Myeloid Sarcoma with Megakaryoblastic Differentiation Arising in the Conjunctiva.

An 87-year-old woman not known to have either a lymphoma or leukemia developed a left multinodular, fish-flesh superior epibulbar and forniceal mass. A biopsy disclosed a blastic tumor with scattered multinucleated immature megakaryoblasts. Immunophenotyping of bone marrow cells revealed strong positivity for CD7, CD31, CD43, CD45, CD61, and CD117; CD71, myeloperoxidase, and lysozyme were also positive in scattered cells. Forty percent of the neoplastic cells were Ki-67 positive. Cytogenetic studies indicated a trisomy 8 (associated with worse prognosis) and a t(12; 17) translocation. Desmin, smooth muscle actin, pancytokeratin, CAM 5.2, adipophilin, tryptase, S100, SOX10, MART1, and E-cadherin were negative, ruling out a nonhematopoietic tumor. The conjunctival lesion was diagnosed as a myeloid sarcoma with megakaryoblastic differentiation, a rare variant. It probably arose from a myelodysplastic syndrome. This is the first case of its type to develop in the conjunctiva.

Lymphoepithelial Carcinoma of the Nasolacrimal Duct: Clinical, Radiologic, and Immunopathologic Features.

Undifferentiated lymphoepithelial carcinoma (exhibiting both begin lymphoid and malignant epithelial components) most commonly arises in the head and neck, especially in the nasopharynx. It may also be encountered in various ocular adnexal sites, including the nasolacrimal duct. A 63-year-old woman developed a swelling in the region of the right lacrimal sac accompanied by epiphora. CT scanning revealed an enlargement of the nasolacrimal duct from the lacrimal sac to the inferior nasal meatus. A biopsy during dacryocystorhinostomy for symptomatic epiphora revealed hypercellular sheets of small lymphocytes which were interpreted as evidence for a chronic dacryocystitis. Two years later the subtotally excised lesion had substantially grown in size. Repeat CT scans demonstrated an inferonasal anterior orbital mass with further enlargement of the nasolacrimal duct with a solid mass in its lumen, and bone erosion. The biopsy combined a rich background of lymphocytes within which were clusters of undifferentiated carcinoma cells that were cytokeratin and p63 positive. Critical review of the earlier biopsy led to the detection of the same cells, but in smaller numbers, that had been overlooked. An awareness of the possibility of lymphoepithelial carcinoma of the lacrimal sac/duct should improve diagnostic accuracy with the aid of immunohistochemistry. Radiation therapy is often successful in managing this highly sensitive malignant tumor.

Giant dacryocystomucopyocele in an adult: a review of lacrimal sac enlargements with clinical and histopathologic differential diagnoses.

Dacryocystocele is an umbrella term that refers to any diffuse, centrifugal enlargement of the lacrimal sac that results from combined proximal and distal obstructions in the tear drainage system. In adults, the presence of mucus in the cyst's contents leads to the modified term of dacryocystomucocele. If infection supervenes, which almost always occurs in protracted cases and adds the clinical dimension of a dacryocystitis, then a dacryocystomucopyocele is created. Dacryocystocele and its congeners are much rarer in adults than in children. We describe a 95-year-old woman with an acquired, enormous dacryocystomucopyocele, larger than any previously reported, that developed over 25 years and produced globe displacement with an associated conspicuous enlargement of the nasolacrimal duct. The aspirated sac fluid was mucopurulent and harbored low-virulence bacterial organisms of the Prevotella and Petosteptococcus species. In infants, dacryocystoceles are transitory as the result of spontaneously reversible factors. In adults, secondary proximal irreversible fibrotic strictures or bony changes around the nasolacrimal duct typically arise from chronic inflammation or low grade infection. Other possible causations of duct obstruction, in addition to florid mucosal edema, include encroachment on the duct by enlarged contiguous ethmoid air cells; a sinus mucocele or sinusitis; idiopathic, post-traumatic or dysplastic bony remodeling of the wall of the duct; and a neoplasm-all of which require some form of surgical intervention, typically dacryocystorhinostomy. The differential diagnosis of medial canthal swellings centered on the lacrimal sac spans malformations, diverticula, dermoid/epidermoid cysts, sac inflammations/infections causing swelling without generalized sac enlargement, encephaloceles and primary epithelial tumors, as well as extrinsic tumors impinging on the sac.

