Echothymia: environmental dependency in the affective domain.

Echothymia is stimulus-bound affective behavior, an echophenomenon in the domain of affect. Like echolalia and echopraxia, it is a concomitant of the environmental dependency associated with dysfunction of the frontal-striatal systems that mediate so-called frontal lobe functions. The authors introduce the definition and phenomenology of echothymia, overview its differential diagnosis and clinical significance, and suggest ways in which understanding echothymia may contribute to clinical management.

Characterization of pneumatic retinopexy failures and the pneumatic pump: a new complication of pneumatic retinopexy.

BACKGROUND: We review a three-year series of cases with pneumatic retinopexy (PR), specifically evaluating failures and suggesting a new mechanism of failure. METHODS: One hundred and thirty-five consecutive patients who underwent PR for rhegmatogenous retinal detachments were included. The primary outcome measure was the characteristics of PR failures. Secondary outcome measures included preoperative clinical characteristics. RESULTS: Seventy-five percent of the patients had successful results with PR; 100% were ultimately reattached with additional surgery. All but 1 failure (97%) occurred within the first postoperative month and 85% occurred by postoperative Day 10. The most common cause of failure was a new detachment from a new break (23 of 35 failures). Risk factors for failure included presenting visual acuity worse than 20/60 (odds ratio [OR] = 1.89), male gender (OR = 1.52), age >60 years (OR = 1.32), >2 breaks (OR = 1.28), pseudophakia/aphakia (OR = 1.20), and detachments after trabeculectomy (OR = 2.43). Lattice degeneration, high myopia (>-6.00 diopters), left eye, detachment after cataract surgery or yttrium aluminum garnet capsulotomy had minimal influence on outcome (all OR < 1.10). CONCLUSION: Characterization of PR failures will improve patient selection. We have named a new complication secondary to a patient performing the steamroller maneuver five times the "pneumatic pump."

The oculocardiac reflex in an adult with a non-displaced orbital floor fracture.

The current dogma is that the oculocardiac reflex from orbital trapdoor fractures occurs only in children and young adults. We present the occurrence of the oculocardiac reflex in an adult with a non-displaced orbital floor fracture. CT demonstrated the adventitia surrounding the inferior rectus trapped in and below the orbital floor fracture. The patient's oculocardiac reflex resolved by early next morning, presumably from the tissue escaping from the orbital floor defect.

Syphilitic posterior placoid chorioretinitis.

We present a case of syphilitic posterior placoid chorioretinitis, an uncommon but distinct ocular manifestation of syphilis. Because of the ocular findings, a lumbar puncture was performed to evaluate for neurosyphilis, which was positive. The ocular symptoms resolved with intravenous penicillin.

Pancreatic panniculitis secondary to acinar cell carcinoma of the pancreas.

We report the case of a 68-year-old white woman who presented with painful, 1- to 4-cm, erythematous nodules located bilaterally on the anterior and medial shins that had progressively developed and worsened over the last month. Workup revealed pancreatic panniculitis (PP) secondary to acinar cell carcinoma of the pancreas (ACCP). The unique clinicopathologic features, differential diagnosis, underlying causes, associated laboratory and clinical findings, pathophysiology, treatments, and appropriate workup for PP also are reviewed.

Yellow corneal ring associated with vitamin supplementation for age-related macular degeneration.

PURPOSE: To report the first described cases of peripheral yellow corneal rings secondary to vitamin supplementation for age-related macular degeneration (ARMD). DESIGN: Retrospective single-center case series. PARTICIPANTS: The eyes of 4 patients taking vitamin supplementation for ARMD were examined at the University of Pittsburgh Medical Center Department of Ophthalmology between January 2010 and April 2011. METHODS: We reviewed the medical records of 4 patients with peripheral corneal rings receiving vitamin supplementation for ARMD. MAIN OUTCOME MEASURES: The presence of peripheral yellow corneal rings, skin findings, and serum carotene levels. RESULTS: Each patient had circumferential, yellow, peripheral corneal rings and exhibited subtle yellowing of the skin most notable on the palms. Serum carotene levels were normal in 2 of the 3 patients and markedly elevated in the last patient in whom it was measured. CONCLUSIONS: It is unclear at this time how to counsel patients with this ocular finding. We suspect that these rings are more common than generally appreciated because they may have a subtle appearance or be misdiagnosed as arcus senilis. We suggest that a formal study be performed on a cohort of patients taking vitamin supplementation for macular degeneration that specifically screens for yellow rings and measures serum carotene levels when they are identified.

