Pediatric fibrocartilaginous embolism inducing paralysis.

BACKGROUND: Fibrocartilaginous embolism (FCE) is a rare cause of pediatric ischemic myelopathy. The pathology is thought to result from fragmentation with embolization into the microvasculature of the radicular artery often secondary to high axial force. While most cases arise in the setting of vigorous activity, our case reveals that FCE can also occur during relative physical inactivity. Additionally, while a majority of cases are associated with neck or back pain, our case also reveals that FCE can present without concurrent pain episodes. We describe a rare case of spinal cord infarction (SCI) likely due to FCE in a 14 year old male. Our patient was sitting with his feet elevated, playing a video game, when he developed sudden difficulty moving his arms. Initially presenting with a negative MRI scan and la belle indifference, our patient was suspected to exhibit functional quadraparesis secondary to psychosomatization/adjustment disorder. Repeat MR imaging 7 days later revealed typical findings for FCE with SCI (irregular, pencil-like T2 hyperintensity in the ventral cervical/upper thoracic cord and owl's eye pattern on axial images). Six months later, the diagnosis of FCE remains predominant. Our patient continues to improve with occupational and physical therapy. Ambulatory efforts and bladder function continue to progress. To improve functional gains, the patient is being considered for a chemodenervation procedure. CONCLUSION: Our case reveals that FCE can occur during physical inactivity and present without concurrent pain. Outcome regarding pediatric fibrocartilaginous embolism is highly variant; however, the two largest outcomes reported were either patient death or discharge.

Critical assessment of pediatric neurosurgery patient/parent educational information obtained via the Internet.

OBJECTIVE The Internet is used frequently by patients and family members to acquire information about pediatric neurosurgical conditions. The sources, nature, accuracy, and usefulness of this information have not been examined recently. The authors analyzed the results from searches of 10 common pediatric neurosurgical terms using a novel scoring test to assess the value of the educational information obtained. METHODS Google and Bing searches were performed for 10 common pediatric neurosurgical topics (concussion, craniosynostosis, hydrocephalus, pediatric brain tumor, pediatric Chiari malformation, pediatric epilepsy surgery, pediatric neurosurgery, plagiocephaly, spina bifida, and tethered spinal cord). The first 10 "hits" obtained with each search engine were analyzed using the Currency, Relevance, Authority, Accuracy, and Purpose (CRAAP) test, which assigns a numerical score in each of 5 domains. Agreement between results was assessed for 1) concurrent searches with Google and Bing; 2) Google searches over time (6 months apart); 3) Google searches using mobile and PC platforms concurrently; and 4) searches using privacy settings. Readability was assessed with an online analytical tool. RESULTS Google and Bing searches yielded information with similar CRAAP scores (mean 72% and 75%, respectively), but with frequently differing results (58% concordance/matching results). There was a high level of agreement (72% concordance) over time for Google searches and also between searches using general and privacy settings (92% concordance). Government sources scored the best in both CRAAP score and readability. Hospitals and universities were the most prevalent sources, but these sources had the lowest CRAAP scores, due in part to an abundance of self-marketing. The CRAAP scores for mobile and desktop platforms did not differ significantly (p = 0.49). CONCLUSIONS Google and Bing searches yielded useful educational information, using either mobile or PC platforms. Most information was relevant and accurate; however, the depth and breadth of information was variable. Search results over a 6-month period were moderately stable. Pediatric neurosurgery practices and neurosurgical professional organization websites were inferior (less current, less accurate, less authoritative, and less purposeful) to governmental and encyclopedia-type resources such as Wikipedia. This presents an opportunity for pediatric neurosurgeons to participate in the creation of better online patient/parent educational material.

Mature Teratoma Confined to the Posterior Fossa.

Mature teratomas located solely in the posterior fossa are rare. We describe a girl who presented with hydrocephalus caused by a posterior fossa tumor that was ultimately diagnosed as a mature teratoma following complete extirpation. Unusual imaging characteristics which produced confusion preoperatively were, however, very consistent with mature teratomas that are encountered in the gonads. Immature elements were universally absent; therefore, extirpation was curative. Hydrocephalus is unlikely to resolve after tumor removal, and cerebrospinal fluid diversion may be required.

