Spleen

Question 1

Which of the following is not an indication for spelenectomy for Non-Hodgkin’s Lymphoma?

A. Signs of hypersplenism

B. Splenomegaly

C. Severe thrombocytopenia

D. Portal vein thrombosis

Answer 1

D. Portal vein thrombosis

Question 2

Spontaneous splenic rupture may occur in which disease entity?

A. Splenic artery aneurysm

B. Felty’s syndrome

C. Sarcoidosis

D. Chronic lymphocytic leukemia

Answer 2

C. Sarcoidosis

Question 3

Overwhelming postsplenectomy infection (OPSI) is commonest after splenectomy for

A. Trauma

B. Non-Hodgkin’s lymphoma

C. Thalassemia

D. Hereditary spherocytosis

Answer 3

C. Thalassemia

Question 4

Which of the following are true regarding wandering spleen?

A. Splenic torsion and infarction are rare occurrences

B. Splenectomy is required in most cases

C. The spleen is attached to a vascular pedicle without the usually mesenteric attachment.

D. Splenic abscess mostly occurs in accessory spleens.

Answer 4

C. The spleen is attached to a vascular pedicle without the usually mesenteric attachment.

Question 5

A 20-year-old patient underwent emergency splenectomy for a ruptured spleen. How soon should he preferentially receive vaccination?

A. Intraoperatively

B. Few hours after surgery

C. Within 5 days after surgery

D. Two weeks after surgery

Answer 5

D. Two weeks after surgery

Question 6

What of the following vaccines are not included in post-splenectomy vaccination regimen?

A. Streptococccus pneumonia vaccine

B. Haemophilus influenza type b vaccine

C. Neisseriae gonorrhea vaccine

D. Neisseriae meningitides vaccine

Answer 6

C. Neisseriae gonorrhea vaccine

Question 7

Splenectomy is indicative for curative response in the following disease entity except?

A. Idiopathic thrombocytopenia purpura

B. Felty’s syndrome

C. Splenic artery syndrome

D. Sickle cell anemia

Answer 7

D. Sickle cell anemia

Question 8

Splenectomy is generally only indicated to decrease cytopenia and anemia for which type of leukemia?

A. Acute myeloid leukemia

B. Chronic myeloid leukemia

C. Chronic myelomonocytic leukemia

D. Chronic lymphocytic leukemia

Answer 8

D. Chronic lymphocytic leukemia

Question 9

Which is true about splenic abscess?

A. Spontaneous splenic rupture can occur in both adults and children.

B. Most splenic abscesses occur ithrough local spread from lungs

C. Splenomegaly is common in splenic abscesses

D. Most common organism involved is anaerobic

Answer 9

A. Spontaneous splenic rupture can occur in both adults and children.

Question 10

Splenectomy is indicated for idiopathic thrombocytopenia purpura in the following situation

A. Partial response only to glucocorticoid therapy after 2 weeks of treatment

B. Patient on platelet counts of only 30,000-50,000/mm3

C. Refractory thrombocytopenia

D. Platelet count of less than 10,000mm3 with no bleeding

Answer 10

C. Refractory thrombocytopenia

Question 11

Most common type of hemolytic anemia for which splenectomy is indicated?

Answer 11

Hereditary spherocytosis

Question 12

Most common glycolytic defect causing congenital nonspherocytic hemolytic anemia?

Answer 12

Pyruvate kinase deficiency

Question 13

Most common RBC enzyme deficiency overall?

Answer 13

Glucose-6-Phosphate Dehydrogenase Deficiency

Question 14

When do alpha-thalassemia and beta-thalassemia present?

Answer 14

Because alpha chains are needed to form both fetal hemoglobin and adult hemoglobin, alpha thalassemia becomes symptomatic In utero or at birth.

By contrast, beta thalassemia becomes symptomatic at 4-6 months because beta chains are involved only In adult hemoglobin synthesis.

Question 15

Which of the following is NOT a location where accessory spleens can be found?

A. Gastrocolic ligament
B. Gerota’s fascia
C. Large bowel mesentery
D. Broad ligament

Answer 15

Answer: B

The most common anomaly of splenic embryology is the accessory spleen.

Present in up to 20% of the population, one or more accessory spleen(s) may occur in up to 30% of patients with hematologic disease.

Over 80% of accessory spleens are
found in the region of the splenic hilum and vascular pedicle.

Other locations or accessory spleens in descending order of frequency are: the gastrocolic ligament, the tail of the pancreas, the greater omentum, the greater curve of the stomach, the splenocolic ligament, the small and large bowel mesentery, the left broad ligament in women, and the left spermatic cord in men. (See Schwartz 10th ed., p. 1424.)

Question 16

Which of the following splenic ligaments is NOT an avascular plane?

A. Gastrosplenic
B. Splenocolic
C. Phrenosplenic
D. Splenorenal

Answer 16

Answer: A
Of particular clinical relevance, the spleen is suspended in position by several ligaments and peritoneal folds to the colon (splenocolic ligament); the stomach (gastrosplenic ligament); the diaphragm (phrenosplenic ligament); the kidney, the adrenal gland, and the tail of the pancreas (splenorenal ligament) (Fig. 34-1).

Whereas the gastrosplenic ligament contains the short gastric vessels, the remaining ligaments are usually avascular, with rare exceptions, such as in a patient with portal hypertension.

The relationship of the pancreas to the spleen also has important clinical implications.

In cadaveric anatomic series, the tail of the pancreas has been demonstrated to lie within 1 cm of the splenic hilum 75% of the time and to actually abut the spleen in 30% o patients.

(See Schwartz 10th ed., Figure 34-2, p. 1425.)

Question 17

All of the following are functions of the spleen EXCEPT

A. Clearance of damaged or aged red blood cells (RBCs) from the blood.

B. Extramedullary site for hematopoiesis and recycling iron.

C. Initiation of adaptive immune response from filtration of lymph.

D. Clearance of encapsulated bacteria from the blood stream.

Answer 17

Answer: C

The spleen has both fast and slow circulation of blood. It is during slow circulation that blood travels through the reticular spaces and splenic cords where it is exposed to contact with splenic macrophages which remove senescent blood cells.

Through this process the spleen is also able to remove erythrocyte inclusions such as Heinz bodies without lysing the cells.

Through the reticuloendothelial system the spleen clears encapsulated bacteria such as pneumococcus and Haemophilus influenzae which are poorly opsonized rom the hepatic reticuloendothelial system.

In addition to these functions the spleen serves as an extramedullary site for hematopoiesis and plays a functional role in the recycling of iron. While the white pulp of the spleen is important in the initiation of the adaptive immune response, material is delivered to the spleen through the blood and not the lymph. (See Schwartz 10th ed., p. 1427.)

Question 18

Which of the following proteins is not altered in hereditary spherocytosis (HS)?

A. Pyruvate kinase
B. Spectrin
C. Ankyrin
D. Band3protein

Answer 18

Answer: A

The underlying abnormality in hereditary spherocytosis (HS) is an inherited dysfunction or deficiency in one of the erythrocyte membrane proteins (spectrin, ankyrin, band 3 protein, or protein 4.2), which results in destabilization of the membrane lipid bilayer.

This destabilization allows a release of lipids from the membrane, causing a reduction in membrane surface area and a lack of deformability, leading to sequestration and destruction of the spherocytic erythrocytes in the spleen.

Although less common than glucose-6-phosphate dehydrogenase (G6PD) deficiency overall, pyruvate kinase deficiency is the most common RBC enzyme deficiency to cause congenital chronic hemolytic anemia. (See Schwartz 10th ed., p. 1429.)

Question 19

Splenectomy is indicated as a treatment in which of the following conditions?

A. Cold-antibody autoimmune hemolytic anemia (AIHA)

B. Hodgkin’sdisease

C. G6PD deficiency

D. Abscesses of the spleen

Answer 19

Answer: D
Autoimmune hemolytic anemias (AIHA) are characterized by destruction of RBCs due to autoantibodies against RBC antigens.

AIHA is divided into warm and cold categories based on the temperature at which the autoantibodies exert their effect.

