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Acromegaly

Acromegalia

Acromegaly is characterized by excessive secretion of growth hormone, usually caused by a somatotroph pituitary adenoma.

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Presentation

The diagnosis of acromegaly is frequently delayed due to the slow progression of signs and symptoms. Patients with large tumors may experience symptoms due to the local mass effects of the pituitary lesion. These include headaches, visual abnormalities, typically bitemporal hemianopsia, and cranial nerve palsies. Hypersecretion of prolactin occurs if its inhibitory feedback from the hypothalamus is disturbed. Pituitary adenomas may also result in decreased secretion of other hormones by the pituitary gland.

Oversecretion of GH and IGF-1 causes acral and soft tissue overgrowth with skin thickening and organomegaly leading to arthritis, abnormal development of facial features, diabetes mellitus, hypertension and other cardiovascular diseases, carpal tunnel syndrome, obstructive sleep apnea, and various other symptoms. Patients present with increased foot size due to abnormal development of feet and increased ring size due to excessive fingers growth. Hyperhidrosis may also occur. Increased urinary secretion of calcium and phosphate is also associated with acromegaly.

Soft Tissue Swelling
  • This leads to coarse facial features, soft tissue swelling (including the tongue), enlargement of the hands and feet, respiratory problems, hypertension, diabetes mellitus, carpal tunnel syndrome, and osteoarthritis.[ncbi.nlm.nih.gov]
  • Injections often improve symptoms of soft tissue swelling, headache, joint pains and sleep apnea. Patients require lifelong treatment with somatostatin.[pietromortini.com]
  • Features that result from high level of GH or expanding tumor include: Soft tissue swelling visibly resulting in enlargement of the hands, feet, nose, lips and ears, and a general thickening of the skin Soft tissue swelling of internal organs, notably[en.wikipedia.org]
  • Soft tissue swelling in the back of the throat can cause snoring and sleep apnea, leading to disturbed sleep and daytime sleepiness.[empoweryourhealth.org]
  • Lanreotide and octreotide also cause tumor shrinkage in 30-50% of patients, and improve soft tissue swelling, headache, joint pains and sleep apnea.[pacificneuroscienceinstitute.org]
Galactorrhea
  • She did not complain of recent headaches, vomiting, visual difficulties, or galactorrhea and was clinically euthyroid. Her pulse rate was 84 beats/min, and her blood pressure was 150/90 mmHg. A visual field assessment did not reveal a defect.[ncbi.nlm.nih.gov]
  • In a series of 48 patients with galactorrhea, hyperprolactinemia and the presence of pituitary adenoma, eight patients (17%) had an increased GH level and clinical acromegaly ( 8 ).[edm.bioscientifica.com]
  • Galactorrhea occurs in some women with acromegaly, usually in association with hyperprolactinemia . However, galactorrhea may occur with GH excess alone, because GH itself stimulates lactation.[merckmanuals.com]
  • […] hyperprolactinemia as well tend to have an earlier onset of disease, lesser acromegalic features, and lower GH levels, but also larger tumors. [22] Women with GH-prolactin–secreting adenomas tend to have higher incidences of menstrual disorders and galactorrhea[emedicine.medscape.com]
  • References: [1] [4] Clinical features Tumor mass effects Headache , vision loss ( bitemporal hemianopsia ) , cranial nerve palsies : Oligomenorrhea , secondary amenorrhea , galactorrhea , vaginal atrophy : Erectile dysfunction , decreased libido, testicular[amboss.com]
Coarse Facial Features
  • This leads to coarse facial features, soft tissue swelling (including the tongue), enlargement of the hands and feet, respiratory problems, hypertension, diabetes mellitus, carpal tunnel syndrome, and osteoarthritis.[ncbi.nlm.nih.gov]
  • facial features and progressive enlargement of hands and feet.[ncbi.nlm.nih.gov]
  • Children with gigantism have few soft tissue effects (eg, peripheral edema, coarse facial features), because of their rapid linear growth.[emedicine.medscape.com]
  • A person with acromegaly usually has large hands and feet, thick lips, coarse facial features, a jutting forehead and jaw, and widely spaced teeth. Often people with acromegaly sweat a lot.[hormone.org]
Congestive Heart Failure
  • Its main features are biventricular hypertrophy, diastolic dysfunction, and in later stages, systolic dysfunction and congestive heart failure.[ncbi.nlm.nih.gov]
  • Acromegalic subjects may develop congestive heart failure , particularly when blood pressure becomes high. At times, excessive overgrowth of bone and cartilage involves the joints and causes pain.[britannica.com]
  • heart failure, which may be due to uncontrolled hypertension or to an intrinsic form of cardiomyopathy attributable to excess GH/IGF-I Increased incidence of colonic polyps and adenocarcinoma of the colon In comparison with acromegalic patients with[emedicine.medscape.com]