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.

The Bell's toll.

A 70-year-old man presented complaining of lid and facial drooping and facial numbness, which progressed over several months. Magnetic resonance imaging of the head showed an enlarged right cavernous sinus. His past medical history was remarkable to squamous cell carcinoma of the face. The patient underwent a craniotomy with biopsy of the cavernous sinus that confirmed malignancy. High index of suspicion in a patient that presents facial drooping and/or numbness should alert ophthalmologists about the occurrence of perineural spread of a previous malignant lesion of the skin.

Retained transorbital foreign body with intracranial extension after pipe bomb explosion.

BACKGROUND: Penetrating brain injuries caused by explosions are survived in extremely rare cases only. However, potential casualties of such cases may be encountered by regular physicians even outside a war zone, e.g., due to an assault or terror blast. There is very limited literature to this end; therefore, we report the successful neurosurgical management of a penetrating head injury due to a pipe bomb explosion. CASE DESCRIPTION: A 19-year-old man was brought to the ER with a swollen, bleeding right orbit, and a severely injured left hand after having sustained an unwitnessed explosion from a self-made pipe bomb. He presented with a GCS (Glasgow Coma Scale) of 15 at time of admission, work-up revealed an intracranial retained metal fragment measuring 5 × 1 × 0.2 cm lodged retro-orbitally and in the skull base. The patient underwent emergent right temporal craniotomy and temporal lobectomy and simultaneous right enucleation before the petrous bone and sphenoid wing lodged metal fragment was successfully removed. CONCLUSION: This case underscores the importance of having a high suspicion for the presence of an intracranial injury and a retained foreign body in the setting of a penetrating head injury. Aggressive and timely workup as well as expeditious surgical management are crucial in these settings and can generate exceptionally good outcomes despite a major trauma.

Shunts to divert aqueous humor to distant epithelialized cavities after keratoprosthesis surgery.

PURPOSE: Glaucoma is a frequent and often severe problem in patients needing keratoprosthesis (KPro) surgery. Standard glaucoma shunts in these cases often develop a very dense capsule that obstructs flow and causes intraocular pressure elevation. Therefore, we developed shunts that deliver aqueous to distant epithelialized cavities where an obstructing capsule is less likely to form. In this retroactive study of such shunts, the risk of postoperative infection (especially endophthalmitis) has been assessed. METHODS: Ahmed shunts were connected with a tube to the lacrimal sac or ethmoid sinuses. In subsequent designs the polypropylene plate was eliminated, the valve was enclosed and a distal tube added (New World Medical Inc, Rancho Cucamonga, CA). The latter shunts were connected to the maxillary sinus or lower lid fornix. Between 2001 and 2005 the devices were implanted in 34 patients with very severe ocular disease. (31 had KPro implanted). Thus, the distal tube was led to the lacrimal sac (2 cases), to the ethmoid sinuses (6), to the maxillary sinus (16 cases), and to the lower lid fornix (10 cases). The patients were followed for cumulatively 145 shunt years, with a mean follow-up of 4 years and 3 months. They were instructed to administer low-dose topical antibiotics indefinitely. RESULTS: Only 1 case of acute bacterial endophthalmitis occurred, thus an incidence of 0.7% per shunt year. A slow-growing Mycobacterium was cultured in a disintegrating eye and 1 bacterial maxillary sinusitis occurred, both in autoimmune diseases. Three valves became exposed and had to be removed (1 replaced). Four eyes developed hypotony, probably from valve failure. CONCLUSIONS: The shunt arrangement, which connects the anterior chamber to potentially microbe-populated cavities or to the lower lid fornix and its flora, might be suspected to allow rapid retrograde invasion of infective agents, resulting in endophthalmitis. In this series of KPro patients, however, the incidence of severe infection was very low, in fact comparable to that after standard trabeculectomy.

Imaging of optic nerve head drusen: improvements with spectral domain optical coherence tomography.

