The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

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.

Robotic-Assisted Tubular Transoral Parapharyngeal Approach to the Ventral Craniovertebral Junction.

BACKGROUND: Transoral robotic surgery (TORS) has become a routine technique for treating benign and malignant lesions of the oropharynx with the advantage of reducing morbidity compared to open surgical techniques. However, TORS has not been used routinely for accessing lesions of the spine. OBJECTIVE: To describe how TORS can be used to access spinal lesions. METHODS: We describe our technique of accessing the parapharyngeal space using the robotic technique, and then dissecting the prevertebral muscles to expose the ventral craniovertebral junction. Tubular retraction with endoscopic visualization is then employed for surgical resection. We then report a case of a 14-yr-old competitive athlete who presented with an osseous lesion of C1, which underwent resection using this novel TORS approach. RESULTS: Our patient underwent successful resection of a lateral C1 osteoid osteoma utilizing a combined TORS/endoscopic approach. She tolerated soft diet immediately and was discharged on postoperative day 2. Postoperative imaging revealed complete resection of the lesion, and she returned to competitive athletics within 6 wk. CONCLUSION: Utilizing this novel, robotic-assisted approach can definitively treat osseous cervical spine lesions while reducing morbidity, allowing for early return to normal diet and minimizing overall length of hospital stay.

Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups. CONCLUSION: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.

Beta-adrenergic blockade for attenuation of catecholamine surge after traumatic brain injury: a randomized pilot trial.

BACKGROUND: Beta-blockers have been proven in multiple studies to be beneficial in patients with traumatic brain injury. Few prospective studies have verified this and no randomized controlled trials. Additionally, most studies do not titrate the dose of beta-blockers to therapeutic effect. We hypothesize that propranolol titrated to effect will confer a survival benefit in patients with traumatic brain injury. METHODS: A randomized controlled pilot trial was performed during a 24-month period. Patients with traumatic brain injury were randomized to propranolol or control group for a 14-day study period. Variables collected included demographics, injury severity, physiologic parameters, urinary catecholamines, and outcomes. Patients receiving propranolol were compared with the control group. RESULTS: Over the study period, 525 patients were screened, 26 were randomized, and 25 were analyzed. Overall, the mean age was 51.3 years and the majority were male with blunt mechanism. The mean Injury Severity Score was 21.8 and median head Abbreviated Injury Scale score was 4. Overall mortality was 20.0%. Mean arterial pressure was higher in the treatment arm as compared with control (p=0.021), but no other differences were found between the groups in demographics, severity of injury, severity of illness, physiologic parameters, or mortality (7.7% vs. 33%; p=0.109). No difference was detected over time in any variables with respect to treatment, urinary catecholamines, or physiologic parameters. Glasgow Coma Scale (GCS), Sequential Organ Failure Assessment, and Acute Physiology and Chronic Health Evaluation scores all improved over time. GCS at study end was significantly higher in the treatment arm (11.7 vs. 8.9; p=0.044). Finally, no difference was detected with survival analysis over time between groups. CONCLUSIONS: Despite not being powered to show statistical differences between groups, GCS at study end was significantly improved in the treatment arm and mortality was improved although not at a traditional level of significance. The study protocol was safe and feasible to apply to an appropriately powered larger multicenter study. LEVEL OF EVIDENCE: Level 2-therapeutic.

