Silicone Sphere Implant Extrusion From Orbital Granulomatosis With Polyangiitis: A Rare Complication in the Anophthalmic Socket.

Orbital implant extrusion is a known complication following evisceration and enucleation. In this case report, we present a 45-year-old woman who presented with a left silicone implant exposure and infection 2 years following evisceration with saddle nose on examination. CT of the maxillofacial bones without contrast showed bilateral soft tissue infiltration around the superior recti muscles, as well as a nasal septum perforation from extensive sinus disease. Left orbitotomy revealed a small fibrotic mass near the orbital roof. Biopsy and serology results were consistent with granulomatosis with polyangiitis.

Management Strategy of a Grade-3 Cerebrospinal Fluid Leak Repair Following Endoscopic Endonasal Transsphenoidal Excision of Pituitary Macroadenoma.

A skull- base defect with grade-3 cerebrospinal fluid (CSF) leak following a pituitary macroadenoma removal is rare and challenging. We provide a simple sample model of multilayer closure with naturally available hard and soft tissue components. Tamponade was provided to the reconstructed site with a simple inflated Foley's catheter bulb. There was no repair failure and cavities were well mucosalised on follow-up. Mucosal and turbinate preservation was fully achieved in this method as no turbinate flaps were raised or large raw surface exposure was there.

Relapse of tagraxofusp treated blastic plasmacytoid dendritic cell neoplasm with loss of CD123 expression.

Tagraxofusp, a CD123-based-targeted immunotherapy, was recently approved to treat blastic plasmacytoid dendritic cell neoplasm (BPDCN) with excellent response. Also, a subset of BPDCN shows resistance to tagraxofusp. These resistant cases continue to express CD123, which forms the basis of the continued utility of tagraxofusp in newer combination chemotherapies to overcome resistance in BPDCN. Herein, we report a case of an elderly male with BPDCN that achieved complete remission on initial primary treatment with tagraxofusp. However, BPDCN relapsed after 1.5 years while on treatment, with loss of CD123 expression. At relapse, the neoplasm was comprehensively immunophenotyped by flow cytometry (performed on both peripheral blood and bone marrow specimen) and by immunohistochemical evaluation of the bone marrow clot section. The neoplasm at relapse was diagnostic of BPDCN with a lack of CD123 expression. This case highlights a potential limitation of current and upcoming tagraxofusp-based multidrug therapies, at least in a subset of refractory BPDCN. We believe our report will serve as a sentinel to incite future investigations involving alternate resistance mechanisms in BDPCN.

Trend in prevalence of coronary artery disease and risk factors over two decades in rural Punjab.

OBJECTIVES: The burden of coronary artery disease (CAD) has increased in the last three decades in low-income and middle-income countries including India. CAD is responsible for 20% deaths in India. The burden of CAD has increased due to a higher prevalence of risk factors related to the changing lifestyle. We studied the change in prevalence of CAD and risk factors over 20 years in a rural area. METHODS: A rural population of adults over the age of 30 years from three villages of Punjab was surveyed for the prevalence of CAD and its risk factors in 1994 and 2014 using similar research methodology. CAD was diagnosed by Epstein and clinical criteria. Blood pressure, anthropometry, ECG and biochemical analysis were carried out. The findings of two surveys were compared with a look at the change in the prevalence of CAD and its risk factors over 20 years. RESULTS: The overall age standardised prevalence of CAD increased from 2.79% in 1994 to 4.06% (p<0.05) in 2014. There was a significant increase in the prevalence of several risk factors including sedentary lifestyle (8.2% vs 41.3%, p<0.001), hypertension (14.5% vs 26.5%, p<0.001), diabetes (4.7% vs 9.7%, p<0.001), obesity (16.6% vs 35.4, p<0.001) and hypercholesterolaemia (7% vs 9.6%, p 0.011). In contrast, cigarette smoking (8.9% vs 3%, p<0.001) and use of desi ghee (51.4% vs 28.5%, p<0.001) decreased. CONCLUSIONS: In a rural population of Punjab, the prevalence of several CAD risk factors like sedentary lifestyle, hypertension, diabetes, obesity and hypercholesterolaemia increased over 20 years. These changes in risk factors were associated with a modest increase in prevalence of CAD.

A Rare Case of Metastatic Desmoplastic Small Round Cell Tumour: Diagnosis and Management.

