Spirituality and Quality of Life among Parents of Adolescents with Spina Bifida.

In this investigational cross-sectional study, we explored the relationships among caregiver burden, depressive symptoms, spirituality, and quality of life (QOL) in 58 parental caregivers of adolescents with spina bifida (SB). A hierarchical linear regression analysis provided evidence that depressive symptoms and caregiver burden were negatively related to QOL scores, but spirituality was positively related. We found a significant interaction between caregiver burden and spirituality, suggesting that spirituality moderates the relationship between caregiver burden and quality of life. The negative effect of caregiver burden on QOL decreased as spirituality increased. These results warrant further research into how caregiver burden and spirituality intersect and impact QOL in parental caregivers of adolescents with SB.

Craniocervical Dissociation in Pediatric Patients: Pearls and Pitfalls of Diagnosis and Management.

AIMS: The aims of this study were to document the injury pattern in pediatric traumatic craniocervical dissociation (CCD) and identify features of survivors. METHODS: Pediatric traumatic CCDs, diagnosed between January 2004 and July 2016, were reviewed. Survivors and nonsurvivors were compared. Categorical and continuous variables were analyzed with Fisher exact and t tests, respectively. RESULTS: Twenty-seven children were identified; 10 died (37%). The median age was 60 months (ranges, 6-109 months [survivors], 2-98 months [nonsurvivors]). For survivors, the median follow-up was 13.4 months (range, 1-109 months). The median time to mortality was 1.5 days (range, 1-7 days). The injury modality was motor vehicle collision in 18 (67%), pedestrian struck in 8 (30%), and 1 shaken infant (3%). For nonsurvivors, CCD was equally diagnosed by plain radiograph and head/cervical spine computed tomography scan. For survivors, CCD was diagnosed by computed tomography in 7 (41%), magnetic resonance imaging in 10 (59%), and none by radiograph. Seven diagnosed by magnetic resonance imaging (41%) had nondiagnostic initial imaging but persistent neck pain. Magnetic resonance imaging was obtained and was diagnostic of CCD in all 7 (P < 0.01). Survivors required significantly less cardiopulmonary resuscitation (P < 0.01), had lower Injury Severity Scores (P < 0.01), higher Glasgow Coma Scale scores (P < 0.01), and shorter transport times (P < 0.01). Significantly more involved in motor vehicle collisions survived (P = 0.04). Nine (53%) had no disability at follow-up evaluation. CONCLUSIONS: In pediatric CCD, high-velocity mechanism, cardiac arrest, high Injury Severity Score, and low Glasgow Coma Scale score are associated with mortality. If CCD is correctly managed in the absence of cardiac arrest or traumatic brain or spinal cord injury, children may survive intact.

The moderating role of spirituality on quality of life and depression among adolescents with spina bifida.

AIM: To investigate the relationships between spirituality, somatic symptom distress/severity, depressive symptoms and quality of life (QOL) for adolescents diagnosed with spina bifida (SB). DESIGN: Exploratory, cross-sectional design. METHODS: Fifty-eight adolescents with SB in southern California were recruited during routine visits to a multidisciplinary clinic at a healthcare university from January 2016-January 2017. Each adolescent completed a series of self-report measures, including the System of Belief Inventory, Somatic Symptom Scale, Patient Health Questionnaire and Pediatric Quality of Life Inventory. Path analysis was performed to examine regression coefficients for each direct and indirect effect. RESULTS: The mediation-moderation analysis showed that depressive symptoms fully mediated the relationship between symptom distress and QOL (B = 0.029 [0.014], CI [0.007, 0.061]) and higher levels of spirituality moderated the relationship between depressive symptoms and QOL (B = 0.052, p = .018). Spirituality was higher for adolescents with greater symptom severity; including shunt status Welch's F(1, 53.689) = 4.174, p = .046, level of lesion F(2,57) = 3.382, p = .041, and ambulation status F(3, 57) = 2.920, p = .042. CONCLUSION: Adolescents with SB who had greater levels of symptom distress experienced significantly higher levels of depressive symptoms and a lower QOL. Contrary to our expectations, adolescents with greater levels of spirituality had a lower QOL when depressive symptoms were mild/moderate, but no differences were noted when depressive symptoms were severe. IMPACT: This study examined the relationship between spirituality and quality of life (QOL) in adolescents with spina bifida, who were experiencing different levels of depressive symptoms and symptom distress/severity. Depressive symptoms appeared to have a more profound effect on QOL than spirituality. Accordingly, we recommend that healthcare professionals actively screen for depressive symptoms when assessing these adolescents and their physical symptoms/distress levels.

