colonic duplications are congenital anomalies reportedly present in 0.2% of the pediatric population . such malformations have been described all along the gastrointestinal tract , although they are rarely found in the colon ( only 5%-6% ) . complete tubular forms are even more unusual . since this condition is usually clinically asymptomatic , diagnosis prior to surgery is difficult and can only be confirmed during a laparotomy . according to some authors , 80% of the cases are symptomatic before the age of two , presenting with acute abdomen secondary to perforation or massive bleeding . moreover , others report that diagnosis in adulthood is also very difficult , because of a very non - specific clinical presentation . we report the case of a complete colonic duplication presenting with  ascites and acute abdomen. the duplication began at the terminal ileum , 4 - 5 centimeters from the ileo - caecal valve ; which showed 2 caeca and 2 appendices , as well as two complete tubular colonic formations , ending in a blind pouch located at the rectal level . one normal anus was present . given the low incidence and unique clinical presentation of this anomaly , we hope that publishing our case will enhance diagnostic precision and contribute to improved surgical treatment . a 6-month - old patient was admitted into the pediatric intensive care unit with an initial diagnosis of urinary septic shock . his personal history reflected the presence of posterior urethral valves and left vesicoureteral reflux ( vur ) , accompanied by severe hydronephrosis and ipsilateral renal atrophy . three months prior to the septic event the patient had undergone a cystoscopic resection of the valves . at the moment of the admission he presented with intense hypotonia , high fever , painful tympanic abdomen on palpation and a severe general state . blood tests showed dehydration and severe metabolic acidosis . over the next 18 hours the patient required vasoactive drugs to maintain hemodynamic signs . physical examination revealed signs of acute abdomen with a large accumulation of fluid between the bowels . the laparotomy revealed a peritoneal cavity filled with serous fluid and the terminal ileum duplicating at 4 - 5 cm distance from the ileo - caecal valve , ending in two caeca and two appendices [ figure 1 ] , which were continued with a total tubular colonic duplication . both colonic tracts had a common wall [ figure 2 ] , intimately connected to each other . the anti - mesenteric edge was hugely dilated and showed clear ischemic signs in its transversal portion , ending in a blind pouch located at the rectal ampulla level , at 3 - 4 cm from the normal anus . we resected the total duplicated gut , excluding the rectum , by combining conventional surgery with the use of a linear mechanical stapler to resect the most distal portion due to its difficult access . the outer fragment shows ischemic signs corresponding to the portion that was removed during surgery the histopathology of the lesion revealed hemorrhagic necrosis of the resected gut . this could be related to the massive dilation and decreased blood supply of the anti - mesenteric gut . the final diagnosis was acute abdomen and shock eighteen days later , following restoration of intestinal passage , the patient had recovered acceptably and was discharged from the hospital , with antibiotic prophylaxis for vur . he was followed up in clinic for a year , with abdominal ultrasounds and also seen by the nephrologists and urologists . at the moment , he is asymptomatic . colonic duplications are the most unusual type of intestinal tract duplications , and total tubular forms are even rarer . several authors describe the high association of this type of anomalies with genitourinary or lower vertebral column , such as prostatorectal fistula , anorectal or scrotal abnormalities and myelomeningocele . the pathogenesis of the entity is not clear , and many theories have been postulated about it . this wide variety of explanations , no fully accepted theory or demonstrated , suggesting that the origin of intestinal duplications may be multi - factorial and that the current molecular genetics research will probably give us an acceptable explanation regarding these anomalies in the near future . in the present case , the duplication was associated with posterior urethral valves , leading to a clinical picture of left hydronephrosis and renal atrophy . this association of lesions should be included in the spectrum of previously reported associations , some of them very similar in urologic nature . the most interesting aspect of this case was its clinical outcome : ascites and acute abdomen with severe hemodynamic and metabolic instability that endangered the patient 's life . this type of clinical debut is rare , usually discovered as a casual finding , because of its asymptomatic course or its clinical appearance in adulthood . the lack of a unique clinical picture of the entity constitutes a serious diagnostic dilemma . either way , there is no uniform pattern of clinical onset , but always seems to appear in the early stages of life . from a morphologic perspective we agree with most authors that cystic duplication at any point along the colon 's anatomy is the most frequent type of anomaly , followed by partial tubular duplications ( next to the main intestinal tract ) very few cases of complete cecoapendicular duplications , such as the one presented here , exist in the literature . with regard to proposed surgical techniques , most of the authors prefer a complete excision of the duplicated tract , although some authors support the creation of a common channel , using mechanical sutures for the common wall resection to avoid the accumulation of fecal material in the duplicated gut , as was described by yucesan . in our case however , this technique could not be used since the ischemic and inflammatory component of the duplicated gut clearly required the excision of the piece .
we report the case of a 6-month - old infant who presented with a complete duplication of the large intestine , debuting clinically with acute abdomen and severe metabolic disorders . 
 we discuss the pathogenesis and morphology of the lesions , diagnostic difficulties and peculiarities of surgical treatment .