Hiding In Plain Sight: Florid Cushing’s Disease Presenting As A Severe Extremity Cellulitis

Abstract

Disclosure: C.M. Godar: None. E.B. Noble: None. N.O. Vietor: None. T.S. Knee: None.

Background: Cushing’s syndrome may rarely present as an emergency known as Florid Cushing’s Syndrome. Patients can exhibit severe hyperglycemia, hypertension, hypokalemia, infections, and hypercoagulability. Cushing’s syndrome is a rare disease, and the constellation of clinical features can be overlooked if clinicians are not aware of the manifestations of hypercortisolism. We present the case of a patient with Cushing’s syndrome that went unrecognized with life-threatening sequelae.

Case presentation: A 52-year-old woman with well-controlled type 2 diabetes and hypertension was admitted to the hospital for severe left lower extremity cellulitis. Prior to hospitalization she had noted rapid weight gain, fatigue, weakness, mental clouding, and moodiness. She was admitted for antibiotics and surgical debridement. The infection persisted despite broad spectrum antibiotics, multiple surgical debridements, and skin grafting. She became bacteremic, and extremity amputation was considered. She additionally developed hypertensive emergency, refractory hypokalemia, and hyperglycemia to 396 mg/dL. Exam was notable for facial plethora, supraclavicular fullness, dorsocervical fat pad, and violaceous abdominal striae. Cushing’s Syndrome was suspected, and labs revealed a significantly elevated random serum cortisol of 60.5mcg/dL (Ref 6.2-19.4), significantly elevated 24H urine cortisol of 2157mcg/24H (Ref 0-50), and ACTH elevated to 81.8pg/mL (Ref 7.2-63.3) that confirmed Cushing’s Disease. MRI sella and octreotide scans did not localize a lesion. Inpatient therapy included multiple antihypertensive agents, insulin drip, aggressive potassium repletion, and initiation of ketoconazole to reduce cortisol levels. Ketoconazole was maximally dosed and she underwent surgical exploration and removal of a small pituitary microadenoma. Following surgery, she developed transient adrenal insufficiency requiring hydrocortisone and she no longer required antihypertensives, insulin, or potassium therapy. Follow up 7 years later has revealed no recurrence of Cushing’s Disease.

Discussion: Cushing’s Syndrome may present with a variety of clinical features and rarely may present as a medical emergency. Delay in diagnosis can lead to Florid Cushing’s Syndrome which carries high risk for morbidity and mortality. This case illustrates the need for clinician awareness of the features of Cushing’s Syndrome: hypertension, hyperglycemia, rapid weight gain, cushingoid exam features, hypokalemia, hirsutism, virilization, infection, and/or hypercoagulable state. Severe hypercortisolism was responsible for this patient’s refractory infection, and if not controlled, she likely would have endured a lower extremity amputation. Rapid detection with elevated random serum and/or urine cortisol and treatment with a cortisol-lowering agent is critical and lifesaving.

Presentation: Thursday, June 15, 2023

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Day 11, Cushing’s Awareness Challenge 2016

Blue and Yellow – we have those colors on ribbons, websites, T-shirts, Cushing’s Awareness Challenge logos and even cars.

This is the yellow PT cruiser I had rented for the Columbus, OH meeting in 2007.  I didn’t ask for yellow.  That’s just what the rental company gave me.  Somehow, they knew.

This meeting is the one when we all met at Hoggy’s for dinner although some of us travelers stayed at this hotel.

I’m the one in yellow and blue.

Later in 2007, I bought my own truly Cushie Car.  I even managed to get a butterfly on the tags.

So, where did all this blue and yellow come from, anyway?  The answer is so easy and without any thought that it will amaze you!

In July of 2000, I was talking with my dear friend Alice, who ran a wonderful menopause site, Power Surge.  We wondering why there weren’t many support groups online (OR off!) for Cushing’s and I wondered if I could start one myself and we decided that maybe I could.

This website (http://www.cushings-help.com) first went “live” July 21, 2000.  It was a one-page bit of information about Cushing’s.  Nothing fancy.  No message boards, no blogs, no wiki, no image galleries…  Certainly no Cushing’s Awareness Challenges.

I didn’t know much about HTML (yet!) but I knew a little from what Alice had taught me and I used on my music studio site.  I didn’t want to put as much work <COUGH!> into the Cushing’s site as I had on the music studio site so I used a now defunct  WYSIWYG (What You See Is What You Get) web editor called Microsoft FrontPage.

One of their standard templates was – you guessed it! – blue and yellow.

TaDa!  Instant Cushie color scheme forever.  Turns out that the HTML that this software churned out was really awful and had to be entirely redone as the site grew.  But the colors stuck.

Now, in this day of mobile web browsers and people going online on their cellphones, the website is being redone yet again.  But the colors are still, and always, blue and yellow.

