Etiology

Congenital duodenal obstruction can occur due to an intrinsic or extrinsic lesion.¹² The most common cause of duodenal obstruction is atresia.⁷ This intrinsic lesion is most commonly believed to be caused by a failure of recanalization of the fetal duodenum resulting in complete obstruction. Early in the fourth week of gestation, the duodenum begins to develop from the distal foregut and the proximal midgut. During the fifth and sixth weeks of gestation, the duodenal lumen temporarily obliterates due to proliferation of its epithelial cells. Vacuolation due to degeneration of the epithelial cells during the 11th week of gestation then leads to duodenal recanalization.¹³ An embryologic insult during this period can lead to an intrinsic web, atresia, or stenosis. The extrinsic form of duodenal obstruction is due to defects in the development of neighboring structures such as the pancreas, a preduodenal portal vein, or malrotation and Ladd’s bands.^14,15

Annular pancreas as an etiology for duodenal obstruction warrants special mention as this form of obstruction is likely due to failure of duodenal development rather than a true constricting lesion. Thus, the presence of an annular pancreas is simply a visible indication of an underlying atresia or stenosis.¹⁶ Between the fourth and eighth week of gestation, the pancreatic buds merge. In annular pancreas, the tip of the ventral pancreas becomes fixed to the duodenal wall forming a nondistensible, ring-like or annular portion of pancreatic tissue surrounding the descending part of the duodenum.¹³ In annular pancreas associated with duodenal obstruction, the distal biliary tree is often abnormal and may open proximal or distal to the atresia or stenosis.^17,18 Other reported biliary abnormalities associated with duodenal obstruction include biliary atresia, gallbladder agenesis, stenosis of the common bile duct, choledochal cyst, and immune deficiency.^19–24

Classification

Anatomically, duodenal obstructions are classified as either atresias or stenoses. An incomplete obstruction, due to a fenestrated web or diaphragm, is considered a stenosis. Most stenoses involve the third and/or fourth part of the duodenum. Atresias, or complete obstruction, are further classified into three morphologic types (Fig. 30-1). Type I atresias account for more than 90% of all duodenal obstructions and contain a lumenal diaphragm that includes mucosal and submucosal layers. A diaphragm that has ballooned distally (windsock) is a type I atresia.^25,26 It is important to understand that the anatomy of the windsock may lead to a portion of the dilated duodenum actually being distal to the actual obstruction (Fig. 30-2). Type II atresias are characterized by a dilated proximal and collapsed distal segment connected by a fibrous cord. Type III atresias have an obvious gap separating the proximal and distal duodenal segments.²⁷

More than 50% of affected patients with duodenal atresia have associated congenital anomalies.²⁸ Approximately 30% are associated with trisomy 21, 30% with isolated cardiac defects, and 25% with other gastrointestinal (GI) anomalies.^29,30 Approximately 45% of patients are premature, and about one-third exhibit growth retardation.^6,7

Pathology

The obstruction can be classified as either preampullary or postampullary, with approximately 85% of obstructions located distal to the ampulla.³⁰ With complete or almost complete obstruction, the stomach and proximal duodenum become significantly dilated. The pylorus is usually distended and hypertrophic. The bowel distal to the obstruction is collapsed, except in the case of a windsock deformity in which the distal bowel is dilated to a variable length depending on the length of the windsock (see Fig. 30-2). In most cases of duodenal obstruction, the gastrointestinal tract can be decompressed proximally. With complete obstruction of the duodenum, the incidence of polyhydramnios ranges from 32–81%.^1,31–34 Growth retardation is also common, presumably from nutritional deprivation from the swallowed amniotic fluid.

Diagnosis

There are multiple benefits to the antenatal diagnosis of duodenal obstruction, including parental counseling. The diagnosis can often be suggested by prenatal ultrasound (US). Sonographic evaluation in fetuses of mothers with a history of polyhydramnios can detect two fluid-filled structures consistent with a double bubble in up to 44% of cases.35–37 Despite duodenal obstruction usually occurring by week 12, the reason for failure of early prenatal detection is not entirely clear. Most cases of duodenal atresia are detected between 7 and 8 months gestation.³⁸ It is currently believed that immature gastric emptying in-utero may contribute to low gastric pressures, failing to dilate the proximal duodenum until later in gestation. While both circular and longitudinal muscle layers are present in the stomach by week 8 of gestation, pressure amplitudes at 25 weeks are only 60% of term gastric pressures.^39,40

The presentation of the neonate with duodenal obstruction varies depending on whether the obstruction is complete or incomplete, and the location of the ampulla of Vater in relation to the obstruction. The classic presentation is that of bilious emesis within the first hours of life in an otherwise stable neonate. In about 10% of cases, however, the atresia is pre-ampullary and the emesis is nonbilious.¹⁵ Abdominal distention may or may not be present. In neonates with duodenal atresia, the abdomen is scaphoid. Aspiration via a nasogastric (NG) tube of more than 20 mL of gastric contents in a newborn suggests intestinal obstruction, as normal aspirate is less than 5 mL.⁴¹ For patients with stenosis, the diagnosis is often delayed until the neonate has started on enteral feeds and feeding intolerance develops with emesis and gastric distention.

In antenatally suspected cases of duodenal obstruction, as well as in neonates with a clinical presentation consistent with a proximal bowel obstruction, an upright abdominal radiograph is usually sufficient to confirm the diagnosis of duodenal atresia. The diagnostic radiographic presentation of duodenal atresia is that of a double bubble sign with no distal bowel gas (Fig. 30-3). The proximal left-sided bubble represents the air and fluid-filled stomach while the dilated proximal duodenum represents the second bubble to the right of midline.⁴² In almost all cases of duodenal atresia, the distal bowel is gasless. However, the presence of distal gas does not necessarily exclude the diagnosis of atresia as there are reports of bifed common bile ducts with insertion of one of the ducts proximal and the other distal to the atretic segment which allows the air to bypass the atresia.⁴³ In neonates whose stomach has been decompressed by either NG aspiration or vomiting, 40–60 mL of instilled air into the stomach will reproduce the double bubble.²⁷ Rarely, the biliary tree is air filled, and a variety of pancreatic and biliary anomalies have been demonstrated (Fig. 30-4).⁴³ At our institution, neonates who present with bilious emesis and a decompressed stomach on plain abdominal films receive a limited upper GI contrast study to exclude malrotation and volvulus. With duodenal stenosis, a double bubble sign is often not present and the diagnosis is usually made with a contrast study (Fig. 30-5).

Management

After the diagnosis is made, appropriate resuscitation is required with correction of fluid balance and electrolyte abnormalities, in addition to gastric decompression. At our institution, all neonates diagnosed with duodenal obstruction receive a complete metabolic profile, complete blood count, coagulation studies, an abdominal and spinal ultrasound, and two-dimensional echocardiography prior to any operation. An emergency operation is only performed in cases where malrotation with concurrent volvulus cannot be excluded.

