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Pituitary Apoplexy

Updated : August 30, 2023





Background

Pituitary apoplexy is a rare but serious medical condition resulting from bleeding or decreased blood supply to the pituitary gland, often in a existing pituitary adenoma. This can cause sudden death of the gland and is characterized by acute ischemic infarction.

The first recorded case of hemorrhage associated with pituitary tumors was reported in 1898 by Pearce Bailey, but the term pituitary apoplexy to describe necrosis and to bleed into pituitary tumors was not introduced until 1950.

Prompt identification and assessment are crucial in many cases of pituitary apoplexy, which is considered a medical and surgical emergency. Treatment usually involves a combination of medical and surgical interventions, depending on the underlying cause and severity of the condition.

Epidemiology

The occurrence of pituitary apoplexy is a medical condition that affects individuals with pituitary adenoma, a type of tumor located in the pituitary gland. Reports on the incidence of pituitary apoplexy vary greatly, ranging from 1.5% to 27.7%, in patients with pituitary adenoma. However, these reports often do not differentiate between symptomatic and asymptomatic cases.

When only symptomatic cases are considered, the incidence of pituitary apoplexy is estimated to be around 10%. Nevertheless, if considering the detection of non-symptomatic intratumoral hemorrhage through neuroimaging studies, the incidence increases to 26%. The occurrence of pituitary apoplexy in pituitary adenomas is relatively rare, estimated at 0.2% annually. Nevertheless, tumors larger than 10 cm and exhibiting rapid growth have a higher risk of apoplexy.

Moreover, pituitary apoplexy is observed more frequently in men than women, with a male-to-female ratio approaching 2:1. In terms of age range, most patients diagnosed with pituitary apoplexy fall between 37-58 years old. It is important to note that the incidence rates of pituitary apoplexy are based on reported cases, and there may be instances where pituitary apoplexy goes undiagnosed or misdiagnosed.

Anatomy

Pathophysiology

Pituitary apoplexy is a rare and life-threatening medical condition that occurs when the pituitary gland is bleeding or necrosis. The pituitary gland is a small, pea-sized gland located at the base of the brain, which produces and secretes hormones that regulate various bodily functions.

Pituitary apoplexy can cause a sudden onset of severe headaches, visual disturbances, and hormonal imbalances. The pathophysiology of pituitary apoplexy is complex and not fully understood, but it is thought to involve a combination of vascular, mechanical, and biochemical factors.

The following is a detailed explanation of the pathophysiology of pituitary apoplexy:

  • Vascular factors: Pituitary apoplexy is often associated with a sudden increase in blood flow to the pituitary gland, resulting in the rupture of blood vessels and bleeding into the gland. This increase in blood flow may be caused by various factors, such as hypertension, use of anticoagulant medications, or pregnancy. In addition, the pituitary gland has a rich blood supply from the internal carotid artery, which makes it vulnerable to ischemic damage or thrombosis of the blood vessels.
  • Mechanical factors: Pituitary apoplexy can also occur due to mechanical stress on the pituitary gland, such as trauma or surgery. For example, a blow to the head can cause the pituitary gland to shift within the sella turcica (a bony structure that houses the pituitary gland), leading to the compression of blood vessels and tissue necrosis.
  • Biochemical factors: Certain conditions or factors can also predispose individuals to pituitary apoplexy by altering the biochemical environment of the pituitary gland. For instance, pituitary adenomas can cause hormonal imbalances that affect the blood vessels and cause tissue damage. In addition, patients with underlying medical conditions such as diabetes mellitus, sickle cell anemia, or hypercoagulable states are at higher risk for pituitary apoplexy due to changes in blood glucose levels or blood viscosity.

These factors can lead to the sudden onset of pituitary apoplexy, characterized by a rapid increase in intrasellar pressure, tissue necrosis, and hormonal dysfunction. The clinical manifestations of pituitary apoplexy can vary depending on the extent and location of the tissue damage but commonly include severe headache, visual disturbances, nausea and vomiting, and hormonal imbalances (such as hypopituitarism or hyperprolactinemia).

Etiology

Pituitary apoplexy often occurs in the presence of a pre-existing pituitary adenoma, a type of tumor that grows on the pituitary gland.
Several factors can predispose or contribute to the development of pituitary apoplexy. Endocrine stimulation tests, used to evaluate the hormonal function, can sometimes trigger the condition.

Treatment with medications such as cabergoline or bromocriptine, which are used to treat conditions like hyperprolactinemia and Parkinson’s disease, may also increase the risk of pituitary apoplexy. Similarly, gonadotropin-releasing hormone treatment, which stimulates ovulation in women undergoing fertility treatments, has been associated with pituitary apoplexy.

Other factors linked to pituitary apoplexy include lumbar fusion surgery performed in the prone position, pituitary irradiation, pregnancy, anticoagulation therapy, thrombocytopenia, and certain medications to treat erectile dysfunction.

Genetics

Prognostic Factors

Clinical History

Clinical History

Pituitary apoplexy can have severe clinical consequences. One of the primary issues that arise in patients with pituitary apoplexy is the reduced secretion of adrenocorticotropic hormone (ACTH). The lack of ACTH secretion can cause the adrenal gland to stop producing cortisol.

