Atypical Spindle Cell and Pleomorphic Lipomatous Tumor of the Ocular Adnexa: Two Cases of a Rare Entity.

Atypical spindle cell and pleomorphic lipomatous tumor (ASCPLT) is a rare lipomatous neoplasm that was recently introduced into the World Health Organization Classification of Soft Tissue and Bone tumors as a distinct entity. ASCPLT has potential for local recurrence but does not metastasize. This biologic behavior separates ASCPLT from its morphologic mimics. Ocular adnexal ASCPLT has not been previously reported. Described herein are two patients with ASCPLT. The subcutaneous orbital rim lesion featured markedly pleomorphic spindle and multinucleated cells. The eyelid lesion was dominated by atypical spindle cells in a background of mature adipocytes. Both neoplasms demonstrated infiltrative margins, rare mitotic figures, immunoreactivity for CD34 and loss of Rb1, and the absence of MDM2 amplification by fluorescence in situ hybridization. Recognition of ASCPLT in the differential of ocular adnexal neoplasms may lead to a re-evaluation of morphologically similar tumors, which may have varied biologic behavior and warrant a different management approach.

Preoperative Ultrasound-Guided Percutaneous Embolization of Orbital Lymphaticovenous Malformations Using Onyx.

The treatment of orbital lymphaticovenous malformations (OLVMs) has evolved from simple surgical resection to a multimodal approach consisting of sclerosing agents, embolization, and resection depending on the specific components that compose the lesion. Here we present a representative case series of 5 nonconsecutive patients who underwent percutaneous embolization of OLVMs with the Onyx Liquid Embolic System (Medtronic, Dublin, Ireland) before surgical resection between 2019 and 2021. OLVMs are rare, benign, congenital lesions that grow with age and can become symptomatic and disabling. Treatment can be conservative or invasive; depending on the clinical presentation and characteristics of the lesions, treatment modalities can vary. Preoperative percutaneous embolization of OLVMs with the Onyx system is a promising technique, as demonstrated in this case series.

MEN 2B masquerading as chronic blepharitis and euryblepharon.

This is a retrospective case description of a single male patient found to have multiple endocrine neoplasia syndrome type 2B (MEN 2B). At the age of 14, he presented for evaluation of ocular irritation and eyelid thickening. Intraoperative assessment revealed mucosal lesions on the anterior dorsum of his tongue and biopsy of multiple eyelid lesions disclosed bilateral submucosal neuromas, leading to the diagnosis of MEN 2B. MEN 2B is a potentially life-threatening syndrome due to the risk of developing medullary thyroid carcinoma (MTC) and pheochromocytoma. The patient was found to have MTC requiring thyroidectomy. We hope to highlight the clinical and histopathologic findings of MEN 2B in an effort to emphasize the importance of its identification in the absence of a family history.

Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review.

BACKGROUND: Fraser syndrome is an autosomal recessive disorder characterized primarily by syndactyly, cryptophthalmos, urinary tract anomalies, ambiguous genitalia, and laryngeal anomalies. A 28-year-old man with Fraser syndrome presented with cryptophthalmos, microphthalmia, lacrimal system dysgenesis, and chronic sinusitis. OBJECTIVE: The patients' clinical condition and surgical treatment are described. A literature review was conducted, and articles relevant to the case are presented. METHODS: Case report. RESULTS: To our knowledge, this is the first published case report of endonasal management of dacryocystoceles in a Fraser syndrome patient. The patient was treated via endoscopic endonasal marsupialization and drainage. CONCLUSION: Fraser syndrome patients may initially present to many different specialties as the spectrum of clinical manifestations is broad. Physicians treating these patients should take a collaborative approach to surgical and medical management.

Self-inflicted enucleations: Clinical features of seven cases.

