Management of a small bowel hemangioma causing intussusception in an infant: A rare case report and literature review

Introduction Hemangiomas of the small intestine are rare and usually present in young people. They are very difficult to diagnose preoperatively. We report a rare case of mixed intestinal hemangioma (IH) causing intussusception in a pediatric patient. Case presentation A 3-month-old girl, with no prior medical or surgical history, was admitted with rectal bleeding and paroxysmal crying due to intermittent abdominal pain. An urgent abdominal ultrasound revealed ileo-ileal intussusception. Operative findings confirmed the intussusception, and a segmental resection of the intussuscepted jejunum was performed. Histopathological examination found a mixed hemangioma. The postoperative course was uneventful. Discussion Clinical presentation may include intestinal bleeding leading to anemia, obstruction, intussusception and perforation. Intussusception caused by small bowel hemangioma is extremely rare. Notably, we didn't find any cases of small bowel hemangioma revealed by intussusception in children. The main treatment for hemangiomas is surgical resection of the affected segment. No evidence in the literature on postoperative recurrence of hemangiomas. Conclusion Intussusception secondary to intestinal hemangiomas is extremely rare. Preoperative diagnosis is challenging as they are often undetectable with traditional techniques. Enhanced awareness and understanding of this condition can facilitate earlier diagnosis and improve management outcomes.


Introduction
Intestinal hemangiomas (IH) are rare, accounting for only 0.05 % of all intestinal neoplasms [1].These growths typically occur in young people, with no preference for either sex [2].The jejunum is the most affected site in the gastrointestinal tract [2].Common symptoms include abdominal pain, bleeding, and chronic anemia [3].Intussusception caused by a small bowel hemangioma is extremely rare.Here, we report a case of a mixed hemangioma of the jejunum in a 3-month-old girl admitted for intussusception.This case report has been reported in line with the SCARE Criteria [4].

Case presentation
A 3-month-old girl, with no prior medical or surgical history, was admitted with rectal bleeding and paroxysmal crying due to intermittent abdominal pain.Clinical examination revealed a mild fever of 38 • C, tachycardia with a heart rate of 180 beats per minute, and signs of dehydration including skin turgor, sunken eyes and depressed fontanelle.The patient also exhibited abdominal bloating, tenderness and hematochezia.A non-contrast abdominal X-ray demonstrated air-fluid levels in the small bowel.An urgent abdominal ultrasound revealed a jejunal intussusception.The patient's general condition rapidly worsened, leading to fatigue and pallor.Given the high probability of necrosis associated with the jejunal intussusception we opted for surgery rather than radiographic reduction, which is not commonly performed for proximal intussusception in our department.After a short period of resuscitation, the patient underwent a midline laparotomy with a presumptive diagnosis of intestinal intussusception.Operative findings confirmed the intussusception, and a segmental resection of the intussuscepted jejunum was performed, followed by an end-to-end anastomosis.Macroscopic examination revealed a large polypoid pedunculated lesion measuring 4.6 × 2.1 × 1.5 cm in the lumen (Fig. 1).Hematoxylin and eosin staining of representative sections showed multiple blood-filled spaces lined by thin endothelial cells and separated by scant inflammatory and connective tissue stroma.The overlying mucosa displayed hyperplastic glands (Fig. 2).Immunohistochemical analysis showed positivity for CD31.The final diagnosis was a mixed hemangioma causing intussusception of the jejunum.The postoperative course was uneventful, and the infant was discharged 7 days after surgery.

Discussion
Hemangioma is a benign vascular proliferation and should be distinguished from vascular malformation which is a lesion with structural anomalies and not a true tumor.IH are rare and account for 7-10 % of all benign neoplasms of small bowel [1].IH may be solitary or multiple as a manifestation of Maffucci syndrome, Klippel-Trenaunay syndrome, disseminated neonatal hemangiomatosis, or blue rubber bleb nevus syndrome [5].A search of PubMed database was performed to find studies on solitary hemangioma of small bowel in patients under 16 years, published before December 2023.We identified 23 cases in total [1,3,.Table I summarizes the patients' data.Clinically, gastrointestinal hemangiomas are symptomatic in 90 % of cases [27].Clinical presentation includes abdominal pain, bleeding, chronic symptoms of anemia, obstruction, perforation, intramural hematoma, and intussusception [8].Intussusception secondary to a small bowel hemangioma is extremely rare.Notably, we didn't find any cases of small bowel hemangioma revealed by intussusception in children, in the English literature.Very few cases causing intussusception were described in adults [28,29].
Hemangiomas are difficult to diagnose preoperatively with traditional techniques like upper and lower endoscopies, but advanced imaging modalities such as capsule endoscopy, double balloon enteroscopy (DBE), computed tomography and magnetic resonance imaging are available options for investigating small bowel lesions.In an emergency setting like ours, the final diagnosis is typically made through histologic examination.Macroscopically, IH is a bluish-purple lesion.Histologically, the lesion is composed of blood-filled spaces of variable sizes and shapes.The lumens are filled with blood cells, lined by thin endothelial cells, and separated by connective tissue stroma.According to the size of affected vessels, hemangiomas are histologically classified as cavernous, capillary, or mixed type [1].The main treatment for hemangiomas is surgical resection of the affected segment.Gastrointestinal hemangiomas usually have a satisfying prognosis, and there is no evidence in the literature on the recurrence of hemangiomas [26].

Conclusion
Intestinal hemangiomas are rare, and those presenting as intussusception are even rarer.Preoperative diagnosis is challenging as they are often undetectable with traditional techniques.Enhanced awareness

Fig. 2 .
Fig. 2. Microscopic examination: multiple various blood-filled spaces which were lined by thin endothelial cells and separated by scant inflammatory and connective tissue stroma.

Table I
Small bowel hemangiomas in infants reported in the literature.