Cryptococcal choroiditis in advanced AIDS with clinicopathologic correlation.

PURPOSE: To describe a case of disseminated cryptococcal meningitis with multifocal choroiditis and provide optical coherence tomography (OCT) findings correlated with described histopathology in a patient with advanced acquired immunodeficiency syndrome (AIDS). OBSERVATIONS: The patient was a 54-year-old man with AIDS who presented with dyspnea and headache followed by acute vision loss. OCT demonstrated a lesion with a small area of fluid that was limited by a more prominent and irregular external limiting membrane with underlying nodular choroidal thickening, mild RPE disorganization, and hyperreflectivity of the overlying photoreceptor layer. Patient was found to have disseminated cryptococcal infection and passed away despite aggressive therapy. Autopsy was performed including bilateral enucleation and a Cryptococcus lesion was confirmed on histopathology. CONCLUSION AND IMPORTANCE: This case highlights the clinical, imaging, and histopathologic findings of cryptococcal choroiditis and provides a review of the updated treatment recommendations for disseminated infection in a patient with advanced AIDS. Although currently fundoscopy has proven most useful in directing the diagnostic algorithm in choroiditis in the setting of advanced immunosuppression, OCT may provide insight into the spread of Cryptococcus within the eye.

Same-Day Versus Next-Day Repair of Fovea-Threatening Primary Rhegmatogenous Retinal Detachments.

PURPOSE: To evaluate the outcomes of same-day versus next-day repair of fovea-threatening rhegmatogenous retinal detachments (FT RRD). DESIGN: Retrospective, multi-surgeon observational case series. METHODS: Operative reports and medical records were reviewed to evaluate a number of visual and anatomic outcomes, including presenting features, intraoperative complications, and postoperative results in the repair of primary FT RRD undergoing same-day versus next-day repair with scleral buckling, pars plana vitrectomy, or both procedures. PARTICIPANTS: A total of 96 consecutive patients (43 same-day, 45 next-day, and eight two days later) were compared. RESULTS: There was no statistically significant difference in visual outcomes between same-day and next-day repair at postoperative months 3 and 6 and at last follow-up (month 3 mean BCVA 20/30 same day; 20/32 next day; p = 0.82). Preoperative vision was strongly correlated with postoperative acuity. Effect of differences in length or type of visual symptoms, location of RRD, gender, or lens status on postoperative month 3 best-corrected visual acuity (BCVA) was not statistically significant. Overall, 85% of patients had a BCVA of 20/40 or better at postoperative month 3. Reoperation rate and intraoperative complications were not statistically different between the two groups. Re-attachment was achieved in all but one patient in both groups. Time in the operating room was longer for same-day surgery (2.98 ± 0.46 hours) compared to next-day surgery (2.54 ± 0.38 hours) (p < 0.001), which was statistically significant even when factoring in the type of surgery performed. However, one case did progress to a macula-off detachment in a superior RRD with breaks found in lattice degeneration. CONCLUSION: Next-day surgery provided equivalent visual outcomes. Emergent, same-day surgery has logistical and resource implications as it may be more expensive, may necessitate rescheduling of previously booked cases, and may limit preoperative examination by the surgeon and perioperative team.

Dilated iris vasculature in the setting of the neonatal hypoxic encephelopathy.

The differential diagnosis of dilated iris vasculature in a neonate includes retinopathy of prematurity with anterior segment plus disease, persistent fetal vasculature, intrauterine cocaine exposure, maternal diabetes, and other pathologies associated with iris neovascularization and ischemia seen in adult populations, such as central retinal vein occlusions, ocular ischemic syndrome, and chronic retinal detachment. We present neonatal hypoxic ischemic encephalopathy as a new etiology of dilated iris vasculature in a male baby who suffered a large in-utero brain vasculature insult three weeks prior to delivery but with normal fundi, no risk factors for retinopathy of prematurity (normal birth weight, and gestational age), and no other explanatory etiologies. The mechanism of the dilated iris vasculature is likely also ischemic and therefore its presence likely portends a poor prognosis. We recommend that the neonatologist evaluate for this sign for this reason and consult ophthalmology to ensure its correct etiology.

