AUTHOR AND EDITOR INFORMATION

Section 1 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

INTRODUCTION

Section 2 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Atresia and stenosis of the colon

The term colonic atresia describes a condition in which a part of the colon has failed to correctly form, and that part is either completely blocked or is altogether missing. Colonic stenosis describes a condition in which a part of the colon is very narrow, resulting in a partial blockage. Other obstructions of the colon that affect newborns include Hirschsprung disease and small left colon syndrome, as well as obturation obstruction, meconium ileus, or meconium plug. These issues are all forms of colonic obstruction; however, they are different from atresia and stenosis and are more completely reviewed in their own articles. 

The colon is the rarest site of atresia in the GI tract. It is a congenital anomaly which may be suggested using prenatal ultrasonography and is usually revealed in affected newborns shortly after birth. Patients usually present with abdominal distention and failure to pass meconium.

Stenosis of the colon is much more common. Patients usually present later in life, most often because of an identifiable event. In congenital stenosis, a narrow segment of colon is observed, but bowel continuity is maintained. A discrepancy between the colonic segments above and below the area of stenosis is present. In acquired stenosis, which is commonly referred to as stricture, what starts as a normal segment becomes narrowed. This is most common in premature babies who have recovered from an episode of necrotizing enterocolitis.

History of the Procedure

Colonic atresia was first recorded in 1673.¹ The first survivor was reported by Gaub in 1922 and was treated using a diverting colostomy.² Potts reported the first survival following primary repair in 1947.³

Problem

The common problem in both atresia and stenosis is intestinal blockage, either partial or complete.

In colonic atresia, the problem is complete bowel obstruction. Gas and stool cannot pass, and the colonic segment above the atresia becomes distended. If left untreated, this leads to perforation. 
In colonic stenosis, the problem is that gas and stool try to pass through a narrow area. While the baby is passing soft baby stools, this may or may not be noticeable. When the baby's diet changes from breast milk or formula to cereals and solid foods, the stool can become thicker and more formed. This may cause stenosis to become symptomatic, leading to distension, feeding intolerance, or failure to thrive. 

In babies who have had necrotizing enterocolitis, stenosis occurs after the original episode has resolved. This may manifest in varying degrees, ranging from minor feeding intolerance and distension to near-complete bowel obstruction.

Frequency

Colon atresia is very rare. Various incidences have been reported, ranging from 1 in 1500 live births⁴ to 1 in 66,000 live births.⁵ Webb (1931) and Benson (1968) cited the incidence as 1 in 20,000, which is most reflective of experiences in the modern era.^{6, 7} In a 1982 report, Powell suggested that colonic atresia represents 5-15% of intestinal atresias,⁸ whereas in 1966, Freeman reported a lower figure of 1.8%.⁹ In 1953, Gross recorded 6 cases of colonic atresia out of 140 cases of intestinal atresia (4.3%) at the Boston Children's Hospital.¹⁰

Multiple atresias are uncommon in the colon; however, colonic atresia may be overlooked when small intestinal atresia is present.¹¹ Rare cases of familial colonic atresia have been described. Animal studies have shown an autosomal recessive pattern of inheritance in cattle. Hereditary multiple intestinal atresia affects both the large and small intestine, whereas nonhereditary multiple intestinal atresia usually spares the colon.¹²

The incidence of colonic stenosis is not readily available because most cases are acquired. In 1953, Gross noted a single colonic lesion in 71 patients with intestinal stenosis.¹⁰ Necrotizing enterocolitis is the most common etiology of postnatal colonic stenosis; narrowing develops in 10-25% of affected patients.^{13, 14, 15}

Etiology

See Relevant Anatomy.

Pathophysiology

See Relevant Anatomy.

Clinical

Patients with colonic atresia or congenital stenosis may sometimes have findings on prenatal ultrasonography, such as dilated bowel loops or the presence of polyhydramnios. Initial physical examination findings are normal in the absence of associated conditions. The anus usually appears normal. Progressive abdominal distention develops. Rectal examination reveals white or pale mucus rather than pigmented meconium. Failure to completely pass meconium suggests atresia, whereas delayed passage of meconium (>24 h) suggests Hirschsprung disease. Patients with colonic atresia may pass meconium normally because the incident that caused the atresia may have occurred after the colon had become filled with meconium.

Colonic stenosis usually follows some form of injury to the colon, and ischemia is considered central to the insult. This injury may be in utero or postnatal. The infant or child may present with symptoms similar to atresia with high-grade stenosis; less stenotic lesions may not become apparent until feeding is undertaken. In those instances, the child's abdomen may become distended with feeding, and stool production is scant, if present. 

