C. Corbett Wilkinson

Biomarkers of Hypercoagulability and Inflammation in Childhood-Onset Arterial Ischemic Stroke

OBJECTIVE To test the hypothesis that acute elevations of biomarkers of hypercoagulability and inflammation are common in children with arterial ischemic stroke (AIS), particularly among etiologic subtypes that carry an increased risk of recurrent stroke. STUDY DESIGN In this prospective/retrospective institutional-based cohort study of acute childhood-onset AIS (n = 50) conducted between 2005 and 2009, D-dimer, factor VIII (FVIII) activity, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were serially evaluated at the time of clinical blood sampling. Patients were classified by stroke subtype as cardioembolic, moyamoya, non-moyamoya arteriopathy, or other. RESULTS Both D-dimer and CRP were frequently elevated in acute childhood-onset AIS and exhibited a decreasing trend with time. Acute D-dimer levels were significantly higher in cardioembolic AIS compared with noncardioembolic AIS (median, 2.04 microg/mL [range 0.54-4.54 microg/mL] vs 0.32 microg/mL [0.22-3.18 microg/mL]; P = .002). At an optimal threshold of > or = 0.50 microg/mL, the sensitivity and specificity of D-dimer for cardioembolic subtype were 78% and 79%, respectively. CONCLUSIONS Our findings identify D-dimer and CRP as candidate biomarkers for etiology and prognosis in childhood-onset AIS. Further studies should investigate the role of these and other biomarkers of hypercoagulability and inflammation in childhood-onset AIS.

Prevalence of cervical spine injury in infants with head trauma.

OBJECT The incidence, type, and severity of pediatric cervical spine injuries (CSIs) are related to age and mechanism of injury. In this study, the authors assessed the prevalence of CSIs in infants with head trauma treated in their institution. METHODS The authors reviewed the medical records of children younger than 1 year of age who presented to The Childrens Hospital with head injuries between January 1993 and December 2007. They excluded infants with head injuries resulting from motor vehicle accidents and known falls from heights greater than 10 ft. For each patient, collected data included age, cause of injury, diagnosis, discharge disposition, and outcome. Relevant imaging data were reviewed, and when appropriate, autopsy reports were also reviewed. RESULTS Nine hundred five infants with head trauma and without a major mechanism/cause were identified. Their mean age was 4.3 months. Of the 905 patients, only 2 cases of CSI were detected, giving a prevalence of 0.2%. The mechanism of injury in these 2 patients was nonaccidental trauma (NAT). CONCLUSIONS The study revealed a very low prevalence of CSIs in infants with head trauma (0.2%). Routine cervical spine imaging in these infants, therefore, appears to have low diagnostic yield. The mechanism of head injury was NAT in the 2 patients who sustained an associated CSI. This supports the need for more stringent cervical spine imaging criteria for the infant with suspected NAT.

Comparison of outcomes following decompressive craniectomy in children with accidental and nonaccidental blunt cranial trauma

OBJECT The goal of this study was to compare clinical outcomes following decompressive craniectomy performed for intracranial hypertension in children with nonaccidental, blunt cranial trauma with outcomes of decompressive craniectomy in children injured by other mechanisms. METHODS All children in a prospectively acquired database of trauma admissions who underwent decompressive craniectomy over a 9-year span, beginning January 1, 2000, are the basis for this study. Clinical records and neuroimaging studies were systematically reviewed. RESULTS Thirty-seven children met the inclusion criteria. Nonaccidental head trauma was the most common mechanism of injury (38%). The mortality rate in patients with abusive brain injury (35.7%) was significantly higher (p < 0.05) than in patients with other causes of traumatic brain injury (4.3%). Children with inflicted head injuries had a 12-fold increase in the odds of death and 3-fold increase in the odds of a poor outcome (Kings Outcome Scale for Closed Head Injury score of 1, 2, or 3). CONCLUSIONS Children with nonaccidental blunt cranial trauma have significantly higher mortality following decompressive craniectomy than do children with other mechanisms of injury. This understanding can be interpreted to mean either that the threshold for decompression should be lower in children with nonaccidental closed head injury or that decompression is unlikely to alter the path to a fatal outcome. If decompressive craniectomy is to be effective in reducing mortality in the setting of nonaccidental blunt cranial trauma, it should be done quite early.

