An unusual presentation of rectal atresia with rectal duplication cyst in a neonate: A case report.

A 10 days old male baby brought with abdominal distension and cystic swelling in the left buttock. Radiological investigations suggested para-rectal abscess or rectal duplication cyst. Patient underwent transverse loop colostomy and later definitive surgery by posterior sagittal approach. Intra-operative findings were suggestive of small atretic rectal segment with duplication cyst of the rectum. To the best of our knowledge, neonatal rectal atresia with rectal duplication cyst has not been reported in English literature.

Repair of Anorectal Malformation

Major advances in the management of anorectal malformations have been achieved during the last 20 years. Alberto Pena introduced the posterior sagittal anorectoplasty (PSARP) in 1982. He divided all the sphincteric muscles at the exact posterior midline and fully exposed the crucial anatomy. He was able to manage the associated fistula under direct vision with minimal urinary tract injury. The rectum could be placed in the middle of the sphincteric muscle complex. Even with PSARP by Alberto Pena himself, only 37.5% of all cases were considered totally continent. Anorectal malformation is still acontinuing challenge for the pediatric surgeons.

Long-term Results of Imperforate Anus Treated by Posterior Sagittal Anorectoplasty

PURPOSE: deVries and Pena in 1982, reported posterior sagittal anorectoplasty (PSARP) as an operative procedure for a high or intermediate imperforate anus (IA). PSARP has gained overall acceptance as a standard operative method for the repair of high and intermediate anorectal malformations. However, reports concerning the long-term outcomes of patients who have undergone a PSARP are still quite few. METHOD: To evaluate the quality of life for IA patients after a PSARP, 26 out of 43 patients who had undergone a PSARP were evaluated by using a questionnaire more than 10 years after closure of colos tomy. The questionnaire was composed of 5 categories; defecation patterns, defecation aids, social life, satisfaction, and problems after the PSARP. RESULTS: There were 23 males and 3 females. Ages were 13-26 years old. There were 17 rectourethral fistulae, 3 rectovesical fistulae, one rectal atresia, one rectovestibular fistula, and 2 rectovaginal fistulae. Follow-up periods were 12-16 years. For defecation patterns, normal defecation was observed in 62% of the patients, accidental soiling in 23%, and continuous soiling in 15%. For defecation aids, 54% of the patients used no defecation aids, 38% used enemas, and 8% used laxatives. In social life, 69% of the patients had no problems, 23% missed school, and 8% missed camp or a trip. As to the patients' own satisfaction, 81% wanted no reoperation and 19% wanted a reoperation. Problems after PSARP were reoperations for an anal transposition in 4 patients and a urethral stricture in one. CONCLUSION: PSARP resulted in good bowel control and satisfaction in the majority of IA patients, but some patients needed supportive measures and wanted a reoperation, if possible.

Posterior sagittal anorectoplasty, Secondary procedure, Anorectal malformation

Eleven patients underwent posterior sagittal anorectoplasty(PSARP) as a secondary procedure. Two of them had rectovaginal fistula and another two had rectocutaneous fistula. Six of the rest complained of frequent fecal soiling and the last one had severe anorectal stricture after perineal anoplasty. Five patients had lived with colostomy until the second operations were carried out. The ages at the time of the secondary PSARP were between 7 months and 29 years. Distal colostogram and MRI were taken to evaluate distal colon, position of the rectum and voluntary muscle. All patients had normal sacrum except one who had anorectal stricture. Seven patients, six with fecal incontinence and the other one with rectovaginal fistula had mislocated anorectums. Three patients, two with rectocutaneous fistula, the other one with anorectal stricture, had abdominal approach to obtain enough length of colon for pull-through procedure. With the posterior midsagittal approach, we could manage all the problems, rectovaginal fistulas, rectocutaneous fistulas, strictures and malpositioned rectums, without difficulty. No patients had serious complications except wound infection in one. All patients were satisfied with the results after redo-PSARPs even though normal continence has been achieved in only one patient. Seven patients who had continuous soiling or rectocutaneous fistula, needed no more diapers even though four of them showed fecal staining under stressful condition and the other three showed intermittent fecal leaking less than once a day. The rest three of the patients maintained their continence with support of drugs and/or enemas because of constipation. The PSARP is a popular procedure as a primary operation; however, our results suggested that this procedure also gave us a good opportunity for management of serious complication developed after primary anoplasties.