Radiological and clinical predictors of scoliosis in patients with Chiari malformation type I and spinal cord syrinx from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Scoliosis is frequently a presenting sign of Chiari malformation type I (CM-I) with syrinx. The authors' goal was to define scoliosis in this population and describe how radiological characteristics of CM-I and syrinx relate to the presence and severity of scoliosis. METHODS: A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°). RESULTS: Based on available imaging of patients with CM-I and syrinx, 260 of 825 patients (31%) had a clear diagnosis of scoliosis based on radiographs or coronal MRI. Forty-nine patients (5.9%) did not have scoliosis, and in 516 (63%) patients, a clear determination of the presence or absence of scoliosis could not be made. Comparison of patients with and those without a definite scoliosis diagnosis indicated that scoliosis was associated with wider syrinxes (8.7 vs 6.3 mm, OR 1.25, p < 0.001), longer syrinxes (10.3 vs 6.2 levels, OR 1.18, p < 0.001), syrinxes with their rostral extent located in the cervical spine (94% vs 80%, OR 3.91, p = 0.001), and holocord syrinxes (50% vs 16%, OR 5.61, p < 0.001). Multivariable regression analysis revealed syrinx length and the presence of holocord syrinx to be independent predictors of scoliosis in this patient cohort. Scoliosis was not associated with sex, age at CM-I diagnosis, tonsil position, pB-C2 distance (measured perpendicular distance from the ventral dura to a line drawn from the basion to the posterior-inferior aspect of C2), clivoaxial angle, or frontal-occipital horn ratio. Average curve magnitude was 29.9°, and 37.7% of patients had a left thoracic curve. Older age at CM-I or syrinx diagnosis (p < 0.0001) was associated with greater curve magnitude whereas there was no association between syrinx dimensions and curve magnitude. CONCLUSIONS: Syrinx characteristics, but not tonsil position, were related to the presence of scoliosis in patients with CM-I, and there was an independent association of syrinx length and holocord syrinx with scoliosis. Further study is needed to evaluate the nature of the relationship between syrinx and scoliosis in patients with CM-I.

The Role of Computed Tomography and Magnetic Resonance Imaging in the Diagnosis of Pediatric Thoracolumbar Compression Fractures.

BACKGROUND: Because of concerns about radiation exposure, some centers consider magnetic resonance imaging (MRIs) the preferred imaging modality for pediatric thoracic and/or lumbar compression fractures. The purpose of this study was to evaluate the sensitivity of computed tomography (CT) and MRI in diagnosing thoracolumbar compression fractures and the utility of MRI in their management. METHODS: Retrospective review identified 52 patients aged 0 to 18 years with 191 thoracic and/or lumbar compression fractures who had both CT and MRI during the initial trauma evaluation. The decision to perform CT and/or MRI was made by the attending pediatric spine surgeon. In all cases the CT scan was performed before the MRI. All imaging studies were reviewed by a board-certified pediatric radiologist and attending pediatric spine surgeon. RESULTS: Only 10 patients (19%) had a single-level injury. Of 42 with multiple compression fractures, 34 (81%) had fractures in contiguous levels, and 8 had noncontiguous injuries. Comparing CT and MRI, there was complete agreement in the number and distribution of fractures in 23 patients (44%). MRI identified additional levels of fracture in 15 patients (29%); 14 (27%) had fewer levels fractured on MRI than CT. Only one patient (2%) had fractures seen on MRI after a normal CT scan. Complete correlation between CT and MRI was seen in 59% (17/29) of patients aged 11 to 18 years, compared with 26% (6/23) of patients younger than 11. CONCLUSIONS: In pediatric patients with mild thoracic or lumbar compression fracture(s), CT scan demonstrates a high sensitivity in determining the presence or absence of a fracture compared with MRI. Although some variability exists between the 2 modalities in the exact number of spinal levels involved, the definitive treatment and outcome were not changed by the addition of MRI. The information that may be obtained from an MRI must be weighed against the increased time and expense of the study, as well as the risks associated with sedation when necessary. LEVEL OF EVIDENCE: Level II-diagnostic study.

Impact of venous thromboembolism chemoprophylaxis on postoperative hemorrhage following operative stabilization of spine fractures.

BACKGROUND: Prophylactic anticoagulation may decrease the risk of venous thromboembolism (VTE) in patients with spine fractures following blunt trauma but comes with the threat of postoperative bleeding in patients requiring stabilization of these fractures. The purpose of this study was to evaluate the impact of preoperative anticoagulation on VTE and bleeding complications in patients with blunt spine fractures requiring operative stabilization. METHODS: All patients with blunt spine fractures requiring operative stabilization over a 6-year period were identified. Patients with a hospital stay of less than 48 hours or missing data were excluded. Patients were stratified by age; severity of shock, spinal cord injury, fracture location, injury severity; and timing and duration of anticoagulation. Outcomes included bleeding complications (wound hematoma/infection and development of epidural hematoma) and VTE (pulmonary embolism and deep venous thrombosis). Outcomes were evaluated to determine risk factors for bleeding complications and VTE in the management of operative spine fractures. RESULTS: Seven hundred five patients were identified: 355 patients received one dose or more of preoperative anticoagulation, and 350 did not receive preoperative anticoagulation. Seventy-two percent were male, with a mean injury severity score and Glasgow Coma Scale score of 21 and 14, respectively. Bleeding complications occurred in 18 patients (2.6%), and 20 patients (2.8%) had VTE. Patients with VTE were more severely injured (Glasgow Coma Scale score of 13 vs 15, p ≤ 0.001 and injury severity score of 27 vs 18, p = 0.008). Despite longer time to mobilization (4 vs 2 days, p < 0.001), patients who received 50% or more of their scheduled preoperative doses had fewer episodes of pulmonary embolism (0.4% vs 2.2%, p < 0.05), with no difference in bleeding complications (2.1% vs 2.9%, p = 0.63) compared to patients who received either no preoperative anticoagulation or less than 50% of their scheduled preoperative doses. CONCLUSIONS: Preoperative anticoagulation in patients with operative spine fractures reduced the risk of pulmonary embolism without increasing bleeding complications. Preoperative anticoagulation is both safe and beneficial in patients with operative spine fractures. LEVEL OF EVIDENCE: Therapeutic study, level IV.