  • In 1993, star wrestler Andre the Giant, who stood at 7 feet and 5 inches, and weighed 500 pounds, died at age 46 of congestive heart failure caused by acromegaly.[abcnews.go.com]
Tall Stature
  • Convert to ICD-10-CM : 253.0 converts approximately to: 2015/16 ICD-10-CM E22.0 Acromegaly and pituitary gigantism Approximate Synonyms Acromegaly Constitutional tall stature Growth hormone overproduction Hypersomatotropic gigantism Overproduction of[icd9data.com]
  • 2018 2019 Billable/Specific Code Applicable To Constitutional gigantism E34.4 ) constitutional tall stature ( ICD-10-CM Diagnosis Code E34.4 Constitutional tall stature 2016 2017 2018 2019 Billable/Specific Code Applicable To Constitutional gigantism[icd10data.com]
  • The same excess of growth hormone in individuals whose epiphyses have not fused will result in gigantism (excessively tall stature).[radiopaedia.org]
  • The onset of IGF-I hypersecretion in childhood or late adolescence results in tall stature (see the image below). (See Clinical Presentation and Workup.)[emedicine.com]
Sleep Apnea
  • KEYWORDS: Acromegaly; Dyspnea; Obstructive sleep apnea syndrome; Vocal cord paralysis[ncbi.nlm.nih.gov]
  • The aim of the ACROSAHS study was to investigate the prevalence of acromegaly and acromegaly comorbidities in patients with sleep apnea symptoms and acral enlargement.[ncbi.nlm.nih.gov]
  • Gordon Endoscopic findings in sleep apnea associated with acromegaly [22.] P. Chanson, J. Timsit, O.[archbronconeumol.org]
  • Acromegaly Growth Hormone Treatment Sleep Apnea Cardiovascular Diseases Bone Diseases Detailed Description: Acromegaly is a rare disease caused by a growth hormone (GH) producing pituitary adenoma.[clinicaltrials.gov]
Snoring
  • Soft tissue within the body can also enlarge, causing increased snoring, sleep apnea, chronic sinus problems, radiculopathy from nerve root entrapment (i.e., carpal tunnel syndrome), joint pain and stiffness, increase in sweating (hyperhydrosis), and[ccpd.ucsf.edu]
  • Other tissue in the body also can become enlarged, causing symptoms such as: Carpal tunnel syndrome Deepening of the voice Diabetes Excessive sweating and changes in body odor Increased risk of colon polyps Joint pain and stiffness Sleep apnea and snoring[ucsfhealth.org]
  • He snored, but had never been evaluated for obstructive sleep apnea. He also experienced excessive sweating and mild fatigue and his lower jaw had enlarged over the years, increasing the spacing between his teeth.[brighamhealthhub.org]
  • They include the following: Broadening of the nose, lips, ears, and forehead; enlarged tongue and increased spacing between front teeth Headaches Hoarse voice; sleep apnea and loud snoring Growth in hands and feet that requires larger shoes, rings, and[pfizer.com]
Hoarseness
  • Ultimately they cause considerable disability (aside from the need for larger rings, gloves, and shoes) including hoarseness , sleep apnea , joint pain, cardiovascular disease, hypertension, insulin resistance , visual impairment and severe headaches.[medicinenet.com]
  • […] the stool Carpal tunnel syndrome Decreased muscle strength ( weakness ) Decreased peripheral vision Easy fatigue Excessive height (when excess GH production begins in childhood) Excessive sweating Headache Heart enlargement, which can cause fainting Hoarseness[nlm.nih.gov]
  • They include the following: Broadening of the nose, lips, ears, and forehead; enlarged tongue and increased spacing between front teeth Headaches Hoarse voice; sleep apnea and loud snoring Growth in hands and feet that requires larger shoes, rings, and[pfizer.com]
Loud Snoring
  • They include the following: Broadening of the nose, lips, ears, and forehead; enlarged tongue and increased spacing between front teeth Headaches Hoarse voice; sleep apnea and loud snoring Growth in hands and feet that requires larger shoes, rings, and[pfizer.com]
Prognathism
  • Craniofacial soft tissue and skeletal changes including mandibular prognathism and disturbed occlusion are typical manifestations of the disease process.[ncbi.nlm.nih.gov]
  • Facial skeleton elongation and mandibular overgrowth were combined with prognathism, malocclusion and overbite. CONCLUSION: Skull and CCJ alterations are of paramount importance when selecting the surgical approach, if surgery is indicated.[ncbi.nlm.nih.gov]
  • There is a tendency towards mandibular overgrowth with prognathism, maxillary widening, tooth separation and jaw malocclusion. The disease also has rheumatologic, cardiovascular, respiratory and metabolic consequences which determine its prognosis.[orpha.net]
  • Symptoms due to excess of GH/IGF-I These include the following: Soft tissue swelling and enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of[emedicine.medscape.com]
  • The mandible also characteristically enlarges resulting in prognathism and gaps between the teeth 10 . This appearance is sometimes referred to as a "lantern jaw" 11 although this merely a descriptive term, not unique to acromegaly.[radiopaedia.org]
