Duodenal Atresia And Stenosis


Article Author:
William Gossman
Benjamin Eovaldi


Article Editor:
Harris Cohen


Editors In Chief:
Lawrence Lee
Michael Firstenberg
Lawrence Greiten


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Kyle Blair
Trevor Nezwek
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Daniyal Ameen
Altif Muneeb
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
5/19/2019 11:08:08 PM

Introduction

Duodenal atresia is a congenital intestinal obstruction that can cause bilious or nonbilious vomiting within the first 24 to 38 hours of neonatal life, typically following the first oral feeding. It is associated with in-utero polyhydramnios and is one of the most common causes of fetal bowel obstruction. Antenatal ultrasound can make the diagnosis. If duodenal atresia is not diagnosed antenatally, then the diagnosis can be made radiographically with plain abdominal x-ray as the first step in evaluation. This may be followed by a controlled contrast exam if needed. Either barium for a limited upper gastrointestinal (UGI) series or water/Pedialyte for an ultrasound evaluation can be performed to confirm the diagnosis. CT plays a limited if any role in the evaluation of duodenal atresia.[1]

Etiology

Obstruction of the duodenum causes duodenal atresia; usually distal to the ampulla of Vater in the second portion of the duodenum. Errors of duodenal re-canalization during the eighth to the tenth week of embryological development is the main cause of duodenal atresia. In duodenal atresia, there is complete obstruction of the duodenal lumen. Duodenal stenosis is the term used for narrowing resulting in an incomplete obstruction of the duodenum lumen. A duodenal web is a rarer cause of duodenal obstruction which tends to cause a windsock deformity of the duodenal lumen.[2]

Epidemiology

Duodenal atresia occurs in 1 in 5000 to 10,000 live births. It is often associated with other anomalies, including trisomy 21/Down syndrome and cardiac malformations. Approximately 30% of children with duodenal atresia have Down syndrome. There is a 3% prevalence of congenital duodenal atresia among patients with trisomy 21/Down syndrome. There is no difference in prevalence between the genders. There is an association with VACTERL, annular pancreas, and other bowel atresias including jejunal atresia, ileal atresia, and rectal atresia.[3]

Pathophysiology

Duodenal atresia is believed to occur because of failure of the epithelial solid cord to recanalize or excessive endodermal proliferation.[4]

History and Physical

Duodenal atresia presents early in life as vomiting; which usually occurs within the first 24 to 38 hours of life after the first feeding and progressively worsens if not treated. Sometimes the vomiting may be projectile; which like pylorospasm and gastroesophageal reflux, may simulate hypertrophic pyloric stenosis.[5][6] The clinical presentation of bilious vomiting points to congenital intestinal obstruction distal to the ampulla of Vater. There are cases of atresia proximal to the ampulla of Vater that present without bilious vomiting. The ampulla of Vater is located in the second or descending portion of the duodenum. Excessive bilious vomiting can cause hypokalemic hypochloremic metabolic alkalosis with paradoxical aciduria, particularly if there is a delay in hydration. Patients have symptoms of abdominal distension and absent bowel movements.

Evaluation

Antenatal imaging will show a double bubble, an echoless stomach filled with amniotic fluid, as well as a second nearby but more distal fluid filled, often circular (but blind ended) structure (the second bubble) that is the obstructed portion of the duodenum.  The use of prenatal ultrasound has allowed for an earlier diagnosis of duodenal atresia.  An advantage of neonatal abdominal ultrasound is that it can be performed in the neonatal intensive care unit or nursery.  When antenatal ultrasound is performed the duodenum is usually not filled with fluid, and the presence of a fluid-filled duodenum suggests duodenal atresia.  If a double-bubble sign is seen on antenatal ultrasound, then it is important for the sonographer to demonstrate a connection between the two fluid-filled structures because foregut duplication cyst, as well as other abdominal cysts, may simulate the appearance of a double-bubble sign.[7][8]

