Extramedullary plasmacytoma of the orbit with intracranial invasion: A case report.

RATIONALE: Plasmacytoma is a disease caused by the proliferation of monoclonal immunoglobulin-producing plasma cells and divided into multiple myeloma, solitary bone plasmacytoma, and extramedullary plasmacytoma. We report a case of orbital extramedullary plasmacytoma invading the dura mater in a patient with exophthalmos and diplopia. PATIENT CONCERNS: A 35-year-old female patient with exophthalmos in the right eye and diplopia visited the clinic. DIAGNOSES: The thyroid function tests showed nonspecific results. Orbital computed tomography and magnetic resonance imaging revealed a homogeneously enhancing orbital mass infiltrating the right maxillary sinus and adjacent brain tissue in the middle cranial fossa through the superior orbital fissure. INTERVENTIONS: To diagnose and alleviate the symptoms, an excisional biopsy was performed, which revealed a plasmacytoma. OUTCOMES: One month after the surgery, the protruding symptoms and eye movement restriction in the right eye improved, and the visual acuity in the right eye was restored. LESSONS: In this case report, we present a case of an extramedullary plasmacytoma that originated in the inferior wall of the orbit and invaded the cranial cavity. To our knowledge, no previous reports have described a solitary plasmacytoma that originated in the orbit, causing exophthalmos and invading the cranial cavity at the same time.

Incidental Hamartoma in an elderly patient: a case report.

BACKGROUND: Neuromuscular hamartoma is a very rare tumor; with only five cases reported in the orbit. It often occurs in infants and young children and involves large peripheral nerves, but there has been only few reports of occurrence in the orbit and adults. CASE PRESENTATION: This paper describes a 70-year-old man with an incidental orbital mass detected by an imaging test and who later developed associated symptoms. The mass was diagnosed as neuromuscular hamartoma. Superior rectus muscle recession and inferior rectus muscle resection were performed in the right eye for hypertropia secondary to postoperative inferior rectus muscle palsy. Hypertropia in the right eye and diplopia improved after surgery, and regular follow-up is underway. CONCLUSION: This is the first case of an incidentally detected orbital mass diagnosed by histologic examination as a neuromuscular hamartoma in an older patient whose proptosis progressed after a long period of inactivity.

Sixteen-and-a-half syndrome with metastatic pons tumor: A case report.

RATIONALE: One-and-a-half syndrome (OAAH) is characterized as the combination of ipsilateral horizontal gaze palsy and internuclear ophthalmoplegia. OAAH syndrome accompanied with 7th and 8th cranial nerve palsy is called 16-and-a-half syndrome. We aimed to report the case of 16-and-a-half syndrome with metastatic pons tumor. PATIENT CONCERNS: A 57-year-old male diagnosed with nonsmall-cell lung cancer (NSCLC) with brain metastasis occurring 15 months ago was referred to our clinic with the chief complaint of horizontal diplopia and right gaze palsy. DIAGNOSIS: According to the patient symptom, ocular examination, and radiographic findings, he was diagnosed as 16-and-a-half syndrome which was caused by brain tumor metastasis from NSCLC. INTERVENTIONS: We referred him to hemato-oncology department and he was treated with radiation and supportive therapy. OUTCOMES: Unfortunately, the patient passed away 1 month later without improvement of ophthalmoplegia. LESSONS: The clinical findings of our case indicate 16-and-a-half syndrome caused by brain tumor metastasis from NSCLC, which to our knowledge has not been previously reported. The case highlights a rare cause of OAAH spectrum disease and the importance of a systemic work-up including associated neurologic symptoms and brain imaging in patients with horizontal gaze palsy.

One-year survivor of adult alveolar rhabdomyosarcoma of the maxillary sinus with orbital extension: Case report.

INTRODUCTION: Rhabdomyosarcoma is uncommon in adults. Adult and maxillary rhabomyosarcoma with direct orbital extension has been rarely reported. To our knowledge, there is no reported case about adult patient with alveolar maxillary rhabdomyosarcoma and orbital extension survived 1 year with intact ocular function. CASE PRESENTATION: A 21-year-old female presented with protrusion of the right eye and an obstructed nasal passage for the past month. Her symptoms were not relieved by oral antibiotic or irrigation. She was referred to our clinic. Computed tomography and magnetic resonance imaging showed a large homogenous well-enhanced mass with surrounding bony erosion and remodeling. The mass extended to the ipsilateral nasal cavity and orbit. Endoscopic biopsy of the nasal cavity confirmed alveolar rhabdomyosarcoma. The maxillary mass was excised using the Caldwell-Luc approach, and the orbital mass was excised using a transconjuctival and transcaruncular approach. A systemic work-up confirmed ipsilateral lymph node metastasis. The patient received 6 cycles of chemotherapy with vincristine, dactinomycin, and cyclophosphamide, as well as 5120 cGy radiotherapy. Her ocular function was intact 1 year after treatment, and magnetic resonance imaging showed complete regression of the tumor. CONCLUSION: Rhabdomyosarcoma, which is usually an aggressive malignancy, should be considered in the differential diagnosis of a rapidly growing orbital mass. Aggressive treatment, including surgery, chemotherapy, and radiation therapy, can increase local remission rates and improve the prognosis.