Babies with necrotizing enterocolitis may show signs of acquired stenosis following their acute episode. When the septic signs of the illness resolve and the child is doing well, feeding is often attempted. Those babies who have formed stenoses usually do not tolerate feeds and become distended. Studies may be performed to confirm the diagnosis and to try to localize the site of narrowing, at the discretion of the surgeon.

Associated conditions

Colonic atresia has been associated with abdominal wall defects and abnormalities of the genitourinary tract.¹⁶ Nonfixation of the colon has been reported.¹⁷ Association with anal atresia¹⁸ and imperforate anus¹⁹ has been reported but is extremely rare. Colonic perforation may occur.²⁰ This is thought to be caused by overdistension of closed colonic loop, with gas and stool trapped between a competent ileocecal valve proximally and the blind-ending colon distally. In 1988, Pohlson et al reported perforation of the terminal ileum in one case, clearly demonstrating that perforation can occur anywhere proximal to the obstructed bowel.²¹

Hirschsprung disease has been present in a small number of cases.^{22, 23, 24, 25} Additional anomalies associated with this pairing include omphalocele²⁶ and absence of a hand.²⁷ In most cases the aganglionosis is discovered after colostomy closure when the distal bowel does not properly function. Some authors have recommended that rectal biopsy be performed at the time of laparotomy,²⁸ whereas others believe that biopsy should be reserved for children who do not pass stool readily upon restoring bowel continuity after resection.^{23, 25} Because colonic atresia is rare and because of the morbidity and mortality associated with missing the diagnosis prior to establishing intestinal continuity, rectal biopsy prior to definitive repair would seem prudent.²⁹

Cardiac conditions that require catheterization may predispose a baby to a mesenteric vascular incident resulting in colonic stenosis. Additional conditions reported in patients with colonic stenosis include cryptophthalmia syndrome (ie, cleft lip and palate, microphthalmia, dysplastic kidneys, proximal jejunal atresia), arthrogryposis, proximal intestinal atresia, neoplasm, and malrotation.^{4, 8} Riley-Day syndrome (ie, familial dysautonomia) has been associated with spontaneous colon ischemia.³⁰ Coloboma, cataracts and facial hemihypertrophy, facial asymmetry with palsy, microphthalmia with partial iridial coloboma, exophthalmia, and bilateral optic nerve hypoplasia all have been reported.⁸ Kim et al (2000) described a case of colonic atresia in monozygotic twins.¹²

INDICATIONS

Section 3 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

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.

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 prior to operation, but no studies are necessary to confirm the need for surgery.

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

RELEVANT ANATOMY

Section 4 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Embryology, anatomy, and pathophysiology

The colon arises from the digestive tube, which is present by the end of the first month of gestation. Rapid elongation begins at 5 weeks' gestation. Over the ensuing 5 weeks, the intestinal tube, separable into cephalad and caudal limbs (based on the relationship to the omphalomesenteric duct), rotates counterclockwise and returns to its familiar position in the abdomen. The proximal caudal limb receives its blood supply from the superior mesenteric artery (SMA), whereas the inferior mesenteric artery (IMA) supplies the distal portion.³¹

The SMA gives rise to the ileocolic, right colic, and middle colic arteries, which supply the ileocecal region, the ascending colon, and the proximal transverse colon, respectively. The left colic artery, arising from the IMA, supplies the left portion of the transverse colon and the descending colon. The middle colic and left colic arteries join near the splenic flexure to form the marginal artery. The sigmoid arteries, rectosigmoid arteries, and branches of the IMA supply the sigmoid colon.

Small bowel and colonic atresias are not believed to occur by the same process as in duodenal atresia, which is suspected to be due to failure of vacuolization of the duodenum and was described by Tandler in 1900. 

In 1955, Louw and Barnard hypothesized that small bowel atresias are caused by prenatal vascular interruption.³² The same mechanism is believed to cause colonic atresia. Thrombosis, volvulus, and herniation with strangulation are all mechanisms that may cause in utero vascular injury and bowel necrosis with subsequent reabsorption. Fairbanks proposed a relationship between fibroblast growth factor 10 expression, vascular development, and colonic atresia.³³

Although the colon receives its blood supply from the SMA and the IMA, much variation among smaller named arterial branches is observed. The portions of the colon most vulnerable to ischemia appear to be the splenic flexure and the ileocecal area. Coincidentally, these areas are the farthest from the major trunks. Nevertheless, atresia and stenosis occur throughout the colon; the wide variation in terminal branches coupled with the complex rotation and fixation of the bowel may create regions of bowel ischemic injury in utero that result in atretic or stenotic lesions.³⁴

Any process that leads to occlusion of branches of these vessels in utero may result in atresia. Compression at the umbilical ring,³⁵ internal hernia, intussusception,³⁶ choledochal cyst,³⁷ volvulus, and thrombosis have all been implicated in the initiation of bowel infarction, leading to disintegration, gradual reabsorption of dead tissue, and sealing of the bowel ends in the fetus as described by Louw in 1964.³⁸ The bowel content is sterile; thus, sepsis does not occur. Meconium is produced throughout the gut during the third trimester; thus, a newborn whose atresia occurs after that time may still pass meconium in the newborn period.

