Grand round Interesting case Pituitary apoplexy

interesting case about
pituitary apoplexy

1. Grand Round 13 Sep 2023

2. Case presentation ผู้ป่วยชายไทย อายุ 68 ปี ภูมิลำเนา อำเภอ พาน จังหวัด เชียงราย อาชีพ - Present illness 1 เดือนก่อนมาโรงพยาบาลเริ่มมี อาการปวดศีรษะเป็นๆ หายๆ ลักษณะการปวดเป็นแบบแน่นๆ กลางศีรษะ ไม่ร้าวไปบริเวณอื่นๆ ปวดกลางคืนมากกว่าช่วงกลางวัน เวลาปวดจะไม่มีเห็นแสงวาบ ไม่มีปวดรอบกระบอกตา ไม่มีตามองเห็นผิดปกติ ไม่มีคลื่นไส้อาเจียน ไม่มีชา ไม่อ่อนแรง อาการไม่ดีขึ้นหลังรับประทานยาแก้ปวด ความรุนแรงของอาการปวดเป็นมากขึ้นเรื่อย ๆ เบื่ออาหาร กินได้น้อย น้ำหนักลดจาก 52กก เหลือ -45 กก ใน 1 เดือน ในช่วง 1 สัปดาห์ก่อนมา ร . พ . ผู้ป่วยรู้สึกเหมือนมีไข้ต่ำ ๆ ตลอด ( แต่ไม่ได้วัดไข้ ) สังเกตว่าปัสสาวะบ่อยขึ้น ต้องเข้าห้องน้ำ เกือบทุก 2 ชั่วโมง แต่ปริมาณ ไม่มาก ไม่มีหิวน้ำบ่อย ไม่มีปากแห้งคอแห้ง อาการปวดศีรษะและอ่อนเพลีย เป็นมากขึ้น เรื่อยๆ ล่าสุด Pain score 7-8 จึงไปตรวจที่คลินิกใกล้บ้าน แพทย์แนะนำให้มา รพ.

3. Past history No underlying disease No drug allergy Deny any herbal or other medications

4. Physical examination Vital signs : Temp 37.3 c BP 92/59 mmHg PR 72/min RR 18/min BW 45 kg Ht. 155 cm BMI 18.73 General appearance : Cachexia, dry lips, look sick HEENT : Mild pale conjunctiva, no icteric sclerae, cervical LN neg. Heart : Regular, HR 70/min, no murmurs. Lungs : Clear Abd : Soft, not tender, no hepatosplenomegaly Ext : no edema Skin : No rash Neuro : Normal consciousness, stiff neck neg, motor grade 5 all, sensory grossly intact. VF : Confrontation ประเมินไม่ได้

5. Problems List Progressive headache Anorexia with weight loss Low grade fever Polyurea

6. Differential Diagnosis

7. Clues for Secondary Headache

8. RED FLAGS SIGNS

9. CBC

10. Lab Chemistry

11. UA+Urine chemistry

12. CT Brain Pre-Post Contrast

13. Hormonal Work up

14. Definite diagnosis Progressive headache from Pituitary macroadenoma with apoplexy Panhypopituitarism ( Central hypothyroid, AI,GH, +/- DI ) SIADH from hypothyroid and adrenal insufficiency True Hyponatremia (Low Sosm , High Uosm , High Urine Na) +/- Central DI

15. Review Pituitary Apoplexy

16. Introduction Pituitary apoplexy is a clinical syndrome, characterized by sudden onset of Headache Visual impairment Decreased consciousness caused by abrupt hemorrhage and/or infarction of the pituitary gland, generally within a pituitary adenoma

17. Anatomy of Pituitary Gland

18. Hormone Secretion of Pituitary Gland Modified from 3 The Netter Collection of Medical Illustrations: Endocrine System Vol.2

19. Pathophysiology of Pituitary Apoplexy 1.Suri, H., Dougherty, C. Presentation and Management of Headache in Pituitary Apoplexy. Curr Pain Headache Rep 23, 61 (2019) 2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..

20. Pathophysiology of Pituitary Apoplexy 1.Suri, H., Dougherty, C. Presentation and Management of Headache in Pituitary Apoplexy. Curr Pain Headache Rep 23, 61 (2019) 2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45..

21. Epidemiology More than 80% have an underlying pituitary adenoma (Can occur in non-adenomatous lesions, such as sellar tuberculoma, sellar metastasis, or normal pituitary gland) Prevalence is about 6.2 cases/100 000 population Incidence is about 0.17 episodes/100,000 person-years Most frequent in the fifth or sixth decade Male : Female is about 2:1 1.Raappana A, Koivukangas J, Ebeling T, Pirilä T. Incidence of pituitary adenomas in Northern Finland in 1992–2007. J Clin Endocrinol Metab . 2010;95:4268–4275 2.FernandezAKaravitakiNWassJA. Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK). Clinical Endocrinology 201072377–382.

22. Precipitating Factors : found in 40% of cases The majority of cases occur spontaneously 1.Rajasekaran S, Vanderpump M, Baldeweg S, et al. UK guidelines for the management of pituitary apoplexy. Clin Endocrinol ( Oxf ). 2011;74:9–20 2.Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45...