Prior to the mid-1970s, duodenojejunostomy was the preferred technique for correcting duodenal atresia or stenosis.7,44,45 Since then, the various techniques utilized include side-to-side duodenoduodenostomy, diamond-shaped duodenoduodenostomy, partial web resection with Heineke–Mickulicz-type duodenoplasty, and tapering duodenoplasty.^44–46 The long side-to-side duodenoduodenostomy, although effective, is associated with a high incidence of anastomotic dysfunction and prolonged obstruction.³⁰ Blind-loop syndrome appears to be more common in patients treated with duodenojejunostomy.⁴⁷ Gastrojejunostomy should not be performed as it is associated with a high incidence of marginal ulceration and bleeding.²⁷

Currently, the preferred technique is either laparoscopic or open duodenoduodenostomy.9–11,30 Originally, a side-to-side anastomosis was performed. A proximal transverse to distal longitudinal (diamond-shaped) anastomosis is now preferred.^{7,44,45,48–50} For the open approach, either a right upper quadrant supraumbilical transverse incision or an umbilical crease incision is utilized.⁴⁹ After mobilizing the ascending and transverse colon to the left, the duodenal obstruction is readily exposed. Malrotation should be evaluated at this point as it can occur in association with congenital duodenal obstruction in up to 30% of patients.¹ A sufficient length of duodenum distal to the atresia is mobilized to allow for a tension-free anastomosis. A transverse duodenotomy is made in the anterior wall of the distal portion of the dilated proximal duodenum and a similar length duodenotomy is made in a vertical orientation on the antimesenteric border of the distal duodenum. The anastomosis is then fashioned by approximating the end of each incision to the appropriate mid-portion of the other incision (Fig. 30-6). Tapering duodenoplasty is generally not necessary as the proximal duodenal dilation usually resolves after relief of the obstruction. Muscular continuity of the duodenal wall suggests a windsock deformity or diaphragm. This finding should precipitate extra vigilance in the operative correction because the dilated and collapsed bowel are both distal to the windsock, and have been anastomosed in error.^26,51

The laparoscopic approach was first described by Rothenberg in 2002.⁹ The standard laparoscopic approach begins with the patient supine and the abdomen is insufflated through the umbilicus. Two other instruments are inserted, one in the baby’s right lower quadrant and one in the right mid-epigastric region, respectively. A liver retractor can be placed in the right or left upper quadrant if necessary. Alternatively, the liver can be elevated by placing a transabdominal wall suture around the falciform ligament and tying it outside the abdomen (Fig. 30-7). The duodenum is mobilized and the location of obstruction is identified. Using the same principles that have been described for the open approach, a standard diamond-shaped anastomosis is created. Although some surgeons will perform the laparoscopic anastomosis with interrupted sutures, this can be technically demanding because of the significant number of sutures required and the thin nature of the distal duodenum. We reported our results using Nitinol U-clips (Medtronic, Minneapolis, MN) to create the duodenoduodenostomy with no leaks and more rapid initiation of feeds when compared to the traditional open approach (Fig. 30-8).^10,11 Although the U-clips are no longer commercially available, this study highlighted the advantages of early feeding. The historical approach to enteral feeding following duodenal atresia repair involved a period of waiting for the gastric output to become less bilious and the volume of gastric drainage to decrease, indicating return of intestinal function. This report showed that the time spent waiting for the gastric output to decrease is likely not necessary as all of the patients undergong the laparoscopic duodenoplasty had initiation of feeds without adverse events after an upper gastrointestinal contrast study on day 5 revealed no leak. When compared to infants undergoing an open operation with the historical postoperative management mentioned previously, there was a marked reduction in hospitalization for the laparoscopically corrected infants, primarily due to the early feeding.

Historically, during repair of duodenal atresia, it has been emphasized that inspecting the entire small bowel to identify a second atresia is important. Given that duodenal atresia and jejunoileal atresia do not share common embryologic etiologies, a multi-institutional review of duodenal atresia patients was undertaken to quantify the incidence of jejunoileal atresia in this population.⁵² In the largest series to date, the rate of concomitant jejunoileal atresia in patients with duodenal atresia was less than 1%. With the low incidence of a concomitant distal atresia, extensive inspection of the entire bowel does not appear necessary.

Early postoperative mortality for duodenal atresia repair has been reported to be as low as 3–5% with the majority of deaths occurring secondary to complications related to associated congenital abnormalities.53,54 Long-term survival approaches 90%.^7,53–55 Long-term complications have been noted following repair and include delayed gastric emptying, severe gastroesophageal reflux, bleeding peptic ulcer, megaduodenum, duodenogastric reflux, gastritis, blind-loop syndrome and intestinal obstruction related to adhesions.³⁰

Etiology

Jejunoileal atresia occurs in approximately 1 in 5,000 live births. It occurs equally in males and females, and about one in three infants is premature.⁵⁶ Although the majority of cases are thought to occur sporadically, familial cases of intestinal atresias have been described.⁵⁷ It is generally accepted that jejunoileal atresia occurs as a result of an intrauterine ischemic insult to the midgut, affecting single or multiple segments of the already developed intestine.^13,58–61 Intrauterine vascular disruption can lead to ischemic necrosis of the bowel with subsequent resorption of the affected segment or segments (Fig. 30-9).

The hypothesis that most cases of jejunoileal atresia occur secondary to vascular disruption during fetal life is derived from experimental as well as clinical evidence. Isolated mesenteric vascular insults and interference with the segmental blood supply to the small intestine were created in fetal dogs, and resulted in different degrees and patterns of intraluminal obstruction, reproducing the spectrum of stenosis and atresia found in humans.62–64 Moreover, the presence of bile, lanugo hair, and squamous epithelial cells from swallowed amniotic fluid distal to an atresia suggests that the atresia occurs subsequent to some event, but that at some time in gestation the intestinal lumen was patent, thus allowing passage of these contents. Additionally, atresias seen in association with other intrauterine vascular insults such as fetal intussusception, midgut volvulus, thromboembolic occlusions, transmesenteric internal hernias, and incarceration or snaring of bowel in an omphalocele or gastroschisis have contributed to wide acceptance of this hypothesis.^58,64–68

The presence of associated extra-abdominal organ abnormalities in jejunoileal atresia is low (<10%) due to its occurrence later in fetal life and the localized nature of the vascular insult.⁶⁹ Rarely, jejunoileal atresia has been found in patients with Hirschsprung disease, cystic fibrosis, malrotation, Down syndrome, anorectal and vertebral anomalies, neural tube defects, congenital heart disease, and other GI atresias.^56,69,70 Methylene blue, previously used for amniocentesis in twin pregnancies, has been implicated in causing small bowel atresia.⁷¹

Although jejunoileal atresias are usually not hereditary, there is a well-documented autosomal recessive pattern of inheritance of multiple atresias.⁷² In these cases, intestinal rotation was normal, mesenteric defects were never observed, and lanugo hairs and squamous cells were not identified distal to the most proximal atresia. All these findings suggest an early intrauterine event. Survival is poor in these infants, even with successful bowel resection.

