Implementation of thyroid eye disease registry in Iran: rationale and research protocol.

BACKGROUND: To describe the implementation of a registry system for patients with thyroid eye disease (TED) in Iran to obtain more information about its nature, prevalence, and annual incidence, as well as extend insight into the etiology, pathogenesis, and eventually make an accurate prognosis of different medical or surgical treatment methods. METHODS: After receiving approval from the Disease Registry Committee of Iran University of Medical Sciences (IUMS) in 2019 and the Ministry of Health and Medical Education (MOHME) in 2020, the protocol was introduced in three consecutive phases at regional, provincial and national levels. The establishment of a registry committee in Rassoul Akram Hospital, one of the medical centers affiliated to IUMS, was the first step to organizing the registry project's main core. The steering committee included six subgroups of required subject fields. The members are experts in developing a guideline, providing a new dataset, drawing an outline for the next steps, and structuring user-friendly software through several panel discussion meetings. The data is collected from clinical and para-clinical/imaging findings, laboratory evaluations, and their selected treatment strategy, retrospectively and prospectively. RESULTS: The purpose is to broaden our knowledge about the profile of TED; accordingly, data related to patients' demographics, thyroid gland disease (status, duration, treatments, and function tests), general medical and ocular history, along with visual/ocular exams resulting TED status are collected and recorded in a 2- language software. The web-based software system is accessible at https://orc.iums.ac.ir . To maintain data security, prioritized user access was defined for different members. Furthermore, diverse methods, such as employing trained staff and utilizing software validation rules, were implemented to control data quality in every step of data collection, entry, and registration. Medical records of retrospective subjects were also evaluated and entered after accuracy verification. CONCLUSION: Iran's TED registry provides practitioners with comprehensive data on natural history and phenotype variations in clinical features and outcomes. It facilitates patient recruitment and, consequently, earlier diagnosis on a large scale which helps improve treatment and quality of life for patients.

Long-Term Results of Oral Propranolol Treatment Protocol for Periocular Infantile Hemangioma: Should There Be Any Contraindication?

PURPOSE: The authors report long-term results of the oral propranolol treatment protocol for periocular infantile hemangioma regardless of conventional indications. METHODS: This is a retrospective study (2008-2018) on children with propranolol treatment protocol for periocular infantile hemangioma and last follow-up (FU) of ≥4 months after treatment and age of >24 months. After systemic evaluation, it was started with 0.5 and increased by 0.5 every 3 days to reach 2 mg/kg/day. If there was no good response, it would be gradually increased up to 3 mg/kg/day. Tapering (0.5 mg/kg/day every 4 weeks) was started when patients were on treatment for ≥6 months and ≥12 months old and there was no change in the infantile hemangioma size for ≥3 months. Rebound growth was based on the parents' report and would be treated by increasing the dose to at least 2 mg/kg/day for 3 months. FU was either in-person or via social media. Change in periocular infantile hemangioma size on the photos was scored by 3 masked observers (visual analog scale) and presented as excellent (≥50% reduction), good (1%-49%), fair (no change), and failure (enlarged). RESULTS: Forty-three patients were included. Orbital involvement was in 49%, ptosis in 58%, and risk of amblyopia in 63%. Mean treatment, tapering, and FU duration were 37, 13, and 74 months, respectively. Mean age at presentation, start of propranolol treatment protocol, tapering, stop, and last FU were 1.5, 5, 29, 42, and 78 months, respectively. Twenty-seven patients received 2, seven 2.5, and nine 3 mg/kg/day. Mean visual analog scale significantly increased from 43% (FU1) to 92% (last FU) when 97.6% of patients showed an excellent response. The remaining skin lesions were observed in 35%. No side effect was reported. CONCLUSIONS: Long-term results of the propranolol treatment protocol for periocular infantile hemangioma showed an excellent response in 97.6% of patients with no side effects. A residual skin lesion was observed in 1/3 of the patient.

Minced Free Fat Graft Versus Pedicle Fat Flap to Efface Orbital Rim Hollow in Lower Blepharoplasty.

