Atresia, Stenosis, and Other Obstruction of the Colon Treatment & Management

Updated: Sep 26, 2017
  • Author: Nelson G Rosen, MD, FACS, FAAP; Chief Editor: Eugene S Kim, MD, FACS, FAAP  more...
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Treatment

Approach Considerations

Like all other intestinal atresias, colonic atresia is fatal if the obstruction is not relieved. A baby with colonic atresia is at risk for dehydration, perforation, and sepsis. Any intestinal atresia requires operative intervention to prevent these complications. [54]

The specific operative indication in colonic atresia is complete bowel obstruction. Once a newborn is identified as having bowel obstruction, no additional information is required to prove the need for surgery. Other studies may be done before the operation, but no studies are necessary to confirm the need for surgery.

When colonic stenosis is very tight, it behaves like colonic atresia. In less severe cases, the child may have chronic problems such as bloating with feeds, cramping, or poor weight gain. Any of these symptoms may be significant enough to warrant either radiologic investigations or operative exploration and repair.

Intestinal obstruction at any level necessitates surgical relief. The timing of surgery in colonic atresia and stenosis depends on the patient’s clinical condition and any associated malformations and comorbidities.

The decision to proceed with primary correction (resection with anastomosis) or stoma diversion depends on the same factors, as well as on the skill and experience of the surgeon and the surgical team. Although no contraindications for surgery are recognized, stoma diversion is the minimum intervention necessary to relieve the obstruction. Severe underlying illness and associated life-threatening malformations may be considered relative contraindications for immediate primary repair.

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Initial Resuscitation and Support

Initial treatment of newborns with colonic atresia is directed toward resuscitation. The child often has a distended abdomen and is dehydrated. Usual treatments carried out before operative intervention include the following:

  • Nasogastric decompression
  • Intravenous (IV) fluid resuscitation
  • IV antibiotic therapy – This is usually administered to all patients, though the indications are not clearly evidence-based; because during the first 3 days of life the intestinal flora is not yet colonized with anaerobes, gram-positive and gram-negative coverage is sufficient at that time

After the prompt initiation of these basic measures, the child should be transferred to a neonatal intensive care unit (NICU) at a center with pediatric surgeons capable of correcting the malformation. Associated abnormalities of other organ systems may require attention and treatment.

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Surgical Therapy

Surgical management of colonic atresia is directed at eliminating the bowel obstruction and establishing intestinal continuity. In selected patients (ie, those with limited comorbidities and limited associated malformations), these goals may be achieved in a single operation by resecting the atretic ends and anastomosing the colon. If the child has significant comorbidities, a diverting enterostomy may be brought out just proximal to the atresia, and intestinal continuity may be restored during a second operation.

Choice of procedure

Some authors have advocated resection with primary anastomosis for right-colon lesions and colostomy diversion with subsequent reconstruction for left-side disease. In theory, the liquid feces in the right colon pose less of a risk to a fresh anastomosis than formed stool would. However, a 1990 study found that survival rates were unaffected by the type of procedure and that outcomes were excellent with either. [7]  A 2009 series reported excellent outcomes for primary anastomosis in 10 of 12 patients, two of whom had gastroschisis. [35]

Colonic atresia associated with Hirschsprung disease can be associated with abnormal distal colonic fixation. [55, 27]  The association of nonfixation with aganglionosis seems significant enough to warrant obtaining biopsy results before establishing intestinal continuity in those selected cases.

Diverting enterostomy alone is always a safe option if the suspicion of aganglionosis is significant and experienced pediatric pathology services are unavailable. Because of the rarity of this association, it may be reasonable to reserve biopsy for patients with intestinal fixation abnormalities, postoperative feeding or elimination difficulties, or failure to thrive.

The question of the necessity for routine rectal biopsy before the establishment of intestinal continuity is not so much controversial as it is difficult to answer definitively. The number of cases in which Hirschsprung disease is associated with colonic atresia is small but nonetheless enough to confirm that the association is greater than random, albeit still exceedingly rare.