No correlations have been found between jejunoileal atresia and parental or maternal disease. However, the use of maternal vasoconstrictive medications, as well as maternal cigarette smoking in the first trimester of pregnancy, has been shown to increase the risk of small bowel atresia.⁷³ Chromosomal abnormalities are seen in less than 1% of the patients with jejunoileal atresia.⁷⁴

Pathology

The Grosfeld classification system separates these defects into four groups, with an additional consideration for type III(b) (Fig. 30-10).⁴ This classification has significant prognostic and therapeutic value as it emphasizes the importance of associated loss of intestinal length, abnormal collateral intestinal blood supply, and concomitant atresia or stenosis.⁷⁵ Regarding classification, the most proximal atresia determines whether the atresia is classified as jejunal or ileal atresia. Multiple atresias are found in up to 30% of patients.^56,76

Pathophysiology

The vascular and subsequent ischemic insult not only causes morphologic abnormalities but also adversely influences the structure and subsequent function of the remaining proximal and distal bowel.58,59,91 The blind-ended proximal bowel is dilated and hypertrophied with histologically normal villi, but without effective peristaltic activity. A deficiency of mucosal enzymes and muscular adenosine triphosphatase has also been found.⁹² At the level of the atresia, the ganglia of the enteric nervous system are atrophic with minimal acetylcholinesterase activity. These changes are most likely secondary to local ischemia. Obstruction alone can elicit similar, but less severe, morphologic and functional abnormalities.⁹²

Experimental studies showing that the intestinal atresia results from ischemic necrosis of the intestine also imply that there is a precarious blood supply to the proximally dilated bowel. This has been confirmed with postmortem injection of barium sulfate into the mesenteric vessels.58,59,85 However, it has also been postulated that the intestine is not ischemic at birth, but rather becomes so only with swallowing air. Distention and increased intraluminal pressure or torsion can then occur. The good results obtained with tapering procedures without resection of the bulbous portion would support the contention that the blood and nerve supply to the bowel adjacent to the atresia is normal.⁵⁸ However, this ischemic insult may interfere with mucosal and neural function. Defective peristalsis is commonly noticed in the atretic area, thus supporting resection of the dilated bulbous proximal end for better function.⁹³ Because the proximal end of the distal atretic bowel has been subjected to a similar insult, a small portion of it should be resected at the time of operative correction as well.

Clinical Manifestations

Prompt recognition of intestinal obstruction in the neonate is paramount due to the possibility of midgut volvulus or an internal hernia with subsequent ischemia. Although prenatal ultrasound is more reliable at detecting duodenal atresia, in recent years it has become useful in diagnosing jejunoileal atresia as well. The ultrasound findings include dilated loops of bowel and polyhydramnios, which may not be present early in gestation or only with very distal obstructions. A fetus with these abnormal findings should elicit a search for familial GI abnormalities as well as referral for prenatal evaluation. The vast majority of patients with jejunoileal atresia will not be diagnosed prenatally.

In neonates with atresia or stenosis, the presenting symptoms are consistent with bowel obstruction, including bilious emesis and abdominal distention. Although the meconium may appear normal, it is more common to see gray plugs of mucus passed via the rectum. Occasionally, if the distal bowel in type III(b) atresia is ischemic, blood may be passed through the rectum.

Intestinal stenosis is more likely to create diagnostic difficulty when compared to intestinal atresia. Intermittent partial obstruction or malabsorption may improve without treatment. Clinical investigations can initially be normal. However, these babies usually develop failure to thrive and ultimately progress to complete intestinal obstruction and require exploration.

Diagnosis

The diagnosis of jejunoileal atresia can usually be made by radiographic examination of the abdomen using swallowed air as contrast. Swallowed air reaches the proximal bowel by one hour and the distal small bowel by three hours in a normal vigorous infant in whom its passage is blocked, but this pattern may be delayed in premature or sick infants with poor sucking.94,95 Jejunal atresia patients can have a few gas-filled and fluid-filled loops of small bowel, but the remainder of the abdomen is gasless (Fig. 30-14). When the atresia is associated with cystic fibrosis, fewer air-fluid levels are evident, and the typical ground-glass appearance of inspissated meconium is seen. A limited-contrast study may be useful if intestinal stenosis is suspected.

As haustral markings are rarely seen in neonates, distal ileal atresia may be difficult to differentiate from colonic atresia (Fig. 30-15). A contrast enema will reveal an unused appearance to the colon. Reliance on intraoperative injection of saline into the large bowel to confirm distal bowel patency may fail to identify an associated colonic or rectal atresia.96,97 If the small bowel atresia occurred late in gestation, the bowel distal to the atresia may have a more normal caliber. Occasionally, air and meconium can accumulate proximal to an atresia, mimicking the radiologic appearance of meconium ileus. Additionally, total colonic aganglionosis may be difficult to differentiate from small bowel atresia.

Ten per cent of babies with jejunoileal atresia present with meconium peritonitis.⁴ The intestinal perforation usually occurs proximal to the obstruction, near the bulbous blind end. The radiologic appearance of a meconium pseudocyst containing a large air/fluid level is related to the late intrauterine bowel perforation. Intraluminal calcification of meconium or intramural calcification in the form of diffuse punctate or rounded aggregations have been reported with intestinal stenosis or atresia.⁹⁸ Meconium calcification in patients with hereditary FMIA produces a ‘string of pearls,’ which is pathognomonic of this condition.^72,88

The clinical and radiologic picture of jejunoileal stenosis is determined by the level and degree of stenosis, and the diagnosis may be delayed for years. Morphologic and functional changes in the proximal obstructed intestine vary depending on the degree of obstruction.

Differential Diagnosis

Diseases that mimic jejunoileal atresia include colonic atresia, midgut volvulus, meconium ileus, duplication cysts, internal hernias, ileus due to sepsis, birth trauma, maternal medications, prematurity, and hypothyroidism.4,99,100 Special investigations including an upper GI contrast study, contrast enema, rectal biopsy, and a delta F508 gene deletion assay or sweat test to exclude associated cystic fibrosis may be needed.^99,101

Management

Delay in diagnosis may lead to impairment of intestinal viability (50%), frank necrosis and perforation (10–20%), fluid and electrolyte abnormalities, and sepsis. Preoperative management should include gastric decompression and fluid resuscitation to correct electrolyte abnormalities and hypovolemia. Antibiotics should be initiated if there is suspicion for perforation or infection.

