Clinical Outcomes in Children With Orbital Cellulitis and Radiographic Globe Tenting.

PURPOSE: Axial displacement of the globe with tenting centered on the optic nerve-globe junction is a predictor of visual loss in adults. The purpose of this study was to determine the visual outcomes of children with orbital cellulitis and globe tenting. METHODS: The records of 46 consecutive children with orbital cellulitis at a single tertiary children's hospital were reviewed retrospectively. Initial and final visual acuities were available for 34 of 46 patients (74%). Globe tenting was defined by an angle of 130° or less at the optic nerve-globe junction as derived from sagittal CT or MRI. Visual acuities of 4 children with globe tenting (mean age, 10.3 ± 3.3 years) were compared with those of 30 children without globe tenting (mean age, 10.8 ± 3.5 years). Final logarithm of the minimum angle of resolution visual acuities were analyzed. RESULTS: The mean posterior globe angle was 124.5° ± 8.0° in patients with globe tenting, compared with 145.6° ± 7.4° in the affected eye of the patients without globe tenting (p = 0.002). Final visual acuity was logarithm of the minimum angle of resolution = 0 following treatment in patients with globe tenting and logarithm of the minimum angle of resolution = 0.02 in patients without tenting (p = 0.70). DISCUSSION: We propose that the increased elastic compliance of the optic nerve sheath and sclera in children may contribute to better visual outcomes. CONCLUSIONS: Pediatric orbital cellulitis with globe tenting may not lead to devastating vision loss as previously seen in adults.

Malignant Peripheral Nerve Sheath Tumor of the Lower Eyelid: Case Presentation and Literature Review.

Solitary benign neurogenic tumors are common in the orbit, but only rarely arise from peripheral nerves in the eyelids. In contrast, malignant tumors of neural or nerve sheath elements are exceedingly rare in the orbit and, to date, have never been reported in the lower eyelid. The authors report a 55-year-old man with multiple recurrent lower eyelid masses initially treated as chalazia then misdiagnosed as neurotropic malignant melanoma on pathology. Diagnosis of malignant peripheral nerve sheath tumor was ultimately confirmed histopathologically after surgical resection. The patient has since undergone multiple resections and adjuvant radiotherapy. Twenty-two months since the last procedure, the patient remains disease-free.

Smith-Lemli-Opitz syndrome and inborn errors of cholesterol synthesis: summary of the 2007 SLO/RSH Foundation scientific conference sponsored by the National Institutes of Health.

In June 2007, the Smith-Lemli-Opitz/RSH Foundation held a scientific conference hosted jointly by Dr. Robert Steiner from Oregon Health & Science University and Dr. Forbes D. Porter from The Eunice Kennedy Shriver National Institute for Child Health and Human Development, National Institutes of Health. The main goal of this meeting was to promote interaction between scientists with expertise in cholesterol homeostasis, brain cholesterol metabolism, developmental biology, and oxysterol and neurosteroid biochemistry, clinicians researching and treating patients with Smith-Lemli-Opitz syndrome, the patient support organization and families. This report summarizes the presentations and discussions at the conference, represents the conference proceedings, and is intended to foster collaborative research and ultimately improve understanding and treatment of Smith-Lemli-Opitz syndrome and other inborn errors of cholesterol synthesis.

A stochastic neuronal model predicts random search behaviors at multiple spatial scales in C. elegans.

Random search is a behavioral strategy used by organisms from bacteria to humans to locate food that is randomly distributed and undetectable at a distance. We investigated this behavior in the nematode Caenorhabditis elegans, an organism with a small, well-described nervous system. Here we formulate a mathematical model of random search abstracted from the C. elegans connectome and fit to a large-scale kinematic analysis of C. elegans behavior at submicron resolution. The model predicts behavioral effects of neuronal ablations and genetic perturbations, as well as unexpected aspects of wild type behavior. The predictive success of the model indicates that random search in C. elegans can be understood in terms of a neuronal flip-flop circuit involving reciprocal inhibition between two populations of stochastic neurons. Our findings establish a unified theoretical framework for understanding C. elegans locomotion and a testable neuronal model of random search that can be applied to other organisms.

Dura splitting decompression for Chiari I malformation in pediatric patients: clinical outcomes, healthcare costs, and resource utilization.

BACKGROUND: Dural splitting decompression may be an effective and safe treatment for Chiari I malformation. OBJECTIVE: To compare clinical outcomes, complications, and resource utilization for patients undergoing Chiari I decompression with or without duraplasty. METHODS: Between 2000 and 2009, the senior author performed 113 Chiari I decompression operations with dural splitting or duraplasty in children less than 18 years of age; 110 were included in a retrospective cohort analysis of safety, efficacy, and treatment cost. Patients without significant syringomyelia underwent dural splitting decompression, and patients with syringomyelia underwent duraplasty. RESULTS: : Sixty-three patients without significant syringomyelia (57%) underwent dural splitting decompression. They were significantly younger than patients undergoing duraplasty (8.3 ± 4.9 years vs 10.4 ± 4.4 years; P < .05). Headaches improved or resolved in most patients in both groups (90.5% vs 93.6%; P = .59). Dysphagia, long tract signs, cranial nerve, and bulbar symptoms also improved similarly in both groups. Three duraplasty patients were treated medically for aseptic meningitis; one underwent reoperation for a symptomatic pseudomeningocele. No patient undergoing dural splitting decompression experienced a cerebrospinal fluid-related complication. Extradural decompression required less operative time than duraplasty (105.5 vs 168.9 minutes, P < .001), a shorter length of stay (2.4 vs 2.8 days, P = .011), and lower total cost for the primary hospitalization ($26 837 vs $29 862, P = .015). CONCLUSION: In this retrospective cohort study, dural splitting decompression was equally effective, safer, and lower cost for treatment of Chiari I malformation without syringomyelia. A multicenter trial with groups balanced for the presence of syringomyelia is necessary to determine whether these results are generalizable.