SPOTTER PRESENTATION CUSHING DISEASE 24-8-24.pptx

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1. SPOTTER PRESENTATION 24-8-24 Dr. P.Anil Kumar Reddy Senior Resident- Neurosurgery. Moderator- Dr. V. V. Ramesh Chandra (Professor & HOD)

2. IDENTIFY THE SPOTTER

3. IDENTIFY THE SPOTTER

4. A 22 years old male patient with no comorbidities, presented with H/o facial puffiness for 6 years associated with abdominal distension, easy fatigability, weight gain. H/o acne/hyperpigmentation over face, chest, feet from 6 years not associated with usage of any drugs. H/o weight gain from 5 years, gained 20 kg in the last 5 years. H/o trauma( lifting heavy weights) 4yrs back followed by severe low backache with no weakness of lower limbs. BRIEF HISTORY

5. Endocrinology Workup Hormone Value Normal range T3 0.6 0.5-5.0 T4 74 54-142 TSH 0.6 0.8-1.7 Hb A1c 8.7 FBS 175 Baseline cortisol 8 am 43.5 6.7-22.6 Baseline cortisol 11 pm 30.5 6.7-22.6 ACTH 60 6.17-58.2 Testosterone 0.76

6. Over Night Dexamethasone suppression test(ONDST) : 1mg dexa @11pm 8 am serum cortisol- 35.31 (6.7-22.6) Low dose dexamethasone test (LDDST)-0.5 mg of dexa every 6 th hrly for 48hrs 8am serum cortisol -27.7 (6.7-22.6) High dose dexa suppression test(HDDST)- 2mg of dexa 6 th hrly for 48hrs 8am serum cortisol- 17.7 (6.7-22.6) more than 50 % suppressed –pituitary as cause. On admission midnight cortisol done, it showed no diurnal drop in cortisol hence proceeded with LDDST, it was not suppressed, ACTH was elevated indicating ACTH as cause hence HDDST was done which showed >50% suppression from baseline indicating pituitary as cause (Cushing’s disease)

7. X ray dorsal spine- BMD-osteoporosis 1) COLLAPSED D12 VERTEBRA 2) ANTERIOR WEDGING OF D11, D10, L1,L2 VERTEBRAL BODIES D12

8. PROVISIONAL DIAGNOSIS- Endogenous Cushing Disease with Pituitary Macrodenoma with Vertebral Fractures due to Secondary Osteoporosis. Surgery – Endoscopic Transnasal Trans-sphenoidal Excision of Macroadenoma. Biopsy:-

9. ASPECT CUSHING DISEASE CUSHING SYNDROME DEFINITION Specific type of Cushing syndrome caused by a pituitary adenoma General condition of chronic cortisol excess CAUSES Endogenous: Adrenal tumors , ectopic ACTH syndrome, pituitary adenomas (Cushing disease) - Exogenous: Use of corticosteroids Pituitary adenoma secreting ACTH CLINICAL FEATURES Similar features as Cushing syndrome - Additional pituitary mass effects like headache, visual disturbances - Central obesity - Hypertension - Diabetes - Osteoporosis - Mood change DIAGNOSIS - High-dose dexamethasone suppression test - CRH stimulation test - MRI of the pituitary gland Screening tests: 24-hour urinary free cortisol, late-night salivary cortisol, low-dose dexamethasone suppression - Imaging: CT/MRI of adrenal glands and/or pituitary TREATMENT Primary treatment: Transsphenoidal surgery to remove pituitary adenoma - Pharmacological management: Cabergoline, pasireotide - Radiotherapy if surgery is incomplete Depends on cause: Surgical resection of tumors , discontinuation of corticosteroids, or pharmacological management - Medications: Ketoconazole, metyrapone PROGNOSIS Varies based on underlying cause; treatment success depends on the etiology and extent of disease Generally good prognosis with successful pituitary adenoma removal; some may require additional treatments

10. Pituitary Macroadenomas- greater than 10mm in diameter. 80-90% of cases of Cushing disease are caused by pituitary macroadenomas. Macroadenomas secrete excessive amounts of adrenocorticotropic hormone (ACTH) , leading to overproduction of cortisol by the adrenal glands. Often cause symptoms due to mass effect , such as headaches, visual disturbances, and hypopituitarism Microadenomas also secrete ACTH but often do so in a less pronounced manner compared to macroadenomas.

11. Cushing disease remission is defined as achieving adrenal insufficiency with glucocorticoid dependency postoperatively Criteria for post op cure – 1. Morning cortisol <5ug/dl (0.43ug/dl on 27-6-24) 2. Undetectable serum ACTH 3. Return of low dose dexamethasone suppression Assessing Post-operative Remission

12. Osteoporosis recovery post treatment. U ntreated Cushing syndrome- 4-6 times risk of osteoporotic fractures Recovery Post-Surgery: B one density often improves over time, usually slow. Pharmacotherapy: Bisphosphonates (e.g., alendronate), S elective estrogen receptor modulators (SERMs), denosumab are commonly used to improve bone density. Calcium and Vitamin D Supplementation: Lifestyle Modifications: Weight-bearing exercises and fall prevention strategies.

13. Cochrane Library(2018) reported a systematic review regarding of percutaneous vertebroplasty for osteoporotic vertebral compression fracture, and showed no demonstrable important clinical benefits compared with the placebo , and did not support a role for vertebroplasty to treat acute or subacute osteoporotic vertebral fractures. Osteoporotic vertebral compression fractures caused by Cushing’s syndrome in young women: case report and literature review Cheng et al. BMC Musculoskeletal Disorders (2023) 24:167 https://doi.org/10.1186/s12891-023-06253-9

14. Preop Post op (6 months)