Complex Unilateral Orbital Dysplasia with Plexiform Neurofibroma, Presented as Unilateral Pulsating Proptosis: Staged Surgery and our Learning
Journal Title: International Journal of Neurology and Neurosurgery - Year 2019, Vol 11, Issue 3
Abstract
Presence of sphenoid wing dysplasia fulfills one of the diagnostic criterias of neurofibromatosis type 1 (NF1). This defect of the skull base can manifests as pulsating proptosis. Associated calvarial defects, especially away from the lambdoid suture are a much rarer presentation. This report describes the successful removal of plexiform neurofibroma followed by reconstruction of the dysplastic sphenoid wing in an NF1 patient. Later on, he underwent blepharoplasty for scarring of the upper eyelid. After successful management of the overall procedure in three stages, the patient achieved improved visual outcome along with acceptable cosmetic appearance.
Authors and Affiliations
Dhiman Chowdhury
Keywords
Related Articles
- EP ID EP669835
- DOI 10.21088/ijnns.0975.0223.11319.11
- Views 65
- Downloads 0
Clinical Profile and Aetiological Analysis of Late Onset Seizure: A Study Conducted At Tertiary Care Institute
Context: The elderly are the most rapidly growing segment of the population. When compared to other age groups, the incidence and prevalence of seizures and epilepsy are higher in the elderly. Clinical manifestations and...
Primary Ewing's Sarcoma of Spine, Its Management & Outcome: A Retrospective Analysis
Objective: PrimaryEwing’ Sarcoma (ES) of spine is rare entity but causes significant morbidity if not diagnosed in time and not managed properly, so the optimal management of primary Ewing’s Sarcoma spine should be analy...
Comparison of Decompressive Craniectomy and Maximal Medical Management with Barbiturates in Severe Traumatic Brain Injury Patients with Refractory ICP: A Randomized Controlled Study
Context: Traumatic brain injury is the major cause of death and disability worldwide. Management of severe traumatic brain injury still remains elusive, some prefer surgical intervention while others advocate medical tre...
Evolution of Post Traumatic Acute Thin SDH into Sub acute or Chronic SDH: Analysis of Symptomatology, Risk Factor and Outcomes
Introduction: The natural course of post traumatic acute thin SDH is different among various patients. Most thin SDH resolve spontaneously, a few progress into chronic subdural hematomas requiring surgical treatment. The...
Clinical Profile of Ocular Myasthenia Gravis: A Prospective Study from Tertiary Care Centre
Background: Ocular myasthenia gravis (OMG) is the most common focal form of myasthenia gravis (MG) characterized by variable weakness and fatigability of extraocular muscles (EOMs), levator, and orbicularis oculi. Most o...