Object Optic pathway/hypothalamic gliomas (OPHGs) are generally benign tumors situated in an exquisitely sensitive brain region. The location and natural history of OPHGs has led to much debate about optimal treatment. This paper revisits the role of and optimal timing of debulking surgery in OPHG. Methods This paper presents a series of cases managed by the neuro-oncology team at Alder Hey Children's Hospital and a single surgeon. Data were collected retrospectively for periods prior to 2009 and prospectively thereafter. Tailored treatment strategies were used, including observation and combinations of surgery, chemotherapy, and radiotherapy. Tumor control rates and outcomes are reviewed. Results Forty-two patients were treated between 1998 and 2011. Their median age at diagnosis was 5 years 7 months. Nineteen patients were positive for neurofibromatosis Type 1 (NF1) and 23 patients were negative for NF1. The median duration of follow-up was 77 months (range 21.8–142.3 months). Presenting symptoms included visual impairment (in 50% of cases), headache (in 24%), and hypothalamic/pituitary dysfunction (in 29%). Twenty-two debulking procedures were performed in 21 patients. Four biopsies (3 open, 1 endoscopic) were also performed. The histological diagnosis was pilocytic astrocytoma in 21 patients and pilomyxoid astrocytoma in 2 patients. Ten patients (Group 1) had primary surgical debulking alone and were then observed. Four patients (Group 2) had surgical debulking, plus planned chemotherapy within 3 months. Seven patients (Group 3) required surgical debulking for progressive disease following a variety of treatments. Patient age had the greatest impact on subsequent tumor progression. In total, 13 patients received chemotherapy, 4 on initial presentation, 4 in combination with surgery, and 5 for further tumor progression. Five patients were treated with radiotherapy, 3 prior to referral to Alder Hey. Eleven patients required shunt insertion for hydrocephalus. Vision was stabilized for 74% of patients. The number of patients with hypothalamic/pituitary dysfunction increased from 12 at presentation to 16 by the end of treatment. The overall survival rate was 93%. Three patients died—1 from tumor progression, 1 from infective complications from tumor biopsy, and 1 from a spontaneous posterior fossa hemorrhage. NF1 was associated with improved outcome—fewer patients required active intervention and rates of visual impairment and/or or hypothalamic/pituitary dysfunction were lower. Conclusions Good long-term survival and functional outcomes can be achieved in children with OPHG. Tumor control was achieved through an individualized approach using surgery, chemotherapy, or radiotherapy in varied combinations. The authors aim to limit radiotherapy to cases involving older children in whom other therapies have failed, due to the well-described and often devastating late effects associated with midline cranial irradiation. Surgery has a clear role for diagnosis, tumor control, and relief of mass effect. In particular, primary surgical debulking of tumor (without adjuvant therapy) is safe and effective. Recent advances in intraoperative MRI may add value and need further assessment.

