Logo Medical Science Monitor

Call: +1.631.470.9640
Closed: National Holiday

Contact Us

Logo Medical Science Monitor Logo Medical Science Monitor Logo Medical Science Monitor

01 August 2009

Orbital tumor as an initial manifestation of Wegener's granulomatosis in children: a series of four cases

Barbara ChipczynskaABCDEFG, Miroslawa GralekABDG, Wojciech HautzBE, Maria Zegadlo-MylikBCD, Beata Kocyla-KarczmarewiczB, Krystyna KanigowskaB, Malgorzata SeroczynskaB, Beata KepaB, Dorota Klimczak-SlaczkaB

Med Sci Monit 2009; 15(8): CS135-138 :: ID: 878127

Abstract

Background
Wegener's granulomatosis (WG) is a rare idiopathic disease in which small and medium-sized arteries are affected by necrotizing granulomatous inflammation. It is associated with a triad of pulmonary (cavitating granulomatous lesions with hemoptysis, cough, and dyspnea), renal (glomerulonephritis with hematuria, proteinuria), and head (otitis media, recurrent sinusitis, eye or orbital involvement) manifestations.
Material and Method
Four children aged 7-11 years diagnosed with WG between 1995-2008 initially presented with unilateral proptosis and ptosis due to orbital tumor. CT or MRI, orbital lesion biopsy, and laboratory tests (ERS, CRP, ANCA) were part of the diagnostic workup. The diagnoses were based on correlation between clinical presentation and diagnostic findings. All four patients had orbital lesions on contrast-enhanced CT and MRI. Two had lesions of the temporal pyramid. Orbital tumor biopsies showed granulomatous lesions in two patients, necrotizing vasculitis with leukocytoclasia in three, and an orbital pseudotumor in one. ESR and CRP were positive in all. ANCA positivity was variable (c-ANCA did not allow WG diagnosis or there were atypical ANCAs). All had blood and protein in the urine, but only one had advanced renal involvement. All were treated with oral steroid and immunosuppression; remission was successful.
Results

Conclusions
WG is often more difficult to diagnose in children than in adults due to frequent absence of its signature features. The absence of the classic triad and atypical laboratory or biopsy findings do not exclude a diagnosis of WG. Orbital demonstration helps achieve early diagnosis and treatment of this potentially fatal rheumatologic disease.

Keywords: Tomography, X-Ray Computed, Orbital Neoplasms - radiography, Contrast Media, Child, Wegener Granulomatosis - pathology

Add Comment 0 Comments

Editorial

01 June 2024 : Editorial  

Editorial: Concerns as Highly Pathogenic Avian Influenza (HPAI) Virus of the H5N1 Subtype is Identified in Dairy Cows and Other Mammals

Dinah V. Parums

DOI: 10.12659/MSM.945315

Med Sci Monit 2024; 30:e945315

0:00

In Press

Animal Research  

Curcumin as a Potential Therapeutic Agent for Mitigating Carbon Monoxide Poisoning: Evidence from an Experi...

Med Sci Monit In Press; DOI: 10.12659/MSM.943739  

Animal Research  

Targeting the Gut-Kidney Axis in Diarrhea with Kidney-Yang Deficiency Syndrome: The Role of Sishen Pills in...

Med Sci Monit In Press; DOI: 10.12659/MSM.944185  

Review article  

A New Pandemic of the XXIst Century: The Growing Crisis of Adolescent Depression in the Digital Age

Med Sci Monit In Press; DOI: 10.12659/MSM.944838  

Review article  

A Review of Wastewater-Based Epidemiology Studies for the Assessment of Over-the-Counter Medicines Used as ...

Med Sci Monit In Press; DOI: 10.12659/MSM.944120  

Most Viewed Current Articles

17 Jan 2024 : Review article   1,652,499

Vaccination Guidelines for Pregnant Women: Addressing COVID-19 and the Omicron Variant

DOI :10.12659/MSM.942799

Med Sci Monit 2024; 30:e942799

0:00

14 Dec 2022 : Clinical Research   1,522,350

Prevalence and Variability of Allergen-Specific Immunoglobulin E in Patients with Elevated Tryptase Levels

DOI :10.12659/MSM.937990

Med Sci Monit 2022; 28:e937990

0:00

16 May 2023 : Clinical Research   690,265

Electrophysiological Testing for an Auditory Processing Disorder and Reading Performance in 54 School Stude...

DOI :10.12659/MSM.940387

Med Sci Monit 2023; 29:e940387

0:00

01 Jan 2022 : Editorial   50,215

Editorial: Current Status of Oral Antiviral Drug Treatments for SARS-CoV-2 Infection in Non-Hospitalized Pa...

DOI :10.12659/MSM.935952

Med Sci Monit 2022; 28:e935952

0:00

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

Medical Science Monitor eISSN: 1643-3750
Medical Science Monitor eISSN: 1643-3750