OBJECTIVE: To demonstrate a new algorithm that can determine the shape, location, and volume of optic nerve head drusen (ONHD), which were imaged with spectral domain optical coherence tomography (SDOCT). METHODS: One exenteration patient and 4 glaucoma patients with bilateral ONHD were recruited from the Massachusetts Eye and Ear Infirmary and from a private practice office. Images were obtained using an experimental SDOCT system developed at the Wellman Center for Photomedicine, Massachusetts General Hospital. With axial resolutions of about 6 mum, SDOCT can obtain 2-dimensional images in 1/29 of a second, compared with commercially available time domain OCT instruments with 10 mum resolution images in 1.28 seconds. The volumes of ONHD were calculated with a new algorithm and were then correlated with visual field mean deviation. RESULTS: SDOCT can display 2-dimensional images comparable with histology and 3-dimensional videos of ONHD. ONHD are signal-poor regions with high-signaled borders. Larger ONHD volumes are directly correlated with larger mean deviation absolute values on Humphrey visual field testing. CONCLUSIONS: SDOCT is a potentially better technique for ONHD imaging and may improve the diagnosis and management of patients with both OHND and glaucoma.

Mirror, mirror on the wall: placebo effects that exist only in the eye of the beholder.

Rationale The extent to which placebo effects can be driven exclusively by subjective impressions of improvement in the absence of any independent corroboration is unclear. Methods Thirty-six self-referred patients were treated with a light therapy device intended to rejuvenate facial skin. At each of eight weekly treatments, participants' facial skin was exposed for 40 seconds to pulses of multispectral LED-generated light in the range of 588 nm wavelength at 0.1 J cm(-2). Outcomes were assessed by participants as well as by the treating doctor and by blinded, expert raters. Results Patients reported robust and statistically significant improvements in seven facial features at the conclusion of the 8-week treatment regimen as well as at 1-month follow-up (for all comparisons, P 0.05). Moreover, effect sizes were close to zero and in the opposite direction from improvement (median d = -0.06 for doctor ratings; and for observer ratings, there was only a 46% success rate at identifying post-treatment as compared with pre-treatment photographs). Conclusion The robust placebo responses documented in this trial were confined to the subjective impressions of the patients. Neither the treating doctor nor blinded, expert raters could detect any improvement. Thus, patients can perceive improvement in medical interventions in the absence of any independent corroboration that improvement has occurred. This result is used a heuristic to more clearly define the components of the placebo response.

In the eye of the beholder--skin rejuvenation using a light-emitting diode photomodulation device.

BACKGROUND: A light-emitting diode (LED) photomodulation system can produce pulses of amber light expected to induce structural skin changes and reverse the effects of photoaging. OBJECTIVE: To reproduce the encouraging results already published. METHODS AND MATERIALS: Facial skin was exposed to pulses of 588+/-10-nm-wavelength light from a photomodulation device for 40 seconds once a week for 8 weeks. Photographs, clinical assessment, and a subjective questionnaire were taken at baseline, at the last follow-up, and 1 month after that. Thirty-six patients' pre- and post-treatment photos were arbitrarily scrambled, and 30 independent blinded observers were asked to pick the post-treatment photo. Two time-point comparisons were evaluated. RESULTS: For every facial characteristic studied and for both time-point comparisons, patients reported highly statistically significant improvements. In extremely sharp contrast, neither the physician's assessment nor the independent observers' evaluation indicated any improvement. CONCLUSION: Although subjective findings are comparable between studies, we were unable to reproduce the objective results of efficacy previously reported. Patients genuinely believed that several of their facial features had improved, even though there was no detectable objective change. Our data therefore suggest that the LED photomodulation treatment from the device tested is a placebo.

Age- and fatigue-related markers of human faces: an eye-tracking study.