In cold-agglutinin disease severe symptoms are uncommon and splenectomy is almost never indicated.

Warm-agglutinin disease presents with mild jaundice as well as symptoms and signs of anemia with one-third to one-half of patients presenting with splenomegaly. Initial treatment is with corticosteroids with splenectomy being second-line therapy with failure of steroids.

Although splenectomy has a 60 to 80% response rate recurrence is common. Hodgkin’s disease is a disorder of the lymphoid system characterized by the presence of Reed-Sternberg cells.

Most patients present with lympadenopathy above the diaphragm with adenopathy below the diaphragm rare on presentation.

Adenopathy below the diaphragm can arise with disease progression and the spleen is often an occult site of spread although splenomegaly is uncommon.

While splenectomy is performed for surgical staging in certain cases including clinical suspicion of lymphoma without evidence of peripheral disease or restaging or suspicion of failure after chemotherapy, staging laparotomy is less commonly performed in the current era of minimally invasive surgery and advanced imaging techniques.

G6PD deficiency is the most common RBC enzyme deficiency and can be characterized by chronic hemolytic anemia, acute intermittent hemolytic episodes, or no hemolysis depending on the variant.

Treatment for G6PD deficiency involves avoidance of drugs known to precipitate hemolysis.

Treatment for splenic abscess involves initiation of broad-spectrum antibiotics with tailoring of antibiotic therapy once culture results become available as well as splenectomy.

While splenectomy is the procedure of choice percutaneous or open drainage are options for patients unable to tolerate splenectomy. (See Schwartz 10th ed., p. 1431.)

Question 20

The disproportionately high rate of overwhelming post-splenectomy infection (OPSI) in thalassemia patients is thought to be due to an immune de ciency. Which of the
following strategies has been shown to reduce mortality?

A. Partial splenectomy.

B. Prophylactic antibiotic therapy.

C. Delaying splenectomy until after 2 years of age.

D. Transfusion to maintain a hemoglobin (HGB) of >9mg/dL.

Answer 20

Answer: A

The increase in infectious complications associated with splenectomy in thalassemia patients is thought to be due to a coexisting immune deficiency that is caused by iron overload.

Iron overload is associated with both thalassemia as well as the transfusions that accompany treatment or thalassemia.

Some investigators have tried partial splenectomy with some success in reducing mortality associated with splenectomy in these patients.

In addition, splenectomy should be delayed until the patient is older than 4 years unless absolutely necessary.

While transfusion to maintain a hemoglobin (HGB) of >9mg/dL is part of the treatment for thalassemia it does not reduce infectious complications associated with splenectomy in these patients.

There is little evidence supporting efficacy of prophylactic antibiotics in asplenic patients in preventing infectious complications associated with splenectomy. (See Schwartz 10th ed., p. 1432.)

Question 21

A 30-year-old woman presents to her primary care provider with complaints of bleeding gums while brushing her teeth as well as menorrhagia and several episodes of epistaxis within the past month. She has been previously healthy with no prior medical problems or surgeries. Examination reveals petechiae and ecchymosis over the lower extremities. Laboratory results show white blood cell (WBC) count 7000/mm3, HGB 14 g/dL, hematocrit (HC ) 42%, and platelet count 28,000/mm3 with numerous megakaryocytes on peripheral smear. First-line therapy for this condition would be

A. Oral prednisone
B. Intravenous (IV) immunoglobulin
C. Rituximab
D. Splenectomy

Answer 21

Answer: A

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a low platelet count and mucocutaneous and petechial bleeding.

The usual first line of therapy for ITP is oral prednisone with most responses occurring within the first 3 weeks after initiating therapy.

Intravenous (IV) immunoglobulin is given for internal bleeding with platelet counts <5000/mm3, when extensive purpura exists, or to increase platelets preoperatively and is thought to work by impairing clearance of immunoglobulin G-coated platelets by competing or binding to tissue macrophage receptors.

Both rituximab and thrombopoietin-receptor antagonists are second-line treatment options. Splenectomy is an option for refractory ITP and can provide a permanent response in about 75 to 85% of patients. (See Schwartz 10th ed., pp. 1432–1433.)

Question 22

The most common physical finding in a patient with hairy cell leukemia (HCL) is

A. Massivesplenomegaly
B. Shortness of breath
C. Abdominal pain
D. Joint pain

Answer 22

Answer: A

Hairy cell leukemia (HCL) is an uncommon blood disorder, representing only 2% of all adult leukemias.

HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in the bone marrow.

These lymphocytes contain irregular hair-like cytoplasmic projections identifiable on the peripheral smear.

Most patients seek medical attention because of symptoms related to anemia, neutropenia, thrombocytopenia, or splenomegaly.

The most common physical finding is splenomegaly, which occurs in 80% of patients with HCL and the spleen is often palpable 5 cm below the costal margin.

Many patients with HCL have ew symptoms and require no specific therapy.

Treatment is indicated
or those with moderate to severe symptoms related to cytopenias, such as repeated infections or bleeding episodes, or to splenomegaly, such as pain for early satiety.

Splenectomy does not correct the underlying disorder, but does return cell counts to normal in 40 to 70% of patients and alleviates pain and early satiety. Newer chemotherapeutic agents (the purine analogues 2ʹ-deoxyco ormycin [2ʹ-DCF] and 2-chlorodeoxyadenosine [2-CdA]) are able to induce durable complete remission in most patients. (See Schwartz 10th ed., p. 1434.)

Question 23

Which of the following is an indication
a patient with chronic myelogenous leukemia (CML)?

A. Failure of chemotherapy to decrease splenomegaly

B. Sequestration requiring transusion

C. Symptomatic relief of early satiety

D. Presence of bcr gene mutation

Answer 23

Answer: C

Chronic myelogenous leukemia (CML) is a disorder of the primitive pluripotent stem cells in the bone marrow, resulting in a significant increase in erythroid, megakaryotic, and pluripotent progenitors in the peripheral blood smear.

The genetic hallmark is a transposition between the bcr gene on chromosome 9 and the abl gene on chromosome 22.

CML accounts or 7 to 15% of all leukemias, with an incidence of 1.5 in 100,000 in the United States.

CML is frequently asymp- tomatic in the chronic phase, but symptomatic patients often present with the gradual onset of fatigue, anorexia, sweating, and left upper quadrant pain and early satiety secondary to splenomegaly.

Enlargement of the spleen is found in roughly one-half of patients with CML.

Splenectomy is indicated to ease pain and early satiety. (See Schwartz 10th ed., p. 1435.)

Question 24

Which of the following is an indication for splenectomy in polycythemia vera (PV)?

A. Failure of aspirin to prevent thrombotic complications

B. Frequent need orphlebotomy

C. Symptoms related to splenomegaly

D. Prevention of progression to myeloid metaplasia

Answer 24

Answer: C

Polycythemia vera (PV) is a clonal, chronic, progressive myeloproliferative disorder characterized by an increase in RBC mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly.

Patients affected by PV typically enjoy prolonged survival compared to others affected by hematologic malignancies, but remain at risk for transformation to myelofibrosis or acute myeloid leukemia (AML).

The disease is rare, with an annual incidence of 5 to 17 cases per million population.

Although the diagnosis may be discovered by routine screening laboratory tests in asymptomatic individuals, affected patients may present with any number of nonspecific complaints, including headache, dizziness, weakness, pruritus, visual disturbances, excessive sweating, joint symptoms, and weight loss.

Physical findings include ruddy cyanosis, conjunctival plethora, hepatomegaly, splenomegaly, and hypertension.

The diagnosis is established by an elevated RBC mass (>25% o mean predicted value), thrombocytosis, leukocytosis, normal arterial oxygen saturation in the presence of increased RBC mass, splenomegaly, low serum erythropoietin (EPO) stores, and bone marrow hypercellularity.

Treatment should be tailored to the risk status of the patient and ranges from phlebotomy and aspirin to chemotherapeutic agents.

