A systematic review of symptomatic hamartomas of the jejunum and ileum.

INTRODUCTION: Gastrointestinal hamartomas are rarely encountered benign growths made of an abnormal mixture of tissues normally found in the body where the growth occurs. Most hamartomas are asymptomatic and diagnosed incidentally. Evidence regarding symptomatic hamartomas of the jejunum and ileum is limited and restricted to anecdotal case reports. We aim to provide a contemporary overview to clarify various aspects linked to symptomatic hamartomas, helping aide management and clinical decision-making.

METHODS: The search terms 'small bowel', 'small intestine', 'jejunum' and 'ileum' were combined with 'hamartoma'. Embase, Medline, PubMed searches and Google Scholar hand-searches were conducted. All English language papers from 1 January 2000 to 1 June 2020 were included.

RESULTS: In total 39 cases were reviewed: 29 adults and 10 paediatric (0-16 years). Presenting symptoms included abdominal pain (87%), vomiting (56%), gastrointestinal bleeding (20.5%) and weight loss (5%); 36 of 39 cases (92%) presented as emergencies. Diagnostic investigations included: abdominal x-ray (49%), endoscopy (38%), computed tomography (56%), magnetic resonance imaging (5%), contrast study (15%) and video capsule endoscopy (5%). Initial management strategy was either open (30) or laparoscopic (7) surgery or endoscopy (2). All cases required surgery for definitive management. Twenty-five cases recorded hamartoma size, and mean maximum diameter was 3.56cm (1.2-8cm) with stricturing in seven cases. Nine of the 39 cases were associated with hamartomatous syndromes. Two mortalities were recorded.

CONCLUSIONS: This is the first published review addressing symptomatic hamartomas of the jejunum and ileum. New data relating to demographic cohort, symptom profile, investigations, management strategies, and morbidity and mortality provide greater insight to those encountering this challenging clinical finding in the future.