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Multivariable analysis of 63 contemporary patients diagnosed with nelson’s syndrome: A nationwide readmission database study

https://doi.org/10.1016/j.jocn.2021.07.052Get rights and content

Highlights

  • We report a contemporary series of Nelson’s syndrome (NS) and explore clinical characteristics of modern NS.

  • Modeling revealed that the absence of primary hypertension served as a significant predictor for NS.

  • NS patients report higher rates of hypoosmorarilty/hyponatremia, hypopituitarism, and sepsis compared to control.

Abstract

Introduction

Nelson’s syndrome (NS) is a rare complication involving enlargement of an adrenocorticotropic hormone (ACTH) producing tumor in the pituitary following bilateral adrenalectomy in Cushing’s syndrome. Here, we explore the epidemiology, complication profiles, and readmission statistics of 63 patients diagnosed with NS.

Methods

The Nationwide Readmission Database was retrospectively queried for all patients diagnosed with NS (n = 63) or receiving total bilateral adrenalectomy (TBA) surgery (n = 275) between 2016 and 2017. Complications, demographics, and predictive factors were queried for all patients involved. Statistical analysis used Mann-Whitney U nonparametric testing was to compare basic demographics and gaussian-fitted multivariable regression analysis with post hoc odds ratios to compare patient predictors of development of NS and complication rates between the two cohorts.

Results

We report the largest contemporary patient series of NS through a nationally-representative inpatient database and explore the clinical characteristics of modern NS patients. Modeling revealed that the absence of primary hypertension served as a significant predictor for NS when compared to the TBA control cohort (OR = 0.88; 95%CI = 0.79–0.99; p = 0.037). In addition, analysis of complications between NS and TBA cohorts revealed that NS patients have significantly higher rates of hypoosmolarity/hyponatremia (OR = 1.42; 95%CI = 1.19–1.71; p = 0.00021), hypopituitarism (OR = 1.94; 95%CI = 1.60–2.36; p < 0.0001), and sepsis (OR = 1.51; 95%CI = 1.14–2.00; p = 0.0046).

Conclusion

Contemporary NS is a rare complication of TBA, and modern cases of NS may differ significantly from cases of NS reported in the mid-1900s. As such, a thorough understanding of patient complications and predictive factors for NS are necessary to fully guide patient management in the modern era.

Introduction

Nelson’s syndrome (NS) describes the enlargement of an adrenocorticotropic hormone (ACTH) producing tumor in the pituitary following bilateral adrenalectomy in Cushing’s disease. It was first described in 1958 as the clinical triad of a corticotroph pituitary adenoma, hyperpigmentation, and elevated plasma ACTH levels [1], [2]. Since then, there have been many advances in pharmaceutical, radiosurgical, and surgical treatments for Cushing’s disease. In modern practice total bilateral adrenalectomy (TBA) is seldom necessary, thereby reducing the incidence of NS [3], [4].

As the practice of bilateral adrenalectomy has become outdated, so follows the literature on the management of Nelson’s syndrome. Modern day experience has become limited to small case series. However, we are able to pool larger sample sizes via administrative hospital databases to query all cases of NS reported in the Nationwide Readmission Databases (NRD) between 2016 and 2017. Here, we aim to provide a deeper understanding of NS within the context of modern advancements in therapeutics.

Section snippets

Data source

We use the 2016 and 2017 Healthcare Cost and Utilization Project (HCUP) NRD, which are large yearly databases that publish national information regarding inpatient demographics, diagnoses, procedures, and readmissions. Hospital admissions are de-identified and represented as unique linkages to allow for accurate patient tracking throughout the calendar year. Patient diagnoses and procedures may be queried through the use of International Classification of Diseases, Tenth Revision (ICD-10) codes

Patient demographics

The mean age of patients diagnosed with NS was 52.6 ± 19.0 years with 71.7% being female and a mean CCI of 2.5 ± 1.9. The mean age of patients with TBA who did not develop NS was 50.8 ± 16.6 years with 55.0% being female and a mean CCI of 2.5 ± 1.8 (Table 2). Pituitary surgery was performed in 13 (20.6%) patients in the NS cohort and 0 (0.0%) patients in the control cohort.

Predictors of NS

Through multivariable modeling, predictors for NS were investigated. Factors including patient frailty, malnutrition, wound

Discussion

We report the largest contemporary patient series of NS and explore the clinical characteristics of modern NS patients. Modeling revealed that the absence of primary hypertension served as a significant predictor for NS when compared to the TBA control cohort. In addition, multivariable analysis was performed to compare complication rates between patients with NS and those who received TBA without a diagnosis of NS, which revealed that NS patients have significantly higher rates of

Conclusion

Contemporary NS is a rare complication of TBA, and modern cases of NS may differ significantly from cases of NS reported in the mid-1900s. Modeling revealed that the absence of primary hypertension served as a significant predictor for NS when compared to the TBA control cohort, and that NS patients have significantly higher rates of hypoosmolarity/hyponatremia, hypopituitarism, and sepsis. Prospective multicenter studies with high patient numbers, granular complications and predictive factors,

Disclosures

There are no conflicts of interest in this study. There are also no disclosures of funding for this study. Institutional Review Board (IRB) approval was not necessary prior to analysis, as the patient cohort was obtained from a publicly available de-identified database.

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.

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