A 26-year-old male without any significant past medical history presented to the hospital with shortness of breath, cough, pleuritic chest pain, and weight loss for the past 3 months. On chest CT, he was found to have extensive mediastinal and hilar lymphadenopathy and multiple pulmonary nodules. On physical examination, a right groin mass was noted which had been slowly growing for the past 2 years. Ultrasound of the groin showed complex solid mass with internal vascular channels. CT guided biopsy of the mass showed desmoplastic small round cell tumour. His hospital course was complicated by hypoxic respiratory failure requiring emergent intubation and ICU admission where he completed one cycle of vincristine, cyclophosphamide, and doxorubicin with subsequent improvement, followed by extubation. His condition continued to improve after second cycle of chemotherapy and he was ultimately discharged in a stable condition to continue outpatient chemotherapy after a 2-month inpatient stay.

Giant ethmoid mucocele presenting as an orbital mass.

Mucocele is a benign and expansile cystic lesion lined with respiratory mucosa of pseudo stratified columnar epithelium. Mucocele most commonly produces bone destruction within the paranasal sinuses. It mostly involves the frontal sinus followed by ethmoid and rarely sphenoid and maxillary sinuses. We report a case of giant ethmoid mucocele who presented with history of progressive unilateral protrusion of eye since 5 years. CT scan revealed a large mucocele of the ethmoid sinus with orbital extension on the same side. He was successfully treated with endoscopic sinus surgery.

A trimodality approach in the management of metastatic low-grade epithelioid hemangioendothelioma of the bone.

A 29-year-old man presented with a 2-week history of severe pain in the left foot with no preceding history of trauma. A left foot radiograph demonstrated a cortical lucency in the mid-distal shaft of the third metatarsal bone. MRI of the left foot showed an expansile lesion in the same location. A staging bone scan showed a focal uptake in the known lucency in the left third metatarsal and in the proximal left femur. A subsequent left hip radiograph demonstrated a lucency in the intertrochanteric region. CT scan of the chest, abdomen and pelvis was unremarkable. A biopsy of the left third metatarsal expansile lesion performed during an incision and curettage procedure revealed an epithelioid haemangioma (EHE) of the bone. MRI of the left hip performed in response to the findings on the bone scan showed metastatic disease in the left intertrochanteric region. A prophylactic left hip fixation surgery with an interlocking intramedullary femoral nail was therefore undertaken to avoid a pathological fracture of the left hip from the metastatic disease. Simultaneously, a left hip biopsy was performed, which also revealed an EHE. The patient underwent external beam radiation to the left femoral head and neck. This was followed by fractionated radiosurgery to the left third metatarsal. Once the left foot wound had healed, the patient subsequently received four cycles of doxorubicin and ifosfamide. A restaging positron emission tomography CT carried out after completion of therapy showed no metabolic evidence of residual primary tumour or metastasis. More than 2 years after completing his trimodality therapy, the patient remains fully functional and symptom free.

A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome.

A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient's skin biopsy was consistent with Langerhans cell histiocytosis.

Isolated Ocular Manifestation of Relapsed Chronic Myelogenous Leukemia Presenting as Myeloid Blast Crisis in a Patient on Imatinib Therapy: A Case Report and Review of the Literature.

Blast phase in chronic myelogenous leukemia (CML) has rarely been reported to involve extramedullary sites like skin, lymph nodes, and central nervous system. Clinical history, characteristic hematologic findings (elevated leukocyte counts, myelocytic predominance, and basophilia), and Philadelphia chromosome are of high diagnostic significance especially in isolated extramedullary presentations. We describe a unique case of CML relapse with blast phase involving the eye. A 66-year-old man with a known diagnosis of CML on imatinib and in molecular remission for 3 years presented with a painful blind eye. Histologic examination revealed diffuse involvement of choroid, iris, vitreous humor, and the optic nerve by blast cells. The blasts expressed CD34, aberrant TdT, and a myeloid phenotype (CD13, CD33, and CD117). Fluorescence in situ hybridization (FISH) of vitreous fluid detected BCR-ABL1 gene rearrangement. Additionally, trisomy 8 and gains of 9 and 22 were seen which were not present in the initial diagnostic marrow study 3 years ago. At relapse, the bone marrow, peripheral blood, and the cerebrospinal fluid were not involved by CML. Patient received induction chemotherapy and single dose prophylactic intrathecal methotrexate and was maintained on antityrosine kinase therapy and eventually underwent allogenic stem cell transplantation.