[5] Discussion
FXIII (also known as fibrin stabilizing factor) deficiency is one of the
rarest factor deficiencies, occurring in approximately 1 in every 2
million people worldwide. Its function includes stabilizing blood clot
formation, aiding in tissue repair, depositing extracellular matrix, and
contributing to the differentiation of osteoblasts [2]. The
deficiency typically presents as excessive and prolonged bleeding from
the umbilical stump, poor wound healing, and can lead to complications
such as intracranial hemorrhage, either spontaneously or
post-traumatically [10]. Additionally, superficial and deep
hematomas are associated with the deficiency, although they are more
commonly observed in older age groups [11].
Diagnosing FXIII deficiency can be challenging, as standard clotting
tests such as PT, aPTT, and INR are usually normal. Key laboratory
investigations for diagnosis include the clot solubility test, FXIII
activity assay, FXIII antigen assay, inhibitor assay, and molecular
diagnostics [2]. This case is unique because we believe it is the
first reported instance of a duodenal hematoma due to FXIII deficiency.
Duodenal hematomas are typically caused by crushing blunt force that
ruptures intramural blood vessels or as a complication of anticoagulant
therapy, endoscopic biopsy, vasculitis, pancreatitis, tumors, or
bleeding disorders [6-9].
Early diagnosis of FXIII deficiency allows for conservative treatment,
avoiding surgery [6, 8, 9, 12, 13]. Treatment options for FXIII
deficiency include fresh frozen plasma (FFP) or cryoprecipitate.
However, with advancements, FXIII concentrate and recombinant FXIII
(rFXIII) are available for severe deficiencies to reduce bleeding
events.
For duodenal hematomas, treatment can be conservative or invasive.
Conservative management involves keeping the patient NPO for bowel rest
and nasogastric (NG) decompression, placing a peripherally inserted
central catheter (PICC), and initiating parenteral nutrition
[14-17]. The duration of conservative management varies, and enteral
nutrition can begin once gastric aspirates are no longer bilious.
Surgical treatment options include hematoma removal or bypass surgery
[18]. In the past, when radiological advancements were not
available, an exploratory laparotomy was performed after blunt abdominal
trauma for evacuation, but this is now an option primarily in cases of
jaundice and delayed bowel obstruction [19]. Invasive surgical
procedures may still be necessary for complications such as uncontrolled
bleeding or panperitonitis [20]. Recent advances include endoscopic
drainage with fistula formation or mucosal puncture, which has shown
success without major complications [21]. In cases of intramural
duodenal hematomas (IMDH), percutaneous catheter insertion can be used
for decompression [22].