Pediatric intracranial gunshot wounds: the Memphis experience.

OBJECTIVE Penetrating brain injury in civilians is much less common than blunt brain injury but is more severe overall. Gunshot wounds (GSWs) cause high morbidity and mortality related to penetrating brain injury; however, there are few reports on the management and outcome of intracranial GSWs in children. The goals of this study were to identify clinical and radiological factors predictive for death in children and to externally validate a recently proposed pediatric prognostic scale. METHODS The authors conducted a retrospective review of penetrating, isolated GSWs sustained in children whose ages ranged from birth to 18 years and who were treated at 2 major metropolitan Level 1 trauma centers from 1996 through 2013. Several standard clinical, laboratory, and radiological factors were analyzed for their ability to predict death in these patients. The authors then applied the St. Louis Scale for Pediatric Gunshot Wounds to the Head, a scoring algorithm that was designed to provide rapid prognostic information for emergency management decisions. The scale's sensitivity, specificity, and positive and negative predictability were determined, with death as the primary outcome. RESULTS Seventy-one children (57 male, 14 female) had a mean age of 14 years (range 19 months to 18 years). Overall mortality among these children was 47.9%, with 81% of survivors attaining a favorable clinical outcome (Glasgow Outcome Scale score ≥ 4). A number of predictors of mortality were identified (all p < 0.05): 1) bilateral fixed pupils; 2) deep nuclear injury; 3) transventricular projectile trajectory; 4) bihemispheric injury; 5) injury to ≥ 3 lobes; 6) systolic blood pressure < 100 mm Hg; 7) anemia (hematocrit < 30%); 8) Glasgow Coma Scale score ≤ 5; and 9) a blood base deficit < -5 mEq/L. Patient age, when converted to a categorical variable (0-9 or 10-18 years), was not predictive. Based on data from the 71 patients in this study, the positive predictive value of the St. Louis scale in predicting death (score ≥ 5) was 78%. CONCLUSIONS This series of pediatric cranial GSWs underscores the importance of the initial clinical exam and CT studies along with adequate resuscitation to make the appropriate management decision(s). Based on our population, the St. Louis Scale seems to be more useful as a predictor of who will survive than who will succumb to their injury.

Results of delayed follow-up imaging in traumatic brain injury.

OBJECTIVE: There is a paucity of scientific evidence available about the benefits of outpatient follow-up imaging for traumatic brain injury patients. In this study, 1 year of consecutive patients at a Level 1 trauma center were analyzed to determine if there is any benefit to routinely obtaining CT of the head at the outpatient follow-up visit. METHODS: This single-institution retrospective review was performed on all patients with a traumatic brain injury seen at a Level 1 trauma center in 2013. Demographic data, types of injuries, surgical interventions, radiographic imaging in inpatient and outpatient settings, and outcomes were assessed through a review of the institution's trauma registry, patient charts, and imaging. RESULTS: Five hundred twenty-five patients were seen for traumatic brain injury in 2013 at Regional One Health in Memphis, Tennessee. One hundred eighty-five patients (35%) presented for outpatient follow-up, all with CT scans of the head. Seven of these patients (4%) showed worsening of their intracranial injuries on outpatient imaging studies; however, surgical intervention was recommended for only 3 of these patients (2%). All patients requiring an intervention had neurological deterioration prior to their follow-up appointment. CONCLUSIONS: These experiences suggest that outpatient follow-up imaging for traumatic brain injury should be done selectively, as it was not helpful for patients who did not exhibit worsening of neurological signs or symptoms. Furthermore, routine outpatient imaging results in unnecessary resource utilization and radiation exposure.