Inappropriate use of infant seating devices increases risks of injury.

PURPOSE: The purpose of our study was to investigate the epidemiology and resulting injuries following falls sustained by infants seated in a variety of seating devices. METHODS: A retrospective chart review of a cohort of infants less than 12 months old who presented to our institution from 1991 to 2010 after a fall from various seating devices was performed. RESULTS: Two hundred five infants were identified, including 146 patients who were admitted to our institution (1991-2010) and 59 patients who were seen and discharged from the ED (2008-2010). Mean age of admitted infants was younger (3.5 vs. 5.3 months). Two patients (1%) required surgery for a depressed skull fracture. Overall, 18% had an intra-cranial hemorrhage. More patients requiring an admission secondary to their injuries fell from a table or counter (42% vs. 27%). CONCLUSION: Falls sustained by children seated in a variety of devices are frequent. Failure to restrain children in seating devices or improperly placing them on a table/counter is associated with more significant injuries. In order to minimize such injuries, it is important to educate caregivers of the risk in utilizing such seating devices.

Nonendoscopic, minimally invasive calvarial vault remodeling without postoperative helmeting for sagittal synostosis.

OBJECT: Multiple surgical procedures have been described for the management of isolated nonsyndromic sagittal synostosis. Minimally invasive techniques have been recently emphasized, but these techniques necessitate the use of an endoscope and postoperative helmeting. The authors assert that a safe and effective, more "minimalistic" approach is possible, avoiding the use of endoscopic visualization and routine postoperative application of a cranial orthosis. METHODS: A single-institution cohort analysis was performed on 18 cases involving infants treated for isolated nonsyndromic sagittal synostosis between 2008 and 2010 using a nonendoscopic, minimally invasive calvarial vault remodeling (CVR) procedure without postoperative helmeting. The surgical technique is described. Variables analyzed were: age at time of surgery, sex, estimated blood loss (EBL), operative time, intraoperative complications, postoperative complications, length of stay, pre- and postoperative cephalic index (CI), clinical impressions, and results of a 5-question nonstandardized questionnaire administered to patient caregivers regarding outcome. RESULTS: Eleven male and 7 female infants (mean age 2.3 months) were included in the study. The mean duration of follow-up was 16.4 months (range 6-38 months). The mean procedural time was 111 minutes (range 44-161 minutes). The mean length of stay was 2.3 days (range 2-3 days). The mean EBL in all 18 patients was 101.4 ml (range 30-475 ml). One patient had significant bone bleeding resulting in an EBL of 475 ml. Excluding this patient, the mean EBL was 79.4 ml (range 30-150 ml). There were no deaths or intraoperative complications; one patient had a superficial wound infection. The mean CI was 69 preoperatively versus 79 postoperatively, a statistically significant difference (p < 0.0001). Two patients were offered helmeting for suboptimal surgical outcome; one family declined and the single helmeted patient showed improvement at 2 months. No patient has undergone further surgery for correction of primary deformity, secondary deformities, or bony irregularities. Complete questionnaire data were available for 14 (78%) of the 18 patients; 86% of the respondents were pleased with the cosmetic outcome, 92% were happy to have avoided helmeting, 72% were doubtful that helmeting would have provided more significant correction, and 86% were doubtful that further surgery would be necessary. Small, palpable, aesthetically insignificant skull irregularities were reported by family members in 6 cases (43%). CONCLUSIONS: The authors present a nonendoscopic, minimally invasive CVR procedure without postoperative helmeting. Their small series demonstrates this to be a safe and efficacious procedure for isolated nonsyndromic sagittal synostosis, with improvements in CI at a mean follow-up of 16.1 months, commensurate with other techniques, and with overall high family satisfaction. Use of a CVR cranial orthosis in a delayed fashion can be effective for the infrequent patient in whom this approach results in suboptimal correction.

