Clinical factors associated with need for neurosurgical care in young children with imaging for macrocephaly: a case control study.

BACKGROUND: Macrocephaly is present in 2.3% of children with important neurosurgical conditions in the differential diagnosis. The objective of this study was to identify clinical associations with actionable imaging findings among children with head imaging for macrocephaly. METHODS: We conducted a case-control study of head imaging studies ordered for macrocephaly among children 24 months and younger in a multistate children's health system. Four neurosurgeons reviewed the images, determining cases to be a 'concern' if neurosurgical follow-up or intervention was indicated. Electronic health records were reviewed to collect patient-level data and to determine if surgery was performed. Controls were matched 3:1 to cases of 'concern' in a multivariate model using conditional logistic regression. RESULTS: In the study sample (n = 1293), 46 (4%) were concern cases, with 15 (1%) requiring surgery. Significant clinical factors associated with neurosurgical concern were bulging fontanel [aOR 7.47, (95% CI: 2.28-24.44), P < 0.001], prematurity [aOR 21.26, (95% CI: 3.76-120.21), P < 0.001], any delay [aOR 2.67, (95% CI: 1.13-6.27), P = 0.03], and head-weight Z-score difference (W_diff, defined as the difference between the Z-scores of head circumference and weight) [aOR 1.70, (95% CI: 1.22-2.37), P = 0.002]. CONCLUSIONS: Head imaging for macrocephaly identified few patients with findings of concern and fewer requiring surgery. A greater head-weight Z-score difference appears to represent a novel risk factor for neurosurgical follow-up or intervention.

Traumatic injury to the unfused anterior arch of C1 in the setting of bipartite atlas.

We present a case of a pediatric patient with congenital unfused anterior and posterior arches of the atlas (C1), also known as bipartite atlas, who sustained a traumatic injury during gymnastics. A computed tomography (CT) scan of the cervical spine raised concern for abnormal separation of the midline cleft of the anterior arch of C1. Subsequent magnetic resonance imaging (MRI) showed focal, edema-like signal in the midline cleft of C1. She was advised by neurosurgery to remain in a hard cervical collar for 6 weeks. She recovered after conservative treatment and returned to gymnastics. This case shows that a congenital unfused anterior arch of the atlas identified on CT after cervical trauma should not always be interpreted as an incidental finding. If a superimposed injury is suspected, MRI helps evaluate for traumatic injury, particularly if it is associated with upper cervical pain and tenderness or pain with neck movements. An additional case reiterates our findings.

Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update.

PURPOSE: The purpose of this study was to review a case of a septal dysembryoplastic neuroepithelial tumor (sDNT) and compare it to cases reported in the current literature. METHODS: We review a case of sDNT and compare with 7 other previously noted cases in the literature. RESULTS: The mainstay treatment is gross total resection, and most patients achieve full clinical resolution. Septal dysembryoplastic neuroepithelial tumor (sDNT) is a rare pediatric disease most commonly presenting as intractable epilepsy or headache. sDNT has been recognized as a genotypically distinct entity from DNT. A high frequency (~ 80%) of mutations of platelet-derived growth factor receptor A (PDGFRA) has been isolated in sDNT and could form the basis for future therapy. MRI is most commonly used to radiographically diagnose the disease and usually demonstrates a lobular interventricular mass involving the septum, potentially extending to the third ventricle. CONCLUSIONS: Our case and literature review validates endoscopic biopsy as a diagnostic and therapeutic intervention.

Frontal burr hole approach for neuroendoscopic resection of craniopharyngioma with the NICO Myriad device: report of two cases.

OBJECTIVE: Craniopharyngiomas are challenging tumors to resect due to their deep location and proximity to vital structures. The perceived benefit of gross total resection may be tempered by the possibility of permanent disability. Minimally invasive techniques may reduce surgical morbidity while still allowing effective resection. The authors describe their initial experience with a neuroendoscopic transcortical, transventricular approach to two craniopharyngiomas. The surgeries were performed through a right frontal burr hole using the NICO Myriad, a side-cutting, aspiration device that fits through the working channel of a standard neuroendoscope. METHODS: The imaging and medical records of two children (a 5-year-old male and a 9-year-old female) undergoing endoscopic resection of a craniopharyngioma with this technique were reviewed. Outcomes, results, and complications were noted. RESULTS: A gross total resection was achieved in both patients. The operative time was 180 and 143 min, respectively. The estimated blood loss was 20 and 50 cm3, respectively. Both patients required a cerebrospinal fluid shunt. There were no surgical complications. CONCLUSIONS: The NICO Myriad is an effective tool that allows a safe minimally invasive endoscopic resection of craniopharyngiomas in patients with amenable anatomy. Surgeons with experience in neuroendoscopy may be able to achieve a gross total resection of these challenging tumors through a minimally invasive burr hole approach.

Severe hydronephrosis caused by an infected intra-abdominal fluid collection in a patient with a ventriculoperitoneal shunt.

