Signs and Symptoms

Acute adrenal insufficiency usually manifests as shock in a patient with undiagnosed chronic adrenal

insufficiency who has been subjected to physiologic stress. Similarly, patients with established adrenal

insufficiency caused by exogenous steroid use may experience crisis if they do not increase

glucocorticoid replacement during times of stress or illness. Symptoms of adrenal insufficiency reflect

glucocorticoid and mineralocorticoid deficiencies. Signs and symptoms of acute insufficiency include

fever, nausea, vomiting, refractory hypotension, and lethargy (Table 77-7). Acute adrenal insufficiency

is a medical emergency and should be suspected in stressed patients with a history of either adrenal

insufficiency or exogenous steroid use. Chronic adrenal insufficiency presents more subtly, with fatigue,

weight loss, anorexia, nausea and vomiting, abdominal pain, and diarrhea.

Diagnosis

Laboratory findings of adrenal insufficiency include hyponatremia, hyperkalemia, azotemia, and fasting

or reactive hypoglycemia. Hypercalcemia may also be present. The rapid ACTH stimulation test is the

best test for both acute and chronic adrenal insufficiency. Synthetic ACTH (250 μg) is administered

intravenously, and plasma cortisol levels are measured 30 and 60 minutes later. Normal peak cortisol

response should exceed 20 μg/dL. Measurement of ACTH by IRMA is then used to distinguish primary

from secondary and tertiary adrenal insufficiency. High plasma concentration of ACTH (>200 pg/dL)

and low plasma cortisol (<10 mg/dL) are diagnostic of primary adrenal insufficiency. Low levels of

plasma ACTH indicate secondary (pituitary) or tertiary (hypothalamic) adrenal insufficiency.

DIAGNOSIS

Table 77-7 Symptoms and Signs of Acute Adrenal Insufficiency

Treatment

Acute adrenal insufficiency is based on clinical suspicion before laboratory confirmation is available.

Intravenous volume replacement with isotonic fluids and immediate intravenous steroid replacement

therapy with 4 mg dexamethasone is essential. A rapid ACTH stimulation test is then performed to

establish the diagnosis of adrenal insufficiency after resuscitation and corticosteroid replacement.

Hydrocortisone acetate is detected in laboratory measurement for cortisol so dexamethasone should be

used for replacement of glucocorticoid function until ACTH testing is complete. Thereafter, 100 mg of

hydrocortisone is administered intravenously every 6 to 8 hours and is tapered to standard replacement

doses as the patient’s condition stabilizes. Mineralocorticoid replacement is not required until oral

intake resumes. Chronic adrenal insufficiency requires both corticosteroid and mineralocorticoid

replacement. Usual daily dosing is 12 mg/m2 of hydrocortisone and 0.05 to 0.10 mg fludrocortisone.

Patients who have known adrenal insufficiency or who have received supraphysiologic doses of

corticosteroid for at least 1 week in the year preceding surgery should receive perioperative stress-dose

corticosteroids. Administration of 100 mg hydrocortisone the morning of major surgery followed by 100

mg of hydrocortisone every 8 hours during the perioperative 24 hours is usually more than sufficient.

Steroids can be rapidly tapered to replacement levels as the patient’s condition permits.

ADRENALECTOMY

Surgical approaches to the adrenal glands include the laparoscopic approach, the anterior

transabdominal approach, and less commonly, the combined thoracoabdominal approach or posterior

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retroperitoneal approach. Either adrenal gland can be removed using any of these approaches. The

choice of approach depends on the suspected pathology, the size of the adrenal lesion, and the expertise

of the surgeon. Small benign appearing tumors that are localized with confidence by imaging studies are

resected using a laparoscopic or posterior approach. Posterior retroperitoneal adrenalectomy, once the

most common method of adrenalectomy is less commonly used since most lesions amenable to this

approach can be removed laparoscopically. Large adrenal masses and those that may harbor malignancy

should be resected using an anterior approach to adequately explore the entire abdomen and gain

sufficient exposure for safe resection. Very large adrenocortical carcinomas may require a

thoracoabdominal approach for en bloc resection with involved adjacent structures. Recently, a

retroperitoneal laparoscopic approach has been advocated as an alternative method for small tumors,

particularly for patients with prior abdominal surgery.

Laparoscopic Adrenalectomy

Laparoscopic adrenalectomy is now the preferred approach to most small, benign adrenal lesions

including functioning and nonfunctioning adenomas and pheochromocytomas. Enthusiasm for the

laparoscopic approach is based on the expectation of decreased postoperative pain, faster rehabilitation,

and fewer complications. Numerous studies have shown the efficacy and safety of this procedure for

tumors up to 6 cm. Larger lesions can be approached by experienced surgeons in select circumstances.

Importantly, expertise in open adrenalectomy is absolutely necessary for the laparoscopic surgeon to

convert to an open procedure and promptly rectify any intraoperative laparoscopic complications.

