Highlights of the 11th Emirates Diabetes and Endocrine (Virtual) Congress, March 4–6, 2021

<b><i>Backgrounds:</i></b> The Eleventh Diabetes and Endocrine Virtual Congress (EDEC 2021) was held on March 4–6, 2021, due to the COVID pandemic. <b><i>Objectives:</i></b> We aimed to present highlights of the congress proceedings. <b><i>Materials and Methods:</i></b> The journal appointed 3 rapporteurs to prepare this congress report. They prepared assigned sections and reviewed the rest of the manuscript, and approved its final version. <b><i>Results:</i></b> The conference contents over 3 days included a wide range of lectures by world-class experts and key opinion leaders on various topical subjects, endocrinology, diabetes care, and metabolism, in addition to original data from submitted abstracts. The clinical approach to managing a pituitary mass, contemporary management of acromegaly, and the relationship between growth hormone and neoplasia were discussed. The virtual EDEC 2021 provided a comprehensive review of topical issues concerning clinical practice and research in diabetes, endocrinology, and metabolism. Predictably, the thyroid took the scene’s center, spanning many subjects from hypothyroidism, pregnancy care, thyroid nodules, and cancer management. Rational approaches to pituitary disease and acromegaly were valuable for practicing endocrinologists. Recent advances in adrenal disease were valuable, practical, and futuristic. The reviews on bone metabolism and calcium homeostasis at large and during the COVID-19 pandemic were fascinating. On the diabetes side, emphasis on the cardiovascular outcomes, the use of newer agents when compelling indications exist, and the role of technology were precious contributions. Some of the classical questions were addressed and revisited, such as endocrinology of thalassemia, precocious puberty, and testosterone replacement. The newer and future lipid-modifying therapies were stimulating, mainly when high-risk patients are considered. Imaging, immunology, and molecular biology took their share in various presentations. <b><i>Conclusions:</i></b> The virtual EDEC 2021 provided a comprehensive review of topical issues concerning clinical practice and research in diabetes, endocrinology, and metabolism. Many new concepts were introduced in diabetes care and endocrinology, bringing the audience to the forefront of research and world-class clinical practice.

Assessing Risk of Severe Complications after Endoscopic Transnasal Transsphenoidal Surgery: A Comparison of Frailty, American Society of Anesthesiologists, and Comorbidity Scores

Objective This study aimed to improve age-independent risk stratification for patients undergoing endoscopic transnasal transsphenoidal (TNTS) approach to pituitary mass resection by investigating the associations between frailty, American Society of Anesthesiologists (ASA), and comorbidity scores with severe complications following TNTS. Design This study is a retrospective review. Setting This review was conducted utilizing the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database. Participants A total of 680 cases of TNTS identified from 2010 to 2013 were included in this study. Main Outcome Measures The modified frailty index (mFI) was calculated to quantify frailty. ASA and Charlson's comorbidity index (CCI) scores were obtained as physiologic status and comorbidity-based prognostic markers. Severe complications were separated into intensive care unit (ICU)-level complications, defined by Clavien–Dindo grade IV (CDIV) criteria, and mortality. Results Overall, 24 CDIV complications (3.5%) and 6 deaths (0.9%) were recorded. Scores for mFI (p = 0.01, R 2 = 0.97) and ASA (p = 0.04., R 2 = 0.87) were significantly correlated with CDIV complications. ASA scores were significantly correlated with mortality (p = 0.03, R 2 = 0.87), as well as independently associated with CDIV complication by multivariable regression models (odds ratio [OR] = 2.96, 95% confidence interval [CI]: 1.35–6.83, p < 0.01), while mFI was not. CCI was not significantly associated with CDIV complications or mortality. A multivariable regression model incorporating ASA had a lower Akaike's Information Criteria (AIC; 188.55) than a model incorporating mFI (195.99). Conclusion Frailty and physiologic status, as measured by mFI and ASA scores respectively, both correlate with ICU-level complications after TNTS. ASA scores demonstrate greater clinical utility than mFI scores; however, as they are more easily generated, uniquely correlated with mortality and independently associated with ICU-level complication risk on multivariable regression analysis.

Recurrent autoimmune hypophysitis treated with rituximab: a case report

Background Autoimmune hypophysitis is a rare condition that often results in enlargement of the pituitary gland and hypopituitarism due to inflammatory infiltration. Management of autoimmune hypophysitis can include long-term hormonal replacement and close control of the inflammatory pituitary mass. Mass-related symptoms in patients with autoimmune hypophysitis are treated with anti-inflammatory therapy, surgery, and/or radiotherapy. Case presentation We present a 25-year-old White man with visual field defects of the right eye, headache, and weight loss. Magnetic resonance imaging showed a sellar mass, and the patient underwent transcranial surgery. Histopathology revealed autoimmune hypophysitis with predominantly CD20 positive B-cell infiltration. Progression of visual field defects necessitated postoperatively anti-inflammatory treatment with prednisolone. Azathioprine was initiated under gradual tapering of prednisolone with stable conditions at first, but relapse followed after dose reduction. Therefore, rituximab treatment was initiated, which resulted in regression of the pituitary mass. Rituximab treatment was discontinued after 25 months. The patient has continuously been in remission for 4 years after rituximab treatment was stopped. Conclusion This case illustrates that rituximab might be an effective alternative treatment in B-cell predominant autoimmune hypophysitis.

