From the Guidelines
Pituitary apoplexy is a rare but serious medical emergency that occurs when there is sudden bleeding into the pituitary gland or when the blood supply to the gland is cut off, causing tissue death, and it is recommended to adopt the recommendations of available adult guidelines for its management in children and young people (CYP) due to limited case reports and case series in this population 1.
Definition and Pathophysiology
Pituitary apoplexy typically affects people with an existing pituitary tumor, though it can occasionally occur in normal pituitary glands. The condition occurs because the pituitary's blood supply is vulnerable to disruption, especially when enlarged by a tumor, and certain triggers like blood pressure fluctuations, anticoagulant use, or head trauma can precipitate the bleeding event.
Symptoms and Diagnosis
Symptoms develop rapidly and may include:
- Severe headache
- Visual disturbances (such as double vision or loss of peripheral vision)
- Nausea
- Vomiting
- In severe cases, reduced consciousness Hormone deficiencies often result because the damaged pituitary can't produce essential hormones that regulate bodily functions.
Treatment and Management
Treatment depends on symptom severity, with some patients requiring urgent neurosurgical intervention to relieve pressure on surrounding structures, while others with milder symptoms may be managed conservatively with hormone replacement therapy.
- Corticosteroids (like hydrocortisone) are typically administered immediately to address potential adrenal insufficiency.
- After the acute phase, patients need long-term endocrinological follow-up as many will require lifelong hormone replacement therapy. In CYP with pituitary apoplexy, early surgery might be beneficial for selected patients, especially considering that paediatric pituitary apoplexy can be more severe than in adults 1.
From the Research
Definition and Characteristics of Pituitary Apoplexy
- Pituitary apoplexy (PA) is an acute, life-threatening clinical syndrome caused by hemorrhage and/or infarction of the pituitary gland 2.
- It is characterized by the sudden onset of headache, and may also be accompanied by nausea, vomiting, visual disturbances, and decreased level of consciousness 2, 3.
- PA can occur at any age, but is more frequent in men aged 50-60, and is rare in children and adolescents 2.
Clinical Presentation and Diagnosis
- The clinical presentation of PA includes neurologic, ophthalmologic, and endocrinologic symptoms, such as sudden-onset severe headache, visual field defects, and pituitary hormone deficiencies 4, 5.
- Magnetic resonance imaging (MRI) is the imaging method of choice to aid in the diagnosis of PA 4, 6.
- The diagnostic approach also includes evaluation of pituitary secretory function 3.
Treatment and Management
- PA is a medical emergency that requires immediate attention and, in many cases, urgent surgical intervention and long-term follow-up 2, 6.
- Treatment options include conservative management with periodic follow-up or neurosurgical intervention, which should be decided by a multidisciplinary team 3, 6.
- Surgical treatment is usually performed through the trans-sphenoidal route, and most patients have a good recovery in visual function and extraocular palsy 6.
Risk Factors and Outcomes
- Precipitating factors for PA include systemic, iatrogenic, and external factors, as well as the presence of an underlying pituitary tumor 2, 6.
- Prior use of dopamine agonists and the presence of oculomotor abnormalities are significant risk factors for PA in patients with nonfunctioning pituitary macroadenomas 5.
- The majority of patients will have residual hormonal deficits that require prolonged hormone replacement therapy, and there is a small but significant risk of recurrent PA and tumor recurrence 6.