Intramural Hematoma of the Small Intestine in a Patient with Severe Hemophilia A. Case Report and Review of the Literature

Prieto, Héctor Marcos; Pérez, Callejas; Bermejo, Javier

doi:10.4172/2161-069x.1000363

Cited by 1 publication

(7 citation statements)

References 33 publications

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“…Intramural hematoma of the gastrointestinal (GI) tract is a rare condition in PWH, despite their bleeding tendency. Bleeding into the walls of the esophagus, stomach, small intestine, or colon has been reported [4,6,26,27]. A hematoma located in the bowel wall can lead to a sudden onset of acute abdominal pain, and patients may present with signs of GI obstruction [3,20].…”

Section: Discussionmentioning

confidence: 99%

“…The most common pathological mechanism of intramural hematoma should be the tearing or rupture of terminal arterial vessels leaving the mesentery to penetrate the muscularis of the intestinal wall [4,7,33]. As the hematoma forms, an osmotic gradient within the wall gradually increases, leading to the gradual separation of the muscularis mucosa from the muscle layer [4,7,34]. This may, in turn, result in paralytic ileus and narrowing or obstruction of the GI lumen [35].…”

Section: Pathological Mechanismmentioning

confidence: 99%

“…Abdominal ultrasonography is effective in evaluating the presence of free air in the abdominal cavity. Nevertheless, abdominal CT is the definitive diagnostic modality [4,7,20]. It can not only identify the site and extent of hematoma but also reveal potential complications, such as intussusception, local abscess, or perforation [7,20,37].…”

Section: Image Findingsmentioning

confidence: 99%

“…Gastrointestinal (GI) bleeding, characterized by bleeding into the lumen of the GI tract, is a frequent complication among people with hemophilia (PWH), with a reported incidence ranging from 10% to 25% [4,5]. However, PWH rarely experience bleeding into the submucosal layer of the GI tract, which is known as intramural hematoma.…”

Section: Introductionmentioning

confidence: 99%

“…However, PWH rarely experience bleeding into the submucosal layer of the GI tract, which is known as intramural hematoma. Furthermore, recurrent cases of intramural hematoma in PWH are even more infrequent [3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Intramural Hematoma of Gastrointestinal Tract in People with Hemophilia A and B

Teng

Kung

Cheng

et al. 2023

JCM

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People with hemophilia (PWH), especially severe hemophilia, often experience bleeding episodes, which occur mostly at major joints. Intramural hematoma of the gastrointestinal (GI) tract is a rare, potentially life-threatening clinical bleeding manifestation in PWH. Prompt identification and timely administration of clotting factor concentrates are of utmost importance for effective management and optimal patient outcomes. In this report, we present the case of a 48-year-old male with severe hemophilia A. The patient developed a spontaneous intramural hematoma of the jejunum, leading to signs of acute abdomen, bloody stool, and paralytic ileus. Conservative management with factor VIII (FVIII) infusion was successfully administered. However, within a span of three months, the patient suffered from a recurrent episode of intramural hematoma, which was again effectively treated with conservative therapy. Subsequently, prophylactic FVIII therapy was administered to the patient, resulting in the absence of recurrence for over three years. Inspired by this case, we conducted a comprehensive review of the relevant literature and gathered data from 79 reported cases of intramural hematoma that were documented between the years 1956 and 2022. We classified these cases based on the site affected within the gastrointestinal (GI) tract (spread across five different locations) and proceeded to conduct a simple pooling analysis on the data collected, which subsequently revealed that the overall mortality rate of intramural hematoma in people with hemophilia (PWH) was found to be 12.2%, while children have a higher mortality rate (23.3%) than adults (4.9%). We hope this case report and literature review increase awareness of this rare bleeding manifestation in PWH, the effectiveness of conservative treatment, and the possibility of prophylaxis against recurrence.

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