Macroglossia
  • Inspection of the soft tissue revealed only macroglossia, although external palpation indicated a subcutaneous stiffness of the submandibular area.[ncbi.nlm.nih.gov]
  • She was observed to have macroglossia, thickened coarse skin, acral enlargement, and newly detected, uncontrolled diabetes. A diagnosis of acromegaly was suspected.[ncbi.nlm.nih.gov]
  • Macroglossia may cause obstructive sleep apnoea leading to daytime tiredness. Dental changes: separation and jaw malocclusion. Excessive sweating (65%) and thick, oily skin, with development of skin tags. Women may have mild hirsutism.[patient.info]
  • […] hemianopsia ) , cranial nerve palsies : Oligomenorrhea , secondary amenorrhea , galactorrhea , vaginal atrophy : Erectile dysfunction , decreased libido, testicular volume Soft tissue effects Doughy skin texture, hyperhidrosis Deepening of the voice, macroglossia[amboss.com]
  • Facies with prognathism and macroglossia, thick lips, prominent brow ridges (Figure 1), deep voice. Normal on auscultation. Normal fundoscopy. The patient reported no changes in body morphology.[revistanefrologia.com]
Widely Spaced Teeth
  • Enlarged bones in the nose and mouth may cause a large tongue and widely spaced teeth and may lead to sleep apnea. Bone and cartilage growth may lead to arthritis.[stanfordhealthcare.org]
  • spaced teeth Large feet (change in shoe size), large hands (change in ring or glove size) Large glands in the skin (sebaceous glands) causing oily skin, thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with swelling, redness[nlm.nih.gov]
  • Symptoms of acromegaly The main signs of acromegaly are: changes to facial features, such as a broadening nose, enlarging jaw and more widely spaced teeth enlarged lips or tongue enlarged hands and feet A pituitary tumour can also cause a range of other[healthdirect.gov.au]
  • spaced teeth Swelling of the bony areas around a joint Additional symptoms that may be associated with this disease: Weight gain (unintentional) Hair, excessive on females Signs and tests: The level of growth hormone is high.[uclahealth.org]
Hypertension
  • Increased mortality has been related to cardiovascular events that could be linked to these hormones and patients suffer from high rates of diabetes and hypertension.[ncbi.nlm.nih.gov]
  • Author information 1 All India Institute of Medical Sciences, New Delhi, India. [email protected] Abstract Acromegaly has several cardiovascular manifestations of which cardiomyopathy (CMP) and hypertension (HTN) are important and contribute to the[ncbi.nlm.nih.gov]
  • We compared clinical features and serum levels of the growth hormone (GH) and insulin-like growth factor-1 (IGF-1), hypertension (HT) and diabetes mellitus (DM) prevalence between the two groups.[ncbi.nlm.nih.gov]
  • Patients with diabetes mellitus were significantly older with an increased frequency of hypertension (p Georg Thieme Verlag KG Stuttgart · New York.[ncbi.nlm.nih.gov]
  • This leads to coarse facial features, soft tissue swelling (including the tongue), enlargement of the hands and feet, respiratory problems, hypertension, diabetes mellitus, carpal tunnel syndrome, and osteoarthritis.[ncbi.nlm.nih.gov]
Heart Failure
  • Its main features are biventricular hypertrophy, diastolic dysfunction, and in later stages, systolic dysfunction and congestive heart failure.[ncbi.nlm.nih.gov]
  • We report a case of acromegaly in a 41 year old female patient who remained undiagnosed for 6 years and presented to us for the first time with symptoms of heart failure.[ncbi.nlm.nih.gov]
  • Here, we describe a case in which HTN and advanced heart failure resolved in an aged patient within a very short span of 2 months from the time of presentation. Copyright 2012 Cardiological Society of India. Published by Elsevier B.V.[ncbi.nlm.nih.gov]
Cardiomegaly
  • […] include: headache, described more often as "head pain" (due to dural tension) muscle pain, often misdiagnosed as fibromyalgia joint pain arthritis vertebral fractures with or without loss of bone mineral density carpal tunnel syndrome cardiovascular cardiomegaly[radiopaedia.org]
  • […] teeth, and enlarging tongue Carpal tunnel syndrome Arthritis Oily skin and excessive sweating Tiredness, fatigue, depression Impotence, loss of libido Interrupted menstrual cycle (amenorrhea) Lactation (galactorrhea) Heart disease and heart enlargement (cardiomegaly[acromegaly.org]
  • Chest X-ray – may show cardiomegaly and osteoporosis . The rest of pituitary function should be investigated with measurement of prolactin, thyroid function, gonadotropins and sex steroids.[myvmc.com]
  • […] swelling of soft tissue in the hands and feet (onset signs) enlarged bones in the skull, face, jaw, hands and feet joint pains pins and needles in the hands headaches gaps forming between the teeth, which may cause a ‘bad bite’ barrel chest enlarged heart (cardiomegaly[betterhealth.vic.gov.au]
Chest Pain
  • pain, palpitations, SOB, previous MI etc.[medicaleducationleeds.com]