Rigid Internal Fixation for Traumatic Cranio-Cervical Dissociation in Infants and Young Children.

STUDY DESIGN: Retrospective review. OBJECTIVE: Evaluate radiographic and clinical outcomes for infants and children, who underwent rigid occipito-cervical fixation for traumatic craniocervical dissociation (tCCD). SUMMARY OF BACKGROUND DATA: Traumatic craniocervical dissociation is devastating. Children are at high risk but make up a large number of survivors. Non-rigid fixation has traditionally been favored over screw and rod constructs due to inherent challenges involved with instrumenting the pediatric craniocervical junction. Therefore, outcomes for rigid occipito-cervical instrumentation in infants and young children with tCCD remain uncertain. METHODS: Retrospective review of children who survived tCCD between 2006 and 2016 and underwent rigid occipito-cervical fixation. RESULTS: Fifteen children, from 8 months to 8 years old (mean, 3.8 yr), were either a passenger (n = 11) or a pedestrian (n = 4) in a motor vehicle accident. Seven patients had weakness: five with quadriplegia, one with hemiparesis, and one with bilateral upper extremity paresis. Ten patients had concurrent C1-2 instability. At last follow-up, four patients had improved motor function: one with bilateral upper extremity paresis and one with hemiparesis regained full strength, one with quadriplegia regained function on one side while another regained function in bilateral upper extremities. All underwent rigid posterior occipito-cervical fixation, with two patients requiring additional anterior and posterior fixation at non-contiguous levels. Fourteen patients were stable on flexion-extension x-rays at a mean follow-up of 31 months (9-1 yr or longer, 7-2 yr or longer). There were no cases of deformity, growth disturbance, or subaxial instability. CONCLUSION: Children who survive tCCD may regain function after stabilization. Rigid internal rod and screw fixation in infants and young children safely provided long-term stability. We advocate using C2 translaminar screws to exploit the favorable anatomy of pediatric lamina to minimize the risks of occipitocervical (OC) instrumentation. LEVEL OF EVIDENCE: 4.

Epilepsy Surgery for Skull-Base Temporal Lobe Encephaloceles: Should We Spare the Hippocampus from Resection?

The neurosurgical treatment of skull base temporal encephalocele for patients with epilepsy is variable. We describe two adult cases of temporal lobe epilepsy (TLE) with spheno-temporal encephalocele, currently seizure-free for more than two years after anterior temporal lobectomy (ATL) and lesionectomy sparing the hippocampus without long-term intracranial electroencephalogram (EEG) monitoring. Encephaloceles were detected by magnetic resonance imaging (MRI) and confirmed by maxillofacial head computed tomography (CT) scans. Seizures were captured by scalp video-EEG recording. One case underwent intraoperative electrocorticography (ECoG) with pathology demonstrating neuronal heterotopia. We propose that in some patients with skull base temporal encephaloceles, minimal surgical resection of herniated and adjacent temporal cortex (lesionectomy) is sufficient to render seizure freedom. In future cases, where an associated malformation of cortical development is suspected, newer techniques such as minimally invasive EEG monitoring with stereotactic-depth EEG electrodes should be considered to tailor the surrounding margins of the resected epileptogenic zone.

Cervical spine injuries in young children: pattern and outcomes in accidental versus inflicted trauma.

BACKGROUND: The aim of the study was to compare the cervical spine (c-spine) pattern of injury and outcomes in children below 3 y with a head injury from confirmed inflicted versus accidental trauma. METHODS: After Institutional Review Board approval, data were prospectively collected between July 2011 and January 2016. Inclusion criteria were age below 3 y, a loss of consciousness, and any one of the following initial head computed tomography (CT) findings (subdural hematoma, intraventricular, intraparenchymal, subarachnoid hemorrhage, or cerebral edema). A protocol of brain and neck magnetic resonance imaging and magnetic resonance angiography was instituted. Brain and neck imaging results, clinical variables, and outcomes were recorded. Data were compared by t-test for continuous and Fisher exact test for categorical variables. RESULTS: 73 children were identified, 52 (71%) with inflicted and 21 (29%) with accidental trauma. The median age was 11 mo; (range: 1-35 mo). Ten (14%) had c-spine injuries, 7/52 (13%) inflicted, and 3/21 (14%) accidental. The mechanism was shaking for all inflicted and motor vehicle accident or pedestrian struck for accidental c-spine injuries. The inflicted group were significantly younger (P = 0.03), had higher Injury Severity Scores (P = 0.02), subdural hematomas (P = 0.03), fractures (P = 0.03), retinal hemorrhages (P = 0.02), brain infarcts (P = 0.01), and required cardiopulmonary resuscitation (P = 0.01). Seven with inflicted trauma died from brain injury (9.5%), one had atlanto-occipital dissociation. Six mortalities (86%) had no c-spine injury. Six with inflicted c-spine injuries survived with neurologic impairment, whereas three with accidental survived without disability, including one atlanto-occipital dissociation. CONCLUSIONS: Compared to accidental trauma, young children with inflicted c-spine injuries have more multisystem trauma, long-term disability from brain injury, and an injury pattern consistent with shaking.