A meta-analysis of spinal surgical site infection and vancomycin powder.

OBJECT: Surgical site infection (SSI) is a serious and costly complication of spinal surgery. There have been several conflicting reports on the use of intrawound vancomycin powder in decreasing SSI in spine surgery. The purpose of this study is to answer the question: "Does intrawound vancomycin powder reduce the rate of SSIs in spine surgery?" METHODS: A comprehensive search of multiple electronic databases and bibliographies was conducted to identify clinical studies that evaluated the rates of SSI with and without the use of intrawound vancomycin powder in spine surgery. Independent reviewers extracted data and graded the quality of each paper that met inclusion criteria. A random effects meta-analysis was then performed. RESULTS: The search identified 9 retrospective cohort studies (Level III evidence) and 1 randomized controlled trial (Level II evidence). There were 2574 cases and 106 infections in the control group (4.1%) and 2518 cases and 33 infections (1.3%) in the treatment group, yielding a pooled absolute risk reduction and relative risk reduction of 2.8% and 68%, respectively. The meta-analysis revealed the use of vancomycin powder to be protective in preventing SSI (relative risk = 0.34, 95% confidence interval 0.17-0.66, p = 0.021). The number needed to treat to prevent 1 SSI was 36. A subgroup analysis found that patients who had implants had a reduced risk of SSI with vancomycin powder (p = 0.023), compared with those who had noninstrumented spinal operations (p = 0.226). CONCLUSIONS: This meta-analysis suggests that the use of vancomycin powder may be protective against SSI in open spinal surgery; however, the exact population in which it should be used is not clear. This benefit may be most appreciated in higher-risk populations or in facilities with a high baseline rate of infection.

Traumatic atlanto-occipital dislocation in children.

Although once considered an invariably fatal injury, improvements in diagnosis and management have made atlanto-occipital dislocation (AOD) a survivable injury. MRI is the preferred imaging modality; occasionally, flexion/extension/distraction fluoroscopy may be required to determine craniovertebral stability. Early surgical stabilization is recommended for all children with AOD. Early occipitocervical fusion using screws in combination with a rod or plate, or sublaminar wires with a contoured rod, coupled with autograft bone, provide immediate stabilization and a high fusion rate. Halo immobilization and traction are contraindicated in the management of AOD in children because of the risk of displacement of the injured occipitocervical joint. Postoperative hydrocephalus is frequent and should be suspected when neurologic decline occurs after fixation. Nearly half of children who survive AOD will have residual neurologic deficits.

Significance of hydrogen breath tests in children with suspected carbohydrate malabsorption.

BACKGROUND: Hydrogen breath tests are noninvasive procedures frequently applied in the diagnostic workup of functional gastrointestinal disorders. Here, we review hydrogen breath test results and the occurrence of lactose, fructose and sorbitol malabsorption in pediatric patients; and determine the significance of the findings and the outcome of patients with carbohydrate malabsorption. METHODS: We included 206 children (88 male, 118 female, median age 10.7 years, range 3-18 years) with a total of 449 hydrogen breath tests (lactose, n = 161; fructose, n = 142; sorbitol, n = 146) into a retrospective analysis. Apart from test results, we documented symptoms, the therapeutic consequences of the test, the outcome and the overall satisfaction of the patients and families. RESULTS: In total, 204 (46%) of all breath tests were positive. Long-term follow-up data could be collected from 118 patients. Of 79 patients (67%) who were put on a diet reduced in lactose, fructose and/or sorbitol, the majority (92%, n = 73) reported the diet to be strict and only 13% (n = 10) had no response to diet. Most families (96%, n = 113) were satisfied by the test and the therapy. There were only 21 tests (5%) with a borderline result because the criteria for a positive result were only partially met. CONCLUSIONS: Hydrogen breath tests can be helpful in the evaluation of children with gastrointestinal symptoms including functional intestinal disorders. If applied for a variety of carbohydrates but only where indicated, around two-third of all children have positive results. The therapeutic consequences are successfully relieving symptoms in the vast majority of patients.