Prenatal maternal use of vasoconstrictive medications, such as cocaine, amphetamines, nicotine, or decongestants, has been suggested to be a risk factor for intestinal atresia formation.³⁹

Congenital stenosis occurs when the bowel injury is incomplete. This may happen when injury occurs close to the bowel wall, allowing collateral blood flow to preserve the injured tissue. Another mechanism is limited ischemia, in which the blood supply is partially or intermittently occluded, resulting in incomplete intestinal injury.

Acquired stenosis is more common than atresia or congenital stenosis. Via the same mechanism of vascular compromise, the injured bowel undergoes healing and scarring with narrowing of the affected intestine. Intense inflammatory reactions, such as those that occur in necrotizing enterocolitis and Crohn disease, may result in stricture. Tuberculosis-associated left colon stricture has also been reported.⁴⁰

Patients who undergo bowel resection for any reason and have a segment of intestine removed and joined by anastomosis may develop stricture at the anastomotic site due to ischemia or technical issue. Surgery is usually required to revise the joined loops.

Colon atresia is typically classified using the 1989 descriptions of intestinal atresia by Bland-Sutton and the 1964 descriptions by Louw.^{41, 38} In type 1 lesions, the bowel and mesentery remain intact, but the bowel lumen is interrupted by a complete membrane (see Media file 1). Type 2 lesions are those in which the bowel is discontinuous, connected by a fibrous cord. In type 3 lesions, the bowel ends are completely separated, and the mesentery has a gap. Stenotic lesions are characterized by intact bowel with incomplete occlusion and require no classification.

In 1990, Davenport et al reviewed 118 cases of colonic atresia. The distribution of the lesions by site was as follows:⁵

• Ascending colon - 33 (28%)
• Hepatic flexure - 4 (3%)
• Transverse colon - 27 (23%)
• Splenic flexure - 30 (25%)
• Descending and sigmoid - 24 (20%)

Two thirds of colon atresias are in the distribution of the IMA. This may relate to lack of collateral blood supply or disease processes that render this portion of the colon more susceptible to injury.

CONTRAINDICATIONS

Section 5 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Intestinal obstruction at any level requires surgical relief. The timing of surgery in colonic atresia and stenosis depends on the patient's clinical condition and their 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 the skill and experience of the surgeon and their team. Although no contraindications to 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 to immediate primary repair.

WORKUP

Section 6 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Lab Studies

In all children with neonatal bowel obstruction should, obtain electrolyte measurement and CBC count and perform a crossmatch prior to operative intervention.

Imaging Studies

• Antenatal ultrasonography may reveal bowel loop distention or polyhydramnios.
• Plain radiography of the abdomen reveals bowel obstruction and may reveal a prominent dilated loop. A prone view shows absence of gas in the rectum, although gas may be present if the film was obtained after the rectum was examined.
• Contrast enema may be performed prior to surgery at the discretion of the surgeon and can be useful in ruling out the presence of other lesions downstream from the atresia. It reveals a microcolon with a rounded proximal end (see Media file 2).
• Radiography in congenital colonic stenosis also reveals an obstructive pattern that may mimic atresia. Contrast enema reveals narrowing of the colon, with limited filling of the dilated proximal colon (see Media file 3).

Diagnostic Procedures

• Rectal biopsy (performed either as a suction biopsy prior to surgical correction or at the time of operative correction) should be considered important in evaluating for associated Hirschsprung disease.^{42, 29}

TREATMENT

Section 7 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Medical Therapy

Initial treatment of newborns with colonic atresia is directed toward resuscitation. The child is often distended and dehydrated. Usual treatments prior to operative intervention include the following:

• Nasogastric decompression
• Intravenous fluid resuscitation
• Intravenous antibiotics

Surgical Therapy

The management of colonic atresia is directed at eliminating the bowel obstruction and establishing intestinal continuity. In selected cases (with limited comorbidities and limited associated malformations), this may be performed in one 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.   

