Adrenal Adenoma Anarchy: A Case of an ACTH-Secreting Pheochromocytoma.

Introduction. Pheochromocytomas are rare neuroendocrine tumors that arise from sympathetic adrenomedullary chromaffin tissue. Depending on the amount of catecholamines they secrete, they have variable presentations. There have been reported cases of adrenocorticotrophic (ACTH) secreting pheochromocytomas that present with severe Cushing syndrome. Here, we present a pheochromocytoma with adrenocorticotrophic hormone (ACTH) cosecretion, which due to its rarity and variable presentation, may be a diagnostic challenge. Presentation. A 64-year-old woman with history of colon cancer presented with new-onset diabetes, worsening hot flashes, and hypertension. On CT imaging she had an enlarging right adrenal nodule (1.7 cm) with 60 Hounsfield units of attenuation and no PET avidity. Biochemical evaluation showed elevated urinary and plasma metanephrines, elevated plasma cortisol levels despite dexamethasone suppression, elevated late-night salivary cortisol, and high-normal adrenocorticotrophic hormone. The patient underwent laproscopic right adrenalectomy, and pathology confirmed pheochromocytoma. Her lab abnormalities and symptoms of hot flashes and hypertension improved postoperatively. Conclusion. This case demonstrates an unusual ACTH-secreting pheochromocytoma with subtle presentation and highlights the importance of obtaining a complete biochemical evaluation of incidental adrenal adenomas.

Aggravation and deliberation over lung herniation.

A Caucasian woman aged 58 years with history of asthma and surgically repaired congenital diaphragmatic hernia presented to the emergency department (ED) with persistent cough, pleuritic chest pain, shortness of breath, in spite of recent treatment for influenza A virus. On physical examination, a large bulge was protruding from her left posterior thorax. She was found to have a large abnormal radiographic lucency on lateral chest X-ray posterior to the thoracic cavity, confirmed with chest CT to represent a large lung herniation in between the left seventh and eighth ribs. The patient was evaluated by a thoracic surgeon and offered surgical repair but ultimately decided on conservative management which to date has been ineffective.

Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) Versus Standard of Care for Postsurgical Lagophthalmos and Exposure Keratopathy: Trends in Visual Outcomes.

PURPOSE: To compare baseline characteristics and visual acuity outcomes in patients treated with prosthetic replacement of the ocular surface ecosystem (PROSE) versus other standard-of-care (SOC) treatments for postsurgical lagophthalmos and exposure keratopathy. METHODS: An institutional review board-approved, retrospective cohort study of 45 consecutive patients (53 eyes) with postsurgical lagophthalmos and exposure keratopathy following oculoplastic or skull base surgeries treated between August 2011 and August 2017 was performed. Patients treated with PROSE (22 patients, 27 eyes) were identified by referrals made to the PROSE treatment program at Weill Cornell Medical College. Patients treated with SOC treatments (23 patients, 26 eyes) were identified by International Classification of Diseases-9 and International Classification of Diseases-10 search of billing records. SOC treatments included ocular surface lubrication (artificial tears and/or punctal plugs), tape tarsorrhaphy and/or moisture chamber, or surgical correction. The primary outcome measure was best-corrected visual acuity converted to logMAR at baseline and at 1, 3, 6, and 12 months. Secondary outcome measures were subjective patient reports of improvement in vision and comfort, as well as presence of punctate epithelial erosions and/or corneal haze on slit-lamp examination before and after treatment. RESULTS: Average age for all patients was 52 ± 22 years (range: 7-87). Twenty-eight (62%) of total patients were male. Baseline corrected logMAR visual acuity was 0.58 ± 0.40 (20/76) for PROSE and 0.27 ± 0.39 (20/37) for SOC cohorts (p < 0.001). Mean number of failed prior treatments was 8.3 ± 3 for PROSE and 2.1 ± 2 for SOC (p < 0.0001). Mean difference in logMAR visual acuity for PROSE versus SOC, respectively, from baseline to 1 month was 0.33 ± 0.26 (3-line improvement) versus 0.01 ± 0.17 (no line improvement; p < 0.0001), to 3 months was 0.31 ± 0.23 (3-line improvement) versus 0.08 ± 0.30 (4-letter improvement; p = 0.0004), to 6 months was 0.31 ± 0.28 (3-line improvement) versus 0.10 ± 0.36 (1-line improvement; p = 0.02), and to 12 months was 0.32 ± 0.28 (3-line improvement) versus 0.12 ± 0.34 (1-line improvement; p = 0.01). CONCLUSIONS: Patients with postsurgical lagophthalmos and exposure keratopathy treated with PROSE are more likely to have failed a higher number of treatments and have worse initial best-corrected visual acuities than those treated with SOC. Prosthetic replacement of the ocular surface ecosystem causes rapid and substantial visual improvement within 1 month of use compared with SOC, with little change beyond this time and sustained best-corrected visual acuity at 3, 6, and 12 months after treatment.

Clinical Experience With PROSE Fitting: Significance of Diagnosis and Age.

OBJECTIVE: To compare ease of adoption of the BostonSight Prosthetic Replacement of the Ocular Surface Ecosystem device, a custom-fit scleral lens, by patients in different age and diagnosis groups. METHODS: In this prospective study, patients were categorized by age as younger than 60 or 60 years and older and by diagnosis as corneal irregularity (CI) or ocular surface disease (OSD). Ease of adoption of the scleral device was assessed by (1) number of devices and visits required to complete the fitting process, (2) time needed for device insertion and removal, (3) adaptation to the device, as assessed by daily wear time and by time needed to achieve full-time wear (defined as 8 hours per day), and (4) patients' subjective rating of ease of device insertion and removal. The length of the fitting process was also assessed. RESULTS: There was no significant difference in the number of devices and visits needed between age group younger than 60 and age group of 60 and older or between CI and OSD groups. Patients in all groups achieved full-time wear in less than 2 weeks. Average wear time per week did not differ significantly between age or diagnosis groups. Similarly, the time needed for daily insertion and removal during the fitting period, as well as patients' subjective rating of ease of device insertion and removal, did not differ between age or diagnosis categories. The length of the fitting process was significantly longer in the OSD group compared with the CI group (P<0.001); however, factors not related to ease of adoption of the scleral device may be responsible for this difference. CONCLUSIONS: Patients in both younger and older patient groups adopted the use of a scleral device with equal ease, as did patients in the CI and OSD diagnosis groups.

Treatment of Refractory Persistent Corneal Epithelial Defects: A Standardized Approach Using Continuous Wear PROSE Therapy.

PURPOSE: To evaluate continuous wear of a fluid-ventilated, gas-permeable scleral PROSE device using a standardized protocol as treatment for refractory persistent corneal epithelial defects in patients with severe ocular surface disease. METHODS: Retrospective review of eight eyes of seven consecutive patients with persistent epithelial defects refractory to traditional therapies. The standardized treatment regimen consisted of: (1) 24-hour-a-day PROSE wear until re-epithelialization was achieved, (2) brief daily device removal, cleaning, disinfection, and reservoir fluid replacement, (3) addition of a benzalkonium chloride (BAK)-free fourth-generation fluoroquinolone antibiotic drop to the reservoir, and (4) transition to long-term, daytime PROSE wear upon re-epithelialization. RESULTS: All eight eyes exhibited resolution of the persistent epithelial defect. No eyes developed microbial keratitis. Four eyes exhibited recurrences; all recurrences promptly responded to reinstitution of continuous wear. CONCLUSIONS: Continuous wear of a PROSE device, using a strictly standardized regimen, constitutes an effective, safe treatment option for refractory persistent epithelial defects.