Traumatic atlanto-occipital dislocation in children: evaluation, treatment, and outcomes.

BACKGROUND: Advancements in emergency care and diagnostic methods have increased the number of children who survive atlanto-occipital dislocation. We report our experience with one of the largest series of pediatric patients with atlanto-occipital dislocation. METHODS: Patients ranging in age from newborn to sixteen years old who had a diagnosis of atlanto-occipital dislocation from 1991 through 2011 were identified. Inclusion criteria were complete radiographic and clinical records and duration of follow-up of at least six months. Basic patient characteristics, mechanism of injury, associated injuries, neurological impairment, surgical treatment and type of implant used for fixation, complications, and clinical and radiographic outcomes were recorded. RESULTS: The fourteen patients who were included (seven male and seven female) had a mean age of 5.2 years at the time of injury. An automobile accident in which the victim was a passenger was the most common mechanism of injury. Twelve patients had associated injuries, with a brain injury in eleven of them, and nearly half sustained a spinal cord injury. According to the Traynelis classification system, eight patients had a type-II (longitudinal) atlanto-occipital dislocation, five had a type-I (anterior) dislocation, and one had a type-III (posterior) dislocation. All patients had posterior occipitocervical fusion with internal fixation. The mean duration of follow-up was 75.4 months. The most common postoperative complication was hydrocephalus, which occurred in four patients. Spinal fusion occurred in all patients by four to six months postoperatively. At the time of the most recent follow-up evaluation, half of the patients had neurological impairment. CONCLUSIONS: More patients with atlanto-occipital dislocation now survive the initial trauma, although most have associated injuries and many have neurological impairment. Our preferred management is early occipitocervical fusion and stabilization. If there is neurological decline after spinal fixation, obstructive hydrocephalus should be suspected.

Multifocal Cryptococcus neoformans var. neoforms: infection, treatment, and monitoring by serial computed tomography in a Schmidt's red-tailed guenon (Cercopithecus ascanius schmidti).

A 9-yr-old, female, intact Schmidt's red-tailed guenon (Cercopithecus ascanius schmidti) presented with an acute swelling, consistent with an abscess over the right hip. Cytology, culture, and polymerase chain reaction (PCR) results from material within the abscess and serum titers were all indicative of a Cryptococcus neoformans infection. Thoracic radiographs and computed tomography (CT) revealed a consolidated right caudal lung lobe associated with an endobronchial mass. Pulmonary Cryptococcus infection and systemic disease was confirmed with positive brush cytology and bronchoalveolar lavage (BAL). Culture and sensitivity, minimum inhibitory concentration values, and serum fluconazole level results were used to develop and monitor treatment efficacy for this animal. Sequential thoracic radiographs, CT images, bronchoscopy, BAL, and serology were used to monitor response to therapy. Five months after initial diagnosis, pregnancy was confirmed and the frequency of immobilizations for recheck serology and diagnostic imaging was decreased to lessen radiation exposure and physiologic stress to the dam and fetus. Long-term administration of oral fluconazole was well accepted and effective at treating the multifocal C. neoformans infection. No long-term adverse effects of therapy were observed, and repeated diagnostics were well tolerated. Advanced imaging modalities and serum drug levels were critical to the assessment, monitoring, and treatment of infection in this animal and should be considered for other cases.

An accessory limb with lipomyelomeningocele in a male.

At 7 months, an infant born with a third limb attached to a lumbosacral mass with an associated lipomyelomeningocele underwent removal of the limb and spinal cord detethering. Depending on the complexity of the pathology and proximity of the limb to viscera, consultation with neurosurgical and surgical colleagues is recommended.

Occipitocervical fusion using a contoured rod and wire construct in children: a reappraisal of a vintage technique.

