Unilateral proptosis in a child-need for prompt diagnosis

Proptosis in a child often presents as a diagnostic dilemma. Proptosis can be secondary to infection or childhood malignancies. It warrants urgent and relevant investigations to facilitate correct diagnosis and treatment. The common causes of proptosis include infection and malignant lesions. Any delay in intervention in either of the causes can lead to significant morbidity or can impair the vision of the child. An orbital neoplasm in the pediatric age group is an uncommon clinical finding which can initially manifest as proptosis. Here, we report a case of a 2-year-old girl, presenting with progressive swelling of the right eye. An incisional biopsy confirmed the diagnosis of embryonal rhabdomyosarcoma (RMS). RMS is an aggressive tumor; hence, early diagnosis and prompt treatment are highly essential to prevent significant morbidity and can save the vision of the child.

UNILATERAL PROPTOSIS - A DIAGNOSTIC DILEMMA

Objective: To study epidemiology, presentation and management of cases with unilateral proptosis presenting at a tertiary care hospital. Study Design: Case series. Place and Duration of Study: Armed Forces Institute of Ophthalmology, Rawalpindi Pakistan, from Jan 2016 to Dec 2017. Methodology: Retrospective analysis of hospital record of patients with unilateral proptosis was carried out. Data was entered and analyzed in SPSS version 22. Chi square test was used for statistical analysis. Results: Thirty-three cases of unilateral proptosis with a mean age of 41.79 ± 3.87 years were included in the study. Pain was the most common association. Proptosis was mild in 42.4%, moderate in 48.5% and severe in 9.1% cases. In 61% cases, diagnosis was made on excisional or incisional biopsy and on the basis of radio imaging in 39% cases. Most common cause of proptosis found was pleomorphic adenoma (12%). Patients with axial and non-axial proptosis were 48.5% and 51.5% respectively. Fourteen cases (42.5%) were managed surgically and 2 cases (6%) were observed. Total of 22 cases (67%) improved after treatment, 8 cases (24%) went into remission, 2 (6%) deteriorated, and 1 (3%) died in the study period. Conclusion: Unilateral proptosis is a diagnostic challenge. Pertinent history taking and detail examination is the key, but associated signs or symptoms not always point towards correct diagnosis. Tissue biopsy provides a conclusive diagnosis especially where radio imaging is doubtful.

A RARE CASE OF EXTRAOCULAR ORBITAL LYMPHOMA IN A YOUNG PATIENT

Orbital lymphoma refers to a lymphoma that commonly involves the conjunctiva, lacrimal gland, eyelid and ocular musculature. Primary non1 Hodgkin's lymphoma (NHL) of the orbit is a rare subset, representing only 1% of all NHL and 8-10% of extranodal NHL. We report a case of 24 year old female presented with redness, swelling, an intermittent pain and gradual protrusion of the right eye (RE) since 6 months. Her vision in RE was 6/60 and in Left eye (LE) 6/6. Intraocular pressure (IOP) was 16 mmHg in Both Eye. On examination, RE was proptosed 30mm approximately. Extraocular movement were restricted in all gazes, conjunctiva was chemosed and congested. LE was within normal limits. No lymph nodes were palpable. Ultrasonography of the right orbit showed a mass in the lateral aspect in extraconal space extending to the superior, lateral and retrobulbar region. CT scan of the right orbit showed a mass encasing the extraocular muscles and displacement of the optic nerve. FNAC reports were suggestive of non-Hodgkin's Lymphoma of the right orbit. She was given 6 cycles of RCHOP regime which includes (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin and prednisolone). Follow-up during each cycle of chemotherapy was done and showed good response initially. Even though rare, primary orbital NHL should be kept in considerat Conclusion- ion in a young female patient with slow growing unilateral Proptosis. After imaging & diagnostic biopsy, patient should be immediately staged & treated. Early diagnosis and treatment improves survival.

Olfactory Neuroblastoma of the Sinonasal Tract with Prominent Orbital Protrusion: A Case Report and Literature Review

Olfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Olfactory neuroblastoma centered in the posterior right orbit with prominent orbital protrusion is even rare. Grading ONB is extremely important as individualized treatment plans must be formulated according to tumor grade. We report the case of a 67-year-old female who presented with the chief complaints of persistent nasal congestion with intermittent epistaxis and unilateral proptosis over the past five years. Radiological imaging was suggestive of a large heterogeneous mass in the right superior nasal cavity with extensions into the right medial orbit, nasopharynx, the right maxillary sinus, the anterior cranial fossa, right ethmoidal, frontal and bilateral sphenoidal sinuses, as well as into the right frontal lobe. Assessment of the radiologic features revealed the diagnostic possibility of olfactory neuroblastoma. A nasopharyngeal biopsy confirmed an olfactory neuroblastoma. Frontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities, paranasal sinuses and right medial orbit from below was done. Evaluation of histopathological characteristics and immunohistochemical findings revealed a diagnosis of WHO grade IV olfactory neuroblastoma. Because of poor economic condition, the patient did not take adjuvant radiotherapy and chemoradiation and post-operative examination. We report a huge ONB centered in the posterior right orbit with prominent orbital protrusion. Magnetic resonance image and computed tomography are helpful for evaluating the appearance and the extent of ONB, as well as grading this tumor, which may aid therapeutic decisions and improve survival.

