Digital Journal of Ophthalmology

A 22-year-old woman with alcoholic pancreatitis and bilateral central scotomata

Tushar Dave — 2024-08-18

A 22-year-old woman presented emergently at Kelowna General Hospital, British Columbia, with a 3-day history of epigastric pain with radiation to her back. This was assessed to be alcoholic pancreatitis, and she was admitted to the hospital for supportive care. Approximately 2 hours into her admission, she reported new-onset, binocular, blurry vision but no pain, flashes, floaters, or scotomata. On the third day of admission, the patient reported new-onset, bilateral, patchy central vision loss. Given the evolution of her vision loss, the ophthalmology service was consulted.

Widefield fundus photograph and optical coherence tomography of vitamin A deficiency retinopathy

Soungmin Cho — 2024-06-23

A 55-year-old woman with end-stage nonalcoholic liver disease presented with nyctalopia. Her best-corrected visual acuity was 20/30 in both eyes. Funduscopic examination showed multiple, midperipheral yellow punctate deposits bilaterally. Optical coherence tomography of the macula revealed an attenuated ellipsoid zone in each eye (Figure, inset). Her vitamin A level was <5 mcg/dL (reference range, 32.5–78.0 mcg/dL). She started vitamin A and was advised to follow up in 4 weeks. Unfortunately, the patient was unable to follow up for repeat fundus examination. In cirrhosis, there is inadequate delivery of bile salts into the intestinal lumen, leading to insufficient absorption of fat-soluble vitamins such as vitamin A. Vitamin A, which is all-trans retinol, is a precursor of rhodopsin and a vital component of visual phototransduction. Thus, vitamin A deficiency commonly leads to nyctalopia. Supplementation of vitamin A improves the visual symptoms.

Frosted branch angiitis in a case of chronic kidney disease

Nafees Fathima — 2024-07-01

A 35-year-old man with history of chronic kidney disease and on dialysis thrice weekly presented at Sankara Eye Hospital, Guntur, with sudden-onset decrease of vision in both eyes. On examination, vision in the right eye was counting fingers; best-corrected visual acuity in the left eye was 6/9. The anterior segment was grossly normal. Fundus examination of the right eye revealed a pale, edematous retina, with extensive subretinal fluid in the macula and panretinal florid perivascular sheathing and hemorrhages, with the appearance of frosted branch angiitis. The fundus of the left eye had flame-shaped hemorrhages and hard exudates along arcades as well as segmentation of blood flow in the inferior branch retinal vein. We made a provisional diagnosis of secondary frosted branch angiitis in the right eye and hypertensive retinopathy in the left eye.

Unilateral congenital prepapillary vascular loop

Júlio Almeida — 2024-07-21

An 18-year-old man presented at Professor Doutor Fernando Fonseca Hospital for a first-time ophthalmology appointment with a complaint of bilateral blurred vision of several months’ duration. He had uncorrected myopia. Fundus examination of his left eye revealed a congenital prepapillary arterial loop with a figure-eight configuration along the Cloquet canal. The fellow eye was unremarkable. Prepapillary vascular loops are congenital retinal vascular anomalies that present as a twisted bundle of vessels projecting into the vitreous cavity and returning to the optic disc, usually with a benign and asymptomatic course. Although most cases are asymptomatic, observation is advised because of possible complications, such as branch retinal artery occlusion and vitreous hemorrhage.

Mirror image: opposite paths of cilioretinal arteries

Dhruval Khurana — 2024-07-22

A cilioretinal artery was noted in each eye during routine eye examination of a 22-year-old man. Using a mobile phone camera equipped with a 20 D lens, an image of each fundus was captured. In the right eye (A), the cilioretinal artery was seen to originate from the upper temporal quadrant, whereas in the left eye (B), it originated from the lower temporal quadrant.