OBJECT: Many methods to stabilize and fuse the craniocervical junction have been described. One of the early designs was a contoured (Luque) rod fixated with wires, the so-called Hartshill-Ransford loop. In this study, the authors report their 20-year experience with this surgical technique in children. METHODS: The authors reviewed the medical records of patients 18 years of age or younger who underwent dorsal occipitocervical fusion procedures between March 1992 and March 2012 at Le Bonheur Children's Hospital using a contoured rod and wire construct. Data on basic patient characteristics, causes of instability, neurological function at presentation and at last follow-up, details of surgery, complications, and radiographic outcome were collected. RESULTS: Twenty patients (11 male) were identified, with a mean age of 5.5 years (range 1-18 years) and a median follow-up of 43.5 months. Fourteen patients had atlantooccipital dislocation, 2 patients had atlantoaxial fracture-dissociations, 2 had Down syndrome with occipitocervical and atlantoaxial instability, 1 had an epithelioid sarcoma from the clivus to C-2, and 1 had an anomalous atlas with resultant occipitocervical instability. Surgical stabilization extended from the occiput to C-1 in 3 patients, C-2 in 6, C-3 in 8, and to C-4 in 3. Bone morphogenetic protein was used in 2 patients. Two patients were placed in a halo orthosis; the rest were kept in a hard collar for 6-8 weeks. All patients were neurologically stable after surgery. One patient with a dural tear experienced wound dehiscence with CSF leakage and required reoperation. Eighteen patients went on to achieve fusion within 6 months of surgery; 1 patient was initially lost to follow-up, but recent imaging demonstrated a solid fusion. There were no early hardware or bone failures requiring hardware removal, but radiographs obtained 8 years after surgery showed that 1 patient had an asymptomatic fractured rod. There were no instances of symptomatic junctional degeneration, and no patient was found to have increasing lordosis over the fused segments. Five (31%) of the 16 trauma patients required a shunt for hydrocephalus. CONCLUSIONS: Despite the proliferation of screw-fixation techniques for craniocervical instability in children, the contoured rod-wire construct remains an effective, less expensive, and technically easier alternative that has been in use for almost 30 years. It confers immediate stability, and therefore most patients will not need to be placed in a halo device postoperatively. A secondary observation in our series was the high (30%) rate of hydrocephalus requiring a shunt in patients with traumatic instability.

The relationship between serum sodium and intracranial pressure when using hypertonic saline to target mild hypernatremia in patients with head trauma.

INTRODUCTION: Limited data suggest mild hypernatremia may be related to lower intracranial pressure (ICP) in patients with traumatic brain injury (TBI). The practice at the study center has been to use hypertonic saline (HTS) to generate a targeted serum sodium of 145 to 155 mEq/l in patients with TBI. The purpose of this study was to determine the relationship between serum sodium values and ICP, and to evaluate the acute effect of HTS on ICP. METHODS: A retrospective review of patients who were admitted to the trauma ICU for TBI, had an ICP monitor placed, and received at least one dose of HTS between January 2006 and March 2011 was performed. Data were collected for up to 120 hours after ICP monitor placement. The primary outcome was the relationship between serum sodium and maximum ICP. Secondary outcomes were the relationship between serum sodium and the mean number of daily interventions for ICP control, and the acute effect of HTS on ICP during the 6 hours after each dose. Linear regression was used to analyze the primary outcome. Analysis of variance on ranks and repeated measures analysis of variance were used to evaluate the number of interventions and the acute effect of HTS on ICP, respectively. RESULTS: Eighty-one patients were enrolled with mean ± standard deviation age of 36 ± 15 years and median Glasgow Coma Scale score of 7 (interquartile range, 4 to 7). A total of 1,230 serum sodium values (range, 118 to 174 mEq/l) and 7,483 ICP values (range, 0 to 159 mmHg) were collected. There was no correlation between serum sodium and maximum ICP (R(2) = 0.0052). The overall mean ± standard deviation number of interventions for elevated ICP per day was 4.2 ± 2.9, 2.9 ± 2.0, and 2.6 ± 2.3 for patients with a mean serum sodium of < 145, 145 to 155, and > 155 mEq/l, respectively (P < 0.001). Regarding the acute effect of HTS on ICP, there was no statistical difference in mean ICP compared with baseline during hours 1 through 6 following HTS doses (baseline, 13.7 ± 8.4 mmHg; hour 1, 13.6 ± 8.3 mmHg; hour 2, 13.5 ± 8.8 mmHg; hour 3, 13.3 ± 8.7 mmHg; hour 4, 13.4 ± 8.7 mmHg; hour 5, 13.4 ± 8.3 mmHg; hour 6, 13.5 ± 8.3 mmHg; P = 0.84). CONCLUSIONS: Serum sodium concentrations did not correlate with ICP values. These results warrant further evaluation and possible reassessment of sodium goals for ICP management in patients with TBI.