  • Symptoms of slow heart rate may include dizziness or lightheadedness, fainting or near-fainting, chest pain, shortness of breath, confusion or memory problems, and weakness or extreme tiredness.[acromegaly.somatulinedepot.com]
  • Serious symptoms such as shortness of breath or chest pain warrant immediate attention. Exams and Tests Acromegaly is not an easy diagnosis, and it is often missed by health care providers.[emedicinehealth.com]
Arthritis
  • Abstract Spontaneous rupture of flexor tendons within the carpal tunnel is rare in the absence of rheumatoid arthritis.[ncbi.nlm.nih.gov]
  • If not treated, acromegaly can lead to heart problems, arthritis, diabetes, vision problems, high blood pressure, or even death.[cedars-sinai.edu]
  • Bone and cartilage growth may lead to arthritis. People with acromegaly can also develop diabetes, high blood pressure, and other problems. The excess production of growth hormone usually is caused by a tumor of the pituitary gland.[stanfordhealthcare.org]
Arthralgia
  • Must be screened for in the presence of pituitary adenoma, profuse sweating, acral growth, coarsening of facial features, and when suspected in conjunction with commonly associated conditions, such as carpal tunnel syndrome, arthralgia, glucose intolerance[bestpractice.bmj.com]
  • Clinical features of acromegaly include soft tissue overgrowth, sweating, headaches and associated with hypertension, diabetes or glucose intolerance, arthralgia and sleep apnea syndrome: prolonged exposure to elevated levels of GH and IGF-I lead to both[ci.nii.ac.jp]
  • The diagnosis of acromegaly, frequently made late because of the insidious nature of the disease, is generally based on symptoms of excess growth hormone, such as acral enlargement, soft-tissue swelling, arthralgia, jaw prognathism, hyperhidrosis, osteoarthritis[erj.ersjournals.com]
  • Common side effects of Somatuline Depot include diarrhea, cholelithiasis, abdominal pain, nausea, injection site reactions, flatulence, arthralgia, and loose stools.[acromegaly.org]
Large Feet
  • […] begins in childhood) Excessive sweating Headache Heart enlargement, which can cause fainting Hoarseness Joint pain , limited joint movement, swelling of the bony areas around a joint Large bones of the face, large jaw and tongue, widely spaced teeth Large[nlm.nih.gov]
  • Symptoms include body odor, easy fatigue, headache, large feet, excessive height, swelling of the bony areas around a joint, widely spaced teeth and sleep apnea. Treatment includes surgery, dopamine agonists and growth hormone antagonists.[gii.co.jp]
Feet Enlargement
  • "Living with Acromegaly Reset the Balance Improve Your Outlook" View and print brochure Symptoms and signs may include: Soft tissue thickening of the palms of the hands and soles of the feet Enlargement of hands (ring size), feet (shoe size) and head[acromegaly.org]
  • Symptoms and signs may include: Soft tissue thickening of the palms of the hands and soles of the feet Enlargement of hands (ring size), feet (shoe size) and head (hat size) Enlargement of forehead (frontal bossing) and jaw (prognathism) with pronounced[acromegaly.org]
Hand Enlargement
  • Symptoms: Enlarged hands Enlarged feet Widened fingers or toes due to skin overgrowth with swelling, redness, and pain Enlarged jaw (prognathism) and tongue Enlarged facial bones Thickening of the skin, skin tags Enlarged sebaceous glands Hoarseness Headache[uclahealth.org]
Hirsutism
  • […] skin (sebaceous glands) causing oily skin, thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with swelling, redness, and pain Other symptoms that may occur with this disease: Colon polyps Excess hair growth in females ( hirsutism[nlm.nih.gov]
  • Acromegaly also causes the Sign of Leser-Trelat (i.e., the eruption of multiple lesions of seborrheic keratosis). 1,2,6-9 Some cutaneous manifestations, such as hirsutism and sebaceous hypersecretion, may result from the free testosterone carrier protein[scielo.br]
  • Others: prominent supraorbital ridge, hirsutism, thick greasy skin, carpal tunnel syndrome, visual field defects, galactorrhoea, hypertension, oedema, heart failure , arthropathy, proximal myopathy, glycosuria.[myvmc.com]
Hyperhidrosis
  • The diagnosis of acromegaly, frequently made late because of the insidious nature of the disease, is generally based on symptoms of excess growth hormone, such as acral enlargement, soft-tissue swelling, arthralgia, jaw prognathism, hyperhidrosis, osteoarthritis[erj.ersjournals.com]
  • Hyperhidrosis may also occur. Increased urinary secretion of calcium and phosphate is also associated with acromegaly. Measurement of: GH GHRH TRH Oral glucose IGF-1 Prolactin Imaging studies include MRI, CT scanning and radiography.[symptoma.com]
  • Symptoms due to excess of GH/IGF-I These include the following: Soft tissue swelling and enlargement of extremities Increase in ring and/or shoe size Hyperhidrosis Coarsening of facial features Prognathism Macroglossia Arthritis Increased incidence of[emedicine.medscape.com]