A rare case of Merkel cell carcinoma of the eyelid

Stefano Gouigoux — 2024-07-22

An 80-year-old man presented at the Eye Clinic of the University of Trieste for evaluation of a rapidly growing left superior eyelid lesion. Past ophthalmologic history was positive for cataract extraction with IOL implantation, performed some years previously. The patient’s medical history was also positive for chronic renal insufficiency, atrial fibrillation, and ischemic heart disease. The patient had undergone excision of a squamous cell carcinoma located on the scalp and several treatments for actinic keratosis, indicating a tendency to develop sun exposure–related tumors. The oculoplastics specialist reported a nodular, tender, elastic lesion, measuring about 1.9 × 1.2 cm and not easily mobile, located just inferior to the lateral third of the left brow (A). The overlying skin was hyperemic, with local telangiectatic vessels. There were no signs of infection and no pain or tenderness. Ultrasonography and Doppler ultrasonography (B) showed a hypoechogenic, 1.2 × 0.7 × 0.5 cm, solid, highly vascularized mass. After surgical resection, pathological evaluation was performed and revealed a “small blue round cell tumor” in the dermis (C [hematoxylin and eosin]), characterized by the following immunohistological essay: CK-20 (D) and BerEp-4 positivity, p-63, CK-5 and CK-6 negativity, high Ki-67 percentage. These findings were consistent with a diagnosis of Merkel cell carcinoma, a very rare and aggressive skin tumor, which occurs infrequently in the periocular region.

An artifact of ultrawide-field retinal imaging due to DSAEK graft marking

Nicholas J. Heller — 2024-07-23

An 82-year-old woman with a history of Fuchs endothelial corneal dystrophy status post Descemet stripping automated endothelial keratoplasty (DSAEK) graft in the left eye was referred to Monmouth Retina Consultants for cystoid macular edema following cataract surgery. On pseudocolor ultrawide-field retinal imaging (Optos UWF) of the right eye a large letter “S” artifact overlying the nasal retina was noted (A). The artifact was also seen on fundus autofluorescence (B). On the slit-lamp examination of the patient’s right eye, the “S” marking was visible on the temporal side of the graft, which was the source of the observed artifact (C). The patient was asymptomatic, and the same letter “S” appearance was not appreciable on a clinical funduscopic examination. During standard endothelial grafting procedures, corneal surgeons typically label the graft’s endothelial surface with a letter using permanent violet marking pen, which helps ensure correct graft orientation.

Dye-enhanced visualization of trabecular meshwork for canal-based minimally invasive glaucoma surgery

Nitika Beri — 2024-07-25

A male patient in his mid-fifties presented to our tertiary care hospital with diagnosis of both eyes moderate primary open angle glaucoma (POAG) with immature senile cataract (IMSC) was controlled on three antiglaucoma medications (AGM) in right eye (RE) and 2 AGMs in left eye (LE). On examination patient had best corrected visual acuity (BCVA) of 6/60 in RE with nuclear sclerosis of grade 2 along with a cup to disc ratio (CDR) of 0.8:1. LE examination showed a BCVA of 6/24 with nuclear sclerosis of grade 1 along with CDR of 0.8:1. On gonioscopy the angles were open in both the eyes up to scleral spur and the trabecular meshwork (TM) was lightly pigmented. Visual field examination by Humphrey Visual Field analyzer (Carl Zeiss Meditec,Dublin,CA,USA) showed moderate glaucoma in both eyes as per Hodapp -Parrish- Anderson criteria. Patient was planned for aqueous angiography guided BANG (Bent Ab-interno Needle Goniectomy) in high flow region followed by phacoemulsification surgery.[1] Indocyanine green (ICG) dye (0.5%) was injected in anterior chamber for performing aqueous angiography and simultaneous staining of the anterior capsule of the lens to facilitate capsulorrhexis.[1,2] On intraoperative gonioscopy it was noted that ICG dye also stained the TM to green color which made it easy to identify and incise the TM during BANG procedure (Figure 1A & 1B). We would like to highlight that ICG dye (0.5%) can aid in enhanced visualization of the TM and facilitate procedures of canal based MIGS by trainee surgeons (Figure 1B).