BACKGROUND: Lower eyelid fat flap repositioning during transconjunctival lower blepharoplasty (TCLB) is a widely accepted technique for preventing postoperative tear trough deformity. Its drawbacks include retraction, limited volume, transcutaneous fixation, and a complex insertion technique. The minced lower eyelid fat graft was introduced to overcome these drawbacks. OBJECTIVES: The aim of this study was to compare the outcome of preperiosteal pedicle fat flaps with minced free fat graft in TCLB. METHODS: Participants who underwent the pedicle fat flap procedure from April 2019 to April 2020 (Group A) and the minced free fat graft from May 2020 to May 2021 (Group B) with at least 6 months of follow-up were included. Subjective (pain, infraorbital numbness) and objective (chemosis, residual skin wrinkles, hyperpigmentation, tear trough deformity, bumps) postoperative outcomes, satisfaction (visual analogue scale score) at the last follow-up, and reoperation rates were compared. RESULTS: There were 142 participants (94% females) with a mean age of 48.4 years (range: 21-71) and a follow-up of 8.2 months (range: 6-36). There were 73 participants in Group A and 69 in Group B, with no significant differences in age (P = .6), sex (P = .7), or follow-up (P = .3). In addition to TCLB, Groups A and B had simultaneous upper eyelid and eyebrow procedures (53% vs 49%, P = .2), lateral canthal plication (77% vs 83%, P = .4), and pinch skin excision (82% vs 88%, P = .3). Groups A and B did not have significant differences in postoperative numbness (6.8% vs 1.4%, P = .2), chemosis (11% vs 10%, P = 1), skin wrinkles (12.3% vs 8.7%, P = .6), hyperpigmentation (1.4% in both groups), bumps (2.7% vs 7.2%, P = .3), tear trough deformity (0.0% in both groups), satisfaction score (97.7% vs 98.1%, P = .4), or reoperation rate (12.3% vs 5.8%, P = .2). CONCLUSIONS: The desired aim of fat redistribution in TCLB seems to be equally achievable with minced fat graft and pedicle fat flap techniques.

Endoscopic-assisted Orbitotomy for Obliteration of Orbital Arteriovenous Fistula Refractory to Endovascular Techniques.

Orbital arteriovenous fistula is a rare acquired disorder. The coincidence of arteriovenous fistula with lymphaticovenous malformation is even rarer. The optimal treatment, thus, is controversial. Surgical approaches vary widely, with associated pros and cons. The purpose of this case report is to describe an orbital arteriovenous fistula in a 25-year-old man with congenital fronto-orbital lymphaticovenous malformation, refractory to endovascular techniques, which was later successfully ablated by a direct endoscopic-assisted orbital approach.

Targeted chemotherapy in retinoblastoma: A step forward from patient survival to globe survival.

BACKGROUND/ OBJECTIVES: Despite being the most common intraocular malignancy in childhood, there is a substantial disparity between developing and developed countries in terms of patient and globe survival in retinoblastoma (Rb). The current study intends to determine patient and globe survival before and after the introduction of the new targeted treatment modalities in a developing country. METHODS: Medical records of 350 patients (516 eyes) with retinoblastoma referred to a tertiary referral center for Rb in Tehran, Iran, were reviewed. In order to compare patient and globe survival before and after the availability of the new treatment modalities, including intra-arterial and intravitreal chemotherapy, the patients were divided into group 1 (2001-2007) and group 2 (2008-2018) based on the calendar period of diagnosis. RESULTS: Two-hundred-twenty-three eyes of 149 patients and 293 eyes of 201 patients were categorized into groups 1 and 2, respectively. The 5-year patient survival was 97% across the current survey, and the overall survival rate was 96% in group 1 and 99% in group 2 (P = 0.08). Overall, 50% of eyes with retinoblastoma underwent enucleation, which was the primary in 63% (116/184) of the unilateral and 30% (99/322) of the bilateral cases. Primary enucleation was significantly lower in group 2 (35%) in contrast to group 1 (50%) (P < 0.001). In addition, globe survival improved significantly in the International Classification of Retinoblastoma Groups D (17% in group 1 vs. 66% in group 2, P < 0.001) and E (1% in group 1 vs. 23% in group 2 P < 0.001) during the two timelines. In enucleated eyes, despite the increased rate of prelaminar involvement in group 2 (13% vs. 2% in group 1, P = 0.003), the rate of high-risk histopathologic findings was similar between the two groups. CONCLUSION: Similar to developed countries, the application of new targeted treatment modalities, including intra-arterial and intravitreal chemotherapy, has been associated with significantly improved globe survival in Rb patients. However, it should be noted that even with the availability of these novel treatment options, the decision for on-time enucleation should not be deferred.

Impact of COVID-19 on the patient referral pattern and conversion rate in the university versus private facial plastic surgery centers.

