Juan Carlos Sánchez España & Roberto Secondi
We present the case of an ulcerative lesion of the eyelid as first presentation of type A lymphomatoid papulosis (LP) in a young adult. LP is a rare cutaneous lymphoproliferative disease with a risk of associated systemic or cutaneous lymphoma.
Mariel Angelou Parulan, Gangadhara Sundar, Yew Kwang Ong, Tseng Tsai Yeo, Victor Lee & Miriam Santiago Kimpo
We report a case of a 2-year-old female who presented with bilateral progressive proptosis, visual loss, nasal obstruction, and breathing difficulty. Magnetic resonance imaging revealed a large sino-orbital mass that was extending to the orbital apex and skull base. An initial diagnosis of rhabdomyosarcoma was made elsewhere on the basis of the presence of round and spindle cell tumor. Subsequent biopsy with immunohistochemical staining was positive for nuclear staining with β-catenin, shifting the diagnosis to a myofibroblastic tumor, favoring desmoid-type fibromatosis. With image guidance, near complete excision of tumor was performed by a multidisciplinary team, while respecting danger zones such as the skull base and the optic nerve. Following a recurrence over 2 months, additional excision was performed with a 6-month treatment of methotrexate and vinblastine. Desmoid tumor is a rare form of soft tissue tumor uncommonly seen in the orbital area. Although benign, it is known to be recurrent and infiltrative. Few data are known and further information will aid in the management of these tumors.
Clara J. Men, Frances Wu, Bradford W. Lee, Jonathan H. Lin, Bobby S. Korn & Don O. Kikkawa
A 5-year-old otherwise healthy girl presented to the oculoplastic service with a painless superotemporal subconjunctival mass in the left eye. Visual acuity was within normal limits, and there was no evidence of proptosis or orbital enlargement. Excision was performed to remove the anterior portion of the mass for alleviation of symptoms. On histopathological analysis, the mass was comprised of fibroadipose tissue consistent with dermolipoma and contained a hard nodule found to be a calcified tooth. In the periocular region, odontogenic choristoma (tooth) is a rare lesion, and has been reported to occur within teratomas, dermoid cysts, and displaced oral embryonic epithelium. We describe an unusual case of a tooth occurring within a sporadic dermolipoma. The clinical presentation, examination, management, and histopathology are reviewed.
Levi N. Kanu, Catherine Y. Liu, Daniel J. Oh, Peter W. MacIntosh & Pete Setabutr
We describe six patients with 12 separate episodes of self-inflicted periocular foreign body injuries, which presented to our institution recently. All patients were male, relatively young (mean 28.5 years old), incarcerated, and had significant underlying psychiatric conditions. The subjects had inserted staples (6), paperclips (2), or other small metallic wire segments (4) into the periocular region. Most cases (9/12) involved concurrent self-inflicted injury to other body parts. Ten cases involved foreign bodies inserted through the palpebral conjunctiva into the upper eyelid, while two cases involved insertion into the orbit. Identification and surgical retrieval of foreign bodies was successful in most cases (9/11) but was not attempted in one case. Self-inflicted periocular injuries, while rare, are challenging cases for which the ophthalmologist should be prepared. A multidisciplinary approach, including psychiatric assessment and treatment, is important for optimal care.
Mohammad Javed Ali, Dilip Kumar Mishra & Milind N. Naik
Urbach-Weithe syndrome is an exceedingly rare multisystem disorder characterized by pathognomonic clinical findings of multiple beaded papules along the eyelid margins and peri-ocular areas and hoarseness of voice secondary to intercellular deposition of periodic acid Schiff (PAS)-positive hyaline material. Lacrimal drainage anomalies are not well defined in this syndrome; however, punctal involvement and acquired nasolacrimal duct obstructions have been reported. We present a patient of Urbach-Weithe syndrome with bilateral punctal and peri-punctal involvement.
S. Kumar & T. Diamond
Paraneoplastic syndrome is a rare but reversible cause of non-thyroid-related extraocular muscle enlargement. We present a 71-year-old lady with diplopia, restricted eye movements, suppressed thyroid-stimulating hormone and enlargement of all extraocular muscles while on thyroxine replacement for hypothyroidism. She had distant history of metastatic breast cancer treated with chemotherapy, surgical resection and tamoxifen. She had negative anti-thyroid autoantibodies and thyroid ultrasound was not consistent with autoimmune thyroid disease. Carcinoembryonic antigen and cancer antigens 15-3, 125 and 72-4 were elevated, and whole-body positron emission tomography-computed tomography showed avid liver, left adrenal and skeletal lesions, with liver biopsy confirming breast cancer recurrence. She received prednisone and chemotherapy (letrozole, palbociclib) and achieved normalisation of eye movements and reduction in her EOME at 9-month follow-up. Our case highlights the importance of exploring paraneoplastic syndrome as a treatable cause of EOME in a patient lacking features of thyroid orbitopathy and autoimmune thyroid disease.
David S. Curragh, Srikandan Kamalarajah, Brendan Lacey, Stephen T. White, Alan A. McNab, George Kalantzis, Peter J. Dolman, Dinesh Selva & Saul N. Rajak
Purpose: We present a series of primary orbital implant replacement for cases of implant exposure to describe our experience of this one-staged surgical approach.
