Nadeem Akthar, Junaid Ashraf, Abid Quddus Qazi, Naeem Khan.
An experience with rectal duplications and review of literature.
Pak J Med Sci Jan ;17(4):245-50.

Summary: We are reporting eight cases of rectal duplications over a period of 11 years from 1989 to year 2000. Three of these cases were associated with colonic duplications and were of tubular variety whereas rests were cystic. Presentation of each was completely different. Mode of presentation, methods of diagnosis and management strategies are discussed along with the review of literature.

Case One: First issue of a 22 years old mother a male baby, was brought at birth with multiple anomalies, these included; dorsal meningocele, hemi vertebrae in the region of 12th dorsal vertebra; anorectal agenesis with two perineal raphies, a diphallus with two urethrae and a Talipes equino varus. While performing de-functioning transverse colostomy a complete double barrel type of a complete duplication of large bowel was noted. Meningocele was also repaired at the same time. On further investigation, an ante grade barium enema revealed high anorectal agenesis of both rectums with no demonstrable urinary tract connection. Cysto-urethrograms simultaneously through both the urinary meati revealed double bladders. An excretion urogram also had similar findings with normal kidneys. On exploratory laparotomy at nine months of age, two blind ending rectums were incorporated into one pouch using auto staplers. At the same time Anoplasty was also performed. Rest of the double barrel colons were left undisturbed. The duplication was noted upto 6 inches of terminal ileums. Double bladders were noted to have one ipsilateral ureters draining in its’ own side. It was also left undisturbed. Colostomy was closed after six weeks of anorectal reconstruction and the patient had reasonable fecal continence. We did not disturb the urinary tract and the child was passing urine with stream from both urinary meati. Both penile urethrae were encased within one penile body. Correction of Talipes was also subsequently achieved.

Case Two: An eight years old girl was admitted for what was thought to be a perineal fistula since birth. In the past, when she was only one year of age, she had her small left adrenal tumour removed in America. There was no medical record of the finding and management. Examination under general anesthesia revealed, a central anal canal of normal caliber and an eccentric smaller opening, which also resembled anus with surrounding skin puckering. The contrast was introduced through both orifices, this revealed double rectum up to descending limbs of pelvic colon. Further investigations could not be performed because parents decided to take the child back to America for further treatment. She had staged surgery in USA with a proximal de-functioning colostomy followed by, through posterior sagittal approach excision of eccentric less developed anal canal with incorporation of two rectums above the sphincter. This was followed by closure of colostomy. Apparently she is well.

Case Three: A newborn baby had a prolapsing bowel form what looked like a bony defect in the sacrum. It was thought to be a rare form of posterior cloacal fissure. A contrast enema through anal canal demonstrated a completely normal rectum and colon separate from the prolapsing large bowel, this was removed through posterior approach and the defect was closed with local rotation flap of gluteal skin. Histology revealed typical rectal tissue and mucosa of cystic duplicated bowel.

Case Four: A five-year-old male child presented with a painless, circular, cystic swelling of one-inch circumference over the sacrococcygeal junction. This was thought to be a midline dermoid and was explored. At operation, a circular cystic lesion with fully developed muscular wall was excised. On opening it contained meconium and histological report was of rectal tissue and mucosa. This was a case of sequestrated cystic rectal duplication.

Case Five: A two and half year old girl, who had presented with a lipomatous mass over the sacrococcygeal area, was thought to have a mature sacrococcygeal teratoma. A rectal examination, however, was insignificant and no intra-pelvic extension was noted. Barium enema also revealed a normal rectum and colon with no displacement. An abdominal and pelvic ultrasound was also normal. But on ultrasound of the mass, a cystic teratoma was suspected. On exploration beside the fatty tissue, fully formed large intestine like structure was found which was removed in its entirety along with surrounding lipomatous tissue. On Histopathology, the structure was reported fully developed encysted rectal duplication in the surrounding tissue there was no teratomatous tissue. Only fibrofatty tissue was present in the surrounding area of the duplication.

Case Six: Another case with a similar presentation as in case 5 was seen in a five year old girl, who had presented with a lump over her sacral area, which was extending eccentrically over the left gluteal surface. On exploration, there was a long encysted duplicated rectum with no internal connection. It contained large amount of mucoid material and on histological examination had rectal mucosa with fully developed muscularis.

Case Seven: A seven-year-old boy who had presented with feeling of fullness and bulge in the perineum, on examination, had a diffuse perineal bulge adjacent to the tip of the coccyx. On rectal examination, there was a cystic swelling in pre-sacral area, the upper limit of which could not be reached. A contrast study revealed indentation and displacement of rectum anteriorly. On ultrasound, a cystic swelling was noted with a possible diagnosis of anterior meningocele. Urinary bladder, urethra and kidneys were normal. The lesion was removed through combined abdominoperineal approach. On examination, it was a sequestrated colonic lesion containing darkish inspissated cast. The child’s recovery was uneventful.

Case Eight: An eight-month-old female presented with two complete sets of vulva. Each had fully developed urethra vagina and patent anal canal. She also had bifidity of spine (split notochord). Barium anima showed double rectums and sigmoid colons. A cystourethrogram from each urethral orifice demonstrated two bladders. Although we could not evaluate uteruses and ovaries but it is presumed that these were also duplicate. Since she was having no symptoms and was passing stool and urine from both side’s parents were reassured and no surgical intervention was considered necessary.

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