Pituitary Apoplexy Kyla Lokitz Morning Report 7/18/05

Pituitary Apoplexy A clinical syndrome resulting from acute hemorrhage or infarction of the pituitary gland characterized by the onset of: A clinical syndrome resulting from acute hemorrhage or infarction of the pituitary gland characterized by the onset of: Headache Headache Vomiting Vomiting Visual Disturbances Visual Disturbances Opthalmoplegia – CN III most common Opthalmoplegia – CN III most common Meningismus Meningismus Fever Fever Decreased Consciousness Decreased Consciousness Death Death

Epidemiology Incidence found to be % in series of surgically treated adenomas in patients with pituitary apoplexy (Semple et al. 2005). Incidence found to be % in series of surgically treated adenomas in patients with pituitary apoplexy (Semple et al. 2005). Pituitary hemorrhage observed in 25% of surgically removed adenomas; however, these patients were without clinical symptoms (Subclinical Pituitary Apoplexy) (Sibal et al. 2005). Pituitary hemorrhage observed in 25% of surgically removed adenomas; however, these patients were without clinical symptoms (Subclinical Pituitary Apoplexy) (Sibal et al. 2005). Hemorrhage and infarction with similar clinical presentation described in nontumerous pituitary glands. Also seen in other pituitary tumors such as craniopharyngiomas and lymphocytic hypophysitis. Hemorrhage and infarction with similar clinical presentation described in nontumerous pituitary glands. Also seen in other pituitary tumors such as craniopharyngiomas and lymphocytic hypophysitis. Cases reported in patients in first through ninth decade although pediatric cases rare. Cases reported in patients in first through ninth decade although pediatric cases rare. Series suggest apoplexy occurs more frequently in males (Lubina et al. 2005). Series suggest apoplexy occurs more frequently in males (Lubina et al. 2005).

Predisposing Factors: Arterial hypertension Arterial hypertension Sudden head trauma Sudden head trauma Cardiac surgery Cardiac surgery Transient elevation of intracranial pressure Transient elevation of intracranial pressure Diabetes Diabetes Acromegaly Acromegaly Cushing’s Syndrome Cushing’s Syndrome Estrogens Anticoagulation Bromocriptine Dynamic pituitary function tests GnRH analogues Radiotherapy

Pathophysiology With rapid growth, tumor outgrows blood supply With rapid growth, tumor outgrows blood supply Compression of the infundibular and superior hypophyseal vessels against the diaphragma sellae Compression of the infundibular and superior hypophyseal vessels against the diaphragma sellae Ischemia of the normal pituitary gland Ischemia of the normal pituitary gland Intrinsic vasculopathy of pituitary tumors Intrinsic vasculopathy of pituitary tumors

Diagnosis Laboratory Data: Laboratory Data: Prolactin, TSH, FT4, FT3, Cortisol, LH, FSH, Testosterone, Estrogen, GH, IGF1 Prolactin, TSH, FT4, FT3, Cortisol, LH, FSH, Testosterone, Estrogen, GH, IGF1 Electrolytes, CBC (patients often present with hyponatremia and leukocytosis) Electrolytes, CBC (patients often present with hyponatremia and leukocytosis) CSF-rbcs, xanthachromia, pleocytosis, increased protein CSF-rbcs, xanthachromia, pleocytosis, increased protein Imaging Imaging X-ray may demonstrate enlarged sella X-ray may demonstrate enlarged sella CT will demonstrate pituitary mass but not sensitive in demonstrating hemorrhage or infarction (i.e. CT diagnostic in only 28% of cases, defined sellar mass in 72% of cases) (Sibal et al. 04) CT will demonstrate pituitary mass but not sensitive in demonstrating hemorrhage or infarction (i.e. CT diagnostic in only 28% of cases, defined sellar mass in 72% of cases) (Sibal et al. 04) MRI is the radiologic mode of choice (i.e. confirmed diagnosis in >90% of cases) (Sibal et al. 04) MRI is the radiologic mode of choice (i.e. confirmed diagnosis in >90% of cases) (Sibal et al. 04)

Treatment Careful monitoring of fluid and electrolyte balance Careful monitoring of fluid and electrolyte balance Replacement of deficient hormone, especially corticosteriods Replacement of deficient hormone, especially corticosteriods Pituitary surgery Pituitary surgery Long term monitoring for hypopituitarism and recurrence of pituitary adenomas Long term monitoring for hypopituitarism and recurrence of pituitary adenomas

Surgical Treatment Indicated with: Diminished level of consciousness Diminished level of consciousness Hypothalmic disturbances Hypothalmic disturbances Visual impairment Visual impairment Some disagreement in literature about timing of surgery; question of improved outcome (in visual field and visual acuity) if operation occurs within 8 days of onset of symptoms (Randeva et al. 1999); other studies demonstrate satisfactory recovery of vision with late surgery (Ayuck et al. 2004, Sibal et al. 2005). Some disagreement in literature about timing of surgery; question of improved outcome (in visual field and visual acuity) if operation occurs within 8 days of onset of symptoms (Randeva et al. 1999); other studies demonstrate satisfactory recovery of vision with late surgery (Ayuck et al. 2004, Sibal et al. 2005).

Conservative Therapy Favored in patients without or with mild neuro- opthalmic signs. Favored in patients without or with mild neuro- opthalmic signs. Retrospective studies have shown spontaneous improvement in neuro-opthalmic symptoms with conservative management (Sibal et al. 2005) Retrospective studies have shown spontaneous improvement in neuro-opthalmic symptoms with conservative management (Sibal et al. 2005)

References Ayuck, J. et al. Acute management of pituitary apoplexy – surgery or conservative management? Clinical Endocrinology 2004, 61: Ayuck, J. et al. Acute management of pituitary apoplexy – surgery or conservative management? Clinical Endocrinology 2004, 61: Levy, A. et al. Pituitary Disease: Presentation, Diagnosis, and Management. J Neurol Neurosur Psychiatry 2004, 75: Levy, A. et al. Pituitary Disease: Presentation, Diagnosis, and Management. J Neurol Neurosur Psychiatry 2004, 75: Lubina A. et al. Management of pituitary apoplexy: clinical experience with 40 patients. Acta Neurochirugica 2005, 147: Lubina A. et al. Management of pituitary apoplexy: clinical experience with 40 patients. Acta Neurochirugica 2005, 147: Ma, R. et al. Fever, headache, and a stiff neck. The Lancet 2004, 363: Ma, R. et al. Fever, headache, and a stiff neck. The Lancet 2004, 363: Randeva, H. et al. Classical pituitary apoplexy: clinical features, management, and outcome. Clinical Endocrinology 1999, 51: Randeva, H. et al. Classical pituitary apoplexy: clinical features, management, and outcome. Clinical Endocrinology 1999, 51: Semple, P. et al. Pituitary apoplexy. Neurosurgery 2005, 56: Semple, P. et al. Pituitary apoplexy. Neurosurgery 2005, 56: