Diverticular Perforation: A Fatal Complication to Forestall in Cushing Syndrome

Sater, Zahraa Abdul; Jha, Smita; McGlotten, Raven; Hartley, Iris R; Lakis, Mustapha El; Araque, Katherine A; Nieman, Lynnette K.

doi:10.1210/jc.2018-00829

Cited by 10 publications

(8 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…As patients with ectopic ACTH production commonly have gross elevation of cortisol levels and those with Cushing's disease potentially a long duration of elevated cortisol level, this may account for the higher incidence of intestinal rupture in our cohort of patients. However, unlike the other series of six patients reported by Sater et al [ 19 ], our six patients had much lower 24-hour UFC levels, including four with Cushing's disease, and could reflect a difference in the referral pattern.…”

Section: Discussioncontrasting

confidence: 88%

“…The immunosuppressive effects of hypercortisolism, whether endogenous or exogenous, may contribute to initial lack of containment of a perforation and impaired healing [ 9 ]. Furthermore, cortisol excess may mask classic symptoms of perforation, leading to a delay in diagnosis and treatment and a probable increase in morbidity and mortality [ 9 ] as emphasized in the recent case series [ 19 ]. Older studies have proposed that masking of symptoms occurs due to a reduction in the availability and concentration of immunoreactive cells and substances to areas of injury or inflammation, as well as from an impairment of interactions required for cellular immune defense [ 2 , 3 , 23 ].…”

Section: Discussionmentioning

confidence: 99%

“…To the best of our knowledge, there are only 14 published case reports of intestinal perforation, 12 with diverticular disease, in patients with Cushing's syndrome associated with elevated ACTH and cortisol levels [ 11 – 19 ], including 11 patients having ectopic ACTH syndrome. Moreover, all previously reported patients [ 11 – 19 ] had a greater than 10-fold elevation of 24-hour urinary free cortisol (UFC) level when measured. The purpose of our study was twofold: first to examine if intestinal perforation occurred more frequently in patients with ectopic ACTH syndrome and second if intestinal perforation only occurred in patients with a greater than 10-fold elevation of 24-hour UFC.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Intestinal Perforation in ACTH-Dependent Cushing’s Syndrome

Shahidi

Phillips

Chik

2019

BioMed Research International

View full text Add to dashboard Cite

Previous studies have linked systemic glucocorticoid use with intestinal perforation. However, the association between intestinal perforation and endogenous hypercortisolism has not been well described, with only 14 previously published case reports. In this study, we investigated if intestinal perforation occurred more frequently in patients with ectopic ACTH syndrome and in those with a greater than 10-fold elevation of 24-hour urinary free cortisol level. Of 110 patients with ACTH-dependent Cushing’s syndrome followed in two clinics in Canada, six cases with intestinal perforation were identified over 15 years. Age of patients ranged from 52 to 72, five females and one male, four with Cushing’s disease and two with ectopic ACTH production, one from a pancreatic neuroendocrine tumor and one from medullary carcinoma of the thyroid. Five had diverticular perforation and one had intestinal perforation from a stercoral ulcer. All cases had their lower intestinal perforation when the cortisol production was high, and one patient had diverticular perforation 15 months prior to the diagnosis of Cushing’s disease. As in previously reported cases, most had hypokalemia and abdominal pain with minimal or no peritoneal symptoms and this occurred during the active phase of Cushing’s syndrome. Whereas all previously reported cases occurred in patients with 24-hour urinary free cortisol levels greater than 10-fold the upper limit of normal when measured and 11 of 14 patients had ectopic ACTH production, only one of our patients had this degree of hypercortisolism and four of our six patients had Cushing’s disease. Similar to exogenous steroid use, patients with endogenous hypercortisolism also have a higher risk of intestinal, in particular diverticular, perforation and should be monitored closely for its occurrence with a low threshold for investigation and surgical intervention. Elective colonoscopy probably should be deferred until Cushing’s syndrome is under control.

show abstract

Section: Discussioncontrasting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Intestinal Perforation in ACTH-Dependent Cushing’s Syndrome

Shahidi

Phillips

Chik

2019

BioMed Research International

View full text Add to dashboard Cite

show abstract

“…In the presence of an unexplained septic state, physicians should also be aware of cortisol-induced gut perforation responsible for peritonitis that can occur without 'contracture' and prescribe abdominal imaging (121,122,123).…”

Section: (E) Infectious Complicationsmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Young

Haissaguerre

Viera-Pinto

et al. 2020

European Journal of Endocrinology

136

169

View full text Add to dashboard Cite

Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.

show abstract

“…There is a well-known association between the treatment with exogenous corticosteroids and intestinal perforation. It was also previously observed that there is an association between intestinal perforation and endogenous glucocorticoid excess due to ACTH dependent CS [ 9 , 10 ]. Interestingly, most of these patients did not have a history of pre-existing diverticular disease.…”

Section: Discussionmentioning

confidence: 84%

A challenging case of Cushing’s disease complicated with multiple thrombotic phenomena following trans-sphenoidal surgery; a case report

et al. 2021

View full text Add to dashboard Cite

Background Cushing’s syndrome occurs due to overproduction of cortisol from adrenal glands. Endogenous hypercortisolemia can occur secondary to adrenocorticotropic hormone (ACTH) dependent as well as independent causes. The presence of non-specific symptoms and signs contributes to a delay in diagnosis. Early identification and prompt definitive management is crucial. It is important to be alert about the post-operative complications including multiple thrombotic phenomena, which can add to the mortality. We report a case of Cushing’s disease in a young female managed with trans-sphenoidal surgery, followed by a challenging post-operative period complicated with multiple thrombotic phenomena, ultimately succumbed. Case presentation A 32-year-old Sri Lankan female presented with overt features of Cushing’s syndrome and diagnosed to have ACTH dependent Cushing’s disease with pituitary microadenoma. She underwent trans-sphenoidal surgery, following which she developed fatal multiple complications including diverticular rupture and ischemic colitis, needing hemicolectomy, followed by a parieto-occipital infarction. Conclusion This case highlights important and aggressive complications associated with Cushing’s syndrome giving rise to a challenging post-operative course. Diverticular rupture had been described in association with hypercortisolemia and this case adds to the existing literature. Post-operative ischemic colitis and stroke which contributed to the death of this patient could have been due to the procoagulant state associated with Cushing’s syndrome, with a high risk during the immediate post-operative period. This emphasizes the need to consider post-operative thromboprophylaxis in patients undergoing surgery for Cushing’s syndrome.

show abstract

Diverticular Perforation: A Fatal Complication to Forestall in Cushing Syndrome

Abstract: Patients with CS can develop spontaneous surgical abdomen with rapid decompensation within hours. Prompt recognition is critical in the successful treatment of these patients.

Cited by 10 publications

References 10 publications

Intestinal Perforation in ACTH-Dependent Cushing’s Syndrome

Intestinal Perforation in ACTH-Dependent Cushing’s Syndrome

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

A challenging case of Cushing’s disease complicated with multiple thrombotic phenomena following trans-sphenoidal surgery; a case report

Contact Info

Product

Resources

About