Bombay Hospital Journal, Vol. 53, No. 4, 2011

Abstract

Conn syndrome is characterised by increased s. aldosterone and decreased plasma

renin sctivity. Pt presents with severe hypokalaemia, hypertension (HTN) and

alkalosis. Females are affected more than males. We, report a case of a 29 yr old male

admitted with severe generalised weakness and difficulty in breathing. He was

intubated and resuscitated with medical management. On investigation, a diagnosis

of adrenal adenoma (Conn syndrome) was established. Subsequently, the pt

underwent Rt adrenalectomy. Pt recovered completely. His s. potassium was

normal without any supplements. He became normotensive without any

antihypertensive drugs. Thus Conn syndrome is rare, can present with life -

threatening complications, but correct diagnosis and timely surgical intervention

can be life saving.

Dept. of Urology, Bombay Hospital, Mumbai.

Prashant Gupta*, Umesh Oza**, Sadanand W. Thatte***

Conn's Syndrome - Under Diagnosed, Surgically Correctable

Introduction

onn syndrome is characterised by Cincreased aldosterone secretion from

adrenal glands, decreased plasma renin

activity, HTN and hypokalaemia. It was

first described by Conn in 1955. The term

primary hyperaldosteronism is used to

describe Conn syndrome and other

aetiologies of primary hypersecretion of

aldosterone (e.g. adrenal hyperplasia).

Primary hyperaldosteronism in general

and Conn syndrome in particular is the

most common form of secondary HTN.

Case Report

A 29 yr old male pt with H/o high BP since 2 yrs

presented with sudden onset of generalised

weakness and difficulty in breathing. He was

admitted in ICU and intubation was done to relieve

respiratory problem. The findings on admission were

as follows - Pulse - 110/min, BP - 170/110 mm Hg,

saturation -100% on ventilator, CBC - WNL, urine rt.

- WNL, electrolytes - Na+ 150, K+ 2.0, pH - 7.52.

The pt was resuscitated. Supportive and

symptomatic treatment was given with correction of

fluid and electrolyte imbalance and alkalosis.

Investigations were done: S. aldosterone 34 ng/dl,

plasma renin activity - 0.04 ng/ml/hr, USG abdomen

showed normal b/l kidneys, mild increased cortical

echotexture and normal arterial flow and the CT scan

showed 1x1cm Rt adrenal adenoma with post

contrast enhancement.[fig-1,2]

Fig 1 Showing small adenoma in Rt.adrenal gland

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Tab Spironolactone 50 mg BD was added in the

antihypertensive regime.

A provisional diagnosis on the basis of above

investigations was made as functional adrenal

adenoma of Rt side.

Laparoscopic Rt. Adrenalectomy was planned

and the pt was taken for the same with due 5anaesthetic precautions. Due to excessive bleeding

during the procedure because of tear of adrenal vein

the LAP procedure was abandoned and an open

transperitoneal Rt. Adrenalectomy was performed.

The post-operative period was uneventful with a good

and dramatic recovery showing an increase in s.

potassium levels without supplements and BP within

n o r m a l l i m i t s w i t h o u t i n c r e a s i n g t h e

antihypertensive doses. The dose of tab.

spironolactone was reduced. The gross specimen

showed a 1cm x 1cm yellow tan nodule.

Microscopically, the specimen shows thinly

encapsulated adrenal tumour composed of compact

nests and broad trebaculae of polyhedral cells

separated by rich network of arborizing thin wall slit -

like vessels. The cells possess pale granular

acidophilic cytoplasm and smooth contoured round

nuclei [fig-3].

Finally diagnosed as aldosterone secreting Rt

adrenal adenoma.

Review of Literature

Two major subtypes can be observed

due to which primary hyperaldosteronism

i.e. increased aldosterone secretion from

adrenals is caused -

1. Unilateral aldosterone producing

adenoma (APA) Or Conn syndrome

(50 - 60% of cases)

2. Idiopathic hyperaldosteronism (IHA)

or bilateral adrenal hyperplasia (40 -

50% of cases).

3. A rare subtype is aldosterone

producing adrenocortical Ca (APAC).

P r e v a l e n c e f o r p r i m a r y

hyperaldosteronism specifically Conn

syndrome are respectively 0.05 - 2% and 40.03 - 1.2% of HTN pts. Mortality is mainly

due to HTN if left untreated for many yrs

and hypokalaemia if disease is severe. The

F: M ratio is 2-2.5:1 and the peak

Bombay Hospital Journal, Vol. 53, No. 4, 2011

Rt.adrenal adenomaHistology of adenoma showing benign

encapsulated tumour

Bombay Hospital Journal, Vol. 53, No. 4, 2011

incidence occur in 3rd - 6th decade of life.

Discussion

Aldosterone acts on the distal renal

tubule, promoting sodium ion exchange

for hydrogen and potassium ions. Water 1and sodium retention lead to HTN.

T h e r e f o r e , p t s w i t h p r i m a r y

hyperaldosteronism usually present with

HTN associated with weakness, polyuria,

polydipsia, muscle cramping, or paralysis.

Pts are hypokalaemic, alkalaemic often

hyponatraemic. Hypokalaemia in primary

hyperaldosteronism is often severe (< 3 9mmol/L). Conn's initial pt. had a

potassium level of 1.6-2.5 mEq/L, but

hyperaldosteronism may also present with

normokalaemia specially in the early

phase of disease. Normal BP is rare but 1,2cases reported. Biochemical diagnosis of

primary hyperaldosteronism is done by

m e a s u r i n g p l a s m a a l d o s t e r o n e

concentration (PAC) in the setting of low 1plasma renin activity (PRA). PAC/PRA

ratio of 30-50 is strongly associated with

this diagnosis.

Once primary hyperaldosteronism has

been identified, the adrenal glands should

be examined radiographically. This can be

done by USG (thin pts) and CT scan or MRI

(overweight pts). Sensitivities range from

53-100% for CT detection of aldosterone

producing adenomas. A recent study

showed similar performance between CT

and MRI for aldosterone producing 3adenomas. Assessment of function may

require isotope NP59 scanning or selective

renal vein sampling for aldosterone in b/l 7adrenal hyperplasia. In the treatment

regime of the pt we added Spironolactone

for increasing s. potassium which was not

improved with oral and iv potassium

supplements. Treatment for primary

hyperaldosteronism may be medical or

surgical depending on the cause and

clinical characteristics of the pt. Most

functional adrenal tumours are benign.

LAP adrenalectomy is often curative in 6case of unilateral adrenal adenoma. Pt

who has high surgical risk, bilateral

adrena l adenoma or id iopath ic

hyperaldosteronism are best managed by

spironolactone conservatively. Most of the

pts with Conn syndrome (91.4%) become 8normotensive after surgery.

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