Acromegaly Flashcards
Acromegaly Symptoms and Signs?
ABCDEFGH-PTSD
Acral enlargement (limb, fingers, ears)
BP - HTN
Cardiac failure / Carpal tunnel syndrome / Colonic polyps
Diabetes
Enlarged organs / Erectyle dysfunction (hypogonadism)
Field defect (visual) + CN palsies
Goitre
Hypopituitarism/Headache
Peripheral neuropathy /
Tags - skin
Sweating / Sleep Apnoea
Diabetes
What are the signs of active disease? (7)
Basically progressive clinical features
Increase in shoe/hand size
Increasing number of skin tags
Increasing BP
Increasing VF loss
Progressive CN palsies
Enlarging Goitres
Worsening DM (Glycosuria)
What are the complications of Acromegaly? (5)
In contrast with signs of active disease, complications are basically established disease state.
- Cardiac: HTN, CCF, Cardiomegaly
- Diabetes
- Hypopituitarism
- Local effects from compression: CN/PN palsies, spinal stenosis, arthritis, VF defect
- Malignancy: CRC, Uterine Leimyomata.
What are the causes of Acromegaly? (3)
GH secreting adenoma (most common, 75% are macro [>1cm])
Rare causes:
Hypothalamic tumours releasing GHRH
Ectopic GH from Carcinoid or SCLC
What are the complications of pituitary hypophysectomy? (3)
Hypopituitarism*** - monitor hormone levels closely and replace them. Remember Vasopressin deficiency can be life-threatening if Teripressin is not appropriately given.
Visual Field defect
Recurrence
Acromegaly - PRICMCP?
P: ABCDEFGH-PTSD
R: Known pituitary, hypothalamic lesions or malignancy (ectopic), FH
I: OGTT, IGF-1, MRI
C: metabolic (HTN, DM, CCF, OSA), malignancy (CRC), pituitary (hypopit, VF, CN), neurological (spinal stenosis, CTS)
Complications of therapy, RTx, Surgery - mainly hypopituitarism associated complications. Sx - CSF leak, stroke, DI.
M: Somatostatin analogs, Surgical Resection (hypophysectomy), RTx
C: was treatment effective, still symptomatic? How is the life affected?
P: Curative post surgery.
Acromegaly - clinical examination?
App: Prognathism, Frontal Bossing, Large Hands/Feets
No sweating or skin tags (axilla)
Arm: Spade-like hands, sweating, CTS (Phalen/Tinnels), HTN, Ulnar nerve thickening (palpate behind medial epicondyle), Proximal myopathy
Eyes: VF defect, CN 346+5, Papilloedema/Optic atrophy, macroglossia, interdental separation
Goitre
CVS + Lung: Displaced Apex, evidence of HF, gyneocomastia (increased PRL)
Abdomen: no organomegaly
MSK: OA in Hips/Knees
Neurological: no evidence of lower limb neurology suggestive of spinal canal stenosis (e.g. foot drop)
To complete: ECG (LVH), Urine dip (diabetes), DRE (PR bleed)
What investigations would you ask for?
T: GH excess - OGTT (75g of oral glucose), GH should normally be suppressed to <1mcg/L in 2 hours + Increased IGF-1
E: exclude other hormone deficiencies - Cortisol, TSH/T4, FSH/LH, prolactin, testosterone. Serum Ca2+ to exclude MEN1 (consider Gastrin)
S: IGF-1 is also marker of severity/activity, MRI-B (Size of the tumour)
S: screen complications - urine dip/fasting glucose (DM), ECG (LVH - if so TTE), PSG (OSA), ambulatory BP monitoring, FOBT/Colonoscopy
What is your approach to managing this patient with Acromegaly?
Confirm Dx: OGTT, IGF-1, MRI-B
A: screen for other hormonal deficiencies, complications of Acromegaly (metabolic, malignant, neurological)
Set specific goals: reduce IGF-1 to normal, suppress GH <2.5mcg/L, minimize symptoms, prevent complications
T: Non-pharm
- Educate - especially complications of hypopituitarism (e.g. adrenal insufficiency), the danger of not taking meds, life-threatening complications (HF, CRC)
- Aggressive Mx CVS risk factors: recommend Mediterranean diet, weight loss, exercise, cessation of smoking, moderation of ETOH, statin/ACEi
- Medical bracelet - hypopituitarism
T: Pharm
- Trans-sphenoidal pituitary surgery is the 1st line - curative (90%) or debulking (perioperative mortality rate <1%). Consider pre-treat with SA.
- Somatostatin analogues: these are 4-weekly S/C injection (inhibits GH release) - indefinite. Can cause biochemical remission (70%) and 70% reduction of the tumour size.
- Cabergoline (D2R agonist)
- GH receptor agonists (Peg-nisomant) - daily S/C.
Involve - other specialties (endocrinologist)
C - ensure F/U and monitor hormones + complications. Fasting glucose, lipids annually. Consider TTE (5-yr), Colonoscopy (at diagnosis + surveillance), screen for MSK + neuro.
Somatostatin analogs side effects? (3)
Mainly GI.
Cholelithiasis
Abdominal pain
Diarrhoea
What screening would you recommend to this acromegalic patient for complications
of the disease?
Metabolic: fasting lipids, glucose, HBA1C, TFT (Goitre). if hypopituitarism regularly test hormones. Ambulatory BP, ECG (LVH), TTE (LV dysfunction), PSG (OSA).
Malignancy: Colonoscopy at diagnosis + surveillance
MSK/Neuro: regular clinical examination and investigate as apprpriate.
3 main post-surgical complications of trans-sphenoidal pituitary surgery?
CSF Rhinorrhea
Diabetis insipidus
Stroke
