Successful surgical intervention to ascending aortic aneurysm with dissection risk in a Patient with Cushing's syndrome and pituitary operation

Giant aortic aneurysm is defined as aneurysm in the aorta greater than 10 cm in diameter. It is a rare finding since most patients will present with complications of dissection or rupture before the size of aneurysm reaches that magnitude. Etiological factors include atherosclerosis, Marfan’s syndrome, giant cell arteritis, tuberculosis, syphilis, HIV-associated vasculitis, hereditary hemorrhagic telangiectasia, and medial agenesis. Once diagnosed, prompt surgical intervention is the treatment of choice. Although asymptomatic unruptured giant aortic aneurysm has been reported in the literature, there has not been any case of asymptomatic giant dissecting aortic aneurysm reported in the literature thus far. We report a case of giant dissecting ascending aortic aneurysm in an asymptomatic young male who was referred to our institution for abnormal findings on physical exam.

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SUN-186 Case Report: Mifepristone Taper in an Individual with Equivocal Cushing’s Syndrome Screening Tests

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.104 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Christopher Martin ◽

David Perez-Molinar ◽

Tyler C Drake

Keyword(s):

Cushing’S Syndrome ◽

Adrenocorticotropic Hormone ◽

Hemorrhagic Stroke ◽

Surgical Intervention ◽

Cushing's Syndrome ◽

Screening Tests ◽

Imaging Studies ◽

Bridge Therapy ◽

Work Up ◽

Dexamethasone Suppression

Abstract We present a 75-year-old male evaluated by the inpatient endocrine service during an admission for hemorrhagic stroke. Approximately 1 year prior to this admission he was started on mifepristone therapy for presumed Cushing’s Syndrome. Initial Cushing’s work-up was equivocal: 1 mg dexamethasone suppression cortisol level of 1.9 and midnight salivary cortisol 167 ng/dl. Random ACTH measurement was not obtained as part of this initial evaluation. Review of prior imaging studies did not demonstrate obvious culprit pituitary nor adrenal lesions. Mifepristone induced hyperaldosteronism, thyroid dysfunction and adrenal insufficiency were demonstrated presumably secondary to cortisol receptor antagonist induced up-regulation of adrenocorticotropic hormone and cortisol. We describe our experience stopping mifepristone and performing re-evaluation. We propose a tapering protocol in the setting of potentially untreated Cushing’s Syndrome and suggest use as a bridge therapy to surgical intervention rather than destination therapy.

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A CASE OF CHRONIC DISSECTING AORTIC ANEURYSM DUE TO TAKAYASU'S ARTERITIS ASSOCIATED WITH CUSHING'S SYNDROME

Nihon Naika Gakkai Zasshi ◽

10.2169/naika.63.163 ◽

1974 ◽

Vol 63 (2) ◽

pp. 163-168 ◽

Cited By ~ 2

Author(s):

Jun MIYAWAKI ◽

Toshio OZAWA ◽

Noboru Handa ◽

TAKUO Fujita ◽

Masaki YOSHIKAWA ◽

...

Keyword(s):

Aortic Aneurysm ◽

Cushing’S Syndrome ◽

Takayasu’S Arteritis ◽

Cushing's Syndrome ◽

Takayasu's Arteritis ◽

Dissecting Aortic Aneurysm

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Low-dose etomidate for the management of severe hypercortisolaemia in different clinical scenarios: a case series and review of the literature

Therapeutic Advances in Endocrinology and Metabolism ◽

10.1177/2042018819825541 ◽

2019 ◽

Vol 10 ◽

pp. 204201881982554

Author(s):

Agnieszka Łebek-Szatańska ◽

Karolina M. Nowak ◽

Wojciech Zgliczyński ◽

Elżbieta Baum ◽

Agnieszka Żyłka ◽

...

Keyword(s):