23. Common clinical features of pituitary apoplexy Frequency (%) Headache >90 Nausea- vomitting 40-80 Visual impairment ( Diplopia,Visula field defects,decreased VA) >50 Altered mental status 13-42 Hypopituitarism >50 Hyponatremia 12-44 Pyrexia 20 Others: Seizure/Hemiparesis/Sudden death Cristina Capatina ,Warrick Inder ,Niki Karavitaki et.al1 MANAGEMENT OF ENDOCRINE DISEASE: Pituitary tumour apoplexyin European Journal of Endocrinology May 2015 Volume 172: Issue 5, European Society of Endocrinology Manel Jemel , Wafa Alaya, Fedia Boubaker , Olfa Berrich and Baha Zantour Submitted: February 6th, 2018 Reviewed: April 16th, 2018 Published: November 5th, 2018 DOI: 10.5772/intechopen.77270 Clinical Presentation

24. Presentations of Headache in Pituitary Apoplexy Nearly half (45.8%) in a retrospective series of 60 patients presented with severe acute thunderclap headache with maximum intensity at onset . Some presented with a gradual onset, unremitting headache of several weeks duration. Few cases throbbing and associated with nausea, phonophobia, and mild photophobia, mimicking migraine Himanshu Suri & Carrie Dougherty. Presentation and Management of Headache in Pituitary Apoplexy; Current Pain and Headache Reports volume 23, Article number: 61 .2019.

25. Diagnostic Evaluation Who Should be Suspected Patients presenting with acute severe headache with or without neuro‐ophthalmic signs Patients known to have a pituitary tumor Patients known to have a pituitary tumor when performing pituitary stimulation tests,

26. Diagnostic Evaluation Role of Computerized Tomography(CT) To rule out SAH in patients with severe, sudden onset headache It shows an intrasellar mass in 80% of cases, but was diagnostic in only 21–28% of cases In non-hemorrhage apoplexy, CT may be non- specific CT provides better hemorrhage detection in hyperacute stage(few hours from onset) than MRI. Briet C1, Salenave S1, Bonneville JF1,et. Al. Pituitary Apoplexy Endocr Rev. 2015 Dec;36(6):622-45...

27. Image Findings Non-contrast CT in Hemorrhage of Pituitary Apoplexy Hyperdense lesion in acute hemorrhage(few hours from onset)

28. Role of Magnetic Resonance Imaging(MRI) Confirm the diagnosis of pituitary apoplexy in over 90% of the patients MRI can identify hemorrhagic and necrotic areas and show the relationship between the tumor and neighboring structures In the first few hours, hemorrhage can be missed on MRI and is better identified with CT

29. Image Findings Normal Pituitary Gland in MRI

30. Image findings Normal Pituitary Gland in MRI

31. Image findings Normal Pituitary Gland in MRI

32. Image Findings CT and MRI in Hemorrhage Apoplexy Modified from Dr Mostafa El- Feky and Assoc Prof Frank Gaillard et al. Timeline diagram of MRI and CT characteristics of intracerebral hemorrhage . Radiopaedia .. 19th Sep 2017

33. Image Findings MRI of Hemorrhage Apoplexy in Acute Stage(12~48hours)

34. Image Findings MRI in Non-hemorrhage Apoplexy In acute phase, both hemorrhagic and non-hemorrhagic apoplexy show high signal on diffusion weighted imaging(DWI) After intravenous contrast, the most common finding in both hemorrhagic and non-hemorrhagic apoplexy is peripheral rim enhancement in acute phase

35. Image Findings MRI in Non-hemorrhage Apoplexy in Hyperacute stage

36. Back to our case

37. Management Therapeutic strategies Supportive measures to ensure hemodynamic stability Empiric corticosteroids replacement Conservative treatment Surgical intervention Best approach is controversial

38. Empiric Corticosteroids Replacement Corticotropic deficiency is the most life-threatening complication, potentially causing severe hemodynamic problems Indication: Hemodynamic instability, impaired consciousness and visual function Treatment Dose : Hydrocortisone 100 mg IV bolus, then 200 mg over 24hr by continuous infusion Tapering dose 50% daily When patient is stabilized switch to Prednisolone

39. Conservative Treatment Some retrospective studies have revealed that in patient with stable visual deficits or improving, a conservative approach is safe, does not result in poor visual or endocrine outcome Indications Patients without any neuro‐ophthalmic signs or improving Operative high risk Treatment Glucocorticoids, replacement of hormone deficits Monitoring of visual function, keep fluids and electrolytes balance.

40. Surgical Intervention Indications Visual fields and acuity impairment Impaired consciousness Deteriorating neurological signs Conservative treatment failure Timing Preferably within the first 7 days of onset of symptoms Complications CSF leakage Damage of normal pituitary

41. Best approach is still controversial

42. Management Adriana Albani , Francesco Ferraù , ,Filippo Flavio Angileri et.al Multidisciplinary Management of Pituitary Apoplexy Int J Endocrinol. 2016 Dec 15.

43. Outcome Motality : 5–15.3% Visual disturbance Complete or significant improvement in visual disturbance occurs in 57–86% Endocrine Hypogonadism remains in 55–79% Hypothyroidism in 45–60% ACTH deficiency in 60–87%

44. Take Home Messages Pituitary tumor apoplexy is a rare, potentially life-threatening clinical syndrome caused by ischemic infarction or hemorrhage, generally into a pituitary tumor The diagnosis should be suspected in all cases with sudden-onset severe headache, with or without neuro-ophthalmic manifestations CT imaging is the most common initial study during the acute onset symptoms MRI having better sensitivity should always be performed in acute and subacute phase

45. Take Home Messages Patient should be under the care of a multidisciplinary team including endocrinologist, neurosurgeon, and ophthalmologist In cases with severe, progressing visual or neurological manifestations, surgical decompression is indicated Patients with mild, stable clinical picture can be managed conservatively The visual and neurological outcomes are favorable in most cases. The endocrinological prognosis is less favorable with many patients requiring replacement therapy and long-term follow-up. The optimal treatment is debate. Prospective randomized trials are necessary