A Case of Orbital Metastasis of Uterine Leiomyosarcoma With Intracranial Extension Presenting With Proptosis.

A 57-year-old woman presented with protrusion of the OS 2 months in duration and decreased vision 1 month in duration. The patient was diagnosed with uterine leiomyosarcoma approximately 3 years before presentation. Lung and liver metastases were diagnosed by biopsy 1 year prior to this visit. CT revealed a solid mass with central necrosis in the left lateral orbit that resulted in orbital shifting, bony destruction, and intracranial extension. The diagnosis was left orbital metastatic leiomyosarcoma, and the patient died 2 months after receiving the diagnosis.

Differences in end-of-life care decision making between patients with and without cancer.

BACKGROUND: Most patients and families do not want invasive life-sustaining procedures when recovery is unlikely. We compared the clinical features of advance directives (ADs) of patients with and without cancer. METHODS: The ADs were obtained from retrospectively reviewing electronic medical records of 699 consecutive patients who died from April 2011 to July 2012. RESULTS: Patients with cancer were more likely to have written ADs: 265 (85.8%) patients with cancer and 277 (71.0%) noncancer patients (P < .001). Significantly more noncancer patients were in the intensive care unit, indicating that they had received or were receiving invasive treatments. Noncancer patients requested life-sustaining treatment more frequently but symptom control less frequently than patients with cancer. CONCLUSION: Advance care planning in patients with incurable, noncancer disease is important to guarantee patient autonomy at the end of life.

A randomized phase II study to assess the effectiveness of fluid therapy or intensive nutritional support on survival in patients with advanced cancer who cannot be nourished via enteral route.

BACKGROUND: Experts advise against parenteral nutrition (PN) for patients with advanced cancer at the end of life. But because many patients and families fear starvation, many physicians administer PN to patients with terminal cancer in Korea. OBJECTIVE: We designed this study to investigate the effect of PN on survival in patients with terminal cancer at the end of life. DESIGN: We planned a randomized phase II study enrolling 116 patients randomized to receive either fluid or PN. SETTING/SUBJECTS: Eligible patients are who could not tolerate enteral feeding and had short life expectancies (<3 months) due to progressive cancer. Patients with functioning bowels were excluded. MEASUREMENTS: The primary end point was overall survival and the secondary end point was total administered calories. RESULTS: We prospectively enrolled 31 consecutive patients and 16 patients were assigned to the PN group. The study ended early because many patients and families were extremely concerned about starvation. The baseline characteristics, including nutritional parameters, were not significantly different between the two groups. The mean administered calories was 374.7 (± 71.7) kcal/d for the fluid group and 1286.8 (± 108.3) kcal/d for the PN group (p<0.001). Median survival was 8 days (95% confidence interval [CI], 5.7-10.3 days) in the fluid group and 13 days (95% CI: 3.1-22.9 days) in the PN group, and this difference was not statistically significant (p = 0.982 by Log-rank test). CONCLUSIONS: This study did not conclusively determine the role of PN for patients with advanced cancer, however, PN support failed to significantly prolong survival in these patients compared to similar patients receiving only fluid.

A congruous superior quadrantanopsia following a junctional scotoma induced by asperogillosis.

A 69-year old man presented to us with decreased vision in his right eye and a relative afferent pupillary defect. Under the presumption that he was suffering from retrobulbar optic neuritis or ischemic optic neuropathy, visual field tests were performed, revealing the presence of a junctional scotoma. Imaging studies revealed tumorous lesions extending from the sphenoid sinus at the right superior orbital fissure, with erosion of the right medial orbital wall and optic canal. Right optic nerve decompression was performed via an endoscopic sphenoidectomy, and histopathologic examination confirmed the presence of aspergillosis. The patient did not receive any postoperative antifungal treatment; however, his vision improved to 20 / 40, and his visual field developed a left congruous superior quadrantanopsia 18 months postoperatively. A junctional scotoma can be caused by aspergillosis, demonstrating the importance of examining the asymptomatic eye when a patient is experiencing a loss of vision in one eye. Furthermore, damage to the distal optic nerve adjacent to the proximal optic chiasm can induce unusual congruous superior quadrantanopsia.

