Spontaneous chronic subdural hematomas in young adults with a deficiency of coagulation factor XIII

Report of three cases

Restricted access

✓ Chronic subdural hematomas (SDHs) generally occur in elderly patients. Its pathogenesis is usually related to head trauma with tearing and rupture of the bridging veins, although in some cases a history of trauma is not recognizable. There are many reports regarding the association between spontaneous chronic SDHs and an alteration in coagulative parameters. A coagulative disorder should be suspected when an unexplained hemorrhage occurs, especially in a young patient. The authors report on three young men with a deficiency in coagulation factor XIII (FXIII) who underwent surgery for chronic SDHs. The role of FXIII in the pathogenesis of chronic SDH is emphasized. In patients with unexplained chronic SDH all coagulation parameters and factors should be screened to identify an eventual coagulative disorder.

Article Information

Address reprint requests to: Giovanni Sabatino, M.D., Department of Neurosurgery, Catholic University, Largo Gemelli 1, 00168 Rome, Italy. email: giosaba@inwind.it.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Case 1. A: An axial T2-weighted magnetic resonance image preoperatively obtained in a 30-year-old man with a 1-month history of progressive frontal headache, revealing hyperintense, bilateral, subdural fluid collections typical of chronic SDH. Case 2. B: A preoperative CT scan obtained in a 35-year-old man with a 1-week history of headache and episodic paresthesias in the upper-right extremity, demonstrating bilateral, hemispheric chronic SDH (typically hypodense in the superior portion together with hyperdensity in the lower portion) together with brain compression. The cortical sulci are not visible. Case 3. C: A preoperative CT scan obtained in a 27-year-old man with a 1-month history of severe headache and progressive motor deficit in the left side, exhibiting a right-hemisphere chronic SDH (typically hypodense) with compression of the ventricle and midline shift toward the left side.

References

  • 1.

    Abbondanzo SLGootenberg JELofts RSMcPherson RA: Intracranial hemorrhage in congenital deficiency of factor XIII. Am J Pediatr Hematol Oncol 10:65681988Abbondanzo SL Gootenberg JE Lofts RS McPherson RA: Intracranial hemorrhage in congenital deficiency of factor XIII. Am J Pediatr Hematol Oncol 10:65–68 1988

  • 2.

    Anwar RMiloszewski KJ: Factor XIII deficiency. Br J Haematol 107:4684841999Anwar R Miloszewski KJ: Factor XIII deficiency. Br J Haematol 107:468–484 1999

  • 3.

    Board PGLosowsky MSMiloszewski KJ: Factor XIII: inherited and acquired deficiency. Blood Rev 7:2292421993Board PG Losowsky MS Miloszewski KJ: Factor XIII: inherited and acquired deficiency. Blood Rev 7:229–242 1993

  • 4.

    Bonnaud ISaudeau Dde Toffol BAutret A: Recurrence of spontaneous subdural haematoma revealing acquired haemophilia. Eur Neurol 49:2532542003Bonnaud I Saudeau D de Toffol B Autret A: Recurrence of spontaneous subdural haematoma revealing acquired haemophilia. Eur Neurol 49:253–254 2003

  • 5.

    Chen JCLevy ML: Causes, epidemiology, and risk factors of chronic subdural hematoma. Neurosurg Clin N Am 11:3994062000Chen JC Levy ML: Causes epidemiology and risk factors of chronic subdural hematoma. Neurosurg Clin N Am 11:399–406 2000

  • 6.

    De Almeida GMLaumer RFahlbusch RSambasivan MFukui MWissinger JP: Subdural hematoma. Surg Neurol 47:251997De Almeida GM Laumer R Fahlbusch R Sambasivan M Fukui M Wissinger JP: Subdural hematoma. Surg Neurol 47:2–5 1997

  • 7.

    Egbring RKroniger ASeitz R: Factor XIII deficiency: pathogenic mechanisms and clinical significance. Semin Thromb Hemost 22:4194251996Egbring R Kroniger A Seitz R: Factor XIII deficiency: pathogenic mechanisms and clinical significance. Semin Thromb Hemost 22:419–425 1996

  • 8.

