Background

Among the long-term complications of infant bowel resection, including necrotizing enterocolitis (NEC), anastomotic and perianastomotic lesions are relatively rare [1]. Anastomotic and perianastomotic ulcers are usually detected during investigations for chronic anaemia or faecal occult blood, and the diagnosis is often delayed by several years. However, in rare cases, ulceration may develop rapidly with massive life-threatening bleeding requiring emergency treatment [2].

Dieulafoy lesions are detected as protruding vessels within a minute mucosal defect or within normal surrounding mucosa. Furthermore, Dieulafoy lesions in the small bowel are uncommon in both children and adults. However, all cases present with overt bleeding, necessitating blood transfusions for serious lower gastrointestinal bleeding that generally requires rapid life-saving haemostatic procedures [3]. We report a 15-year-old girl with a history of surgery for meconium obstruction without mucoviscidosis in infancy who rapidly developed massive bleeding from an ileal perianastomotic Dieulafoy lesion and required emergency laparotomy for haemostatic procedures.

Case presentation

A 15-year-old girl born prematurely with a low birth weight (696 g) developed meconium obstruction without mucoviscidosis when she was 5 days old. A 3-cm section of her small intestine, including a stenotic lesion 35 cm from the ligament of Treitz, was resected. An ileostomy was performed 65 cm from the ligament of Treitz. The ileostomy was reversed 6 months later, with no further complications.

A day prior to the current presentation, the patient experienced intermittent lower abdominal pain and was emergently transported to a local hospital with presyncope and convulsions, which both occurred at 07:00 on the day of the consultation. There were no abnormal findings on head computed tomography (CT) or abdominal ultrasonography. Her haemoglobin level was 11.8 g/dL (reference range: 11.6–14.8 g/dL). The patient was sent home. Massive melena occurred at approximately 14:00. Furthermore, she had difficulty maintaining an upright position and was therefore again urgently transported to the local hospital. Her haemoglobin level had decreased to 6.7 g/dL over the 7-h period since her morning visit to the hospital. Although blood transfusion was started, massive melena persisted, and she was transferred to our hospital at 23:00. The patient’s height and weight were 160 cm and 45 kg, respectively. Her Glasgow Coma Scale score was 14/15 (eye opening 3, best verbal response 5, best motor response 6), her blood pressure was 109/51 mmHg, and her pulse was 76 bpm in the Trendelenburg position. However, her blood pressure dropped to 73/36 mmHg, her consciousness further decreased, and she went into shock during the examination. Mild oppressive pain in the lower abdomen was noted. There was no increase in C-reactive protein, and the patient also tested negative for coronavirus, norovirus, adenovirus, and faecal bacteria. Contrast-enhanced abdominopelvic CT examination showed massive bleeding in the colon, but the source was not identifiable. The patient was subsequently diagnosed with haemorrhagic shock due to lower gastrointestinal bleeding. Thereafter, emergency endoscopy was performed under general anaesthesia. In the operating room, systolic blood pressure dropped into the 60s, necessitating an intraoperative transfusion of 1600 mL of blood. Although no abnormalities were detected in the upper gastrointestinal tract, lower gastrointestinal endoscopy revealed massive blood flow from the terminal ileum, and the patient was diagnosed with small bowel haemorrhage. The decision was made to perform open surgery. Intraoperative findings included irregular dilation in the ileum 40 cm from the ileocecal junction, and a scar presumed to be the anastomotic site was also noted (Fig. 1). Dark red stagnant intestinal content was clearly observed on fluoroscopy. An incision made in the direction of the long axis revealed microulceration with exposed vessels on the mesentery and persistent bleeding (Fig. 2). Since suturing alone was not sufficient to achieve haemostasis, a cuneiform incision was made at the same site, and haemostasis was achieved. Histopathological examination of the surgical specimen showed microulceration with a partial epithelial defect and inflammatory cell infiltration (Fig. 3). No abnormalities were observed in the surrounding membranes. An ileal Dieulafoy lesion was diagnosed based on the appearance of slightly distended arteries and veins in the submucosa of the ulcerated site. The postoperative course was uneventful. The patient was discharged on day 11 of hospitalization. She recovered well enough to participate in her school’s athletic competition 2 months later.