When cortisol levels drop rapidly due to reduced ACTH secretion, the patient may experience a range of symptoms known as adrenal crisis. These symptoms can be severe and may include nausea and vomiting, abdominal pain, bradycardia, and hypotension.

The patient may also experience hypothermia, lethargy, and in some cases, coma. The adrenal crisis can be life-threatening, requiring immediate medical attention to prevent further complications.

Physical Examination

Physical Examination

Pituitary apoplexy can cause a sudden onset of headaches, typically located behind the eyes. While the exact cause of the headache is not entirely understood, several theories have been proposed to explain its underlying mechanism.
In addition to headaches, patients with pituitary apoplexy may experience other symptoms, such as hemianopia, decreased visual acuity, ptosis, diplopia, nausea and vomiting, hormonal dysfunction, and altered mental status.

Double vision is a common complaint among patients with this condition and is typically caused by extrinsic compression of one or several extraocular nerves. The oculomotor nerve is the most commonly affected, leading to lateral eye deviation and ptosis, sometimes accompanied by pupillary dilation of the affected eye.

The condition may also cause hormonal imbalances, depending on which part of the pituitary gland is affected. This can lead to symptoms such as fatigue, weakness, weight loss, and changes in sexual function. Additionally, patients may experience neurological deficits, such as numbness or limb weakness, or difficulty speaking or understanding language.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential Diagnoses
• Aneurysm
• Basilar Artery Infarct
• Cavernous sinus thrombosis
• Hypertensive Encephalopathy
• Ophthalmoplegic migraine
• Rathke’s cleft cyst
• Retrobulbar neuritis
• Subarachnoid Hemorrhage
• Temporal Arteritis

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

When managing patients with pituitary apoplexy, it is crucial to assess their fluid and electrolyte balance, ensure they are hemodynamically stable, and administer corticosteroids even if they do not present symptoms of adrenal crisis. The recommended initial dose is an intravenous bolus of hydrocortisone, ranging from 100-200 mg, followed by continued administration of 50-100 mg every 6 hours or a continuous intravenous infusion of 2-4 mg/hour.

Decompressive surgery can be delayed but should be performed within a week if visual acuity defects appear stable. For improving or stable ophthalmoplegia, a conservative strategy may be considered. Microscopic endonasal or sublabial transsphenoidal surgery is commonly used, while a craniotomy is preferred for large tumors extending over the chiasm or laterally to the temporal fossa. Endoscopic endonasal approaches are also effective for managing pituitary apoplexy.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

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Pituitary Apoplexy

Updated : August 30, 2023




Pituitary apoplexy is a rare but serious medical condition resulting from bleeding or decreased blood supply to the pituitary gland, often in a existing pituitary adenoma. This can cause sudden death of the gland and is characterized by acute ischemic infarction.

The first recorded case of hemorrhage associated with pituitary tumors was reported in 1898 by Pearce Bailey, but the term pituitary apoplexy to describe necrosis and to bleed into pituitary tumors was not introduced until 1950.

Prompt identification and assessment are crucial in many cases of pituitary apoplexy, which is considered a medical and surgical emergency. Treatment usually involves a combination of medical and surgical interventions, depending on the underlying cause and severity of the condition.

The occurrence of pituitary apoplexy is a medical condition that affects individuals with pituitary adenoma, a type of tumor located in the pituitary gland. Reports on the incidence of pituitary apoplexy vary greatly, ranging from 1.5% to 27.7%, in patients with pituitary adenoma. However, these reports often do not differentiate between symptomatic and asymptomatic cases.

When only symptomatic cases are considered, the incidence of pituitary apoplexy is estimated to be around 10%. Nevertheless, if considering the detection of non-symptomatic intratumoral hemorrhage through neuroimaging studies, the incidence increases to 26%. The occurrence of pituitary apoplexy in pituitary adenomas is relatively rare, estimated at 0.2% annually. Nevertheless, tumors larger than 10 cm and exhibiting rapid growth have a higher risk of apoplexy.

Moreover, pituitary apoplexy is observed more frequently in men than women, with a male-to-female ratio approaching 2:1. In terms of age range, most patients diagnosed with pituitary apoplexy fall between 37-58 years old. It is important to note that the incidence rates of pituitary apoplexy are based on reported cases, and there may be instances where pituitary apoplexy goes undiagnosed or misdiagnosed.

Pituitary apoplexy is a rare and life-threatening medical condition that occurs when the pituitary gland is bleeding or necrosis. The pituitary gland is a small, pea-sized gland located at the base of the brain, which produces and secretes hormones that regulate various bodily functions.

Pituitary apoplexy can cause a sudden onset of severe headaches, visual disturbances, and hormonal imbalances. The pathophysiology of pituitary apoplexy is complex and not fully understood, but it is thought to involve a combination of vascular, mechanical, and biochemical factors.

