Periorbital Outcomes and Vision Risk Stratification in Treacher Collins Syndrome.

BACKGROUND: We examined ophthalmologic outcomes and periorbital management in patients with Treacher Collins syndrome (TCS). METHODS: A retrospective cohort study was performed of children with TCS treated from 2009 to 2023 at our center. Demographics and medical history were collected. Primary outcomes were periorbital findings, surgical burden, and visual acuity. Patients were stratified by risk of vision loss based on ophthalmologic findings. RESULTS: Among 50 subjects, mean follow-up was 10.5±5.9 years. Periorbital findings included downslanting palpebral fissures (100%), eyelash deficiency (70%), eyelid coloboma (54%), and significant refractive error (50%). Twenty (40%) individuals underwent 86 periorbital procedures, including lateral canthopexy (n=23 operations) and malar/zygomatic reconstruction/augmentation (n=20). Lateral canthopexies accompanied by malar fat grafting were less likely to require reoperation (0% versus 72%, p=0.004). Subjects with eyelid coloboma were more likely to have exposure keratopathy (30% versus 4%, p=0.020). Grouped by risk of vision loss, 58% were "High" risk, 18% were "Moderate", and 24% were "Low". Among 78 eyes of 39 subjects, vision was "Good" in 90%, "Fair" in 5%, and "Poor" in 6%. Vision risk grading was associated with visual acuity outcomes (n=29) (p=0.050). CONCLUSIONS: In assessing long-term ophthalmological outcomes, we noted good vision despite significant periorbital anomalies. Though most eyelid colobomas can be managed with lubrication, those threatening corneal integrity should be repaired in infancy. As timely intervention may prevent visual impairment, we recommend perinatal ophthalmologist evaluation for all children with TCS. Our proposed vision risk scale may serve as a helpful paradigm by which to contemplate vision-related issues.

The Impact of Primary Tumor Site on Survival in Mycosis Fungoides.

Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma (CTCL), but little is known about the influence of anatomic location of the primary disease site on overall survival (OS) and disease-specific survival (DSS). The purpose of this study was to examine the significance of primary tumor site on survival in MF. A search of the Surveillance, Epidemiology, and End Results (SEER) database was conducted for patients with a diagnosis of MF with a specified primary site from 2000 to 2019. Prognostic factors including demographic and tumor characteristics were examined using Cox regression models. Further research is needed to fully investigate primary disease site as a prognostic indicator, including a deeper dive into MF of all stages and subtypes.

Endobronchial Forceps Retrieval of Embedded Inferior Vena Cava Filters: Retrieval of 535 Filters at a Single Center.

PURPOSE: To report results of 16 years of using the endobronchial forceps technique to remove embedded inferior vena cava (IVC) filters. MATERIALS AND METHODS: Between January 2005 and June 2021, 534 patients (310 women and 224 men) with a mean age of 52 years (standard deviation [SD] ± 16 years) presented for complex filter retrieval of 535 tip- or strut-embedded IVC filters. Tip-embedded filters were diagnosed on rotational venography. Filters were considered strut-embedded if they were closed-cell filters with dwell times of >6 months. The filter was dissected from the IVC using rigid bronchoscopy forceps and removed through a vascular sheath. RESULTS: The endobronchial forceps technique was successful in 530 of 537 retrieval attempts on an intention-to-treat basis (98.7%); a total of 530 filters were retrieved. There were 7 failures: (a) 5 failed retrieval attempts (2 that were retrieved successfully in subsequent procedures) and (b) 2 for which retrieval was not attempted. The mean filter dwell time was 1,459 days (SD ± 1,617 days). Laser sheaths were not used for any removal. Filters included herein were 137 Celect (94 Celect and 43 Celect Platinum), 99 Günther Tulip, 72 Option (48 Option and 24 Option Elite), 68 G2, 45 G2X/Eclipse, 42 Denali, 30 OptEase, 29 Recovery, 7 Meridian, and 6 ALN with Hook filters. Thirty-four minor (6.3%) and 11 major (2%) adverse events (AEs) occurred, which did not result in permanent sequelae. CONCLUSIONS: Use of endobronchial forceps for removal of tip- and strut-embedded retrievable IVC filters is effective and has low AE rates.