  • Headache , vision loss ( bitemporal hemianopsia ) , cranial nerve palsies : Oligomenorrhea , secondary amenorrhea , galactorrhea , vaginal atrophy : Erectile dysfunction , decreased libido, testicular volume Soft tissue effects Doughy skin texture, hyperhidrosis[amboss.com]
  • […] thickened and may feel doughy Thick and hard nails Deepening of forehead creases and skin folds on the face Noticeably large pores, acne-like comedones (blackheads) and thick swollen eyelids Increased body hair ( hypertrichosis ), excessive sweating ( hyperhidrosis[dermnetnz.org]
Skin Thickening
  • […] movement, swelling of the bony areas around a joint Large bones of the face, large jaw and tongue, widely spaced teeth Large feet (change in shoe size), large hands (change in ring or glove size) Large glands in the skin (sebaceous glands) causing oily skin[nlm.nih.gov]
  • Hands and feet become enlarged; facial features are exaggerated as the jaw lengthens and the nose and forehead grow thicker; the skin thickens; and most internal organs enlarge.[britannica.com]
  • Oversecretion of GH and IGF-1 causes acral and soft tissue overgrowth with skin thickening and organomegaly leading to arthritis, abnormal development of facial features, diabetes mellitus, hypertension and other cardiovascular diseases, carpal tunnel[symptoma.com]
  • Symptoms may include: swelling of the hands and feet facial features become coarse as bones grow body hair becomes coarse as the skin thickens and/or darkens increased perspiration accompanied with body odor protruding jaw voice deepening enlarged lip[hopkinsmedicine.org]
Hypertrichosis
  • Oily skin with large pores, hypertrichosis, and excessive sweating are common features. Pigmented skin tags, acanthosis nigricans, and psoriasis are also encountered.[ncbi.nlm.nih.gov]
  • (found in approximately one half of acromegaly patients): Unlike virilizing disorders, hypertrichosis of acromegaly does not affect the beard area Oily skin (acne is not common) Hyperpigmentation (40% of patients) Acanthosis nigricans (a small percentage[emedicine.medscape.com]
  • Enlarged swollen feet and hands The skin is thickened and may feel doughy Thick and hard nails Deepening of forehead creases and skin folds on the face Noticeably large pores, acne-like comedones (blackheads) and thick swollen eyelids Increased body hair ( hypertrichosis[dermnetnz.org]
  • […] the skin (including cutis verticis gyrata ) internal organs (especially heart and kidneys) vocal cords, resulting in a characteristic thick, deep voice and slowing of speech skull, frontal bossing mandible: prognathism with gaping teeth skin changes hypertrichosis[radiopaedia.org]
  • […] speech Generalized expansion of the skull at the fontanelle Pronounced brow protrusion, often with ocular distension (frontal bossing) Pronounced lower jaw protrusion (prognathism) with attendant macroglossia (enlargement of the tongue) and teeth spacing Hypertrichosis[en.wikipedia.org]
Hyperpigmentation
  • […] face Noticeably large pores, acne-like comedones (blackheads) and thick swollen eyelids Increased body hair ( hypertrichosis ), excessive sweating ( hyperhidrosis ), and oily skin ( seborrhoea ) Increased number of skin tags and darkening of the skin ( hyperpigmentation[dermnetnz.org]
  • […] including cutis verticis gyrata ) internal organs (especially heart and kidneys) vocal cords, resulting in a characteristic thick, deep voice and slowing of speech skull, frontal bossing mandible: prognathism with gaping teeth skin changes hypertrichosis hyperpigmentation[radiopaedia.org]
  • Hyperpigmentation occurs in roughly 40% of cases and almost always in photoexposed areas. It is probably due to the associated increase of melanotrophic hormone. Acanthosis negricans occurs in 10% of cases. Hypertrophic scarring is common.[scielo.br]
  • […] no conclusive studies exist to substantiate this finding Hypertrichosis (found in approximately one half of acromegaly patients): Unlike virilizing disorders, hypertrichosis of acromegaly does not affect the beard area Oily skin (acne is not common) Hyperpigmentation[emedicine.medscape.com]
  • […] expansion of the skull at the fontanelle Pronounced brow protrusion, often with ocular distension (frontal bossing) Pronounced lower jaw protrusion (prognathism) with attendant macroglossia (enlargement of the tongue) and teeth spacing Hypertrichosis, hyperpigmentation[en.wikipedia.org]
Frontal Bossing
  • The forehead and overlying skin is thickened, sometimes leading to frontal bossing. There is a tendency towards mandibular overgrowth with prognathism, maxillary widening, tooth separation and jaw malocclusion.[orpha.net]
  • Markers Typically shows elevated levels of: growth hormone IGF-1 (insulin growth factor 1) Plain radiograph Skull Calvarial thickening, particularly of the inner table 10 , frontal bossing, enlarged paranasal sinuses (especially frontal sinuses) and an[radiopaedia.org]
  • Untreated acromegaly results in marked bony and soft tissue changes including an altered facial appearance (frontal bossing, prognathism), enlargement of the hands and feet, sleep apnea, and carpal tunnel syndrome.[acromegaly.org]
