Overview
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Acromegaly is a slowly progressive, debilitating endocrinopathy resulting from excess secretion of growth hormone, usually from a benign macroadenoma of the anterior pituitary gland, and characterized by overgrowth of soft tissues and bone and cartilage of skeleton (nose, jaw, hands, fingers, feet, toes). Excess growth hormone before puberty (epiphyseal closure) leads to gigantism (<5% of acromegalics).
Usual Treatment
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Surgery—primary therapy:
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Transsphenoidal pituitary microsurgery versus transcranial; transsphenoidal more common and preferred, with less morbidity. Smaller tumors (<10 mm diameter) yield probable cure. Otolaryngologists often assist neurosurgeons with access using sublabial or endonasal approach.
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Pituitary radiation—reserved for persistent postsurgical disease or when surgery is contraindicated.
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Medical—adjunctive therapy or for nonsurgical candidates, effective if adenoma cells have dopamine and/or somatostatin receptors:
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Dopamine agonists—bromocriptine and cabergoline.
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Somatostatin analogue—octreotide and lanreotide, inhibits GH release.
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Assessment Points
| System | Effect | Assessment by Hx | PE | Test |
|---|---|---|---|---|
| HEENT | Bone and soft tissue overgrowth of head and neck | TMJ arthritis Hoarseness Deep voice | Enlarged frontal, nasal bones Enlarged sinuses Macroglossia with glossoptosis Prognathism Hypertrophy of larynx Vocal cord thickening and edema Subglottic narrowing Enlarged thyroid gland (25%) with possible tracheal compression/deviation Recurrent laryngeal nerve paralysis | Indirect laryngoscopy Lateral neck x-rays CT of neck |
| CV | CAD PVD LV dysfunction Cardiomyopathy | Chest pain Htn CHF Dysrhythmias Diastolic dysfunction | Htn CHF Dysrhythmias Cardiomegaly Diastolic dysfunction | CXR ECG ECHO |
| RESP | Airway soft tissue overgrowth Upper airway and small airway narrowing | Obstructive sleep apnea (60% of pts) | Barrel chest with kyphosis | PFTs (if indicated) Sleep study |
| RENAL | Increased GI Ca 2+ absorption Hypercalciuria Increased total body Na + | Urolithiasis | Peripheral edema | |
| ENDO | Increased BMR | Heat intolerance | Hyperhidrosis | To diagnose acromegaly: ↑ 24 h GH levels Best screening test: ↑ serum IGF I Definitive test: Oral glucose tolerance test (GH levels do not ↓) |
| Hyperprolactinemia (some adenomas secrete GH and prolactin) Hyperthyroidism (3–7%) Insulin resistance (80%) Glucose intolerance (30–45%) Overt DM (15–25%) Hypertriglyceridemia (20–45%) Hyperphosphatemia Colon polyps/malignancy | Men: ↓ Libido, impotence Women: Menstrual abnormalities | Enlarged thyroid (25%) | TFTs Glucose Cholesterol, triglycerides Phosphorus Colonoscopy | |
| CNS | Pituitary mass effect | Headache Hypersomnolence Visual disturbances | CT MRI (with gadolinium) to determine tumor size +/− extrasellar expansion | |
| PNS | Carpal tunnel syndrome | Paresthesias | Median nerve compression | EMG, NCV |
| MS | Bone and soft tissue overgrowth Osteoporosis Myopathy | Arthralgias Osteoarthritis (knees, hips, shoulders, lumbosacral spine) Fatigue, weakness | Enlarged hands and feet Hip, knee, shoulder, low back pain Muscle weakness | X-ray |
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