Treatment of Sagittal Craniosynostosis Using Osteoplastic Parietal Craniotomy Flap With Distraction Osteogenesis: A Variation on Technique.

ABSTRACT: Late presentation sagittal craniosynostosis presents a unique challenge due to the decreased ability of the skull to repair the bony defects created by standard of care techniques. Distraction osteogenesis is a viable strategy to correct this defect in late presenting cases. The authors describe a variation in technique in which the temporalis muscle origin is retained, creating an osteoplastic bone flap with retained vascularity through the tem-poralis muscle. This may improve postoperative bony healing of bony defects in this compromised population. The authors present two patients who presented to them late with sagittal synostosis who were treated with distraction osteogenesis in which vascular continuity to the parietal bones is preserved through the temporalis muscle.

Beta Hairpin Peptide Hydrogels as an Injectable Solid Vehicle for Neurotrophic Growth Factor Delivery.

There is intense interest in developing novel methods for the sustained delivery of low levels of clinical therapeutics. MAX8 is a peptide-based beta-hairpin hydrogel that has unique shear thinning properties that allow for immediate rehealing after the removal of shear forces, making MAX8 an excellent candidate for injectable drug delivery at a localized injury site. The current studies examined the feasibility of using MAX8 as a delivery system for nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF), two neurotrophic growth factors currently used in experimental treatments of spinal cord injuries. Experiments determined that encapsulation of NGF and BDNF within MAX8 did not negatively impact gel formation or rehealing and that shear thinning did not result in immediate growth factor release. ELISA, microscopy, rheology, and Western blotting experiments collectively demonstrate the functional capabilities of the therapeutic-loaded hydrogels to (i) maintain a protective environment against in vitro degradation of encapsulated therapeutics for at least 28 days; and (ii) allow for sustained release of NGF and BDGF capable of initiating neurite-like extensions of PC12 cells, most likely due to NGF/BDGF signaling pathways. Importantly, while the 21 day release profiles could be tuned by adjusting the MAX8 hydrogel concentration, the initial shear thinning of the hydrogel (e.g., during injection) does not induce significant premature loss of the encapsulated therapeutic, most likely due to effective trapping of growth factors within structurally robust domains that are maintained during the application of shear forces. Together, our data suggests that MAX8 allows for greater dosage control and sustained therapeutic growth factor delivery, potentially alleviating side effects and improving the efficacy of current therapies.

Fracture of the anterior arch of atlas after minor trauma of the immature spine postlaminectomy.

Torticollis is a common complaint in the pediatric emergency department. Here, we report what we believe to be the first example in a young child of a fracture of the anterior arch of the atlas associated with an acquired, postsurgical defect of the posterior arch. A brief review of pediatric cervical spine injuries and fractures is presented. Atlas laminectomy may predispose patients to isolated atlas fractures even with minor trauma. Those fractures, however, are stable and treated by hard cervical orthosis.

Spinal cord injury without radiographic abnormality and the Chiari malformation: controlled observations.

BACKGROUND: Case reports have suggested an association between the Chiari malformation type 1 (CM1) and spinal cord injury without radiographic abnormality (SCIWORA). No controlled observations have appeared. We report a case-control study. METHODS: The Kids' Inpatient Database (KID) is a product of the Healthcare Utilization Project of the Agency for Healthcare Research and Quality. For the years 1997, 2000, 2003, 2006, and 2009, the KID includes discharge data abstracted from almost 14 million pediatric admissions. We hypothesized that CM1 predisposes children to SCIWORA in the cervicomedullary region, and we reasoned that, if this hypothesis were true, then KID admissions coded for upper cervical SCIWORA would be associated with CM1 more frequently than admissions coded for lower cervical SCIWORA. RESULTS: After exclusion of admissions with a variety of confounding diagnostic codes, we identified 754 admissions coded for upper cervical SCIWORA and 424 coded for lower cervical SCIWORA. Concurrent coding for CM1 was more prevalent among admissions coded for upper cervical SCIWORA than for lower cervical SCIWORA, but the difference did not attain statistical significance (p = 0.093; Fisher's exact test). CONCLUSIONS: These controlled observations fail to support the hypothesis that CM1 is associated with SCIWORA in childhood. If there is a real association, in the context of recent estimates of the prevalence of CM1 in the pediatric population, the magnitude of the incremental risk must be very small.