We report the clinical presentation, radiography, and management outcomes of autoenucleations (AE). Charts of 7 patients evaluated at 4 institutions with AE were reviewed. Four males and three females had a mean age of 50 years (range 26-72 years). The etiologies were psychosis secondary to underlying mental illness (6, 88%) and substance use (1, 12%), and the mechanism was largely blunt digital injury (6, 88%). Three (43%) AE patients suffered bilateral enucleations. Common concomitant injuries included eyelid lacerations (5, 71%) and optic nerve avulsion (3, 43%). Radiography was utilized for all of the study patients with computed tomography as the most common (5, 71%), followed by ultrasound (1, 14%), and magnetic resonance imaging with CT angiography (1, 14). Orbital exploration was performed in the management of all patients. Orbital implants were placed in 4 (57%) patients. Patients were followed for a mean of 1.9 months (range 1-4 months). Autoenucleation affects both genders and is commonly associated with eyelid lacerations, optic nerve avulsion, and intracranial hemorrhage. The association with intracranial hemorrhage is consistent with prior reports of internal carotid artery injury following shearing of the optic nerve. Autoenucleation cases were seen secondary to mental or substance induced psychosis, and these patients may be at risk for future injuries such as AE of the contralateral globe. The common causes for psychosis reported our patient group include schizophrenia, depression, schizoaffective disorder, and methamphetamine-induced psychosis, which corroborates with similar cases in the literature. Two of three cases of bilateral AE suffered sequential AE where the contralateral globe was enucleated days apart. All patients suffering AE should have full medical, psychiatric, neurologic, and radiologic evaluation and monitoring while under care. When evaluating patients with obvious ocular injury, accompanying intracranial injuries should be ruled out in a timely fashion before pursuing surgical intervention.

Giant Ocular Horn Occurring in a 10-Year-Old Female.

Cutaneous horns uncommonly involve the periocular region. Involvement of the ocular surface is particularly rare. The authors present a patient who underwent a perinatal buccal mucosal graft for corneal perforation due to congenital corneal ectasia, most likely resulting from Peters anomaly. She developed a giant ocular horn 10 years later.

Solitary Extramedullary Plasmacytoma of the Orbital Apex and Dura Masquerading as Idiopathic Inflammation.

This is a retrospective case description of a single female patient found to have a primary solitary extramedullary plasmacytoma of the orbital apex and temporal/parietal dura. These monoclonal plasma cell neoplasms account for fewer than 5% of plasma cell neoplasms. Although most commonly found in the head and neck, orbital and dural solitary extramedullary plasmacytomas are extremely uncommon. The authors present a case of an extramedullary plasmacytoma involving both of these structures in a middle-age otherwise healthy female to highlight this rare entity and elucidate current treatment modalities and future considerations.

Bilateral optic nerve edema presenting as initial manifestation of thyroid eye disease.

A 48-year-old smoker with a history of hyperthyroidism treated 10 years prior to presentation with radioactive iodine ablation of the thyroid gland presented to his ophthalmologist with a 2-week history of transient loss of vision in the right eye occurring for 1 to 2 hours each morning. He denied ocular pain, diplopia or change in the prominence of one or both eyes. Examination revealed 2 mm of relative proptosis on the right, bilateral temporal flare and lower lid retraction. There was minimal upper lid retraction and no evidence of lid lag. Ocular motility was full. Dilated fundoscopic examination revealed bilateral optic nerve edema, right more than left. CT of the orbit demonstrated enlargement of the extraocular muscles bilaterally with marked enlargement of the right medial rectus and left inferior rectus muscles resulting in crowding at the orbital apex bilaterally. Laboratory testing revealed the patient to be hyperthyroid. The patient was treated with high dose oral steroids followed by orbital radiation. Hyperthyroidism was managed by the patient's primary care physician. Visual symptoms rapidly improved with oral steroids and orbital radiation. Optic nerve edema completely resolved. Repeat CT imaging demonstrated a reduction in the enlargement of the extraocular muscles with relief of bilateral optic nerve compression.

Sebaceous Carcinoma of the Ocular Region: The 2014 Professor Winifred Mao Lecture.