    Gerlach RRaabe AZimmermann MSiegemund ASeifert V: Factor XIII deficiency and postoperative hemorrhage after neurosurgical procedures. Surg Neurol 54:2602652000Gerlach R Raabe A Zimmermann M Siegemund A Seifert V: Factor XIII deficiency and postoperative hemorrhage after neurosurgical procedures. Surg Neurol 54:260–265 2000

  • 9

    Gootenberg JE: Factor concentrates for the treatment of factor XIII deficiency. Curr Opin Hematol 5:3723751998Gootenberg JE: Factor concentrates for the treatment of factor XIII deficiency. Curr Opin Hematol 5:372–375 1998

  • 10.

    Ichinose A: Physiopathology and regulation of factor XIII. Thromb Haemost 86:57652001Ichinose A: Physiopathology and regulation of factor XIII. Thromb Haemost 86:57–65 2001

  • 11.

    Jansen JWHaverkate FKoopman JNieuwenhuis HKKluft CBoschman TA: Influence of factor XIIIa activity on human whole blood clot lysis in vitro. Thromb Haemost 7:1711751987Jansen JW Haverkate F Koopman J Nieuwenhuis HK Kluft C Boschman TA: Influence of factor XIIIa activity on human whole blood clot lysis in vitro. Thromb Haemost 7:171–175 1987

  • 12.

    Konig SASchick UDohnert JGoldammer AVitzhum HE: Coagulopathy and outcome in patients with chronic subdural haematoma. Acta Neurol Scand 107:1101162003Konig SA Schick U Dohnert J Goldammer A Vitzhum HE: Coagulopathy and outcome in patients with chronic subdural haematoma. Acta Neurol Scand 107:110–116 2003

  • 13.

    Kudo HKuwamura KIzawa ISawa HTamaki N: Chronic subdural hematoma in elderly people: present status on Awaji Island and epidemiological prospect. Neurol Med Chir 32:2072091992Kudo H Kuwamura K Izawa I Sawa H Tamaki N: Chronic subdural hematoma in elderly people: present status on Awaji Island and epidemiological prospect. Neurol Med Chir 32:207–209 1992

  • 14.

    Larsen PDWallace JWFrankel LSCrisp D: Factor XIII deficiency and intracranial hemorrhages in infancy. Pediatr Neurol 6:2772781990Larsen PD Wallace JW Frankel LS Crisp D: Factor XIII deficiency and intracranial hemorrhages in infancy. Pediatr Neurol 6:277–278 1990

  • 15.

    Muszbek LAdany RMikkola H: Novel aspects of blood coagulation factor XIII. Structure, distribution, activation and function. Crit Rev Clin Lab Sci 33:3574211996Muszbek L Adany R Mikkola H: Novel aspects of blood coagulation factor XIII. Structure distribution activation and function. Crit Rev Clin Lab Sci 33:357–421 1996

  • 16.

    Pavithran KThomas M: Spontaneous resolution of subdural hematoma in haemophilia. Haematologica 87: ELT032002Pavithran K Thomas M: Spontaneous resolution of subdural hematoma in haemophilia. Haematologica 87: ELT03 2002

  • 17.

    Stroobandt GFrasen PThauvoy CMenard E: Pathogenetic factors in chronic subdural hematoma and causes of recurrence after drainage. Acta Neurochir 137:6141995Stroobandt G Frasen P Thauvoy C Menard E: Pathogenetic factors in chronic subdural hematoma and causes of recurrence after drainage. Acta Neurochir 137:6–14 1995

  • 18.

    Ugur SUzel NDundar KKaya UAyan IGedikoglu G: Intraspinal hemorrhage in a child with factor XIII deficiency. Pediatr Emerg Care 7:2312331991Ugur S Uzel N Dundar K Kaya U Ayan I Gedikoglu G: Intraspinal hemorrhage in a child with factor XIII deficiency. Pediatr Emerg Care 7:231–233 1991

  • 19.

    Yamashima TFriede RL: Why do bridging veins rupture into the virtual subdural space? J Neurol Neurosurg Psychiatry 47:1211271984Yamashima T Friede RL: Why do bridging veins rupture into the virtual subdural space? J Neurol Neurosurg Psychiatry 47:121–127 1984

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 132 132 13
Full Text Views 143 143 2
PDF Downloads 77 77 1
EPUB Downloads 0 0 0

PubMed

Google Scholar