The downside to taking a staged approach if Hirschsprung disease is a possibility is minimal in comparison with the risk of multiple operations and increased mortality when the association is missed before the anastomosis. Understanding both sides of this issue allows the surgeon to choose the approach that best serves their patient.

In congenital colonic stenosis, one usually finds less difference in the sizes of the proximal and distal limbs, making resection with primary anastomosis the preferred treatment. In acquired colonic stenosis following necrotizing enterocolitis, primary excision and anastomosis is the treatment of choice for stable patients without life-threatening comorbidities.

Hamzaoui et al reported using a transanal approach, similar to the transanal technique for Hirschsprung disease, after exploratory laparotomy with colostomy at birth in two patients with rectal and low sigmoid atresia (operated on at 6 and 3 months of age, respectively). [56]  No intraoperative or postoperative complications were noted at 2-year follow-up, and neither patient had fecal incontinence after closure of the colostomy.

Preparation for surgery

Basic preoperative laboratory measurements for all neonates should include a complete blood count (CBC), electrolyte assessment, and a crossmatch. If time permits, a contrast enema can be quite useful for assessing the colon and excluding any other narrowing, which can be difficult to accomplish during surgery.

All children who undergo laparotomy need adequate analgesia. Narcotics should be administered to babies only in a monitored setting (ie, a NICU with apnea, pulse oximetry, and cardiac monitors). No baby should be prevented from receiving adequate analgesia simply because he or she cannot articulate the need for it.

Primary repair

A transverse supraumbilical laparotomy is usually performed. The abdomen is eviscerated, and the bowel is inspected. The atresia should be readily identified by the dilated bulbous proximal portion with a microcolon on the other side (see the images below). A large mesenteric or intestinal gap may be observed between the proximal and distal ends, reflecting the region that would have been supplied by the vessel that sustained the antenatal vascular accident (the hypothetical cause of the atresia).

Intraoperative photograph displays proximal dilate Intraoperative photograph displays proximal dilated segment in patient with type 3 atresia of distal transverse colon. Courtesy of Richard Glick, MD.
Small portion of bowel held on right looks like ap Small portion of bowel held on right looks like appendix but is actually distal segment for patient with type 3 atresia at distal transverse colon. Courtesy of Richard Glick, MD.

The abdomen is then fully explored. The full intestinal length is examined for the presence of other atresias. This may require passing a catheter distally into the open end of the distal segment and distending the bowel with saline to ensure that the lumen is patent. The presence of multiple atresias may necessitate multiple resections; to minimize intestinal loss and the number of anastomoses, these atresias should be assessed collectively.

The dilated proximal portion of the colon is then resected. As a consequence of dysmotility, the dilated proximal bowel functions poorly. Resecting the proximal intestine back to an area of normal caliber is essential for postoperative function and, by lessening the size discrepancy between the two portions of the bowel to be joined, facilitates the creation of the anastomosis.

Only a minimal amount of distal bowel is removed. However, the atretic end is thickened and should not be used in closure; accordingly, that end is resected. The distal colon is usually divided on a bias, with more bowel removed on the antimesenteric side than on the mesenteric side (to promote better blood supply at the anastomosis). The antimesenteric side is usually opened further to counter the size discrepancy with the proximal end.

If a mesenteric gap is present, it is usually closed primarily, if possible.

Diversion with primary repair at second stage

In this operation, reconstruction is accomplished in much the same manner as in a primary repair (see above).

As in a primary repair, the proximal intestine is resected back to an area where bowel caliber is normal. This end is then brought out onto the anterior abdominal wall and matured as an ostomy.

If the patient is critically ill, the distal segment may be left completely undisturbed until the patient’s status changes in the future. If the patient is stable, the end of the distal is removed, and the fresh end is brought up to the abdominal wall skin as a mucous fistula.

