Rectal atresia and stenosis: Unique anorectal malformations

Division of Pediatric Surgery, Colorectal Center for Children, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Journal of Pediatric Surgery (Impact Factor: 1.39). 06/2012; 47(6):1280-4. DOI: 10.1016/j.jpedsurg.2012.03.036
Source: PubMed


Rectal atresia/stenosis is a rare disorder in the spectrum of anorectal malformations and is particularly associated with a presacral mass. These patients are born with a normal anal canal but have a stricture or complete atresia located a few centimeters proximal to the dentate line. We present a surgical technique for the management of these patients, as well as their unique clinical concerns and outcomes.
We reviewed the records of 14 patients with rectal atresia and 3 with rectal stenosis. We describe a novel technique that we have developed for the preservation of the anterior dentate line that was performed in the last 13 patients.
Rectal atresia/stenosis was associated with a presacral mass in 5 patients (29%). Definitive repair was completed using a circular rectorectal anastomosis in the first 4 patients and an anterior dentate line sparing procedure in the last 13. All patients older than 3 years have demonstrated the ability to have voluntary bowel movements.
With the largest reported series of rectal atresia/stenosis, we have demonstrated a safe and effective technique for repair. Preoperative evaluation must be thorough because a significant number of these patients will have an associated presacral mass.

29 Reads
  • Source
    • "Laparotomy revealed CA and confirmed Wingspread low subtype ARM. While Wingspread high subtype ARM may involve rectal atresia, our case had no evidence of fistulae , presacral mass or anal musculature atresia also commonly associated with high Wingspread classification (Hamrick et al. 2012; Osifo et al. 2014). This fact implies a multifactorial cause of the deformities, with rectal atresia occurring distinctly from anal malformation. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Colonic atresia and anorectal malformation are rare congenital anomalies individually. Few reports of the conditions combined in a single patient have been published in the literature. Neither colonic atresia, anorectal malformation or a combination of the disorders has previously been reported in the Haitian population. A 5-day-old female presented with feculent emesis, failure to pass stool since birth and an imperforate and stenotic anus. Exploratory laparotomy revealed colorectal atresia distal to a malformed cecum and a Wingspread low subtype anorectal malformation without any associated urogenital fistulae. Temporizing percutaneous ileal drainage was followed by second-stage anal perforation and dilation, ileal J-pouch and pull through. This is the first reported case of colonic atresia, anorectal malformation or the combination of the disorders among the Haitian population and one of only a handful of such cases reported worldwide. Although vascular accidents in utero have been implicated as the etiology of colonic atresia, simultaneous presence of anorectal malformation suggests a multifactorial cause. Investigation for multisystem abnormalities is warranted. Two-staged operative correction is considered the best treatment; however, long-term postoperative outcomes are uncertain. The coexistence of colonic atresia and anorectal malformation is a very rare occurrence and presents unique clinical and operative challenges. Investigation for additional congenital abnormalities is appropriate, and although two-stage operative correction is considered the best treatment, long-term outcomes are uncertain.
    Full-text · Article · Apr 2014 · SpringerPlus
  • Source
    • "As smaller lesions can be missed by using ultrasound only, we have changed our protocol. Nowadays, we routinely perform pelvic MRI before PSARP e on the 2nd day of life in case of primary repair operation or at about 3 months of age in case of colostomy as recommended by Hamrick et al. [13]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: We report a unique coincidence of malignant tumor projected via anus in an infant girl operated on for congenital anal atresia without fistula. The case seems to be different from Currarino syndrome. After five month of uneventful follow-up after posterior sagittal anorectoplasty (PSARP), a rapidly enlarging polyp-like bulge protruded from the anus. Blood tumor markers were negative. X-ray computed tomography (CT scan) revealed a tumor located subcutaneously between the coccygeal bone and rectum. The intraoperative biopsy showed malignancy. By perineal approach, the tumor was radically resected en-bloc with anus, part of neorectum, and distal fragment of coccygeal bone. Mature descending colostomy was performed. The postoperative course was uneventful. The final histological diagnosis revealed embryonic malignant teratoma. The bone scintigraphy was negative. Additional chemotherapy was applied without complication. After one year follow-up free of tumor recurrence or metastasis, the perineal anus reconstruction was performed by combined abdominal and posterior sagittal approach. Anal dilatation program was started on 10th postoperative day when the stoma was closed. Five months after the last surgery, the girl presents bowel control and defecates regularly 2–3 times a day without complaints, constipation or cicatricial anal stenosis. Oncologic surveillance is negative.
    Full-text · Article · Aug 2013 · Journal of Pediatric Surgery Case Reports
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Rectal atresia or stenosis is an extremely rare anorectal malformation associating a normal anal canal with a stricture or a complete rectal atresia. We describe a case of rectal atresia in a newborn female presenting with an abdominal distension and failure of passing meconium.
    Full-text · Article · Oct 2014 · Pan African Medical Journal
Show more