Early Celecoxib Use in Spontaneous Intracerebral Hemorrhage is Associated with Reduced Mortality.

BACKGROUND: Hemorrhagic strokes constitute 10-15% of all strokes and have the worst mortality and morbidity of all subtypes. Mortality and morbidity of spontaneous intracerebral hemorrhage (sICH) are often secondary to the effects of inflammation, brain edema, and swelling. Studies have shown that celecoxib, a selective cyclooxygenase 2 (COX-2) inhibitor, reduces perihematomal edema formation and inflammation. This study aimed to examine the impact of celecoxib on sICH outcomes. METHODS: TriNetX, a multi-institutional research database, was retrospectively queried to identify patients with sICH. Outcomes in patients who received celecoxib within 5 days (cohort 1) were analyzed and compared to those in patients who did not receive celecoxib (cohort 2). The primary end point was mortality within 1 year of sICH. Secondary end points included ventilator dependence, tracheostomy, percutaneous endoscopic gastrostomy tube placement, craniotomy, deep venous thrombosis, pulmonary embolism, ischemic stroke, transient ischemia attack, myocardial infarction, and seizures. Further analysis was performed to assess these outcomes for patients treated with ibuprofen, a nonselective COX inhibitor. RESULTS: After propensity score matching, 833 patients were identified in each cohort based on celecoxib use. Mortality at 1 year was significantly reduced in patients with sICH receiving celecoxib compared to those who did not (13.33% vs. 17.77%; p = 0.0124). Risks of ventilator dependence, tracheostomy, percutaneous endoscopic gastrostomy tube placement, craniotomy, deep venous thrombosis, pulmonary embolism, ischemic stroke, transient ischemia attack, myocardial infarction, and seizures were not significantly increased in patients who received celecoxib within 5 days of sICH compared to those who did not receive celecoxib. There was no significant difference in mortality between patients based on ibuprofen administration. CONCLUSIONS: There exists a growing interest in using COX-2 as a potential target strategy for neuroprotection in patients with sICH, with some evidence of a mortality benefit in small cohort studies. This study shows that early celecoxib use is associated with decreased mortality in patients with sICH.

Awake craniotomies in the pediatric population: a systematic review.

OBJECTIVE: Awake craniotomy (AC) is employed to maximize tumor resection while preserving neurological function in eloquent brain tissue. This technique is used frequently in adults but remains poorly established in children. Its use has been limited due to concern for children's neuropsychological differences compared with adults and how these differences may interfere with the safety and feasibility of the procedure. Among studies that have reported pediatric ACs, complication rates and anesthetic management vary. This systematic review was performed to comprehensively analyze outcomes and synthesize anesthetic protocols of pediatric ACs. METHODS: The authors followed PRISMA guidelines to extract studies that reported AC in children with intracranial pathologies. The Medline/PubMed, Ovid, and Embase databases were searched from database inception to 2021, using the terms ("awake") AND ("Pediatric*" OR "child*") AND (("brain" AND "surgery") OR "craniotomy"). Data extracted included patient age, pathology, and anesthetic protocol. Primary outcomes assessed were premature conversion to general anesthesia, intraoperative seizures, completion of monitoring tasks, and postoperative complications. RESULTS: Thirty eligible studies published from 1997 to 2020 were included that described a total of 130 children ranging in age from 7 to 17 years who had undergone AC. Of all patients reported, 59% were male and 70% had left-sided lesions. Procedure indications included the following etiologies: tumors (77.6%), epilepsy (20%), and vascular disorders (2.4%). Four (4.1%) of 98 patients required conversion to general anesthesia due to complications or discomfort during AC. In addition, 8 (7.8%) of 103 patients experienced intraoperative seizures. Furthermore, 19 (20.6%) of 92 patients had difficulty completing monitoring tasks. Postoperative complications occurred in 19 (19.4%) of 98 patients and included aphasia (n = 4), hemiparesis (n = 2), sensory deficit (n = 3), motor deficit (n = 4), or others (n = 6). The most commonly reported anesthetic techniques were asleep-awake-asleep protocols using propofol, remifentanil or fentanyl, a local scalp nerve block, and with or without dexmedetomidine. CONCLUSIONS: The findings of this systematic review suggest the tolerability and safety of ACs in the pediatric population. Although pediatric intracranial pathologies pose etiologies that certainly may benefit from AC, there is a need for surgeons and anesthesiologists to perform individualized risk-benefit analyses due to the risks associated with awake procedures in children. Age-specific, standardized guidelines for preoperative planning, intraoperative mapping, monitoring tasks, and anesthesia protocols will help to continue minimizing complications, while improving tolerability, and streamlining workflow in the treatment of this patient population.