PURPOSE: To compare the number of referrals and conversion rate between the pandemic and pre-pandemic period. METHODS: The number of referrals and conversion rate between the 10-month pandemic (March-December 2020) and pre-pandemic (March-December 2019) were evaluated in the two university (mainly non-cosmetic) and private (mainly cosmetic) facial plastic surgery centers. Demographics and monthly number and type (cosmetic and non-cosmetic) of the referrals and surgeries were recorded from the both and cosmetic facial injections (botulinum toxin and filler) and the source of referrals (web- and non-web-based) from the private center. The conversion rate was a ratio of the number of the surgeries to the number of referrals. RESULTS: The number of referrals declined by 7.7% in the private center which was significantly higher for the non-cosmetic (26%) than the cosmetic (0.5%) referrals. It was 32% in the university center. The private center conversion rate significantly (P < 0.001) decreased for both the cosmetic (60%) and non-cosmetic (82%) procedures. It was not significantly different between the cosmetic (65%) and non-cosmetic (58%) procedures in the university center. However, the number of cosmetic facial injections (11%) and the web-based referral source (4%) increased. The recovery was better for the number of referrals (better in the private center) than the conversion rate. CONCLUSION: The fall in the conversion rate was statistically significant in the private center. While the number of referrals recovered to almost the pre-pandemic level, the conversion rates, despite recovery, remained at a lower level at the end.

A Systematic Review on the Role of Antioxidants in Thyroid Eye Disease.

Purpose: To systematically review the role of antioxidants in management of patients with thyroid eye disease (TED). Methods: A literature search of the electronic databases was performed without restrictions on the date of publication till the end of March 2021, using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Clinical trials, case-control studies, cohorts, case series, case reports, and experimental (including in vitro) studies in the English language were included. The primary outcome in human studies was improvement in severity, activity scores, and/or quality of life scores. There was a decrease in the level of H2O2-dependent oxidative stress, Hyaluronic acid release, reactive oxygen species, cell proliferation, or antifibrotic/antiproliferative actions in the in vitro studies. Results: Out of 374 initially screened articles, 157 studies were selected, the full texts of 82 were reviewed, and 14 papers were finally included. There were 4 clinical and 10 in vitro studies from 1993 to 2018. While ß-carotene, retinol, Vitamin E, Vitamin C, melatonin, resveratrol, N-acetyl-l-cysteine, and quercetin showed some efficacy in in vitro studies; allopurinol, nicotinamide, pentoxifylline, and selenium (Se) were effective in both clinical and experimental reports. Se was the only recommended antioxidant based on one high-level randomized controlled trial. Conclusion: While different antioxidants could potentially be effective in the management of TED, no strong recommendation for any or combination of antioxidants could be made to be implemented in the daily practice.

Prevalence of Masked versus Unmasked Blepharoptosis in Subjects Seeking for Blepharoplasty and or Eyebrow Lift.

PURPOSE: To report the prevalence of unmasked versus masked in subjects seeking for eyelid and eyebrow rejuvenation. METHODS: This retrospective study included subjects (>18 years) who were seeking for blepharoplasty and/or eyebrow lift procedures in the year 2020. They were either having No ptosis, Unmasked or Exposed ptosis (complaining of simultaneous droopy eyelid, which was confirmed on examination), or Masked or Hidden ptosis (not complaining of simultaneous droopy eyelid, but its presence was revealed on the examination). Margin reflex distance 1 (MRD1) and levator function (LF) were recorded. Associated ptosis was defined as MRD1 of ≤3 mm and regarded as mild (3 and 2.5 mm), moderate (2 and 1.5 mm), and severe (≤1 mm). RESULTS: Included were 1401 subjects with a mean age of 47.2 years. The prevalence of masked and unmasked ptosis was 31% (90% unilateral) and 15.6% (59% bilateral), respectively. A significantly younger mean age (45.6 vs. 59.7 years), higher MRD1 (2.6 vs. 1.9 mm), and better LF (13.3 vs. 11.3 mm) were observed in the masked vs. unmasked ptosis group. More than 80% of the subjects with masked ptosis presented with indirect signs. A majority of both groups (68.8% of masked and 63.5% of unmasked ptosis) were mild to moderate. CONCLUSION: Almost half of the subjects seeking for eyelid and eyebrow rejuvenation had either masked (2/3) or unmasked (1/3) associated ptosis. Masked ptosis was mostly unilateral and presenting with indirect signs of ptosis.

Optic Nerve Head Vessel Density Changes from Graves' Disease without TED to TED Dysthyroid Optic Neuropathy: Does Optic Nerve Head Ischemia Play a Role?