Many authors advocate resection with primary anastomosis for right colon lesions and colostomy diversion with subsequent reconstruction for left-sided atresia and stenosis. Theoretically, the liquid feces in the right colon pose less risk to a fresh anastomosis than formed stool. However, in practice, Davenport et al noted that the type of procedure had no effect on survival rates, and outcomes were excellent using either technique.⁵

Colonic atresia associated with Hirschsprung disease is often, but not exclusively, associated with abnormal distal colonic fixation.^{43, 29} The association of nonfixation with aganglionosis seems significant enough to warrant obtaining biopsy results prior to 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.

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.

Preoperative Details

Basic preoperative laboratory measurements for all neonates should include a CBC count, electrolyte assessment, and a crossmatch. If time permits, a contrast enema can be quite useful in assessing the colon and excluding any other narrowings in that area, which can sometimes be difficult intraoperatively. 

A rectal biopsy should be performed if possible to eliminate the slight chance of Hirschsprung disease, but all care should be taken to ensure that it does not delay prompt operative intervention. 

Intraoperative Details

Primary repair in one stage

• Laparotomy: A transverse supraumbilical laparotomy is usually performed. The abdomen is eviscerated, and the bowel is inspected. The atresia should be readily visible by the dilated bulbous proximal portion and a microcolon on the other side. A large mesenteric or intestinal gap may be observed between the proximal and distal ends, reflective of the region that would have been supplied by the vessel that had the prenatal vascular accident, which hypothetically caused the atresia. 
• Full exploration: The abdomen must now be fully explored. The full intestinal length must be 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 the lumen is patent. The presence of multiple atresias may require multiple resections and should be assessed together to minimize intestinal loss and the number of anastomoses.
• Proximal segment: In preparing to put the two ends of the colon together, the dilated proximal portion must be resected. The dilated proximal bowel functions poorly due to dysmotility. Resecting the proximal intestine back to an area of normal caliber is essential for postoperative function and lessens the size discrepancy when performing the anastomosis.
• Distal segment: Minimal distal bowel is removed, although the atretic end is thickened and should not be used in closure. That end is resected, and the distal colon is usually divided on a bias, removing more bowel on the antimesenteric side than 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.
• Mesenteric gap: If present, the mesenteric gap is usually closed primarily, if possible.

Diversion with primary repair at a second stage 

The operation is much the same as for primary repair above. 

• Proximal segment: The proximal end of the atresia is again resected back to a normal-caliber bowel. This end is then brought out onto the anterior abdominal wall and matured as an ostomy.
• Distal segment: If the patient is critically ill, the distal segment may be left completely undisturbed until the patient's status changes in the future. For stable patients, the distal end of the atresia should have its end removed, and the fresh end should be brought up to the abdominal wall skin as a mucous fistula.
• Ostomy location and maturation: Both ostomies can be brought out at the same location, which may be at the corner of the laparotomy incision or may be through a separate incision. They may also be separated by a skin bridge; however, that 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.
• Mucous fistula: The mucous fistula should not be matured like a mushroom but should be flush with the skin and should only be large enough to be able to admit a catheter for irrigations 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 the surgeon prefers a skin bridge between the ostomy and mucous fistula, it should be either small enough to be directly covered by the ostomy appliance wafer or should be far enough away as to not interfere with ostomy appliance placement.

Postoperative Details

The neonate who has undergone surgical correction of atresia or stenosis requires 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. This requires significant hydration 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.

All children who undergo laparotomy need adequate analgesia. Narcotics should only be administered to babies in a monitored setting (ie, a neonatal intensive care unit with apnea, pulse-oximetry, and cardiac monitors). No baby should be prevented from getting adequate analgesia simply because they cannot articulate the need for it.

Antibiotics are usually administered to all patients, although the indications are not clearly evidence based. Within the first 3 days of life, the intestinal flora is not yet colonized with anaerobes, so gram-positive and gram-negative coverage is sufficient at that time. 

Early parenteral nutrition should be started 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 intravenous 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 prior to removal as a final test to see if the child can truly tolerate their own intestinal gases and secretions.

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

Patients who have undergone a colostomy require specific care for the stoma and its output. Return of bowel function in these patients is usually faster after colostomy 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.

Follow-up

Follow-up imaging studies are not performed unless indicated by clinical issues. Children who have had a stoma created usually undergo contrast enema prior to stomal closure to evaluate the distal limb 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 distal segments. Close follow-up is essential to ensure that they are appropriately managed.

COMPLICATIONS

Section 8 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Complications of surgery for colonic atresia and stenosis are those of any bowel resection with stoma creation or anastomosis. Wound infection or incisional hernia can occur after any surgery. 

