Paediatric angiodysplasia of the jejunum: a case report and review of the literature

Introduction

Gastrointestinal angiodysplasia (GIAD) is a vascular abnormality of the mucosa or submucosa of the alimentary tract that most commonly occurs in the ascending colon and cecum.^1,2 It is thought to be due to the degenerative process of ageing, thus it is rare in the paediatric population. The majority of patients are asymptomatic, but lesions occasionally bleed causing melena, hematochezia, haematemesis, anaemia or rarely massive bleeding and hypotension. ³ Investigations into acute bleeding depend on the mode of presentation and include imaging techniques such as computerized tomography (CT) angiogram, upper and lower gastrointestinal endoscopy or laparotomy. Radiographical and surgical management in children should only be considered in acute, severe cases when more conservative measures have failed, as it is invasive and associated with higher mortality rates. ⁴

This paper presents an unusual case study of a 10-year-old girl who presented with an acute, severe upper and lower gastrointestinal bleed and haemodynamic instability, the subsequent surgical challenges encountered and how these were addressed.

Case report

A previously well 10-year-old girl presented with fresh rectal bleeding, cramping lower abdominal pain and tenesmus. The patient's medical and family histories were unremarkable, but it was noted that she had fallen off her bike the previous day. On physical examination she was tachycardic, pale and experiencing episodes of reduced consciousness. Initial blood results showed a haemoglobin of 6.7 g/dL. Fluid resuscitation and blood transfusions were initiated. Despite active resuscitation, she remained haemodynamically unstable and continued to pass fresh, red blood rectally; therefore, she was transferred to theatre for upper gastrointestinal endoscopy, colonoscopy and subsequent laparotomy. During surgery a copious amount of blood was seen throughout the gastrointestinal tract, but no active bleeding point was identified. An incidental appendicectomy was performed. The bleeding appeared to have stopped and, as she was haemodynamically stable, she returned to intensive care.

Postoperatively her haemoglobin dropped from 12 g/dL to 6.4 g/dL over approximately six hours despite transfusion. A total of 173 mL of blood was aspirated from the nasogastric tube, confusing the clinical picture of upper and lower gastrointestinal bleeding. A CT angiogram was ordered as she was haemodymically stable. The CT angiogram images demonstrated potential bleeding in the jejunum, but it could not confirm whether this was due to a single or multiple bleeding points (Figure 1). Embolization was not performed, as the risk of bowel infarction would have been significant if there were multiple bleeding points over a length of small bowel. As a result, a second laparotomy was performed.

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Figure 1

CT angiogram axial plane of the gastrointestinal lesion. This is an axial plane CT angiogram image taken in the arterial phase. It shows extravasation of contrast indicating active bleeding into the lumen of the jejunum

At repeat laparotomy a copious saline lavage of the whole small bowel was performed to allow optimal views during small bowel endoscopy. This was done through a duodenal enterotomy and the re-opened appendix stump. The whole length of the small bowel was visualized and the only abnormality – and potential bleeding point – was located at 100 cm from the duodenojejunal flexure (Figure 2). This was a small, cherry-like lesion that was not bleeding. To confirm whether this was the source of bleeding, this section of bowel was isolated using soft bowel clamps for 10 minutes. Prior to releasing the bowel clamps, repeat endoscopy of the remaining small bowel proximal and distal to the clamped segment demonstrated no blood present. When the bowel clamps were released fresh blood was noted in this segment of the bowel, confirming this to be the source of bleeding. A resection of 30 cm of jejunum containing the lesion and a primary anastomosis were performed.

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Figure 2

Endoscopic image of the angiodysplasia in the mid-jejunum. The lesion was identified at 100 cm from the duodenojejunal flexure. The cherry-red, flat spot surrounded by the pale white ‘halo sign’ is indicative of GIAD

Following surgery she remained clinically stable and was discharged after five days. At follow-up six months later she has had no further gastrointestinal bleeding.

Discussion

In the adult population acute gastrointestinal haemorrhage is a common medical emergency with a mortality rate of about 10%. ⁵ GIAD is also common in the adult population and particularly those over 60, with two-thirds over 70 years of age.^1–3,6 GIAD and acute rectal haemorrhage rarely occur in the paediatric population.^7,8 To our knowledge this is the first case of an acute gastrointestinal haemorrhage in a child due to GIAD of the jejunum.

This is an unusual presentation as GIAD lesions are normally asymptomatic and incidentally found; however, when lesions bleed they may present with melena, hematochezia or anaemia. ³ Massive upper and lower gastrointestinal bleeding and hypotension associated with GIAD, as in this patient, are very uncommon. The fresh upper and lower gastrointestinal bleeding confused the clinical picture and caused difficulty in determining the level of bleeding in this patient. The site of this lesion, the jejunum, was also unusual as approximately 80% occur in the cecum and ascending colon. ¹

This patient's presentation posed a diagnostic challenge. Normally imaging and endoscopy are the first line investigation; however, the patient needs to be clinically stable. Endoscopy also has limited use in terms of location and so a combination of upper, lower, deep small bowel or capsule endoscopy methods may be used. Surgery is rarely required for rectal bleeding in the paediatric population and should be reserved for acute emergency cases, continuing and worsening digestive haemorrhage, ineffective endoscopic and angiographic haemostasis or massive bleeding. ⁹ Complete small bowel endoscopy allowed visualization of any potential lesions along the gastrointestinal tract and on-table small bowel preparation with a copious saline lavage is recommended. In this case only one lesion was identified in the jejunum. Initially this was not bleeding, possibly due to the warm saline wash. However, by isolating it using soft bowel clamps for 10 minutes it was proven that this was the source of bleeding, and this technique is to be recommended to any similar case where the exact source of bleeding is difficult to identify.

This case highlights the importance of considering angiodysplasia in the upper gastrointestinal region in children and the surgical management options to consider in order to prevent a potentially fatal outcome.

Funding: The authors have not received any financial support.

Author contributions: All authors have read and approved the publication of this manuscript.