ACROMEGALY PAED

8/7/2019 ACROMEGALY PAED

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ACROMEGALYACROMEGALYACROMEGALYACROMEGALY

To be reported by:To be reported by:Mr. Elder Pascual PaedMr. Elder Pascual Paed

To be reported to:To be reported to:

Mr. Mikhail ´Miloµ AlmirolMr. Mikhail ´Miloµ Almirol



February 12. 201102:15:45 am 2

Definition

� is a syndrome that results when the

pituitary gland produces excessgrowth hormone after epiphysealplate closure at puberty.



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� Facial aspect of a patient with acromegaly.

The cheekbones are obvious, the foreheadbulges, the jaw is enlarged and facial linesprominent. The forehead and overlying skinis thickened, which may lead to frontalbossing.



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� As compared with the hand of a typicalperson (left), the hand of a patient withacromegaly (right) is enlarged, the fingersare widened, thickened and stubby, andthe soft tissue is thickened.



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� Mandibular overgrowth leads toprognathism maxillary widening, teethseparation and jaw malocclusion.



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� Photo of teeth showing classic teethgapping due to acromegaly on lower

jaw.



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Several Types� Pituitary-tumor-caused acromegaly

� Non-pituitary-tumor-caused

acromegaly� Ectopic acromegaly

� Acromegaly due to growth hormone-

secreting pituitary adenoma



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Incidence Rate:

� 816 per year, 68 per month, 15 perweek, 2 per day, 0 per hour, 0 perminute, 0 per second



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Prevalence

� Estimated 40-60 people per 1 millionpopulation have acromegaly in the US



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Risk Factors



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Causes� Pituitary adenoma - the overproduction of

growth hormones is caused by a benign

tumor of the pituitary gland.

� Other tumors - acromegaly is caused notby pituitary tumors but by tumors of thepancreas, lungs, and adrenal glands.



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Causes� Pituitary gigantism - This condition of

growth hormone excess is rare in children

and is referred to as pituitary gigantism,because the excessive growth hormoneproduces excessive growth of bones andthe child can achieve excessive height;

from 2.1 to 2.7 m (6'11" to 8'11") instature by adulthood if left untreated.



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Pathophysiology

Increased GHRH fromhypothalamus.

Hypothalamus Willstimulate pituitary torelease GH

GH act to release IGF-1 from

Liver that helps on growth anddevelopment.

If decrease canlead tohyperplasia ofchondrocytesand it will

differentiateinto bones andalso to othertissue

Will lead to

hyperplasia andhypertrophy



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Signs and Symptoms

� Soft tissue swelling visibly resulting

in enlargement of the hands, feet,nose, lips and ears, and a generalthickening of the skin.



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Signs and Symptoms

� Soft tissue swelling of internalorgans, notably the heart with

attendant weakening of itsmuscularity, and the kidneys, also thevocal cords resulting in a

characteristic thick, deep voice andslowing of speech



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Signs and Symptoms� Generalized expansion of the skull at

the fontanelle

� Pronounced lower jaw protrusion withattendant macroglossia (enlargementof the tongue) and teeth gapping



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Signs and Symptoms� Mild Symptoms - initially, the onset of acromegaly

is often slow and insidious� Headaches

� blurred vision� enlarged hands� enlarged feet� bone overgrowth

� swollen hands� swollen feet



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Priority Nursing

Diagnosis



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Common Complication� Severe headache

� Enlarged heart� Hypertension� DM� Heart Failure� Kidney failure

� Compression of the optic chiasm leading to loss ofvision in the outer visual fields (typically bitemporalhemianopia)

� Increased palmar sweating and sebum productionover the face (seborrhea) are clinical indicators of

active growth hormone (GH) producing pituitarytumors. These symptoms can also be used to monitorthe activity of the tumor after surgery althoughbiochemical monitoring is confirmatory.



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Diagnostic Tests� Medical imaging

� Medical laboratory

� CT scan - of pituitary or otherorgans, seeking the tumor

� GHRH blood test - useful to detect

non-pituitary tumors



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Diagnostic Tests� Other pituitary hormones have to be

assessed to address the secretory effects

of the tumor as well as the mass effect ofthe tumor on the normal pituitary gland.They include TSH .

