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Abstract
Subgaleal hematomas are usually seen after high-velocity head trauma; however, their presence following trivial injury should raise suspicion regarding an associated clotting disorder. Here we describe an interesting patient who presented to us with bilateral proptosis associated with a subgaleal hematoma due to fibrinogen and factor X deficiency. This condition can result in serious bleeding diathesis often necessitating multiple surgeries to control haemorrhage.
Our patient, an 11-year-old male child presented to us 4 weeks after a minor head injury. He had a gradually progressive soft swelling on his forehead with proptosis of both eyes. Aspiration of the swelling at a nearby private hospital had revealed blood. The swelling recurred over a span of 2 weeks.
On neurological examination, there were no positive findings. On local examination, he had a large, non-tender subgaleal hematoma over the bilateral fronto-temporo-parieto-occipital region of his head extending to both eyes. There was associated bilateral subconjunctival haemorrhage, periorbital ecchymosis and gross proptosis. The vision was affected on the right side, finger counting at 1 foot. Left side vision was intact. Extraocular movements were restricted in both eyes in all planes. CT scan of the brain showed subgaleal hematoma over the bilateral fronto-temporo-parieto-occipital region with an extension over the supraorbital ridge into the bilateral orbit beneath the orbital roof, there were bilateral relatively symmetrical areas of subperiosteal haemorrhages involving superior aspect of orbit causing proptosis and inferior displacement of the globes. On haematological examination, his haemoglobin was 5 gm/dl. Platelet count was 240,000/cumm. On coagulation profile, prothrombin time was 45 seconds (normal range 11.1–14.5 seconds) with an activated partial thromboplastin time of 39.5 seconds (normal range 28.1–39.7 seconds), and thrombin time was 58 seconds. The plasma fibrinogen levels were < 100 mg/dl (normal range- 200-400 mg/dl). D-dimer was strongly positive. The patient also had factor X deficiency. The peripheral blood smear had a normocytic normochromic picture.
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References
- Kakizaki H, Selva D, Asamoto K, et al Orbital septum attachment sites on the levator aponeurosis in Asians and whites. Ophthal Plast Reconstr Surg 2010;26(4):265-8.
- Lee KS, Bae HG, Yun IG. Bilateral proptosis from a subgaleal hematoma. Case report. J Neurosurg 1988;69(5):770-1.
- Gioia VM, Linberg JV, McCormick SA. The anatomy of the lateral canthal tendon. Arch Ophthalmol 1987;105(4):529-32.
- Kim D, Taragin B. Subgaleal hematoma presenting as a manifestation of Factor XIII deficiency. Pediatr Radiol 2009;39:622-4.
- Chotirmall SH, Pearson E, Saad AZ, et al. Posttraumatic subgaleal hematoma with orbital extension associated with clopidogrel usage in an elderly patient: case report. J Am Geriatr Soc 2007;55(1):135-6.
- Pomeranz AJ, Ruttum MS, Harris GJ. Subgaleal hematoma with delayed proptosis and corneal ulceration. Ann Emerg Med 1995;26(6):752-4.
- Procianoy F, Filho MB, Cruz AAVE, et al. Subperiosteal hematoma and orbital compression syndrome following minor frontal trauma in sickle cell anemia: case report. Arq Bras Oftalmol 2008;71(2):262-4.
- Antón J, Pineda V, Martin C, et al. Posttraumatic subgaleal hematoma: a case report and review of the literature. Pediatr Emerg Care 1999;15(5):347-9.
- Hayes T. Dysfibrinogenemia and thrombosis. Arch Pathol Lab Med 2002;126(11):1387-90.
- Kotlin R, Reicheltova Z, Maly M et al. Two cases of congenital dysfibrinogenemia associated with thrombosis-Fibrinogen Praha III and Fibrinogen Plzen. Thromb Haemost 2009;102(3):479-86.
- Asselta R, Duga S, Tenchini ML. The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost 2006;4(10):2115-29.
- Powell JS, Rodgers GM. Inherited coagulation disorders. In: Greer JP, Arber DA, Glader B, et al, eds. Wintrobe’s clinical hematology. 13th edn. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins 2013:1122-17.
- Francis JL, Armstrong DJ. Acquired dysfibrinogenaemia in liver disease. J Clin Pathol 1982;35(6):667-2.
- Gralnick HR, Givelber H, Abrams E. Dysfibrinogenemia associated with hepatoma. Increased carbohydrate content of the fibrinogen molecule. N Engl J Med 1978;299(5):221-6.