Clinical diagnosis of ventriculoperitoneal shunt failure among children with hydrocephalus.

OBJECTIVE: To define the significance of various symptoms and signs in the diagnosis of ventriculoperitoneal shunt failure and infection. METHODS: The observations that form the basis of this study were made in the course of 2 multicenter, prospective, randomized, controlled clinical trials of technical aspects of ventriculoperitoneal shunt surgery-the Shunt Design Trial and the Endoscopic Shunt Insertion Trial. At registration, basic demographic and baseline clinical data were recorded. At scheduled follow-up visits 3 months and 1, 2, and 3 years after surgery and at unscheduled visits, the presence or absence of various symptoms or signs was recorded. At each visit, the neurosurgeon-investigator made a determination about whether the shunt had reached an end point: mechanical obstruction, infection, overdrainage, or loculation of the ventricular system. Observations at the last follow-up visit for each patient constituted the data for the current study. Sensitivities, specificities, and likelihood ratios were calculated for each symptom and sign as tests for shunt failure from any cause and for failure by infection. Decision trees were constructed to analyze the relationships of various symptoms and signs in the diagnosis of shunt failure and infection. RESULTS: Observations were available for analysis from 647 patient visits. A total of 248 shunts were judged to have failed (38%), and 55 were judged specifically to have failed by infection (8.5%). Bulging fontanel, fluid collection along the shunt, depressed level of consciousness, irritability, abdominal pain, nausea and vomiting, abnormal shunt pump test, accelerated head growth, and headache were strongly associated with shunt failure. Fever was strongly associated with shunt infection. Gross signs of wound infection were associated with shunt infection but were observed infrequently. Decision tree analysis confirmed the salience of bulging fontanel as a predictor of shunt failure. Fever and time since initial surgery were powerful predictors of shunt infection. Irritability emerged as an important observation in the identification of both shunt failure and shunt infection. Among children who underwent initial shunt insertion after 2 months of age, the absence of irritability, nausea/vomiting, and headache were powerful and generalizable predictors of the absence of shunt failure or infection. CONCLUSIONS: Analysis of symptoms and signs of ventriculoperitoneal shunt complications can inform clinical judgment in the assessment of children with hydrocephalus.

Monte Carlo simulation of cerebrospinal fluid shunt failure and definition of instability among shunt-treated patients with hydrocephalus.

OBJECT: The authors undertook the present study to estimate the number of operations that patients with hydrocephalus will require within 10 years of diagnosis based on published survival data for cerebrospinal fluid (CSF) shunts. METHODS: Survival data for CSF shunts from several previously published sources were formatted as life tables spanning a 10-year period in monthly intervals. The monthly sequence of fractions of shunts failing was taken as the basis for a Monte Carlo simulation. Month by month for each virtual patient the computer simulation called up a random number between 0 and 1. If the random number was greater than the fraction of shunts failing in that monthly interval, the shunt survived. If the random number was less than or equal to the fraction of shunts failing in that interval, the shunt failed. When a virtual patient's shunt failed, that patient was returned to the first interval in the life table and began again. For every virtual patient this process continued for 120 months, and the number of shunt operations during the 10-year epoch was counted. Probability distributions were estimated for numbers of shunt operations. Expected numbers of shunt operations were calculated, as were the 95th and 99th percentiles. RESULTS: Four sources of CSF shunt survival data were used. Expected numbers of shunt operations ranged between 2.43 and 3.93 over 10 years. Estimated 95th percentiles ranged between five and 11, and estimated 99th percentiles ranged between eight and 15. CONCLUSIONS: New patients with hydrocephalus can expect to undergo between two and four operations for insertion or revision of CSF shunts in the first 10 years after diagnosis. Patients who undergo more than 15 shunt operations in 10 years are statistical outliers. A focused study of such patients may yield useful suggestions for complication avoidance and improvement in the quality of life of children with hydrocephalus.

