panhypopituitarism

Pattawan Wongwijitsook

Maharat Nakhon Ratchasima hospital

17 Nov 2013

PITUITARY GLAND

(HYPOPHYSIS CEREBRI)

The master of endocrine glandsmaster of endocrine glands

It is a small oval structure 1 cm in diameter.

PITUITARY GLAND

X-RAY SKULL: LATERAL VIEW SAGITTAL SECTION OF HEAD & NECK

Hypophyseal fossa

Sphenoidal air sinus

Pituitarygland

A fold of dura mater (Diaphragma sellae) covers the pituitary

gland & has an opening for passage of infundibulum

(pituitary stalk) connecting the gland to hypothalamus.

BLOOD SUPPLY OF PITUITARY GLAND

ARTERIES: ARTERIES: Superior & inferior hypophyseal arteries

(branches of internal carotid artery)

VEINS:VEINS: Hypophyseal veins drain into Cavernous Sinuses.

PITUITARY GLAND

• Anterior pituitary

• Posterior pituitary

ANTERIOR PITUITARY CELLS

Anterior pituitary cell hormone effect

somatotrophs GH -Stimulate general body growth

-Regulate metabolism

thyrothrophs TSH -control secretion and others

activity of thyroid gland

gonadotrophs FSH

LH-Stimulate secretion of estrogen

and progesterone

- secretion of testosterone

- maturation of oocyte in ovary

and sperm in testes

Pituitary society

Anterior pituitary cells hormone effect

Lactothrophs prolactin - Initiated milk

production in

mammary gland

corticothrophs ACTH

MSH

-Stimulate adrenal

cortex secrete

glucocorticoid

-Affect skin

pigmentation

Posterior pituitary

(storage from hypothalamus)

Oxytocin

ADH

-Contract uterine

(delivery)

- milk inject (breast

feeding)

- control body water

balancePituitary society

•

Harrison’s 18th edition

hypopituitary

• Definition

Partial or complete deficiency of one or

more pituitary hormone.

It may arises as a congenital defect during the

development of the pituitary gland or as a result of acquired

disease of the pituitary gland . The parasellar structures , or

the hypothalamus.

Williams Textbook of endocrinology 10 edition

Epidermiology

• Prevalence 45 cases per 100,000

• Incidence of about 4 cases per 100,000 per

year in the normal population.

• Pituitary adenomas or incidentalomas found

27% of postmortem studies

10% of MRI studies

Williams Textbook of endocrinology 10 edition

Epidermiology

• No specific gender , ethnic , geographic or age

group

• Associated 1.8 fold higher mortality compared

with an age- and sex- match population.

• Cardiovascular and cerebrovascular death rate

are higher

Williams Textbook of endocrinology 10 edition , pituitary society

hypopituitary

• The clinical presentation of anterior pituitary

Acquired pituitary insufficiency

- degree of hormone deficiency

- the number of hormone impaired

- the rapidity of onset

Williams Textbook of endocrinology 10 edition

hypopituitary

• The clinical presentation of anterior pituitary

Congenital pituitary insufficiency

- the eariler the age of onset

- the greater thr severity of thyroid ,

gonadal , adrenal , growth , or water

disturbance

hypopituitary

• The resilience of the individual pituitary cell

lineages in the presence of each causes also

differs.

• Prolactin deficiency is rare except for

complete pituitary destruction or genetic

syndrome

Williams Textbook of endocrinology 10 edition

hypopituitary

• The lactotrophs cell is often hyperfunctional

as a result of decrased tonic inhibitory signals.

• GH was diminished function prior than others

trophic hormones.

• The corticotrophs and thyrotrophs cell are

usaully the last to lost function.

Williams Textbook of endocrinology 10 edition

Hypopituitary causes

Symptom of hypopituitarism

Symptom of hypopituitarism

Gonadotropin Deficiency

Women

• Oligomenorrhea or

amenorrhea

• Loss of libido

• Vaginal dryness or

dyspareunia

• Loss of secondary sex

characteristics (estrogen

deficiency)

Men

• Loss of libido

• Erectile dysfunction

• Infertility

• Loss of secondary sex characteristics (testosterone deficiency)

• Atrophy of the testes

• Gynecomastia (testosterone deficiency)

ACTH Deficiency

• Results in hypocortisolism

– Malaise

– Anorexia

– Weight-loss

– Gastrointestinal disturbances

– Hyponatremia

• Pale complexion

– Unable to tan or maintain a tan

• No features of mineralocorticoid deficiency

– Aldosterone secretion unaffected

TSH Deficiency

• Hypothyroidism

• Atrophic thyroid gland

Prolactin Deficiency

• Inability to lactate postpartum

• Often 1st manifestation of Sheehan syndrome

Growth Hormone Deficiency

• Adults– Often asymptomatic

– May complain of

– Fatigue

– Degrees exercise tolerance

– Abdominal obesity

– Loss of muscle mass

• Children– GH Deficiency

– Constitutional growth delay

hypopituitary

• Two thirds of pituitary macroadenoma ,

craniopharyngioma , and others parasellar lesion �

pituitary reserve function.

