scholarly journals Atlanto-axial rotary instability (Fielding type 1): characteristic clinical and radiological findings, and treatment outcomes following alignment, fusion, and stabilization

2020 ◽  
Author(s):  
Fraser C. Henderson ◽  
Robert Rosenbaum ◽  
Malini Narayanan ◽  
Myles Koby ◽  
Kelly Tuchman ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Radiological Findings ◽  
Rotary Instability

scholarly journals Screening and Treatment Outcomes in Adults and Children With Type 1 Diabetes and Asymptomatic Celiac Disease: The CD-DIET Study

Diabetes Care ◽  
2020 ◽  
Vol 43 (7) ◽  
pp. 1553-1556 ◽  
Author(s):  
Farid H. Mahmud ◽  
Antoine B.M. Clarke ◽  
Kariym C. Joachim ◽  
Esther Assor ◽  
Charlotte McDonald ◽  
...  
Keyword(s):  
Type 1 Diabetes ◽  
Celiac Disease ◽  
Treatment Outcomes ◽  
Adults And Children ◽  
Diet Study

scholarly journals Moyamoya syndrome associated with neurofibromatosis type I in a pediatric patient

2011 ◽  
Vol 129 (2) ◽  
pp. 110-112 ◽  
Author(s):  
Luiz Guilherme Darrigo Júnior ◽  
Elvis Terci Valera ◽  
André de Aboim Machado ◽  
Antonio Carlos dos Santos ◽  
Carlos Alberto Scrideli ◽  
...  
Keyword(s):  
Moyamoya Disease ◽  
Autosomal Dominant ◽  
Genetic Disorder ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Moyamoya Syndrome ◽  
Radiological Findings ◽  
Convulsive Seizures ◽  
Age Range

CONTEXT: Neurofibromatosis type 1 (NF-1) is the most prevalent autosomal dominant genetic disorder among humans. Moyamoya disease is a cerebral vasculopathy that is only rarely observed in association with NF-1, particularly in the pediatric age range. The present study reports an occurrence of this association in an infant. CASE REPORT: An eight-month-old female presented convulsive seizures with clonic movements. The patient suffered an ischemic stroke with hemiparesis. Magnetic resonance imaging revealed radiological findings compatible with moyamoya disease. The diagnosis of NF-1 was made at the age of 20 months. CONCLUSION: Despite the rarity of this association in childhood, children with focal neurological symptoms and a diagnosis of NF-1 deserve to be investigated for moyamoya syndrome.

scholarly journals Orbital Apex Lesions: A Diagnostic and Therapeutic Challenge

2017 ◽  
Vol 79 (04) ◽  
pp. 386-393 ◽  
Author(s):  
Sung-Woo Cho ◽  
Won-Wook Lee ◽  
Dae Ma ◽  
Ji-hoon Kim ◽  
Doo Han ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Clinical Characteristics ◽  
Inflammatory Pseudotumor ◽  
Definitive Treatment ◽  
Fungal Sinusitis ◽  
Orbital Apex ◽  
Radiological Findings ◽  
Presenting Symptoms ◽  
Invasive Fungal Sinusitis ◽  
Significant Difference

Objective To analyze the clinical characteristics of and treatment outcomes for orbital apex lesions according to their pathological diagnosis and identify clinical characteristics that could aid in their differential diagnosis. Design Retrospective analysis design was used for this study. Setting The study was conducted in a single tertiary institution. Participants Patients with pathologically confirmed lesions centered in the orbital apex who were admitted between January 2011 and December 2015. Main Outcome Measures Clinical characteristics, including demographics, predisposing factors, presenting symptoms, radiological findings, intraoperative findings, biopsy results, and treatment outcomes. Results Nine patients with invasive fungal sinusitis, six with inflammatory pseudotumor, and six with neoplastic or tumorous lesions were enrolled. The most common presenting symptom was orbital pain or headache, followed by ophthalmoplegia and vision loss, which exhibited overall recovery rates of 62.5% and 33.3%, respectively, after definitive treatment. The prognosis was worse for patients with invasive fungal sinusitis. There was no significant difference in age, underlying medical conditions, absolute neutrophil count, C-reactive protein level, and radiological findings among the three groups. Grossly necrotic tissues around the orbital apex area at biopsy were more frequently found in patients with invasive fungal sinusitis than in the other patients. In most cases, pain ameliorated after surgical intervention. There were no surgery-related morbidities. Conclusions Lesions centered in the orbital apex included invasive fungal sinusitis, inflammatory pseudotumor, and tumorous lesions. However, clinical features that clearly differentiated chronic invasive fungal sinusitis from inflammatory pseudotumor could not be identified. Our findings suggest that prompt biopsy is warranted for timely diagnosis, symptom relief, and early implementation of definitive treatment.

Congenital giant plexiform neurofibroma with occipital calvarial dysplasia in association with meningoencephalocele in neurofibromatosis Type 1 and segmental neurofibromatosis

2013 ◽  
Vol 12 (5) ◽  
pp. 458-464 ◽  
Author(s):  
Ravi Dadlani ◽  
Venkatraman Sadanand ◽  
Nandita Ghosal ◽  
Alangar S. Hegde
Keyword(s):  
Plexiform Neurofibroma ◽  
Neurofibromatosis Type ◽  
Transverse Sinus ◽  
Calvarial Defect ◽  
Review Of Literature ◽  
Radiological Findings ◽  
First Case ◽  
Rare Lesion ◽  
Vascular Channels

Giant plexiform neurofibroma (GPNF) of the scalp is an extremely rare lesion reported in association with neurofibromatosis. Occipital location of GPNF is even more infrequent, especially in association with occipital dysplasia (OD). The authors report 2 pediatric cases of GPNF associated with OD. The first case had an associated meningoencephalocele, and the second had large vascular channels within the lesion and the dominant ipsilateral transverse sinus lying in the center of the calvarial defect. The authors present these 2 unusual cases with a review of literature and discuss the radiological findings, theories of etiopathogenesis of the OD, and management dilemmas.

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