As in essential thrombocythemia (ET) splenectomy is not helpful in the early stages of disease and is best reserved for late-stage patients in whom myeloid metaplasia has developed and splenomegaly-related symptoms are severe.
(See Schwartz 10th ed., pp. 1435–1436.)

Question 25

Which of the following is the most common etiology of splenic cysts worldwide?

A. Bacterial in ection
B. Trauma
C. Parasitic infection
D. Congenitalanomaly

Answer 25

Answer: C

Splenic cysts are rare lesions. The most common etiology for splenic cysts worldwide is parasitic infestation, particularly echinococcal.

Symptomatic parasitic cysts are best treated with splenectomy, though selected cases may be amenable to percutaneous aspiration, instillation of protoscolicidal agent, and reaspiration.

Nonparasitic cysts most commonly result
rom trauma and are called pseudocysts; however, dermoid, epidermoid, and epithelial cysts have been reported as well.

The treatment of nonparasitic cysts depends on whether or
not they produce symptoms.

Asymptomatic nonparasitic cysts may be observed with close ultrasound follow-up to exclude significant expansion.

Patients should be advised of the risk of cyst rupture with even minor abdominal trauma if they elect nonoperative management or large cysts.

Small symptomatic nonparasitic cysts may be excised with splenic preservation, and large symptomatic nonparasitic cysts may be unroofed.

Both of these operations may be performed laparoscopically. (See Schwartz 10th ed., pp. 1436–1437.)

Question 26

Which of the following is an indication for surgical treatment of asplenic aneurysm?

A. Pregnancy
B. Size >1.5 cm
C. History of thrombocytopenia
D. History of neutropenia

Answer 26

Answer:A

Although rare, splenic artery aneurysm (SAA) is the most common visceral artery aneurysm.

Women are our times more likely to be a ected than men.

The aneurysm usually arises in the middle to distal portion o the splenic artery.

The risk of rupture is between 3% and 9%; however, once rupture occurs, mortality is substantial (35–50%).

According to a recent series, mortality is significantly higher in patients with underlying portal hypertension (>50%) than in those without it (17%).

SAA is particularly worrisome when discovered during pregnancy, as rupture imparts a high risk o mortality to both mother (70%) and fetus (95%).

Most patients are asymptomatic and seek medical attention based on an incidental radiographic finding. About 20% of patients with SAA have symptoms of left upper quadrant pain.

Indications or treatment include presence of symptoms, pregnancy, intention to become pregnant, and pseudoaneurysms associated with inflammatory processes.

For asymptomatic patients, size greater than 2 cm constitutes an indication for surgery. Aneurysm resection or ligation alone is acceptable or amenable lesions in the mid-splenic artery, but distal lesions in close proximity to the splenic hilum should be treated with concomitant splenectomy.

An excellent prognosis follows elective treatment. Splenic artery embolization has been used to treat SAA, but painful splenic infarction and abscess may follow. (See Schwartz 10th ed., p. 1438.)

Question 27

A 45-year-old man presents to the emergency department with emesis of bright red blood. Laboratory results include HGB 10 g/dL, HC 30%, platelets 300,000/mm3, international normalized ratio (INR) 1.0, aspartate transaminase (AST) 30 U/L, alanine transaminase (ALT) 45 U/L, and albumin 4.0 g/dL. After appropriate resuscitation he undergoes esophagogastroduodenoscopy (EGD) which is notable for gastric varices. What is the appropriate treatment for this condition?

A. Transjugular intrahepatic portosystemic shunt

B. Variceal band ligation

C. Splenorenal shunt

D. Splenectomy

Answer 27

Answer: D

While portal hypertension is most commonly a result o cirrhosis it can result rom other causes such as splenic vein thrombosis.

Patients with splenic vein thrombosis can present with bleeding rom gastric varices in the setting of normal liver function test results.

These patients also often have a history of pancreatic disease.

Portal hypertension secondary to splenic vein thrombosis is potentially curable with splenectomy. (See Schwartz 10th ed., p. 1438.)

Question 28

Which of the ollowing is NOT part of the triad seen with Felty syndrome?

A. Rheumatoidarthritis (RA) B. Splenomegaly
C. Neutropenia
D. Thrombocytopenia

Answer 28

Answer: D

The triad of rheumatoid arthritis (RA), splenomegaly, and neutropenia is called Felty syndrome. It exists in approximately 3% of all patients with RA, two-thirds of which are women.

Immune complexes coat the surface of WBCs, leading to their sequestration and clearance in the spleen with subsequent neutropenia.

This neutropenia (<2000/mm3) increases the risk for recurrent infections and often drives the decision for splenectomy.

The size of the spleen is variable,
from nonpalpable in 5 to 10% of patients, to massive enlargement in others.

The spleen in Felty syndrome is our times heavier than normal.

Corticosteroids, hematopoietic growth factors, methotrexate, and splenectomy have all been used to treat the neutropenia of Felty syndrome.

Responses to splenectomy have been excellent, with over 80% of patients showing a durable increase in WBC count.

More than one-half of patients who had infections prior to surgery did not have any infections after splenectomy.

Besides symptomatic neutropenia, other indications or splenectomy include transfusion- dependent anemia and profound thrombocytopenia. (See Schwartz 10th ed., p. 1438.)

Question 29

Which of the ollowing is the most effective prevention strategy against OPSI?

A. Vaccination 2 weeks after splenectomy

B. Vaccination 2 weeks before splenectomy

C. Daily antibiotic prophylaxis

D. Carrying a reserve supply of antibiotics or self-administration

Answer 29

Answer: B

Asplenic patients have an increased susceptibility to infection or the remainder of their lives and although the overall lifetime risk of OPSI is low the consequences can be devastating.

Patients undergoing splenectomy or hematologic or malignant indications have a greater risk of OPSI than patients undergoing splenectomy or trauma or iatrogenic injury, and OPSI is more common in children than adults.

Providers need to have a high index of suspicion when evaluating asplenic patients or possible infection.

Patient education and vaccinations against encapsulated pathogens is the mainstay of preventative therapy.

Patients should be vaccinated 2 weeks prior to elective splenectomy in order to optimize antigen recognition and processing.

If splenectomy is performed emergently vaccinations are given postoperatively with an attempt to delay administration for 2 weeks to avoid the transient immunosuppression associated with surgery.

There is little evidence supporting efficacy of prophylactic antibiotics in asplenic patients and vaccination remains the most effective prevention strategy. (See Schwartz 10th ed., p. 1439.)

Question 30

All of the following are true regarding laparoscopic splenectomy EXCEPT

A. It is associated with shorter hospital stays.

B. It is associated with increased intraoperative blood loss.

C. It is associated with decreased morbidity.

D. Patients are positioned in the right lateral decubitus position or the 45° right lateral decubitus position.

Answer 30

Answer: B

Laparoscopic splenectomy has become the favored procedure versus open splenectomy or elective splenectomy over the past two decades and is now considered the gold standard or elective splenectomy in patients with normal-sized spleens.

With experienced surgeons laparoscopic splenectomy is associated with decreased intraoperative blood loss, shorter hospital length o stay, and lower morbidity rates as compared to open splenectomy.

Laparoscopic splenectomy is often per-
ormed with the patient in the right lateral decubitus position, patients are sometimes placed in a 45° right lateral decubitus position to acilitate easier access or concomitant procedures such as laparoscopic cholecystectomy. (See Schwartz 10th ed., p. 1440.)

Question 31

What is the most common complication following open splenectomy?

A. Pancreatitis
B. Left lower lobe atelectasis
C. Pleural effusion
D. Wound infection

Answer 31

Answer: B

Complications following splenectomy can be divided into pulmonary, hemorrhagic, infectious, pancreatic, and thromboembolic.

Pulmonary complications include left lower lobe atelectasis, pleural effusion, and pneumonia with left lower lobe atelectasis being the most common complication overall.

Hemorrhagic complications include intraoperative hemorrhage, postoperative hemorrhage, and subphrenic hematoma.

Infectious complications include subphrenic abscess and wound infection.

Placement of a drain in the left upper quadrant can be associated with postoperative subphrenic abscess and is there ore not routinely recommended.