Technique for autologous nasal mucosa transplantation in severe ocular surface disease.

PURPOSE: To describe the detailed surgical technique used for harvesting and transplantation of autologous nasal mucosa grafts for ocular surface reconstruction, to evaluate the clinical results and utility of autologous nasal mucosa grafts in ocular surface diseases. METHODS: Autologous nasal mucosa was harvested from either the middle or inferior turbinate. It was transplanted on 11 eyes with severe ocular surface diseases including 4 eyes with chemical burns, 2 eyes with thermal burns, 1 eye with Stevens-Johnson syndrome, 1 eye with erythema multiforme major, and 3 eyes with extensively recurred pterygiums. Clinical outcomes were assessed based on ocular manifestation, epithelization, and visual acuity. RESULTS: The mean follow-up period was 19.9 months (range 13-32 months). Nasal mucosal tissues were safely excised, and their mucosal harvest sites were completely healed in 2 weeks without postoperative complications. All nasal mucosa autograft survived successfully with rapid epithelization, well-developed horizontal vascularization, and abundant mucin secretion. Clinical success, intact ocular surface epithelium without epithelial erosion more than 4 weeks, was achieved in all eyes. There was no recurrence of significant ulceration, conjunctivalization, fibrovascularization, symblepharon, or pterygium. CONCLUSIONS: Goblet cell transplantation using autologous turbinate nasal mucosa presents a promising treatment method in patients with severe ocular surface disorders.

Recurrent occlusion of laser iridotomy sites after posterior chamber phakic IOL implantation.

We report a case of recurrent occlusion of laser iridotomy (LI) sites after a Visian ICL (Implantable contact lens version 4, Staar Surgical AG, Nidau, Switzerland) implantation. A 45-year-old woman had bilateral ICL implantation after placement of two peripheral LI sites in each eye to prevent pupillary block. At one month after the operation, severe narrowing or occlusion of four LI sites occurred. After this, although she received four additional LIs at postoperative months 1, 6, 9 and 10 in both eyes, the narrowing or occlusion recurred. Mild chronic anterior chamber inflammation was observed intermittently throughout the follow-up period. We performed clear lens extraction in both eyes (at postoperative month 11 in the left eye and month 26 in the right eye) due to recurrent occlusion of the LI sites and excess trabecular meshwork pigment deposition presumably caused by the four repeated LIs. Recurrent obstruction of LI sites can occur after ICL implantation. These problems were unresolvable despite four repeated laser iridotomies. The risks associated with anterior uveitis must be considered when planning an ICL implantation.

Iris and trabecular meshwork pigment changes after posterior chamber phakic intraocular lens implantation.

PURPOSE: To evaluate the changes in laser iridotomies, intraocular pressure (IOP), angle structures, and pupil diameter after implantable contact lens (ICL) (Version 4 Staar Surgical) implantation in myopic Asian eyes. SETTING: Department of Ophthalmology, Chung-Ang University Yongsan Hospital, Seoul, Korea. METHODS: The ICL was implanted in 81 eyes of 43 patients with spherical equivalent greater than -6.00 diopters. Gonioscopy examination was performed 1 day after laser iridotomy and 6 and 12 months after ICL implantation. The changes in laser iridotomy sites, IOP, and pupil diameter were also evaluated. RESULTS: Ten eyes (12.3%) had occlusion or narrowing of the laser iridotomy sites. The angle width was more than 30 degrees in all cases preoperatively but narrowed to less than 20 degrees in 16 eyes (19.8%) 6 months postoperatively. The mean pigment, measured by the semiquantitative method (grade 0 to 4), was 2.03 in the inferior angle, 0.18 in the nasal angle, 0.12 in the temporal angle, and 0.00 in the superior angle 12 months postoperatively. The pigment in all quadrants increased temporally as a result of the laser iridotomy or iris rubbing by the ICL; however, the pigments absorbed gradually and decreased to values before laser iridotomy 12 months postoperatively. There was a temporary increase in IOP from the instillation of steroid eyedrops 1 week and 1 month postoperatively; IOP returned to the preoperative level and remained there 12 months postoperatively. There was a significant decrease in pupil diameter 1 and 3 months postoperatively; the diameter returned to the preoperative size at 6 and 12 months. Pigment dispersion syndrome and pigmentary glaucoma were not seen. CONCLUSION: Implantation of the ICL narrowed the angle width but did not increase trabecular pigmentation compared with values after laser iridotomy, indicating ICL implantation is safe regardless of the pigmentary changes in the trabecular meshwork.