Outcome of Surgical Treatment of 200 Children With Cushing's Disease

Lonser, Russell R.; Wind, Joshua J.; Nieman, Lynnette K.; Weil, Robert J.; DeVroom, Hetty L.; Oldfield, Edward H.

doi:10.1210/jc.2012-3604

Cited by 131 publications

(180 citation statements)

References 22 publications

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“…Data from large surgical series indicate that over 90% of ACTH-adenomas are microadenomas (< 1 cm in diameter) with a mean diameter of 6 mm at time of diagnosis. 46,58 However, the small adenoma size and a reduced signal-to-noise imaging ratio at the sella-sphenoid bone-air interface contribute to the 40%-60% lack of detection of CD-associated adenomas on MR imaging at time of clinical and biochemical diagnosis. 20,46 To best inferior Petrosal sinus sampling…”

Section: Mr Imagingmentioning

confidence: 99%

“…77,92 When adenomas reach a diameter of approximately 3 mm, a surrounding microscopic tissue envelope develops that corresponds to a rim of compressed gland adjacent to the edge of the adenoma (histological pseudocapsule) that can be used to identify small adenomas and that facilitates selective enucleation of both small and large adenomas. 46,58,77 Selective adenomectomy using the histological pseudocapsule to define the boundaries of the adenoma achieves immediate and lasting remission in 97% of adult and 98% of pediatric CD patients.…”

Section: Preoperative Mr Imaging Findingsmentioning

confidence: 99%

“…20,58,98 Because of the small size of most adenomas in CD, these risks occur less likely than after surgery for larger pituitary tumors. absence of hypocortisolism after surgery Within 48 hours of surgery, most patients in remission from CD develop a glucocorticoid withdrawal syndrome associated with circulating cortisol levels of 2 mg/dl or less (see Measures of Biochemical Remission below).…”

Section: Risks Of Surgerymentioning

confidence: 99%

“…Specifically, low (below 2 mg/dl) morning serum cortisol, low urine free cortisol levels (below 20 mg/24 hours), low serum ACTH (less than 5 pg/ml), and/or low midnight salivary cortisol within the 1st week have been used to predict lasting success. 10,14,56,58 Recent data indicate that a morning serum cortisol level less than 1 mg/dl (on postoperative Day 3, 4, or 5 off of exogenous steroids) is the best predictor of lasting biochemical remission (96% positive predictive value). However, higher subnormal values do not exclude lasting remission.…”

Section: Measures Of Biochemical Remissionmentioning

confidence: 99%

“…20,58,85,92 Pituitary function is assessed within 2 weeks of surgery by measuring total T 4 and prolactin. If T 4 is similar to preoperative values and prolactin is greater than 4 ng/ml, the pituitary is considered functional.…”

Section: Postsurgical Endocrinological Managementmentioning

confidence: 99%

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Cushing's disease: pathobiology, diagnosis, and management

Lonser

Nieman

Oldfield

2017

JNS

Self Cite

150

114

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Cushing's syndrome (CS) is caused by prolonged supraphysiological levels of circulating cortisol. Cushing's disease (CD) is the most common etiology (70%-80% of CS cases) of endogenous CS. It is caused by a pituitary adenoma that secretes adrenocorticotropic hormone (ACTH), which stimulates secretion of cortisol by the adrenal glands. If not effectively treated, CD is associated with hypertension, diabetes, obesity, osteoporosis, vascular disease, and shortened life span. Successful resection of a CD-associated ACTH-secreting pituitary adenoma results in immediate biochemical remission and preservation of pituitary function. Early identification of CD by clinical findings, endocrinological evaluation, and imaging studies is critical for diagnosis and effective surgical management. endocrinological and Clinical Features endocrinological Features Normal PhysiologyInsight into the normal and pathophysiological mechanisms of control of the hypothalamic-anterior pituitaryadrenal axis is critical to understanding the pathologic, diagnostic, and therapeutic features of CD (Fig. 1). 52 Under normal physiological conditions (circadian rhythm, where cortisol levels peak in early morning and nadir late at night), systemic inflammation (cytokine), or stress (physiological or psychological), corticotropin-releasing hormone (CRH; a 41-amino acid peptide) is released from the paraventricular hypothalamic nucleus via the median eminence. From the median eminence, CRH is transported in the hypophyseal portal venous system to the pituitary gland, where it binds to the CRH receptor-1 (CRH-R1) on pituitary corticotroph cells. CRH binding to CRH-R1 receptors activates adenylate cyclase and stimulates proopiomelanocortin (POMC) gene expression in the corticotroph cells.17,66 Proopiomelanocortin preprohormone is processed into ACTH (a 39-amino acid peptide) and b-lipotropin (a 93-amino acid peptide). Products of b-lipotropin include b-endorphin and a-lipotropin. ACTH is secreted into the systemic circulation and binds to receptors in the adrenal cortex, where it stimulates production and secretion of glucocorticoids, including cortisol. 83 Under normal conditions, circulating cortisol provides physiological control of this endocrine axis by its negative feedback inhibition on hypothalamic CRH and pituitary ACTH secretion. Cushing's disease (CD) is the result of excess secretion of adrenocorticotropic hormone (ACTH) by a benign monoclonal pituitary adenoma. The excessive secretion of ACTH stimulates secretion of cortisol by the adrenal glands, resulting in supraphysiological levels of circulating cortisol. The pathophysiological levels of cortisol are associated with hypertension, diabetes, obesity, and early death. Successful resection of the CD-associated ACTH-secreting pituitary adenoma is the treatment of choice and results in immediate biochemical remission with preservation of pituitary function. Accurate and early identification of CD is critical for effective surgical management and optimal prognosis. The authors review the cu...