  • Symptoms In acromegaly, elevated growth hormone causes an increase in shoe and ring size, enlargement of the mandible leading to an underbite, frontal bossing, and enlargement of the nose.[ccpd.ucsf.edu]
Thick Lips
  • The facial aspect is characteristic and includes a widened and thickened nose, prominent cheekbones, forehead bulges, thick lips and marked facial lines. The forehead and overlying skin is thickened, sometimes leading to frontal bossing.[orpha.net]
  • A person with acromegaly usually has large hands and feet, thick lips, coarse facial features, a jutting forehead and jaw, and widely spaced teeth. Often people with acromegaly sweat a lot.[hormone.org]
  • Facies with prognathism and macroglossia, thick lips, prominent brow ridges (Figure 1), deep voice. Normal on auscultation. Normal fundoscopy. The patient reported no changes in body morphology.[revistanefrologia.com]
Nose Enlargement
  • Symptoms of acromegaly The main signs of acromegaly are: changes to facial features, such as a broadening nose, enlarging jaw and more widely spaced teeth enlarged lips or tongue enlarged hands and feet A pituitary tumour can also cause a range of other[healthdirect.gov.au]
  • Due to excess of GH: Gradual change in appearance due to the effects on cartilage and soft tissues: enlargement of hands and feet (increase in ring and shoe size), frontal bossing, thickening of the nose, enlarged tongue (macroglossia), growth of the[patient.info]
Mandibular Prognathism
  • Craniofacial soft tissue and skeletal changes including mandibular prognathism and disturbed occlusion are typical manifestations of the disease process.[ncbi.nlm.nih.gov]
Headache
  • This highlights the use of monthly pasireotide in resolving headaches and improved biochemical control in a patient with acromegaly.[ncbi.nlm.nih.gov]
  • Furthermore, unrelated analgesic and biochemical effects of SSAs supported a complex pathogenesis of acromegaly-associated headache.[ncbi.nlm.nih.gov]
  • It has been established in a prospective study that the size of a pituitary tumor does not determine the headache presentation. 2 Moreover, the phenotype of headache presentations is wide, including migraine and the trigeminal autonomic cephalalgias,[nejm.org]
  • Abstract A young man with subtle clinical features suggestive of hypersomatotropism presented with acute-onset severe headache. Relevant investigations confirmed polycythaemia and growth hormone (GH)-secreting pituitary macroadenoma with apoplexy.[ncbi.nlm.nih.gov]
  • She did not complain of recent headaches, vomiting, visual difficulties, or galactorrhea and was clinically euthyroid. Her pulse rate was 84 beats/min, and her blood pressure was 150/90 mmHg. A visual field assessment did not reveal a defect.[ncbi.nlm.nih.gov]
Bitemporal Hemianopsia
  • In addition, bitemporal hemianopsia was observed on campimetry. After further examination, the patient was diagnosed with acromegaly. 2016 Japan Society of Obstetrics and Gynecology. KEYWORDS: acromegaly; amenorrhea; postpartum[ncbi.nlm.nih.gov]
  • These include headaches, visual abnormalities, typically bitemporal hemianopsia, and cranial nerve palsies. Hypersecretion of prolactin occurs if its inhibitory feedback from the hypothalamus is disturbed.[symptoma.com]
  • References: [1] [4] Clinical features Tumor mass effects Headache , vision loss ( bitemporal hemianopsia ) , cranial nerve palsies : Oligomenorrhea , secondary amenorrhea , galactorrhea , vaginal atrophy : Erectile dysfunction , decreased libido, testicular[amboss.com]
  • Vision problems can include: A loss of the outer peripheral vision, called a bitemporal hemianopsia When severe, a patient can only see what is directly in front of them Many patients do not become aware of their visual loss until it is quite severe.[pietromortini.com]
  • Headache Superior Spread leads to compression of the Optic Chiasm leading to bitemporal hemianopsia . Inferior Spread leads to sphenoid sinus invasion. Lateral Spread leads to Cavernous Sinus invasion.[theartofmed.wordpress.com]
Amenorrhea
  • KEYWORDS: acromegaly; amenorrhea; postpartum[ncbi.nlm.nih.gov]
  • References: [1] [4] Clinical features Tumor mass effects Headache , vision loss ( bitemporal hemianopsia ) , cranial nerve palsies : Oligomenorrhea , secondary amenorrhea , galactorrhea , vaginal atrophy : Erectile dysfunction , decreased libido, testicular[amboss.com]
  • This can cause symptoms such as diminished sexual function, loss of menstrual periods (amenorrhea), and symptoms of an underactive thyroid gland. A pituitary problem diagnosed based upon these symptoms before the symptoms of acromegaly are apparent.[empr.com]
  • ) and jaw (prognathism) with pronounced under- or overbite, spreading teeth, and enlarging tongue Carpal tunnel syndrome Arthritis Oily skin and excessive sweating Tiredness, fatigue, depression Impotence, loss of libido Interrupted menstrual cycle (amenorrhea[acromegaly.org]