Intravascular fasciitis of the scalp as a complication of ICP monitor placement: a case report and review of the literature.

BACKGROUND/IMPORTANCE: There are only 56 documented cases of intravascular fasciitis, a rare variant of nodular fasciitis. Of these cases, only 2 involved the scalp. This lesion is amenable to surgical resection, making it important to differentiate it from soft tissue malignancies of the scalp. CLINICAL PRESENTATION: We report an unusual case of intravascular fasciitis involving the scalp at the site of an intracranial pressure (ICP) monitor of a 13-year-old male patient. The lesion was surgically excised with no recurrence upon 1-month follow-up. CONCLUSION: Intravascular fasciitis is a benign, reactive proliferation of soft tissue that may arise at sites of prior trauma. It appears as a soft, painless, mobile lesion, and immunohistochemical studies are required to differentiate it from malignant lesions. The standard of care is surgical resection of the lesion.

First-time tethered cord release among adults with myelomeningocele: an analysis of people in the National Spina Bifida Patient Registry.

OBJECTIVE: The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates. METHODS: Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression. RESULTS: Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16-0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20-0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50-9.88, p = 0.005). CONCLUSIONS: Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors' prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.

Mental health and substance use disorders in young adults with spina bifida.

OBJECTIVE: Although medical advances have allowed most patients with spina bifida (SB) to survive into adulthood, these patients may have physical impairments, urological complications, infections, and neurocognitive deficits. These factors can cause psychological distress and impact the transition from pediatric to adult care. There remains limited research on mental health disorders (MHDs) and substance use disorders (SUDs) in SB patients during this vulnerable transition period. This study aimed to investigate the 10-year incidence of MHDs and SUDs in 18- to 25-year-old patients with SB. METHODS: TriNetX, a federated de-identified database, was retrospectively queried to identify 18- to 25-year-old patients with SB. The presence of MHDs and SUDs based on ICD-10 codes in SB patients (cohort 1) was analyzed and compared with those of patients without SB (cohort 2). Subgroup analysis was performed on SB patients with hydrocephalus and neurogenic bladder (NB). SB patients were further compared to patients with a spinal cord injury (SCI). RESULTS: After propensity score matching, the authors identified 1494 patients in each cohort. SB patients were more likely to have depression (OR 1.949, 95% CI 1.64-2.317), anxiety (OR 1.603, 95% CI 1.359-1.891), somatoform disorders (OR 2.102, 95% CI 1.052-4.199), and suicidal ideations or attempts and self-harm (OR 1.424, 95% CI 1.014-1.999). The prevalence of attention-deficit/hyperactivity disorder (ADHD) and eating disorders were comparable between cohorts. SB patients also had increased rates of nicotine dependence (OR 1.546, 95% CI 1.22-1.959) but not of alcohol or opioid disorders. In SB patients, the presence of hydrocephalus and NB was not associated with significantly increased rates of any measured MHDs or SUDs. When compared with SCI patients, SB patients were more likely to have anxiety (OR 1.377, 95% CI 1.028-1.845) and ADHD (OR 1.875, 95% CI 1.084-3.242). However, SB patients had lower rates of nicotine dependence (OR 0.682, 95% CI 0.482-0.963) and opioid-related disorders (OR 0.434, 95% CI 0.223-0.845). SB and SCI patients shared similar rates of depression, suicidal ideations or attempts and self-harm, and alcohol-related disorders. CONCLUSIONS: Young adults with SB have higher prevalence rates of MHDs and SUDs compared with the general population. Therefore, incorporation of mental health and substance use management is critical to facilitate transition to adulthood.

Direct ventriculoatrial shunt in a pediatric patient: case report and technical note.

