Avascular necrosis of femoral heads post-adrenal surgery for Cushing’s syndrome: a rare presentation

Summary Avascular necrosis (AVN) is a rare presenting feature of endogenous hypercortisolism. If left untreated, complete collapse of the femoral head may ensue, necessitating hip replacement in up to 70% of patients. The majority of the described patients with AVN due to endogenous hypercortisolaemia required surgical intervention. A 36-year-old female, investigated for right leg pain, reported rapid weight gain, bruising and secondary amenorrhoea. She had abdominal adiposity with violaceous striae, facial plethora and hirsutism, atrophic skin, ecchymosis and proximal myopathy. Investigations confirmed cortisol excess (cortisol following low-dose 48h dexamethasone suppression test 807nmol/L; 24h urinary free cortisol 1443nmol (normal<290nmol)). Adrenocorticotrophic hormone (ACTH) was <5.0pg/mL. CT demonstrated subtle left adrenal gland hypertrophy. Hypercortisolaemia persisted after left adrenalectomy. Histology revealed primary pigmented micronodular adrenal disease. Post-operatively, right leg pain worsened and left leg pain developed, affecting mobility. MRI showed bilateral femoral head AVN. She underwent right adrenalectomy and steroid replacement was commenced. Four months after surgery, leg pain had resolved and mobility was normal. Repeat MRI showed marked improvement of radiological abnormalities in both femoral heads, consistent with spontaneous healing of AVN. We report a case of Cushing’s syndrome due to primary pigmented nodular adrenocortical disease, presenting with symptomatic AVN of both hips. This was managed conservatively from an orthopaedic perspective. Following cure of hypercortisolaemia, the patient experienced excellent recovery and remains symptom free 4 years after adrenalectomy. This is the first report of a favourable outcome over long-term follow-up of a patient with bilateral AVN of the hip, which reversed with treatment of endogenous hypercortisolaemia. Learning points AVN of femoral head can be a presenting feature of hypercortisolism, both endogenous and exogenous. Rarely, treatment of hypercortisolaemia can reverse AVN without the need for orthopaedic intervention. Primary pigmented nodular adrenal disease is a rare cause of ACTH-independent Cushing’s syndrome.

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Abstract #125: Oncocytic Adrenal Tumor Presenting as Cushing’s Syndrome-Rare Presentation of a Rare Tumor

Endocrine Practice ◽

10.1016/s1530-891x(20)42481-4 ◽

2015 ◽

Vol 21 ◽

pp. 14

Author(s):

Subodh Banzal ◽

Sonal Banzal ◽

Abhishek Singhai

Keyword(s):

Cushing’S Syndrome ◽

Adrenal Tumor ◽

Cushing's Syndrome ◽

Rare Tumor ◽

Rare Presentation

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Dexmedetomidine for Conscious Sedation in Bilateral Inferior Petrosal Sinus Sampling

Research & Innovation in Anesthesia ◽

10.5005/jp-journals-10049-0015 ◽

2016 ◽

Vol 1 (2) ◽

pp. 61-62

Author(s):

Neeraj Barnwal ◽

Raylene Dias ◽

Rahul Mamde

Keyword(s):

General Anesthesia ◽

Cushing’S Syndrome ◽

Conscious Sedation ◽

Cushing's Syndrome ◽

Inferior Petrosal Sinus ◽

The Novel ◽

Rare Presentation ◽

Inferior Petrosal Sinus Sampling ◽

Ectopic Adrenocorticotropic Hormone ◽

Acth Secreting

ABSTRACT Cushing's syndrome in an acromegalic patient is a very rare presentation. To differentiate a Cushing's disease from Cushing's syndrome due to ectopic adrenocorticotropic hormone (ACTH) secreting tumors, inferior petrosal sinus sampling (IPSS) is required. Acromegaly patients have associated airway abnormalities posing a challenge to administration of anesthesia. Traditionally, most IPSS was done under general anesthesia. But now it is being recognized that general anesthesia for this procedure has its own implications and hence conscious sedation is being used for this purpose. We describe our experience with the novel agent dexmedetomidine for conscious sedation in this procedure. How to cite this article Barnwal N, Dias R, Mamde R. Dexmedetomidine for Conscious Sedation in Bilateral Inferior Petrosal Sinus Sampling. Res Inno in Anesth 2016;1(2):61-62.

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Commentary on article: Adrenal crisis in treated patients with Cushing’s syndrome

Acta Endocrinologica ◽

10.1530/eje-19-0475 ◽

2019 ◽

Vol 181 (4) ◽

pp. C13-C15 ◽

Cited By ~ 1

Author(s):

David J Torpy

Keyword(s):

Cushing’S Syndrome ◽

Withdrawal Syndrome ◽

Cushing's Syndrome ◽

Individual Variability ◽

Adrenal Crisis ◽

High Dose ◽

Adrenal Surgery ◽

Acth Secretion ◽

Risk Period ◽

Surgery Rates

A study has examined the rates of adrenal crises in patients treated with pituitary or adrenal surgery. Rates were substantial (approximately 9 per 100 patient years), perhaps representing suppression of corticotrope ACTH secretion and deprivation of normal corticotrope number postoperatively. Hormone withdrawal syndrome may have contributed to the rates of apparent adrenal crises given the definition used. Higher rates were seen in patients given relatively high dose glucocorticoids postoperatively in one of the two centres where patients were treated – perhaps some of the patients in the high dose centre had longer periods of corticotrope suppression from exogenous glucocorticoids, increasing the risk period for adrenal crises. The question of optimal glucocorticoid dose and weaning rate after cure of Cushing’s syndrome remains a balance between weaning at a rate sufficiently rapid to allow resumption of normal corticotrope function thereby preventing adrenal crises and providing sufficient glucocorticoid support to avoid hormone withdrawal syndrome or even precipitating an adrenal crisis, in the vulnerable 4–6 month period after successful surgery. There is likely to be considerable inter-individual variability in optimum glucocorticoid dose and weaning rate so that close clinical and biochemical monitoring is currently a practical approach.

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