- Levy J, Pettei MJ, Weitz JI. Dysfibrinogenemia in obstructive liver disease. J Pediatr Gastroenterol Nutr 1987;6(6):967-70.
- Dear A, Brennan SO, Sheat MJ, et al. Acquired dysfibrinogenemia caused by monoclonal production of immunoglobulin lambda light chain. Haematologica 2007;92(11):e111-7.
- O’Kane MJ, Wisdom GB, Desai ZR, et al. Inhibition of fibrin monomer polymerisation by myeloma immunoglobulin. J Clin Pathol 1994;47(3):266-8.
- Casini A, Blondon M, Lebreton A, et al. Natural history of patients with congenital dysfibrinogenemia. Blood 2015;125(3):553-61.
- Rohyans JA, Miser AW, Miser JS. Subgaleal hemorrhage in infants with hemophilia: report of two cases and review of the literature. Pediatrics 1982;70(2):306-7.
- Menegatti M, Peyvandi F. Factor X deficiency. Semin Thromb Hemost 2009;35(4):407-15.
References
Kakizaki H, Selva D, Asamoto K, et al Orbital septum attachment sites on the levator aponeurosis in Asians and whites. Ophthal Plast Reconstr Surg 2010;26(4):265-8.
Lee KS, Bae HG, Yun IG. Bilateral proptosis from a subgaleal hematoma. Case report. J Neurosurg 1988;69(5):770-1.
Gioia VM, Linberg JV, McCormick SA. The anatomy of the lateral canthal tendon. Arch Ophthalmol 1987;105(4):529-32.
Kim D, Taragin B. Subgaleal hematoma presenting as a manifestation of Factor XIII deficiency. Pediatr Radiol 2009;39:622-4.
Chotirmall SH, Pearson E, Saad AZ, et al. Posttraumatic subgaleal hematoma with orbital extension associated with clopidogrel usage in an elderly patient: case report. J Am Geriatr Soc 2007;55(1):135-6.
Pomeranz AJ, Ruttum MS, Harris GJ. Subgaleal hematoma with delayed proptosis and corneal ulceration. Ann Emerg Med 1995;26(6):752-4.
Procianoy F, Filho MB, Cruz AAVE, et al. Subperiosteal hematoma and orbital compression syndrome following minor frontal trauma in sickle cell anemia: case report. Arq Bras Oftalmol 2008;71(2):262-4.
Antón J, Pineda V, Martin C, et al. Posttraumatic subgaleal hematoma: a case report and review of the literature. Pediatr Emerg Care 1999;15(5):347-9.
Hayes T. Dysfibrinogenemia and thrombosis. Arch Pathol Lab Med 2002;126(11):1387-90.
Kotlin R, Reicheltova Z, Maly M et al. Two cases of congenital dysfibrinogenemia associated with thrombosis-Fibrinogen Praha III and Fibrinogen Plzen. Thromb Haemost 2009;102(3):479-86.
Asselta R, Duga S, Tenchini ML. The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost 2006;4(10):2115-29.
Powell JS, Rodgers GM. Inherited coagulation disorders. In: Greer JP, Arber DA, Glader B, et al, eds. Wintrobe’s clinical hematology. 13th edn. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins 2013:1122-17.
Francis JL, Armstrong DJ. Acquired dysfibrinogenaemia in liver disease. J Clin Pathol 1982;35(6):667-2.
Gralnick HR, Givelber H, Abrams E. Dysfibrinogenemia associated with hepatoma. Increased carbohydrate content of the fibrinogen molecule. N Engl J Med 1978;299(5):221-6.
Levy J, Pettei MJ, Weitz JI. Dysfibrinogenemia in obstructive liver disease. J Pediatr Gastroenterol Nutr 1987;6(6):967-70.
Dear A, Brennan SO, Sheat MJ, et al. Acquired dysfibrinogenemia caused by monoclonal production of immunoglobulin lambda light chain. Haematologica 2007;92(11):e111-7.
O’Kane MJ, Wisdom GB, Desai ZR, et al. Inhibition of fibrin monomer polymerisation by myeloma immunoglobulin. J Clin Pathol 1994;47(3):266-8.
Casini A, Blondon M, Lebreton A, et al. Natural history of patients with congenital dysfibrinogenemia. Blood 2015;125(3):553-61.
Rohyans JA, Miser AW, Miser JS. Subgaleal hemorrhage in infants with hemophilia: report of two cases and review of the literature. Pediatrics 1982;70(2):306-7.
Menegatti M, Peyvandi F. Factor X deficiency. Semin Thromb Hemost 2009;35(4):407-15.