Pancreatic complications include pancreatitis, pseudocyst formation, and pancreatic fistula and often result from intraoperative trauma to the pancreas during dissection of the splenic hilum.

Thromboembolic complications include deep vein thrombosis and portal vein thrombosis. (See Schwartz 10th ed., p. 1444.)

Question 32

Which of the ollowing patients is at highest risk for OPSI?

A. A 30-year old who underwent splenectomy for ITP

B. A 25-year old who underwent splenectomy for iatrogenic bleeding after a total colectomy.

C. A 3-year old who underwent splenectomy or hereditary spherocytosis.

D. A 4-year old who underwent splenectomy due to bleeding after a motor vehicle crash.

Answer 32

Answer: C

While the all lifetime risk of OPSI is low (ranging from <1–5%) the consequences are serious.

The reason for splenectomy is the single most influential determinant of OPSI risk.

There is evidence that those who undergo splenectomy
for hematologic disease are far more susceptible to OPSI than patients who undergo splenectomy for trauma or iatrogenic reasons.

When taking age into consideration children who are 5 years of age or younger and adults who are 50 years of age or older seem to be at an elevated risk.

The interval since splenectomy also seems to be a Factor with the greatest risk occurring in the first 2 years after splenectomy, however, it is important to remember that cases of OPSI can occur decades later and asplenic patients remain at lifelong risk. (See Schwartz 10th ed., p. 1445.)

Question 33

Which of the ollowing asplenic patients should receive prophylactic antibiotic therapy to protect against OPSI?

A. A 35-year-old man undergoing a tooth extraction.

B. A4-year-old child who recentlyunderwent splenectomy.

C. A 15-year-old boy who underwent splenectomy at
age 13.

D. There is little evidence supporting efficacy of prophylactic antibiotics.

Answer 33

Answer:D

Antibiotic therapy in asplenic patients falls into three categories: deliberate therapy for established or presumed infections, prophylaxis in anticipation of invasive procedures, and general prophylaxis.

There is little evidence supporting efficacy of prophylactic antibiotics in anticipation of invasive procedures or efficacy of general prophylaxis and guidelines are not uniform.

Common recommendations include daily antibiotics until 5 years of age or at least 5 years after splenectomy with some advocating continuing antibiotics until young adulthood, however, there is little evidence supporting efficacy.

It is unlikely that randomized controlled trials on this issue will be performed due to the low incidence of overwhelming post- splenectomy infection as well as its serious consequences. (See Schwartz 10th ed., p. 1445.)

Question 34

Which of the following statements regarding splenic anatomy is true?

A. The splenic ligaments are all avascular.

B. The tail of the pancreas is often contained in the splenorenal ligament.

C. The average weight of the adult spleen is 300 g.

D. The first branches of the splenic artery are the short gastric arteries.

E. The accessory spleens are most commonly found in the greater omentum.

Answer 34

ANSWER: B

COMMENTS: Although the majority of the splenic ligaments are in fact avascular, the gastrosplenic ligament contains the short gastric vessels.

Additionally, in the case of portal hypertension, other splenic ligaments may become vascularized.

The tail of the pancreas may be injured during splenectomy because it often lies within the splenorenal ligament.

The average weight of the adult spleen is 150 g (range, 75 to 300 g).

The first branches of the splenic artery are the pancreatic branches and then the short gastric, the left gastroepiploic (which may also give rise to the short gastric arteries), and the terminal splenic branches.

The splenic artery divides into segmental branches that enter the trabeculae of the spleen.

There are two types of anatomy of the splenic artery: the distributive and magistral.

The distributive subtype is much more common (70% of individuals) and is characterized by a short splenic artery trunk and multiple long branches entering the spleen.

Conversely, the magistral type has a long main trunk that divides relatively near the hilum.

Accessory spleens are common, especially in patients with hematologic disorders, and are found in 15%–35% of patients.

In decreasing order of frequency, they are found in the splenic hilum, gastrosplenic ligament, splenocolic ligament, splenorenal ligament, greater omentum and mesentery, and the left pelvis along the left ureter or by the left testis or ovary, but they have been identified anywhere within the peritoneal cavity.

Question 35

Which statement regarding the segments and function of the
spleen is true?

A. The white pulp consists of lymphatic sheaths surrounding vessels and is where B-lymphocyte precursors mature before migrating to the red pulp.

B. The white pulp usually constitutes 50% of a normal spleen.

C. The major function of the red pulp is to store old or defective erythrocytes for future use.

D. The marginal zone is the zone usually absent except in the presence of lymphoma.

E. The spleen can act as a large reservoir of platelets, erythrocytes, and other lymphatic cells.

Answer 35

ANSWER: E

COMMENTS: The white pulp of the spleen consists of lymphatic sheaths that usually surround splenic blood vessels.

The white pulp contains plasma cells and lymphocytes and functions as the immune center of the spleen.

The lymphatic sheaths contain mostly T cells, as well as some B-cell follicles that can be either primary or secondary (after stimulation by antigen).

The white pulp makes up approximately 25% of a normal spleen.

The red pulp is the largest component of the spleen and represents a network of sinuses that filter the blood.

Here, red cells are removed from the circulation and destroyed.

The junction between the red pulp and white pulp is the marginal zone, which allows additional antigen presentation and contributes further to the lymphatic functions of the spleen.

The spleen serves as a large reservoir. As much as 30% of an individual’s platelets reside in the normal spleen.

In disease states such as portal hypertension, splenic sequestration can trap an even larger proportion of the body’s circulating cells, including as much as 90% of the total number of platelets.

Question 36

A 49-year-old woman with Felty’s syndrome undergoes successful splenectomy. Several years after surgery, examination of her peripheral blood smear would reveal which one of the following to be true?

A. Howell-Jolly bodies, which are suggestive of the presence of an accessory spleen

B. Stippling, spur cells, and target cells because of the lack of filtration

C. High levels of properdin and tuftsin

D. No change in the level of antibodies needed to clear organisms as in the presplenectomy state

E. Red blood cells undergoing maturation more quickly

Answer 36

ANSWER: B

COMMENTS: Howell-Jolly bodies are abnormal cytoplasmic inclusions within red blood cells.

They are seen in individuals who have undergone splenectomy because normally they are removed by a functioning spleen, and thus their absence would suggest the presence of an accessory spleen.

Stippling, spur cells, and target cells are all functionally altered erythrocytes that are normally cleared from the circulation by the spleen and thus are commonly seen following splenectomy.

Properdin and tuftsin are important opsonins manufactured in the spleen. Properdin helps initiate the alternative pathway of complement activation, which is particularly useful for fighting encapsulated organisms.

Tuftsin enhances the phagocytic activity of granulocytes.

Asplenic individuals lack the ability to produce these substances. The spleen is the initial site of immunoglobulin (Ig) M synthesis in response to bacteria. Without this primary defense mechanism, asplenic individuals require increased levels of antibodies to clear organisms relative to the presplenectomy state.

Erythrocytes do not undergo maturation more quickly after splenectomy.

As part of its “pitting” function, the spleen removes cytoplasmic inclusions [particles such as nuclear remnants (Howell-Jolly bodies), insoluble globin precipitates (Heinz bodies), and endocytic vacuoles] from within circulating red blood cells.

Felty’s syndrome is an uncommon disorder marked by splenomegaly, neutropenia, and rheumatoid arthritis.

Patients may have thrombocytopenia and anemia, with a predisposition to infections.

Splenectomy in patients with Felty’s syndrome is beneficial in correcting the anemia and neutropenia associated with this syndrome.

Question 37

A 39-year-old male comes to you with easing bruising and mucosal bleeding. His complete blood count (CBC) shows a hemoglobin level of 8.2 g/dL and platelet count of 56,000/ mm3. You obtain a peripheral smear that shows increased megakaryocytes and reticulocytes. Which of the following is associated with this condition?

