Ileal cavernous haemangioma in an adult presenting as a rare cause of small bowel obstruction

Abstract

Haemangiomas of the small bowel are a very rare entity and rarely considered as an aetiology for an intestinal obstruction. Contrast-enhanced CT is the investigation of choice but the lesion can be confused with malignancy or rarely a neuroendocrine tumour. Commonly it presents as abdominal pain with anaemia and/or melaena. With patients presenting without obstruction or acute gastrointestinal bleed, capsule endoscopy has shown to be a useful diagnostic tool.

We present here our experience of managing a case of a patient with ileal haemangioma who presented with a subacute small bowel obstruction and underwent a laparoscopic-assisted ileal segmental resection with side-to-side anastomosis. The lesion was a cavernous haemangioma on histopathological analysis.

BACKGROUND

Haemangioma is a benign tumour of vascular origin. It is a rare tumour with an incidence of 0.05% of all gastrointestinal tumours,¹ usually seen in the young with equal male to female ratio. The primary presentation of these haemangiomas is gastrointestinal bleeding with uncertain origin. It can also present as obstruction, intussusception, intramural haematoma, perforation and platelet sequestration.²

Depending on the size and type of vessels involved, haemangioma is a cavernous, capillary or mixed-type tumour. The most common one is the cavernous type and racemose haemangioma being the least common one.^{3 4} Most commonly it is found in the small bowel. Contrast-enhanced CT (CECT) is the investigation of choice to plan management approach. Capsule endoscopy is more accurate^5–7 but cannot be used in patients with acute or subacute obstruction. Preoperative diagnosis is difficult due to small superficial biopsies at endoscopy not yielding specific histological characteristics.^{7 8} Diagnostic laparoscopy with partial or segmental bowel resection is the optimal treatment approach here for symptomatic lesions.

We present our experience of encountering an ileal haemangioma in a 45-year-old woman who presented with recurrent attacks of subacute intestinal obstructions.

Case presentation

A 45-year-old woman was referred from Dubai with recurrent episodes of vomiting with abdominal pain for the past 6 months which was now increasing in frequency. Bowel habits were normal but she had a tendency for constipation for which she took on and off laxatives. She had no history of rectal bleeding or melaena. She had a loss of appetite for the last 2 years and significant weight loss in the last 6 months. She was admitted once for subacute bowel obstruction which was settled by conservative treatment.

Investigations

On referral to us, she had a CECT which showed a growth in her bowel in the mid-ileal region with increased vascularity most likely suggestive of a tumour of the small intestine. Blood investigations including complete blood count, liver and renal function tests were normal. Her haemoglobin was 125 g/L (normal range 120-155 g/L). Serum carcinoembryonic antigen was 2.1 ng/mL (normal range is <2.5 ng/mL). As the patient was symptomatic and the growth area was inaccessible by colonoscopy, it was decided to go ahead with the laparoscopic approach for this growth.

Differential diagnosis

The differentials were ileal carcinoma and neuroendocrine tumour because of the hypervascularity seen on CECT.

Treatment

The patient was advised diagnostic laparoscopy with ileal segmental resection for the same. On diagnostic laparoscopy, the mid-ileal abnormality was seen. Considering the hypervascularity, the involved loop of ileum was brought out through the supraumbilical port (figure 1). An extracorporeal resection of the entire segment was done taking the mesentery at the base. The patient had an ileoileal extracorporeal anastomosis and closure of ports.

Figure 1.

(A) Laparoscopic view of the haemangioma in the ileal segment and (B) extracorporeal ileal segment.

Outcome and follow-up

She recovered well and was discharged home on the 5th day. The resected specimen was sent for histopathology which confirmed it was a cavernous haemangioma (figure 2) with no atypia or malignancy. The lumen was markedly narrowed because of the dilated vessels and hypertrophy of the muscles. At 1-year follow-up, the patient was asymptomatic with the resolution of constipation.

Figure 2.

(A–D) Microscopic sections of the affected ileal segment which show numerous dilated blood vessels showing red blood cells in submucosa and muscularis propria.

Discussion

Haemangiomas of the small bowel are a very rare entity and show an incidence of 0.05% of all gastrointestinal tumours.¹ Haemangioma is a pseudotumour of vascular origin, congenital and benign. Haemangioma is classified into cavernous, capillary or mixed tumour,³ based on their biological characteristics into haemangioma and vascular malformations. Gastrointestinal haemangioma presents as single, multiple, or associated with a cutaneous haemangioma or various other syndromes such as blue rubber bleb nevus syndrome, Klippel-Trenaunay-Weber syndrome and Maffucci syndrome.⁹ They can also be high-flow and low-flow types, based on the angiographic findings.^{6 10 11}

Small intestinal haemangiomas are rarely reported in the literature. We analysed available literature by using the Medical Subject Headings words and PubMed search of terms viz ‘hemangiomas’, ‘capsule endoscopy’, ‘gastrointestinal hemangiomas’ and ‘gastrointestinal bleed’. We found around 23 cases of haemangiomas of various types in the literature until now. A summary is depicted in table 1 below. We found cavernous haemangiomas being the most common histology. The mean age is 40.65 years. The male to female ratio is 1.55:1. Jejunum is the most common site, 34.7%; ileum, 25.08%; stomach, 13.04%; and colon and rectum, 21.73%.

Table 1.