Related Factors of Delirium After Transsphenoidal Endoscopic Pituitary Adenoma Resection

Highlights

  • Aim to identify independent risk factors for postoperative delirium after pituitary adenoma surgery.
  • Select matched subjects by Propensity Score Matching to reduce potential biases caused by variables.
  • Enhance preoperative communication to minimize the occurrence of delirium, for patients at high risk of postoperative delirium.
  • Minimize surgery duration and general anesthesia, optimize perioperative sedation regimen.
  • Reducing unnecessary or excessive protective physical restraints.

Abstract

Objectives

The primary aim of this study is to explore the factors associated with delirium incidence in postoperative patients who have undergone endoscopic transsphenoidal approach surgery for pituitary adenoma.

Methods

The study population included patients admitted to Tianjin Huanhu Hospital’s Skull Base Endoscopy Center from January to December 2022, selected through a retrospective cohort study design. The presence of perioperative delirium was evaluated using the 4 ‘A’s Test (4AT) scale, and the final diagnosis of delirium was determined by clinicians. Statistical analysis included Propensity Score Matching (PSM), χ2 Test, and Binary Logistic Regression.

Results

A total of 213 patients were included in this study, and the incidence of delirium was found to be 29.58 % (63/213). Among them, 126 patients were selected using PSM (delirium:non-delirium = 1:1), ensuring age, gender, and pathology were matched. According to the results of univariate analysis conducted on multiple variables, The binary logistic regression indicated that a history of alcoholism (OR = 6.89, [1.60–29.68], P = 0.010), preoperative optic nerve compression symptoms (OR = 4.30, [1.46–12.65], P = 0.008), operation time ≥3 h (OR = 5.50, [2.01–15.06], P = 0.001), benzodiazepines for sedation (OR = 3.94, [1.40–11.13], P = 0.010), sleep disorder (OR = 3.86, [1.40–10.66], P = 0.009), and physical restraint (OR = 4.53, [1.64–12.53], P = 0.004) as independent risk factors for postoperative delirium following pituitary adenoma surgery.

Conclusions

For pituitary adenoma patients with a history of alcoholism and presenting symptoms of optic nerve compression, as well as an operation time ≥3 h, enhancing communication between healthcare providers and patients, improving perioperative sleep quality, and reducing physical restraint may help decrease the incidence of postoperative delirium.

Introduction

In clinical practice, patients admitted to the intensive care unit (ICU) during the postoperative period after endoscopic transsphenoidal tumorectomy of pituitary adenoma often experience episodes of delirium. According to a recent retrospective analysis conducted at a single center, the incidence of postoperative delirium among these patients was found to be 10.34 % (n = 360) [1]. Delirium is a common complication following neurosurgery, characterized by acute distraction, confusion in thinking, sleep disorders, and cognitive decline. The incidence of delirium in admitted patients after neurosurgery has been reported to be 19 %, with a range of 12 % to 26 % depending on clinical features and the methods used for delirium assessment [2], [3], [4]. The incidence of postoperative delirium varied across different types of neurosurgical diseases, as reported in a meta-analysis [2]. Specifically, the incidences were 8.0 % for patients with neurological tumors, 20 % for those undergoing functional neurosurgery, 24.0 % for microvascular decompression patients, 19.0 % for traumatic brain injury patients, 42.0 % for neurovascular patients, and 17.0 % for the mixed population undergoing neurosurgery procedures. Furthermore, the incidence rates of delirium in intensive care units (ICUs), general wards, or both combined were found to be 24.0 %, 17 %, and 18 %, respectively.

The aforementioned issue not only leads to prolonged hospital stays and increased healthcare costs, but also exerts a significant impact on patient consciousness and cognitive function. Therefore, early and accurate identification of delirium in post-neurosurgical patients is crucial. However, due to frequent co-occurrence with primary brain injury, related complications can also lead to cognitive impairment or even decreased levels of consciousness, posing challenges for timely and precise identification of delirium. Currently, the primary focus lies in the prevention of delirium within the neurosurgical ICU setting. Early identification and comprehensive pre-surgical assessment are positively significant measures for preventing postoperative delirium occurrence [5], [6]. In this study, a retrospective cohort design was employed to collect pertinent data and statistically analyze the incidence of delirium, as well as its associated influencing factors, among patients admitted to the neurosurgical ICU for pituitary adenoma treatment. And now it is reported as follows.

Section snippets

Patient selection

A retrospective cohort study design was employed to select 213 pituitary adenomas admitted to the Skull Base and Endoscopy Center of Tianjin Huanhu Hospital between January 2022 and December 2022 as the subjects for investigation, with a review of their medical records. The mean age was (50.03 ± 15.72) years, ranging from 20–79 years old (Fig. 1). Informed consent was obtained from all patients or their families, ensuring compliance with the requirements stated in the Declaration of Helsinki.

Inclusion criteria

a.