Cervical spine imaging for young children with inflicted trauma: Expanding the injury pattern.

AIM: The purpose of this study was to document the incidence and pattern of cervical spine (c-spine) injuries in children below 36months with inflicted trauma. METHODS: An IRB approved, prospective cohort study was performed between July 2011 and January 2016. Inclusion criteria were: age below 36months, loss of consciousness after inflicted trauma, and one initial head computed tomography finding: a subdural, intraventricular, intraparenchymal, subarachnoid hemorrhage, diffuse axonal injury, hypoxic injury, or cerebral edema. A protocol of brain and neck magnetic resonance imaging and angiography was obtained within 48h. Variables were compared by t-test and Fisher-exact test. RESULTS: There were 53 children (median age: five months; range: 1-35months), 38 males (71.7%), of which seven died (13.2%). C-spine injury was identified in 8 (15.1%): ligamentous injury (2), vertebral artery shear injury (1), atlantooccipital dissociation (AOD) (1), cord injury with cord epidural hematoma (2), and isolated cord epidural hematoma (2). Retinal hemorrhages (p=0.02), shaking (p=0.04), lower Glasgow coma score (GCS) (p=0.01), brain infarcts (p=0.01), and hypoxic/ischemic injury (p=0.01) were associated with c-spine injury. One with AOD died. Six had significant disability. CONCLUSION: For small children with inflicted trauma, the c-spine injury incidence is 15.1%. The injury pattern includes retinal hemorrhages, shaking, lower GCS, and brain injury. Evaluation of shaken infants should include c-spine imaging. LEVEL OF EVIDENCE: Level 2 A- This is a prospective cohort study with complete follow-up to hospital discharge or death. In all cases, inflicted trauma was confirmed. Owing to the nature of child abuse, the precise time of injury is not known. All children underwent a strict imaging protocol on arrival to hospital that was supervised on a prospective basis.

Deep brain stimulation for the treatment of childhood dystonic cerebral palsy.

OBJECT: Deep brain stimulation (DBS) for dystonic cerebral palsy (CP) has rarely been reported, and its efficacy, though modest when compared with that for primary dystonia, remains unclear, especially in the pediatric population. The authors present a small series of children with dystonic CP who underwent bilateral pallidal DBS, to evaluate the treatment's efficacy and safety in the pediatric dystonic CP population. METHODS: The authors conducted a retrospective review of patients (under the age of 18 years) with dystonic CP who had undergone DBS of the bilateral globus pallidus internus between 2010 and 2012. Two of the authors independently assessed outcomes using the Barry-Albright Dystonia Scale (BADS) and the Burke-Fahn-Marsden Dystonia Rating Scale-movement (BFMDRS-M). RESULTS: Five children were diagnosed with dystonic CP due to insults occurring before the age of 1 year. Mean age at surgery was 11 years (range 8-17 years), and the mean follow-up was 26.6 months (range 2-42 months). The mean target position was 20.6 mm lateral to the midcommissural point. The mean preoperative and postoperative BADS scores were 23.8 ± 4.9 (range 18.5-29.0) and 20.0 ± 5.5 (range 14.5-28.0), respectively, with a mean overall percent improvement of 16.0% (p = 0.14). The mean preoperative and postoperative BFMDRS-M scores were 73.3 ± 26.6 (range 38.5-102.0) and 52.4 ± 21.5 (range 34.0-80.0), respectively, with a mean overall percent improvement of 28.5% (p = 0.10). Those stimulated at least 23 months (4 patients) improved 18.3% (p = 0.14) on the BADS and 30.5% (p = 0.07) on the BFMDRS-M. The percentage improvement per body region yielded conflicting results between rating scales; however, BFMDRS-M scores for speech showed some of the greatest improvements. Two patients required hardware removal (1 complete system, 1 unilateral electrode) within 4 months after implantation because of infections that resolved with antibiotics. CONCLUSIONS: All postoperative dystonia rating scale scores improved with pallidal stimulation, and the greatest improvements occurred in those stimulated the longest. The results were modest but comparable to findings in other similar series. Deep brain stimulation remains a viable treatment option for childhood dystonic CP, although young children may have an increased risk of infection. Of particular note, improvements in the BFMDRS-M subscores for speech were comparable to those for other muscle groups, a finding not previously reported.