Next-generation sequencing technology is available to many clinical laboratories; however, it is not yet widely used in routine microbiology practice. To demonstrate the feasibility of using whole-genome sequencing in a routine clinical microbiology workflow, we sequenced the genome of every organism isolated in our laboratory for 1 day. Whole-genome sequencing of microbes is an important tool for infectious disease researchers (1, 2). It has been used to understand the virulence of organisms of uncertain provenance and investigate the molecular basis of host-pathogen interactions (3-6). Whole-genome sequencing has also been used to evaluate genetic relationships among strains recovered in potential outbreaks of Mycobacterium tuberculosis, Staphylococcus aureus, and other human pathogens (7-9). However, despite tremendous advances in benchtop next-generation sequencing instrumentation, laboratory automation, and bioinformatics tools, whole-genome sequencing of microbes has not yet been widely adopted by clinical microbiology laboratories to identify unknown organisms.To test the feasibility of integrating whole-genome sequencing into the routine workflow of a clinical laboratory and identify potential impediments to this approach, we sequenced the genome of every organism recovered from cultures in our microbiology laboratory on a single day. In total, 130 samples were collected from 116 patient cultures (Table 1). A sample was defined as a loopful of colonial material (approximately 5 colonies) taken from solid agar. Whenever possible, each organism was sampled individually if multiple organisms were present on a plate. If organisms could not be sampled individually, then the mixture was sampled in toto. The study set included 107 samples collected from the aerobic bacteriology bench (Table 1, samples ADL101 to ADL124, ADL201 to ADL229, and ADL301 to ADL354), 9 samples collected from the anaerobic bacteriology bench (samples ADL401 to ADL409), and 14 samples collected from the acid-fast bacilli and mycology bench (samples ADL501 to ADL514).Bacteria and yeast were collected from the agar plate using a sterile 1-l calibrated loop and transferred to a 2-ml tube containing 1 ml Tris-EDTA and 0.1-mm silica spheres (Lysing matrix B; MP Biomedicals, Santa Ana, CA). Fungal isolates were collected in tubes containing 2-mm zirconia beads and 1.6-mm aluminum oxide particles (Lysing matrix J). Cells were lysed using ballistic disintegration (FastPrep96 automated homogenizer; MP Biomedicals), and genomic DNA was extracted using standard methods (DNeasy 96 blood and tissue kit; Qiagen Inc., Valencia, CA). The quantity and quality of genomic DNA were confirmed (Qubit 2.0 fluorometer; Invitrogen, Life Technologies, Grand Island, NY), and dual-indexed sequencing libraries were prepared according to the manufacturer's instructions (NexteraXT DNA sample preparation kit; Illumina Inc., San Diego, CA). The first 96 libraries were sequenced using a 2-by 250-bp paired-end protocol (MiSeq personal sequencer; Illumina). Since yeast and...

AIS catheters can reduce the number of shunt infections seen in clinical practice in certain subgroups. This has had a significant impact on the neonatal hydrocephalic population. The high risk of shunt infection after a period of external ventricular drainage raises the issue of emergence of bacterial resistance.

Object Skull base tumors in children are rare but require complex approaches with potential morbidity to the developing craniofacial skeleton, in addition to tumor-related morbidity. Reports of long-term clinical and functional outcome following skull base approaches in children are scarce. The authors report long-term outcome in children with tumors undergoing multidisciplinary skull base surgery. Methods A retrospective analysis was undertaken of children undergoing surgery at a single institution between 1998 and 2008 for benign and malignant lesions of the anterior, middle, or posterior cranial base. Patients with craniopharyngioma, pituitary tumors, and optic glioma were excluded. Histology, surgical morbidity, length of hospital stay, progression-free survival, and adjuvant therapy were recorded. Functional and cognitive outcome was assessed prospectively using the Late Effects Severity Score (LESS). Results Twenty-three children ranging in age from 13 months to 15 years underwent skull base approaches for resection of tumors during the study period. The median follow-up duration was 60 months. Tumor types included meningioma, schwannoma, rhabdomyosarcoma, neuroblastoma, angiofibroma, and chordoma. Complete resection was achieved in 12 patients (52%). Thirteen patients (57%) had benign histology. The median hospital stay was 7 days. There were 3 deaths, 1 perioperative and 2 from tumor progression. Two patients had CSF leakage (9%) and 2 developed meningitis. Two children (9%) had residual neurological deficit at last follow-up evaluation. Thirteen (59%) of 22 surviving patients received adjuvant therapy. The majority of the patients remain in mainstream education and 19 of the 20 surviving children have an LESS of 3 or lower. Conclusions Children tolerate complex skull base procedures well, with minimal surgical-related morbidity as well as good long-term tumor control rates and functional outcomes from maximal safe resection combined with adjuvant treatment when required.

We report a fatal case of meningeal melanocytoma in the cerebello-pontine angle in a 10-year-old child. This case exemplifies the vascular nature of these lesions even with minimal vascular blush on angiography. An updated literature search is presented, the results of which highlight the need for close follow-up and adjuvant treatment following subtotal resection.