A. Splenomegaly must exist for this condition to occur.

B. All patients with this condition will have reticulocytosis.

C. Early satiety is an important symptom that may suggest splenomegaly.

D. Left upper quadrant pain is the most common symptom in this process.

E. The disease will not correct with splenectomy.

Answer 37

ANSWER: C

COMMENTS: The criteria for the diagnosis of hypersplenism are (1) documented anemia, thrombocytopenia, or leukopenia; (2) normal compensatory response from the bone marrow to correct the cytopenia; and (3) correction of the cytopenia by splenectomy.

In patients with splenomegaly, all activities of the spleen are thought to be markedly accentuated. Although splenomegaly is almost always associated with hypersplenism, not all cases of hypersplenism are associated with splenomegaly. Patients may have an anatomically normal spleen with abnormal function.

The most frequently reported symptom of splenomegaly is early satiety from the mass effect of the enlarging spleen on the stomach.

The left upper quadrant pain is more frequently associated with conditions that cause an acute distension of the splenic capsule such as abscess or hematoma formation after trauma.

Question 38

A 24-year-old female is to undergo an elective splenectomy for β-thalassemia. Which of the following recommendations should you make to her regarding vaccinations?

A. She should be vaccinated against Streptococcus pneu- moniae, Neisseria meningitidis, and Haemophilus influenzae 14 days before splenectomy.

B. She should be vaccinated against S. pneumoniae, N. meningitidis, and H. influenzae at the time of splenectomy.

C. She should be vaccinated against S. pneumoniae, N. meningitidis, and H. influenzae 1 year after splenectomy.

D. She should be vaccinated against S. pneumoniae alone 14 days after splenectomy and given a booster dose of N. meningitidis vaccine.

E. She should be vaccinated against S. pneumoniae alone 14 days before splenectomy and given a booster dose of N. meningitidis vaccine.

Answer 38

ANSWER: E

COMMENTS: Asplenic patients are at an increased risk of severe sepsis following splenectomy.

A series of case reports in the 1980s and 1990s reports about a 3.2% incidence of postsplenectomy sepsis.

Patients are at the highest risk in the first year after splenectomy.

Since the spleen has an important immunologic role in the opsonization of encapsulated organisms, asplenic patients are at an increased susceptibility to these bacteria—S. pneumoniae, H. influ- enzae, and N. meningitidis.

The most common organism that causes postsplenectomy sepsis is S. pneumoniae.

The current guidelines recommend that all patients receive the S. pneumoniae vaccine 14 days prior to surgery.

The ideal timing for vaccination after trauma splenectomy or unanticipated splenectomy during surgery for other reasons should be 14 days postoperatively.

The patient in this question is 24 years old and has already presumably received her Haemophilus influenza type B (HiB) vaccine and therefore does not need to be redosed.

If she had not been vaccinated against HiB, the vaccination would be indicated. She has also most likely received an N. meningitidis vaccine as well and therefore only needs a booster dose.

Question 39

A 4-year-old male with sickle cell anemia undergoes splenectomy for sequestration crisis. His mother wants to know if he is now at an increased risk for infection and would like to know if he is going to require lifelong antibiotics. How do you advise her?

A. He needs to take lifelong daily prophylaxis with amoxicillin.

B. He needs daily prophylaxis with amoxicillin until age 5 and 1 year after splenectomy.

C. Antibiotics prophylaxis is not indicated.

D. Antibiotic prophylaxis is not indicated unless he becomes immunocompromised.

E. He only needs to take antibiotics if he develops a fever.

Answer 39

ANSWER: B

COMMENTS: Patients and families need to be educated that the asplenic state carries a small risk of overwhelming and life-threatening infection.

Sepsis in this population carries a 50% mortality risk.

It is crucial that asplenic patients adhere to vaccination and antibiotic prevention guidelines and inform all caregivers of their asplenic state.

It is recommended that all asplenic children under age 5 receive daily oral antibiotic prophylaxis with a penicillin derivative or antibiotic with similar coverage.

If children receive a splenectomy at age 4, it is recommended that they continue prophylaxis for 1 year following splenectomy when the rate of infection is the highest.

Question 40

A 43-year-old man has thrombocytopenia, ecchymosis, and a history of melena. His primary doctor suspects that he might have idiopathic thrombocytopenia purpura (ITP). Which of the following is true about this condition?

A. It is characterized by a low platelet count, mucosal hemorrhage, normal bone marrow, and an enlarged spleen.

B. It is caused by a splenic overproduction of IgM, which attacks the platelet membrane and causes platelet destruction.

C. The bone marrow often hypertrophies to counteract the increased platelet destruction.

D. It affects young men more commonly than women.

E. Diagnosis requires exclusion of other causes of
thrombocytopenia.

Answer 40

ANSWER: E

COMMENTS: ITP is a disorder of increased platelet destruction caused by autoantibodies to platelet membrane components.

This results in platelet phagocytosis in the spleen, and the bone marrow does not adequately compensate for this increased destruction.

Although ITP is characterized by a low platelet count, mucosal hemorrhage, and relatively normal bone marrow (not hyperactive), the spleen is not enlarged.

The autoantibodies are IgG antibodies, not IgM, directed against the platelet fibrinogen receptor.

The mechanism underlying the use of intravenous (IV) IgG for the treatment of ITP is that IgG saturates the fibrinogen receptors so that they will not bind and thus destroy platelets.

This autoimmune disorder affects women more commonly than men.

A diagnosis of ITP requires exclusion of other potential causes of thrombocytopenia such as drugs, myelodysplasia, thrombotic thrombocytopenia purpura (TTP), systemic lupus erythematosus, lymphoma, and chronic disseminated intravascular coagulation.

Question 41

A 37-year-old female has just completed treatment with steroids for ITP. Her platelet count is currently 37,000/mm3. She is asymptomatic. Which of the following is the next step in her management?

A. Splenectomy
B. Rituximab
C. Intravenous immunoglobulin (IVIG) 
D. Observation
E. Eltrombopag

Answer 41

ANSWER: D

COMMENTS: The initial treatment for ITP is high-dose steroids, usually 1 mg/kg of prednisone a day.

The goal of therapy is to induce remission and increase platelet counts to >100,000/mm3.

This is effective in roughly 75% of patients.

Treatment is initiated when platelet counts fall to less than 20,000/mm3 or less than 50,000/mm3 in patients who are symptomatic.

Although the initial response to steroid therapy is good, it only persists in 15%–25% of patients.

Patients who are symptomatic and fail steroid therapy should then progress to receiving IVIG; if this fails at correction of thrombocytopenia, then elective splenectomy is the next step in management.

Not all patients respond to splenectomy.

For these patients, rituximab (a monoclonal antibody against CD20 on B cells) and eltrombopag (thrombopoietin receptor agonist) are fourth-line options.

The patient in this question is asymptomatic and therefore can be managed with observation.

Question 42

Which of the following is true of thrombocytopenic purpura?

A. It is characterized by widespread occlusion of arterioles and capillaries by hyaline membranes composed of platelets and fibrinogen.

B. Splenectomy is curative and is the first line for treatment.

C. Central nervous system manifestations such as intracere- bral hemorrhage are exceedingly rare.

D. The pathogenesis is well understood and is marked by antibodies to vessel wall antigen.

E. Plasmapheresis is only used as a salvage therapy in the treatment of TTP.

Answer 42

ANSWER: A

COMMENTS: This patient has thrombotic thrombocytopenic purpura.

The disease is characterized by occlusion of arterioles and capillaries by hyaline deposits of aggregated platelets and fibrin.

The pathogenesis is not well understood or described; the most accepted theory is that it is an autoimmune disease that causes there to be antibodies to small vessel antigens.

The success of plasma- pheresis as a treatment supports this idea, but a signal antibody has not been identified.

The first-line therapy is plasmapheresis.

Fresh- frozen plasma and high-dose corticosteroids may be used to control bleeding.

Splenectomy is not curative and is considered only for salvage therapy.

Mortality rates in patients with TTP can approach 50%, mostly from intracranial hemorrhage or renal failure.

The disease can have a rapidly fulminant course. Most long-term survivors of TTP have undergone splenectomy.