An unusual primary conjunctival plasmacytoma

Dario Marangoni — 2024-07-27

A 57-year-old man with past medical history of chronic alcoholic pancreatitis was referred to the Oculoplastics Service of the Ophthalmology Clinic at Ospedale Maggiore, Trieste, for evaluation of a lower eyelid neoformation of recent onset. On examination, a sessile, reddish-pink, conjunctival lesion measuring 7 × 4 mm was observed in the middle one-third of the right lower eyelid fornix. The lesion was mobile with respect to the tarsus and displayed superficial telangiectatic vessels (A). Histopathological analysis of the excised lesion showed proliferation of mature plasma cells in a trabecular pattern. Immunohistochemistry showed positivity for CD38 (B), CD56, CD79a, CD117, CD138, MUM1/IRF4, and cyclin D1, revealing a nonsecretory plasmacytoma. Conjunctival plasmacytomas are exceedingly rare plasma cell neoplasms that can be isolated (primary) or associated with bone marrow plasmacytosis or multiple myeloma (secondary). Depending on the size and localization, treatment options include local excision, external beam irradiation, or radiotherapy after surgical excision. Long-term follow-up with periodic systemic evaluations, is necessary to confirm that conjunctival involvement is not an early manifestation of multiple myeloma. After referral for hematological evaluation, a bone marrow biopsy and PET/CT scan were performed to rule out secondary lesions; results were negative, confirming the diagnosis of primary conjunctival plasmacytoma. The patient has been monitored for 24 months after diagnosis, and no evidence of local recurrence or systemic disease has been detected.

Atypical bilateral posterior polymorphous corneal dystrophy – a perpendicular presentation

Aashna P. Doshi — 2024-07-28

A 25-year-old woman presented at V N Desai Hospital, Mumbai, with occasional blurring of vision in both eyes. There was no history of forceps use or difficulty during normal delivery at birth. On examination, best-correct visual acuity in both eyes was 20/25. Slit-lamp examination of the right eye (A) showed two central, vertically oriented, parallel, bandlike lesions with serrated edges in the Descemet membrane. The left eye (B) had two central, horizontally oriented, parallel bands with a few fine vesicles in the intervening endothelial surface of the cornea. The remainder of the examination was unremarkable. The patient was diagnosed with posterior polymorphous corneal dystrophy (PPCD) in a variation where one eye has the classic horizontal orientation, and the other eye has a variant oriented vertically. PPCD is a rare, autosomal dominant corneal endothelial disorder with variable manifestations. Abnormal thickened Descemet membrane laid down by endothelial cells manifests as vesicular, bandlike or diffuse patterns at the posterior cornea. The exact incidence of PPCD is unknown because of its typically asymptomatic nature. Most patients have a bilateral, nonprogressive, asymptomatic disease, but asymmetric presentation is known to occur in many cases.

Glistening aqueous and pseudohypopyon—phacolysis without raised intraocular pressure

Aashna Doshi — 2024-07-28

Phacolytic glaucoma is a rare secondary open-angle glaucoma. A 70-year-old woman presented at V N Desai Hospital, Mumbai, with gradual, progressive diminution of vision in her left eye of 6 months’ duration. Best-corrected visual acuity (BCVA) in the left eye was counting fingers close to the face. Slit-lamp examination of the left eye revealed minimal conjunctival congestion, clear cornea, and a deep anterior chamber with multiple refractile particles (white arrow) forming a pseudohypopyon (black arrow) and a hypermature (Morgagnian) cataract with an intact anterior lens capsule. Intraocular pressure (IOP) measured 14 mm Hg by Goldmann applanation tonometry. With lens aging, there is stretching of the anterior lens capsule. Degeneration of lens fibers of a hypermature cataract forms soluble, high-molecular-weight proteins that leak through microscopic openings of the intact lens capsule. These leaked proteins are phagocytosed by macrophages and can be seen as refractile particles in the anterior chamber, milky aqueous, pseudohypoyon, or white deposits on the anterior lens capsule. They can block the trabecular meshwork, resulting in raised IOP, which manifests as acute pain, redness of conjunctival hyperemia, and corneal edema. Cataract extraction is the definitive treatment for phacolytic glaucoma. Our patient had not developed raised IOP and had a clear cornea. She underwent successful small-incision cataract surgery in the left eye. Postoperative BCVA was 20/40. The classical pathophysiological findings in this case are not easily appreciated in more typical presentations with dense corneal edema due to elevated IOP.

