Intramural duodenal haematoma with mucosal prolapse causing intestinal obstruction

Abstract

Intramural small bowel haematoma is a rare cause of intestinal obstruction in children. Coagulation disorders or anticoagulant therapy are the most common cause followed by blunt trauma. A one and half-year-old boy, with known case of gastro-oesophageal reflux disease had undergone upper gastrointestinal endoscopy and biopsy. Four days later, he presented to us with clinical features of small bowel obstruction. CT of the abdomen showed features suggestive of haemorrhagic duplication cyst. Explorative laparotomy revealed 15–20 cm of bowel from the third part of the duodenum to proximal jejunum filled with clotted blood and thinned out serosa. There was no evidence of intraluminal blood in the distal bowel loops. Resection of the involved bowel with primary anastomoses was done. Histopathological examination of bowel revealed intramural haematoma associated with prolapse of the mucosa. There was no evidence of duplication cyst or vascular malformations. Postoperative period was uneventful.

Background

Intramural small bowel haematoma is a rare occurrence in childhood. Blunt trauma to the abdomen is the most common cause in children. Spontaneous small bowel haematomas have been reported in children with coagulation disorders or those on anticoagulant therapy.¹ Iatrogenic causes like upper gastrointestinal (GI) endoscopy and biopsy have also been described in children with coagulopathy.² The presentation may vary from mild, vague abdominal pain to intestinal obstruction. Often the diagnosis is established after imaging or surgical exploration. Imaging features may mimic other cystic masses of the abdomen. Herein we present a rare case of a duodenojejunal intramural haematoma leading to mucosal prolapse and small bowel obstruction in a child with no underlying bleeding diathesis.

Case presentation

A 1½ -year-old boy had non-bilious vomiting since birth and was being treated for gastro-oesophageal reflux disease. He underwent upper GI endoscopy for his persistent symptoms in another institution. Endoscopy showed features of antral gastritis. Gastric and duodenal biopsies were performed. Four days later, he presented with multiple episodes of bilious vomiting and epigastric pain. There were no other associated symptoms. He was admitted to a hospital elsewhere. Ultrasonography of the abdomen was done with features suggestive of a mesenteric cyst with volvulus. The child was transferred to our hospital paediatric surgical unit for further management. He was haemodynamically stable at presentation. He had persistent bilious vomiting with diffuse tenderness of the abdomen.

Investigations

Laboratory studies revealed iron-deficiency anaemia (haemoglobin—8.9 and low red blood cell indices). Serum creatinine and electrolytes including bicarbonate were within normal limits. Coagulation parameters (prothrombin time and activated partial thromboplastin time) were normal. CT of the abdomen with intravenous contrast revealed a hypodense elongated lesion extending from the third part of the duodenum to the proximal jejunum with abrupt narrowing of proximal jejunal loops, with features suggestive of a haemorrhagic duplication cyst (figure 1).

Figure 1.

Contrast-enhanced CT abdomen showing elongated thick-walled hypodense lesion (*) in close relation to the third and fourth part of the duodenum and proximal jejunum with luminal compression.

Treatment

Conservative management was attempted with nasogastric aspiration and intravenous fluids. He had high nasogastric aspirates and started developing signs of peritonism. Hence, it was decided to take him up for an emergency laparotomy. The stomach, the first and second part of the duodenum were normal. Fifteen centimetres of bowel from the third part of the duodenum to proximal jejunum was dilated, thinned out and filled with clotted blood. Serosa was deeply discoloured, congested and perforated (figure 2). Distal bowel loops were collapsed. There was no peritoneal contamination. There was no evidence of blood within the lumen of proximal or distal loops. The diseased bowel was resected and an end-to-end dudenojejunal anastomoses were done. The resected bowel was sent for histopathological examination.

Figure 2.

Intraoperative photo showing haematoma with serosal tear of the proximal jejunum.

Outcome and follow-up

Gross examination of the bowel revealed an intramural haematoma associated with mucosal prolapse and luminal narrowing (figure 3). Microscopic examination revealed extensive recent haemorrhage within the submucosa and muscularis propria. Crypt villus architecture was well preserved. There was no evidence of duplication cyst or vascular malformations. The postoperative period was uneventful. Oral diet was commenced at 72 hours. The child was discharged on proton pump inhibitors and was doing well. Six weeks after surgery, he presented with clinical symptoms of adhesive intestinal obstruction with bilious vomiting and abdominal distension. Conservative management was attempted, but as there was no clinical improvement, he was taken up for re-exploratory laparotomy. There were extensive small bowel adhesions predominantly involving the distal ileum with a small fresh perforation of the proximal ileum. No peritoneal contamination was noted. Resection of the perforated segment with primary anastomosis was done. The child recovered uneventfully and was discharged. The child was doing well on subsequent follow-up visits.