Application of computer-assisted design in craniofacial reconstructive surgery using a commercial image guidance system. Technical note.

The technology of digital image guidance systems has transformed many aspects of neurosurgery, including intracranial tumor surgery, functional neurosurgery, and spinal surgery. Despite the central role of imaging studies in diagnosis and treatment planning, intraoperative image guidance has so far had very limited application to the surgical correction of craniofacial deformities, particularly those associated with craniosynostosis. The authors report an example of the marriage of computer-assisted design methods to a commercially available neurosurgical image-guidance system in the treatment of a case of anterior plagiocephaly due to unilateral coronal synostosis. They discuss the steps that must yet be taken to make this technology applicable to the management of craniosynostosis in infants.

Detected and overlooked cervical spine injury in comatose victims of trauma: report from the Pennsylvania Trauma Outcomes Study.

OBJECT: This study was undertaken to determine whether a clinically useful rule could be formulated for identifying the presence of traumatic brain injury (TBI) in patients who are at exceptionally low risk of cervical spine injury. METHODS: The Pennsylvania Trauma Outcomes Study database was searched for cases of TBI in which the admission Glasgow Coma Scale (GCS) score was less than or equal to 8. Cases of cervical injury were identified based on diagnostic codes. Associations between cervical injury and various clinical variables were tested using chi-square analysis. The probability of cervical injury was modeled using logistic regression. Decision tree models were constructed. Statistical determinants of overlooked cervical injury were examined. The prevalence of cervical injury among 41,142 cases of TBI was 8%. Mechanism of injury, thoracolumbosacral (TLS) fracture, age, limb fracture, admission GCS score, hypotension, and facial fracture were associated with cervical injury and were incorporated into the following logistic regression model: probability = 1/(1 + exp[4.248 - 0.417 X mechanism -0.264 X age - 0.678 X TLS - 0.299 X limb - 0.218 X GCS - 0.231 X hypotension - 0.157 X facial]). The results of applying this model provided a rule for cervical spine clearance applicable to 28% of the cases with a negative predictive value (NPV) of 97.0%. Decision tree analysis yielded a rule applicable to 24% of the cases with an NPV of 98.2%. The prevalence of overlooked cervical injury in all individuals with severe TBI was 0.3%; the prevalence of overlooked cervical injury in patients with cervical injury was 3.9%. Overlooked cervical injury was less common in patients with associated TLS fractures (odds ratio 0.453, 95% confidence interval 0.245-0.837). CONCLUSIONS: This analysis identified no acceptable rule to justify relaxing vigilance in the search for cervical injury in patients with severe TBI. Provider vigilance and consequent rates of overlooked cervical injury can be affected by environmental cues and presumably by other behavioral and organizational factors.

Intrathecal baclofen overdose and withdrawal.

Intrathecal baclofen (ITB) therapy is being used increasingly to treat medically intractable spasticity in children with cerebral palsy and spinal cord injuries. Baclofen overdose and withdrawal are potentially life-threatening complications of pump and spinal catheter system malfunction. We report a case of a 12-year-old boy, on long-term ITB therapy, who presents to our emergency department with an overdose of ITB, which is followed by withdrawal symptoms. The patient initially presented obtunded and in respiratory arrest. His symptoms of respiratory arrest, obtundation, fixed pupils, and hypotension mimicked other diagnoses, such as head trauma. The history obtained from the family about the pump reservoir being refilled just before the onset of symptoms led to the diagnosis. During hospitalization, as the patient recovered from the overdose, he began to experience symptoms of baclofen withdrawal, including hypertension, hyperthermia, and hallucinations. The pump was found to be disconnected and was revised. The patient was discharged home without permanent sequelae. With increased use of ITB, emergency medicine physicians must be aware of the mechanics of these pumps and the management of baclofen toxicity and withdrawal.

