Hamad M. Alsulaiman , Dalal R. Fatani , Osama Al Sheikh , Sahar Elkhamary , Azza Maktabi , Hind M. Alkatan & Silvana Artioli Schellini
The authors are describing two rare cases of dacryops involving the Krause accessory lacrimal glands and affecting the orbit. In both cases, the lesion was located in the inferior fornix, with no preceding trauma, surgery or trachomatous conjunctival scarring. The first case involved a clear orbital extension with evidence of optic nerve compression. The second case extended from the anterior orbit to the limbus. Both had surgical removal with good outcome. As Krause gland dacryops are similar to other orbital cysts, clinical, imaging and histopathologic evaluation are essential to confirm the final diagnosis.
Sung Eun Kim , Ji Hyun Kim & Suk-Woo Yang
A liposarcoma of the orbit is extremely rare and a metastatic liposarcoma of the orbit is even rarer. Histologically, metastatic tumors frequently resemble the appearance of the primary lesion or may be less differentiated. In the present paper, we report on a case of a metastatic liposarcoma of the orbit and brain in which dedifferentiated transformation occurred from the primary tumor. The primary liposarcoma was a myxoid liposarcoma in the thigh, which had been resected 6 years ago. The metastatic orbital liposarcoma was poorly differentiated compared to the primary tumor and the tumor in the brain was even more poorly differentiated and anaplastic than the tumor in orbit. The tumor in the orbit and brain was completely removed and postoperative radiotherapy and chemotherapy were performed.
Sruti S. Akella , Jinesh Shah & Anne Barmettler
Sinus pericranii (SP) is a rare malformation involving aberrant connections between the dural venous sinuses and extracranial veins. Classically considered congenital pathology, there are reported instances of post-traumatic SP in adults. Very rarely, the upper eyelid is involved and the resulting ptosis can obstruct the visual axis. In these cases, surgical removal may be warranted. Because of the high risk of massive intraoperative hemorrhage, careful preoperative identification and diagnostic workup is essential. The authors report a patient with new onset, asymmetric eyelid ptosis referred for evaluation, and subsequently diagnosed with SP. Through a multi-disciplinary assessment, deferring surgical intervention was determined to be appropriate management. The lesion was monitored clinically, and at 6 months follow-up, there were no noted adverse outcomes.
Jin Kyun Oh , Davinder K. Chandhoke & Roman Shinder
Shoaib Ugradar, Daniel B. Rootman, Mohammed Ali Khan
A teenaged boy presented with a 2-week history of a red left eye and extraocular movement restriction with a history of contact lens wear but denied a history of trauma. His visual acuity was 20/50, and a tender red nodule on the inferotemporal sclera (Fig A) with anterior vitritis were found. Magnetic resonance imaging revealed enhancement of the lateral and inferior recti and adjacent fat (Fig B). An intraocular foreign body (IOFB) embedded in the inferotemporal retina (Fig C) was found on fundoscopy and was later removed following pars plana vitrectomy, resolving the intraocular and orbital inflammation. Final visual acuity was 20/25. Gross examination confirmed the IOFB to be an eyelash (Fig D). (Magnified version of Fig A–D is available online at http://www.aaojournal.org).
Montolío-Marzo, Santiago; González-Valdivia, Hugo; Casas-Gimeno, Estér; Sebastian-Chapman, Laura; Prat-Bartomeu, Joan
To describe the management of dermoid cysts in a pediatric referral hospital.
Retrospective review of 115 patients with pathological diagnosis of dermoid cyst in a pediatric referral hospital between 2003 and 2019.
One hundred fifteen patients, 51 (44.3%) males, and 64 (55.7%) females were retrospectively reviewed. There were fifty-one (44.3%) right eyes and 64 (55.7%) left eyes. Mean age at surgery was 39.15 months (5.6 months–16.4 years). One hundred four (90.4%) lesions were superficial, and 11 (9.6%) were deep. Quadrant location was 63 (54.8%) superior-temporal, 45 (39.1%) superior-nasal, 4 (3.5%) inferior-temporal, and 2 (1.7%) in the nasal inferior quadrant. Most patients only had aesthetic concerns, but 3 (2.61%) showed lacrimal drainage obstruction symptoms, 2 (1.74%) had proptosis, and 1 case spontaneously drained to the cutaneous surface. Imaging was performed in 51 (44.3%) patients. Regarding to their radiodensity, 71.9% had low density content, 28.1% high density, and only 1 patient showed full liquid content; 10.9% showed heterogeneous content; 53.1% showed bone remodeling. Every patient but 1 underwent surgery for a barely accessible asymptomatic retrobulbar cyst. Nine cysts (7.8%) were breached during surgery. Three recurrences were found (2.6%), but only 1 was related to intraoperative breach.
Dermoid cysts are the most common benign periorbital tumors in the pediatric population. Imaging is required for evaluation of lesions in atypical locations, deep or fixed to underlying tissue. Surgical removal is the gold standard of treatment. Multidisciplinary approach may be required in the most complex cases. After surgery, few complications and recurrences were found in our series.
Allister S. Lee, Princeton W. Y. Lee, Anthony Allworth, Tai Smith & Timothy J. Sullivan
To report the spectrum of fungal infections involving the orbit encountered in an Australian subtropical population with respect to presentation, host risk factors, involved pathogens, treatment and outcomes.