BACKGROUND /IMPORTANCE: The safety of direct cardiac shunts has been historically described in the pediatric population before the introduction of silastic catheters but are rarely utilized in modern practice. Herein, we describe several technical nuances regarding the placement of a direct ventriculoatrial catheter in a pediatric patient, including the creation of a sternal divot to accommodate for the movement of the catheter during growth. CLINICAL PRESENTATION: We report a complex case of a 2-year-old former premature infant with multiple systemic congenital abnormalities, including tracheal atresia (type 2), complete atrioventricular septal defect status post repair, and shunted hydrocephalus. She developed multiple shunt malfunctions secondary to abdominal malabsorption and shunt infections. CONCLUSION: Multiple options for distal shunt placement, including the atrium via open and endovascular techniques, the abdomen, gallbladder, and pleura, were considered, but the direct cardiac placement was felt to be the safest option given the patient's coexisting conditions. Placement requires a multidisciplinary team. Special consideration should be made for linear growth in children.

Pregnancy in spina bifida patients: a comparative analysis of peripartum procedures and complications.

PURPOSE: Spina bifida (SB) is caused by a failure in neural tube closure that can present with lower extremity sensory deficits, paralysis, and hydrocephalus. Medical advances have allowed increased pregnancies among SB patients, but management and pregnancy-associated complications have not been thoroughly investigated. The objective is to delineate peripartum procedures and complications in patients with SB. METHODS: A national de-identified database, TriNetX, was retrospectively queried to evaluate pregnant SB patients and the general population. Procedures and complications were investigated using corresponding ICD-10 and CPT codes within 1 year of pregnancy diagnosis. RESULTS: 11,405 SB patients were identified and compared to 9,269,084 non-SB patients. SB patients were significantly more likely to undergo cesarean delivery (1.200; 95% CI [1.133-1.271]) and less likely to receive neuraxial analgesia (0.406; 95% CI [0.383-0.431]). Additionally, patients with SB had an increased risk of seizures (3.922; 95% CI [3.529-4.360]) and venous thromboembolism (VTE) (3.490; 95% CI [3.070-3.969]). Risks of preeclampsia and hemorrhage were comparable. SB patients with hydrocephalus and Chiari malformation type 1 (CM-1) or type 2 (CM-2) were compared to patients without these comorbid conditions. This sub-group analysis showed a significantly increased risk of having cesarean deliveries (SB with hydrocephalus: 12.55%, S.B. with CM-1 or CM-2: 12.81% vs. SB without hydrocephalus or CM, 6.16%) and VTE (3.74%, 2.43% vs. 0.81%). There were also increased risks of hemorrhage and seizures and decreased use of neuraxial analgesia, but the sample size was insufficient. CONCLUSION: SB patients were more likely to undergo cesarean section and exhibit peripartum complications compared to those without SB.

Fatty filum terminale and low-lying conus medullaris in Gollop-Wolfgang complex: a case report and review of literature.

BACKGROUND/IMPORTANCE: Gollop-Wolfgang complex is a rare skeletal dysplasia with only 200 cases reported in the literature. This disorder is usually associated with several extraosseous anomalies. This report describes the first case of a fatty filum terminale and a low-lying conus medullaris in a patient with this complex. A review of the current literature of the Gollop-Wolfgang complex accompanies this case, highlighting the documented extraosseous anomalies seen in this complex. CLINICAL PRESENTATION: We report a case of an 18-month-old patient with Gollop-Wolfgang complex who underwent cord untethering with release of the filum terminale after extensive workup showed the presence of a dyssynergic bladder and radiological evaluation revealed a fatty filum terminale and low-lying conus medullaris. CONCLUSION: Gollop-Wolfgang complex is a skeletal dysplasia usually associated with several extra skeletal anomalies. Our report describes the first case of a fatty filum terminale and low-lying conus medullaris in this complex, as well as provides an overview of the documented anomalies seen in this disorder. A multidisciplinary approach is recommended when treating these infants in order to ensure that occult manifestations of the complex are not missed.

Development of Biodegradable Osteopromotive Citrate-Based Bone Putty.

