Familial Idiopathic Pan-Colonic Varices Found Incidentally in a Young Patient with a Hepatic Flexure Tumor.

BACKGROUND Colonic varices are rare entity that often results from portal vein hypertension and hepatic cirrhosis. In the absence of underlying pathology, they are termed "idiopathic colonic varices". They are usually an incidental finding; however, they can present with varying degrees of lower gastrointestinal bleeding. There is only one reported case in the literature of colonic varices with a concomitant colonic tumor; our patient is the second one with such a presentation. We report a case of this rare combination with the outcomes of the elected surgical management and review the literature. CASE REPORT A 24-year-old male was referred to our hospital with a 1-month history of colicky abdominal pain. His family history is remarkable of 2 relatives with colonic varices. A computed tomography scan of the abdomen and pelvis showed a hepatic flexure colonic mass. Colonoscopy revealed pancolonic varices. Biopsy from the lesion revealed adenocarcinoma. Options were discussed with the patient to undergo only a right hemicolectomy for his cancer or a total colectomy to include the colonic segment involved with varices, and he elected the first option, with no complications upon 1 year follow up. CONCLUSIONS Idiopathic pan-colonic varices are rare pathology. Their presence with colonic tumor presents a dilemma as to whether a subtotal/total colectomy is needed on the premise that a limited resection may carry the risk of subsequent bleeding. In the literature, the only similar case to ours had brisk postoperative bleeding, while ours did not experience such a complication.

A Rare Case of Adult-Onset Rectosigmoid Hypoganglionosis.

BACKGROUND Intestinal hypoganglionosis is very rare and accounts for 3% to 5% of all classified congenital intestinal innervation disorders. Isolated hypoganglionosis of the colon is a particularly rare form of the disease, and differential diagnosis includes association with Hirschsprung's disease and chronic intestinal pseudo-obstruction (CIPO) related to visceral myopathies. Most cases are diagnosed at an early age or in childhood with only a few cases reported in adults. CASE REPORT We report a case of isolated hypoganglionosis of the rectum and sigmoid presenting as an emergency with acute intestinal obstruction in a 20-year-old male patient. A history of chronic constipation was reported since childhood, but this condition had never been investigated. A preoperative CT scan showed a megasigmoid and megarectum. A Hartmann's procedure was performed. The patient made a slow recovery and was discharged on the 12th postoperative day in good condition. Histology showed features consistent with isolated hypoganglionosis, and a full thickness rectal biopsies taken 2 months later confirmed the diagnosis. CONCLUSIONS Isolated hypoganglionosis in an adult is very rare, and a high index of suspicion is warranted in young patients with a history of chronic constipation to avoid delayed presentation as an emergency.