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pediatric proptosis

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https://www.readbyqxmd.com/read/28700400/treatment-of-pediatric-igg4-related-orbital-disease-with-tnf-%C3%AE-inhibitor
#1
Sanjai Jalaj, Kristen Dunbar, Ashley Campbell, Michael Kazim
The authors describe a 9-year-old female who presented with swelling, proptosis, and tenderness of the right upper eyelid and MRI imaging demonstrating right lacrimal gland enlargement. After failing treatment with corticosteroids, the patient underwent a biopsy that was consistent with IgG4-related orbital disease. She was subsequently successfully treated with adalimumab (TNF-α inhibitor). This is the first case report of the successful use of a TNF-α inhibitor for the treatment of IgG4-related orbital disease in a child...
July 10, 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/28596822/ethmoid-malformation-associated-with-pediatric-nasal-polyposis-and-allergic-fungal-sinusitis
#2
Cameron G Hanson, Todd B Proctor
A 17-year-old female with allergic fungal sinusitis and nasal polyposis presented with epistaxis in the emergency room. On examination, right-sided proptosis and irregular nasal obstruction were observed in the right nasal cavity. CT imaging revealed massive right-sided polyposis and significant ipsilateral malformation with boney architecture preservation of the ethmoidal labyrinth and lamina papyracea. The patient was treated surgically with symptomatic improvement. These findings indicate a unique malformation of the ethmoid while the patient was in development...
June 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28576543/pycnodysostosis-at-otorhinolaryngology
#3
Tekin Baglam, Adem Binnetoglu, Muhammet Fatih Topuz, Nilay Baş Ikizoglu, Refika Ersu, Serap Turan, Murat Sarı
AIM: Pycnodysostosis is a rare autosomal, recessive, skeletal dysplasia caused by a mutation in the cathepsin k gene. Pycnodysostosis is characterized by short stature, characteristic facial appearance (delayed closure of fontanelles and cranial sutures, mandibular hypoplasia and angle disorder, blue sclera), and acroosteolysis of the distal phalanges. Our aim was to describe the otorhinolaryngologic findings, differential diagnoses, various treatment options, and followup in eight cases of pycnodysostosis...
April 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28558378/pial-arteriovenous-fistula-and-capillary-malformation-arteriovenous-malformation-associated-with-rasa1-mutation-2-pediatric-cases-with-successful-surgical-management
#4
A Jessey Chugh, Asim Shahid, Sunil Manjila, Deepak Gulati, Nicholas C Bambakidis
We present case reports of 2 pediatric patients who were both found to have pial arteriovenous fistulas (AVFs) with subsequent genetic analysis revealing mutations in the RASA1 gene. Considering their family history of distinct cutaneous lesions, these mutations were likely inherited as opposed to de novo mutations. Patient 1 had large capillary malformations on the left side of the face and neck, associated with macrocephaly, and presented at the age of 32 months with speech delay, right-sided weakness, and focal seizures involving the right side of the body...
May 31, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28520318/-image-of-the-month-a-pediatric-case-of-unilateral-proptosis-and-cystic-lymphangioma
#5
L Adouane, Ch Chantrain, M Lewin, P Philippet, M Thimmesch
No abstract text is available yet for this article.
May 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28468165/orbital-reconstruction-in-neuroectodermal-tumor-of-the-orbit-multimodal-treatment-approach
#6
Jorge Ernesto Cantini A, Diana M Díaz López, Esteban Francisco Hernandez Florez
INTRODUCTION: Primitive neuroectodermal tumors of peripheral origin are very rare, and orbital neuroectodermal tumors are even more uncommon. Only 25 patients with primary orbital involvement in the pediatric age group have been reported. METHODS: In this article, the authors describe their experience in the multimodality treatment approach to treat neuroectodermal tumor of the orbit. The authors also present a male patient 3-year old presenting with a neuroectodermal tumor of the right orbit causing rapidly progressive proptosis...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28427053/growing-skull-fracture-and-the-orbitocranial-variant-nuances-of-surgical-management
#7
Vikram Singh, Gopalakrishnan M Sasidharan, Dhananjaya Ishwar Bhat, Bhagavatula Indira Devi
AIMS: Growing skull fracture (GSF) is a rare complication resulting from diastatic enlargement of a pediatric skull fracture. Orbitocranial GSF is a distinct variant with more complex management issues due to the displacement of the globe. This study aims to discuss surgical considerations in the management of GSF, with particular reference to orbitocranial variants. METHODS: We conducted a retrospective analysis of 7 children operated for GSFs at our institute from 2008 to 2015...