Spontaneous Regression of a Massive Sporadic Chiasmal Optic Pathway Glioma.

Optic pathway gliomas have highly variable and unpredictable natural histories, including spontaneous regression, making management decisions difficult and controversial. We report a case of substantial spontaneous regression of an optic pathway glioma involving the chiasm in a 15-month-old boy. Our case is particularly striking given the massive size at presentation with chiasmal, bilateral optic tract, and midbrain involvement, along with clinical ophthalmologic signs for which the majority of practitioners may have recommended treatment on presentation.

A potential life-saving diagnosis--recognizing Turcot syndrome.

A previously healthy 9-year-old girl presented with ataxia, headaches, and nausea of 1 month's duration. Magnetic resonance imaging demonstrated a large posterior fossa mass. Posterior segment examination revealed pigmented ocular fundus lesions (POFLs), which included cometoid dark lesions with depigmented tails and smaller, dark midperipheral lesions. The patient underwent resection for a medulloblastoma. Because of these specific retinal lesions in combination with her medullobastoma, a diagnosis of Turcot syndrome was made and subsequently confirmed by genetic testing. Turcot syndrome is one of the familial adenomatous polyposis (FAP) syndromes. This diagnosis may be life-saving because 100% of FAP patients develop colon cancer that can be cured only early with timely colectomy.

Retinal dysplasia of holoprosencephaly.

Retinal dysplasia occurs in the setting of sporadic and syndromic holoprosencephaly, which often has associated ocular malformations. The pathology of this dysplasia, which includes rosettes, has been previously described. However, its funduscopic findings have not been well documented. The authors present the fundus images of a patient with severe holoprosencephaly with retinal dysplasia and bilateral optic nerve colobomas that resulted in death 2 weeks after birth.

Donor tissue characteristics in preparation of DMEK grafts.

PURPOSE: The aim of the study was to determine donor tissue characteristics that contribute to longer peeling times or tissue tears during peeling during Descemet membrane endothelial keratoplasty (DMEK) graft preparation. METHODS: The data analyzed included donor age, gender, postmortem tissue age, contralateral eye data, peel time, and peel complications of 116 consecutive DMEK donor tissues, which were prepared by a single surgeon with the submerged cornea using backgrounds away (SCUBA) technique. RESULTS: The mean peel time was 10.1 ± 3.3 minutes (range, 6-24 minutes). Tears were noted in 7 cases (6%) and occurred more commonly with longer peel times. Three of the torn grafts had either peripheral tears or small, noncentral buttonholes or horseshoe tears and were successfully transplanted, for a transplant rate of 97%. However, the host stroma in these cases took longer to clear postoperatively. The donor gender and postmortem day of peeling did not have any statistical significance for the peeling time or tear rate (P > 0.05). Interestingly, tissue from donors older than 65 years had slightly faster peel times, but this difference did not reach statistical significance (≤65 = 11.0 ± 4 minutes; >65 = 9.3 ± 3.1 minutes; P = 0.07) and had equal rates of graft tears (6%). Most importantly, however, there was a strong correlation between peel times for the 2 eyes from the same donor (r = 0.78, P = 0.03), and the risk of a tear during peeling in the second eye was much greater if the first graft also had a tear during preparation (odds ratio = 7.2, P = 0.02). CONCLUSIONS: Exclusion of the contralateral eye tissue when there was difficulty in graft peeling for the first eye may prove to be a unique quality of DMEK donor tissue.

Recurrent ocular involvement in pediatric atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (HUS) is a subtype of thrombotic microangiopathy associated with complement alternative pathway dysregulation. It is clinically characterized by a relapsing course and a poor prognosis. Multiple organ systems are commonly affected by thrombotic microangiopathy in pediatric atypical HUS; however, ocular involvement is rarely reported. The case of an 11-year-old girl diagnosed as having atypical HUS who presented with bilateral central retinal vein occlusions with macular subhyaloid hemorrhage during her initial onset and ophthalmoplegia, diplopia, and optic disc edema during her relapsing episode 1 year later is described. All ocular manifestations occurred in the convalescence phase of atypical HUS. No other extrarenal complications were found and full recovery was achieved following typical treatment for atypical HUS (ie, plasma infusion, steroid, and supportive therapy). This is thought to be the first reported case of recurrent ocular involvement in pediatric atypical HUS.

