Urgent and Emergent Pituitary Conditions PANKAJ A. GORE, MD DIRECTOR, BRAIN AND SKULL BASE T UMOR SURGERY PROVIDENCE B R AIN AND S PINE I NSTITUTE
Urgent and Emergent Pituitary Conditions Neurosurgical Conditions Pituitary Apoplexy Visual Loss Hydrocephalus Endocrine Conditions Secondary Adrenal Failure Hypothyroidism
Case 1 43 y/o with low libido and low testosterone
Case 1 Acute diplopia 1 day prior to scheduled surgery Severe HA and left CN 6 palsy
Case 1 Urgent Endoscopic Endonasal Surgery for resection of lesion Resolution of diplopia 6 months postop Returned to mountain climbing On thyroid, testosterone and growth hormone supplementation
Case 1 Postop Imaging
Pituitary Apoplexy Sudden/Rapid Onset of Headache (often progressive) Tumor Infarct or Hemorrhage Visual loss (50-60%) Cranial Neuropathy (80%) Diplopia (CN III or VI) Ptosis (CN III) Nausea/Vomiting
Pituitary (Adenoma) Apoplexy - Incidence Estimates from 1-26% Moller-Goede et al. Eur J Endocr, 2011 574 pts, retrospective review PA in 10-16% non-functioning macroadenomas PA in 5% clinically active macroadenomas Increased incidence in setting of anticoagulation and antiplatelet medication
Pituitary Apoplexy - Pathophysiology Foulad et al. Pituitary Gland Anatomy, Medscape
Pituitary Apoplexy - Imaging
Pituitary Imaging - MRI
Pituitary Apoplexy - Management Admission to ICU Pituitary labs -Suggestion of functional tumor affects surgical plan Hydrocortisone (100 mg IV q6) Thyroid Supplementation Smaller lesions without visual compromise can be conservatively managed
Pituitary Apoplexy Surgical Indications Visual Compromise More likely to recover with surgical debulking Recovery can be incomplete Cranial nerve deficit More rapid recovery with surgery Usually complete recovery with time Non-prolactinoma Functional Lesion
Sheehan Syndrome Hypertrophic pituitary gland is susceptible to infarction from hypovolemia and/or sepsis Acute presentation or visual compromise is rare Delayed hypopituitarism is common Lactation deficiency Amenorrhea due to gonadatropin deficiency
Pituitary Apoplexy - Outcomes Visual Acuity and field deficit improvement more likely with early surgery (<48 hrs) Endocrine outcomes are equivalent 80% will require hormone supplementation of 1 or more axes Corticosteroids 40-85% Thyroid 50-70% Desmopressin 6-25% Testosterone 40-80%
Case 2 22 y/o M with one month of visual loss, OS > OD Rapidly progressive severe OS loss over last week Sent to ED by optho No galactorrhea + decreased energy Prolactin -5506 IGF-1 506
Case 2
Case 2 Surgical plan Endoscopic Endonasal Surgery for aggressive debulking via sellar and transtubercular approach Lumbar Drain, Fat graft and Naso-septal Flap
Case 2 -Postop OS vision improved immediately after surgery D/C home on POD5 3 month postop VF, VA normal On cabergoline and hydrocortisone
Visual Loss Classically bitemporal visual field loss starting in superior quadrants
Visual Loss
Visual Recovery 95% of patients have VF/VA recovery Gnanalingham et al. J Neurol Neuosurg Psych 2005 35% full recovery of VFs 60% partial recovery VF impairment preop affects recovery on MV analysis
Recommendations Visual acuity or field deficits related to pituitary tumors should be urgently referred to neurosurgery Rapid changes (deterioration over several weeks or faster should be seen emergently)
Case 3 67 y/o attorney with 48 hrs of cognitive changes and imbalance
Case 3
Case 3 - Approach Interhemispheric Transcallosal Transventricular Trans-choroidal approach to IIIrd ventricle
Case 3 Outcome Neurologically intact EVDs weaned and hydrocephalus resolved Returned to playing tennis a few weeks after surgery Persistent low-grade DI on desmopression On cortisol, thyroxine and testosterone supplementation Fractionated RT recommended for residual
Case 3 Outcome 9 month postop MRI slight progression Referred for RT.
Hydrocephalus Rare with pituitary lesions Usually requires giant macroadenoma, craniopharyngioma or other lesion occupying 3 rd ventricle Recommendations: Urgent/Emergent neurosurgery referral
Case 4 25 y/o male endurance runner who noted fatigue at the end of a race Mild hyponatremia found 1 month later confusion and gait difficulties Na = 95 ICU admission and correction of hyponatremia Found to have secondary adrenal insufficiency and hypothyroidism Na normalized with initiation of hydrocortisone and thyroid hormone
Case 4 - Imaging
Case 4 - Approach Endoscopic Endonasal Approach Lumbar Drain and Abdominal Fat Graft D/C home POD 4 Path = Rathke s cleft cyst
Case 4 Outcome Neurologically intact Doing well and working No DI Maintained on preop hydrocortisone and thyroid hormone
Secondary Adrenal Insufficiency Failure of ACTH production resulting in a hypoadrenal state Sx: Chronic, worsening fatigue Muscle weakness Weight loss Nausea, vomiting, diarrhea Hypotension Hyponatremia Exacerbation by stress or illness can lead to Addisonian Crisis
9Is of pituitary insufficiency Iatrogenic Injury Infection Infarction Irradiation Invasion Infiltration Immunologic Idiopathic
Pituitary Tumors and AI Mass effect on gland Apoplexy Typically macroadenoma Post-surgical manipulation Chronic steroid admin after surgery Withdrawal of steroids Failure to stress dose Cushing s disease patients post-surgery
~18% of pituitary incidentalomas manifest ACTH/cortisol axis deficiency
Labs 8am cortisol Good screening study > 12 mcg/dl = normal 8-12 = likely normal < 8 further workup ACTH stim test Baseline ACTH and cortisol level Rise in cortisol > 18 mcg/dl after 30-60 min ** may be normal in secondary AI
Treatment Stress/Illness Initial: 100 mg hydrocortisone IV q6 Reduce quickly to 15-20 qam, 10 mg qpm Dexamethasone wont interfere with ACTH stim test
Hypothyroidism 28% of pituitary incidentalomas have deficiency in TSH/thyroid axis Myxedema coma > 60 yoa 90% in winter months Usually presents with cognitive changes 20% mortality Administer T4 100-500 microgram IV Also give hydrocortisone 100 mg IV
Summary Beyond apoplexy, pituitary tumors can have other neurologically threatening consequences. Apoplexy, visual changes and/or hydrocephalus merit urgent referral to neurosurgery Adrenal Failure and Hypothyroidism can also manifest in the setting of pituitary tumors and have life threatening consequences
Thank you Contact Information: Office: 503-935-8500 Mobile: 503-501-8331 Email: pgore@orclinic.com