2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28403132/orbital-reconstruction-in-neuroectodermal-tumor-of-the-orbit-multimodal-treatment-approach
#8
Jorge Ernesto Cantini A, Diana M Díaz López, Esteban Francisco Hernandez Florez
INTRODUCTION: Primitive neuroectodermal tumors of peripheral origin are very rare, and orbital neuroectodermal tumors are even more uncommon. Only 25 patients with primary orbital involvement in the pediatric age group have been reported. METHODS: In this article, the authors describe their experience in the multimodality treatment approach to treat neuroectodermal tumor of the orbit. The authors also present a male patient 3-year old presenting with a neuroectodermal tumor of the right orbit causing rapidly progressive proptosis...
April 11, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28058158/a-rare-cause-of-proptosis-in-childhood-langerhans-cell-histiocytosis
#9
Mustafa Vatansever, Esra Vatansever, Erdem Dinç, Ayça Sarı, Tuba Kara
A three-year-old male patient was admitted to the clinic with proptosis in his right eye. He had a history of fever with an unknown etiology. In examination, right proptosis was observed and an immobile mass was palpated at the lateral wall of the right orbita. Eye movement was unrestricted in all directions and anterior and posterior segment examination was normal in both eyes. On computed tomography, diffuse bone destruction and expansion was observed in the right orbital lateral wall and other cranial bones...
August 2016: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/27817916/orbital-periorbital-plexiform-neurofibromas-in-children-with-neurofibromatosis-type-1-multidisciplinary-recommendations-for-care
#10
Robert A Avery, James A Katowitz, Michael J Fisher, Gena Heidary, Eva Dombi, Roger J Packer, Brigitte C Widemann
TOPIC: Children and adults with neurofibromatosis type 1 (NF1), a common autosomal dominant condition, manifest a variety of ophthalmologic conditions. Plexiform neurofibromas (PNs) involving the eyelid, orbit, periorbital, and facial structures (orbital-periorbital plexiform neurofibroma [OPPN]) can result in significant visual loss in children. Equally important, OPPNs can cause significant alteration in physical appearance secondary to proptosis, ptosis, and facial disfigurement, leading to social embarrassment and decreased self-esteem...
January 2017: Ophthalmology
https://www.readbyqxmd.com/read/27630510/neuroblastoma-in-early-childhood-a-rare-case-report-and-review-of-literature
#11
Ritesh R Kalaskar, Ashita R Kalaskar
Neuroblastoma is an extremely rare pediatric neoplasm whose prognosis becomes poor and poor as the age advances. It can be sporadic or nonfamilial in origin. It is primarily a tumor of abdominal origin from where it metastasis to lymph nodes, liver, intracranial and orbital sites, and central nervous system. There is no standard dental treatment protocol for the management of neuroblastoma due to its poor survival rate and rarity. However, dental treatment may follow the protocol of preventive and restorative...
July 2016: Contemporary Clinical Dentistry
https://www.readbyqxmd.com/read/27587345/-orbital-and-periorbital-cellulitis-in-children-epidemiological-clinical-therapeutic-aspects-and-course
#12
A Daoudi, S Ajdakar, N Rada, G Draiss, I Hajji, M Bouskraoui
Orbital cellulitis in children is a rare but potentially serious condition. The goal of this study is to analyze the epidemiological, clinical, therapeutic aspects and typical course of orbital and periorbital cellulitis in children, so as to propose a clinical management protocol adapted to our context. During the retrospective study period (2008-2014), 28 cases were hospitalized in the pediatric department at the Mohammed VI university medical center in Marrakech. Eighty-five percent of the cases were diagnosed as preseptal cellulitis, and 15% as retroseptal cellulitis...
September 2016: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/27537249/optic-gliomas-in-neurofibromatosis-type-1
#13
Emily Parkhurst, Sridevi Abboy
PURPOSE: To examine the incidence, presentation, and outcome of optic gliomas in children with neurofibromatosis type 1 (NF1) in Southern California Kaiser Permanente. METHODS: The authors queried the Southern California Kaiser Permanente electronic medical record database to find patients diagnosed as having NF1. Genetics, ophthalmology, and imaging medical records of patients with optic glioma were reviewed. RESULTS: A total of 708 patients younger than 21 years had a diagnosis of NF1 in Southern California Kaiser Permanente and 30 (4...
November 1, 2016: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/27413373/predictive-factors-of-development-of-graves-ophthalmopathy-for-patients-with-juvenile-graves-disease
#14
Dalia Jarusaitiene, Rasa Verkauskiene, Vytautas Jasinskas, Jurate Jankauskiene
Background. Due to low incidence of Graves' ophthalmopathy (GO) among children, the manifestation is poorly analyzed, posing a risk to late identification of insidious disease. Purposes. To identify predictive factors that may influence the development of GO in pediatric and young patients with Graves' disease (GD). Methods. A cross-sectional study of patients newly diagnosed with pediatric or juvenile GD during 2002-2012 was conducted at the Hospital of Lithuanian University of Health Sciences. Ocular evaluation was based on European Group on Graves' Orbitopathy survey...