Surgical Considerations

The operative management of intestinal atresias is based on the location of the lesion, anatomic findings, associated conditions noted at operation, and the length of the remaining instestine.⁵⁶ Resection of the dilated and hypertrophied proximal bowel (see Fig. 30-12B), with primary end-to-end anastomosis with or without tapering of the proximal bowel, is the most common technique.^{4, 77,100}

As recently as the 1950s, the surgical mortality for newborns with intestinal atresia was 80% to 90%.56,99 This high mortality rate was mostly related to late presentation and dysmotility of the proximal dilated bowel which led to complications related to chronic obstruction and inanition. Fortunately, the current survival rate is greater than 90%.⁵⁶ The understanding that the proximal bowel is dysfunctional, improvements in the anastomotic technique and suture material, and the development of total parenteral nutrition (TPN) are the primary reasons for this significantly improved survival in recent years. Currently, only infants with severe associated congenital abnormalities or short bowel syndrome should not have a good prognosis.

Operative Considerations

The repair of small intestinal atresia can be undertaken via several approaches. One option is to evaluate using a laparoscopic approach, with subsequent resection and anastomosis performed in an extra-corporeal fashion. Although this approach seems attractive, it can be difficult to identify the atresia due to the markedly dilated small intestine and the small working space of the neonate’s abdominal cavity. To overcome these limitations, we explore the abdomen through the umbilicus. With this technique, the umbilical skin is incised and the fascia is opened vertically in the midline to the extent allowed by the umbilical skin incision. The small intestine can be exteriorized relatively easy through the umbilical incision (see Figs 30-12C and 30-13B). In a retrospective report, a circumumbilical incision for neonatal surgery was found to as effective as the transverse abdominal incision with less morbidity and better cosmetic results.¹⁰² The traditional transverse supra- or infraumbilical incision is also appropriate. Regardless of the approach, access to the entire intestine and peritoneal cavity is necessary. Careful inspection of the entire bowel is performed and the site and type of obstruction should be noted as well as any other abnormalities. In addition, the length of bowel should be assessed. The most distal limb of the atretic bowel can then be cannulated with a red rubber catheter and irrigated with warm saline to evaluate for distal obstruction. Continuity of the colon can be established preoperatively by a contrast enema or with a prepositioned transrectal catheter placed prior to prepping.¹⁰³ Failure to adequately evaluate for distal obstruction or stenosis can lead to postoperative complications, including an anastomotic leak. If present, malrotation should be corrected with a Ladd procedure. Because the length of functional bowel has important prognostic significance, and determines the most appropriate method of repair, the length of functional bowel should be carefully measured along the antimesenteric border and documented in the operative report.

Delayed intestinal function in the blind proximal atretic segment as well as functional obstruction after performance of a side-to-side anastomosis without resection of the dilated proximal atretic bowel have been described.¹⁰⁰ Therefore, if the length of functional bowel is adequate, the bulbous hypertrophied proximal bowel should be resected to approximately normal caliber bowel. Ultimately, the goal is to restore bowel continuity while maintaining both intestinal function and length. Intestinal imbrication has also been shown to be an effective method to reduce the caliber of the dilated bowel while maintaining mucosal absorptive surface.¹⁰⁴ Regarding the distal segment, a short length (4–5 cm) of bowel is obliquely resected, leaving the mesenteric side longer than the antimesenteric aspect. An incision along the antimesenteric border to create a ‘fish mouth’ may be needed to create an adequate distal enterotomy for the anastomosis.

Although there are multiple techniques for the anastomosis, we generally perform a one-layer modification of the end-to-back technique using 5-0 or 6-0 sutures. Once the anastomosis is completed, the suture line is tested for leaks, and reinforcing sutures are placed as needed. The mesenteric defect is repaired with careful attention to avoid rotation or kinking of the anastomosis, or injury to the blood supply. A temporary enterostomy should be performed if there is a question of bowel viability.⁵⁶ However, neither decompressive gastrostomy nor transanastomotic stents are usually needed.^105,106

Similar techniques are used for stenosis and jejunoileal membranes. Procedures such as transverse enteroplasty, excision of the membrane, and bypassing techniques are not recommended primarily because they fail to remove the abnormal segments of bowel, and may produce blind-loop syndromes.

Prognostic Factors

The normal small bowel length in term neonates is approximately 250 cm. In preterm infants, it ranges from 160–240 cm. With the development of TPN, special enteral diets and pharmacologic management of short gut syndrome, previous estimates that a small bowel length of 100 cm or more is necessary to sustain oral intake and survival may no longer be applicable. Preservation of bowel length at the expense of a poorly functioning anastomosis should be avoided.

If proximal resection will lead to significant, or unacceptable bowel loss, tapering or plication of the dilated bowel is a useful technique.104,107 Tapering enteroplasty as far proximal as the second portion of the duodenum can be accomplished by resecting an antimesenteric strip of the dilated proximal bowel.¹⁰⁸ During tapering duodenojejunoplasty, particularly with type III atresias, the duodenum is de-rotated, thus allowing a direct caudal descent from the stomach which decreases the risk for obstruction. Additionally, the mesentery should be maximally opened, while meticulously protecting the small bowel blood supply. During this process, the cecum can be mobilized to the left which results in a broader mesentery and also allows the anastomosis to lie in manner that will help avoid kinking.¹⁰⁹ The tapering can be safely performed up to 35 cm.¹⁰⁷ The tapered bowel may then be anastomosed to the distal bowel or exteriorized as a stoma.

A primary anastomosis may be contraindicated in cases of peritonitis, volvulus with vascular compromise, meconium ileus, or type III(b) atresia.110,111 Under these circumstances, exteriorization of both ends of the atresia may be needed.

Intestinal atresia encountered in a baby with gastroschisis may be single or multiple, and may be located in either the small or large bowel. In a series from our institution, 12.6% of 199 patients with gastroschisis had an associated atresia.¹¹² The most common location for the atresia was jejunoileal and most were type III(a). Our current management algorithm for patients with gastroschisis and atresia is to first assess the extent of reactive change (peel) on the intestine. If there is minimal peel, primary anastomosis may be an option. This is rare and should be considered only in the most optimal situations. In nearly all instances, the atresia should be left undisturbed at the initial operation. After fascial closure is accomplished, management should include gastric decompression and TPN support with subsequent atresia repair four to six weeks later.