Adults with myelomeningocele and other forms of spinal dysraphism: hospital care in the United States since the turn of the millennium.

OBJECTIVE The natural history and management of myelomeningocele (MM) in children is fairly well understood. There is a deficiency of knowledge regarding the care of adults, however, even though there are now more adults than children living with MM. The purpose of this study was to characterize the hospital care of adults with MM and hydrocephalus on a nationwide population base. Adults with other forms of spina bifida (SB) were studied for contrast. METHODS The Nationwide Inpatient Sample for the years 2001, 2004, 2007, and 2010 was queried for admissions with diagnostic ICD-9-CM codes for MM with hydrocephalus and for other forms of SB. RESULTS There were 4657 admissions of patients with MM and 12,369 admissions of patients with SB in the sample. Nationwide rates of admission increased steadily for both MM and SB patients throughout the study period. Hospital charges increased faster than the health care component of the Consumer Price Index. Patients with MM were younger than patients with SB, but annual admissions of MM patients older than 40 years increased significantly during the study period. With respect to hospital death and discharge home, outcomes of surgery for hydrocephalus were superior at high-volume hospitals. Patients with MM and SB were admitted to the hospital more frequently than the general population for surgery to treat degenerative spine disease. CONCLUSIONS Patients with MM and SB continue to require neurosurgical attention in adulthood, and the demand for services for older patients with MM is increasing. Management of hydrocephalus at high-volume centers is advantageous for this population. Patients with MM or SB may experience high rates of degenerative spine disease.

Progressive syringomyelia controlled by treatment of associated hydrocephalus in an infant with birth injury. Case report.

The author's aim in reporting this case is to extend awareness of the importance of management of associated hydrocephalus among patients with syringomyelia to the posttraumatic state. A full-term infant was delivered by cesarean section because of transverse lie. In the 2nd week of life, hypotonia affecting the lower limbs and the left upper limb was recognized. On MR imaging, posterior fossa subdural hemorrhage and spinal cord injury at the T-1 and T-2 levels were evident. Sequential imaging studies demonstrated progressive myelomalacic changes in the cervical spinal cord and eventual development of syrinx cavities as far cephalad as C-3. Hydrocephalus developed simultaneously as well. A ventriculoperitoneal cerebrospinal fluid shunt was inserted at 22 months of age. Six months after shunt insertion, MR imaging demonstrated regression of the posttraumatic syringomyelia. The patient was stable from an imaging standpoint at 16 months after shunt insertion, and she has continued to make developmental progress. The importance of treatment of associated hydrocephalus is widely recognized in the management of syringomyelia associated with the Chiari malformations. It should be considered in the management of posttraumatic syringomyelia as well.

Intrathecal baclofen overdose followed by withdrawal: clinical and EEG features.

Intrathecal baclofen therapy is increasingly used to alleviate medically intractable spasticity in children with cerebral palsy, spinal cord injuries, and generalized dystonia. Complications like overdose or withdrawal can occur and could be the result of pump malfunction (device-related) or refilling and programming mistakes (human errors). This report describes a case, with emphasis on electroencephalographic changes, of a 12-year old male on long-term intrathecal baclofen therapy who had sequential occurrence of both acute inadvertent baclofen overdose followed by withdrawal symptoms. During baclofen intoxication, electroencephalography documented periodic generalized epileptiform discharges, occasionally followed by intermittent electro-decremental responses on a background of diffuse delta slowing (1-2 Hz). During withdrawal, mild generalized slowing during wakefulness was observed along with the appearance of high-amplitude, sharply contoured delta activity resembling frontal intermittent rhythmic delta activity in sleep. To our knowledge, this temporal profile of electroencephalographic features during baclofen intoxication followed by withdrawal has not been described before in pediatric patients. It is important for treating physicians to recognize the evolution of this electroencephalographic pattern in order to avoid misinterpretation of diagnosis and prognosis.

Birth injuries of the brachial plexus.

