causes may be a penetrating gastric ulcer posteriorly or retroperitoneal fibrosis.

The diagnosis is made in patients who have upper gastrointestinal bleeding with only gastric varices

on endoscopy. Because there is no portal vein hypertension and no cirrhosis of the liver, there are no

esophageal varices. The spleen may or may not be enlarged but again there are no other signs and

symptoms of cirrhosis. Definitive diagnosis in the past has been made by a celiac angiogram that

demonstrates absence of the splenic vein with collateral flow via the gastric veins. Magnetic resonance

angiogram/venograms, as well as high-resolution ultrasound, may also make the diagnosis.

Splenectomy is curative as it removes the blood flow via the splenic artery through this organ. This

removes all the excess collateral flow, which leads to the venous hypertension and the associated

symptoms resolve. Whenever splenic vein thrombosis is diagnosed, even if the patient has not had an

episode of bleeding, elective splenectomy should be performed as a prophylactic measure.

Patients with portal hypertension and cirrhosis or partial vein thrombosis have a complex collection

of symptoms that include hypersplenism with splenomegaly and thrombocytopenia and anemia.

However, the operative risk associated with portal vein hypertension is excessive and splenectomy is

virtually never indicated in this setting.

Splenic Artery Aneurysm

Splenic artery aneurysm is uncommon even though it is the second most frequent abdominal artery to

undergo aneurysmal changes.144 Splenic aneurysms occur twice as often in women than in men. Patients

can be divided into two distinct groups. First, there are elderly patients whose aneurysms are

manifestations of atherosclerosis. Second, young women have apparent congenital predisposition to

form splenic artery aneurysms. In these young women, there is an increased risk of rupture of these

aneurysms during pregnancy. Inflammatory processes such as pancreatitis may involve the splenic

artery and occasionally lead to aneurysm, but more frequently lead to acute bleeding.

Splenic artery aneurysms are typically asymptomatic and may be initially identified as a widened rim

of calcification in the left upper quadrant defining the aneurysm boundaries. They may also be

discovered as incidental findings on CT scan. If symptomatic, patients experience left upper quadrant

pain, nausea, and vomiting. The presence of symptoms suggesting pending aneurysmal rupture and

urgent splenectomy with ligation of the splenic arteries is indicated.

When a calcified atherosclerotic splenic artery aneurysm is discovered in a patient older than 60 years

with no splenomegaly and no symptoms, surgical excision is not indicated and the aneurysm may be

followed to look for signs of enlargement. In younger patients who have a symptomatic aneurysm

identified, particularly young women of child-bearing age, an elective splenectomy is recommended to

prevent rupture. Nonsurgical approaches include embolization of the splenic artery in patients who are

at poor risk for open laparotomy.144

MISCELLANEOUS INDICATIONS FOR SPLENECTOMY

Splenectomy may be performed infrequently for miscellaneous indications, including splenic cysts,

infectious cysts, and splenic abscesses. Also included in the discussion of this category is the frequently

described wandering spleen that is a normal spleen that lacks the normal ligamental attachments to

keep it in the left upper quadrant. This can present in a variety of ways from an undiagnosed mass to a

torsion of the splenic hilum causing acute abdominal pain.

Table 73-10 Primary Neoplasms and Cysts of Spleen

Cysts of the Spleen

2013

The splenectomy is performed as described earlier often for hematologic malignancies, including

multiple forms of leukemias and lymphoma primarily for symptoms of hypersplenism or splenomegaly,

or as a staging procedure. Splenectomy for primary neoplasms of the spleen is an infrequent indication.

These primary neoplastic processes are listed in Table 73-10.

Hemangioma is most common benign primary neoplasm of the spleen. It is often an incidental finding

and may be solitary or multiple. During operation, it can be identified as a more intensely bluish-purple

colored lesion when seen from the surface compared to the reddish-purple color of the splenic

parenchyma. Hemangiomas can be identified with excellent sensitivity and specificity by MRI

characteristics. It is ill-advised to perform a biopsy of a lesion that may be a splenic hemangioma.145,146

Hemangiomas of the spleen rarely cause symptoms, but massive hemangiomas either rupture

spontaneously or make the spleen more susceptible to a traumatic rupture. In cases of massive

hemangioma with capsular distension with pain, either a splenectomy or partial splenectomy may be

indicated.

Hemangioendothelioma is a neoplasm that is thought to be slightly more aggressive than the typical

benign hemangioma. It is felt to be pathologically an intermediate lesion between benign hemangioma

and malignant angiosarcoma. It is not felt to have metastatic potential and generally is an incidental

finding. Larger lesions may cause symptoms or be noted for their size and rupture either spontaneously

or with minor trauma, all circumstances in which patients with these lesions may undergo splenectomy.

Lymphangiomas also occur in the spleen and are much less common than hemangiomas. They may be

multiple or solitary and can be identified by a lighter color and compressibility when seen in surgery.

Two mass lesions that occur in the spleen that are not true neoplasms are hamartomas and

inflammatory pseudotumors. Hamartomas are focal development of abnormalities that occur in spleen

and other solid organs such as the liver. Hamartomas contain normal cellular elements and are again

nonneoplastic but have a random fibrotic organization.147 The major significance of hamartomas is that

they are identified either at laparotomy as incidental findings or they are seen as incidental lesions on

CT scans done for other reasons.148 Inflammatory pseudotumors are described throughout most organs

and have also been described in the spleen. These sometimes are quite large with a wide variety of

reactive cells. A subcategory of inflammatory pseudotumors of the spleen is related to mycobacterial

infection, particularly in HIV-positive patients. There have been reports of primary splenic pregnancy

that is considered the rarest site of ectopic pregnancies.