With type III(b) atresia, restricting bands along the free edge of the distal coiled and narrow mesentery should be divided to optimize the blood supply. The bowel should be returned to the abdomen with careful inspection of the mesentery to prevent torsing the single marginal artery and vein. In cases of questionable intestinal viability, improved long-term results have been achieved with resection and tapering of the dilated proximal bowel with limited resection of the distal bowel.113,114

Bowel-length conservation methods, such as multiple anastomoses for multiple atresias, may result in increased morbidity. A silicone (Silastic) catheter stent can be used with multiple primary anastomoses and serves as a conduit for radiologic evidence of anastomotic integrity, luminal patency, and enteral feeding.¹¹⁵ If multiple atresias are grouped closely together and there is adequate bowel length, a single resection and anastomosis can be performed.

No attempt at any bowel lengthening procedures should be entertained at the initial operation. However, such procedures may ultimately obviate the need for prolonged TPN in patients with short gut syndrome.

Postoperative Care

Parenteral nutrition is mandatory and should begin as soon as possible, and should continue until the infant is tolerating full enteral feeds.

Enteral feedings can be initiated when the gastric aspirate is clear, output is minimal, and the infant is stooling. At our institution, enteral feeding is usually started through a feeding tube at a rate of 20 mL/kg/day of breast milk or formula in a continuous fashion. The feeds are increased by 20–30 mL/kg/day. Oral intake is started when the baby is alert, able to suck, and tolerating at least 8 mL of tube feeds per hour.

Transient GI dysfunction is frequently observed in infants with jejunal and ileal atresia, and its etiology is multifactorial.4,116 Lactose intolerance, malabsorption (owing to stasis with bacterial overgrowth), and diarrhea may be significant in infants who have undergone repair of type III(b) atresia, or in those with short bowel syndrome after surgery for multiple atresias. Regular monitoring for clinical signs of intestinal overload or intolerance is required. Water-loss stools, increasing frequency of stooling, hematochezia, fecal-reducing substances, or a decreased stool pH warrant biochemical evaluation of the stool for disaccharide or monosaccharide intolerance.¹¹⁷ Unintentional injury to the mucosa can be caused by sugars, high-osmolarity feeds, oral medications, and bacterial or viral infections. Pharmacologic control of altered GI function may hasten adaptation. Loperamide hydrochloride decreases intestinal peristaltic activity and cholestyramine is effective in binding bile salts.^117,118 Cholestyramine should not be given unless water-loss stools are evident. Vitamin B12 and folic acid should be given regularly to the patient without a terminal ileum to prevent megaloblastic anemia.

Functional outcome ultimately depends on the following factors: (1) the location of the atresia (the ileum adapts to a greater degree than the jejunum); (2) the maturity of the intestine (the small intestine in a premature infant still has time for maturation and growth); and (3) the length of the small intestine, which can be difficult to determine accurately after birth.¹¹⁹ The ileocecal valve is critically important as it allows for more rapid intestinal adaptation when the residual small bowel length is short.

1. Irving, IM. Duodenal atresia and stenosis: Annular pancreas. In: Lister J, Irving IM, eds. Neonatal Surgery. 3rd ed. London: Buttersworths; 1990:424.

2. Adeyemi, D. Neonatal intestinal obstruction in a developing tropical country: Patterns, problems, and prognosis. J Trop Pediatr. 1989; 35:66–70.

3. Cywes, S, Davies, MRQ, Rode, H. Congenital jejuno-ileal atresia and stenosis. S Afr Med J. 1980; 57:630–639.

4. Grosfeld, JL. Jejunoileal atresia and stenosis, section 3: The small intestine. In: Ravitch MM, Welch KJ, Benson CD, et al, eds. Pediatric Surgery. Chicago: Year Book Medical; 1986:838.

5. Kimura, K, Loening-Baucke, V. Bilious vomiting in the newborn: Rapid diagnosis of intestinal obstruction. Am Fam Physician. 2000; 61:2791–2798.

6. Chhabra, R, Suresh, BR, Weinberg, G, et al. Duodenal atresia presenting as hematemesis in a premature infant with Down syndrome. Case report and review of the literature. J Perinatol. 1992; 12:25–27.

7. Grosfeld, JL, Rescorla, FJ. Duodenal atresia and stenosis: Reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg. 1993; 17:301309.

8. Adzick, NS, Harrison, MR, de Lorimier, AA. Tapering duodenoplasty for megaduodenum associated with duodenal atresia. J Pediatr Surg. 1986; 21:311–312.

9. Rothenberg, SS. Laparoscopic duodenoduodenostomy for duodenal obstruction in infants and children. J Pediatr Surg. 2002; 37:1088–1089.

10. Valusek, PA, Spilde, TL, Tsao, K, et al. Laparoscopic duodenal atresia repair using surgical U-clips: A novel technique. Surg Endosc. 2007; 21:1023–1024.

11. Spilde, TL, St Peter, SD, Keckler, SJ, et al. Open vs. laparoscopic repair of congenital duodenal obstructions: A concurrent series. J Pediatr Surg. 2008; 43:1002–1005.

12. Ladd, WE. Congenital obstruction of the duodenum in children. N Engl J Med. 1931; 206:277–283.

13. Moore, KL, Persaud, TVN. The digestive system. In The Developing Human, 8th ed, Philadelphia: WB Saunders; 2007:218.

14. Schnaufer, L. Duodenal atresia, stenosis and annular pancreas. In: Welch RJ, Randolph JG, et al, eds. Pediatric Surgery. Chicago: Year Book Medical; 1986:929.

15. Shawis, R, Antao, B. Prenatal bowel dilatation and the subsequent postnatal management. Early Hum Dev. 2006; 82:297–303.

16. Elliot, GB, Kliman, R, Elliot, KA. Pancreatic annulus: A sign or a cause of duodenal obstruction? Can J Surg. 1968; 11:357.

17. Gourevitch, A. Duodenal atresia in the newborn. Ann R Coll Surg Engl. 1971; 48:141–158.

18. Jona, JZ, Belin, RP. Duodenal anomalies and the ampulla of Vater. Surg Gynecol Obstet. 1976; 143:565–569.

19. Irving, IM, Rickham, PP. Duodenal atresia and stenosis: Annular pancreas. In: Rickham PP, Lister J, Irving IM, eds. Neonatal Surgery. 2nd ed. Boston: Butterworths; 1978:355.

20. Brereton, RJ, Cudmore, RE, Bouton, JM. Double atresia of the duodenum. Z Kinderchir. 1980; 31:60–65.

21. Coughlin, JP, Rector, FE, Klein, MD. Agenesis of the gallbladder in duodenal atresia: Two case reports. J Pediatr Surg. 1992; 27:1304.

22. Davenport, M, Saxena, R, Howard, E. Acquired biliary atresia. J Pediatr Surg. 1996; 31:1721–1723.

23. Moore, SW, de Jongh, G, Bouic, P, et al. Immune deficiency in familial duodenal atresia. J Pediatr Surg. 1996; 31:1733–1735.