CASE: The authors report a case of a 10-year-old boy with myelodysplasia and shunted hydrocephalus who developed acute severe hydronephrosis presumed secondary to a large abdominal cerebrospinal fluid (CSF) collection. METHOD: The patient had recently begun having trouble catheterizing his Monti channel, and had been diagnosed and treated for an Escherichia Coli urinary tract infection. He presented with abdominal pain, and an abdominal CT demonstrated severe hydronephrosis and a large abdominal fluid collection presumed to be a CSFoma. The shunt was externalized, and fluid consistent with CSF was removed by aspiration of the shunt catheter at the time of surgery and by interventional radiology placement of a pigtail drain into the remaining fluid collection. Endoscopic evaluation of the Monti channel revealed evidence of healed perforation. A suprapubic catheter was placed and the Monti channel remained catheterized. The shunt was eventually converted to a ventriculoatrial shunt. A postoperative renal ultrasound later demonstrated marked improvement in the hydronephrosis and mild caliectasis. The suprapubic catheter and the indwelling Monti catheter were removed. The patient resumed catheterization of the Monti channel. RESULT: Follow-up ultrasounds have shown stable, mild hydronephrosis, consistent with the patient's baseline. CONCLUSIONS: Practitioners should have a high index of suspicion for potential shunt contamination and CSFoma development in patients with Monti channels and a history of difficult catheterization.

Cloacal exstrophy with extensive Chiari II malformation: case report and review of the literature.

INTRODUCTION: Cloacal exstrophy is a rare condition characterized by a defect in the abdominal wall with extrusion of malformed abdominal contents. The normal separation of the genitourinary and gastrointestinal systems does not occur. While patients with cloacal exstrophy have a high incidence of spinal defects, cranial defects are rare. Chiari malformation has been rarely reported in children with cloacal exstrophy, although the embryogenesis is unknown. OBJECTIVE: In this report, the authors describe a child with cloacal exstrophy and a large myelocystocele. The child also had an extensive Chiari II malformation. RESULTS: We review the relevant embryology and suggest a possible mechanism for Chiari formation in this patient.

Concurrent dermoid and epidermoid cysts in an adolescent patient: a case report.

Dermoid and epidermoid cysts are benign lesions of ectodermal origin which are pathologically distinct entities, although often clinically indistinguishable. Cyst location, mobility, and appearance on MRI can help distinguish the two, however the distinction is mostly academic since both types have similar management. Co-occurrence of dermoid and epidermoid cysts together in the same patient has not been observed in the literature, however one case of an epidermoid cyst evolving into a dermoid cyst has been documented. In this case report, we identify a 16-year-old male with three separate cysts of the scalp and leg which, after histopathological analysis following surgical resection, were found to represent both dermoid and epidermoid cysts. We offer potential explanations for this rare occurrence in the absence of a genetic syndrome and highlight the importance of performing a thorough work-up of patients with multiple cysts.

Onyx Embolization of a Distal Middle Cerebral Artery Pseudoaneurysm in a Five-Week-Old: A Case Report and Review of Current Treatment Options.

Intracranial pseudoaneurysms secondary to traumatic birth are a rare finding in infants. Definitive diagnosis of such findings is challenging, and no standard management is delineated for management of pseudoaneurysms in the pediatric population. Commonly attempted treatments include endovascular embolization or surgical clipping. A 5-week-old female presented with a two day history of right hand focal seizures. The patient was found to have a dysplastic superficial intra-axial aneurysm arising from the distal left middle cerebral artery (MCA) branch in the setting of a left posterior frontal lobe hemorrhage noted on brain magnetic resonance imaging/magnetic resonance angiography (MRI/MRA). The patient underwent diagnostic cerebral angiogram demonstrating a left distal MCA pseudoaneurysm, which was treated with Onyx embolization. Post-embolization period was complicated by recurrent left central localized seizures and a left hemispheric temporoparietal hemorrhagic infarction. The patient was managed on levetiracetam, phenytoin, phenobarbital with stable seizure control. Herein, we highlight the youngest case to date of a 5-week-old infant with a left distal MCA pseudoaneurysm treated with Onyx embolization. Pseudoaneurysmal incidence, diagnosis and accepted management is discussed.

Pediatric Otogenic Sigmoid Sinus Thrombosis: Case Report and Literature Reappraisal.

The purpose of this study was to review our experience with a single case of mastoiditis associated with sigmoid sinus thrombosis and increased intracranial pressure, and to review the experience of others through prior publications. We reviewed a case of a 6-year-old boy with an acute otitis media and mastoiditis, with associated ipsilateral sigmoid sinus and contralateral distal transverse sinus thrombosis. Based on the literature and our own experience, we conclude that most children with dural sinus thrombosis from acute otitis media and mastoiditis, in the setting of increased intracranial pressure, attain a good neurological outcome with a conservative neurosurgical approach. The mainstay of treatment seems to be appropriate antibiotic coverage and anticoagulation. Surgeries such as external ventricular drain, serial lumbar punctures, intracranial pressure monitor, and endovascular thrombectomy are reserved for patients with neurological deterioration despite maximal medical treatment.