� IGF1 provides the most sensitive and

useful lab test for the diagnosis ofacromegaly.



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Diagnostic Tests� Growth hormone blood test (fasting)

� Oral glucose tolerance test

� MRI scan - of pituitary or otherorgans, seeking the tumor



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Managements� Medical Managements

� bromocriptine (Parlodel®).

(uses to reduce both GH secretion andtumor size. )

� octreotide (Sandostatin®).

is a synthetic form of a brain hormone,somatostatin, that stops GH production.



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Surgical

� Endonasal Transphenoidal surgery - reaching thepituitary through an incision in the nasal cavitywall. The wall is reached by passing through thenostrils with microsurgical instruments.

� Transphenoidal surgery - during which an incisionis made into the gum beneath the upper lip.Further incisions are made to cut through theseptum to reach the nasal cavity, where the

pituitary is located.



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ebruary 12. 201102:15:45 am 25

Nursing Management

� Instruct the client to take bromocriptineorally.� octreotide must be injected under the skin

every 8 hours for effective treatment.

� Provide safety� Physical Assessment� Monitor Vital Signs� Monitor I & O·s



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ebruary 12. 201102:15:45 am 26

Preventions

� None, but early treatment mayprevent complications of the disease

from getting worse.



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ebruary 12. 201102:15:45 am 27

Reference� Acromegaly and Gigantism". Merck.com.

http://www.merck.com/mmhe/sec13/ch162/ch162e.html .Retrieved 2010-10-26.

� Link text, Historical footnote in regards to Marfan's.

� Wikipedia.com� Ask.com� Googlephilippines.com� About.com



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ebruary 12. 201102:15:45 am 28

Q&A� 1. is a syndrome that results when the

pituitary gland produces excess growthhormone after epiphyseal plate closure at

puberty.

A. Macromegaly SyndromeB. Acromegaly Syndrome

C. Micromegaly SyndromeD. NOTA



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ebruary 12. 201102:15:45 am 29

Answer to 1B. Acromegaly - is a syndrome that

results when the pituitary gland

produces excess growth hormoneafter epiphyseal plate closure atpuberty.



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30

2. initially, the onset of acromegaly isoften slow and insidious

A. Primary SymptomsB. Slight Symptoms

C. Mild SymptomsD. Severe Symptoms



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Answer to 2.� C. Mild Symptoms - initially, the

onset of acromegaly is often slow and

insidious



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� 3. during which an incision is made intothe gum beneath the upper lip. Further

incisions are made to cut through theseptum to reach the nasal cavity,where the pituitary is located.

A. Transphenoidal surgeryB. Endotracheal surgeryC. Pituitary Imagery Surgery

D. Endonasal Transphenoidal surgery



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Answer to 3� A. Transphenoidal surgery -

during which an incision is made into

the gum beneath the upper lip.Further incisions are made to cutthrough the septum to reach the nasalcavity, where the pituitary is located.



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� 4. Mandibular overgrowth leads to _________,__________, and _________.

A. prognathiasm mandilary sharpening, teethdarkening, jaw lock

B. prognosism maximal shortening, teethovergrowth, jaw malocclusion

C. prognathism mandilary widening, teethseparation, jaw malocclusion

D. prognathism mandilary obstruction, teethseparation, jaw formation



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Answer to 4� C. Mandibular overgrowth leads to

prognathism maxillary widening,

teeth separation and jawmalocclusion.



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5. Which of the following is/are theeffect/s to the teeth of the pateinthaving acromegaly syndrome in the lower

jaw.

A. Whitening of the teethB. Yellow Forming Spots on the teeth

C. Darkening of the gums and teethD. Gapping Of the teeth



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Answer to 5� D. Gapping Of the teeth

� Photo of teethshowing classicteeth gapping

due toacromegaly onlower jaw.



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THANK YOU!

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ACROMEGALY PAED