The burden of bone fractures demands development of effective biomaterial solutions, while additional acute events such as noncompressible bleeding further motivate the search for multi-functional implants to avoid complications including osseous hemorrhage, infection, and nonunion. Bone wax has been widely used in orthopedic bleeding control due to its simplicity of use and conformation to irregular defects; however, its nondegradability results in impaired bone healing, risk of infection, and significant inflammatory responses. Herein, a class of intrinsically fluorescent, osteopromotive citrate-based polymer/hydroxyapatite (HA) composites (BPLP-Ser/HA) as a highly malleable press-fit putty is designed. BPLP-Ser/HA putty displays mechanics replicating early nonmineralized bone (initial moduli from ≈2-500 kPa), hydration induced mechanical strengthening in physiological conditions, tunable degradation rates (over 2 months), low swelling ratios (<10%), clotting and hemostatic sealing potential (resistant to blood pressure for >24 h) and significant adhesion to bone (≈350-550 kPa). Simultaneously, citrate's bioactive properties result in antimicrobial (≈100% and 55% inhibition of S. aureus and E. coli) and osteopromotive effects. Finally, BPLP-Ser/HA putty demonstrates in vivo regeneration in a critical-sized rat calvaria model equivalent to gold standard autograft. BPLP-Ser/HA putty represents a simple, off-the-shelf solution to the combined challenges of acute wound management and subsequent bone regeneration.

Bioinspired Super-Strong Aqueous Synthetic Tissue Adhesives.

Existing tissue adhesives and sealants are far from satisfactory when applied on wet and dynamic tissues. Herein, we report a strategy for designing biodegradable super-strong aqueous glue (B-Seal) for surgical uses inspired by an English ivy adhesion strategy and a cement particle packing theory. B-Seal is a fast-gelling, super-strong, and elastic adhesive sealant composed of injectable water-borne biodegradable polyurethane (WPU) nanodispersions with mismatched particle sizes and counterions in its A-B formulation. B-Seal showed 24-fold greater burst pressure than DuraSeal®, 138-fold greater T-pull adhesive strength than fibrin glue, and 16-fold greater lap shear strength than fibrin glue. In vivo evaluation on a rat cerebrospinal fluid (CSF) rhinorrhea model and a porcine craniotomy model validated the safety and efficacy of B-Seal for effective CSF leak prevention and dura repair. The plant-inspired adhesion strategy combined with particle packing theory represents a new direction of designing the next-generation wet tissue adhesives for surgeries.

Malignant spindle cell tumors of the posterior fossa in children: case series and review of management.

OBJECTIVE: The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population. METHODS: The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with "posterior fossa" or "spindle cell tumors" or "Ewing sarcoma" or "high-grade" or "spindle cell sarcoma" or "leptomeningeal melanocytoma" as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome. RESULTS: The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease. CONCLUSIONS: A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.

Aneurysmal and arteriovenous malformation hemorrhage during pregnancy: An update on the epidemiology, risk factors and prognosis.

INTRODUCTION: Intracranial hemorrhage (ICH) is a devastating condition with a high rate of morbidity and mortality. Aneurysm or arteriovenous malformation (AVM) rupture are two common etiologies leading to ICH. Here we provide an update on ICH during pregnancy with a focus on those caused by aneurysm or AVM rupture. METHODS: Here we systematically review 25 studies reported in the literature to provide an update on ICH during pregnancy focusing on aneurysm or AVM rupture. We also reviewed the prognosis of ICH during puerperium. RESULTS: Discrepancies exist between studies supporting or refuting the hypothesis of a higher rate of ICH during pregnancy, obscuring the overall rate of aneurysm and AVM rupture in pregnant ICH patients. However, risk factors such as maternal age and hypertension have shown to increase the frequency of ICH in pregnant patients. We also show increased morbidity and mortality in patients suffering from preeclampsia/eclampsia. DISCUSSION: ICH is rare, but the various studies demonstrating its increased frequency, morbidity, and mortality during pregnancy should raise our awareness of this condition. The management and treatment decisions for a pregnant ICH patient should follow the same principles as nonpregnant patients, but with the knowledge that not all medications are appropriate for use in the pregnant patient. Although there seems to be a higher frequency of AVM rupture, further research must be conducted in order to fully determine the effects of pregnancy on aneurysm and AVM ruptures.

Tethered spinal cord among individuals with myelomeningocele: an analysis of the National Spina Bifida Patient Registry.