Indirect cerebral revascularization with a temporoparietal fascial flap in pediatric moyamoya patients: a novel technique and review of current surgical options.

Moyamoya syndrome is a progressive occlusive disease of the cerebral vessels. There are a variety of surgical treatments directed at revascularizing the ischemic brain in pediatric moyamoya disease. Many reports of varying success with both direct and indirect type of procedures can be found in medical literature. We present a novel technique, encephalo-TPF-synangiosis (ETS) with a pedicled bone flap, for indirect moyamoya revascularization in pediatric patients. A three-quarters osteoplastic temporal craniotomy was created. A pedicled temporoparietal fascial flap was passed intracranially through the temporalis muscle and placed into contact with the pial surface. The bone flap was the reaffixed to the skull. We performed 8 ETS in 6 patients. This is a well-vascularized, highly reliable method that offers broad-based surface area for revascularization. We also offer a composite overview of current surgical indirect revascularization techniques.

Three-percent saline administration during pediatric critical care transport.

OBJECTIVES: The purpose of this study was to describe the administration of 3% saline (3%S) during pediatric critical care transport. METHODS: A retrospective study was performed on pediatric patients who underwent critical transport to Loma Linda University Children's Hospital from January 1, 2003, to June 30, 2007, and were given 3%S. Patients' demographics, admission diagnosis, route and amount of 3%S administration, serum electrolytes, vital signs, radiographic data, and Glasgow Coma Scale scores were collected and analyzed. RESULTS: A total of 101 children who received 3%S infusions during pediatric critical care transport were identified. Mean patient age was 5.9 years, and mean patient weight was 27.6 kg. The main indications for infusing 3%S were suspected cerebral edema (41%), intracranial bleed with edema (51%), and symptomatic hyponatremia (6%). The amount of 3%S bolus ranged from 1.2 to 24 mL/kg, with a mean of 5.4 mL/kg. Serum electrolytes before and after 3%S infusion demonstrated significant increases in sodium, chloride, and bicarbonate levels (P < 0.05). A significant reduction was also seen in serum urea nitrogen levels and anion gap. Radiographic imaging performed before 3%S infusion demonstrated findings consistent with concerns of increased intracranial pressure such as intracranial bleed and cerebral edema. The route of initial 3%S infusions was mainly through peripheral intravenous lines (96%). No complications related to the 3%S delivery such as local reactions, renal abnormalities, or central pontine myelinolysis were observed. CONCLUSIONS: It seems 3%S may be administered safely during pediatric critical transport and administration routes can include peripheral lines. With the importance of initiating therapy early to improve patient outcomes, the use of 3%S may benefit transported children with brain injury and suspected intracranial hypertension.

Encephalocraniocutaneous lipomatosis: a review of its clinical pathology and neurosurgical indications.

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome whose hallmark lesions are benign lipomas of the brain and spinal cord. The authors present a case of a male infant with ECCL who had extensive brainstem and spinal cord lipomas. The management of this patient's hydrocephalus, cervicomedullary compression, tethered cord, and scoliosis over the course of his first 2 years of life is described. This case report and review of the literature is presented to provide a synopsis of the problems likely to be encountered by neurosurgeons who treat patients with this syndrome.

Endoscopic fenestration of a cerebellopontine angle arachnoid cyst resulting in complete recovery from sensorineural hearing loss and facial nerve palsy.

Cerebellopontine angle arachnoid cysts are usually asymptomatic, but are frequently found incidentally because of increased use of neuroimaging. Nevertheless, as these cysts enlarge, they may compress surrounding structures and cause neurological symptoms. Patients may present with vague, nonspecific symptoms such as headache, nausea, vomiting, and vertigo. Cranial nerve palsies, including sensorineural hearing loss and facial weakness, although rare, have also been reported in association with posterior fossa arachnoid cysts. Although surgery for these entities is controversial, arachnoid cysts can be treated surgically with open craniotomy for cyst removal, fenestration into adjacent arachnoid spaces, shunting of cyst contents, or endoscopic fenestration. Reversal of sensorineural hearing loss following open craniotomy treatment has been described in the literature in only 1 adult and 1 pediatric case. Improvement in facial weakness has also been reported after open craniotomy and arachnoid cyst fenestration. The authors report the first case of complete recovery from sensorineural hearing loss and facial weakness following endoscopic fenestration in a patient with a cerebellopontine angle arachnoid cyst.

Atlanto-occipital dislocation with retroclival hematoma in a pediatric patient presenting to the emergency department.