Short-term mortality following surgical procedures for the diagnosis of pediatric brain tumors: outcome analysis in 5533 children from SEER, 2004–2011

OBJECT Thirty-day mortality is increasingly a reference metric regarding surgical outcomes. Recent data estimate a 30-day mortality rate of 1.4-2.7% after craniotomy for tumors in children. No detailed analysis of short-term mortality following a diagnostic neurosurgical procedure (e.g., resection or tissue biopsy) for tumor in the US pediatric population has been conducted. METHODS The Surveillance, Epidemiology and End Results (SEER) data sets identified patients ≤ 21 years who underwent a diagnostic neurosurgical procedure for primary intracranial tumor from 2004 to 2011. One- and two-month mortality was estimated. Standard statistical methods estimated associations between independent variables and mortality. RESULTS A total of 5533 patients met criteria for inclusion. Death occurred within the calendar month of surgery in 64 patients (1.16%) and by the conclusion of the calendar month following surgery in 95 patients (1.72%). Within the first calendar month, patients < 1 year of age (n = 318) had a risk of death of 5.66%, while those from 1 to 21 years (n = 5215) had a risk of 0.88% (p < 0.0001). By the end of the calendar month following surgery, patients < 1 year (n = 318) had a risk of death of 7.23%, while those from 1 to 21 years (n = 5215) had a risk of 1.38% (p < 0.0001). Children < 1 year at diagnosis were more likely to harbor a high-grade lesion than older children (OR 1.9, 95% CI 1.5-2.4). CONCLUSIONS In the SEER data sets, the risk of death within 30 days of a diagnostic neurosurgical procedure for a primary pediatric brain tumor is between 1.16% and 1.72%, consistent with contemporary data from European populations. The risk of mortality in infants is considerably higher, between 5.66% and 7.23%, and they harbor more aggressive lesions.

Ossifying lipoma of the cervical spine

The authors present the case of a 21-month-old girl with a posterior cervical subcutaneous/spinal lipoma that contained heterotopic bone. The patient demonstrated no neurological deficits and the lesion was resected without complication. The lesion was determined to be an ossifying lipoma. The literature on ossifying lipomas and osteolipomas is reviewed and the differences between the two are enumerated.

Use of magnetic resonance imaging to detect occult spinal dysraphism in infants

OBJECTIVE Cutaneous stigmata or congenital anomalies often prompt screening for occult spinal dysraphism (OSD) in asymptomatic infants. While a number of studies have examined the results of ultrasonography (US) screening, less is known about the findings when MRI is used as the primary imaging modality. The object of this study was to assess the results of MRI screening for OSD in infants. METHODS The authors undertook a retrospective review of all infants who had undergone MRI of the lumbar spine to screen for OSD over a 6-year period (September 2006-September 2012). All images had been obtained on modern MRI scanners using sequences optimized to detect OSD, which was defined as any fibrolipoma of the filum terminale (FFT), a conus medullaris ending at or below the L2-3 disc space, as well as more complex lesions such as lipomyelomeningocele (LMM). RESULTS Five hundred twenty-two patients with a mean age of 6.2 months at imaging were included in the study. Indications for imaging included isolated dimple in 235 patients (45%), asymmetrically deviated gluteal cleft in 43 (8%), symmetrically deviated (Y-shaped) gluteal cleft in 38 (7%), hemangioma in 28 (5%), other isolated cutaneous stigmata (subcutaneous lipoma, vestigial tail, hairy patch, and dysplastic skin) in 31 (6%), several of the above stigmata in 97 (18%), and congenital anomalies in 50 (10%). Twenty-three percent (122 patients) of the study population had OSD. Lesions in 19% of these 122 patients were complex OSD consisting of LMM, dermal sinus tract extending to the thecal sac, and lipomeningocele. The majority of OSD lesions (99 patients [81%]) were filar abnormalities, a group including FFT and low-lying conus. The rate of OSD ranged from 12% for patients with asymmetrically deviated gluteal crease to 55% for those with other isolated cutaneous stigmata. Isolated midline dimple was the most common indication for imaging. Among this group, 20% (46 of 235) had OSD. There was no difference in the rate of OSD based on dimple location. Those with OSD had a mean dimple position of 15 mm (SD 11.8) above the coccyx. Those without OSD had a mean dimple position of 12.2 mm (SD 19) above the coccyx (p = 0.25). CONCLUSIONS The prevalence of OSD identified with modern high-resolution MRI screening is significantly higher than that reported with US screening, particularly in patients with dimples. The majority of OSD lesions identified are FFT and low conus. The clinical significance of such lesions remains unclear.

Cerebrospinal fluid shunt operations without cranial bandaging. Clinical article.

OBJECT Cranial bandages are commonly applied over scalp incisions immediately after cerebrospinal fluid (CSF) shunt surgery, putatively to prevent complications, particularly infection. These bandages require resources, consume the time of healthcare workers, and incur non-negligible expenses. It is therefore both reasonable and important to examine the efficacy of cranial bandaging. METHODS The combined experience of 3 neurosurgeons over 6.75 years with using no cranial bandaging after operations for implantation or revision of CSF shunts is the basis of this report. These data were prospectively accrued and retrospectively analyzed. RESULTS The infection rate was 4.2% (95% CI 3.1-5.6%) for 1064 operations performed without postoperative cranial bandaging after either shunt insertion or revision surgery through clean or clean-contaminated wounds. The age distribution extended from premature infants through adults 77 years of age. CONCLUSIONS The results of this investigation support the position that bandaging scalp wounds after CSF shunt implantation or revision surgery adds no benefit beyond the easier, simpler, faster, and cheaper practice of using antibiotic ointment as a dressing without bandaging.

