You are in: eMedicine Specialties > Pediatrics: Surgery > General Surgery Duodenal Atresia and Stenosis: Surgical PerspectiveArticle Last Updated: Oct 1, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Nicola Lewis, MBBS, FRCS, Specialist Registrar, Department of Surgery, Birmingham Children's Hospital, UK Coauthor(s): Philip Glick, MD, MBA, Professor, Departments of Surgery, Pediatrics, and Gynecology and Obstetrics, Vice-Chairperson for Research and Development, Department of Surgery, State University of New York at Buffalo Editors: Robert K Minkes, MD, PhD, Professor of Surgery, University of Texas Southwestern; Chief of Surgical Services, Children's Medical Center of Dallas-Legacy; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine; Andre Hebra, MD, Chief, Division of Pediatric Surgery, Medical University of South Carolina; Professor of Surgery and Pediatrics, Medical University of South Carolina; H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina; Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago Author and Editor Disclosure Synonyms and related keywords: duodenal atresia, stenosis, congenital duodenal obstruction, gastrojejunostomy, duodenojejunostomy, duodenoduodenostomy, duodenoplasty, intrinsic duodenal obstruction, high intestinal obstruction, upper intestinal obstruction, sustained vomiting, bilious vomiting, nonbilious vomiting, loss of fluid, loss of electrolytes, hyperalimentation, Down syndrome, esophageal atresia, malrotation, anterior portal vein, second distal web, anorectal anomalies, intestinal atresias, cloacal anomalies, renal tract anomalies, polyhydramnios, choledochal cyst, failure to thrive INTRODUCTIONSection 2 of 12
  Fonkalsrud reviewed 503 cases of congenital duodenal obstruction treated between 1957 and 1967.1 Of patients who were surgically treated, 64% survived. Deaths were attributed to associated malformations, respiratory complications, prematurity, and anastomotic complications. More recent survival rates for infants born with duodenal atresia or stenosis are approximately 90-95%.2, 3 Increased survival rates can be attributed to advances in respiratory care, hyperalimentation, improved pediatric anesthesia, improvements in the recognition and management of associated anomalies, and more refined surgical techniques (eg, the diamond-shaped anastomosis4). History of the ProcedureIn 1733, Calder described the first 2 recorded cases of duodenal atresia. The first successfully treated case was reported by Vidal in 1905; a gastrojejunostomy was performed. In 1914, Ernest performed the first successful duodenojejunostomy in an infant with duodenal atresia. Current surgical management more commonly includes duodenoduodenostomy and duodenoplasty. ProblemSee Pathophysiology. FrequencyThe incidence of duodenal atresia is 1 case per 5,000-10,000 live births. EtiologyMost cases of duodenal atresia are sporadic. Investigations of familial cases of duodenal atresia suggest an autosomal recessive inheritance in these individuals.1, 5 PathophysiologyIn 1900, Tandler described the traditionally accepted theory on the normal development of the duodenum.6 The duodenum develops from the caudal part of the foregut and the cranial part of the midgut. At 4 weeks' gestation, it consists of an epithelial tube surrounded by mesenchyme. At 5-6 weeks' gestation, the epithelium proliferates while the surrounding mesenchymal walls are still narrow; the epithelial cells fill the lumen, completely obliterating it. Subsequent epithelial apoptosis at 8-10 weeks' gestation leads to vacuolation and recanalization of the duodenum. Failure of vacuolation may lead to intrinsic duodenal obstruction. ClinicalIn 38-55% of patients, intrinsic duodenal obstruction is associated with another significant congenital anomaly.1, 7, 8, 9 Approximately 30% of cases are associated with Down syndrome, and 23-34% of cases are associated with isolated cardiac defects. Esophageal atresia may be present in 7-12% of patients.10 Other GI anomalies include malrotation, anterior portal vein, second distal web, anorectal anomalies, intestinal atresias, cloacal anomalies, and renal tract anomalies. Duodenal atresia is associated with prematurity and low birth weight.11, 8 Rarely, duodenal atresia is seen as a part of Feingold syndrome.12 Duodenal atresia Duodenal atresia is prenatally detected in 32-57% of patients.7, 13 Sonographic features of high intestinal obstruction (ie, duodenal obstruction with a dilated stomach [double-bubble sign]) become apparent in the third trimester. Polyhydramnios develops in 32-59% of cases; in the presence of polyhydramnios, normal findings on ultrasonography of the fetus do not exclude duodenal atresia.14, 7, 15 A similar appearance can be observed in fetuses with a choledochal cyst, external duodenal compression, and a normal stomach with a sharp incisura. Approximately 80% of cases are prenatally diagnosed with confirmation following delivery.13 Prenatal diagnosis of duodenal atresia should lead to a search for other associated anomalies and amniocentesis for karyotype analysis. Following delivery, a thorough physical examination should be performed, including careful examination of the anus. Healthy newborn infants have gastric aspirates that measure less than 5 mL. Congenital intestinal obstruction is associated with gastric aspirates that measure greater than 30 mL.16 An infant with a gastric aspirate that measures greater than 30 mL in the delivery room or newborn nursery should be evaluated for duodenal atresia and other causes of upper intestinal obstruction. Symptoms of upper intestinal obstruction commence within the first 24 hours after birth. However, patients may present hours or days after delivery. Sustained vomiting (bilious or nonbilious) is the most common symptom, occurring in approximately 85% of cases.2, 1, 3 Nonbilious vomiting occurs when atresia is present above the papilla of Vater. Vomiting is associated with variable dehydration, changes in serum electrolytes, and weight loss. Normal meconium may be observed in the early stages.14, 3 The high level of the obstruction makes global abdominal distension an infrequent finding, but fullness in the epigastrium, caused by the dilated duodenum and stomach, may be noted. Differentials include malrotation and volvulus, intestinal atresia or stenosis in other locations, and extrinsic duodenal obstruction, duodenal duplication, or congenital bands. Duodenal stenosis The incomplete nature of the obstruction in duodenal stenosis results in a variable and often delayed presentation. It usually results in recurrent episodes of vomiting, aspiration, or failure to thrive. Some patients present in adulthood with gastroesophageal reflux, peptic ulceration, or obstruction of the duodenum proximal to the stenosis by a bezoar. INDICATIONSSection 3 of 12
