Question of the Week

Question :
Posted On : 27 Mar 2018
a 5 year old girl, previously healthy, has been running fever for last 3 weeks. she was brought to emergency with progressively increasing respiratory distress. on exam, she is pale, with swelling of face, neck and arms. x ray chest shows normal heart size with wide mediastinum.
Expert Answer :
History seems incomplete. However, this girl probably has superior vena cava syndrome. Malignancy is most common cause for SVC syndrome in children. Anemia suggests some degree of bone marrow involvement.
Most likely she has a non-hodgkin lymphoma with SVC syndrome. At this age it is also not uncommon to have superior mediastinal syndrome {compression of trachea} as trachea is pliable in this age.
This IS a medical emergency. Things to do on urgent basis
1} Manage with supplemental oxygen
2} Do not let her lie down completely, even for CT scan. This can be life threatening for her. Situation is dynamic and can change on minute to minute or hourly basis.
3} If not comfortable or facilities not availbale, refer to higher center.
4} Packed red cell {whole blood if Packed red cells not available} transfusion to increase oxygen carriage.
5} Accurate fluid balance {intake, output}
6} Try doing lymph node biopsy. I presume she must have other lymph nodes.
7} If condition deteriorating fast, then give steroids empirically. Downside is, it is likely to hamper correct diagnosis but I will say saving life is a priority given the clinical description and dynamic situation. Radiotherapy is another alternative but generally steroids work very well and radiotherapy is rarely required.
8} Manage according to diagnosis

I do not have all details to suggest more than this at this time point.
All the best.
Answer Discussion :
SVS wth mediastinal mass, Lymphoma
3 years ago
Evelin Dafni
Lymphoma leading to superior venacaval syndrome
3 years ago
M Yousif
acute haemolytic anemia
3 years ago
rana sleiman
SVC sd,R/O Lymphoma
3 years ago
Anoop K
Superior venacaval syndrome due to mediastinal mass probably lymphoma
3 years ago

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