OBJECTIVE: The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics. METHODS: The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Cox proportional hazards models were constructed to identify relationships between TCR rates and sex, functional lesion level, ambulation status, treated hydrocephalus, and prior Chiari decompression. RESULTS: Of 6339 patients with information about their operations, 1366 (21.5%) underwent TCR, with significant variability between centers. The majority (75.8%) underwent a single TCR. The annual TCR rate was linear between birth and 13 years (1.8%/year) but declined sharply from 14 to 21 years (0.7%/year). There was no period of time at which the TCR rate accelerated. There were no significant differences in TCR rates between males and females. TCR rate was not related to functional lesion level but was lower among nonambulators compared with community ambulators (p = 0.005) and among those with treated hydrocephalus (HR 0.30, p < 0.001), and higher among those having prior Chiari decompression (HR 1.71, p < 0.001). CONCLUSIONS: These results extend the results of prior single-institution studies, demonstrate significant treatment variability between institutions, and challenge the traditional concept that tethering is related to spinal cord stretching due to spinal growth.

Origin and Use of Hydrogen Peroxide in Neurosurgery.

Hydrogen peroxide (H2O2) is a chemical with a wide range of applications. This includes its use in the medical field, in which its use has been ubiquitous but is most useful as an antiseptic and in achieving hemostasis. Neurosurgeons have been using H2O2 for well over a century, primarily for its hemostatic and antiseptic effects. This is in spite of the fact that the actual effectiveness of H2O2 as an antiseptic is questionable, and its use, in general, may be more dangerous than it appears. We review the application of H2O2 in medicine generally and, more specifically, in neurosurgery. This review outlines the reasoning behind the use of H2O2 as an antiseptic and details why it may not be as effective as one might think. We also detail its use as a hemostatic agent in neurosurgery, reviewing a number of techniques in which it has been useful in this role. Finally, we review the documented cases of complications associated with the use of H2O2 in neurosurgery. Ultimately, we conclude that the use of H2O2 in neurosurgery be reconsidered because of its lack of effectiveness as an antiseptic and potentially fatal complications.

Phrenic Nerve Stimulator Placement via the Cervical Approach: Technique and Anatomic Considerations.

BACKGROUND: Diaphragmatic pacing via phrenic nerve stimulation can help improve breathing and facilitate mechanical ventilation weaning in patients with respiratory failure secondary to brainstem injury, high cervical spinal cord injury, or congenital central hypoventilation. Devices can be placed utilizing several techniques; however, nuances regarding placement are not well published. OBJECTIVE: To describe our experience with phrenic nerve stimulator placement via the cervical approach with a focus on surgical anatomy, variations, and technique. METHODS: Placement of phrenic nerve stimulator via a cervical approach is described in detail. RESULTS: Successful placement of phrenic nerve stimulator without complication. CONCLUSION: The cervical approach for the placement of a phrenic nerve stimulator is a safe and effective option for patients. Detailed knowledge of anatomy and anatomic variations is required. Potential advantages and disadvantages are discussed.

Congenital Spinal Cysts: An Update and Review of the Literature.

Congenital spinal cysts are rare and encompass a wide variety of diseases including arachnoid, enterogenous, teratomatous, neurenteric, foregut, bronchogenic, epithelial, ependymal, dermoid, and epidermoid cysts. Here, we elucidate the epidemiology, pathology, pathogenesis, and diagnostic findings of the most common congenital spinal cysts, followed by a discussion of their presentation and treatment options. Differentiating the cause of each lesion is crucial for targeted clinical and surgical management for the patient. Our review describes how arachnoid cysts can be observed, fenestrated, percutaneously drained, or shunted; however, the primary goal for neurenteric, dermoid, and epidermoid cysts is removal. Further, we discuss how patient presentation is dependent on the rate of growth and location of compression on the spinal cord and nerve roots. However, although many of these lesions are discovered incidentally on imaging, the spectrum of possible symptoms include pain, weakness, ataxia, bladder incontinence, and progressive or acute neurologic deficits. We present and review the histology and imaging of a variety of cysts and discuss how although the goal of treatment is resection, the risks of surgery must be considered against the benefits of complete resection in each case.

Identifying the Misshapen Head: Craniosynostosis and Related Disorders.