PURPOSE: To investigate the facial cues that are used when making judgments about how old or tired a face appears. DESIGN: Experimental study. PARTICIPANTS: Forty-seven subjects: 15 male and 32 female participants, ranging from age 18 to 30 years. METHODS: Forty-eight full-face digital images of "normal-appearing" patients were collected and uploaded to an eye-tracking system. We used an Applied Science Laboratories (Bedford, MA) Eye Tracker device associated with gaze-tracking software to record and calculate the gaze and fixation of the participants' left eye as they viewed images on a computer screen. After seeing each picture, participants were asked to assess the age of the face in the picture by making a selection on a rating scale divided into 5-year intervals; for fatigue judgments we used a rating scale from 1 (not tired) to 7 (most tired). MAIN OUTCOME MEASURES: The main outcome measure was gaze fixation, as assessed by tracking the eye movements of participants as they viewed full-face digital pictures. RESULTS: For fatigue judgments, participants spent the most time looking at the eye region (31.81%), then the forehead and the nose regions (14.99% and 14.12%, respectively); in the eye region, participants looked most at the brows (13.1%) and lower lids (9.4%). Participants spent more time looking at the cheeks on faces they rated as least tired than they did on those they rated as most tired (t = 2.079, P<0.05). For age judgments, the eye region (27.22%) and then the forehead (15.71%) and the nose (14.30%) had the highest frequencies of interest; in the eye region, the brows and lower lids also had the highest frequencies of interest (11.40% and 8.90%, respectively). Participants looked more at the brows (t = -2.63, P<0.05) and glabella (t = -3.28, P<0.01) in those faces they rated as looking the oldest. CONCLUSIONS: This study supports the hypothesis that age and fatigue judgments are related to preferential attention toward the eye region. Consequently, these results suggest that aesthetic or functional surgery to the eye region may be one of the most effective interventions in enhancing the appearance of an individual. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Recognizing the protruding eye.

UNLABELLED: It is currently unknown how much exophthalmos may be noticeable to an observer. The authors determined the threshold for detection of exophthalmos may be 4 millimeters. PURPOSE: To determine the threshold for detection of exophthalmos by an observer. METHODS: The Massachusetts Eye and Ear Infirmary Ophthalmic Plastics imaging database was used to select 28 photographs of patients with unilateral exophthalmos measuring between 1 to 11 mm for the study group and 28 photographs of patients without exophthalmos for the control group. One hundred ophthalmology attendings, residents, medical students, and technicians reviewed each photograph. Participants commented on whether the patient appeared "normal" or "abnormal." RESULTS: Eighty-one percent of the control patients were correctly identified as "normal." In comparison, 60% of patients with 1 mm of exophthalmos (p < 0.001), 53% of patients with 2 mm of exophthalmos (p < 0.001), 46% of patients with 3 mm of exophthalmos (p < 0.001), 35% of patients with 4 mm of exophthalmos (p < 0.001), and 40% of patients with 5 mm of exophthalmos (p < 0.001) were identified as "normal." The vast majority of patients (91.9%, p < 0.001) with 6 mm of exophthalmos were identified as "abnormal," and almost all patients (97.9%, p < 0.001) with more than 6 mm of exophthalmos were also described as having an "abnormal" appearance. CONCLUSIONS: Greater than half of the patients with 1-2 mm of exophthalmos appear as "normal" as the control patients. In comparison, the majority of patients with 4-5 mm of exophthalmos and nearly all the patients with 6 mm of exophthalmos and greater appear "abnormal." Our data suggests that the point at which exophthalmos becomes clinically perceptible to the majority of observers is 4 mm. There may be patients with 3 mm of exophthalmos and greater with orbital pathology being "missed" on cursory external examinations by general ophthalmologists, optometrists, and general practitioners.

A lacrimal sac abscess incision and drainage technique.

A comfortable, anatomically based lacrimal sac abscess incision and drainage technique is described. The records of 52 patients were reviewed. The procedure was relieving and well tolerated because of adequate infraorbital and anterior ethmoidal nerve blocks. To promote rapid resolution, both components of the abscess were drained: the distended lacrimal sac and its associated submuscular pocket. The contiguous cavities were packed and allowed to heal by secondary intention. Of 49 cases, 39 (79.6%) were done as outpatient procedures and 41 (83.7%) were performed under locoregional anesthesia. Edema completely resolved by a median of 7 days. A repeat drainage procedure within 1 month was required in only 4 of 48 cases (8.3%). Fistulas and ectropion were not found. Four of 16 patients (25.0%) who did not eventually receive a definitive procedure (dacryocystorhinostomy or dacryocystectomy) developed a recurrent lacrimal sac abscess after complete resolution of the primary episode.