Platelet transfusion does not control the bleeding, and therapy should be focused on high-volume plasmapheresis.

Question 43

A 17-year-old male is being worked up for jaundice and anemia. His peripheral smear shows spherocytes. His Coomb’s test is negative, and an osmotic fragility test is positive. Other than his jaundice, he denies any other symptoms. Right upper quadrant ultrasound shows a normal gallbladder without stones. What is the best surgical option to offer this patient?

A. Surgery is not indicated
B. Splenectomy
C. Cholecystectomy
D. Splenectomy and cholecystectomy 
E. Bone marrow biopsy

Answer 43

ANSWER: B

COMMENTS: Hereditary spherocytosis (HS) is inherited in an autosomal dominant fashion and is the most common of the con- genital hemolytic anemias.

It affects around 1/5000 individuals.

There are numerous genetic defects responsible for this disease that affect proteins on the cytoskeleton of red blood cells—spectrin and ankyrin.

This causes loss of flexibility and makes them more sus- ceptible to destruction in the spleen.

Diagnosis is made by peripheral smear, which will exhibit spherocytes.

Spherocytes can also be seen in other hemolytic anemias, but a Coomb’s test will be negative only with HS.

The osmotic fragility test is diagnostic. Treat- ment is splenectomy, which should be delayed until children are at least 4 to 6 years of age.

Moreover, 30%–60% of patients with HS will develop gallstones.

Concomitant splenectomy and cholecystectomy is recommended in patients with gallstones.

The patient in this question is symptomatic from his HS and requires splenectomy; he does not have gallstones on ultrasound and therefore does not need his gallbladder removed at this time.

Question 44

Which of the following hematologic disorders is unlikely to respond to splenectomy?

A. Idiopathic thrombocytopenic purpura

B. Autoimmune hemolytic anemia secondary to warm antibodies

C. Autoimmune hemolytic anemia secondary to cold antibodies

D. HS

E. Thalassemia

Answer 44

ANSWER: C

COMMENTS: Hematologic disorders that respond to splenectomy are conditions of cells that are destroyed in the spleen.

The spleen contains macrophages with Fc fragments that bind to IgG. ITP is caused by IgG antibodies to platelets; autoimmune hemolytic anemias are either caused by IgG (warm) or IgM (cold) antibodies. IgM is not cleared in the spleen but in the liver.

Therefore splenectomy is not helpful in patients with an autoimmune hemolytic anemia due to cold antibodies.

Patients with HS and thalassemia do benefit from splenectomy.

Question 45

A 47-year-old woman with a history of a radical mastectomy 20 years previously has had a long-standing lymphedema of her upper extremity on the treated side. She is now com- plaining of a reddish blue nodule on her arm and dyspnea on exertion. Which one of the following is not true regarding her condition?

A. The lymphatic system is a collection of small lymphatic vessels that parallel the major blood vessels and may contain red blood cells, bacteria, and proteins.

B. The lymphatic system has valves.

C. Her reddish blue nodule probably represents a benign condition related to a long-standing lymphedema.

D. Extrinsic factors such as muscle contraction, arterial pulsation, and respiratory movement aid in the movement of lymph flow.

E. Measurement of the protein content of edema fluid can be used to assess the lymphatic function of her arm.

Answer 45

ANSWER: C

COMMENTS: This patient has a lymphangiosarcoma following mastectomy complicated by untreated chronic lymphedema, or Stewart-Treves syndrome.

This malignant tumor is rare and typically occurs in patients with long-standing lymphedema.

It is characterized by a rapid and aggressive course and a tendency to metastasize to the lungs early, as suggested by her history of dyspnea.

Treatment often involves multimodality therapy, and amputation of the limb may be necessary.

The lymphatic system begins as a network of valveless capillaries in the superficial dermis that drain into a secondary system of valved vessels in the deep or subdermal layer, which then drain into major lymphatic channels that parallel the major blood vessels.

Intradermal lymphatics can be evaluated by the intradermal injection of blue dye.

Lymphangiography is rarely done as a mapping technique for patients with lymphedema because it may worsen the symptoms.

Proteins, red blood cells, and lymphocytes that make their way into the extracellular fluid readily enter the lymphatic vessels.

Measurement of the protein content of edema fluid in an extremity can be used to assess the status of lymphatic function.

The protein content should be less than 1.5 mg/dL; higher values suggest declining lymphatic return.

Question 46

A 61-year-old heavy smoker undergoes computed tomography (CT) of the abdomen after a motor vehicle accident. The scan reveals a 4-cm irregular hypodensity in the spleen without other associated lymphadenopathy or masses. Which of the following statements is true regarding primary and metastatic tumors of the spleen?

A. Vascular neoplasms, including hemangiomas and angiosarcomas, are the most common primary tumors of the spleen.

B. With the exception of lymphomas, the spleen is rarely a site of metastatic involvement from primary tumors.

C. Splenectomy is often curative for primary tumors of the spleen.

D. A laparoscopic approach to splenectomy for malignancy is associated with inferior outcomes.

E. Most splenic metastases are symptomatic.

Answer 46

ANSWER: A

COMMENTS: Not infrequently, lung cancer metastasizes to the spleen. Other common primary tumors that may metastasize to the spleen include breast cancer and melanoma, as well as ovarian, gastric, and colon cancers.

The most common primary splenic tumors are vascular in origin and include hemangiomas (benign) and angiosarcomas (malignant).

They later may be associated with environ- mental exposure to vinyl chloride or thorium dioxide. Most splenic metastases are asymptomatic and are found at autopsy in about 7% of cancer patients. Occasionally, secondary tumors in the spleen may cause symptomatic splenomegaly or splenic rupture.

By the time that metastases are detected, splenectomy is rarely curative, but it may be palliative in appropriately selected symptomatic patients or be a reasonable therapy for isolated splenic metastases.

The laparoscopic approach to splenectomy is appropriate for most splenic tumors.

Question 47

A 32-year-old woman comes to the emergency department complaining of pain in her foot and calf. She reports that her left leg has been swollen for the last 15 years. She has a temperature of 101.5°F and reports that she had a splinter removed from her leg 1 week earlier. Her left lower extremity is swollen from the foot to the inguinal ligament, and she has erythema of the foot and calf. In addition to cellulitis, what is the most likely underlying diagnosis?

A. Chronic venous insufficiency

B. Deep venous thrombosis

C. Lymphedema praecox

D. Meige’s disease

E. Milroy’s disease

Answer 47

ANSWER: C

COMMENTS: Chronic venous insufficiency is usually bilateral and marked by signs of venous stasis such as hemosiderin deposits and possibly ulceration.

Deep venous thrombosis would be manifested acutely and would be unlikely to have such a long-standing history.

Swelling of an extremity from a pathologic condition of the lymphatic system is classified as primary or secondary lymphedema.

Primary lymphedema is an uncommon condition and is not related to any extrinsic process. Primary lymphedema is divided into three groups, depending on age at diagnosis.

With an onset before completion of the first year of life, it is called congenital lymphedema (or Milroy’s disease if associated with a family history).

Lymphedema praecox refers to the onset of primary lymphedema before the age of 35.

It is the most common form of primary lymphedema and affects women four times more commonly than men.

Onset is usually during puberty, and 70% of cases are unilateral, with the left side more com- monly affected than the right.

Lymphedema tarda (or Meige’s disease) refers to swelling of the legs that occurs after the age of 35. It is the least common form of primary lymphedema.

Secondary lymphedema can be the result of multiple disease processes, including but not limited to infection, trauma, filariasis, lymph node dissection, and exposure to radiation.

The mainstay of lymphedema treatment is conservative and nonoperative.

The goals of therapy are the prevention of infection and reduction of subcutaneous fluid volume with the use of decongestive massage therapy, pneumatic compression devices, and fitted elastic stockings.

Diuretics are not used routinely but may be useful in women with premenstrual fluid retention.

Recurrent lymphangitis is common following injury, and streptococci are the usual offending organisms. Penicillin is an appropriate therapy.

Rarely, a protein-losing enteropathy attributed to lymphatic obstruction of the small bowel develops in patients with lymphedema.

