Recurrent intraparenchymal brain hemorrhage in an infant with factor XIII deficiency

Factor XIII deficiency is an autosomal recessive disorder with an incidence of 1 in 1-5 million. On activation factor XIII stabilizes clot formation by cross-linking fibrin strands. Deficiency is characterized by severe bleeding due to impairment in clot formation. We describe a case of a child pres...

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Bibliographic Details
Published inJournal of pediatric and neonatal individualized medicine Vol. 10; no. 2; p. e100201
Main Authors Devdeep Mukherjee, Dibyendu De, Satyajit Das, Gopikrishna Kurasa
Format Journal Article
LanguageEnglish
Published Hygeia Press di Corridori Marinella 01.05.2021
Subjects
acute on chronic bleeding
clot solubility test
cryoprecipitate
factor xiii deficiency
intraparenchymal bleeding
neurosurgery
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Summary:Factor XIII deficiency is an autosomal recessive disorder with an incidence of 1 in 1-5 million. On activation factor XIII stabilizes clot formation by cross-linking fibrin strands. Deficiency is characterized by severe bleeding due to impairment in clot formation. We describe a case of a child presenting with drowsiness following a trivial fall and trauma to the head. An emergency CT scan of the brain was suggestive of acute on chronic (recurrent) hemorrhage. Intraoperatively he was noted to have an intraparenchymal multiloculated cystic cavity with a thinned out cortex in the right frontal region. The child underwent right frontal craniotomy and decompression of an intracerebral hemorrhage. He was subsequently diagnosed to have factor XIII deficiency. He is presently on monthly cryoprecipitate prophylaxis with which he has not experienced a recurrence of a similar episode.
ISSN:2281-0692
DOI:10.7363/100201