Workup

Measurement of:

  • GH
  • GHRH
  • TRH
  • Oral glucose
  • IGF-1
  • Prolactin

Imaging studies include MRI, CT scanning and radiography. Radiography reveals increase in length and thickness of certain bones like mandible and ribs. Mainly membranous bones are increased in size in radiographs of acromegalic patients. Increased cartilage growth is also evident. Histological findings are also important diagnostic tool and reveal different types of tumors in acromegaly.

Hyperprolactinemia
  • The data support the hypothesis that hyperprolactinemia may have contributed to stimulating somatomedin C and sustaining the normal growth rate in this child. acromegaly growth hormone pituitary adenoma hyperprolactinemia Received December 31, 1987.[pediatrics.aappublications.org]
  • CONCLUSION: The most important factors affecting gonadal functions, excluding central hypogonadism, are hyperprolactinemia and the duration of the indolent period before diagnosis of acromegaly.[ncbi.nlm.nih.gov]
  • The objective of this study was to investigate the effect of hyperprolactinemia and high levels of insulin-like growth factor-I (IGF-I) on bone resorption and their relation with receptor activator of nuclear factor-κB ligand (RANKL) and osteoprotegerin[ncbi.nlm.nih.gov]
  • Tumor damage to the pituitary stalk may cause hyperprolactinemia due to loss of inhibitory regulation of prolactin secretion by the hypothalamus.[emedicine.medscape.com]
  • Medication-induced hyperprolactinemia and hypothyroidism were excluded. Initial head CT demonstrated a 2 mm pituitary microadenoma and a partial empty sella.[edm.bioscientifica.com]
Hypertriglyceridemia
  • […] of facial features Prognathism Macroglossia Arthritis Increased incidence of obstructive sleep apnea Increased incidence of glucose intolerance or frank diabetes mellitus, hypertension, and cardiovascular disease Hyperphosphatemia, hypercalcuria, and hypertriglyceridemia[emedicine.medscape.com]
  • A 2009 study suggests that in patients with acromegaly, insulin resistance and hyperinsulinemia are positively correlated with the level of disease activity. [17] Hypertriglyceridemia is found in 19-44% of patients.[emedicine.com]
Polyps
  • Among the 146 ACRO patients, 68% were subjected to at least one colonoscopy and in 32% of the cases a polyp was detected during the procedure.[ncbi.nlm.nih.gov]
  • Twelve patients had adenoma (41.4%), eight patients had hyperplastic polyps (27.6%), three patients had sessile serrated adenoma/polyps (10.3%), and three patients had colon cancer (10.3%).[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Benign neoplasms were found in majority of patients with acromegaly (71.6%) most notably: nodular goiter and colon polyps; malignant lesions were rare (4.5%). Only every fifth patient suffered from no neoplastic proliferations.[ncbi.nlm.nih.gov]
  • Colonic polyps. Vertebral fractures, possibly due to low-quality bone despite high bone mass.[patient.info]
  • […] ring or glove size) Large glands in the skin (sebaceous glands) causing oily skin, thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with swelling, redness, and pain Other symptoms that may occur with this disease: Colon polyps[nlm.nih.gov]

Treatment

Acromegaly can be treated surgically as well as by medications. Surgical removal of adenomas is quite successful nowadays [11] [12]. If surgery is not successful regarding GH hypersecretion then radiation therapy is used. Medications include mostly analogues of somatostatin like octreotide, which decrease the secretion of growth hormones and IGF-1, because somatostatin has an inhibitory effect on GH [13]. Dopamine agonists like bromocriptine are also employed as a pharmacological treatment because they bind to dopamine type 2 receptors in the pituitary gland and cause depressed secretion of GH [14].

Transsphenoidal surgery is used to completely remove the tumor. It is an efficient procedure and uses nasal approach to remove the tumor. After treatment, patients should be monitored throughout their life for their GH and IGF-1 levels. Also, patients should be evaluated for severe GH deficiency after surgery [15].

Prognosis

Mortality and morbidity rates are increased in acromegaly. Increased secretion of GH and IGF-1 has somatic and metabolic effects affecting all organ systems and patients may develop sleep apnea, hypertension, cardiomyopathy, nerve root compression, carpal tunnel syndrome, diabetes mellitus, and various other cerebrovascular, cardiovascular, and respiratory disorders [10]. In addition, the pituitary lesion may cause symptoms including headache and visual field defects. Early diagnosis and management of complications improves the long-term outcome [10].

Etiology

Acromegaly is caused by:

  • Increased secretion of growth hormone (GH) caused by pituitary adenomas in most cases.
  • Hypothalamic tumors which result in increased secretion of growth hormone releasing hormone (GHRH).
  • IGF-1 (insulin-like growth factor 1) oversecretion, which is a growth regulating substance produced by the liver under the effect of growth hormone [2].
  • In some instances, tumors of the adrenal glands or lungs may cause ectopic secretion of GH, rarely of GHRH.

Epidemiology

The prevalence of acromegaly in Europe has been reported to be 30-70:1,000,000 [3] [4], however, recent studies observed higher numbers [4] [5] [6]. Acromegaly affects both males and females equally. The mean age for diagnosis is 40 years in males and 45 years in females.

Sex distribution
Age distribution

Pathophysiology

Growth hormone, which is released by the adenohypophysis, is regulated by the hypothalamus through growth hormone releasing hormone and somatostatin. GH exhibits its regulatory effects by insulin-like growth factor I, which is produced by the liver under the influence of GH. Most cases of acromegaly are caused by pituitary adenomas, resulting in increased secretion of GH and insulin-like growth factor I subsequently. Both cause increased growth of soft tissues, bones and viscera. Because somatostatin decreases the secretion of GH, drugs mimicking the actions of somatostatin are given to acromegalic patients to decrease GH.