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Section: Mr Imagingmentioning

confidence: 99%

Section: Preoperative Mr Imaging Findingsmentioning

confidence: 99%

Section: Risks Of Surgerymentioning

confidence: 99%

Section: Measures Of Biochemical Remissionmentioning

confidence: 99%

Section: Postsurgical Endocrinological Managementmentioning

confidence: 99%

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Cushing's disease: pathobiology, diagnosis, and management

Lonser

Nieman

Oldfield

2017

JNS

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150

114

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Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing's Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

Raff

Sharma

Nieman

2014

Comprehensive Physiology

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127

105

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The hypothalamic-pituitary-adrenal (HPA) axis is a classic neuroendocrine system. One of the best ways to understand the HPA axis is to appreciate its dynamics in the variety of diseases and syndromes that affect it. Excess glucocorticoid activity can be due to endogenous cortisol overproduction (spontaneous Cushing’s syndrome) or exogenous glucocorticoid therapy (iatrogenic Cushing’s syndrome). Endogenous Cushing’s syndrome can be subdivided into ACTH-dependent and ACTH-independent, the latter of which is usually due to autonomous adrenal overproduction. The former can be due to a pituitary corticotroph tumor (usually benign) or ectopic ACTH production from tumors outside the pituitary; both of these tumor types overexpress the proopiomelanocortin gene. The converse of Cushing’s syndrome is the lack of normal cortisol secretion and is usually due to adrenal destruction (primary adrenal insufficiency) or hypopituitarism (secondary adrenal insufficiency). Secondary adrenal insufficiency can also result from a rapid discontinuation of long-term, pharmacological glucocorticoid therapy because of HPA axis suppression and adrenal atrophy. Finally, mutations in the steroidogenic enzymes of the adrenal cortex can lead to congenital adrenal hyperplasia and an increase in precursor steroids, particularly androgens. When present in utero, this can lead to masculinization of a female fetus. An understanding of the dynamics of the HPA axis is necessary to master the diagnosis and differential diagnosis of pituitary-adrenal diseases. Furthermore, understanding the pathophysiology of the HPA axis gives great insight into its normal control.

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The expanded endonasal approach in pediatric skull base surgery: A review

London

Rangel

Walz

2020

Laryngoscope Investig Oto

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Objective: Surgery of the pediatric skull base has multiple unique challenges and has seen recent rapid advances. The objective of this review is to assess key issues in pediatric skull base surgery (SBS), including anatomic limitations, surgical approaches, reconstruction techniques, postoperative care, complications, and outcomes.Data Sources: PubMed literature review.Review Methods: A review of the literature was conducted to assess the challenges, recent advances, and reported outcomes in pediatric SBS.Results: The pediatric skull base presents multiple anatomic challenges, including variable patterns of pneumatization, narrow piriform aperture width, and narrow intercarotid distance at the level of the cavernous sinus but not the superior clivus. These issues may be particularly challenging in patients less than 2 years of age. Endoscopic endonasal approaches in the sagittal and coronal plane have been applied to the pediatric skull base while open approaches may still be necessary in the setting of extensive intracranial or orbital disease, as well as disease lateral to critical neurovascular structures. While the nasoseptal flap was initially called into question for pediatric cases, it has been shown through multiple reports to be a feasible and robust reconstructive option. Complications and outcomes often depend upon the pathology. In children, response to noxious stimuli, ability to avoid Valsalva, and adherence to nasal precautions is variable. The use of lumbar drains is more common in pediatric than adult patients.Conclusion: While the pediatric skull base presents unique challenges, outcomes data support that endoscopic endonasal approaches are a pertinent surgical technique in appropriately selected patients. Level of Evidence: 3aK E Y W O R D S endoscopic endonasal approach, pediatric skull base surgery, skull base reconstruction, surgical outcomes

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Outcome of Surgical Treatment of 200 Children With Cushing's Disease

Cited by 131 publications

References 22 publications

Cushing's disease: pathobiology, diagnosis, and management

Cushing's disease: pathobiology, diagnosis, and management

Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing's Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

The expanded endonasal approach in pediatric skull base surgery: A review

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