CASE REPORT Blindness following soft tissue trauma in a child with haemophilia A B.R. Chrcanovic 1 & L.N. Souza 2,3 1 Oral and Maxillofacial Surgery, Belo Horizonte, Minas Gerais, Brazil 2 Emergency Unit, Hospital João XXIII, Belo Horizonte, Minas Gerais, Brazil 3 Centro Universitário Newton Paiva, Belo Horizonte, Minas Gerais, Brazil Abstract Traumatic retrobulbar haemorrhage, if not promptly recognised and treated, may result in acute vision loss because of compression of the optic nerve and its vascular supply. Bleeding disorders or anticoagulant therapy may contribute to the rapid onset of this phenomenon, thus making urgent diagnosis and treatment even more critical. This article reports a case of retrobulbar haemorrhage in an 8-year-old boy with factor VIII deficiency (haemophilia A or classic haemophilia) who delayed in seeking professional help and consequently developed permanent blindness. Minor injuries in patients with bleeding disorders or anticoagulant therapy were proven to result in severe complications, especially when these affect the eye. Key words: blindness, haemophilia A, optic nerve injury, orbital trauma, retrobulbar haemorrhage Correspondence to: Bruno Ramos Chrcanovic Division of Maxillofacial Surgery Emergency Unit Hospital João XXIII Av. Raja Gabaglia, 1000/1209 Gutierrez Belo Horizonte Minas Gerais 30380-090 Brazil Tel.: +55 31 91625090 Fax: +55 31 32920997 email: brunochrcanovic@hotmail.com Accepted: 18 September 2009 doi:10.1111/j.1752-248X.2009.01051.x Clinical relevance The early diagnosis and prompt treatment following soft tissue trauma in the eye may prevent amaurosis or even restore vision. For this reason, the emergency medical staff should be aware of the signs and symp- toms of the onset of retrobulbar haemorrhage in ‘at-risk’ inpatients, and a subsequent computed tomography scan assessment should be performed in any suspected case. Introduction Bleeding inside the orbit can produce a rapidly evolving retrobulbar haemorrhage, a rare but potentially sight- threatening complication of injury or operation near the orbit, which, if left untreated, almost inevitably leads to blindness 1–4 . Therefore, early detection and immediate treatment are desirable. Immediate decom- pression should be performed through surgery 3–5 . However, pharmacological management may pro- duce a satisfactory outcome when used as the sole treatment 2 . Haemophilia A is the most severe of inherited bleed- ing disorders, as it results from factor VIII deficiency and is transmitted in an X-linked recessive manner 6 . However, a significant number of cases (approximately 30%) also arise as a result of spontaneous mutation 7 . Factor replacement therapy has contributed much to the management of acute bleeding episodes and the prevention of long-term sequelae 6 . The modern approach consists of the intravenous administration of a concentrate factor in a prophylactic form, two or three times a week to prevent spontaneous bleeding 8 . The following report concerns a child with haemo- philia A, who suffered a minor trauma directly to one eye, causing a total loss of vision because of his delay in seeking professional assistance. Oral Surgery ISSN 1752-2471 149 Oral Surgery 2 (2009) 149–152. © 2010 The Authors Journal compilation © 2010 Blackwell Munksgaard