Empty sella syndrome

Empty sella syndrome
ICD-9	253.8
DiseasesDB	31523
MeSH	D004652

WikiDoc Resources for Empty sella syndrome
Articles
Most recent articles on Empty sella syndrome Most cited articles on Empty sella syndrome Review articles on Empty sella syndrome Articles on Empty sella syndrome in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Empty sella syndrome Images of Empty sella syndrome Photos of Empty sella syndrome Podcasts & MP3s on Empty sella syndrome Videos on Empty sella syndrome
Evidence Based Medicine
Cochrane Collaboration on Empty sella syndrome Bandolier on Empty sella syndrome TRIP on Empty sella syndrome
Clinical Trials
Ongoing Trials on Empty sella syndrome at Clinical Trials.gov Trial results on Empty sella syndrome Clinical Trials on Empty sella syndrome at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Empty sella syndrome NICE Guidance on Empty sella syndrome NHS PRODIGY Guidance FDA on Empty sella syndrome CDC on Empty sella syndrome
Books
Books on Empty sella syndrome
News
Empty sella syndrome in the news Be alerted to news on Empty sella syndrome News trends on Empty sella syndrome
Commentary
Blogs on Empty sella syndrome
Definitions
Definitions of Empty sella syndrome
Patient Resources / Community
Patient resources on Empty sella syndrome Discussion groups on Empty sella syndrome Patient Handouts on Empty sella syndrome Directions to Hospitals Treating Empty sella syndrome Risk calculators and risk factors for Empty sella syndrome
Healthcare Provider Resources
Symptoms of Empty sella syndrome Causes & Risk Factors for Empty sella syndrome Diagnostic studies for Empty sella syndrome Treatment of Empty sella syndrome
Continuing Medical Education (CME)
CME Programs on Empty sella syndrome
International
Empty sella syndrome en Espanol Empty sella syndrome en Francais
Business
Empty sella syndrome in the Marketplace Patents on Empty sella syndrome
Experimental / Informatics
List of terms related to Empty sella syndrome

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Empty sella syndrome (abbreviated ESS) is a disorder that involves the sella turcica, a bony structure at the base of the brain that surrounds and protects the pituitary gland. ESS is a condition that is often discovered during tests for pituitary disorders, when radiological imaging of the pituitary gland reveals a sella turica that appears to be empty.

Classification

There are two types of ESS: primary and secondary.

• Primary ESS happens when a small anatomical defect above the pituitary gland increases pressure in the sella turica and causes the gland to flatten out along the interior walls of the sella turica cavity. Primary ESS is associated with obesity and high blood pressure in women. The disorder sometimes results in a build-up of fluid pressure inside the skull and the pituitary gland may be smaller than usual.

• Secondary ESS is the result of the pituitary gland regressing within the cavity after an injury, surgery, or radiation therapy. Individuals with secondary ESS due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as the ceasing of menstrual periods, infertility, fatigue, and intolerance to stress and infection.

Associated conditions and diagnosis

In children, ESS may be associated with early onset of puberty, growth hormone deficiency, pituitary tumors, or pituitary gland dysfunction. MRI scans are useful in evaluating ESS and differentiating it from other disorders that produce an enlarged sella.

Differential Diagnosis

• Autoimmune factors
• Cavernous sinus thrombosis
• Communicating hydrocephalus
• Congenital absence of the diaphragma sellae
• Diabetes Mellitus
• Familial
• Head trauma
• Increased intracranial pressure
• Radiation therapy complication
• Meningitis
• Pituitary adenoma
• Primary in middle-aged obese women
• Rupture of intrasellar or parasellar cyst
• Sheehan's Syndrome
• Surgery
• Vascular diseases

Differentiating Empty sella syndrome from other diseases:

Empty sella syndrome needs to be differentiated from other diseases causing hypopituitarism.