Moyamoya in a Patient with Smith-Magenis Syndrome

Occurrence of moyamoya syndrome in a patient with Smith-Magenis syndrome (SMS) has previously been reported once in a 10-year-old Asian female. We report a second case of moyamoya in a patient with SMS, in a now 25-year-old Asian female diagnosed with both conditions as a child. In addition to describing her medical and surgical history, we provide a detailed report of her omental transposition, in which the omental circulation was anastomosed to the superior thyroid artery and external jugular vein. To our knowledge, this is the first report of omental transposition for moyamoya in which omental vessels are anastomosed to vessels in the neck, as well as the second report of moyamoya in a patient with SMS.

Frontal and occipital horn ratio is associated with multifocal intraparenchymal hemorrhages in neonatal shunted hydrocephalus

OBJECTIVE Placement of a cerebrospinal fluid diversion device (i.e., shunt) is a routine pediatric neurosurgical procedure, often performed in the first weeks of life for treatment of congenital hydrocephalus. In the postoperative period, shunt placement may be complicated by subdural, catheter tract, parenchymal, and intraventricular hemorrhages. The authors observed a subset of infants and neonates who developed multifocal intraparenchymal hemorrhages (MIPH) following shunt placement and sought to determine any predisposing perioperative variables. METHODS A retrospective review of the electronic medical record at a tertiary-care childrens hospital was performed for the period 1998-2015. Inclusion criteria consisted of shunt placement, age < 30 days, and available pre- and postoperative brain imaging. The following data were collected and analyzed for each case: ventricular size ratios, laboratory values, clinical presentation, shunt and valve type, and operative timing and approach. RESULTS A total of 121 neonates met the inclusion criteria for the study, and 11 patients (9.1%) had MIPH following shunt placement. The preoperative frontal and occipital horn ratio (FOR) was significantly higher in the patients with MIPH than in those without (0.65 vs 0.57, p < 0.001). The change in FOR (∆FOR) after shunt placement was significantly greater in the MIPH group (0.14 vs 0.08, p = 0.04). Among neonates who developed MIPH, aqueductal stenosis was the most common etiology (45%). The type of shunt valve was associated with incidence of MIPH (p < 0.001). Preoperative clinical parameters, including head circumference, bulging fontanelle, and coagulopathy, were not significantly associated with development of MIPH. CONCLUSIONS MIPH represents an underrecognized complication of neonatal shunted hydrocephalus. Markers of severity of ventriculomegaly (FOR) and ventricular response to CSF diversion (∆FOR) were significantly associated with occurrence of MIPH. Choice of shunt and etiology of hydrocephalus were also significantly associated with MIPH. After adjusting for corrected age, etiology of hydrocephalus, and shunt setting, the authors found that ∆FOR after shunting was still associated with MIPH. A prospective study of MIPH prevention strategies and assessment of possible implications for patient outcomes is needed.

Proposed caudal appendage classification system; spinal cord tethering associated with sacrococcygeal eversion.

IntroductionThe most commonly used classification system for caudal appendages (aka human tails) dates from the 1980s and classifies appendages (tails) as either true tails or pseudotails. Advances in neuroimaging since the 1980s, however, as well as an ever-increasing number of reported cases, have made this system outdated. Sacrococcygeal eversion is a condition in which the distal sacral and coccygeal vertebrae are curved in a retroverted rather than anteverted direction. It can give rise to one type of caudal appendage. Sacrococcygeal eversion has never been associated with spinal cord tethering in any previously published reports.MethodsWe reviewed all cases of caudal appendage encountered by pediatric neurosurgeons at Children’s Hospital Colorado since 2000 in which the appendage would be classified as a true tail by the most commonly used system mentioned above. We also reviewed cases of sacrococcygeal eversion encountered since 2000 by the same group of pediatric neurosurgeons. We searched the hospital electronic medical record system for additional appendages using the terms “caudal appendage” and “persistent human tail.”ResultsWe found 9 “true” tails (as classified by the most commonly used system). All 9 were associated with tethering or possible tethering of the spinal cord and 6 were associated with a low-lying conus medullaris. There were 8 cases of sacrococcygeal eversion, including 2 associated with Apert or Pfeiffer syndrome and fibroblast growth factor receptor 2 (FGFR2) mutations; these have previously been reported. There was a single case of sacrococcygeal eversion associated with Goldenhar or Turner syndrome; the former was associated with a potentially tethering lesion. Four cases of sacrococcygeal eversion not associated with any known syndrome were also found; two of these were associated with tethering or potentially tethering lesions.ConclusionsMost so-called true tails are likely cutaneous markers for spinal dysraphism and spinal cord tethering and are not remnants of the embryonic human tail. Sacrococcygeal eversion can be associated with spinal cord tethering. Based on our cases, and on review of the literature, we devised a five-category classification system for caudal appendages: (1) soft-tissue caudal appendages, (2) bony caudal appendages, (3) bony caudal prominences, (4) true tails, and (5) “other” caudal appendages.