Summary of GI tract haemangiomas.^{5 6}

Sr no	Age (years)/sex	Preoperative diagnosis	Location in the GI tract	Treatment approach used
Cavernous haemangiomas
1	3/M	OGD, CECT	Stomach	Sleeve gastrectomy
2	47/M	VCE, CECT	Ileum	Partial small bowel resection
3	56/F	VCE, CECT	Ileum	Laparoscopic excision of the tumour
4	66/M	VCE, DBE	Jejunum	Excision of lesion
5	20/F	CECT	Sigmoid colon	Sigmoid resection
6	18/M	Colonoscopy, MRI	Rectum	Conservatively managed
7	32/M	CECT, MRI	Ileum	Laparoscopic excision
8	65/M	Colonoscopy, CECT	Left colon and rectum	Conservatively managed
9	62/M	VCE, DBE	Jejunum	Laparoscopic resection
10	52/M	CECT, VCE, DBE	Ileum	Laparoscopic resection
11	20/M	CECT	Jejunum	Segmental resection
12	44/M	VCE	Jejunum	Laparoscopic segmental resection
13	54/F	Colonoscopy, MRI, CECT	Rectum	Laparoscopic-assisted excision
14	22/F	Colonoscopy, CECT	Rectum	Laparoscopic-assisted excision
15	57/M	CECT	Jejunum	Segmental resection
Capillary
1	73/M	VCE, DBE	Ileum	Laparoscopic excision
2	75/M	VCE, DBE	Jejunum	Laparoscopic excision
3	11/F	Angiography	Jejunum	Laparoscopic excision
Racemose haemangioma
1	31/F	Angiography	Jejunum	Laparoscopic excision
2	54/F	OGD, CECT	Stomach	Local excision of lesion
No details on histopathology available
1	29/M	CECT	Ileum	Segmental bowel resection
2	2 days/ F	OGD	Stomach	Conservative and propranolol
3	44/F	Colonoscopy, MRI	Rectal	Conservatively managed

CECT, contrast-enhanced CT; DBE, double-balloon enteroscopy; GI, gastrointestinal; OGD, oesophago-gastroduodenoscopy; VCE, video capsule endoscopy.

Chronic anaemia due to persistent low bleeding and massive gastrointestinal bleed is rarely reported. Around half of the patients either present with anaemia, melaena and/or gastrointestinal bleed. Unlike in other cases reported in the literature, our patient did not have anaemia, hence the nature of tumours and association with anaemia should be pondered on.^{1 6} Twenty-three patients from literature analysis presenting symptoms of melaena in 11 patients, and anaemia in 8.⁶ However, shock and intestinal obstructions caused by gastrointestinal haemangioma were only observed in two patients. Among these, 15 were cavernous haemangioma, 3 cases of capillary haemangioma, 2 cases of racemose haemangioma, 1 case of hemolymphangioma and 1 case of hemangiolymphangioma.⁶

Phleboliths (50% of cases), obstructions or perforations may be identified on a simple abdominal X-ray.^{1 12 13} In acute conditions, CECT is the investigation of choice in the preoperative diagnosis of gastrointestinal haemangioma. Enhancement is seen in the tumour due to high vascularity.^{1 12 14}

Small haemangioma in the small bowel is usually not picked up in upper and lower gastrointestinal endoscopies; and even if seen, the superficial small biopsy may not yield results. Histologically ‘endothelialised muscularis mucosae’ feature of haemangioma is shown to be characteristic and can be used to have a high suspicion especially if endoscopy-guided biopsy is done to diagnose the lesion.¹⁵

Most of the benign tumours are incidental and are found on imaging but a small proportion of haemangioma is symptomatic.¹² Capillary haemangioma consists of conglomerates of small, thin-walled vessels. Haemangiomas may not always bleed and the person may have normal haemoglobin as they can be asymptomatic without active bleed or symptoms of obstruction.³ Newer imaging modalities for small bowel lesions include wireless capsule endoscopy, double-balloon enteroscopy, multiphase CT enterography and magnetic resonance enterography.^{1 13} Small bowel capsule endoscopy is a highly sensitive non-invasive imaging test but cannot be used for acute or subacute obstruction.¹³ In our case, the patient had a subacute intestinal obstruction, therefore, capsule endoscopy was an appropriate option.

The larger the size of the haemangioma, the more will be the muscle hypertrophy, which may cause luminal narrow, giving rise to mechanical obstruction.

We had CECT done but because the mass had a heterogeneous appearance with hypoattenuation and hyperattenuation without phleboliths, the differential in our CECT report was of the neuroendocrine tumour instead of a haemangioma. Similarly, in most patients with haemangioma, preoperative diagnosis may be difficult.

Diagnostic laparoscopy is the investigation of choice for most symptomatic bowel lesions where the diagnosis is not clear. Excisional biopsy is preferred in most patients but for larger lesions, the best approach seems to resect the whole lesion and perform an entero-enteric anastomosis, as it not only gives us a confirmed diagnosis but also can potentially treat the symptoms and disease.⁶ For vascular lesions, an extracorporeal dissection of mesentery and side-to-side anastomosis is a safer approach, less time-consuming and more economical. Hard lesions with peritoneal deposits, presence of ascites or associated lymphadenopathy should raise suspicion of malignancy. Where there is a high suspicion of malignancy, a frozen section can be done or surgically it should be excised based on principles of operating in case of malignancy.^{1 2 6} Surgical resection of the affected segment is the mainstay treatment for haemangioma as is generally localised.^{1 6}

Patient’s perspective.

I thank my doctors, especially the whole team who helped us and guided us through this difficult situation. My wife used to get admitted with these vague and acute episodes of pain and we used to get afraid as there was no proper diagnosis and cure available. but now that we have done the whole treatment and my wife is cured we thought it would be a good idea to report our case for experience sake for other doctors.

Learning points.

• Gastrointestinal haemangioma is rare and its presentation as the subacute intestinal obstruction is even rarer.

• Ileal haemangioma should be considered a differential diagnosis and should be suspected in a highly vascular lesion.

• Diagnostic laparoscopy is the investigation of choice as laparoscopic surgical resection; if needed, both can be done at the same time.