Comparative analysis of tranexamic acid use in minimally invasive versus open craniosynostosis procedures.

BACKGROUND: Intraoperatively administered tranexamic acid (TXA) lessens blood loss during orthopedic and cardiovascular surgery. Its use for craniosynostosis surgery warrants investigation. Therefore, we analyzed our use of TXA during minimally invasive (MI) and open craniosynostosis procedures. METHODS: Fifty-six patients were retrospectively studied: 20 in the MI group, 10 receiving TXA; 36 in the open group, 16 receiving TXA. Study variables were weight-adjusted estimated blood loss (EBL) and calculated blood loss (CBL), transfusion and incidence, transfusion volume, and complications. Calculated blood loss was determined by a novel formula based on red cell mass. RESULTS: In the MI group, median EBL was significantly lower for TXA recipients (9.62 vs 15.94 mL/kg, P = 0.0231), whereas median CBL was not (36.59 vs 34.12 mL/kg, P = 0.7976). Transfusion incidences were 80% TXA versus 100% control (P = 0.4737). Median transfusion volume trended lower (10.76 vs 19.43 mL/kg, P = 0.0723).In the open group, median EBL and CBL for TXA recipients were lower but not significantly different than for nonrecipients (21.86 vs 23.40 mL/kg, P = 0.7416; 53.54 vs 80.13; P = 0.3137). All patients had a transfusion. Median transfusion volume for TXA recipients versus nonrecipients was 34.01 versus 40.35 mL/kg (P = 0.3137). Tranexamic acid greatly minimized the range of EBL and CBL in both surgical groups. There was a significant correlation between the CBL and EBL (P < 0.0001). There were no adverse events. CONCLUSIONS: Intraoperative TXA administration is safe with modest benefit suggested, especially in the MI group. Calculated blood loss correlated well with EBL at lower blood loss volumes, implicating it as a potential measurement of true blood loss.

Comparison of spring-mediated cranioplasty to minimally invasive strip craniectomy and barrel staving for early treatment of sagittal craniosynostosis.

The treatment of sagittal craniosynostosis has evolved from early strip craniectomy to total cranial vault remodeling and now back to attempts at minimally invasive correction. To optimize outcomes while minimizing morbidity, we currently use 2 methods of reconstruction in patients younger than 9 months: spring-mediated cranioplasty (SMC) and minimally invasive strip craniectomy with parietal barrel staving (SCPB). The purpose of this study was to compare the safety and efficacy of the 2 methods. Hospital records of our first 7 SMCs and our last 7 SCPBs were analyzed for demographics, the type of operation performed, estimated blood loss, transfusion requirements, operative time, length of stay in the intensive care unit, length of hospital stay, preoperative cephalic index, postoperative cephalic index, and complications. The techniques were then compared using analysis of variance.All 14 patients successfully underwent cranial vault remodeling with significant improvement in cephalic index. Demographics, length of stay in the intensive care unit (P = 0.15), preoperative cephalic index (P = 0.86), and postoperative cephalic index (P = 0.64) were similar between SMC and SCPB. Spring-mediated cranioplasty had statistically significantly shorter operative time (P = 0.002), less estimated blood loss (P < 0.001), and shorter length of hospital stay (P = 0.009) as compared with SCPB. Complications included 1 spring dislodgment in an SMC that did not require additional management and 1 undercorrection in the SCPB group. Both SMC and SCPB are safe, effective means of treating sagittal craniosynostosis. Spring-mediated cranioplasty has become our predominant means of treatment of scaphocephaly in patients younger than 9 months because of its improved morbidity profile.

Cutaneous vascular anomalies associated with neural tube defects: nomenclature and pathology revisited.