Various surgical procedures have been described for the treatment of lymphedema, including removal of skin, subcutaneous tissue, and fascia, followed by split-thickness skin graft reconstruction (the Charles operation); excision of strips of skin and subcutaneous tissue, followed by primary closure; and the creation of buried dermal flaps.

All these procedures are associated with significant failure rates.

Question 48

A 64-year-old female presents to you with a palpable lymph node in her right axilla. Her family history is positive for a maternal aunt with breast cancer diagnosed at age 78 and a paternal grandmother who died from breast cancer at 42. What is the most likely etiology of this mass?

A. Breast cancer
B. Melanoma
C. Lymphoma
D. Reactive lymphadenopathy 
E. Lung cancer

Answer 48

ANSWER: D

COMMENTS: Unilateral lymphadenopathy is a physical finding that should prompt a thorough evaluation, because a minority of patients (∼25%) will have an underlying malignancy.

The most common diagnosis in the setting of this presentation is reactive lymphadenopathy.

Of those that do have an underlying malignancy, lymphoma is the most common followed by a carcinoma.

Women with this presentation should have a formal breast examination and examination of the contralateral axilla.

Women over the age of 35 should also undergo a mammography.

The best imaging modality for the axilla is ultrasound, which can reliably predict lymph nodes suspicious for malignancy. A biopsy is warranted in patients with lymph nodes concerning for malignancy. For patients with a non- concerning history and physical examination and ultrasound showing benign-appearing lymph nodes, observation is appropriate.

Question 49

What is the next step in the management of the patient in Question 15?

A. Excisional biopsy
B. Breast examination and mammogram 
C. Fine-needle aspiration (FNA)
D. Ultrasound
E. Observation

Answer 49

ANSWER: B

COMMENTS: Unilateral lymphadenopathy is a physical finding that should prompt a thorough evaluation, because a minority of patients will have an underlying malignancy.

The most common diagnosis in the setting of this presentation is reactive lymphade- nopathy.

Of those that do have an underlying malignancy, lym- phoma is the most common followed by a carcinoma; only about 1% of women with breast cancer will present this way.

Women with this presentation should have a formal breast examination and examination of the contralateral axilla.

Women over the age of 35 should also undergo a mammography.

The best imaging modality for the axilla is ultrasound, which can reliably predict lymph nodes suspicious for malignancy.

A biopsy is warranted in patients with lymph nodes concerning for malignancy.

The patient in this question is a 64-year-old female with a positive family history of breast cancer; while the most likely diagnosis is reactive lymphadenopathy, an occult breast cancer must be excluded.

This patient should undergo a thorough breast examination and mammogram.

Question 50

Regarding lymphedema after breast cancer surgery, which of
the following is true?

A. It can occur after sentinel lymph node biopsy.

B. Exercise worsens lymphedema.

C. Weight loss does not have an effect on lymphedema.

D. Any therapy to reduce lymphedema should be delayed until the patient has completely healed from surgery.

E. Laser therapy has not been shown to decrease rates of lymphedema.

Answer 50

ANSWER: A

COMMENTS: Lymphedema is the accumulation of protein-rich fluid in tissue with inadequate lymphatic drainage.

Lymphedema occurs at rates reported from 20% to 30% after mastectomy and up to as high as 7% after sentinel lymph node biopsy.

Patients at risk of lymphedema should maintain a normal body weight, as obesity is a known risk factor for lymphedema.

Additionally, exercises specifically aimed at improving lymphedema have been developed and have been shown to be beneficial in the treatment and preven- tion of lymphedema.

Many institutions have initiated specific exercise regimens as soon as 48 h after surgery.

Question 51

A 56-year-old male presents to the emergency department complaining of nonspecific abdominal pain. A CT scan is ordered that shows a hypoattenuating well-defined intra- splenic lesion, with a thin wall and no peripheral enhancement. How would you manage this patient?

A. Splenectomy

B. Magnetic resonance imaging (MRI) of the abdomen

C. Angioembolization

D. Observation

E. Aspirate contents and then replace with hypertonic saline

Answer 51

ANSWER: D

COMMENTS: Splenic cysts are common lesions seen across all age groups and are often multifocal.

The diagnosis is usually made incidentally on ultrasound or CT scan.

Splenic cysts usually have no clinical significance unless they reach a large size and cause expansion of the splenic capsule.

Elective splenectomy has been performed for large symptomatic cysts.

Additional benign lesions of the spleen include hemangiomas, lymphangiomas, pseudotumors, hamartomas, and echinococcal cysts.

All of these lesions, except echinococcal cysts, can be managed expectantly until symptomatic from mass effect.

The echinococcal cyst is the only para- sitic cyst involving the spleen. The incidence of echinococcal cyst in the spleen compared with the liver is a ratio of approximately 30:1.

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

The treatment for echinococcal cysts can include splenectomy.

However, if there is a high risk of rupture, contents can be aspirated and replaced with hypertonic saline.

Angioembolization is not used for the treatment of splenic lesions; its use is reserved for cases of splenic bleeding.

Question 52

You are consulted on a 29-year-old male admitted to the medical intensive care unit (MICU) with a past medical history of IV drug abuse but otherwise healthy. He is febrile with a temperature of 102.3°F, heart rate of 117 beats/min, and blood pressure of 94/62 mmHg. A CT scan was obtained that shows a lesion with a peripherally enhancing rim. After a 2-L resuscitation, his heart rate decreases to 102 beats/min, and his blood pressure improves to 108/70 mmHg. How would you manage this patient?

A. IV antibiotics and observation

B. IV antibiotics alone

C. IV antibiotics and percutaneous drainage

D. IV antibiotics and splenectomy

E. Observation alone

Answer 52

ANSWER: D

COMMENTS: Splenic abscess is associated with significant mor- tality, which in most series ranges between 40% and 100%.

Typically, pathogenesis is caused by seeding of the spleen by bacteria from endocarditis or IV drug abuse.

There have been some instances where the abscess is spread by a direct contamination of an intraperitoneal source.

In 80% of cases additional sources of infection are identified, but in 20% of cases only the spleen is identified as an infectious source.

Enteric organisms account for two-thirds of splenic abscesses, and Staphylococcus and Strepto- coccus cause the remainder of the cases.

Presenting symptoms are generally fever and leukocytosis, with possible left upper quadrant tenderness.

Plain abdominal films may show gas in the left upper quadrant.

Otherwise ultrasound or CT scan can be diagnostic.

Splenectomy is the treatment of choice if the patient is stable enough to undergo laparotomy.

If patients are too unstable to undergo laparotomy or have multiple sources of infection, then percutaneous drainage is an option.

The patient in this question stabilized with fluids and had the spleen as his sole source of infection; therefore laparotomy with splenectomy would be an appropriate approach to management.

Question 53

After failing medical therapy, a 46-year-old woman with ITP is referred to you for splenectomy. She is very interested in a laparoscopic procedure. Review of her CT scan shows a normal-sized spleen and normal splenic vascular anatomy. Which of the following is true about laparoscopic splenectomy?

A. Operative mortality rates are the same regardless of the underlying disease type.

B. Laparoscopic splenectomy has similar success rates as open splenectomy, except when performed for ITP.

C. The rate of conversion from laparoscopic to open splenectomy is 0%–20%.

D. Laparoscopic splenectomy can be considered for spleen sizes up to 35 cm.

E. Laparoscopic splenectomy results in a higher incidence of splenosis than does the open approach.

Answer 53

ANSWER: C

COMMENTS: Laparoscopic splenectomy is increasingly being selected as a technique when elective splenectomy is indicated.

The operative morbidity and mortality rates after splenectomy are higher for patients with the malignant hematologic disease than for those with benign disease.

The risk of postoperative portal venous thrombosis is greatest for patients with myelopro- liferative disorders.

For idiopathic thrombocytopenic purpura, laparoscopic splenectomy has success rates similar to those of open splenectomy.

Regardless of the surgical approach, when splenectomy is performed for hematologic disease, a careful search for accessory spleens must be performed.