Laser-induced subhyaloidal and macular hemorrhage with complete recovery following vitrectomy

Arash Daneshtalab — 2024-08-21

An 11-year-old boy presented with decreased vision after accidental exposure to a handheld blue laser pointer directed at his right eye. Best-corrected visual acuity in that eye was hand motions. Posterior segment examination revealed hemorrhaging in both the subhyaloidal space and the intramacular layers of the affected eye with sedimented blood in the subhyaloidal space. Raster OCT of the affected eye just above the sedimented blood level revealed two distinct membranes: a highly reflective membrane consistent with the ILM, and an overlying irregular, low-reflective membrane corresponding to the posterior hyaloid interface. A hyperreflective and well-defined lesion (clotted hemorrhage) was surrounded by hyperreflective dots (red blood cells) beyond the ILM. Additionally, a dome-shaped hyperreflective lesion corresponding to subhyaloidal hemorrhage was observed. Four days after the incident, the patient underwent a standard, 23-gauge, three-port, pars plana vitrectomy, with concurrent ILM peeling and aspiration of liquefied blood. Postoperatively, he was started on betamethasone eye drops 4 times daily for 2 weeks, followed by a taper. At 5 weeks’ follow-up, best-corrected visual acuity in the affected eye improved to 20/25, and fundus examination revealed complete resolution of premacular hemorrhage, without postoperative complications. Opacification of the RNFL was visible in the prefoveal area, but without obvious scarring. Corresponding OCT showed attenuation and irregularity of the inner retinal layers, including the RNFL, primarily on the temporal side of the fovea. The most likely source of hemorrhage in this case was a sub-branch of a retinal vessel, given the absence of any obvious chorioretinal laser burn damage.

Excision of an intrascleral cyst guided by anterior segment optical coherence tomography

Corrina P. Azarcon — 2024-07-06

A 4-year-old girl presented with an enlarging, congenital, intrascleral cyst of the left eye. Intraoperative anterior segment optical coherence tomography was used to visualize and to assess the extent of the cyst, facilitating safe excision. The cyst was completely removed, and the defect was covered with an amniotic membrane graft, with a good outcome.

Secondary glaucoma as a late complication of a primary iris stromal cyst

David Oh — 2024-08-03

An 18-year-old woman with a congenital iris stromal cyst was referred for sudden-onset increased intraocular pressure (IOP) that did not respond to medical therapy. Ultrasound biomicroscopy demonstrated synechial angle closure from 9:00 to 12:00 in addition to a large cyst occupying much of the inferior angle. After implantation of an Ahmed glaucoma valve, her visual acuity returned to baseline, and IOP normalized. Although primary iris cysts are generally stationary and have rarely been reported to cause ocular complications, secondary glaucoma can present as a rare complication of primary iris cysts. This case highlights the importance of serial examinations to monitor iris cysts and consideration of glaucoma as a late complication.

Bilateral anterior lens capsule ruptures in Alport syndrome: case series and literature review

Danielle Gouws — 2024-08-04

We present 3 cases of bilateral anterior lens capsule rupture, all leading to a subsequent diagnosis of Alport syndrome. Clinicians should be alert to the ocular and systemic features of Alport syndrome, especially when presented with a spontaneous rupture of the anterior lens capsule in young males. Ophthalmologists are often the first contact for patients with Alport syndrome, and a sound knowledge of the associated features will enable timely referral to other members of a multidisciplinary team required to treat such patients. Diagnosis and subsequent testing of affected families further aids in genetic counseling and early, comprehensive treatment.

Primary anaplastic extramedullary plasmacytoma in the lacrimal sac

Abha Gupta — 2024-09-30

We report the case of a 60-year-old man diagnosed with unilateral, solitary, extramedullary plasmacytoma located in the right lacrimal sac fossa, with no systemic involvement. This rare plasma cell dyscrasia is usually found in the respiratory tract. Clinical and radiological features of the mass and histopathological results are described. The patient was successfully treated with complete surgical excision. There were no clinical or radiological indications of disease recurrence at 10 months’ follow-up.