Birth injuries of the brachial plexus are fairly common, but the majority of affected newborns make quick recoveries without any specific intervention. A minority suffer more severe injuries that lead to varying degrees of life-long disability. Happily, modern microsurgical techniques permit reconstruction of certain plexus injuries and, in carefully selected patients, can restore voluntary activity to target muscle groups. To what degree reanimation of paralyzed muscles improves function and quality of life for these children is a more important matter that has not yet been addressed at the level of modern standards of evidence. Brachial plexus reconstruction is only a first step in the multidisciplinary process needed to optimize long-term functional outcomes for severely affected infants.

Detected and overlooked cervical spine injury among comatose trauma patients: from the Pennsylvania Trauma Outcomes Study.

OBJECT: A rule for identifying patients with traumatic brain injury (TBI) who are at exceptionally low risk of cervical spine injury might be clinically useful. The goal in this study was to research case records to determine whether such a rule would be practicable. METHODS: The Pennsylvania Trauma Outcomes Study database was used to find patients with TBI in whom Glasgow Coma Scale (GCS) scores at admission were 8 or less. Cases of cervical spine injury were identified from diagnostic codes. Associations between these injuries and a variety of clinical variables were tested using chi-square analysis. The probability of a cervical spine injury in these patients was modeled by logistic regression. Decision tree models were constructed and statistical determinants of overlooked cervical spine injury were examined. The prevalence of cervical spine injury among 41,142 cases of TBI was 8%. The mechanism of injury, presence of thoracolumbosacral (TLS) spinal, limb and/or facial fracture, patient age, GCS score at admission, and the presence of hypotension were all factors associated with cervical spine injury. These were incorporated into the following logistic regression model: probability of cervical spine injury = 1/(1 + exp[4.030 - 0.417*mechanism - 0.264*age - 0.678*TLS - 0.299*limb + 0.218*GCS score - 0.231*hypotension - 0.157*facial]). This model yielded a rule for clearance of 28% of cases, with a negative predictive value (NPV) of 97%. Decision tree analysis yielded an easily stated rule for clearance of 24% of cases, with an NPV of 98.2%. The prevalence of overlooked cervical spine injury among all patients with severe TBI was 0.3%; the prevalence of overlooked cervical spine injury among patients in whom it was later diagnosed was 3.9%. Overlooked cervical spine injury was less common among patients with associated TLS fractures (odds ratio 0.453, 95% confidence interval 0.245-0.837). CONCLUSIONS: No acceptable rule for relaxation of vigilance in the search for cervical spine injury among patients with severe TBI has been identified. Levels of provider vigilance and consequent rates of overlooked cervical spine injury can be affected by environmental cues and presumably by other behavioral and organizational factors.

Pediatric spinal injury in the US: epidemiology and disparities.

OBJECT: In the US, race and economic status have pervasive associations with mechanisms of injury, severity of injury, management, and outcomes of trauma. The goal of the current study was to examine these relationships on a large scale in the setting of pediatric spinal injury. METHODS: Admissions for spinal fracture without or with spinal cord injury (SCI), spinal dislocation, and SCI without radiographic abnormality were identified in the Kids' Inpatient Database (KID) and the National Trauma Data Bank (NTDB) registry for 2009. Patients ranged in age from birth up to 21 years. Data from the KID were used to estimate nationwide annual incidences. Data from the NTDB were used to describe patterns of injury in relation to age, race, and payor, with corroboration from the KID. Multiple logistic regression was used to model rates of mortality and spinal fusion. RESULTS: In 2009, the estimated incidence of hospital admission for spinal injury in the US was 170 per 1 million in the population under 21 years of age. The incidence of SCI was 24 per 1 million. Incidences varied regionally. Adolescents predominated. Patterns of injury varied by age, race, and payor. Black patients were more severely injured than patients of other races as measured by Injury Severity Scale scores. Among black patients with spinal injury in the NTDB, 23.9% suffered firearm injuries; only 1% of white patients suffered firearm injuries. The overall mortality rate in the NTDB was 3.9%. In a multivariate analysis that included a large panel of clinical and nonclinical factors, black race retained significance as a predictor of mortality (p = 0.006; adjusted OR 1.571 [1.141-2.163]). Rates of spinal fusion were associated with race and payor in the NTDB data and with payor in the KID: patients with better insurance underwent spinal fusion at higher rates. CONCLUSIONS: The epidemiology of pediatric spinal injury in the US cannot be understood apart from considerations of race and economic status.