24. Mali, V, Wagener, S, Sharif, K, et al. Foregut atresias and bile duct anomalies: Rare, infrequent or common?!. Pediatr Surg Int. 2007; 23:889–895.

25. Bill, AH, Jr., Pope, WM. Congenital duodenal diaphragm. Surgery. 1954; 35:482–486.

26. Rowe, M, Buckner, D, Clatworthy, HW, Jr. Wind sock web of the duodenum. Am J Surg. 1968; 116:444–449.

27. Magnuson, DK, Schwartz, MZ. Stomach and duodenum. In: Oldham KT, Colombani PM, Foglia RP, et al, eds. Principles and Practice of Pediatric Surgery. Philadelphia: Lippincott Williams & Wilkins, 2004.

28. Kimble, RM, Harding, J, Kolbe, A. Additional congenital anomalies in babies with gut atresia or stenosis: When to investigate, and which investigation. Pediatr Surg Int. 1997; 12:565–570.

29. Mustafawi, AR, Hassan, ME. Congenital duodenal obstruction in children: A decade’s experience. Eur J Pediatr Surg. 2008; 18:93–97.

30. Escobar, MA, Ladd, AP, Grosfeld, JL, et al. Duodenal atresia and stenosis: Long-term follow-up over 30 years. J Pediatr Surg. 2004; 39:867–871.

31. Fonkalsrud, EW, DeLorimier, AA, Hays, DM. Congenital atresia and stenosis of the duodenum: A review compiled from the members of the Surgical Section of the American Academy of Pediatrics. Pediatrics. 1969; 43:79–83.

32. al-Salem, AH, Khwaja, S, Grant, C, et al. Congenital intrinsic duodenal obstruction: Problems in the diagnosis and management. J Pediatr Surg. 1989; 24:1247–1249.

33. Longo, MF, Lynn, HB. Congenital duodenal obstruction: Review of 29 cases encountered in a 30-year period. Mayo Clin Proc. 1967; 42:423–430.

34. Kimble, RM, Harding, JE, Kolbe, A. Does gut atresia cause polyhydramnios? Pediatr Surg Int. 1998; 13:115–117.

35. Stubbs, TM, Horger, EO. Sonographic detection of fetal duodenal atresia [Letter]. Obstet Gynecol. 1989; 73:146.

36. Akhtar, J, Guiney, EJ. Congenital duodenal obstruction. Br J Surg. 1992; 79:133–135.

37. Bittnecourt, DG, Barini, R, Marba, S, et al. Congenital duodenal obstruction: Does prenatal diagnosis improve the outcome? Pediatr Surg Int. 2004; 20:582–585.

38. Lawrence, MJ, Ford, WD, Furness, ME, et al. Congenital duodenal obstruction: Early antenatal ultrasound diagnosis. Pediatr Surg Int. 2000; 16:342–345.

39. Dumont, RC, Rudolph, CD. Development of gastrointestinal motility in the infant and child. Gastroenterol Clin North Am. 1994; 23:655–671.

40. Berseth, CL. Gestational evolution of small intestinal motility in preterm and term infants. J Pediatr. 1989; 115:646–651.

41. Britton, JR, Britton, HL. Gastric aspirate volume at birth as an indicator of congenital intestinal obstruction. Acta Paediatr. 1995; 84:945–946.

42. Traubici, J. The double bubble sign. Radiology. 2001; 220:463–464.

43. Kassner, EG, Sutton, A, De Groot, TJ. Bile duct anomalies associated with duodenal atresia: Paradoxical presence of small bowel gas. Am J Roentgenol Radium Ther Nucl Med. 1972; 116:577–583.

44. Kimura, K, Mukohara, N, Nishijima, E, et al. Diamond-shaped anastomosis for duodenal atresia: An experience with 44 patients over 15 years. J Pediatr Surg. 1990; 25:977–979.

45. Weber, TR, Lewis, JE, Mooney, D, et al. Duodenal atresia: A comparison of techniques of repair. J Pediatr Surg. 1986; 21:1133–1136.

46. Singh, SJ, Dickson, R, Baskaranathan, S, et al. Excision duodenoplasty: A new technique for congenital duodenal obstruction. Pediatr Surg Int. 2002; 18:75–78.

47. Rescorla, FJ, Grosfeld, JL. Duodenal atresia in infancy and childhood: Improved survival and long-term follow-up. Contemp Surg. 1988; 33:22–27.

48. Takayashi, Y, Tajiri, T, Masumoto, K, et al. Umbilical crease incision for duodenal atresia achieves excellent cosmetic results. Pediatr Surg Int. 2010; 26:963–966.

49. Ein, SH, Kim, PC, Miller, HA. The late nonfunctioning duodenal atresia repair-A second look. J Pediatr Surg. 2000; 35:690–691.

50. Kimura, K, Tsugawa, C, Ogawa, K, et al. Diamond-shaped anastomosis for congenital duodenal obstruction. Arch Surg. 1977; 112:1262–1263.

51. Richardson, WR, Martin, LW. Pitfalls in the surgical management of the incomplete duodenal diaphragm. J Pediatr Surg. 1969; 4:303–312.

52. St. Peter, SD, Little, DC, Barsness, KS, et al. Should we be concerned about jejunoileal atresia during repair of duodenal atresia? J Laparoendoscopic Adv Surg Tech. 2010; 20:773–775.

53. Feggetter, S. A review of the long-term results of operations for duodenal atresia. Br J Surg. 1969; 56:68–72.

54. Stauffer, UG, Irving, I. Duodenal atresia and stenosis–long-term results. Prog Pediatr Surg. 1977; 10:49–60.

55. Kokkonen, ML, Kalima, T, Jaaskelainen, J, et al. Duodenal atresia: Late follow-up. J Pediatr Surg. 1988; 23:216–220.

56. Dalla Vecchia, LK, Grosfeld, JL, West, KW, et al. Intestinal atresia and stenosis: A 25-year experience with 277 cases. Arch Surg. 1998; 133:490–496.

57. Kumaran, N, Shankar, KR, Lloyd, DA, et al. Trends in the management and outcome of jejuno-ileal atresia. Eur J Pediatr Surg. 2002; 12:163–167.

58. Louw, JH. Congenital intestinal atresia and stenosis in the newborn. Observations on its pathogenesis and treatment. Ann R Coll Surg Engl. 1959; 25:209–234.

59. Louw, JH, Barnard, CN. Congenital intestinal atresia: Observations on its origin. Lancet. 1955; 269:1065–1067.

60. Abrams, JS. Experimental intestinal atresia. Surgery. 1968; 64:185–191.

61. Puri, P, Fujimoto, T. New observations on the pathogenesis of multiple intestinal atresias. J Pediatr Surg. 1988; 23:221–225.