Rathke’s Cleft Cyst Abscess—An Unusual Guest in The Sella

Abscess formation within a Rathkes’s cleft cyst (RCC) is extremely rare, particularly at a young age. We report the case of a young girl with abscess formation in RCC. A 21-year-old female presented with headache, vomiting, visual deterioration and features suggestive of hypopituitarism. She had bitemporal hemianopia with impairment of visual acuity. MRI revealed a cystic lesion in the sella with suprasellar extension and peripheral rim enhancement. On the basis of history and imaging, this was indistinguishable from more commonly encountered pituitary pathology. She underwent transsphenoidal decompression, which revealed yellowish purulent material that when cultured grew Staphylococcus epidermidis. Histological examination revealed numerous neutrophils and cyst wall lining with features characteristic of RCC. Postoperatively, she received antibiotics and replacement therapy for hypopituitarism. Three months later, she experienced deterioration in visual fields. Considering persistent disease, she underwent redo surgery which revealed similar findings. Postsurgery, pituitary MRI revealed an empty sella syndrome. Thereafter, follow-up for 1 year was stable with permanent diabetes insipidus and multiple pituitary hormone deficiency on supplementation. Although uncommon, we recommend considering RCC abscess as a differential diagnosis of a pituitary mass lesion, as predicting its presence can be difficult preoperatively. Persistent or recurrent disease is common in these cases, so timely diagnosis and adequate surgical drainage leads to lower morbidity and mortality.

Factitious Cushing’s syndrome, hypopituitarism, and self-provoked skin lesions: when the skin mirrors the soul

Summary Factitious Cushing’s syndrome (CS) is a very rare form of Münchausen syndrome. Its presentation and course are extremely heterogeneous, and diagnosis is generally challenging. We report the case of a 52-year-old woman who was initially investigated because of the occurrence of cushingoid features. Nevertheless, endocrine work-up showed very low morning plasma ACTH and serum cortisol levels. In addition, it also demonstrated central hypopituitarism and hypogonadotropic hypogonadism. Head MRI showed a small pituitary mass. Based on these results, and probably overlooking the initial clinical suspicion, general practitioner (GP) referred the patient to our Endocrine Unit for hypopituitarism. At inspection, moon face, central obesity, and bruising were evident. Multiple ulcerative skin lesions were also concentrated in the right arm and leg. Dermatology evaluation suggested that the lesions were self-provoked. For several days, the patient denied the assumption of corticosteroids, but we finally discovered that the GP’ nurse had prescribed betamethasone without the GP’s knowledge for about 2 years. In conclusion, the surreptitious assumption of corticosteroids is very rare, but the physicians should be aware that pituitary function could be impaired by high doses of corticosteroids, mimicking hypopituitarism. In these patients, a multidisciplinary approach and environmental investigation can be useful to diagnose factitious CS. Learning points Surreptitious assumption of corticosteroids can cause heterogeneous presentation, ranging from Cushing’s syndrome to multiple hypopituitarism. Suppression of ACTH and cortisol levels in a patient with cushingoid features firstly suggests surreptitious assumption of corticosteroids. A multidisciplinary approach can be extremely useful in patients with suspected factitious Cushing’s syndrome. Sometimes, to prove surreptitious assumption of corticosteroids needs environmental investigation.

Subclinical Acromegaly: to Treat or Not to Treat?

Objective Patients with acromegaly usually present with the classical signs of acromegaly, whereas patients without the specific signs or symptoms are rarely diagnosed. This unique entity can be named "subclinical acromegaly". Methods This was a retrospective study. Our study group consisted of 8 patients (6 females) with incidentally diagnosed acromegaly, most following head MRI for unrelated reasons and without the specific signs of acromegaly. Results Mean age at diagnosis was 50.1 ± 16.3 years. Baseline IGF-1 ranged between 1.27–2.08 x upper limit of normal (ULN). MRI depicted a pituitary microadenoma in 6 patients, one patient presented with an intrasellar macroadenoma and one had no visible pituitary mass. Mean calculated SAGIT clinical score was 4.6. Three patients underwent trans-sphenoidal resection; two achieved hormonal remission and one improved but did not normalize IGF-1 following surgery. Five patients (including one following surgery) were given somatostatin analogs, and three normalized IGF-1. Several patients improved clinically following treatment, reporting improvement in snoring, hypertension, or weight loss, and pituitary adenoma decreased in size in 2 patients that responded to medical treatment. Conclusions we report a series of 8 patients with very mild and subclinical acromegaly. It is uncertain whether all such patients will gain clinical benefit from treatment, but some experienced clinical improvement due to treatment.

Recurrent cavernous sinus thrombosis: a rare complication of Cushing disease

A 14-year-old girl who presented in 2017 with headache, unilateral right eye ptosis and secondary amenorrhoea had an initial workup consistent with non-functioning pituitary macroadenoma. She underwent debulking of pituitary tumour in October 2017. Postoperatively, she developed recurrent cavernous sinus thrombosis. In view of recurrent thrombosis, she was reinvestigated and was found to have adrenocorticotropic hormone-dependent Cushing. Follow-up MRI 1 year after initial presentation showed that there was structural recurrence of pituitary macroadenoma. She subsequently underwent a petrosal craniotomy for debulking of tumour. Postsurgery she remained biochemically Cushingnoid. MRI 5 months after second surgery showed an enlarging pituitary mass which was deemed inoperable. A multidisciplinary meeting discussion consensus for treatment included radiotherapy and somatostatin analogue, pasireotide. She completed 30 cycles of radiotherapy and MRI post radiotherapy showed reduction in the size of the macroadenoma. Currently, she is waiting for pasereotide initiation.