Incidental findings on brain and spine imaging in children.

In recent years, the utilization of diagnostic imaging of the brain and spine in children has increased dramatically, leading to a corresponding increase in the detection of incidental findings of the central nervous system. Patients with unexpected findings on imaging are often referred for subspecialty evaluation. Even with rational use of diagnostic imaging and subspecialty consultation, the diagnostic process will always generate unexpected findings that must be explained and managed. Familiarity with the most common findings that are discovered incidentally on diagnostic imaging of the brain and spine will assist the pediatrician in providing counseling to families and in making recommendations in conjunction with a neurosurgeon, when needed, regarding additional treatments and prognosis.

Syringomyelia complicating myelomeningocele: review of the evidence.

Syringomyelia is frequently found in association with myelomeningocele. Although often asymptomatic, it can, in exceptional cases, cause severe morbidity. The author performed a structured literature review to address the following aspects of this clinical problem. What is the natural history? There are data concerning the imaging prevalence, the autopsy prevalence, and the prevalence of clinically active syringomyelia among patients with myelomeningocele, but literature provides no description of the course of this condition over time. What is the clinical significance? That correlations among symptoms, signs, treatments, and imaging findings are poorly described is no surprise in view of the large fraction of patients with syringomyelia who are asymptomatic. There is, however, an impressive mass of anecdotal evidence associating progressive syringomyelia with untreated (or inadequately treated) hydrocephalus. How to make the diagnosis? There is no disagreement that magnetic resonance imaging is the diagnostic modality of choice, but the literature provides very little guidance about who should undergo this investigation. There is no analysis of the costs and benefits of screening or periodic surveillance. What is the best treatment? Many surgical procedures have been reported to be useful as components of complex algorithms relating clinical factors and imaging data to treatment recommendations. There have been no controlled studies. There have been no prospective studies. There have been no multiinstitutional studies. No studies in which outcomes have been evaluated using objective, validated instruments. No studies in which the robustness of treatment effects over time have been documented. The complexity of treatment selection in contemporary practice makes syringomyelia unsuitable for randomized controlled trials of different therapies. Prospective cohort studies are feasible, however, and hold the potential to address many important questions about natural history and patient outcomes.

Ultrasonographic screening in infants with isolated spinal strawberry nevi.

OBJECT: The authors performed spinal ultrasonography and/or magnetic resonance (MR) imaging in 20 consecutive newborns with spinal strawberry nevi. METHODS: In 15 patients the strawberry nevi were isolated and in five they were associated with other cutaneous markers of occult spinal dysraphism (OSD). In four of five patients with additional cutaneous markers, but in none of those without, MR imaging and surgical exploration demonstrated OSD. The authors found that strawberry nevi in isolation do not appear to indicate underlying dysraphic states. The sparse clinical literature on this topic, which is reviewed, confirms an association between OSD and strawberry nevi presenting in conjunction with other cutaneous signatures. By contrast, spinal strawberry nevi occurring alone may not indicate the presence of underlying dysraphism. CONCLUSIONS: A prospective study of larger numbers of patients with isolated strawberry nevi, undergoing MR imaging evaluation, is necessary to determine whether neuroimaging screening in these patients is indicated.

Recognizing neurosurgical conditions in the pediatrician's office.

Clinical problems with potential neurosurgical ramifications, such as headache and abnormalities of head size and shape, arise often in general pediatric practice. Other neurosurgical issues may manifest themselves less frequently and more insidiously. In either case the pediatrician who is alert to spectrum of the presentations of neurosurgical conditions will direct investigations and referrals efficiently and inspire the confidence of the patient and the family.