Extrapituitary tumours including neuroendocrine tumors are rare causes and may also increase the secretion of GH in the same way and causing the same results [7] [8] [9]. Tumors associated with GHRH production such intracranial gangliocytomas and disruption of the somatostatin feedback pathway also lead to acromegaly.

Prevention

Acromegaly can not be prevented. Early diagnosis should is important to avoid severe complications.

Summary

Acromegaly is most often caused by adenomas of the pituitary gland. If the tumour occurs before puberty, linear growth increases, a condition known as pituitary gigantism. After adolescence, when the fusion of the epiphyseal growth plates has occured, acromegaly develops. The disorder causes acral and soft tissue overgrowth, characterized by markedly increased growth of hands and feet. Also, bones and viscera increase in size including nose, cranium, supraorbital ridges, forehead and vertebrae, causing symptoms such as macrognathia, kyphosis, goiter and left ventricular hypertrophy [1].

Patient Information

Definition

Acromegaly is usually caused by a tumor of the pituitary gland which results in increased growth hormone secretion. This leads to secondary effects due to growth hormone overproduction.

Cause

The common cause of acromegaly is a pituitary adenoma that causes increased growth hormone (GH) and subsequent insulin-like growth factor 1 (IGF-1) secretion.

Signs and symptoms

The symptoms by which the acromegaly patient can be recognized are increased bone growth, hunched back, increased blood pressure, protrusion of jaw and forehead and increased growth of extremities.

Diagnosis

Diagnosis is made by measuring levels of GH and IGF-1 in the blood. Also radiographic and other imaging techniques are used.

Treatment

Acromegaly can be treated medically as well as surgically. Medical treatment is by use of somatostatin analogues, dopamine agonists and growth hormone antagonists. Radiation is also used.

References

Article

  1. Giustina A, Chanson P, Bronstein MD, Klibanski A, Lamberts S, Casanueva FF, et al. A consensus on criteria for cure of acromegaly. J Clin Endocrinol Metab. Jul 2010;95(7):3141-8.
  2. Soares BS, Eguchi K, Frohman LA. Tumor deletion mapping on chromosome 11q13 in eight families with isolated familial somatotropinoma and in 15 sporadic somatotropinomas. J Clin Endocrinol Metab. Dec 2005;90(12):6580-7.
  3. Bengtsson BA, Edén S, Ernest I, et al. Epidemiology and long-term survival in acromegaly. A study of 166 cases diagnosed between 1955 and 1984. Acta Med Scand 1988; 223:327.
  4. Ribeiro-Oliveira A Jr, Barkan A. The changing face of acromegaly--advances in diagnosis and treatment. Nat Rev Endocrinol 2012; 8:605.
  5. Fernandez A, Karavitaki N, Wass JA. Prevalence of pituitary adenomas: a community-based, cross-sectional study in Banbury (Oxfordshire, UK). Clin Endocrinol (Oxf) 2010; 72:377.
  6. Daly AF, Rixhon M, Adam C, et al. High prevalence of pituitary adenomas: a cross-sectional study in the province of Liege, Belgium. J Clin Endocrinol Metab 2006; 91:4769.
  7. Melmed S, Ezrin C, Kovacs K, et al. Acromegaly due to secretion of growth hormone by an ectopic pancreatic islet-cell tumor. N Engl J Med 1985; 312:9.
  8. Beuschlein F, Strasburger CJ, Siegerstetter V, et al. Acromegaly caused by secretion of growth hormone by a non-Hodgkin's lymphoma. N Engl J Med 2000; 342:1871.
  9. Altstadt TJ, Azzarelli B, Bevering C, et al. Acromegaly caused by a growth hormone-releasing hormone-secreting carcinoid tumor: case report. Neurosurgery 2002; 50:1356.
  10. Melmed S, Casanueva FF, Klibanski A, Bronstein MD, Chanson P, Lamberts SW, et al. A consensus on the diagnosis and treatment of acromegaly complications. Pituitary. 2013 Sep. 16(3):294-302.
  11. Ross DA, Wilson CB. Results of transsphenoidal microsurgery for growth hormone-secreting pituitary adenoma in a series of 214 patients. J Neurosurg 1988; 68:854.
  12. Fahlbusch R, Honegger J, Buchfelder M. Surgical management of acromegaly. Endocrinol Metab Clin North Am 1992; 21:669.
  13. Shimon I, Yan X, Taylor JE, et al. Somatostatin receptor (SSTR) subtype-selective analogues differentially suppress in vitro growth hormone and prolactin in human pituitary adenomas. Novel potential therapy for functional pituitary tumors. J Clin Invest 1997; 100:2386.
  14. Sandret L, Maison P, Chanson P. Place of cabergoline in acromegaly: a meta-analysis. J Clin Endocrinol Metab. 2011 May. 96(5):1327-35.
  15. Ronchi CL, Giavoli C, Ferrante E, et al. Prevalence of GH deficiency in cured acromegalic patients: impact of different previous treatments. Eur J Endocrinol 2009; 161:37.

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Last updated: 2017-08-09 17:55