Posttraumatic Subperiosteal Orbital Hematoma With Intracranial Hemorrhage in A Male Infant

Background: To the best of our knowledge, there has been no report of subperiosteal orbital hematoma combined with intracerebral hemorrhage in pediatric patients.Case presentation: A healthy 9-month-old boy with left superior tarsal conjunctival prolapse was admitted to the emergency room after left periorbital blunt trauma. Computer tomography of the brain showed a left orbital roof fracture and subperiosteal orbital hematoma with acute intracerebral hemorrhage. He received emergency craniotomy. Three days after the craniotomy, the subperiosteal hematoma and proptosis increased. An anterior orbitotomy with orbital subperiosteal hematoma evacuation and drain placement were performed. Nine months after surgery, the left blepharoptosis and conjunctival prolapse improved and fix and follow were good in his both eyes. However, a new-onset intermittent exotropia and left hypotropia were detected. Conclusions: Orbital subperiosteal hematoma must be considered in the differential diagnosis of acute unilateral proptosis after trauma. In infants and children, a thorough work-up to detect concomitant intracerebral hemorrhage is necessary. Prompt surgical removal of hematoma is recommended to prevent complications

Invasive Aspergillosis of Right Maxillary Sinus with Orbital Extension in an Immunocompetent Individual

Secondary involvement of orbit in aspergillosis following paranasal sinus fungal infection is rare. A case of invasive aspergillosis of right maxillary sinus in a 31-year-old immunocompetent male patient was referred to Ophthalmology OPD with unilateral proptosis. Patient developed proptosis in right eye since six months, which was insidious in onset, progressive, not associated with painful movements. It showed regression since last one month. On examination, best corrected visual acuity was 6/12 in right eye with light projection accurate in all quadrants on presentation, with progressive diminution of vision over 15 days, normal colour vision, pupil was normal in size, reacting to light. Extraocular movements were reduced on lateral side. Left eye examination was within normal limit. Magnetic resonance imaging showed involvement of intra and extraconal compartment upto apex of orbit of right eye, pushing optic nerve medially, with erosion of lamina papyrecea. Intravenous amphotericin B was started and functional endoscopic sinus surgery with maxillary sinus debridement was done. Regular follow-up was kept to prevent the optic nerve involvement.

Unilateral proptosis in a 3-year-old child – A case report

Optic nerve gliomas (ONGs) are now regarded as true neoplasm with the ability to invade locally, but are relatively rare. Gliomas arise from astrocytes of the optic nerve and visual pathway. We describe a case of a 3-year-old bo y with painless progressive protrusion left eye for 1 month. The child was evaluated clinically and required investigations were carried out, the findings were consistent with the diagnosis of ONG. Magnetic resonance imaging is diagnostic to see its extension and to plan its management. An integrated approach should involve neuro-ophthalmologists, hemato-oncologist, oculoplastic surgeons, neurosurgeons, and radiation oncologists.

Posterior Scleritis Mimicking Orbital Cellulitis: A Report of the Three Cases

Objective: To report on three cases of posterior scleritis presenting as orbital cellulitis. Case Report: The authors retrospectively reviewed the medical records of three patients who were diagnosed as posterior scleritis but initially presented as orbital cellulitis. All patients were young males who presented with pain, unilateral proptosis, eyelid swelling with redness, and limitation of ocular movement. Initially, the diagnosis of orbital cellulitis was suspected and patients had been treated with systemic antibiotics but with no clinical improvement. Systemic work-up for infections and autoimmune diseases were negative. The orbital computed tomography (CT) scans showed severe posterior eyewall thickening with associated soft tissue edema. Ultrasonography showed a typical T-sign, compatible with posterior scleritis. Specific cause of scleritis or an association with systemic disease was not established. Systemic corticosteroids (1 mg per kg per day) were administered, and all patients improved within several days. Conclusion: Posterior scleritis is a rare and severe ocular inflammatory disorder that can mimic orbital cellulitis. Early detection and prompt treatment is crucial. Keywords: Posterior scleritis, Orbital cellulitis, Non-specific orbital inflammation, Orbital computed tomography scan, T-sign ultrasonography