Cushing’S Syndrome ◽

Surgical Intervention ◽

Therapeutic Option ◽

Case Series ◽

Cushing's Syndrome ◽

Medical Intervention ◽

Infectious Complications ◽

Mortality And Morbidity ◽

Clinical Scenarios ◽

Lowering Drug

Background: Severe Cushing’s syndrome (SCS) is associated with acute cardiovascular, metabolic and infectious complications. It is considered an emergency, requiring an immediate diagnosis, together with a broad spectrum of supportive and hypocortisolaemic treatments. Surgical intervention, aimed at removing the source of cortisol or adrenocorticotropic hormone (ACTH), is the optimal treatment in most cases of Cushing’s syndrome. However, in hypercortisolaemic states, surgical intervention has high rates of perioperative mortality and morbidity. Oral adrenal steroidogenesis inhibitors, even if more effective in combination, are not always efficient enough or well tolerated. Despite their common use, a more potent, parental, immediate, and thus life-saving, therapy is necessary. Methods: The authors present three different clinical scenarios of etomidate treatment in patients hospitalized in the third reference endocrinological centre in Poland between 2016 and 2017. Results: Patients with Cushing’s disease, ectopic Cushing’s syndrome and adrenocortical carcinoma presented with severe hypercortisolaemia and exacerbated cortisol-dependent comorbidities. In these three cases, etomidate acted as an accurate, well tolerated and effective cortisol-lowering drug for several days or even months. Patients were monitored in a general ward setting, and no side effects of the therapy were observed. Conclusions: In doses far lower than those used for anaesthesia, etomidate works as a useful cortisol-lowering therapy in patients intolerant to or unable to take oral medications. Additionally, if urgent, the most potent and effective medical intervention is necessary, and clinicians should be aware of such a therapeutic option.

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Presentation of ACTH Independent Cushing’s as Severe Insulin Resistance During Pregnancy

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.307 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A152-A152

Author(s):

Sobia Faisal ◽

Manmeet Kaur ◽

Ashish Samat

Keyword(s):

Insulin Resistance ◽

Pregnant Woman ◽

Cushing’S Syndrome ◽

Adrenal Mass ◽

Surgical Intervention ◽

Cushing's Syndrome ◽

Average Weight ◽

Diagnostic Dilemma ◽

Severe Insulin Resistance ◽

Free Cortisol

Abstract Introduction: Cushing’s syndrome is very rare in pregnancy with around 200 cases reported in literature. It remains a diagnostic dilemma as pregnancy itself is a hypercortisolemic state. Several clinical characteristics of Cushing’s syndrome such as insulin resistance and hirsutism may overlap with normal variants of pregnancy and therefore leading to a missed diagnosis. We hereby present a case of a young pregnant woman with severe insulin resistance, with workup consistent with Cushing’s syndrome. Clinical Case: This was a 27-year-old pregnant woman with DM type 2 diagnosed about 7 years ago. During her pregnancy she was started on insulin and was later admitted to the hospital at 32 weeks for inpatient management of pre-eclampsia and uncontrolled hyperglycemia. Endocrine was consulted for severe insulin resistance and persistent hyperglycemia. On exam patient was noted to be obese with facial plethora, frontal balding and significant facial hirsutism. During the pregnancy she had noted significant weight gain with substantial worsening of frontal balding and hirsutism. Her current pregnancy was spontaneous but was after many years of trying to conceive. Prior pregnancy 8 years ago was uncomplicated with no history for GDM at that time. Review of recent pre-pregnancy imaging revealed left adrenal mass measuring 3.5 cm with 40 HU which measured around 2 cm 8 years ago. Biochemical work up revealed elevated 8 am cortisol at 35.4 ug/dL (ref range 6.2 - 19.4) with suppressed ACTH <5 pg/mL (6 – 50). Testosterone was high at 299 ng/dL (8 – 48) and DHEAS was low normal at 37 mcg/dL (18 – 391). Plasma and 24 hour urine metanephrine collection results were normal. 24-hour urine free cortisol was measured and was elevated at 628.5 mcg/24(4.0–50.0). Midnight salivary cortisol was 0.87 mcg/dL (<OR=0.09). Patient was managed conservatively with plan for surgical intervention post-delivery. Pregnancy was complicated by PPROM at 35 weeks with subsequent cesarean section. Baby was of average weight but developed mild hyperbilirubinemia which required short NICU stay. On reevaluation at 4-week postpartum period, repeat biochemical workup revealed elevated cortisol of 22.5 mcg/dL, ACTH <5 pg/ml, elevated 24-hour urine cortisol 163.9 mcg/24h with appropriate urinary creatinine. Testosterone level was improved postpartum to 9 ng/dL. DHEAS was 39 mcg/dL. Repeat imaging with CT with adrenal protocol revealed left adrenal mass measuring 2.3 cm with 20 HU with absolute washout of 79%. She was started on ketoconazole is now awaiting surgical intervention. Conclusion: We suspect that this patient may have had pregestational subclinical Cushing’s syndrome which eventually progressed to Cushing’s syndrome during pregnancy. Although this is a rare finding during pregnancy, workup for Cushing’s should be considered in pregnant women with severe insulin resistance, hirsutism, hypertension and pre-eclampsia.