• Intrasellar aneurysm , pituitary metastasis ,

parasellar meningioma , optic glioma , hypothalamic

astrocytoma � pituitary failure

Williams Textbook of endocrinology 10 edition

hypopituitary

• Prolactin should be measure because many

pt. with hypopituitarism also present with

secondary hyperprolactinemia.

• 25% of pt. loss of pituitary function after

surgery

Williams Textbook of endocrinology 10 edition

Screening for pituitary failure

• Pt with hypothalamic or pituitary mass lesion.

• Developmental craniofacial abnormality.

• Inflammatory disorder

• Brain granulomatous disease

• Prior head or neck radiation

Williams Textbook of endocrinology 10 edition

Screening for pituitary failure

• Prior skull base surgery

• Head trauma

• Newly discover empty sella

• Experienced pregnancy- associated

hemorrhage or blood pressure change

To prevent long term morbidity Williams Textbook of endocrinology 10 edition

investigaton

www.Pituitarysociety.org

investigation

www.pituitarysociety.org

Pituitary test

• ล

Harrison’s 18th edition

Pituitary test

Harrison’s 18th edition

Pituitary test

Harrison’s 18th edition

treatment

Harrison’s 18th edition

treatment

Harrison’s 18th edition

Acquire hypopituitary

• Pituitary apoplexy

• Cranial irridation

• Lymphocytic hypophysitis

• Empty sellar

• Sheehan syndrome (pituitary necrosis)

• ETC.

Pituitary apoplexy

• Acute intrapituitary hemorrhage

• Occur spontaneous in preexitting adenoma ,

postpartum (sheehan syndrome)

• Associated DM , HT , sickle cell anemia , shock

, warfarin use

• Endocrine emergency

• hypopituitary is very common after apoplexy

Harrison’s 18th edition

Pituitary apoplexy

• pituitary apoplexy occurs within pituitary

adenomas

• it may also occur in non-adenomatous or

even the normal pituitary gland

• especially during pregnancy � sheehan’s

syndrome � pituitary necrosis

Harrison’s 18th edition

Pituitary apoplexy

• Acute symptom : severe headache , nausea vomitting , bilateral visual change (bitemporal hemianopsia) , opthalmoplegia

• Others symptoms : severe hypoglycemia hypotension , loss of consciousness , death

• Diagnosis : Pituitary CT , MRI

intratumural or sellar hemorrhage , deviated pituitary stalk and compress pituitary tissue

Harrison’s 18th edition

Pituitary apoplexy

Acute phase MRI PITUITARY

Pituitary apoplexy

• Management

no evidence impair consciousness or visual loss : conservative corticosteroid if adrenal insufficiency

significant visual loss , severe opthalmoplegia or loss of consciousness : urgent surgical compression

• Visual recovery � the length of time after acute event

Harrison’s 18th edition

Cranial irridation

• Whole brain or head and neck therapeutic

irridation

• Two third of pt develop hormone deficiency

after a median dose 50 Gy (5000 rad) direct

base skull

• GH is most common. Follow by gonadotropin

and ACTH

Harrison’s 18th edition

Lymphocytic hypophysitis

• Most common in postpartum woman.

• Suggests a selective autoimmune process target to

specific cell types.

• Hyperprolactinemia with MRI :prominence pituitary

mass like adenoma.

• Most manifestration of progressive mass effects with

headache and visual disturbance.

• Resolve after several month of glucocorticoid

treatment.

Harrison’s 18th edition

Empty sella

• Develop insidiously.

• Pituitary mass undergo clinically silent infarct.

• CSF filling the dural herniation

• Usually normal pituitary function.

• Functional pituitary adenoma may arise within the

rim of pituitary tissue.

• Not always visible in MRI

Harrison’s 18th edition

Sheehan syndrome

• Ischemic pituitary necrosis due to severe

postpartum hemorrhage , Vasospasm,

thrombosis and vascular compression of the

hypophyseal arteries.

• about 3% for women above 20 years of age,

almost two-thirds of whom had delivered

babies at home.

Indian J Endocrinol Metab. 2011 September;

15(Suppl3): S203–S207.

Pathophysiology of sheehan’s

Indian J Endocrinol Metab. 2011 September;

15(Suppl3): S203–S207.

Sheehan syndrome

• the average time between the previous

obstetric event and diagnosis of SS was 13

years.

• Lactation failure is a very common clinical

feature

• The absence of amenorrhea or the presence

of postpartum lactation, however, does not

rule out the diagnosis.

Indian J Endocrinol Metab. 2011 September;

15(Suppl3): S203–S207.

Sheehan syndrome

• The main involvement was the secretion of

growth hormone (GH) and prolactin (90–

100%).

• deficiencies in cortisol secretion,

gonadotropin and thyroid stimulating

hormone (TSH) ranged from 50 to 100%

• Treatment : hormone therapy �

glucocorticoid , GH etc.

Indian J Endocrinol Metab. 2011 September;

15(Suppl3): S203–S207.

Thank youThank you