title: Recurrent spontaneous splenic rupture in a patient with congenital factor XIII deficiency.
abstract: We describe an unusual presentation of factor XIII (FXIII) deficiency in a 17-year-old boy who was diagnosed with this congenital deficiency at the age of 18 months. He had a history of spontaneous splenic rupture 8 years ago, which was managed conservatively. He now presented with sudden severe abdominal and left shoulder pain for 1 day, with no history of antecedent trauma. He was in shock, and examination revealed diffuse peritonitis. A computed tomography scan showed a grade IV splenic laceration. He was taken as an emergency to the operating room where he was found to have a shattered spleen, and a splenectomy was performed. He received cryoprecipitate transfusions perioperatively. After an uneventful recovery, the patient was discharged. To the best of our knowledge, this is the first described case of a recurrent splenic rupture in a patient with FXIII deficiency.
authors.affiliation: Departments of *Surgery †Radiology ‡Oncology, The Aga Khan University, Karachi, Pakistan.
authors.forename: Amir H
authors.initials: AH
authors.lastname: Shariff
authors.forename: Muhammad
authors.initials: M
authors.lastname: Waqas
authors.forename: Basit
authors.initials: B
authors.lastname: Salam
authors.forename: Muhammad
authors.initials: M
authors.lastname: Arshad
authors.forename: Salman N
authors.initials: SN
authors.lastname: Adil
mesh_terms: D015746:Abdominal Pain / Q000097:blood / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D005177:Factor XIII Deficiency / Q000097:blood* / Q000151:congenital / Q000473:pathology*; D006801:Humans; D008297:Male; D010538:Peritonitis / Q000097:blood / Q000473:pathology / Q000601:surgery; D012008:Recurrence; D012422:Rupture, Spontaneous; D013156:Splenectomy; D013158:Splenic Diseases / Q000097:blood* / Q000473:pathology* / Q000601:surgery