BACKGROUND: Lumbosacral cutaneous vascular anomalies associated with neural tube defects are frequently described in the literature as "hemangiomas." The classification system for pediatric vascular anomalies developed by the International Society for the Study of Vascular Anomalies provides a framework to accurately diagnose these lesions. OBJECTIVE: To apply this classification to vascular cutaneous anomalies overlying myelodysplasias. METHODS: A retrospective analysis of patients with neural tube defects and lumbosacral cutaneous vascular lesions was performed. All eligible patients had detailed histopathologic analysis of skin and spinal cord/placode lesions. Clinical and radiologic features were analyzed. Conventional histology and GLUT-1 immunostaining were performed to differentiate infantile capillary hemangiomas from capillary vascular malformations. RESULTS: Ten cases with cutaneous lesions associated with neural tube defects were reviewed. Five lesions were diagnosed as infantile capillary hemangiomas based upon histology and positive GLUT-1 endothelial reactivity. These lesions had a strong association with dermal sinus tracts. No reoperations were required for residual intraspinal vascular lesions, and overlying cutaneous vascular anomalies involuted with time. The remaining 5 lesions were diagnosed as capillary malformations. These occurred with both open and closed neural tube defects, did not involute, and demonstrated enlargement and darkening due to vascular congestion. CONCLUSION: The International Society for the Study of Vascular Anomalies scheme should be used to describe the cutaneous vascular lesions associated with neural tube defects: infantile capillary hemangiomas and capillary malformations. We advocate that these lesions be described as "vascular anomalies" or "stains" pending accurate diagnosis by clinical, histological, and immunohistochemical evaluations.

Combined C-arm fluoroscopy and C-arm cone beam computed tomography for the evaluation of patients with possible intrathecal baclofen delivery system malfunctions.

BACKGROUND: Evaluating intrathecal baclofen (ITB) delivery systems for potential malfunction can be challenging. The catheter systems are prone to myriad complications that are frequently difficult to ascertain by conventional imaging techniques. Newer imaging technologies and their combinations can be used to identify such problems, define surgical indications, and focus operative planning. C-arm fluoroscopy and C-arm cone beam CT performed in one imaging session represents one such combination that has great utility. OBJECTIVE: We present a case series of ITB catheter evaluations using combined C-arm fluoroscopy (CF) and C-arm cone beam CT (CCBCT). METHODS: We retrospectively analyzed 7 pediatric patients who underwent ITB catheter systems evaluations by the use of combined CF and CCBCT. Study variables included indications for evaluation, imaging results, interventions, correlation of surgical findings with imaging, and clinical outcome. RESULTS: Three patients had intact and patent catheter systems. Four patients demonstrated various problems of the catheter systems, including disconnection, microfracture, fracture with segment migration, and subdural migration. Dosage adjustments improved all patients with normal studies. Surgery was guided by the imaging, and all operative patients improved after targeted interventions. Intraoperative findings correlated perfectly with imaging. CONCLUSION: Combined CF and CCBCT proved highly effective in the evaluation of our patients with potential ITB system malfunctions. This technique is advocated for such evaluations because it accurately defines problems with connectivity, integrity, and position of catheter systems. When surgical intervention is required, this information aids in operative planning.

Langerhans cell histiocytosis in a patient with stage 4 neuroblastoma receiving oral fenretinide.

Langerhans cell histiocytosis (LCH) has previously been reported in association with other malignancies. The pathogenesis of LCH and its relationship to other malignancies is poorly understood. We present a novel case of a child who developed an LCH bone lesion while receiving a Phase I protocol therapy with oral fenretinide/Lym-X-Sorb (4-HPR/LXS) powder for neuroblastoma.

Hypothalamic-opticochiasmatic gliomas mimicking craniopharyngiomas.

The differential diagnosis of suprasellar masses in the pediatric age group includes craniopharyngiomas and hypothalamic-opticochiasmatic gliomas (HOCGs). These tumors frequently display unique features on computed tomography and magnetic resonance imaging. We review two cases of pediatric suprasellar HOCGs with preoperative imaging characteristics resembling those of craniopharyngioma. HOCGs mimicking craniopharyngiomas represent a diagnostic and operative challenge to the pediatric neurosurgeon. Although an accurate leading pathologic diagnosis can frequently be made with preoperative neuroimaging, the neurosurgeon must be prepared for discovery of another tumor type and have plans to proceed accordingly.