Diseases	Onset	Manifestations					Diagnosis
		History and Symptoms				Physical examination	Laboratory findings	Gold standard	Imaging	Other investigation findings
		Trumatic delivery	Lactation failure	Menstrual irregularities	Other features
Sheehan's syndrome	Acute	++	++	Oligo/amenorrhea	Symptoms of: Adrenal insufficiency Hypothyroidism	Breast tissue atrophy Decreased axillary and pubic hair growth	Pancytopenia Eosinophilia Hyponatremia Low fasting plasma glucose Decreased levels of anterior pituitary hormones in blood	Clinical diagnosis Most senitive test: Low baseline prolactin levels w/o response to TRH	CT/MRI: Sequential changes of pituitary enlargement followed by: Shrinkage and necrosis leading to decreased sellar volume or empty sella	Pituitary hormone stimulation tests (Metoclopramide and clomiphene citrate stimulation tests)
Lymphocytic hypophysitis	Acute	+/-	+	Oligo/amenorrhea	Associated with autoimmune conditions Generalized headache Retro-orbital or Bitemporal pain Mass lesion effect such as visual field defects	DI Autoimmune thyroiditis	Decreased pituitary hormones(Gonadotropins most common) Hyperprolactinemia(40%) GH excess	Pituitary biopsy: lymphocytic infiltration	CT & MRI: Features of a pituitary mass Diffuse and homogeneous contrast enhancement	Assays for: Anti-TPO Anti-Tg Ab
Pituitary apoplexy	Acute	+/-	++	Oligo/amenorrhea	Severe headache Nausea and vomiting Paralysis of eye muscles (diplopia) Changes in vision	Visual acuity defects CN palsies (nerves III, IV, V , and VI)	Decreased levels of anterior pituitary hormones in blood.	MRI	CT scan without contrast: Hemorrhage on CT presents as a hyperdense lesion MRI: If inconclusive CT	Blood tests may be done to check: PT/INR and aPTT Pituitary hormonal assay
Empty sella syndrome	Chronic	-	+	Oligo/amenorrhea	Erectile dysfunction Headache Low libido	Signs of raised intracranial pressure may be present Nipple discharge	Decreased levels of pituitary hormones in blood.	MRI	Empty sella containing CSF	Pituitary hormone stimulation tests (Metoclopramide and clomiphene citrate stimulation tests)
Simmonds' disease/Pituitary cachexia	Chronic	+/-	+	Oligo/amenorrhea	Cachexia Premature aging	Progressive emaciation Loss of body hair	Decreased levels of anterior pituitary hormones in blood.	MRI	Done to rule out any pituitary cause	Pituitary hormone stimulation tests (Metoclopramide and clomiphene citrate stimulation tests)
Hypothyroidism	Chronic	+/-	-	Oligomenorrhea/menorrhagia	Cold intolerance Constipation	Dry skin Bradycardia Hair loss Myxedema Delayed relaxation phase of deep tendon reflexes	Low T3,T4 Normal/ low TSH Rest of pituitary hormone levels WNL	TSH levels	Done to rule out any pituitary cause	Assays for anti-TPO and anti-Tg Ab FNA biopsy
Hypogonadotropic hypogonadism	Chronic	-	-	Oligo/amenorrhea	Hot flushes Energy and mood changes Decreased libido	Breast tissue atrophy Decreased maturation of vaginal mucosa	Low estrogen, testosterone High FSH/LH	FSH LH	Done to rule out any pituitary cause	Genetic tests  (karyotype) Measurement of total and free testosterone and 17-hydroxyprogesterone concentrations
Hypoprolactinemia	Chronic	-	+	-	Infertility Subfertiliy	Puerperal agalactogenesis	No workup is necessary	Decreased prolactin levels	Done to rule out any pituitary cause	Prolactin assay in 3rd trimester LH, FSH Thyrotropin and free thyroxine
Panhypopituitarism	Chronic	-	+	Oligo/amenorrhea	Polyuria Polydipsia Features of hypothyroidism and hypoadrenalism	Growth failure B/L hemianopsia Papilledema	All pituitary hormones decreased	MRI	Done to rule out any pituitary cause	Left hand and wrist radiograph for bone age
Primary adrenal insufficiency/Addison's disease	Chronic	-	-	-	Hypoglycemia Hypotension	Dehydration Hyperpigmentation loss of pubic and axillary hair	Hyponatremia with/without hyperkalemia Plasma renin activity to aldosterone ratio	Abdominal CT	Abdominal CT	Serum cortisol testing Serum ACTH testing Anti-adrenal Ab testing
Menopause	Chronic	-	+/-	Oligo/amenorrhea	Hot flashes Insomnia Weight gain and bloating Mood changes	Vaginal atrophy Loss of pelvic muscle tone	↑ FSH ↓ Estradiol and inhibin	FSH > LH	Normal	Endometrial biopsy

Diagnosis

Patient #1: MR images demonstrate an expanded and empty sella

• 
• 
• 

Treatment

Unless the syndrome results in other medical problems, treatment for endocrine dysfunction associated with pituitary malfunction is symptomatic and supportive. In some cases, surgery may be needed.

Prognosis

ESS is not a life-threatening illness.

External links

• Template:NINDS