62. Koga, Y, Hayashida, Y, Ikeda, K, et al. Intestinal atresia in fetal dogs produced by localized ligation of mesenteric vessels. J Pediatr Surg. 1975; 10:949–953.

63. Tibboel, D, van der Kamp, AW, Molenaar, JC. An experimental study of the effect of an intestinal perforation at various developmental stages. Z Kinderchir. 1982; 37:62–66.

64. Khen, N, Jaubert, F, Sauvat, F, et al. Fetal intestinal obstruction induces alteration of enteric nervous system development in human intestinal atresia. Pediatr Res. 2004; 56:975–980.

65. Amoury, RA, Ashcraft, KW, Holder, TM. Gastroschisis complicated by intestinal atresia. Surgery. 1977; 82:373–381.

66. Grosfeld, JL, Clatworthy, HW, Jr. The nature of ileal atresia due to intrauterine intussusception. Arch Surg. 1970; 100:714–717.

67. Murphy, DA. Internal hernias in infancy and childhood. Surgery. 1964; 55:311–316.

68. Vassy, LE, Boles, ET, Jr. Iatrogenic ileal atresia secondary to clamping of an occult omphalocele. J Pediatr Surg. 1975; 10:797–800.

69. Sweeney, B, Surana, R, Puri, P. Jejunoileal atresia and associated malformations: Correlation with timing of in-utero insult. J Pediatr Surg. 2001; 36:774–776.

70. Moore, SW, Rode, H, Millar, AJW, et al. Intestinal atresia and Hirschsprung’s disease. Pediatr Surg Int. 1990; 5:182–184.

71. Nicolini, U, Monni, G. Intestinal obstruction in babies exposed in-utero to methylene blue. Lancet. 1990; 336:1258–1259.

72. Guttman, FM, Braun, P, Garance, PH, et al. Multiple atresias and a new syndrome of hereditary multiple atresias involving the gastrointestinal tract from stomach to rectum. J Pediatr Surg. 1973; 8:633–640.

73. Werler, MM, Sheehan, JE, Mitchell, AA. Association of vasoconstrictive exposures with risks of gastroschisis and small intestinal atresia. Epidemiology. 2003; 14:349–354.

74. Cywes, S, Davies, MR, Rode, H. Congenital jejuno-ileal atresia and stenosis. S Afr Med J. 1980; 57:630–639.

75. Davies, MR, Louw, JH, Cywes, S, et al. The classification of congenital intestinal atresias [letter]. J Pediatr Surg. 1982; 17:224.

76. Baglaj, M, Carachi, R, Lawther, S. Multiple atresia of the small intestine: A 20-year review. Eur J Pediatr Surg. 2008; 18:13–18.

77. Louw, JH. Congenital intestinal atresia and severe stenosis in the newborn. S Afr J Clin Sci. 1952; 3:109–129.

78. Seashore, JH, Collins, FS, Markowitz, RI, et al. Familial apple peel jejunal atresia: Surgical, genetic, and radiographic aspects. Pediatrics. 1987; 80:540–544.

79. Mishalany, HG, Der Kaloustian, VM. Familial multiple-level intestinal atresias: Report of two siblings. J Pediatr. 1971; 79:124–125.

80. Weitzman, JJ, Vanderhoof, RS. Jejunal atresia with agenesis of the dorsal mesentery with “Christmas tree” deformity of the small intestine. Am J Surg. 1966; 111:443–449.

81. Zerella, JT, Martin, LW. Jejunal atresia with absent mesentery and a helical ileum. Surgery. 1976; 80:550–553.

82. Smith, MB, Smith, L, Wells, JW, et al. Concurrent jejunal atresia with “apple peel” deformity in premature twins. Pediatr Surg Int. 1991; 6:425–428.

83. DeLorimier, AA, Fonkalsrud, EW, Hays, DM. Congenital atresia and stenosis of the jejunum and ileum. Surgery. 1969; 65:819–827.

84. Dickson, JA. Apple peel small bowel: An uncommon variant of duodenal and jejunal atresia. J Pediatr Surg. 1970; 5:595–600.

85. Jimenez, FA, Reiner, L. Arteriographic findings in congenital abnormalities of the mesentery and intestines. Surg Gynecol Obstet. 1961; 113:346–352.

86. Tsujimoto, K, Sherman, FE, Ravitch, MM. Experimental intestinal atresia in the rabbit fetus. Sequential pathological studies. Johns Hopkins Med J. 1972; 131:287–297.

87. Komuro, H, Amagai, T, Hori, T, et al. Placental vascular compromise in jejunoileal atresia. J Pediatr Surg. 2004; 39:1701–1705.

88. Bilodeau, A, Prasil, P, Cloutier, R, et al. Hereditary multiple intestinal atresia: Thirty years later. J Pediatr Surg. 2004; 39:726–730.

89. Hasegawa, T, Sumimura, J, Nose, K, et al. Congenital multiple intestinal atresia successfully treated with multiple anastomoses in a premature neonate: Report of a case. Surg Today. 1996; 26:849–851.

90. Puri, P, Guiney, E, Carroll, R. Multiple gastrointestinal atresias in three consecutive siblings: Observations on pathogenesis. J Pediatr Surg. 1985; 20:22–24.

91. Baglaj, SM, Czernik, J, Koryszko, J, et al. Natural history of experimental intestinal atresia: Morphologic and ultrastructural study. J Pediatr Surg. 2001; 36:1428–1434.

92. Pickard, LR, Santoro, S, Wyllie, RG, et al. Histochemical studies of experimental fetal intestinal obstruction. J Pediatr Surg. 1981; 16:256–260.

93. Doolin, EJ, Ormsbee, HS, Hill, JL. Motility abnormalities in intestinal atresia. J Pediatr Surg. 1987; 22:320–324.

94. Cremin, BJ, Cywes, S, Louw, JH. Small intestine. In: Cremin BJ, Cywes S, Louw JH, eds. Radiological Diagnosis of Digestive Tract Disorders in the Newborn: A Guide to Radiologists, Surgeons, and Paediatricians. London: Butterworths; 1973:62.

95. Wasch, MG, Marck, A. The radiographic appearance of the gastrointestinal tract during the first day of life. J Pediatr. 1948; 32:479–489.

96. Benson, CD, Lofti, MW, Brogh, AJ. Congenital atresia and stenosis of the colon. J Pediatr Surg. 1968; 3:253–257.

97. Jackman, S, Brereton, RJ. A lesson in intestinal atresias. J Pediatr Surg. 1988; 23:852–853.

98. Aharon, M, Kleinhaus, U, Lichtig, C. Neonatal intramural intestinal calcifications associated with bowel atresia. AJR Am J Roentgenol. 1986; 130:999–1000.