The complication surgeons are most concerned about is anastomotic leak. 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 the patient may rapidly progress from mild illness into full-blown septic shock. Radiography may reveal intra-abdominal free air. CT scanning could reveal abscesses. Anastomotic leak requires exploration and diverting enterostomy.

Intra-abdominal abscesses may occur without leak but are rare. Intraoperative bleeding that leads to postoperative clot can be a setup for abscess, which may be amenable to drainage by 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.

OUTCOME AND PROGNOSIS

Section 9 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Survival of patients with colonic atresia and stenosis is related to the patient's condition before surgery, technical difficulties with colonic anastomosis, sepsis, and associated anomalies.^{44, 11}

Whereas older series reported high mortality rates for colonic atresia, modern series report survival of all patients, except those with significant life-threatening comorbidities.

Patients with Hirschsprung disease and colonic atresia have more complicated courses and a mortality rate of 10%.²⁹ 

Colonic stenosis outcomes have also improved significantly since Gross reported the death of the single patient treated at the Boston Children's Hospital before 1952.¹⁰ Improvement in resuscitation and perioperative care presently results in survival rates of approximately 90%.^{45, 1}

Outcomes of Surgery in Colonic Atresia and Stenosis

*Ostomy, resection and staged anastomosis months later
^† Resection with primary anastomosis
^‡Cecotomy, resection of diaphragm

FUTURE AND CONTROVERSIES

Section 10 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Summary

Colonic atresia and congenital stenosis are uncommon lesions. Infants present with abdominal distension and vomiting and often fail to pass meconium. Associated anomalies are common, and their severity directly affects outcome. The combination of Hirschsprung disease and colonic atresia remains rare; however, missing the association prior to reconnecting the intestinal tract can lead to reoperations, poor outcome, and increased mortality. 

Surgical correction is the mainstay of therapy for atresia and stenosis. In the absence of significant comorbidity, primary resection and anastomosis is the recommended surgical treatment and appears to have an equivalent outcome to resection with creation of a colostomy and subsequent closure.

Controversies

The question of the necessity for routine rectal biopsy prior to establishing intestinal continuity is not so much controversial as it is difficult to definitively answer. The small number of cases that associate Hirschsprung disease with colonic atresia is such that the association is greater than random but still exceedingly rare. The downside to taking a staged approach if Hirschsprung disease is a possibility is minimal when compared with the risk of multiple operations and increased mortality when the association is missed prior to anastomosis. Knowing both sides of this issue allows the surgeon to choose the approach that best serves their patient.

ACKNOWLEDGMENTS

Section 11 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

The authors would like to acknowledge Richard Glick, MD, for the images of his patient with distal transverse colonic atresia.

MULTIMEDIA

Section 12 of 13  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

Media file 1:  Colonic atresia, type 1 (sigmoid colon). The dilated colon abruptly tapers to unused distal segment.
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Media file 2:  Contrast enema showing microcolon with dilated proximal colon (colonic atresia of sigmoid colon).
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Media type:  Radiograph

Media file 3:  Contrast enema revealing colonic stenosis at the hepatic flexure.
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Media type:  Radiograph

Media file 4:  Abdominal radiograph of a baby with colonic atresia at the distal transverse colon. The colon proximal to the atresia is visible as a large dilated loop of intestine running obliquely across the abdomen.
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Media type:  Radiograph

Media file 5:  This intraoperative photograph displays the proximal dilated segment in a patient with type 3 atresia of the distal transverse colon.
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Media type:  Photo

Media file 6:  The small portion of bowel held on the right looks like an appendix but is actually the distal segment for the patient in the previous image, with type 3 atresia at the distal transverse colon.
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Media type:  Photo

REFERENCES

Section 13 of 13

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• Acknowledgments
• Multimedia
• References

1. Oldham KT. Atresia, stenosis and other obstructions of the colon. In: O' Neill JA, Jr, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, eds. Pediatric Surgery. St. Louis, Mo: Mosby; 1998:1361-68.
2. Gaub OC. Congenital stenosis and atresia of the intestinal tract above the rectum; with a report of an operated case of atresia of the sigmoid in an infant. Tr Am S A. 1922;40:582.
3. Potts WJ. Congenital atresia of the intestine and colon. Surg Gynecol Obstet. 1947;85:14.
4. Evans CH. Collective review: atresias of the gastrointestinal tract. Surg Gynecol Obstet. 1955;92:1-8.
5. Davenport M, Bianchi A, Doig CM, Gough DC. Colonic atresia: current results of treatment. J R Coll Surg Edinb. Feb 1990;35(1):25-8. [Medline].
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Article Last Updated: Apr 28, 2008