Pediatric care providers, pediatricians, pediatric subspecialty physicians, and other health care providers should be able to recognize children with abnormal head shapes that occur as a result of both synostotic and deformational processes. The purpose of this clinical report is to review the characteristic head shape changes, as well as secondary craniofacial characteristics, that occur in the setting of the various primary craniosynostoses and deformations. As an introduction, the physiology and genetics of skull growth as well as the pathophysiology underlying craniosynostosis are reviewed. This is followed by a description of each type of primary craniosynostosis (metopic, unicoronal, bicoronal, sagittal, lambdoid, and frontosphenoidal) and their resultant head shape changes, with an emphasis on differentiating conditions that require surgical correction from those (bathrocephaly, deformational plagiocephaly/brachycephaly, and neonatal intensive care unit-associated skill deformation, known as NICUcephaly) that do not. The report ends with a brief discussion of microcephaly as it relates to craniosynostosis as well as fontanelle closure. The intent is to improve pediatric care providers' recognition and timely referral for craniosynostosis and their differentiation of synostotic from deformational and other nonoperative head shape changes.

Long-term causes of death among pediatric patients with cancer.

BACKGROUND: The objectives of this study were to characterize the risk of death (1) from the primary cancer vs competing cause of death; and (2) from various causes of death vs the general poplation. The relative risk of death after a pediatric cancer diagnosis versus the general population and the risk of death from a primary cancer diagnosis versus competing causes of death. METHODS: This retrospective, population-based study used the Surveillance, Epidemiology, and End Results database (1980-2015) and included patients aged 0 to 19 years at the time of diagnosis. Observed deaths were calculated; the risk of death versus the general population was assessed with standardized mortality ratios (SMRs). Competing risk models for the cause of death were performed. RESULTS: There were 58,356 patients who were diagnosed, and the mortality rate was 22.8%. To assess causes of death, 6996 patients who died during the study period were included (45,580 total person-years at risk): 5128 (73%) died of their primary cancer, and 1868 (27%) died of a competing cause. Among all patients, the rate of death from the index cancer was higher than the rate of death from another cause within the first 5 years after diagnosis. The risk of death from a nonprimary cancer began to supersede the rate of death from the primary cancer 10 years after diagnosis for patients with germ cell tumors, lymphomas, and sarcomas. SMRs for the primary cancer were highest within the first 5 years after diagnosis for all cancers (SMRs, 100-50,000; P < .0001). The risk of death from competing causes (heart disease, suicide, and sepsis) was elevated (SMR, >100; P < .001). The risk of dying of heart disease was high, especially for patients with astrocytomas (SMR, 47.84; 95% confidence interval [CI], 27.87-76.59) and neuroblastomas (SMR, 98.59; 95% CI, 47.28-181.32). The risk of dying of suicide was high in most patients, particularly for those with osteosarcomas (SMR, 111.40; 95% CI, 2.82-620.69), Hodgkin lymphomas (SMR, 62.35; 95% CI, 34.89-102.83), and gonadal germ cell tumors (SMR, 28.97; 95% CI, 12.51-57.09). CONCLUSIONS: The cause of death for patients with gonadal germ cell tumors, lymphomas, and sarcomas is more commonly a secondary cancer or noncancerous cause than the primary disease; their risk of death from competing causes (heart disease, suicide, and sepsis) rises throughout life.

Suprasellar central nervous system ganglioneuroblastoma: a case in a 9-year-old child and review of the literature.

PURPOSE: Intracranial ganglioneuroblastomas are incredibly rare neuroectodermal tumors with only 8 described cases total, 5 of those having imaging findings METHODS: Here we present a 9-year-old female patient with 4 months progressive headaches, personality changes, and vomiting. We also present a review of the current literature of intracranial ganglioneuroblastomas. RESULTS: Imaging demonstrated a partially calcified suprasellar mass measuring 4.6 × 6.3 × 5 cm composed of both solid and cystic components, diagnosed to be a ganglioneuroblastoma, with mass effect on the lateral and 3rd ventricles, with a midline shift of right to left of 6-7 mm. She was treated with subtotal surgical resection, an intensive chemotherapeutic regimen, and radiation and has no residual disease on imaging 1 year and 4 months status post-surgery. CONCLUSION: To our knowledge, this is the first case of a ganglioneuroblastoma to mimic a craniopharyngioma based upon imaging findings and suprasellar location. As these cases are extremely rare, an optimal therapeutic regimen has not been defined. However, a combination of surgical resection, chemotherapy, and radiation therapy can be effective, as shown here with successful treatment and no evidence of residual disease.