PURPOSE: To compare the retinal peripapillary capillary (RPC) vessel density (VD) between 5 groups of patients on the spectrum of thyroid eye disease (TED). METHOD: This is a prospective comparative study looking at 5 groups of patients who were consecutively included from January 2018 to March 2021. They were composed of: Healthy volunteers (39 eyes and 20 subjects), patients with Graves' disease without TED (26 eyes and 13 patients), mild TED (28 eyes and 14 patients), moderate-severe TED (30 eyes and 17 patients), and TED with dysthyroid optic neuropathy (DON) (21 eyes and 12 patients). Ocular and periocular examination, visual field indices, RPC-VD (with optical coherence tomography angiography), and retinal nerve fiber layer, and macular ganglion cell complex thickness were recorded. RESULT: An initial insignificant (0.5 < p < 0.9) rise in the peripapillary-VD (pp-VD) and whole image-VD (wi-VD) from the healthy subject to the Graves' disease without TED was followed by a significant (p = 0.001) fall in RPC-VD in different severity grades of the TED. Paired comparison between the 5 groups showed that the statistically significant fall from the Graves' disease group occurred in the moderate-severe and DON groups (0.001 ≤ p ≤ 0.04). No variable significantly affected the VD (0.08 ≤ p ≤ 0.9). A lower wi-VD and pp-VD were significantly (0.001 ≤ p ≤ 0.009) correlated with the impaired optic nerve functional and structural tests. The sensitivity and specificity of wi-VD (81% and 76%) and pp-VD (69% and 71%) for detecting the DON were statistically significant (p < 0.001). CONCLUSION: Despite an insignificant rise in the wi- and pp-VD from the healthy volunteers to the patients with Graves' disease without TED, VD showed a declining trend in the course of patients with TED, which was statistically significant in the moderate-severe TED and DON groups.

Long-term Results of Palpebral Fissure Transfer With No Lower Eyelid Spacer in Chronic Progressive External Ophthalmoplegia.

PURPOSE: To evaluate the long-term outcomes of the palpebral fissure transfer (PFT) technique without lower eyelid spacer in patients with chronic progressive external ophthalmoplegia (CPEO). DESIGN: Retrospective interventional case series. METHODS: Consecutive patients with CPEO with PFT surgery (2006-2017) and a minimum follow-up of 24 months were included. The PFT included simultaneous levator resection or frontalis sling (based on the levator function and Bell's phenomenon) and lower eyelid retractor recession without spacer. Primary outcome measures included the change in margin reflex distance 1 (MRD-1), MRD-2, and palpebral fissure height (PFH) in the course of time. Potential predictors of keratopathy were also explored. RESULTS: There were 32 patients (64 eyelids) with the mean age and follow-up of 34.1 years (range 15-56 years) and 37.9 months (range 24-72 months), respectively. Mean MRD-1 significantly increased from -0.9 mm to 3.4 mm in postoperative weeks 1 and 2 and 1.8 mm at the last follow-up. Mean MRD-2 significantly decreased from 5.6 mm to 3.7 mm in postoperative weeks 1 and 2 and 5 mm at the last follow-up. Eyes with frank lower eyelid retraction (MRD-2 >5 mm) gained more reduction in MRD-2 compared with those with no sclera show (-0.9 mm vs -0.3 mm, P < .001). Reoperation was performed in 9 eyelids for under- (n = 5) and overcorrection (n = 4). Persistent keratopathy was observed in 3 eyes (4.5%). CONCLUSION: Lower eyelid retractor recession without spacer yields significant protective corneal coverage at the critical early postoperative period when the risk of keratopathy is highest.

Short- and Long-term Patient Satisfaction and Complications in 650 Endoscopic Forehead Lift Procedures.

PURPOSE: The aims are to compare short- versus long-term patient satisfaction and report temporary versus permanent complications in 650 patients with endoscopic forehead lift procedure (EFL). METHODS: This is a retrospective study on all of the consecutive patients with EFL. Patients with previous trauma and surgery and less than 2 years follow up were excluded. Short- (6 months) and long-term (≥2 years) patient satisfaction (visual analog score [VAS], 0-100) were recorded. Patients' perspectives on temporary versus permanent complications were also documented. RESULTS: Mean age and follow up were 46.4 and 7.1 (2-13) years, respectively. Long-term satisfaction (79.9) was significantly lower than the short term (96.6). The long-term satisfaction decreased in 95.7%, increased in 2.7%, and remained the same in 1.6% of the patients. Intraoperative skin laceration occurred in 3 patients (0.5%). Mean time of forehead numbness recovery was 2.3 months. Temporary complications were itching (13.7%), headache (6.3%), unilateral facial nerve palsy (5.8%), acne (3.2%), and remained staples (1.7%). Permanent complications included undercorrection (7.1%), alopecia (4.2%), forehead irregularities (2.3%), surprised look (2.2%), incision site complications (2%), and glabellar depression (0.9%). Reoperation (1.2%) was performed for undercorrection and alopecia. While short-term satisfaction was significantly lower in patients with temporary facial nerve paresis, long-term satisfaction was lower in patients with undercorrection and reoperation. CONCLUSION: A high satisfaction scores of 96.6 and 80 were observed in the short- and long-term follow up after the EFL. Frequency of temporary and permanent postoperative complications was 30.3% and 15.8%. Reoperation rate was 1.2%.