Figure 73-12. The visceral surface of a spleen with a true congenital splenic cyst.

Cysts of the spleen are relatively common lesions seen across all age groups, may be multifocal, and

are not typically of parasitic origin. The diagnosis of splenic cysts can be ascertained by ultrasound or

CT scan. These benign lesions have no clinical significance unless they reach a large size. As discussed

earlier in the history of splenic surgery, the first reported case of a successful elective splenectomy was

done for a splenic cyst. This was an enormous cyst at the tip of the spleen and was felt to be likely an

ovarian mass and found to be arising from the spleen at laparotomy (Fig. 73-12). Today there would be

a tendency to do a cyst enucleation or partial splenectomy for peripheral cystic lesions that are large

and symptomatic. An alternative approach is unroofing the cyst and leaving a portion of the cyst wall in

place and this can frequently be done laparoscopically.149

The only parasitic cyst involving the spleen is from Echinococcus granulosus or a hydatid cyst. The

incidence of echinococcal cyst in the spleen compared to the liver is a ratio of approximately 30:1.

2014

However, the spleen is the third most common site of echinococcal cysts behind the liver and lung.150

Diagnosis should be suspected in patients in areas such as New Zealand, Australia, and parts of the

Western United States where echinococcal disease is common. The complement fixation test is the most

reliable serologic study for this organism. The treatment of echinococcal cysts is splenectomy. As with

echinococcal cysts of the liver, it is of utmost importance not to rupture the cyst and expose the patient

to the scoleces. For large and peripheral cysts in which risk of rupture is high, the contents of the cyst

should be carefully aspirated and replaced with hypertonic saline.151 In a recent report from Greece,

surgeons preserved 8 out of 19 spleens with echinococcal cysts by doing partial splenectomy.150

Abscess of the Spleen

Splenic abscess is uncommon but is an important disease because it is associated with a significant

mortality rate and may be cured by splenectomy.152 In most series, the mortality rate ranges between

40% and 100%.153 The typical patient has hematogenous seeding of the spleen by bacteria from a

remote source such as endocarditis or from intravenous drug abuse.154 There may be in some cases

direct spread of infection from adjacent intra-abdominal sources. Finally, splenic trauma treated

conservatively may eventually result in an infected splenic hematoma. In 80% of the cases, there is an

additional source of infection in locations other than the spleen, and in only 20% of cases is the splenic

abscess the sole source of sepsis identified. Enteric organisms account for two-thirds of the splenic

abscesses and staphylococcus and streptococcus comprise the remainder of the cases.

Patients typically present with signs and symptoms of sepsis including fever, malaise, and

leukocytosis. When the spleen is the sole site of infection, patients will have significant left upper

quadrant tenderness and guarding. Abdominal x-ray films may show gas in the spleen and an ultrasound

or CT scan with contrast will be diagnostic to show an abscess with reactive rim.

The treatment of choice for patients who can undergo a laparotomy and have the splenic abscess as an

isolated or prominent component of septic syndrome is splenectomy. If the spleen is the only source of

infection, removal of the spleen should be curative and result in the recovery of the patient. If patients

have multiple sites of infection or are too sick to undergo laparotomy, percutaneous drainage of splenic

abscesses may be attempted but this is not successful because of frequent spillage and assimilation of

bacteria in the left upper quadrant of the abdomen.

Ectopic Spleen (Wandering Spleen)

Ectopic spleens, or the so-called wandering spleens, occur because of either extreme laxity or absence of

the normal ligaments that attach the spleen to the left upper quadrant. This allows the spleen to

essentially drop to the lower abdomen in either the right lower quadrant or left lower quadrant by the

force of gravity attached by its vascular pedicle.155 This condition of a wandering spleen occurs 13 times

more frequently in women than in men. The wandering spleen presents most commonly in adults but

can cause symptoms in children. The diagnosis may be made by palpable lower abdominal mass

confirmed by a CT scan or a nuclear imaging spleen scan to be splenic tissue. The ectopic spleen creates

symptoms typically when there is torsion of the pedicle causing acute ischemia and acute pain. The

treatment for a wandering spleen as an incidental finding at laparotomy or if the torsion can be

corrected and the spleen appears to be viable is to do a splenopexy tacking it to its native position in the

left upper quadrant. If the spleen appears infarcted, then a splenectomy must be performed.156 The

wandering spleen is ideally suited for laparoscopic splenectomy as it is generally free from ligaments or

attachments and other organs.157

SPLENECTOMY: OPERATIVE TECHNIQUE

The choices of operations available currently to approach the spleen in an elective manner are open

splenectomy, laparoscopic splenectomy, or partial splenectomy. Table 73-11 lists indications for

splenectomy and whether a partial splenectomy is indicated. Certain principles apply to all patients

undergoing elective splenectomy. First, all patients should receive appropriate preoperative vaccination

with Pneumovax (pneumococcal polyvalent vaccine) and possibly also vaccination against Haemophilus

influenzae and Neisserian meningococcus 10 to 14 days prior to the procedure if possible. Patients must

have appropriate blood products ready as often patients are anemic and thrombocytopenic due to

hypersplenism as described earlier.

2015