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Review
. 2021 Aug 17;14(8):e241613.
doi: 10.1136/bcr-2021-241613.

Spontaneous pyohaemothorax in a teenager with von Willebrand disease: a case report and review of literature

Vaishnavi Divya Nagarajan  1   2 , Asha Shenoi  3 , Lucy Burgess  2 , Vlad C Radulescu  4
Affiliations
Review

Spontaneous pyohaemothorax in a teenager with von Willebrand disease: a case report and review of literature

Vaishnavi Divya Nagarajan et al. BMJ Case Rep. .

Abstract

An 18-year-old man with a history of type 3 von Willebrand disease (VWD) presented with a spontaneous pyohaemothorax. Type 3 VWD may present with both mucocutaneous and deep-seated bleeds, such as visceral haemorrhages, intracranial bleeds and haemarthrosis. There have been very few cases described in children of spontaneous pyohaemothorax. Management of this patient was challenging due to risks of bleeding following surgical drainage, requiring constant replacement with von Willebrand factor concentrate, while monitoring factor VIII levels to balance the risks of thrombosis.

Keywords: haematology (incl blood transfusion); paediatrics (drugs and medicines).

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Complete opacification of the left hemithorax with right-sided shift of the mediastinum seen in chest X-ray.
Figure 2
Figure 2
CT image showing a very large left pleural fluid collection with Hounsfield units above that of simple fluid and in the setting of coagulopathy likely representing a large haemothorax. This is displacing the heart and other mediastinal structures significantly to the right.
Figure 3
Figure 3
Graph showing levels of measured factor VIII and VWF levels in relation to dosing of Humate-P. VWF, von Willebrand factor.
See this image and copyright information in PMC

References

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