Caecal Diverticulum Causing Catastrophic Gastrointestinal Bleeding in a Child: A Case Report.

Solitary caecal diverticulae are rare in children and presentation with massive gastrointestinal (GI) bleeding is seldom reported. We present the case of a 13-year-old boy with a two-year history of abdominal pain and multiple inconclusive investigations presenting with a life threating lower GI bleed. We also review the literature surrounding solitary caecal diverticulae and caecal duplication cysts (CDCs).

Introduction

Profuse gastrointestional (GI) bleeding in children, leading to haemodynamic instability is most commonly associated with a Meckel’s diverticulum. Herein, we report a rare cause of profuse gastrointestinal bleeding in an older child.

Case Report

A 13-year-old boy (90kg) presented to a referring hospital with a three-day history of bloody diarrhoea, right iliac fossa pain and shortness of breath. On presentation, he was hemodynamically unstable (BP 80 systolic, HR 140 bpm) with a haemoglobin of 34g/L (130-180g/L). His coagulation screen was normal. He was commenced on tranexamic acid, transferred to the paediatric surgical centre and received a massive blood transfusion (6 units). There, a computed tomography angiogram was undertaken which did not demonstrate active bleeding but demonstrated an abnormal tubular structure arising from the ileocaecal valve (Figure 1a).

Figure 1

a. CT angio showing the relation of the caecum (Ca) to the solitary caecal diverticulum b. Resected specimen demonstrating the caecum (Ca), CD (dashed line) and area of perforation (arrow) were adherent to gonadal vessels c. Histology showing all three layers of diverticulum wall (CD) and hypertrophic gastric mucosa (GM)

He reported a two-year history of intermittent abdominal pain with episodes of bloody stool and constipation. In the preceding year, he had had a normal colonoscopy and oesophago-gastro-duodenoscopy. Oesophageal, gastric, duodenal, colonic and terminal ileal biopsies showed no histological abnormalities. His faecal calprotectin was mildly elevated at 90 ug/g and his haemoglobin was 103 g/L at that time. Magnetic resonance enterography had demonstrated terminal ileal thickening with no other areas of inflammation. There had been no concerns on antenatal scans.

In the paediatric surgical centre he underwent a laparotomy which demonstrated an outpouching at his caecum. This outpouching was adherent to his gonadal vessels (Figure 1b). No typical appendix was visualised. The rest of his small bowel and liver were unremarkable. He had a limited right hemicolectomy and primary anastomosis.

Histopathology demonstrated a perforated solitary caecal diverticulum with heterotopic gastric mucosa (HGM) (Figure 1c). No appendix was definitively identified.

At two-month follow-up, he reported to be completely pain-free with no further episodes of GI bleeding.

Discussion

We report a rare case of caecal diverticulum with HGM resulting in life threating GI bleeding in an older child. HGM in a rectal diverticulum causing hematochezia in a 14 year old girl has been reported 1. We identified a single case report of CDC as a cause of rectal bleeding and haemoglobin drop in a 9-month old male 2. This was diagnosed on colonoscopy and ultimately resected2. Interestingly, they reported benign glandular lining within the cyst wall2; we were able to confirm HGM in our specimen. Heterotopic gastric mucosa can be found anywhere in the GI tract from mouth to anus. It is typically seen in the oesophagus, duodenum, Meckel’s diverticulum or duplication cysts3.

Profuse GI bleeding resulting in haemodynamic instability does present in children. It is most often described in Meckel’s diverticulum which is typically in the absence of abdominal pain, has a quoted incidence of between 1 and 4% and the majority are resected in those under the age of 4 years 4. Vascular malformations arising from the GI tract have been reported to cause rectal bleeding in children occasionally5. In our patient, GI bleeding from longstanding inflammatory bowel disease had been ruled out on previous investigations. Colorectal carcinoma is seldom seen in children and may cause GI bleeding. It is associated with genetic syndromes such as familial adenomatous polyposis (FAP) and can present with a range of symptoms, unfortunately often late6. Carcinoid tumours have also been reported to cause massive lower GI bleeding in adults although there are few reports in children7.

A tubular CDC was also considered but they typically do not communicate with the colonic lumen although it is likely their underlying pathogenesis is shared. Several theories exist as to the origin of enteric duplication cyst. It is suggested that could be secondary to failure in regression of embryonic diverticula, errors of splitting of the notochord or failure in recanalization of the primitive intestine8.

Enteric duplication cysts are rare in children having an estimated incidence from neonatal autopsies of 1/4500 8. Colonic duplication cysts account for only 13% of enteric duplication cyst. The majority (80%) of these are cystic, located on the mesenteric side of the bowel and occur in the caecum 8. They contain all layers of colonic wall including muscularis propria9. They rarely contain gastric mucosa therefore GI bleeding is infrequent, a more common presentation being that of appendicitis8,9. Similarly, solitary congenital caecal diverticulum are rare, include all layers of the caecal wall and tend to present with pain rather than life-threatening haemorrhage10.

Conclusion

We report a rare cause of life threating GI bleeding in an older child due to a perforated caecal diverticulum with HGM. Patients with ongoing symptoms and negative investigations may benefit from diagnostic laparoscopy.