Their appearance may mimic that of a lymph node, and they may more easily be palpated than visualized, thus giving rise to concern that the laparoscopic approach may overlook some accessory spleens.

The conversion rate in an open procedure is reported to range from 0% to 20%. Conversion is usually secondary to bleeding, but extensive adhesions, obesity, and splenomegaly may also be factors.

Spleens up to 20 to 25 cm in size are amenable to laparoscopic splenectomy.

A splenic size of 35 cm is generally too large for a laparoscopic approach.

The laparoscopic approach does not result in a higher incidence of splenosis (autotransplan- tation and subsequent growth of splenic fragments from an injured spleen that may remain functional and occasionally cause pain or symptoms related to a mass effect).

Question 54

A 14-year-old girl is involved in a high-speed car accident and has a mildly distended abdomen, a seatbelt sign, and a positive focused abdominal sonography for trauma (FAST) examination. She is tachycardic and found to have a splenic injury on CT. She fails the nonoperative management over the next day because of transfusion requirements. At the time of exploration, which of the following is most correct?

A. Eighty percent of the spleen can be sacrificed at splenorrhaphy before total splenectomy should be performed.

B. Although available techniques for splenorrhaphy include argon beam coagulation, fibrin glue, and mattress suturing with pledgets, mesh wrap should not be done because of the increased risk for infection.

C. Splenorrhaphy is especially advantageous in the setting of pancreatic or hollow viscus injury because it decreases the occurrence of subphrenic abscess.

D. Splenorrhaphy may be attempted safely in patients with severe head injuries.

E. Splenorrhaphy is best used only for grades I and II injuries.

Answer 54

ANSWER: C

COMMENTS: Splenorrhaphy is a useful tool to allow preservation of the spleen.

Only one-third of the spleen is required for retention of its immunologic benefit; injuries requiring the sacrifice of more than two-thirds of the spleen are best treated by splenectomy.

Available tools for splenorrhaphy include all those listed in choice B.

The use of a mesh wrap has not contributed to an increased incidence of infection following splenorrhaphy, even in the setting of associated hollow viscus injury.

It is true that splenorrhaphy decreases the incidence of abscess formation following pancreatic or hollow viscus injury.

In unstable patients or those with severe head injuries, expeditious splenectomy should be performed if needed instead of a partial splenectomy or splenorrhaphy because the latter tends to be more time-consuming.

There is no grade restriction for performing splenorrhaphy after injury, and it may be done as long as one-third of the spleen remains viable for continued immunologic function.

Question 55

A 23-year-old sustains blunt force trauma in a high-speed
motor vehicle accident. Abdominal CT demonstrates a subcapsular hematoma involving 60% of the surface area of the spleen without obvious injury to the hilum. The patient has a heart rate of 110 beats/min and blood pressure of 105/60 mm Hg. Correct statements regarding the manage- ment of this patient include which of the following?

A. A Kehr’s sign is a contraindication to nonoperative management given the high associated severity of injury.

B. Diagnostic peritoneal lavage is not sensitive for the detection of splenic injury.

C. For patients with moderate to severe splenic injuries managed nonoperatively, follow-up abdominal CT is indicated in 2 to 3 days.

D. Angiography is required in all patients with splenic injury to exclude unsuspected areas of active hemorrhage not seen on CT.

E. Nonoperative management may be pursued, provided that the patient is hemodynamically stable; the injury can be clearly classified by imaging, and transfusion requirements remain less than six units of packed red blood cells.

Answer 55

ANSWER: C

COMMENTS: This patient has a grade III splenic injury.

Kehr’s sign (pain referred to the left shoulder) does correlate highly with splenic injury but does not mandate operative management.

Diagnostic peritoneal lavage is perhaps too sensitive in detecting significant splenic injury and has largely been replaced by ultrasound.

Follow-up scans are recommended to exclude any vascular blush not seen on initial imaging because of sampling error with larger cuts or subsequent lysis of clot.

Angiography, with possible angio-embolization, should be pursued if a vascular blush appears on CT in patients with grade III and higher injuries or in patients with any grade of injury if frank hemorrhage from the splenic artery is seen.

It is not required for all patients. Criteria for non-operative management include hemodynamic stability, documented CT classification of injury, absence of additional injuries necessitating surgery, and transfusion of two or fewer units of red blood cells (not six units).

The success of nonoperative management is reported to be 70%–90% for children and 40%–50% for adults treated in trauma centers.

The differing success rates may be related to both anatomic considerations and mechanisms of injury.

Question 56

4 peritoneal extensions (ligaments) of the spleen?

Answer 56

1) Splenocolic
2) Splenophrenic (relatively avascular)
3) Splenorenal (splenic vessels, tail of pancreas)
4) Gastrosplenic (short gastric vessels)

Question 57

Splenic blood supply?

Answer 57

1) Splenic artery
- off celiac trunk, multiple pancreatic branches, short gastric, left gastroepiploic, terminal splenic

2) Splenic vein
- Inferior to artery, posterior to pancreatic tail, body
- Joins SMV behind pancreatic neck > portal vein

Question 58

Spleen histology?

Answer 58

Red pulp (75%)

• Large numbers of venous sinuses draining into splenic veins
• Sinuses surrounded and separated by reticulum where macrophages lie
• Serves as dynamic filtration system where macrophages remove microorganisms, cellular debris, Ag and Ab complexes, senescent erythrocytea

White pulp

• Periarticular lymphatic sheaths
• Comprised of T lymphocytes and intermittent aggregations of B lymphocytes or lymphoid follicles.

Question 59

Splenic function?

Answer 59

• Early hematopoiesis
• Mechanical filtration of senescent erythrocytes
• Infection control
  —> Pathogens with RBCs: Malaria, Bartonella
  —> Clearance on unopsonized noningested bacteria from circulation
  —> Microorganisms without specific host antibody

Question 60

Immunologic function of spleen?

Answer 60

Major site of opsonins:

1) Tuftsin
- enhances phagocytic activity
- spleen is major cleavage sure—> decreased neutrophil function

2) Properdin
- initiates alternative pathway of complement activation

Question 61

Indications for splenectomy?

Answer 61

Most common INDICATION:
- Trauma to the spleen

Most common ELECTIVE INDICATION:

1) ITP
2) Hereditary spherocytosis
3) Autoimmune hemolytic anemia
4) Thrombotic thrombocytopenic purpura

Question 62

Diagnosis of splenic trauma?

Answer 62

1) Hx and PE
- peritoneal signs (42-72% accurate)
- bruising over LUQ
- Kehr sign: LUQ pain with referred left shoulder pain
- Hypotension, tachycardia (hemorrhage)
- Confounding factors: head, SCI, substance abuse)

2) DPL
- 1L crystalloid infusion
—> >100,000 RBCs, 500 WBC
- Sensitivity 99%
- Specificity 95-98%

3) CT scan- GradingI: Hematoma (subcapsular <10% of SA)
: Laceration (capsular tear <1cm parenchyma depth)II: Hematoma (subcapsular 10-50% SA; intraparenchymal <5cm diameter)
: Laceration (capsular tear 1-3cm parenchyma depth not involving a trabecular vessel)III: Hematoma (subcapsular, >50% SA; ruptured subcapsular or parenchyma hematoma); intraparenchymal hematoma >5cm or expanding
: Laceration (>3cm parenchyma depth, or involving trabecular vessels)

IV: Laceration involving segmental or hilar vessels producing major devascularization (>25% of spleen)

V: Laceration (completely shattered spleen)
: Vascular (hilar vascular injury which devascularizes spleen)

4) Ultrasound
- FAST
- Sensitivity 90-93%
- Specificity 99%

Question 63

Indications for splenectomy in trauma?

Answer 63

1) Unstable patient (SBP <90, HR>120bpm, unresponsive to 1-2L crystalloids)
2) Extensive injury with continued bleeding
3) Bleeding from hilar injury
4) Other life threatening injuries
- Divide short gastric arteries (avoid injury to stomach)
- Divide splenic artery + vein (avoid tail of pancreas)
- No drain needed.