Laparoscopic adrenalectomy begins with induction of general anesthesia, placement of a urinary

catheter and orogastric tube, and positioning of the patient in the lateral decubitus position with the

affected side up. Exposure is facilitated by extension of the operating table at the patient’s waist. A total

of three or four intraperitoneal ports (one camera, one retractor particular for the right side, and two

working ports) are placed at least 5 cm apart in a transverse line from the lateral edge of the rectus

sheath to the midaxillary line between the costal margin and iliac crest (Fig. 77-11A). Four-quadrant

exploration of the peritoneal cavity is first performed with the videoscope through the medialmost port,

and the videoscope is then transferred to the middle port. A retractor is placed through the medial most

port to retract the viscera medially. Operating instruments are alternately placed within the abdomen

through the lateral ports.

Laparoscopic adrenalectomy proceeds similar to the open anterior approach. Right adrenalectomy

begins with mobilization of the liver to open the retroperitoneum and allow retraction of the right lobe

of anteriorly and medially using the most medial port. The adrenal gland is then identified

posterolateral to the inferior vena cava and superior to the kidney. Dissection usually begins by

developing the plane between right adrenal and inferior vena cava using careful blunt dissection, with

small vessels sequentially coagulated using electrosurgical or harmonic energy. The right adrenal vein is

identified, carefully dissected and then doubly clipped and divided. Dissection then continues

circumferentially around the gland with additional small vessels coagulated or clipped. The adrenal

gland subsequently is placed in a bag and removed through an expanded port.

Left adrenalectomy is performed with mirror-image patient and port-site orientation as right

adrenalectomy. After port placement, dissection begins with mobilization of the splenic flexure of the

colon and spleen from the left retroperitoneum. Subsequent medial retraction of the spleen, tail of the

pancreas, and stomach is accomplished with the fan or similar retractor if necessary. Often after full

mobilization, no retractor is necessary, and thus only 3 ports are needed. The left adrenal gland is

identified in perinephric fat at the superior renal pole. Intraoperative laparoscopic ultrasound can be

useful for this on the left side, particularly in Cushing syndrome with the associated increased truncal

fat. Once identified, left adrenal gland dissection proceeds circumferentially around the gland, with

identification, ligation and division of the left adrenal vein at the inferiormost aspect of the dissection.

Care is needed to identify and avoid the inferior phrenic vein, which courses along the medial aspect of

the left adrenal gland. Following circumferential dissection, the gland is removed.

Anterior Approach

Anterior, open adrenalectomy begins with positioning the patient in reverse Trendelenburg position

with elevation of the right flank. An ipsilateral or bilateral subcostal incision allows access to either

adrenal and facilitates exploration of the abdomen (Fig. 77-11B). The abdomen is opened and explored

for evidence of metastatic disease, including biopsy or excision of suspicious lesions. Resection of the

right adrenal gland proceeds with full mobilization and anteromedial retraction of the right hepatic lobe

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(Fig. 77-12A). It is important to fully expose the retrohepatic inferior vena cava. In order to uncover the

inferior vena cava, the right kidney and the right adrenal gland, the hepatic flexure and transverse colon

and are retracted inferiorly, usually without need for extensive mobilization. A Kocher maneuver of the

duodenum is seldom necessary as the adrenal gland is superior to this area. The retroperitoneal space is

entered behind the liver to expose the adrenal gland. Dissection of the gland proceeds from its

inferomedial aspect, where small feeding arteries are individually clipped and divided. The vena cava is

carefully dissected along its lateral border, which allows identification of the right adrenal vein where it

drains directly into the inferior vena cava from the anterior aspect of the adrenal gland. The adrenal

vein is ligated and divided close to the vena cava. If necessary because of hemodynamic changes during

an operation for a pheochromocytoma, the adrenal vein is identified and ligated early to avoid

catecholamine surges and blood pressure fluctuations during manipulation of the gland. Once the

adrenal vein is ligated, arterial feeding vessels are clipped and divided sequentially, beginning at the

superolateral aspect of the gland and continuing medially.

Resection of the left adrenal gland requires mobilization of the spleen, tail of pancreas and left colon.

The left colon is freed from its peritoneal attachments and is reflected inferiorly. The spleen is then

delivered from the left upper quadrant medially, and the splenocolic ligament is divided. The spleen,

stomach, and pancreatic tail are retracted medially en bloc to expose the left kidney and adrenal (Fig.

77-12B). The left adrenal vein is ligated and divided at its junction with the left renal vein. The gland is

dissected and the arterial vessels are ligated and divided sequentially, beginning at the superolateral

aspect of the gland and continuing medially.

Thoracoabdominal Approach

The thoracoabdominal approach is used for large adrenal lesions with invasion of surrounding structures

including the liver and diaphragm on the right side, and the spleen, pancreatic tail, stomach, and

diaphragm on the left. The patient is positioned with the ipsilateral flank raised and arm extended

cephalad. Incision is made in the tenth or eleventh intercostal space beginning at the posterior axillary

line and is extended toward the midline of the abdomen (Fig. 77-11C). Retractors are placed and

subsequent adrenalectomy is performed as for the anterior approach.

