CASE REPORT

Idiopathic colonic varices

Sasha Reiderman¹, Cristian Nicolás Lucas², Pablo Roberto Catalano³.

Sanatorio Franchín, Buenos Aires, Argentina.

1 General Surgery Resident

2 Staff Member of the Coloproctology Section and the General Surgery Service

3 Head of the Coloproctology Section and Staff Member of the General Surgery Service

ABSTRACT

Colonic varices are very rare and an exceptional cause of recurrent lower GI bleeding. In most cases they are associated with portal hypertension. They are considered idiopathic when the etiopathogenesis is due to a congenital vascular disease. These have a greater familial association and are more frequently pancolonic than in non-idiopathic cases.

Colonic varices are diagnosed by colonoscopy. The presence of clinical risk factors for prothrombotic disease should be investigated and imaging studies perform to rule out portal hypertension or associated venous obstruction. Treatment is mainly symptomatic.

We report a case of idiopathic colonic varices in a 16-year-old female patient who presented with lower gastrointestinal bleeding. The aim is to evaluate the behavior of a very rare condition and to review the related literature

Key words: colonic varices, lower gastrointestinal bleeding, angiodysplasias.

INTRODUCTION

Colonic varicose veins are a rare entity. They are usually detected by colonoscopy and in most cases are associated with portal hypertension.¹ The incidence of colonic varices, regardless of their cause, is estimated at 0.07% and they are diagnosed by routine or emergency colonoscopy. They are an exceptional cause of recurrent lower GI bleeding.²

Idiopathic colonic varicose veins are submucosal venous dilatations of the colon, whose etiopathogenesis is due to a congenital vascular disease, in the absence of a triggering cause and without another associated medical condition. They are extremely rare, and more frequent in men with a mean age of 41 years at diagnosis. They have a greater familial association and are more frequently pancolonic than in non-idiopathic cases.³ There is very little information in the literature, with around 30 cases reported by English authors.⁴

Idiopathic colonic varices must be differentiated from those secondary to portal hypertension, either due to cirrhosis or other causes associated with portal vein obstruction.^5-7

During colonoscopy due to active bleeding, insufflation can collapse these vascular malformations, which can be confused with normal mucosa, polyps, or even carcinomas.^8,9

The gold standard for diagnosis is mesenteric angiography. This method makes it possible to identify varices and, in the event of bleeding greater than 5ml/min, to detect the specific site and perform embolization treatment.⁹

CASE

A 16-year-old female patient, with no personal or family history of interest, went to the emergency room due to rectal bleeding of 7 days of evolution, last episode 6 hours ago, palpitations and syncope. She denies urine output disturbances and weight loss. She refers to having consulted the previous week for a similar picture without hemodynamic alterations, being diagnosed with bleeding due to hemorrhoidal disease, for which she received medical treatment.

On admission, she presented tachycardia, functional class III, dyspnea, palpitations, and  hypovolemia grade 2 of the American College of Surgeons classification. Her abdomen was soft, slightly tender on the right flank. The digital rectal examination was positive for bright red blood.

Admission laboratory: hematocrit 25%, hemoglobin 8.6g/dl, white blood cells 7,400/mm3, platelets 226,000/mm3, prothrombin 90.5%, KPTT 30.6 seconds. Transfusion of 1 unit of red blood cells and expansion with crystalloids were indicated. With the patient compensated and without signs of active bleeding, it was decided to carry out complementary studies.

Esophagogastroduodenoscopy reported mild erosive gastritis, with no signs of active bleeding. Colonoscopy showed a prominent, tortuous bluish venous cord from the cecum to the descending colon that diminishes with insufflation, with no evidence of active bleeding.

Diagnosis: colonic varices with predominant involvement of the right, transverse, and descending colon. (Fig. 1 A and B)

A

B

Figure 1. Colonoscopy. Tortuous varices of the colon are seen in the

submucosa. A. Lesions with a nodular appearance. B. Serpiginous vessels.

The angiotomography reported changes in the vascular structures, images compatible with varicose veins involving the right colon and extending to the rectum. There was also marked involvement of the terminal ileum and changes in the thickness of  branches of the mesenteric vessels, some dilated, especially the superior and inferior mesenteric veins. No stenotic areas are seen in the colon or small intestine. Rectum and regional fat with no alterations. Although an increase in vasculature was observed at this level, it was less than that seen mainly in the right colon and terminal ileum. The caliber of the portal vein was 12 mm and that of the splenic vein was 4 mm. No venous thrombosis was observed (Fig. 2 A and B).

B

A

Figure 2. CT angiography. A. Coronal section. Colonic varices and thickening and dilation of branches of the superior and inferior mesenteric veins. B. Sagittal section. Colonic varicose veins.

Abdominal ultrasound showed a liver with preserved echostructure, and portal and lower extremity Doppler ultrasound showed normal flow velocity in the portal vein and inferior vena cava, without signs of portal hypertension or lower extremity thrombosis.

Liver function, serology for viral or autoimmune liver diseases, and imaging studies of parenchymal structure were normal. The presence of markers compatible with prothrombotic phenomena was ruled out.

The patient evolved without evidence of new bleeding. Oral diet and diosmin 500 mg orally every 8 hours were indicated, and the patient was discharged to continue with outpatient monitoring.

During follow-up, the patient presented an isolated episode of rectal bleeding at his home, with no other associated symptoms and stable hematocrit and hemoglobin values.

Direct relatives were asked to perform a colonoscopy to assess the probable familial incidence, ruling out evidence of colonic varicose veins in all cases.

DISCUSSION

Lower gastrointestinal bleeding secondary to colonic varices is a rare entity. Many patients present with multiple bleeding episodes before reaching the correct diagnosis. They can present with pictures of intermittent or severe hematochezia that indicate the study of the gastrointestinal tract or be incidental findings during a colonoscopy.¹⁰ Bleeding originates with a higher incidence in the territory of the inferior mesenteric vein and less in the superior mesenteric vein.¹¹ Our patient presented a first episode of mild rectal bleeding that recurred severely a week later. Once the diagnosis of colonic varicose veins was made by colonoscopy and confirmed with CT angiography, the presence of associated diseases was investigated, without finding a cause of portal hypertension, so the diagnosis of idiopathic colonic varices with ileal involvement was made. The absence of pathology in the relatives studied ruled out a family origin. Familial idiopathic colonic varices are a very rare congenital venous malformation with a familial incidence of 28%.¹² Due to the low frequency of this condition the treatment is not clearly defined. A diet that does not cause constipation and even the use of laxatives and iron supplements can be recommended. In cases of active bleeding with hemodynamic stability, treatment with sclerotherapy or rubber band ligation is described. C When the bleeding is not self-limiting and puts the patient's hemodynamic stability at risk, invasive treatment should be evaluated, either through hemodynamics or surgery with resection of the affected colonic segment.^3,13

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