Intramural Haemorrhage and Haematoma as the Cause of Ileus of the Small Intestine in a Haemophiliac.

INTRODUCTION: The most frequent sites of bleeding in patients with haemophilia are the soft tissues, the joints, the urinary tract, but much more rarely the gastrointestinal tract. The complications of intramural bleeding are acute intestinal obstruction, but also rupture of the haematoma in the lumen or the peritoneal space. CASE REPORT: We present the case of a haemophiliac patient who was admitted as an emergency due to distended abdomen, nausea, vomiting and the clinical picture of ileus. The native abdomen in a standing position presented air fluid levels with moderate distension of the accompanying bowel loops. A nasal probe was inserted and the symptoms of ileus disappeared, but after taking food by mouth, the picture of ileus returned. CT of the abdomen and pelvis was performed, which showed circular, high density thickening of the walls in places in the area of the jejunum, indicating haemorrhage, but also the formation of haematoma in the wall structure. After administering factor VIII, the symptoms of ileus ceased, and the patient recovered completely.
CONCLUSION: This unusual presentation of haemophilia with bleeding in the wall of the small intestine is very rare and has only been seen in a few cases in the world. CT diagnosis defined the cause of the obstruction and saved the patient from an unnecessary surgical procedure.

1. INTRODUCTION

Haemophilia is a congenital blood ailment characterized by the lack of coagulating factor VIII (haemophilia A) or factor IV (haemophilia B).

Severe forms of haemophilia may present with bleeding. The most frequent sites of bleeding are the soft tissue, the joints, the urinary tract, and much more rarely the gastrointestinal tract (1). Gastrointestinal haemorrhage has an incidence of 17.5–25% in haemophiliacs and causes death in 4% of this population overall (2).

The complications of intramural bleeding are acute intestinal obstruction, but also the rupture of the haematoma in the lumen or in the peritoneal space (3). This case presents a patient with haemophiliac ileus of the small intestine, where CT diagnostics defined the cause of the obstruction and saved the patient unnecessary surgery.

2. CASE PRESENTATION

The patient was admitted in the evening as an emergency case, due to a distended abdomen, nausea, vomiting and the clinical picture of ileus. The native abdomen in a standing position presented air fluid levels with moderate distension of the relevant bowel loops (Figure 1).

Figure 1

Air fluid levels with moderate distension of the relevant bowel loops.

A nasal probe was inserted and the symptoms of ileus disappeared, but after taking food by mouth, the picture of ileus returned.

Laboratory test results: WBC 11/00, RBC 6.35, HCT 0.503, HGB 166. CRP 68.60.

a-PTT 84.8 (RV:26-28 s), Antihaemophilic factor A (AHG-A), F-VIII < 0.05 (RV:0.7-1.5 j).

CT of the abdomen and pelvis was performed, which showed circular high density thickening of the walls in places in the area of the jejunum, indicating haemorrhage, but also the formation of haematoma in the wall structure. The presence in places of air fluid levels suggested disturbed passage through the bowels (Figures 2 and 3).

Figures 2 and 3

In the area of the jejunum circular high density thickening of the walls in places, indicating haemorrhage, but also the formation of haematoma in the wall structure.

After administering factor VIII the symptoms of ileus disappeared, and the patient gradually recovered completely.

3. DISCUSSION

Bleeding in the gastrointestinal tract in haemophiliacs is not rare, and is usually manifest either in melena or per rectum. Bleeding less often occurs in the intestinal wall, with the symptoms of intestinal obstruction. In this case, intramural bleeding had occurred in the small intestine (Figure 2) and the intramural haematoma was the cause of paralytic ileus and intestinal obstruction.

The most probable physiopathology of intramural haematomas of the bowel is characterized by the spread of the terminal arterial vessels as they leave the mesentery and penetrate the muscular layer of the intestinal wall (3).

An intramural haematoma may also cause intussusception, as another form of ileus (4). As bleeding is the most frequent cause of acute abdominal pain in haemophiliacs, an acute abdomen should be deemed to be bleeding, until proven otherwise by diagnostic methods (CT).

The diagnostic imaging technique of choice is computed tomography (5). It is a very useful method in diagnosing intramural haematoma, defining the area of the haemorrhage and revealing possible complications. Most intramural haematomas can be treated conservatively and spontaneous resolution occurs, where bleeding is treated by prompt compensation of factor VIII. CT helps to differentiate a surgical cause of acute abdomen from bleeding. Correct diagnosis and a multi-discipline approach are imperative in order to avoid unnecessary explorative procedures.