Figure 3.

Gross pathological specimen showing submucosal haematoma (club) with mucosal prolapse (arrow) leading to luminal obstruction.

Discussion

Trauma is the most significant cause of small bowel intramural haemorrhage in children. There are case reports of sporadic spontaneous intramural haematoma in children who were either on warfarin or low molecular weight heparin therapy.^{1 3} Other rare causes include bleeding disorders and malignancies. This condition has been well documented in adults who are on anticoagulation therapy. The incidence is reported to be around 1 in 2500. It can affect any part of the bowel, but small bowel, especially jejunum, is most commonly involved.^{4 5} It is said to be more common in patients on warfarin therapy, and a male preponderance has been noted in the limited available case series.⁶

Upper GI endoscopy with biopsy sampling or therapeutic injection for ulcers has also been reported to cause intramural haematomas.^{7 8} Incidence is estimated at 1:1250 upper GI endoscopies. There are only 18 published cases of children with intraduodenal haematomas following endoscopic biopsy.⁹ Children with leukaemia or post bone marrow transplantation with low platelet counts are especially at risk.¹⁰ Our patient had an upper GI endoscopy prior to the onset of symptoms but had no other predisposing factor and no evidence of any bleeding disorder.

The exact pathophysiology behind the development of intramural haematomas is not clear. The submucosa has a rich vascular supply, and the relatively fixed position of the duodenum makes it prone to developing a haematoma. It has been postulated that endoscopic biopsies in the duodenum can cause shearing of the mucosa from the submucosa resulting in extensive bleeding of submucosal vessels.² If the bleeding progresses, it can lead to intraluminal, mesenteric or retroperitoneal haemorrhage. Haemorrhagic ascites is also known to occur.¹¹

In our patient, haematoma had developed distal to the biopsy site, and there was no evidence of haematemesis or melena, indicating that the bleeding was exclusively intramural. This was supported by the histopathological examination. Mucosal intussusception, secondary to mucosal prolapse, causing intestinal obstruction has also been only rarely described.

These children initially present with complaints of mild abdominal pain. There is a gradual progression of symptoms with the onset of severe pain and vomiting indicating the development of obstruction. The delay between the injury and onset of obstructive symptoms is explained by the gradual increase in the haematoma due to the progression of bleeding and absorption of fluids secondary to the increased osmolarity.^{12 13} Symptoms of gastrointestinal bleeding and jaundice are also rarely seen. Jaundice may be due to external compression or from haematoma breakdown.

Contrast-enhanced CT is the mainstay of diagnosis. The characteristic findings include circumferential wall thickening, intramural hyperdensity, luminal narrowing and intestinal obstruction. Abbas et al gave more specific description consisting of thickening of the wall greater than 1 cm, with a partial reduction to total obstruction of the passage, the ‘pseudo-kidney’ and ‘coiled spring’ signs, generally, in short segments (average 23 cm) with profuse dilation of the flaps.¹⁴

Ultrasonogram (USG) is of questionable diagnostic value. It can be used as a first-line screening investigation and is the investigation of choice for follow-up for resolution of the haematoma. The sonographic evidence of intramural bleed includes a thickened and echogenic submucosal layer. Upper gastrointestinal series used to be done previously to demonstrate the obstruction but has become obsolete now. The most common differential diagnosis include duplication cysts of the bowel, Meckel’s diverticulum and lymphangioma.¹¹

The primary line of management for an intramural haematoma is conservative. This includes bowel rest, nasogastric aspiration, intravenous fluids and correction of coagulation parameters. There are multiple case series documenting a successful trial of conservative management in adults.⁵ It is justifiable in patients with only partial obstruction and who are clinically stable. The haematoma usually resolves within a few weeks.¹⁵ In the case of large haematomas and complete obstruction, surgery is the mainstay of treatment. Simple evacuation of the haematoma has been proven to be inadequate, and resection with primary anastomosis or additional bypass procedures are usually indicated.¹³ There are also reports of successful image-guided drainage (USG or CT) in select cases, but long-term follow-up is not available.⁹ There are no long-term reported complications.

Small bowel haematoma is a rare clinical condition. It should be considered in any child presenting with an acute abdomen who is on anticoagulant therapy or following endoscopy. Early diagnosis is important to have a good outcome.

Learning points.

• Intramural small bowel haematoma should be considered in any child presenting with abdominal pain or features of obstruction following an endoscopy.

• The risk is higher in children with coagulopathy or other predisposing conditions.

• Contrast-enhanced CT scan is the imaging modality of choice.

• Conservative management may be attempted in haemodynamically stable children, but surgery should not be delayed in those presenting with peritonitis or obstruction.