The following is a detailed explanation of the pathophysiology of pituitary apoplexy:

  • Vascular factors: Pituitary apoplexy is often associated with a sudden increase in blood flow to the pituitary gland, resulting in the rupture of blood vessels and bleeding into the gland. This increase in blood flow may be caused by various factors, such as hypertension, use of anticoagulant medications, or pregnancy. In addition, the pituitary gland has a rich blood supply from the internal carotid artery, which makes it vulnerable to ischemic damage or thrombosis of the blood vessels.
  • Mechanical factors: Pituitary apoplexy can also occur due to mechanical stress on the pituitary gland, such as trauma or surgery. For example, a blow to the head can cause the pituitary gland to shift within the sella turcica (a bony structure that houses the pituitary gland), leading to the compression of blood vessels and tissue necrosis.
  • Biochemical factors: Certain conditions or factors can also predispose individuals to pituitary apoplexy by altering the biochemical environment of the pituitary gland. For instance, pituitary adenomas can cause hormonal imbalances that affect the blood vessels and cause tissue damage. In addition, patients with underlying medical conditions such as diabetes mellitus, sickle cell anemia, or hypercoagulable states are at higher risk for pituitary apoplexy due to changes in blood glucose levels or blood viscosity.

These factors can lead to the sudden onset of pituitary apoplexy, characterized by a rapid increase in intrasellar pressure, tissue necrosis, and hormonal dysfunction. The clinical manifestations of pituitary apoplexy can vary depending on the extent and location of the tissue damage but commonly include severe headache, visual disturbances, nausea and vomiting, and hormonal imbalances (such as hypopituitarism or hyperprolactinemia).

Pituitary apoplexy often occurs in the presence of a pre-existing pituitary adenoma, a type of tumor that grows on the pituitary gland.
Several factors can predispose or contribute to the development of pituitary apoplexy. Endocrine stimulation tests, used to evaluate the hormonal function, can sometimes trigger the condition.

Treatment with medications such as cabergoline or bromocriptine, which are used to treat conditions like hyperprolactinemia and Parkinson’s disease, may also increase the risk of pituitary apoplexy. Similarly, gonadotropin-releasing hormone treatment, which stimulates ovulation in women undergoing fertility treatments, has been associated with pituitary apoplexy.

Other factors linked to pituitary apoplexy include lumbar fusion surgery performed in the prone position, pituitary irradiation, pregnancy, anticoagulation therapy, thrombocytopenia, and certain medications to treat erectile dysfunction.

Clinical History

Pituitary apoplexy can have severe clinical consequences. One of the primary issues that arise in patients with pituitary apoplexy is the reduced secretion of adrenocorticotropic hormone (ACTH). The lack of ACTH secretion can cause the adrenal gland to stop producing cortisol.

When cortisol levels drop rapidly due to reduced ACTH secretion, the patient may experience a range of symptoms known as adrenal crisis. These symptoms can be severe and may include nausea and vomiting, abdominal pain, bradycardia, and hypotension.

The patient may also experience hypothermia, lethargy, and in some cases, coma. The adrenal crisis can be life-threatening, requiring immediate medical attention to prevent further complications.

Physical Examination

Pituitary apoplexy can cause a sudden onset of headaches, typically located behind the eyes. While the exact cause of the headache is not entirely understood, several theories have been proposed to explain its underlying mechanism.
In addition to headaches, patients with pituitary apoplexy may experience other symptoms, such as hemianopia, decreased visual acuity, ptosis, diplopia, nausea and vomiting, hormonal dysfunction, and altered mental status.

Double vision is a common complaint among patients with this condition and is typically caused by extrinsic compression of one or several extraocular nerves. The oculomotor nerve is the most commonly affected, leading to lateral eye deviation and ptosis, sometimes accompanied by pupillary dilation of the affected eye.

The condition may also cause hormonal imbalances, depending on which part of the pituitary gland is affected. This can lead to symptoms such as fatigue, weakness, weight loss, and changes in sexual function. Additionally, patients may experience neurological deficits, such as numbness or limb weakness, or difficulty speaking or understanding language.

Differential Diagnoses
• Aneurysm
• Basilar Artery Infarct
• Cavernous sinus thrombosis
• Hypertensive Encephalopathy
• Ophthalmoplegic migraine
• Rathke’s cleft cyst
• Retrobulbar neuritis
• Subarachnoid Hemorrhage
• Temporal Arteritis

When managing patients with pituitary apoplexy, it is crucial to assess their fluid and electrolyte balance, ensure they are hemodynamically stable, and administer corticosteroids even if they do not present symptoms of adrenal crisis. The recommended initial dose is an intravenous bolus of hydrocortisone, ranging from 100-200 mg, followed by continued administration of 50-100 mg every 6 hours or a continuous intravenous infusion of 2-4 mg/hour.

Decompressive surgery can be delayed but should be performed within a week if visual acuity defects appear stable. For improving or stable ophthalmoplegia, a conservative strategy may be considered. Microscopic endonasal or sublabial transsphenoidal surgery is commonly used, while a craniotomy is preferred for large tumors extending over the chiasm or laterally to the temporal fossa. Endoscopic endonasal approaches are also effective for managing pituitary apoplexy.

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