The definitive management of patients with intrinsic duodenal obstruction is surgical correction. RELEVANT ANATOMYSection 4 of 12
Duodenal atresia or stenosis usually occurs in the first or second part of the duodenum, most often near the papilla of Vater. The common bile duct may open into an intraluminal mucosal web. The 3 anatomic types of duodenal atresia as described by Gray and Skandalakis (see Media file 1) are as follows:
Various biliary tract and pancreatic anomalies have been demonstrated in patients with duodenal atresia or stenosis. These include stenosis and duplication of the distal common bile duct, choledochal cysts, and annular pancreas. Of note, air in the distal duodenum and gallbladder on plain radiography is suggestive of a bifid common bile duct. Double duodenal atresia or stenosis is less frequently reported.17 CONTRAINDICATIONSSection 5 of 12
In the patient with associated tracheoesophageal fistula, ligation of the fistula should precede correction of the duodenal atresia. This can be performed on 2 occasions or simultaneously. Repair of the atresia prior to ligation of the tracheoesophageal fistula could lead to duodenal rupture. WORKUPSection 6 of 12
Lab Studies
Imaging Studies
Diagnostic Procedures
TREATMENTSection 7 of 12
Surgical therapyThe operative management of duodenal atresia is determined by the anatomic findings and associated anomalies noted upon laparotomy. Bypass procedures for duodenal atresia or stenosis include duodenoduodenostomy or duodenojejunostomy. Type 1 duodenal atresia can also be managed by simple duodenotomy with web excision. Preoperative detailsOrogastric decompression of the stomach and fluid resuscitation should be promptly initiated. Orogastric losses are monitored and replaced. Broad-spectrum antibiotics and 1 mg vitamin K are administered. Parenteral nutrition is instituted on the first day of life via a peripherally inserted central catheter. When stable, the infant is taken to the operating room. Intraoperative details
Postoperative detailsThe orogastric tube is left on free drainage. The patient is not given oral feedings until bowel sounds are heard, stool is passed, and the gastric drainage is limited (<1 mL/kg/h of clear or pale-green fluid). This may take 7-10 days but can be prolonged in the premature infant with other significant anomalies that require venous access for parenteral nutrition. Oral feedings are gradually introduced, commencing with clear fluids and aspirating the stomach prior to each feed. These infants can be placed right-side down after feeds to enhance gastroduodenal emptying. Follow-upPoor peristalsis in the proximal duodenum leads to functional obstruction in some patients. This may predispose the patient to blind loop syndrome and duodenogastric reflux. Alkaline biliary reflux leads to gastritis and peptic ulceration. Reports of functional obstructions occurring more than 2 decades after primary repair suggest that survivors of duodenal atresia should receive the following long-term follow-up care:
COMPLICATIONSSection 8 of 12
Early postoperative complications are frequently related to prematurity, coexisting congenital anomalies, and parenteral nutrition. Intestinal obstruction secondary to adhesions may also occur in the early postoperative period. Long-term complications occur at any time from a few months to years following the primary procedure. Hence, long-term follow-up is compulsory for infants treated for intrinsic duodenal obstruction. Late complications include the following:20, 21, 22
OUTCOME AND PROGNOSISSection 9 of 12
Current survival rates for infants with duodenal atresia or stenosis are 90-95%. Higher mortality rates are associated with prematurity and multiple congenital abnormalities. Postoperative complications are reported in 14-18% of patients; some require reoperation.2 Possible indications for reoperation include anastomotic leak, functional duodenal obstruction, adhesions, and missed atresias. Long-term follow-up of these patients reveals that most of these patients are asymptomatic with a normal nutritional status. In a 1988 study, Kokkonen et al found a poor correlation between symptoms and radiologic and endoscopic findings; the megaduodenum failed to return to normal caliber, and duodenogastric reflux and duodenal dysmotility persisted decades after the initial surgery in asymptomatic patients.21 Approximately 12% of patients develop late complications. Late deaths occur in approximately 6% of patients, and 50% of these are related to complex cardiac conditions. Less than 10% of patients require fundoplication for gastroesophageal reflux, and less than 10% require revision of the initial repair.23 Dysmotility disorders associated with megaduodenum can be managed with an antimesenteric tapering duodenoplasty or duodenal plication. FUTURE AND CONTROVERSIESSection 10 of 12
Duodenal webs can be diagnosed and excised by an expert surgical endoscopist. This is feasible in patients who present after the neonatal period with duodenal stenosis. The morbidity and complications associated with a megaduodenum may require further surgical intervention. Transanastomotic feeding tubes or gastrostomies were used in the past but have been demonstrated to offer no clear advantage and, instead, result in a delay in establishing oral feedings and an increase in the duration of hospitalization.24, 3, 25 MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
Duodenal Atresia and Stenosis: Surgical Perspective excerpt Article Last Updated: Oct 1, 2008 | ||||||||||||||||||||||||||