Fig. 1
figure 1

Surgical findings: Irregular intestinal dilation and dark-red stagnant intestinal content were observed in the ileum 40 cm from the ileocecal junction. A scar assumed to be the anastomotic site from prior surgery was noted at the same site

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Fig. 2
figure 2

A 2-cm incision made along the long axis revealed microulceration with exposed vessels on the mesentery and persistent bleeding. Since suturing alone was not sufficient to achieve haemostasis, a cuneiform incision was made at the same site, allowing haemostasis to be achieved

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Fig. 3
figure 3

Intraoperative histopathological findings: A 25 × 20 × 10 mm specimen of the small bowel consistent with ulcer; HE 4X magnification. Microulceration, with a partial epithelial defect and inflammatory cell infiltration, was detected. A slightly dilated artery was identified in the submucosal layer [yellow arrow]

HE: haematoxylin and eosin staining.

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Discussion and conclusions

Acute massive lower gastrointestinal bleeding, as in our present patient, is a rare long-term complication after infant bowel resection.

We treated a 15-year-old girl who had experienced rapid onset of massive bleeding from an ileal perianastomotic Dieulafoy lesion and required an emergency laparotomy for haemostatic procedures. She had a history of surgery for meconium obstruction without mucoviscidosis in infancy. It is important to keep anastomotic or perianastomotic ulcers in mind in patients who have a history of intestinal resection during the neonatal period.

The incidence of symptomatic anastomotic ulcers in the paediatric population is approximately 0.3%, and such ulcers are a relatively rare complication of bowel resection [1]. However, anastomotic or perianastomotic ulcers are known to cause chronic anaemia and occult bleeding, which are both potential long-term complications after bowel resection in infancy. Sudden massive lower gastrointestinal bleeding is rare. However, Dieulafoy lesions can produce serious lower gastrointestinal bleeding requiring rapid life-saving haemostatic procedures [2].

As our patient initially presented with presyncope and convulsions, gastrointestinal bleeding was not suspected at the first visit. However, melena manifested 7 h later, and she went into shock 9 h thereafter. This patient’s course showed that a perianastomotic Dieulafoy lesion can be life-threatening and that an urgent haemostatic procedure is required. Sudden massive bleeding might render children especially susceptible to haemodynamic instability, and the onset may have an atypical course. Caution is thus required in patients with a history of abdominal surgery in the neonatal period, even those who have been well over the long term since the operation.

To our knowledge, this is the second report describing a patient who developed sudden massive lower gastrointestinal bleeding long after surgery performed in infancy [2].

Our search of PubMed from 1992 to 2020 for anastomotic or perianastomotic ulcers in paediatric populations, with the search terms “anastomotic ulcers”, “perianastomotic ulcers”, “infant” and “neonatal”, yielded nine reports in English describing the symptoms of these lesions (Table 1) [1, 2, 4,5,6,7,8,9,10]. Of the 35 cases presented in Table 1, including ours, two had a sudden onset, while the onset was more gradual in the other cases. Of all the cases, 60% had no symptoms of overt bleeding, 66% eventually had to undergo surgery, and 59% developed recurrences during follow-up. Some reports described little or no follow-up after the operation performed in infancy, but this may not be clinically appropriate. Given the high percentage of recurrent cases, regular follow-up is highly recommended for children who undergo surgery in infancy [2].

Table 1 Reports of anastomotic or perianastomotic ulcers after infant bowel resection in children
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Video capsule endoscopy and double-balloon endoscopy have both been reported to be safe and effective in children as endoscopy methods for the small intestine. Video capsule endoscopy might be useful as postoperative follow-up for children because it is easy and noninvasive, and a patency capsule test can reduce the risk of retention [10,11,12].

The pathogenesis of Dieulafoy lesions involves both congenital and acquired factors, such as abnormal distribution and dilation of submucosal capillaries, microaneurysms, and arteriovenous malformations combined with mucosal injury secondary to mechanical factors and other causes [13, 14].

The pathogenesis of the lesion in our present case was a perianastomotic site from the surgery performed in infancy. The patient also suffered from intermittent lower abdominal pain the day before the visit, suggesting microulceration caused by mechanical triggers as well as changes in the vascular distribution near the anastomotic site as the probable underlying causes. Moreover, we suspected that intestinal ischaemia occurred during the operation performed when she was an infant.

We conclude that although sudden massive lower gastrointestinal bleeding requiring emergency care is a rare long-term complication after infant bowel resection, Dieulafoy lesions can produce serious bleeding requiring rapid life-saving haemostatic procedures. Long-term follow-up is recommended after surgery in infancy due to the high percentage of recurrences of anastomotic ulcers.