Propensity score matching

The present study enrolled a total of 213 patients with pituitary tumors, among whom 63 exhibited symptoms related to delirium while the remaining 150 did not. Consequently, the incidence rate of delirium was determined to be 29.58 % in this cohort of patients admitted to the intensive care unit following pituitary tumor surgery. The univariate analysis revealed no significant differences in age (≥65y old, 23.8 % vs. 23.3 %, P = 0.940) and gender (male, 49.2 % vs. 56.7 %, P = 0.318) between the

Background of perioperative delirium in transsphenoidal endoscopic pituitary adenoma surgery

The pituitary gland is situated within the sella turcica and comprises two distinct components. The anterior pituitary, known as the adenohypophysis, functions as an endocrine organ responsible for secreting growth hormone, prolactin, adrenocorticotropic hormone, thyrotropin, follicle-stimulating hormone and luteinizing hormone. On the other hand, the posterior pituitary, referred to as the neurohypophysis, serves as a direct extension of the hypothalamus and acts as a storage site for

Conclusions

To enhance the evaluation of postoperative patients at risk of delirium, it is anticipated that optimizing doctor-nurse-patient communication and minimizing unnecessary and indiscriminate protective measures will mitigate the incidence of delirium following pituitary tumor surgery. This study is a single-center prospective study conducted at our institution, which has several inherent limitations. A large-scale multicenter prospective study is anticipated to further investigate the associated

Limitations

There are multiple factors that influence the occurrence of delirium following neurosurgery. This retrospective study solely focused on analyzing and comparing general patient data, medical history, and potential perioperative factors contributing to delirium, without considering any other known or unknown variables in this analysis. The pituitary gland functions as a neuroendocrine organ involved in the regulation of neuroendocrine processes. Changes in hormone levels following surgery for

Funding

All authors affirm that this study was conducted without any fund support from external organizations.

CRediT authorship contribution statement

Shusheng Zhang: Writing – original draft, Methodology, Investigation, Formal analysis, Data curation, Conceptualization. Yanan Chen: Writing – original draft, Investigation, Data curation. Xiudong Wang: Validation, Supervision, Project administration, Methodology, Conceptualization. Jun Liu: Software, Formal analysis, Data curation. Yueda Chen: Validation, Supervision, Methodology, Investigation. Guobin Zhang: Writing – review & editing, Validation, Supervision, Methodology, Conceptualization.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

References (21)

There are more references available in the full text version of this article.

Day 10, Cushing’s Awareness Challenge

This is one of the suggestions from the Cushing’s Awareness Challenge post:

What have you learned about the medical community since you have become sick?

This one is so easy. I’ve said it a thousand times – you know your own body better than any doctor will. Most doctors have never seen a Cushing’s patient, few ever will in the future.

If you believe you have Cushing’s (or any other rare disease), learn what you can about it, connect with other patients, make a timeline of symptoms and photographs. Read, take notes, save all your doctors notes, keep your lab findings, get second/third/ten or more opinions.  Make a calendar showing which days you had what symptoms.  Google calendars are great for this.

This is your life, your one and only shot (no pun intended!) at it. Make it the best and healthiest that you can.

When my friend and fellow e-patient Dave deBronkart learned he had a rare and terminal kidney cancer, he turned to a group of fellow patients online and found a medical treatment that even his own doctors didn’t know. It saved his life.

In this video he calls on all patients to talk with one another, know their own health data, and make health care better one e-Patient at a time.

7a4e4-maryoonerose

Day 9, Cushing’s Awareness Challenge

Uh, Oh – I’m nearly a day late (and a dollar short?)…and I’m not yet sure what today’s topic will be.  I seem swamped by everything lately, waking up tired, napping, going to bed tired, waking up in the middle of the night, traveling, work, starting all over again…and my DH was recently diagnosed with cancer which makes everything more hectic and tiring.

It’s been like this since I was being diagnosed with Cushing’s in the mid-1980’s.  You’d think  things would be improved in the last 29 years.  But, no.

My mind wants things to have improved, so I’ve taken on more challenges, and my DH has provided some for me (see one of my other blogs, MaryOMedical).

Thank goodness, I have only part-time jobs (4 0f them!), that I can mostly do from home.  I don’t know how anyone post-Cushing’s could manage a full-time job!

I can see this post morphing into the topic “My Dream Day“…

I’d wake up refreshed and really awake at about 7:00AM and take the dog out for a brisk run.

Get home about 8:00AM and start on my website work.

Later in the morning, I’d get some bills paid – and there would be enough money to do so!

After lunch, out with the dog again, then practice the piano some, read a bit, finish up the website work, teach a few piano students, work on my church job, then dinner.

After dinner, check email, out with the dog, maybe handbell or choir practice, a bit of TV, then bed about 10PM

Nothing fancy but NO NAPS.  Work would be getting done, time for hobbies, the dog, 3 healthy meals.

Just a normal life that so many take for granted. Or, do they?

me-tired