99. Hays, DM. Intestinal atresia and stenosis. In: Ravitch M, ed. Current Problems in Surgery. Chicago: Year Book Medical; 1969:3.

100. Louw, JH. Resection and end-to-end anastomosis in the management of atresia and stenosis of the small bowel. Surgery. 1967; 62:940–950.

101. Blanck, C, Okmian, L, Robbe, H. Mucoviscidosis and intestinal atresia. A study of four cases in the same family. Acta Paediatr Scand. 1965; 54:557–565.

102. Suri, M, Langer, JC. A comparison of circumbilical and transverse abdominal incisions for neonatal abdominal surgery. J Pediatr Surg. 2011; 46:1076–1080.

103. McKee, MA, Jejunoileal Atresia edsitorsOldham KT, Colombani PM, Foglia RP, et al, eds. Principles and Practice of Pediatric Surgery. Lippincott Williams & Wilkins: Philadelphia, 2004; 1149.

104. de Lorimier, AA, Harrison, MR. Intestinal plication in the treatment of atresia. J Pediatr Surg. 1983; 18:734–737.

105. Holder, TM, Gross, RE. Temporary gastrostomy in pediatric surgery. Experience with 187 cases. Pediatrics. 1960; 26:36–41.

106. Howard, ER, Othersen, HB. Proximal jejunoplasty in the treatment of jejunoileal atresia. J Pediatr Surg. 1973; 8:685–690.

107. Ramanujan, TM. Functional capability of blind small bowel loops after intestinal remodeling techniques. Aust N Z J Surg. 1984; 54:145–150.

108. Kimura, K, Perdzynski, W, Soper, RT. Elliptical seromuscular resection for tapering the proximal dilated bowel in duodenal or jejunal atresia. J Pediatr Surg. 1996; 31:1405–1406.

109. Kling, K, Applebaum, H, Dunn, J, et al. A novel technique for correction of intestinal atresia at the ligament of Treitz. J Pediatr Surg. 2000; 35:353–355.

110. Grosfeld, JL, Ballantine, TV, Shoemaker, R. Operative management of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg. 1979; 14:368–375.

111. Touloukian, RJ. Intestinal atresia. Clin Perinatol. 1978; 5:3–18.

112. Snyder, CL, Miller, KA, Sharp, RJ, et al. Management of intestinal atresia in patients with gastroschisis. J Pediatr Surg. 2001; 36:1542–1545.

113. Festen, S, Brevoord, JC, Goldhoorn, GA, et al. Excellent long-term outcome for survivors of apple peel atresia. J Pediatr Surg. 2002; 37:61–65.

114. Waldhausen, JH, Sawin, RS. Improved long-term outcome for patients with jejunoileal apple peel atresia. J Pediatr Surg. 1997; 32:1307–1309.

115. Chaet, MS, Warner, BW, Sheldon, CA. Management of multiple jejunoileal atresias with an intraluminal Silastic stent. J Pediatr Surg. 1994; 29:1604–1606.

116. Haller, JA, Jr., Tepas, JJ, Pickard, LR, et al. Intestinal atresia. Current concepts of pathogenesis, pathophysiology, and operative management. Am Surg. 1983; 49:385–391.

117. Dowling, RH. Small bowel adaptation and its regulation. Scand J Gastroenterol Suppl. 1982; 17:53–74.

118. Remmington, M, Malagelada, JR, Zinsmeister, A, et al. Abnormalities in gastrointestinal motor activity in patients with short bowels: Effect of a synthetic opiate. Gastroenterology. 1983; 85:629–636.

119. Rode, H, Millar, AJW. Jejuno-ileal atresia and stenosis. In: Puri P, ed. Newborn Surgery. 2nd ed. London: Hodder Arnold; 2003:445.

120. Boles, ET, Jr., Vassy, LE, Ralston, M. Atresia of the colon. J Pediatr Surg. 1976; 11:69–75.

121. Benson, CD, Lotfi, MW, Brogh, AJ. Congenital atresia and stenosis of the colon. J Pediatr Surg. 1968; 3:253–257.

122. Powell, RW, Raffensperger, JG. Congenital colonic atresia. J Pediatr Surg. 1982; 17:166–170.

123. Croaker, GD, Harvey, JG, Cass, DT. Hirschsprung’s disease, colonic atresia, and absent hand: A new triad. J Pediatr Surg. 1997; 32:1368–1370.

124. Kim, PC, Superina, RA, Ein, S. Colonic atresia combined with Hirschsprung’s disease: A diagnostic and therapeutic challenge. J Pediatr Surg. 1995; 30:1216–1217.

125. Davenport, M, Bianchi, A, Doig, CM, et al. Colonic atresia: Current results of treatment. J R Coll Surg Edinb. 1990; 35:25–28.

126. Williams, MD, Burrington, JD. Hirschsprung’s disease complicating colon atresia. J Pediatr Surg. 1993; 28:637–639.

127. Karnak, I, Ciftci, AO, Senocak, ME, et al. Colonic atresia: Surgical management and outcome. Pediatr Surg Int. 2001; 17:631–635.

128. Sui, KL, Kwok, WK, Lee, WY, et al. A male newborn with colonic atresia and total colonic aganglionosis. Pediatr Surg Int. 1999; 15:141–142.

129. Cox, SG, Numanoglu, A, Millar, AJ, et al. Colonic atresia: Spectrum of presentation and pitfalls in management. A review of 14 cases. Pediatr Surg Int. 2005; 21:813–818.

130. Louw, JH. Investigations into the etiology of congenital atresia of the colon. Dis Colon Rectum. 1964; 7:471–478.

131. Santulli, TV, Blanc, WA. Congenital atresia of the intestine: Pathogenesis and treatment. Ann Surg. 1961; 154:939–948.

132. Rescorla, FJ, Grosfeld, JL. Intestinal atresia and stenosis: Analysis of survival in 120 cases. Surgery. 1985; 98:668–676.

133. Watts, AC, Sabharwal, AJ, Mackinlay, GA, et al. Congenital colonic atresia: Should primary anastomosis always be the goal? Pediatr Surg Int. 2003; 19:14–17.

134. DeFore, WW, Garcia-Rinaldi, R, Mattox, KL, et al. Surgical management of colon atresia. Surg Gynecol Obstet. 1976; 143:767–769.

135. Pohlson, EC, Hatch, EI, Jr., Glick, PL, et al. Individualized management of colonic atresia. Am J Surg. 1988; 155:690–692.

136. Hamzaoui, M, Ghribi, A, Makni, W, et al. Rectal and sigmoid atresia: Transanal approach. J Pediatr Surg. 2012; 47:E41–E44.