Orbital Mass in A 9Year Old Girl
Journal Title: Surgery & Case Studies: Open Access Journal - Year 2019, Vol 1, Issue 5
Abstract
A 9-year-old girl presented with 4-month history of swelling and discoloration on the right upper eyelid and inability to open the eyes completely (Figure 1). She was previously fit and well with no past medical history of note. She was initially diagnosed with periorbital cellulitis at the local hospital and was treated with antibiotics and antihistamines for four weeks with no improvement. Initial investigations showed elevated platelet count of 508, and raised inflammatory markers - ESR of 67 mm/hr and CRP of 40 mg/L. The CT scan was suggestive of a mass lesion on the upper eyelid (Figure 2). Biopsy done by the ophthalmology team showed soft tissue fibro-inflammatory mass with reactive lymphoid aggregates and dense histiocytic and eosinophilic inflammation centred around variable calibre vessels. No atypical leukemic/ lymphoid infiltrates were seen. Histiocytic cells were positive for CD160 and negative for Langerin. Hence a diagnosis of small vessel vasculitis was suggested. The child was then referred to our tertiary pediatric rheumatology service. On examination the child was apyrexial with normal vital signs. She had tender, erythematous swelling of the right upper eyelid and fullness around the right cheek (Figure 3). She had normal vision with blocked right nostril, reduced sensation of smell, and taste. The systemic examination was completely normal. The antibody testing showed negative ANA, and weakly positive p-ANCA, but MPO and PR3 were negative. An MRI of the orbit with contrast showed significant enlargement of the intra -orbital mass with enhancement in the infratemporal and pterygopalatine fossa and masticator compartment, and induration and enhancement of the fat of the right cheek (Figure 4). The possibility of IGG4 disease was discussed at the multi-disciplinary team (MDT) meeting involving pathology, oncology, and ophthalmology teams. the biopsy specimens were reviewed. This confirmed evidence of vasculitis with the presence of fibrinoid necrosis with mostly lymphocytes infiltrating the blood vessel wall. There were >10 IgG4 positive cells per high power field, but the total cells did not represent 40% of plasma cells. Her serum IgG levels were normal as well as the IgG subtypes. With a provisional diagnosis of localised IgG4 related disease, she received weaning course of steroids and weekly subcutaneous methotrexate (15mg/m2) with very good response.
Authors and Affiliations
S Deepak, K Warrier
Keywords
Related Articles
- EP ID EP639329
- DOI 10.32474/SCSOAJ.2019.01.000121
- Views 89
- Downloads 0
Use of Skin Grafts for the Correction of a Circumcision Complication
Background: Circumcision is largely performed and is considered to be technically simple and safe but it may occasionally lead to important complications. Aim: To report the case of a patient who underwent a circumcision...
Scientific Student Societies: A Way of Scientific Research Vocations Boosting
Born in Russia at the end of the XIXth century the Scientific Student Societies have developed a rather original way of initiation and involvement of the young in the scientific research during High School and University...
The History of Opcab (Off Pump) Coronary Surgery
After the advent of the heart-lung machine, few surgeons continued to use the OPCAB technique, Among those were Benetti and Buffolo from South America, who published in the early 1990s the first two large series on OPCAB...
Early diagnosis Gorlin-Goltz syndrome: A Rare Case Report
Gorlin-Goltz syndrome, is an uncommon, autosomal dominant inherited disorder, which is characterized by numerous basal cell carcinoma (known as nevoid basal cell carcinoma syndrome (NBCCS)). The common manifestations inc...
Intestinal Obstruction by Blind Vove Iess Riobamba Hospital 2014
Introduction: Cecal volvulus is the second most common volvulus among colonic volvulus, usually presenting as bowel obstruction. Case Presentation: A 65 year old female presented with abdominal pain, nausea, vomiting, ab...