CASE REPORT An unusual complication of ice skating and the emergence of a previously undiagnosed bleeding disorder C. J. MAINWARING,* J. PLEYDELL-PEARCE,  J. CHANA,* J. EVANS,* A. ROY* and H. LEWIS* *Haematology and  Paediatrics, North Hampshire Hospital, Basingstoke, UK Summary. We report the case of an 8-year-old boy with no prior abnormal bleeding history who presented with severe central abdominal pain fol- lowing a freak accident at a local ice rink. Clinical examination confirmed a tender periumbilical mass. An ultrasound scan confirmed a large haemorrhagic fluid collection adjacent to the second part of his duodenum that was causing a subacute small-bowel obstruction. He was found to have a persistently prolonged prothrombin time between 17.3 and 18.1 s but normal liver function tests. There was no suggestion of dietary vitamin K deficiency. Fur- ther investigations confirmed factor VII deficiency with levels between 30.4 and 33.6 IU dL )1 . His prothrombin time did not normalize with intraven- ous vitamin K. He was subsequently treated with three 30 lg kg )1 body weight doses of novoseven at 4-h interval and made an excellent recovery. The haematoma virtually resolved completely confirmed by a follow-up ultrasound scan 3 months after the initial event. Keywords. factor VII deficiency, intestinal haema- toma, novoseven Introduction Factor VII (FVII) deficiency is one of the rare autosomal recessive inherited bleeding disorders. Severe deficiency with FVII levels of <2 IU dL )1 has an incidence of approximately one in 300 000 to one in 500 000. The FVII gene is found on chromosome 13 mapping to 13q34. There are approximately 120 known mutations with missense mutations account- ing for approximately 80% of cases. Five polymor- phisms within the FVII gene are known to affect plasma FVII levels. The most important is Arg353Gln polymorphism within exon 7 that affects 20% of the UK population. Being heterozygous for this is associated with an approximate 25% reduc- tion in plasma FVII levels. It is well known that there is a poor correlation between the FVII activity and the bleeding phenotype. There is generally a predi- lection towards mucosal-type bleeding including easy bruising, gum bleeding, epistaxes and menorrhagia. Not surprisingly, musculoskeletal, neurological and gastrointestinal bleeds tend to occur more often in patients with severe deficiency. To our knowledge, small-bowel haematomas even after trauma have not previously been described in a patient with mild FVII deficiency. Generally, it is thought that a FVII level of at least 20 IU dL )1 is required for effective haemos- tasis in the management of bleeding episodes or surgery. Although not licensed, recombinant factor VIIa (novoseven) is the preferred treatment for the management of bleeds prior to invasive procedures plus operations. Case report A previously fit and healthy 8-year-old boy was admitted as an acute emergency to the paediatric ward with a 4-h history of severe central abdominal pain and vomiting. Approximately, 24 h prior to admission he had been skating at a local ice rink and had fallen on his back and inadvertently pulled his 90-kg father on top of him causing blunt abdominal trauma. His past medical history was unremarkable including an uneventful appendicectomy 6 weeks earlier. He had undergone no other operations or dental extractions in the past and had no symptoms Correspondence: C. J. Mainwaring, North Hampshire Hospital, Aldermaston Road, Basingstoke RG24 9NA, UK. Tel.: +44 1202 442938; fax: +44 1202 448659; e-mail: cjmainwaring@tiscali.co.uk Accepted after revision Haemophilia (2006), 12, 551–554 DOI: 10.1111/j.1365-2516.2006.01309.x Ó 2006 The Authors Journal compilation Ó 2006 Blackwell Publishing Ltd 551