Cushings Syndrome

Examination
Exposure remove shirt
Seated
Face
Round or moon facies
Plethora
Telangiectasia
Cataracts, anaemia
Oral thrush, buccal pigmentation
irsutism
!cne
"ec#
Supraclavicular fat pads
$orsal hump or buffalo hump %interscapular fat pad&
'pper limbs
ands
clubbing, nicotine staining, h(perpigmentation
R!, S)E
*ruising
Paper( thin s#in %use + index fingers in a circular fashion&
Peripheral ,asting of 'ls
Proximal m(opath(
!canthosis nigricans
!bdomen
Truncal obesit(
Purple striae
)o,er )imb
Edema
*ruising
Stand the patient up
)oo# for #(phoscoliosis
Palpate-percuss for tenderness %osteoporosis and vertebral collapse&
!s# the patient to s.uat and then stand up
Re.uest
*P
'rine dipstic#
Examine the lungs for asthma and pulmonar( fibrosis
/isual field assessment although ma0orit( of pituitar( adenoma are
microadenoma
Panh(popituirism
1E" t(pe 2
Presentation
Sir, this patient has got Cushing3s s(ndrome4 There is presence of moon5facies
,ith facial plethora and telengiectasia4 There is presence of hirsutism, acne, oral
thrush and cataracts or con0unctival pallor4 This is associated ,ith supraclavicular as
,ell as interscapular fat pad deposition4 There is truncal obesit( associated ,ith
purple striae4 There is bruising of the s#in and the s#in is paper( thin s#in ,ith
proximal m(opath( and lo,er limb edema4 There is no evidence of acanthosis
nigricans4 There is #(phoscoliosis ,ith tenderness of the spine4
There ,as no clinical evidence of R! such as s(mmetrical deforming
pol(arthropath( or S)E4
6 ,ould li#e to complete the examination b(
Respirator( examination for evidence of asthma or pulmonar( fibrosis
*P
$1
!s# histor( of exogenous steroid inta#e
/irilisation deepening of voice, breast atroph(, clitoromegal(
Questions
7hat are the causes of Cushing3s s(ndrome8
Rule of 9:;2:
9:< exogenous and 2:< endogenous
of the 2:< endogenous
9:< !CT dependent and 2:< !CT5independent %adrenal adenoma and
carcinoma&
of the 9:< !CT dependent
9:< are Pituitar(%Cushing3s disease& and 2:< are ectopic !CT
%bronchial carcinoid, small cell lung ca, pancreatic carcinoma, non5
teratomatous ovarian tumor&
of the Pituitar( adenoma
9:< are microadenoma
2:< are macroadenoma
!CT independent
!drenal adenoma
!drenal carcinoma
1icro-macronodular adrenal h(perplasia
Part of Carne( complex %pigmented s#in lesions ,ith endocrine and
mesench(tmal tumors&
1cCune !lbright s(ndrome
7hat are the causes of PseudoCushing3s8
$O!
$epressions, drugs
OCPs, obesit(
!lcoholism, acute illness
7hat are purple striae8
Purple striae are due to the ,ea#ening and disruption of the collagen fibres of
the dermis leading to exposure of the underl(ing vascular tissue4 The( can be found
on the abdomen, the upper arms and on the medial aspects of the thighs4
7hat are the signs suggesting ectopic !CT secretion8
!bsence of Cushingoid habitus, prominent edema and h(pertension and
mar#ed muscle ,ea#ness4
7hat are the features that suggest adrenal carcinoma8
/irilisation in the female, g(naecomastia in a male and a palpable abdominal
mass4
7hat is the significance of h(perpigmentation in a Cushingoid patient8
6t implies that Cushing3s s(ndrome is due to !CT excess due to presence of
1S li#e activit( of the !CT molecule4
7hat is "elson3s s(ndrome8
"elson s(ndrome occurred formerl( as a result of bilateral adrenalectom( for
Cushing3s disease
Resulting in absent negative feedbac# of cortisol on the pituitar( adenoma, ,ith
expansion of the pituitar( adenoma ,ith headache, bitemporal hemianopia and
panh(popit eventuall(
Occurs in +:< of such patients in the past
(perpigmentation occurs due to melanoc(te stimulating component of the
precursor molecule of !CT4
o, ,ould (ou investigate this patient8
Screen ,ith
+= urinar( cortisol or
overnight dexamethasone suppression test
2mg bet,een 22pm to 2+ midnight
Serum Cortisol at > am the follo,ing morning %?+mcg-dl&
Confirm diagnosis ,ith a lo, dose dexamethasone suppression test
$etermine the cause ,ith
Plasma !CT
igh dose dexamethasone suppression test
6maging studies %1R6 pituitar( or CT adrenals&
Others
C@R if ectopic !CT suspected
!@R for adrenal calcification
CR test %distinguish ectopic CR vs Cushing3s disease&
6nferior petrosal sinus sampling %distinguish primar( and ectopic source of
!CT ,hen above tests are inconclusive&
o, ,ould (ou manage this patient8
Treatment is directed at the primar( cause of the s(ndrome;
Exdogenous steroids
7ithdra,al if possible
6f not possible
1onitor for complications and treat them
*P
$1
P'$
Osteoporosis
Endogenous
!CT dependent
Cushing3s disease
Transphenoidal h(poph(sectom(
Transfrontal h(poph(sectom(
Pituitar( irradiation
*ilateral adrenalectom( ,ith pituitar( irradiation
Ectopic !CT
)ocate the source and treat appropriatel(
6f unable to, adrenalectom( or medical therap(
!CT independent
!denoma-carcinoma unilateral adrenalectom(
(perplasia bilateral adrenalectom(
1edical therap( onl( used if surgical not possible, eg metastatic adrenal
carcinoma or ectopic !CTA can use mitotane, #etoconaBole, met(rapone,
aminogluthithemde, trilostane and etomidate4