Sebaceous carcinoma is a malignancy arising in the periocular region that can lead to blindness and tumor-related metastases. This study is a review of published literature and personal experience. This malignancy can arise from the sebaceous units in the tarsus (meibomian glands), in association with the cilia (Zeis glands), in the brow, and in the caruncle. There is a tendency for diffuse intraepithelial growth (pagetoid spread) that can be clinically invisible. Detection before lymph node metastasis is critical. This malignancy often masquerades as chronic unilateral conjunctivitis or blepharitis, typically in older patients. Management includes a 2-step approach with step 1 focused on eyelid and conjunctival map biopsies to determine the full extent of solid deep tumor and pagetoid spread. After complete review of all biopsies, step 2 is performed using local resection for all deep tumor, cryotherapy to pagetoid disease, and reconstruction. In most cases, the posterior lamella of eyelid is sacrificed with tumor removal, whereas the anterior lamella of the eyelid can be saved. After removal and cryotherapy, immediate reconstruction, using clean instruments, with buccal membrane graft for the posterior lamella and skin flap for the anterior lamella, is developed. For persistent or recurrent pagetoid disease, cryotherapy, topical mitomycin C, or plaque radiotherapy is provided. Exenteration is sometimes necessary. Sebaceous carcinoma, if detected early, can be managed with carefully planned map biopsy to determine tumor extent, followed by local resection, cryotherapy, and eyelid reconstruction. Orbital exenteration is occasionally necessary.

Wooden intraorbital foreign body injuries: clinical characteristics and outcomes of 23 patients.

PURPOSE: To describe the clinical characteristics, interventions, and visual outcomes of orbital injuries associated with wooden foreign bodies. METHODS: A retrospective case review of orbital injuries managed at Wills Eye Institute and Massachusetts Eye and Ear Infirmary was conducted between 1992 and 2006. RESULTS: The clinical course and management for a total of 23 intraorbital wooden foreign body injuries were reviewed. The distribution of wood included pencil (39%), tree branch/plant matter (35%), and other treated wood (26%). About half of the subjects (52%) presented with preoperative vision between 20/20 and 20/40. Almost all [corrected] of the subjects with preoperative vision between 20/20 to 20/40 retained vision in that range postoperatively (92%). [corrected] Time from injury to presentation was highly variable, ranging from 24 hours to 17 months (mean, 62 days; median, 3 days). Forty-three percent of subjects presented within 24 hours of injury. The site of foreign body found within the orbit was superior (26%; n = 6), medial 30% (n = 7), inferior (26%, n = 6), posterior (9%; n = 2), and lateral (4%; n = 1). Preliminary radiographic interpretation for foreign body was definite in 61% (n = 14), possible in 22% (n = 5), and absent in 13% (n = 3). CONCLUSIONS: Young men are at particularly high risk for wood intraorbital foreign body. There was a relatively equal distribution of wood type. The time from injury to presentation was variable, ranging from <1 day to over a year. Almost half of the subjects presented within 24 hours of injury. In patients with a known site of penetration, almost half occurred in the conjunctiva, notably without presence of eyelid laceration, emphasizing the need to check the conjunctiva and fornices closely. Preliminary radiographic readings often miss or are inconclusive in detecting the foreign body. The shape, location, serial examinations, and particularly the use of quantitative CT are extremely helpful in distinguishing retained wood foreign body from other low-density signals of air or fat.

Alveolar rhabdomyosarcoma presenting as an acute orbital mass in the medial rectus muscle.

Rhabdomyosarcoma is the most common pediatric primary neoplasm in the orbit, often presenting with rapid proptosis and orbital symptoms. We describe a 15-year-old girl who presented with an acute mass in her medial rectus muscle that was subsequently diagnosed as widely disseminated alveolar rhabdomyosarcoma. To our knowledge, this represents the first reported case in which an enlarged extraocular muscle was the initial manifestation of disseminated alveolar rhabdomyosarcoma.

Incidence of infraorbital hypesthesia and sinusitis after orbital decompression for thyroid-related orbitopathy: a comparison of surgical techniques.