Risk of deep venous thrombosis in elective neurosurgical procedures: a prospective, Doppler ultrasound-based study in children 12 years of age or younger.

OBJECTIVE The risk of venous thromboembolism (VTE) from deep venous thrombosis (DVT) is significant in neurosurgical patients. VTE is considered a leading cause of preventable hospital deaths and preventing DVT is a closely monitored quality metric, often tied to accreditation, hospital ratings, and reimbursement. Adult protocols include prophylaxis with anticoagulant medications. Children's hospitals may adopt adult protocols, although the incidence of DVT and the risk or efficacy of treatment is not well defined. The incidence of DVT in children is likely less than in adults, although there is very little prospectively collected information. Most consider the risk of DVT to be extremely low in children 12 years of age or younger. However, this consideration is based on tradition and retrospective reviews of trauma databases. In this study, the authors prospectively evaluated pediatric patients undergoing a variety of elective neurosurgical procedures and performed Doppler ultrasound studies before and after surgery. METHODS A total of 100 patients were prospectively enrolled in this study. All of the patients were between the ages of 1 month and 12 years and were undergoing elective neurosurgical procedures. The 91 patients who completed the protocol received a bilateral lower-extremity Doppler ultrasound examination within 48 hours prior to surgery. Patients did not receive either medical or mechanical DVT prophylaxis during or after surgery. The ultrasound examination was repeated within 72 hours after surgery. An independent, board-certified radiologist evaluated all sonograms. We prospectively collected data, including potential risk factors, details of surgery, and details of the clinical course. All patients were followed clinically for at least 1 year. RESULTS There was no clinical or ultrasound evidence of DVT or VTE in any of the 91 patients. There was no clinical evidence of VTE in the 9 patients who did not complete the protocol. CONCLUSIONS In this prospective study, no DVTs were found in 91 patients evaluated by ultrasound and 9 patients followed clinically. While the study is underpowered to give a definitive incidence, the data suggest that the risk of DVT and VTE is very low in children undergoing elective neurosurgical procedures. Prophylactic protocols designed for adults may not apply to pediatric patients. Clinical trial registration no.: NCT02037607 (clinicaltrials.gov).

Rapidly enlarging low-grade fibromyxoid sarcoma with intracranial extension in a 5-year-old girl: case report.

Low-grade fibromyxoid sarcoma (LGFMS) is a rare mesenchymal tumor that is characterized by a benign histology but potentially aggressive clinical behavior, with a high rate of recurrence and metastasis. It primarily occurs in young adults in the extremities, inguinal area, neck, or chest wall. There are rare reports of intracranial LGFMS in adults. In this report, the authors present the case of a 5-year-old girl who presented with a rapidly enlarging frontal scalp mass. Pathological examination of the resected mass demonstrated LGFMS. To the authors' knowledge, this is the only reported case of intracranial LGFMS in a child.

An important role of CD80/CD86-CTLA-4 signaling during photocarcinogenesis in mice.

Although previous studies have shown that altered B7 costimulation plays a critical role in UV irradiation-induced regulation of immunity, the individual roles of the B7 receptors (CD28 and CTLA-4) or the B7 family members (CD80 and CD86) have not been explored. Thus, we investigated CTLA-4 signaling during photocarcinogenesis of chronically UV-B-exposed mice using an antagonistic anti-CTLA-4 Ab. Anti-CTLA-4-treated mice developed significantly fewer UV-induced tumors. Moreover, anti-CTLA-4 treatment induced long-lasting protective immunity because progressively growing UV tumors inoculated into anti-CTLA-4- and UV-treated mice that had not developed tumors were rejected. Next, we used mice deficient for CD80, CD86, or both in photocarcinogenesis studies to assess the relative contributions of these CTLA-4 ligands. Double-deficient mice showed significantly reduced UV-induced skin tumor development, whereas CD86(-/-) mice produced skin cancer earlier compared with CD80(-/-) and control mice. The growth of UV-induced tumors appears to be controlled by UV-induced suppressor T cells, because CD80(-/-)/CD86(-/-) mice had strongly reduced numbers of UV-induced CD4(+)CD25(+) suppressor T cells. In vitro, CTLA-4 blockade inhibited the suppressor activity of UV-induced CD4(+)CD25(+) T cells, suggesting that reduced photocarcinogenesis might be due to decreased numbers or function of suppressor T cells. Together, these data indicate that blocking CD80/86-CTLA-4 signaling induced immune protection against the development of UV-induced skin tumors. Furthermore, CD86-mediated costimulation appears to play a more critical role in the protection against photocarcinogenesis than CD80.