2016: International Journal of Endocrinology
https://www.readbyqxmd.com/read/27291943/characteristics-of-superior-orbital-subperiosteal-abscesses-in-children
#15
Lourdes Quintanilla-Dieck, Sivakumar Chinnadurai, Steven L Goudy, Frank W Virgin
OBJECTIVES/HYPOTHESIS: Superior pediatric orbital subperiosteal abscesses (SPAs) are less common than medial ones, and clinical features specific to patients with superior SPAs have not been well defined. Clinical characteristics between patients with superior and medial SPAs are compared to determine whether superior location is a risk factor for surgical intervention. STUDY DESIGN: Retrospective cohort study. METHODS: The target population consisted of patients diagnosed with an SPA and seen by the pediatric otolaryngology service at a tertiary children's hospital between January 2010 and October 2014...
June 12, 2016: Laryngoscope
https://www.readbyqxmd.com/read/27230830/prechiasmatic-transection-of-the-optic-nerve-in-optic-nerve-glioma-technical-description-and-surgical-outcome
#16
Hamid Borghei-Razavi, Shunsuke Shibao, Uta Schick
Optic pathway glioma (OPG) encompasses a spectrum of findings ranging from lesions confined to the optic nerve only, lesions affecting the optic chiasm and hypothalamus, and lesions with diffuse involvement of a large part of the optic pathway and neighboring structures. The majority of pediatric low-grade astrocytomas in the optic/chiasmatic region are typical pilocytic astrocytoma. The rest of them (10 %) may be other gliomas such as fibrillary pilomyxoid astrocytoma (grade 2 WHO). The postsurgical local recurrence rate of 55 to 76 % has been reported in some histological subtypes such as pilomyxoid astrocytoma (grade 2)...
January 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/27112168/orbital-malignant-peripheral-nerve-sheath-tumor-with-heterologous-elements-in-a-15-year-old-girl
#17
Hayyam Kiratli, Irem Koç, Diclehan Orhan
A 15-year-old girl presented with painful proptosis in the right eye. Imaging studies demonstrated an inferiorly located orbital mass. Histopathological examination of the exenteration specimen was consistent with malignant peripheral nerve sheath tumor. This case is a rare example of orbital malignant peripheral nerve sheath tumor presumably arising from the infraorbital nerve with heterologous histologic elements.
April 23, 2016: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/27082859/optic-pathway-gliomas-clinical-manifestation-treatment-and-follow-up
#18
Abolhasan Ertiaei, Sara Hanaei, Zohreh Habibi, Ehsan Moradi, Farideh Nejat
OBJECTIVE: Optic pathway gliomas (OPG) are infrequent pediatric brain tumors that affect the optic nerve and the visual pathway in the brain. A wide spectrum of signs and symptoms, such as visual impairment, nystagmus, proptosis, and visual loss, may occur at different stages. Other manifestations, such as hydrocephalus, diancephalic syndrome, neurologic deficits, and growth and developmental delays, may be present as well. Surgical resection, chemotherapy, and radiotherapy are used as treatment strategies...
2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/26914936/ultrasound-and-mr-imaging-findings-in-prenatal-diagnosis-of-craniosynostosis-syndromes
#19
Eva I Rubio, Anna Blask, Dorothy I Bulas
BACKGROUND: Craniosynostosis syndromes are uncommonly encountered in the prenatal period. Identification is challenging but important for family counseling and perinatal management. OBJECTIVE: This series examines prenatal findings in craniosynostosis syndromes, comparing the complementary roles of US and MRI and emphasizing clues easily missed in the second trimester. MATERIALS AND METHODS: Six prenatal cases evaluated from 2002 through 2011 were retrospectively reviewed...
May 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/26905453/traumatic-orbital-encephalocele-presentation-and-imaging
#20
Leslie A Wei, Tabassum A Kennedy, Sean Paul, Timothy S Wells, Greg J Griepentrog, Mark J Lucarelli
OBJECTIVE: Traumatic orbital encephalocele is a rare but severe complication of orbital roof fractures. We describe 3 cases of orbital encephalocele due to trauma in children. METHODS: Retrospective case series from the University of Wisconsin - Madison and Medical College of Wisconsin. RESULTS: Three cases of traumatic orbital encephalocele in pediatric patients were found. The mechanism of injury was motor vehicle accident in 2 patients and accidental self-inflicted gunshot wound in 1 patient...
2016: Orbit
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