Surgical Splenic Problems In The Child

Question 1

Name 2 anatomical abnormalities of the spleen:

Answer 1

1. Wandering spleen
   Lack of ligamentous attachments to the diaphragm, colon and retroperitoneam.
   Episodic pain is felt and an abdominal mass from torsion and infarction of the spleen.
2. Accessory spleen
   Most common anatomic abnormality of the spleen
   Located near the splenic hilum
   • Important not to be missed when performing a splenectomy for ITP
     > Idiopathic thrombocytopenic pupura

Question 2

What are 2 management solutions for wandering spleen?

Answer 2

Splenopexy:

• Surgically fixing an ectopic or floating spleen
• Done is the spleen is viable

Resection:
- If spleen is necrotic

Question 3

What does ITP stand for?

Answer 3

Idiopathic thrombocytopenia pupura

Question 4

Name 2 functional abnormalities of the spleen.

Answer 4

Hyposplenism

Hypersplenism

Question 5

Hypersplenism can be primary or secondary:

- What are causes of primary Hypersplenism?

Answer 5

Typically caused by:
1. Hereditary spherocytosis
(Which is a disorder of the membrane, of your RBC, causing them to be shaped like spheres instead of flattened discs that curve inward.)

2.Idiopathic thrombocytopenic purpura
Low levels of platelets

Question 6

Hypersplenism can be primary or secondary:

- Name one cause of secondary Hypersplenism.

Answer 6

Portal hypertension

Question 7

Name 3 causes of hyposplenism

Answer 7

1. Splenectomy
2. Asplenia
3. Sickle cell Anaemia w/splenic infarcts and eventual involution of spleen

Question 8

What is a consequence of hyposplenism?

Answer 8

Increased Infection

Question 9

How is hyposplenism detected?

Answer 9

Demonstrating Howell-Jolly bodies in the blood smear.

Question 10

What is hereditary spherocytosis?

Answer 10

Which is a disorder of the membrane, of your RBC, causing them to be shaped like spheres instead of flattened discs that curve inward.

Autosomal dominant disease

Question 11

Which cytoskeletal protein deficiency is seen in hereditary spherocytosis ?

Answer 11

Spectrin

Question 12

Name 5 clinical presentations of hereditary spherocytosis:

Answer 12

Jaundice
Anaemia
Splenomegaly 
Elliptocytosis. 
Pigmented gallstones

Question 13

Describe RBC found in patients presenting with hereditary spherocytosis:

Answer 13

Abnormal spherical shape.
Decreased wall flexibility.
- Premature sequestration of the spleen

Question 14

What are 2 management procedures for hereditary spherocytosis?

Answer 14

Splenectomy

Cholecystectomy

Question 15

What is idiopathic thrombocytopenic purpura (ITP)?

Answer 15

It is an autoimmune disease of unknown aetiology.

Antiplatelet antibodies, IgG, bind to platelet associated antigens causing:
- Destruction of platelets by phagocytosis in the spleen.

Acute ITP is seen in children after a viral infection
- ITP in Children: usually self-limiting

Question 16

What medical therapy is required for patients with ITP?

Answer 16

Corticosteroids

IV Immunoglobulins

Question 17

What medical procedure is required for patients that do not Escondido to medical therapy for ITP?

Answer 17

Splenectomy

Question 18

What is the genetic cause of sickle cell Anaemia?

Answer 18

Valine is substituted for Glutamic acid on the beta chain of Hemoglobin A
- This results in hemoglobin S

Question 19

Describe the RBC of patients with sickle cell:

Answer 19

RBC prone to become rigid and sickle in a hypoxia and acidic environment.

Question 20

What are the consequences of sickle cell disease?

Answer 20

Acute splenic sequestration crisis

• Rapidly enlarging spleen
• Severe Anaemia
• Thrombocytopenia

This is an indication for splenectomy.

Question 21

What is thalassemia?

Answer 21

This is a Haemoglobinopathy

- Associated with abnormal production of alpha and beta chains.

Question 22

What is a consequence of thalassemia?

Answer 22

Severe erythrocyte sequestration May occur within the spleen.

Question 23

How is thalassemia managed?

Answer 23

Splenectomy decreases transfusion requirements

Partial splenectomy might be useful in order to avoid postsplenectomy sepsis. 10% of all patients that undergo total splenectomies endure this.

Question 24

What is the purpose of a splenectomy?

Answer 24

Surgical splenectomies are used to ameliorate the sequele of hematological diseases.
(Prevent development of a pathological condition resulting from a prior disease)

• Can be done open or laparoscopically

Question 25

What important precautions should be taken before a splenectomy to prevent postspleectomy septic syndrome?

Answer 25

Immunization against

• Steptococcus pneumonia
• Meningococcus
• Haemophilus influenza B

Question 26

What important precautions should be taken after a splenectomy due to the danger of infection being highest in this time?

Answer 26

Prophylactic antibiotics