Recurrent ocular involvement in pediatric atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (HUS) is a subtype of thrombotic microangiopathy associated with complement alternative pathway dysregulation. It is clinically characterized by a relapsing course and a poor prognosis. Multiple organ systems are commonly affected by thrombotic microangiopathy in pediatric atypical HUS; however, ocular involvement is rarely reported. The case of an 11-year-old girl diagnosed as having atypical HUS who presented with bilateral central retinal vein occlusions with macular subhyaloid hemorrhage during her initial onset and ophthalmoplegia, diplopia, and optic disc edema during her relapsing episode 1 year later is described. All ocular manifestations occurred in the convalescence phase of atypical HUS. No other extrarenal complications were found and full recovery was achieved following typical treatment for atypical HUS (ie, plasma infusion, steroid, and supportive therapy). This is thought to be the first reported case of recurrent ocular involvement in pediatric atypical HUS.

Endocyclophotocoagulation as an adjuvant to vitreoretinal surgery in cases with concomitant glaucoma.

BACKGROUND AND OBJECTIVE: To evaluate the efficacy of endocyclophotocoagulation (ECP) in patients undergoing pars plana vitrectomy for vitreoretinal disease with medically uncontrolled glaucoma. PATIENTS AND METHODS: This is a retrospective review of 12 patients who underwent 270° ECP following vitrectomy. RESULTS: Ten of the 12 patients had excellent intraocular pressure (IOP) control postoperatively, without medication in four patients and with reduced medication in six patients. IOP decreased from 26.3 ± 12.9 to 17.9 ± 6.0 at 1 month (P = .03) and 16.9 ± 4.4 at 3 months postoperatively (P = .02). Best corrected visual acuity was improved by 1 month postoperatively. The number of glaucoma agents decreased from 2.7 ± 1.1 to 1.3 ± 1.3 (P = .03). One patient with neovascular glaucoma developed hypotony. Postoperative inflammation or discomfort did not exceed what was expected for a standard vitrectomy in any patient. CONCLUSION: ECP can be effectively combined with vitrectomy; however, ECP produced unpredictable results in the two patients with neovascular glaucoma.

Chronic keratitis with intrastromal epithelioid histiocytes: a new finding in Muckle-Wells syndrome.

PURPOSE: To report a new corneal finding in Muckle-Wells syndrome (MWS) and its subsequent treatment. METHODS: The medical chart of a 45-year-old woman with a history of MWS was reviewed. She presented with progressive bilateral stromal edema and scarring that had been worsening over the past year. The left eye, which had more severe stromal scarring and a best spectacle-corrected visual acuity (BSCVA) of hand motion, had a history of cataract removal with lens implantation, argon laser trabeculoplasty, and trabeculectomy 2 years previously for open-angle glaucoma. The right eye had also undergone argon laser trabeculoplasty and trabeculectomy and had a BSCVA of 20/100. RESULTS: The patient underwent penetrating keratoplasty in the more severely affected left eye. Nine months postoperatively the patient had a BSCVA of 20/40 in the eye that had undergone transplantation. The pathologic specimen demonstrated a chronic keratitis with focal calcification of Bowman layer, localized Descemet membrane breaks, marked endothelial loss, and pronounced intrastromal epithelioid histiocytes without evidence of amyloidosis. CONCLUSIONS: To the best of our knowledge, this case highlights a new association with MWS. Epithelioid histiocytes have been demonstrated in other parts of the body in patients with MWS including the skin and joints, which is sometimes accompanied by amyloidosis.

Pilomatrixoma in a patient with bilateral retinoblastoma.

A 3-year-old girl with a history of bilateral retinoblastoma presented with a new right lower periorbital mass that showed calcifications on ultrasound. She had previously undergone systemic and intra-arterial chemotherapy for retinoblastoma but had no evidence of active disease for at least 6 months previously. Her family and oncologists feared that this mass was an extraocular metastasis of her retinoblastoma. On excision, it was diagnosed as a pilomatrixoma, an uncommon benign neoplasm that originates from the matrix of the hair root. This is the first reported case of pilomatrixoma in a patient with retinoblastoma.