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Abstract 14920: Predictors of Adverse Outcomes Following Elective Ascending Aortic Aneurysm Repair: A Nationwide Cohort Study

Circulation ◽

10.1161/circ.142.suppl_3.14920 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Sebastian E Beyer ◽

Kamal R Khabbaz ◽

Alec Schmaier ◽

Eric A Secemsky ◽

Brett Carroll

Keyword(s):

Aortic Aneurysm ◽

Hospital Mortality ◽

Surgical Intervention ◽

Adverse Outcomes ◽

Complication Rates ◽

Probit Regression ◽

Ascending Aortic Aneurysm ◽

Procedural Volume ◽

Elective Repair ◽

Secondary Outcomes

Introduction: Elective surgical intervention for ascending aortic aneurysm (AA) is performed when the risk of a future aortic catastrophe outweighs the risk of surgery; however, the data of complication rates following elective repair are limited. Methods: We analyzed adults >18 years of age hospitalized for elective ascending AA surgical intervention in the 2016 and 2017 Nationwide Readmissions Databases. The primary outcome was in-hospital mortality. Secondary outcomes were acute ischemic stroke (AIS), acute myocardial infarction (AMI) and non-elective 30-day readmission. Bayesian lasso probit regression models were used to identify independent predictors of primary and secondary outcomes. Results: Among 4,073 patients hospitalized for elective surgical repair, 28.9% were female. Mean age was 63.0 ± 12.4 years. Concomitant aortic valve surgery was performed in 67%. Overall, 72 (1.8%) died during the initial hospitalization (Figure A). Predictors of in-hospital mortality are shown in Figure B. Of patients alive at discharge, 482 (11.8%) were readmitted within 30 days, with a mortality rate of 2.3% during readmission. Predictors of non-elective 30-day readmission included non-urban localization (Change in probability: 32.5%, 21.7 - 45.7%), chronic liver disease (18.1%, 11.4 - 27.0%), and chronic neurological disorder (15.9%, 12.1 - 20.1%). Main causes of readmissions were arrythmias (20.3%), procedural complications (14.5%), and peri-, endo-, or myocarditis (9.3%). While procedural volume varied between one and 68 procedures / institution, it was not associated with in-hospital mortality, AIS, AMI, or 30-day readmission (p>0.05 for all). Conclusions: One in 25 patients will have a significant complication or die following elective ascending aortic repair and 1 in 8 will be readmitted within 30 days. The risk of surgery compared to the risk of a future aortic catastrophe should be thoroughly assessed prior to recommendation of an ascending AA repair.

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An unusual combination of persistent silent truncus arteriosus Type-II with ascending aortic aneurysm

Journal of Surgical Case Reports ◽

10.1093/jscr/rjaa216 ◽

2020 ◽

Vol 2020 (7) ◽

Author(s):

Mohamed Farah Yusuf Mohamud ◽

Mohamed Abdullahi Mohamud ◽

Abdinafic Mohamud Hussein ◽

Ismail Gedi İbrahim

Keyword(s):

Aortic Aneurysm ◽

Surgical Intervention ◽

Pulmonary Arteries ◽

Truncus Arteriosus ◽

Cardiac Anomaly ◽

Type Ii ◽

Shortness Of Breath ◽

Ascending Aortic Aneurysm ◽

Congenital Cardiac Anomaly ◽

Vascular Occlusive Disease

Abstract Truncus arteriosus (TA) is a relatively uncommon cyanotic congenital cardiac anomaly accounting for 1.3% of all congenital cardiac malformations. TA associated with an ascending aortic aneurysm is an extremely rare congenital cardiovascular abnormality. A 15-year-old male presented with shortness of breath and cough for 2 weeks. Radiological examinations showed that the ascending aorta and main pulmonary arteries originate from a single truncus with ascending aortic aneurysm. TA has a poor prognosis if remain unrepaired and surgical intervention is necessary to avoid severe pulmonary vascular occlusive disease.

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Impending rupture of aortic aneurysm eroding skin and sternum: a rare condition

Asian Cardiovascular and Thoracic Annals ◽

10.1177/0218492319833280 ◽

2019 ◽

Vol 27 (4) ◽

pp. 307-309

Author(s):

Pramote Porapakkham

Keyword(s):

Aortic Aneurysm ◽

Chest Wall ◽

Surgical Intervention ◽

Rare Condition ◽

Anterior Chest Wall ◽

Ascending Aorta ◽

Ascending Aortic Aneurysm ◽

Rare Presentation ◽

Impending Rupture ◽

History Of

A rare presentation of ascending aortic aneurysm eroding into the anterior chest wall and skin is described. Only a few reports of this lethal condition associated with luetic disease have been published previously. A 72-year-old man with a history of blunt chest injury subsequently developed a saccular aneurysm of the ascending aorta protruding out of the anterior chest wall. He was successfully treated with a surgical intervention.

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