Urgent and Emergent Pituitary Conditions

Transcription

1 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

2 Urgent and Emergent Pituitary Conditions Neurosurgical Conditions Pituitary Apoplexy Visual Loss Hydrocephalus Endocrine Conditions Secondary Adrenal Failure Hypothyroidism

3 Case 1 43 y/o with low libido and low testosterone

4 Case 1 Acute diplopia 1 day prior to scheduled surgery Severe HA and left CN 6 palsy

5 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

6 Case 1 Postop Imaging

7 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

8 Pituitary (Adenoma) Apoplexy - Incidence Estimates from 1-26% Moller-Goede et al. Eur J Endocr, 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

9 Pituitary Apoplexy - Pathophysiology Foulad et al. Pituitary Gland Anatomy, Medscape

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11 Pituitary Apoplexy - Imaging

12 Pituitary Imaging - MRI

13 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

14 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

15 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

16 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%

17 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 IGF-1 506

18 Case 2

19 Case 2 Surgical plan Endoscopic Endonasal Surgery for aggressive debulking via sellar and transtubercular approach Lumbar Drain, Fat graft and Naso-septal Flap

20 Case 2 -Postop OS vision improved immediately after surgery D/C home on POD5 3 month postop VF, VA normal On cabergoline and hydrocortisone

21 Visual Loss Classically bitemporal visual field loss starting in superior quadrants

22 Visual Loss

23 Visual Recovery 95% of patients have VF/VA recovery Gnanalingham et al. J Neurol Neuosurg Psych % full recovery of VFs 60% partial recovery VF impairment preop affects recovery on MV analysis

24 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)

25 Case 3 67 y/o attorney with 48 hrs of cognitive changes and imbalance

26 Case 3

27 Case 3 - Approach Interhemispheric Transcallosal Transventricular Trans-choroidal approach to IIIrd ventricle

28 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

29 Case 3 Outcome 9 month postop MRI slight progression Referred for RT.

30 Hydrocephalus Rare with pituitary lesions Usually requires giant macroadenoma, craniopharyngioma or other lesion occupying 3 rd ventricle Recommendations: Urgent/Emergent neurosurgery referral

31 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

32 Case 4 - Imaging

33 Case 4 - Approach Endoscopic Endonasal Approach Lumbar Drain and Abdominal Fat Graft D/C home POD 4 Path = Rathke s cleft cyst

34 Case 4 Outcome Neurologically intact Doing well and working No DI Maintained on preop hydrocortisone and thyroid hormone

35 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

36 9Is of pituitary insufficiency Iatrogenic Injury Infection Infarction Irradiation Invasion Infiltration Immunologic Idiopathic

37 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

38 ~18% of pituitary incidentalomas manifest ACTH/cortisol axis deficiency

39 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 min ** may be normal in secondary AI

40 Treatment Stress/Illness Initial: 100 mg hydrocortisone IV q6 Reduce quickly to qam, 10 mg qpm Dexamethasone wont interfere with ACTH stim test

41 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 T microgram IV Also give hydrocortisone 100 mg IV

42 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

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