A retrospective chart review was performed on all adult patients with orbital mycosis treated by the senior author (TJS) from 1986 to 2017 in a tertiary setting.
Thirty cases of fungal infection involving the orbit were included in this case series. Of these, 26 patients had invasive disease and four patients had non-invasive disease. Causative organisms included mucormycosis (16), aspergillus (8) and other fungi (7). Common risk factors included haematological disorders or malignancy, neutropenia, corticosteroid use and diabetes mellitus. Mucormycosis in three immunocompetent patients was caused by Apophysomyces elegans. Orbital apex syndrome was observed in approximately one third of patients at initial ophthalmological assessment. Amphotericin B was used in most cases of mucormycosis, while there was a more varied spectrum of anti-fungal use in other fungal infections. Seven patients with mucormycosis proceeded to orbital exenteration with a survival rate of 43%. No patients with other orbital fungal infections were exenterated.
Orbital mycoses are not only opportunistic but true pathogenic infections. While initial symptoms may be varied, the development of orbital apex syndrome should raise suspicion for this condition, regardless of patient immune status or age. Survival and visual outcomes are often poor with invasive disease. Multidisciplinary team management with early orbital specialist involvement is essential.
Vickie Lee, Parizad Avari, Ben Williams, Petros Perros, Colin Dayan
The Royal College of Physicians (RCP) and Thyroid Eye Disease Amsterdam Declaration Implementation Group (TEAMeD-5) have the common goal of improving access to high quality care for thyroid eye disease (TED). The TEAMeD-5 programme recommends all patients with moderate-to-severe TED should have access to multidisciplinary clinics (MDT) with combined Ophthalmology and Endocrinology expertise.
The British Oculoplastic Surgery Society represents oculoplastic surgeons who usually lead TED care in the UK. A two-stage survey of the membership was conducted to ascertain current practice of existing resources.
Seventy percent (45/65) of respondents in Survey 1 were aware of current RCP guidance, but only 49% (22/45) rated it as a good means of improving access to comprehensive TED service. Sixty percent (39/65) of respondents are working in a multidisciplinary TED clinic with co-location of ophthalmologists and endocrinologists. Care for TED appears not to be provided in a multidisciplinary context in up to 31% (20/65). Thirty five (54%) of the respondents rated their relationship with endocrinology colleagues as good. Best practice guidelines recommend routine quality of life assessments but only 6/28 (21%) of respondents use this modality in current practice. Six percent (4/65) of areas appear not to be using intravenous steroids. In many areas (25%, 16/65), second-line immunosuppression is provided in a different trust and in 8% (5/65), it appears not to be used at all.
This survey is a ‘snapshot’ of current TED management in the UK and findings suggest scope for improvement. We recommend a framework for more robust collaboration across specialties and propose standards endorsed by multidisciplinary stakeholder societies.
Aylin Garip Kuebler, Caroline Wiecha, Lukas Reznicek, Annemarie Klingenstein, Kathrin Halfter, Siegfried Priglinger & Christoph Hintschich
To evaluate the effectiveness of steroid-pulse therapy and three-wall orbital decompression in patients with dysthyroid optic neuropathy (DON).
Twenty-five patients (46 eyes) with a diagnosis of DON between 2008 and 2015 were included in the study. The first group (7 patients, 16 eyes) consisted of patients with a steroid-pulse treatment only and the second group (18 patients, 30 eyes) included patients with medical and surgical decompression.
Twenty patients were female; five patients were male. After the diagnosis of DON, all patients were treated with steroid-pulse treatment (intravenous 500 mg prednisolon twice/week for 4 weeks, 250 mg twice/week for 2 weeks) as a first-line treatment (medical decompression). In 30 eyes (18 patients) out of 46 eyes, (25 patients) an orbital decompression was needed to preserve the optic nerve function. In those therapy-resistant cases (surgical decompression group), the orbital decompression led to statistically significant improvements in best-corrected visual acuity (BCVA), protan and tritan value of the color vision (p = 0.007, p < 0.0001, p = 0.019, respectively, comparison of first visit to last visit).
According to our data, the mild cases of DON with better initial visual acuity (in our case series mean: 0.3 logMAR) seem to respond well to steroid treatment. However, therapy-resistant cases with an impaired initial BCVA (in our case series, mean: 0.6 logMAR) seem to need the surgery to preserve the optic nerve function. In conclusion, this retrospective study confirms the effectiveness of surgical decompression in therapy-resistant cases of DON.
Paul D. Langer, Francesco P. Bernardini
The unexpected, catastrophic emergence of the novel coronavirus has forced medical specialties the world over to adapt quickly to a new medical reality, and ophthalmology has been no exception. In fact, each subspecialty within ophthalmology has made its own short-term adaptations during the epidemic so as to continue caring for patients while keeping transmission of the virus to a minimum.
As we write this perspective in mid-April 2020, the United States is at or near the peak of the crisis, whereas Italy is approximately 2 weeks past its peak. At this point in the outbreak, our field, oculofacial plastic and orbital surgery, has adjusted to the pandemic in the following ways, with some predicted long-term effects resulting from these changes.