Methods: This study reports on a one-stage technique which involved the removal of the exposed implant or dermis fat graft (DFG) and insertion of a secondary (replacement) in the same procedure, with a variety of materials, including autologous tissue. Re-exposure in a socket where a DFG was placed was defined as a new defect in the newly epithelialized conjunctiva or dehiscence of the dermis-conjunctiva junction. All cases of primary replacement for the management of exposed orbital implant, porous and non-porous, were included, even when there were clinical signs suggestive of infection. The primary outcome was the rate of re-exposure, requiring additional surgical procedures. Infection following primary replacement was a secondary outcome.
Results: Seventy-eight patients had primary replacement for the management of an exposed orbital implant. 6.4% had re-exposure at a mean follow-up of 49.7 months (9.1% for ball implants and 4.5% for DFG). The rate of exposure was higher in those with prior signs of infection than those without (8% vs. 3.6%). Re-exposure occurred in 4.5% of cases with DFG implantation, 4.3% of cases with non-porous implants and in 20% of cases with porous implants.
Conclusion: Primary replacement for management of exposed orbital implant, porous and non-porous, has a high rate of successful outcome even in cases with presumed or confirmed infection.
Rijul S. Kshirsagar, Priscilla Q. Vu & Jonathan Liang
Purpose: Endoscopic surgeries, such as dacryocystorhinostomy (DCR), are increasingly performed for orbital and lacrimal conditions. This study describes and compares recent trends in endoscopic DCR with open, or external, DCR in the United States (US).
Methods: Medicare-Part-B National Summary data files were analyzed from 2000 to 2015 for temporal and geographic trends in endoscopic and external DCR. Medicare Physician and Other Supplier public use files detailing provider information were collected and analyzed from 2012 to 2015.
Results: Between 2000 and 2015, the number of external DCRs remained relatively unchanged (8008 to 7086, −0.7% average annual growth), while the number of endoscopic DCRs steadily increased (881 to 1674, 4.6% average annual growth). The greatest number of endoscopic DCRs were performed in the South Atlantic region, whereas the Mountain region had the greatest number per capita. From 2000 to 2015, the average payment per procedure for external DCR was $526.63, compared with $512.45 for endoscopic DCR. Of endoscopic DCRs performed from 2012 to 2015, 831 (79%) were performed by Ophthalmology, 184 (18%) were performed by Otolaryngology, and the remainder by other subspecialties.
Conclusions: The number of endoscopic DCR surgeries increased over the last 15 years while the number of external DCR surgeries remained stable and continued to surpass endoscopic procedures. While ophthalmologists perform the overwhelming majority of endoscopic DCR, otolaryngologists are performing a growing number.
Xiaodong Zheng, Tomoko Goto, Atsushi Shiraishi & Yumi Nakaoka
Purpose: To report a new method to analyze sagittal images of the upper eyelid by anterior segment optical coherence tomography (AS-OCT).
Methods: Ninety subjects equally divided into young (20–39 years), middle age (40–59 years), and elderly (60–79 years) group were studied. A sagittal image of the upper eyelid was obtained by AS-OCT (CASIA2, Tomey, Japan) and the following parameters were determined: upper eyelid thickness (UET), the distance between two parallel lines tangential to the apexes of the cornea and the upper eyelid; the upper eyelid angle (UEA) was the angle formed by a line running tangential to the apex of the cornea and a line passing from the apex of the cornea to the apex of the upper eyelid; and the cross-sectional area (CSA), the area from the edge of the eyelid to a point 6 mm above the corneal apex. All parameters were compared among groups and correlation analyses were studied between age and eyelid parameters.
Results: The UET was 2.09 ± 0.58 mm, 2.63 ± 1.04 mm and 2.83 ± 0.83 mm in the young, middle age and elderly group, respectively. The difference between the young and the elderly group was significant (P = 0.014). The elderly group had a significantly larger UEA than that of young group (P = 0.022). The elderly group also had significantly larger CSA than the other two groups (all P < 0.01). The age was significantly correlated with all eyelid parameters.
Conclusions: AS-OCT can be used as a non-invasive and quantitative method of evaluating sagittal images of the upper eyelid.
Mansooreh Jamshidian-Tehrani, Amin Nabavi, Abolfazl Kasaee, Narges Hasanpoor, Eahsan Elhami, Shervin Sharif-Kashani, Ahamd Masoumi, M. Hossein Nowroozzadeh & Ali Sadeghi-Tari
Purpose: To evaluate alterations in orbital color Doppler imaging (CDI) parameters and their correlation to disease activity and severity in patients with thyroid eye disease (TED).
Methods: Seventy-six orbits of 45 TED patients and 40 orbits of 40 normal controls were enrolled in this cross-sectional study. According to clinical activity score (CAS), patients were categorized to active (CAS ≥ 3) or inactive disease (CAS < 3). Patients were also classified as having mild, moderate or severe disease. Peak systolic velocity (PSV), end diastolic velocity (EDV), and resistance index (RI) in ophthalmic artery and central retinal artery, and maximum and minimum velocity in superior ophthalmic vein and central retinal vein were determined in all subjects.
Results: There was a significant difference in maximum velocity of superior ophthalmic vein and EDV and RI of ophthalmic artery between patients with TED and normal subjects. Superior ophthalmic vein maximum and minimum velocity and ophthalmic artery RI were significantly higher in patients with active disease than inactive cases. Disease severity did not affect the blood flow parameters independently. A cutoff point of 3.99 cm/s in superior ophthalmic vein maximum velocity yielded a sensitivity of 91.2% and specificity of 81.2% in detecting active disease.
Conclusion: Retrobulbar blood flow is altered in TED and is related to disease activity. Superior ophthalmic vein maximum velocity could be helpful in differentiation of active and inactive cases.