Posterior Approach

With the advent of laparoscopic adrenalectomy, the posterior approach is infrequently used. When

laparoscopic adrenalectomy is not an option, typically because of extensive prior transperitoneal

operations, the posterior approach is better tolerated and allows faster postoperative recovery

compared with the anterior approach. A posterior approach becomes increasingly more difficult as

tumor size increases and is not recommended for excision of large pheochromocytomas, adrenal tumors

greater than 6 cm, or adrenal carcinoma.

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Figure 77-11. Incisions for right adrenalectomy. Shown are typical incisions for (A) laparoscopic approach, (B) open, anterior

approach, (C) thoracoabdominal approach, and (D) posterior, open approach. Incisions for left adrenalectomy are positioned

opposite.

Figure 77-12. Anterior approach to right (A) and left (B) adrenalectomy. Note position of phrenic vein in relationship to the left

adrenal vein and tumor.

The patient is placed prone on the operating table and flexed at the waist, which allows the

abdominal contents to fall away from the retroperitoneum. The incision is made from the midline at the

tenth rib and is extended inferolaterally to the superior border of the posterior iliac crest (Fig. 77-11D).

Dissection proceeds through the subcutaneous fat and latissimus dorsi muscle to the lumbodorsal fascia.

This fascia is incised longitudinally, and the underlying sacrospinalis muscle. The sacrospinalis muscle is

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retracted medially, and the twelfth rib and vascular bundle are resected as far medially as possible. The

twelfth intercostal nerve is preserved and gently retracted superiorly. The retroperitoneum is entered

and the diaphragm is bluntly elevated from Gerota fascia, the pleura are separated from the diaphragm,

and the diaphragm is then divided. Gerota fascia is incised, and the kidney is retracted inferiorly to

expose the adrenal gland. The arterial blood supply is controlled first by clipping and ligating numerous

arterial vessels, which course posteriorly. The adrenal vein, located deep to the arteries, is ligated as it

is encountered. On the right, the adrenal vein exits from the anterior aspect of the gland and courses to

the inferior vena cava. Once the adrenal vein is divided the gland is then freed circumferentially from

its lateral to medial aspect. The inferior border of the gland is dissected last to maintain attachment to

the kidney and allow inferior retraction of the gland. Finally, repair of the diaphragm and any

incidental pleural defects, reapproximation of the lumbodorsal fascia, and closure of the skin.

Posterior Retroperitoneal Laproscopic Adrenalectomy

Posterior retroperitoneal laproscopic adrenalectomy also known as posterior retroperitoneoscopic

adrenalectomy maintains the advantages of a laparoscopic approach while avoiding the abdominal

cavity. This approach is ideal for patients with prior abdominal surgery or for bilateral adrenalectomy

as both adrenal glands can be removed without repositioning the patient.

Posterior retroperitoneal laparoscopic adrenalectomy begins with placing the patient in the prone

jackknife position on a spinal table allowing the abdominal contents to hang forward. The hips and

knees are positioned at 90-degree angles to the spine and femur and carefully padded. The first port is

placed just below the tip of the 12th rib; after the incision is made the retroperitoneal space is entered

sharply, a space is created bluntly with the index finger, and a blunt 10-mm trocar is placed. A second

medial 10-mm trocar is placed along the paraspinous muscle at a 45-degree angle toward the adrenal

gland. A lateral 5-mm port is placed 5 cm lateral to the first port in a similar fashion.

Pneumoretroperitoneum to 20 to 24 mm Hg is created with CO2

to create an adequate working space.

The videoscope is transferred from the initial middle port to the medial port to complete the dissection.

The retroperitoneal space beneath the diaphragm is created bluntly and sharply. The superior border of

the kidney is immediately identified and Gerota fascia is entered. The upper pole of the kidney is

retracted caudally and the tissue superior to the kidney which contains the adrenal gland is completely

separated from the kidney. The medial dissection is then begun by carefully bluntly dissecting medial to

the adrenal gland identifying the adrenal vein and dividing it between clips. Identification of the

adrenal vein, particularly on the right side, is easier than with the transabdominal laparoscopic

approach. Dissection is completed circumferentially around the gland using electrosurgical or harmonic

energy to coagulate any small vessels. Once the gland is completely free, it is placed in a bag and

removed through the middle port.

Bibliography

OMIM Online Mendelian Inheritance in Man [database online] #202300. Baltimore, MD: Johns Hopkins;

2005. Available at: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id5202300. Accessed June

9, 2005.

OMIM Online Mendelian Inheritance in Man [database online] #171300. Baltimore, MD: Johns Hopkins;

2005. Available at: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id5171300. Accessed June

9, 2005.

Bravo EL, Tagle R. Pheochromocytoma: state-of-the-art and future prospects. Endocr Rev 2003;24:539–

553.