Atlanto-occipital dislocation is a rare, oftentimes fatal injury sustained from high-impact trauma. It is seen more often in children compared with adults. In the past decade, there are more pediatric survivors presenting to the emergency department for treatment. This case reviews the presentation, diagnosis, and treatment of a child who survived this injury.

Pineal anlage tumor in a 5-month-old boy.

Pineal tumors are rare neoplasms that are categorized into pineoblastomas, pineocytomas, and pineal parenchymal tumors of intermediate differentiation. Pineal anlage tumors are primary pineal tumors with neuroepithelial and ectomesenchymal differentiation and without endodermal differentiation. The authors review the literature and report the case of a 5-month-old boy with a pineal anlage tumor. This is only the sixth case of a pineal anlage tumor reported in the English-language literature adding to the understanding of this tumor's presentation, immunomorphological and molecular characteristics, embryological origin, radiological appearance, treatment outcome, and prognosis.

Improved neurological function in a paediatric patient following Onyx embolization of a cervical glomus arteriovenous malformation.

Spinal cord intramedullary arteriovenous malformations (AVMs) pose a therapeutic challenge. Because of their complex angioanatomy, surgical excision of these lesions is difficult at best. Over the past decade, endovascular therapy has been established as an alternative treatment modality. As an embolic agent, N-butyl cyanoacrylate (NBCA) posed several problems such as difficulty of use and unpredictable performance. Onyx (ev3, Irvine, California, USA), an alternative liquid embolic agent, possesses several advantageous properties, such as increased control of agent delivery, over previous embolic agents like NBCA. However, reports of Onyx use in treating spinal intramedullary AVMs are still rare, especially in paediatric patients. We report a paediatric patient with glomus-type spinal intramedullary AVM treated successfully with Onyx with intermediate-term outcome.

Patient with congenital optic nerve meningocele presenting with left orbital cyst.

INTRODUCTION: Meningocele of the optic nerve is a rare condition defined as dilatation of the optic nerve by spinal fluid not due to other pathology, classically presenting with headaches or progressive visual decline and usually follows a rapidly progressive course. DISCUSSION: Surgical decompression is the standard treatment with improvement or arrest of progression in most cases. We describe a case of a child with multiple congenital anomalies including a left optic nerve meningocele that progressively expanded and caused displacement of the orbit laterally resulting in severe cosmetic deformity and complete blindness in the left eye. We describe our surgical decompression as well as review the literature on optic nerve meningocele.

Pathophysiology of tethered cord syndrome and similar complex disorders.

Tethered cord syndrome (TCS) is a stretch-induced functional disorder of the spinal cord due to the fact that its caudal portion is anchored by an inelastic structure. The functional lesion of TCS is generally situated in the lumbosacral cord, and many authors have shown that the syndrome is reversible via surgery to untether the cord. To clarify the expressions relevant to TCS, such as "cord tethering" and "tethered cord," the authors have formulated three categories. These categories include cases that show the anatomical appearance of spinal cord stretching. Among them, Category 1 is isolated to represent the "true TCS." The authors focus their discussion of the pathophysiology of TCS on Category 1 to explain the impaired oxidative metabolism and electrophysiological derangements within the tethered spinal cord, which is the primary intrinsic cause of the dysfunction. Furthermore, they extend the discussion to the extrinsic (outside the spinal cord) factors and other complex conditions that mimic TCS.

Further characterization of traumatic subdural collections of infancy. Report of five cases.

Bilateral convexity and interhemispheric subdural hematomas are common neuroimaging patterns seen in infants who have sustained nonaccidental head injuries (NAHIs). These collections often appear aschronic or acute-on-chronic on computerized tomography (CT) studies. To determine the nature of these extraaxial fluid collections and their relationship to cerebrospinal fluid (CSF) dynamics, the authors studied five infants with suspected NAHI in whom symptomatic bilateral mixed- or low-density subdural collections were revealed on imaging studies; the patients underwent burr hole evacuation of the hematoma and external drainage. Once decompression was achieved, radiotracer was injected into the lumbar subarachnoid space, and the subdural drainage system was monitored for appearance of the isotope. In all five cases, the radiotracer moved rapidly from the lumbar subarachnoid space into the convexity subdural space and then into the external drainage system. This indicated the possibility that some of these mixed-density subdural collections were acute blood mixed with CSF rather than acute-on-chronic collections arising from rebleeding subdural membranes. The authors propose that, during infancy, tears in the loosely adherent arachnoid envelope at the main arachnoid granulation site along the superior sagittal sinus may result in a considerable amount of CSF mixing with acute blood in the subdural space, creating a hematohygroma.