PURPOSE: To compare the incidence of postoperative infraorbital (V2) hypesthesia and sinusitis between transantral/Caldwell-Luc and transconjunctival orbital decompression for thyroid-related orbitopathy. METHODS: A retrospective review of all orbital decompressions from January 1994 to January 2001 performed by one surgeon was done. Seventy-eight patients (133 orbits) with thyroid-related orbitopathy underwent orbital decompression for compressive optic neuropathy, exposure keratopathy, or uncontrolled intraocular pressure. Main outcome measures were the presence of V2 hypesthesia and sinusitis determined at 3 postoperative evaluations (1 to 2 weeks, 1 to 3 months, 3 to 6 months). RESULTS: The incidence of V2 hypesthesia in the transconjunctival group declined from 29.2% (19/65) to 6.1% (4/65), whereas the incidence of V2 hypesthesia in the transantral/Caldwell-Luc group decreased from 68.2% (43/63) to 46% (29/63) over the postoperative course. The rate of sinusitis in the transconjunctival group stayed constant at 6.1% (4/65) at early and late postoperative visits, whereas the transantral group rate of sinusitis declined from 33.3% (21/63) to 9.5% (6/63). CONCLUSIONS: Transconjunctival orbital decompression results in a statistically significant decrease of early and late postoperative infraorbital hypesthesia and early postoperative sinusitis when compared with the transantral approach.

Optic nerve sheath meningiomas and advanced treatment options.

PURPOSE OF REVIEW: Recent trends in the management of optic nerve sheath meningiomas have shifted towards fractionated conformal external beam radiotherapy. The purpose of this paper is to review the current literature on this subject and review the clinical characteristics and natural history of optic nerve sheath meningiomas. RECENT FINDINGS: Articles published within the last 2 years have reported the efficacy of using fractionated radiation therapy alone. A total of 62 patients in three studies have been reported; 40.3% retained stable visual acuity, 41.2% percent had improvement in their vision, and 18.4% percent had a decrease in visual acuity. Median follow-up time ranged from 22 to 51 months in these studies. SUMMARY: Optic nerve sheath meningiomas may be safely managed with fractionated external beam radiotherapy in select patients with possible improvement or stabilization of visual acuity. This represents an enormous improvement in a disease with typically poor visual prognosis.

Rediseño de perfil del profesional de la carrera de Bioquimica: determinación de las necesidades actuales del campo laboral / Redesign of profile of the professional of the Biochemical studies, determination of the present necessities of the labor field, Faculty of Ciencias Farmaceuticas y Bioquímicas, UMSA, La Paz, Bolivia: management 2001

Pregunta de investigación. ¿Como aportar a diseñar el perfil profesional dela carrera de Bioquímica de la Facultad de Ciencias Farmacéuticas y Bioquímicas de la Universidad Mayor de San Andrés a través del conocimineto de las necesidades actuales del campo laboral y de la sociedad en cuanto se refieren al profesional bioquímico. Bolivia gestión 2001?.Objetivo de la investigación. Contribuir al conocimiento de las necesidades actuales del campo laboral y de la sociedad en lo que se refiere al profesional bioquímico que aporten a diseñar el perfil del profesional de la carrera de Bioquímica de la Facultad de CienciasFarmacéuticas y Bioquímicas UMSA, Bolivia, Gestión 2001.Material y métodos.Se aplico un estudio de tipo cualitativo-descriptivo. El preente estudio fue realizado en la ciudad de La Paz, Facultad de Ciencias Farmacéuticas y Bioquímicas, UMSA. Para poder cumplir con los objetivos planteados se utilizaron los siguientes métodos: Método teóricos: Descriptivo y de análisis documental. Métodos empíricos: Entrevista personal semiestructurada y exploratoria. Encuesta-cuestionario, en grupo, mixta ( conpreguntas cerradas y abiertas). Resultados. La identificación de los requerimientos de la oferta de empleo muestra que con más frecuencia se solicitan conocimientos generales en todas las áreas del campo laboral de bioquímico, en comparación al requerimiento de conocimientos y habilidades en una área específica, que es requerida en mucha menos frecuenci. En este mismo sentido, las instituciones o laboratorios que actualmente ocupan a profesionales bioquímicos exclusivos a una sola área del ejercicio profesional del bioquímico conrresponden a un porcentaje relativamente bajo (21 porciento). De la misma manera se ha establecido principalmente la necesidad de conocimientos teóricos, práctica de procedimientos generales básicos, como puntos clave en el desarrollo de las actividades profesionales.