compared with normal controls. The surgical management of this group of patients included posterior decompressive (44%), combined transoral and posterior decompressive (31%), combined posterior decompressive and posterior fusion (8%), and multiple posterior decompressive procedures in the same patient (5%). The authors conclude that pediatric patients with a Chiari malformation should be specifically examined for evidence of additional craniovertebral malformations so that procedures are directed at correcting both the comorbidities and the herniation of the cerebellar tonsils through the foramen magnum.

Operative intervention for the treatment of instability at the craniovertebral junction in patients with Down's syndrome has become somewhat controversial because some authors have reported high surgery-related complication rates and suggested that the incidence of neurological abnormality associated with this abnormal motion may be low. In this report, the authors describe the clinical and radiographic findings in 33 patients treated at their institution. Common presenting symptoms included neck pain (14 patients), torticollis (12 patients), and myelopathy manifested as hyperreflexia (21 patients), or varying degrees of quadriparesis (11 patients). Four patients suffered acute neurological insults, two after receiving routine general anesthetics for minor surgical procedures and two other patients following minor falls. Atlantoaxial instability was the most common abnormality documented on radiography (22 patients). Atlantooccipital instability (15 patients) was also frequently observed and was coexistent with the presence of atlantoaxial luxations in 14 patients. A rotary component of the atlantoaxial luxation was present in 13 cases. In 17 patients bony anomalies were present, the most frequent of which was os odontoideum (10 patients). Twenty-four patients underwent operative intervention, and successful fusion was achieved in 23. In six of nine patients with basilar invagination, reduction was achieved with preoperative traction and thus avoided the need for ventral decompressive procedures. There were no cases of postoperative deterioration, and 22 patients made excellent or good recoveries. The results of this series highlight the clinicopathological phenomena of craniovertebral instability in patients with Down's syndrome and suggest that satisfactory outcomes can be achieved with a low rate of surgical morbidity.

✓ Guidelines are proposed for surgical management of symptomatic abnormalities of the craniocervical junction. Experience with 17 recent cases is described. Gas or metrizamide (Amipaque) myelograms with pluridirectional tomograms revealed the etiology and mechanisms of compression of the cervicomedullary junction, as well as its reducibility. Stabilization was the goal in treatment of reducible lesions. Decompression of the cervicomedullary junction was paramount in irreducible cases. Ventral compression was treated in nine patients by transoral transpalatine resection of the odontoid-clivus complex, and all nine improved. A posterior decompression was carried out when bone impingement was present from the dorsal aspect. Fusion was performed in cases in which stability was not achieved by either procedure.

✓ Cranial settling and basilar invagination with medullospinal compression is believed to have resulted in the production of a syrinx in a case of Paget's disease of the cranium. This mechanism of compression at the craniovertebral junction resembles the development of syringomyelia in Chiari malformations presenting in adult life.