Sickle Cell Disease + Brain Tumour (10)

Question 1

What is sickle cell disease?

Answer 1

• Sickle cell disease is a hereditary hemoglobinopathy
  
  • caused by a point mutation in β-globin that promotes the polymerization of deoxygenated hemoglobin,
  • leading to
    
    1. red cell distortion
    2. hemolytic anemia
    3. microvascular obstruction
    4. tissue damage.

Question 2

A lady with sickle cell disease had a head injury and was accidentally found to have a 3.8 cm temporal mass on CT.

Why is the patient immunocompromised?

Answer 2

The patient is immunocompromised due to autosplenectomy, which increases susceptibility to infection with encapsulated organisms.

Question 2

What are the complications of sickle cell disease?

Answer 2

• Vaso-occlusive crisis: triggered by infection, dehydration, acidosis, affecting the bones (painful bone crisis as in hand-foot syndrome), lungs (acute chest syndrome), brain (stroke and retinopathy), spleen (autosplenectomy).
• Sequestration crisis: in children, massive entrapment of sickle cell in the spleen will lead to rapid splenic enlargement and hypovolemic shock.
• Aplastic crisis: due to infection of red cell progenitors by parvo-virus.
• Chronic tissue hypoxia: organ damage (spleen, heart, kidney, lungs).
• Increased susceptibility of infection with encapsulated organisms.

Question 3

What is autosplenectomy?

Answer 3

Autosplenectomy is a process in which the spleen shrinks progressively due to chronic erythrostasis, fibrosis, and splenic infarction caused by red pulp congestion that results from the trapping of sickled red cells in the cords and sinuses.

Question 4

What are the surgical relevance of sickle cell disease?

Answer 4

Gallstones, autosplenectomy, avascular bone necrosis, osteomyelitis, pulmonary hypertension, and heart failure.

Question 5

What are the most common organisms causing infection after splenectomy?

Answer 5

Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis.

Question 6

What is the most common brain tumor in elderly?

Answer 6

• High-grade: Gliomas and glioblastoma multiforme, Medulloblastoma.
• Low-grade: Meningiomas, Acoustic neuromas, Neurofibroma, Pituitary tumors, Pineal tumors, and Craniopharyngiomas.
• Secondaries.

Question 7

What are the manifestations of brain tumors?

Answer 7

• Headache, which is typically worse in the mornings.
• Nausea and vomiting.
• Seizures.
• Progressive focal neurological deficits, for example, diplopia associated with a cranial nerve defect, visual field defects, neurological deficits affecting the upper and/or lower limb.
• Cognitive or behavioral symptoms.
• Symptoms relating to the location of mass, for example, frontal lobe lesions associated with personality changes, disinhibition, and parietal lobe lesions might be associated with dysarthria.
• Papilledema (absence of papilledema does not exclude a brain tumor).

Question 8

What if the brain tumor is left untreated?

Answer 8

Death

Question 9

What did the biopsy show?

Answer 9

The biopsy showed squamous cells, indicating metastatic SCC.

Question 10

What are the possible primary sources of metastatic SCC?

Answer 10

SCC of skin, lung, esophagus, nasopharynx, and cervix.

Question 11

What common organism caused post-biopsy wound infection?

Answer 11

Staphylococcus aureus.

Question 12

What is the cause of glucose in wound discharge?

Answer 12

CSF communication.