Both ostomies can be brought out at the same location, either through the corner of the laparotomy or through a separate incision; they may also be separated by a skin bridge. Location, however, is nonessential. What is most important is that the colostomy be matured and positioned on the abdominal wall in such a way that an appliance can be securely placed around it.

The mucous fistula should not be matured like a mushroom; rather, it should be flush with the skin and only large enough to admit a catheter for irrigation or contrast radiography. If it is placed immediately next to the colostomy with no skin bridge, it should be small enough to allow appliance placement over both. If a skin bridge between ostomy and mucous fistula is preferred, it should be either small enough to be directly covered by the ostomy appliance wafer or far enough away not to interfere with ostomy appliance placement.

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Postoperative Care

Neonates who have undergone surgical correction of atresia or stenosis require neonatal critical care in conjunction with the pediatric surgical care.

In the early postoperative period, the intestine and soft tissues absorb fluid, which surgeons refer to as third-space losses. These losses necessitate rehydration to ensure the maintenance of intravascular volume and adequate tissue perfusion. Inadequate hydration can lead to pressor administration, which can impair intestinal perfusion. Third-space losses usually persist for 24-48 hours if no source of sepsis is present.

Patients who have undergone a colostomy require specific care for the stoma and its output. Bowel function usually returns more quickly in these patients than in those who undergo resection and anastomosis. Colostomy closure is performed electively at a future date. Most surgeons prefer to wait a minimum of 6-8 weeks to allow intraperitoneal inflammation to subside.

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Complications

Surgical treatment of colonic atresia and stenosis is associated with essentially the same complications as any operation involving bowel resection with stoma creation or anastomosis. Surgical site infection or incisional hernia can occur after any surgery.

The complication surgeons are most concerned about is anastomotic leakage. Leaks may manifest at any time but usually become noticeable several days to a week after surgery. Signs may be as subtle as mild tachycardia and fever or as obvious as rapid progression from mild illness to full-blown septic shock. Radiography may reveal intra-abdominal free air. Computed tomography (CT) may reveal abscesses. Anastomotic leakage calls for exploration and diverting enterostomy.

Intra-abdominal abscesses may occur without leakage but are rare. Intraoperative bleeding that leads to postoperative clotting can set the stage for abscess, which may be amenable to drainage by means of interventional radiology.

Anastomotic narrowing may occur and may be related to technical error, ischemia, or leakage. Patients who undergo colostomy may have stoma narrowing, prolapse, or parastomal herniation.

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Diet

Early parenteral nutrition is appropriate for in all patients. Patients with atresia, despite having their bulbous dysmotile proximal portions resected, can have prolonged postoperative ileus and usually benefit from early parenteral nutrition.

If a nasogastric tube was placed during the operation, it usually remains in place, at the discretion of the surgeon. The most traditional approach is to keep a nasogastric tube to suction (taking care to replace its output with IV fluids) until the child is passing flatus or stool. When that occurs, the tube may be placed to bedside gravity drainage; if that is well tolerated, the tube may be removed. Some surgeons clamp the tube before removal as a final test to see if the child can truly tolerate his or her own intestinal gases and secretions.

When the tube is removed, the oral diet may be started. Most surgeons start with a clear liquid, such as sugar water or a balanced electrolyte solution. When that is well tolerated, the diet is advanced to either breast milk or formula.

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Long-Term Monitoring

Follow-up imaging studies are not performed unless indicated by clinical issues. Children who have had a stoma created usually undergo a contrast enema before stomal closure so that the distal limb can be evaluated for stricture or obstruction.

Follow-up in the office is essential. Many of these babies have residual intestinal motility problems that may manifest in either the proximal or the distal segment. Close follow-up is essential to ensure that they are appropriately managed. Failure to thrive or long-standing intestinal motility issues may be a sign of missed Hirschsprung disease and should prompt a biopsy. [42]

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