The initial postnatal radiographic evaluation for diagnosing duodenal atresia is a plain abdominal x-ray.  In duodenal atresia, there is gas in the stomach and the proximal duodenum but an absence of gas distally in the small or large bowel.  A plain abdominal x-ray may reveal the double-bubble sign which is seen postnatally as a large radiolucent (air-filled) stomach usually in the normal position to the left of the midline, and a smaller more distal bubble to the right of midline which represents a dilated duodenum.  A double-bubble sign on an abdominal x-ray is a reliable indicator of duodenal atresia.  Other causes of intestinal obstruction may simulate a double-bubble sign.  Annular pancreas is the second most common cause of duodenal atresia.  Jejunal or more distal obstruction may have dilation more distally or more than two bubbles may be present.[8]

In patients with Down syndrome, the x-ray double-bubble has a higher positive predictive value because the prevalence of duodenal atresia in Down syndrome is much higher than in the general population and further evaluation with additional modalities is not always needed before proceeding to surgery.  Duodenal stenosis can also cause the double-bubble sign; however, if stenosis is present, at least a small amount of gas may be present distal to the obstruction.  The absence of gas in the stomach on abdominal x-ray is a sign of esophageal atresia without tracheoesophageal fistula.  At times there may be distal air distal to a duodenal atresia that has entered distal bowel via an anomalous biliary tree.  The radiographic appearance of the double-bubble sign should prompt immediate surgical consultation.  For cases of suspected duodenal atresia not identified antenatally, barium fluoroscopy can be used to assess the gastrointestinal tract.  If necessary further evaluation with ultrasound or upper gastrointestinal series (UGI) may be performed.  More recently, neonatal ultrasound has been shown to be very helpful in clarifying the anatomy, especially using the fluid as a contrast agent.[9]  A possible mimic of the double-bubble sign is the pseudo-double-bubble sign created by the curved configuration of the stomach where the two bubbles represented normal fluid in the proximal the and distal stomach.

Barium contrast is administered sometimes via an orogastric or nasogastric tube under fluoroscopy to evaluate the esophagus, stomach, and duodenum.  Only a controlled amount of barium is placed to confirm obstruction.  It is then removed by nasogastric tube to prevent reflux and potential aspiration.  The main purpose of the UGI is to differentiate between duodenal atresia and midgut volvulus; an important distinction, because midgut volvulus requires emergency surgery, whereas duodenal atresia can be managed on an urgent basis.[10]

Pediatric CT plays a very limited if any role in diagnosis and evaluation of duodenal atresia.  However, its 2D reconstruction may allow for further evaluation of bowel layout in confusing cases.  Neonatal CT is more technically challenging as it may require sedation, bundling and, starting a neonatal intravenous line for contrast administration.  Additionally, CT does involve ionizing radiation and may be more difficult to interpret because there is less abdominal fat separating abdominal viscera compared to older patients.

Treatment / Management

Treatment involves nasogastric suction to decompress the stomach and surgery to correct the obstructing lesion. Duodenoduodenostomy is the typical surgery performed. Doudenodoudenostomy can be performed as an open or laparoscopic procedure. Doudenodoudenostomy is a type of bypass procedure that is said to be technically demanding for a laparoscopic approach. A diamond-shaped anastomosis is constructed with a proximal transverse to the distal longitudinal duodenal anastomosis. The bypass procedure avoids damage to the pancreas, main pancreatic duct, accessory pancreatic duct, and common bile duct. Before surgery, the stomach and proximal duodenum are decompressed with an orogastric tube, and intravenous fluid resuscitation is performed. Possible complications of duodenoduodenostomy include gastroesophageal reflux, megaduodenum, and impaired duodenal motility. A complete operative evaluation includes looking for additional areas of intestinal obstruction.[11]

Differential Diagnosis

It is helpful to know if the vomitus is bilious or not in developing a differential diagnosis. Primary considerations for cases of bilious vomiting in neonates are duodenal atresia, duodenal stenosis, annular pancreas, but particularly malrotation of the intestine caused by midgut volvulus whereby superior mesenteric artery flow to the distal bowel is compromised. Midgut volvulus usually presents days later in life than duodenal atresia. In babies with Down syndrome, it is important to look for other gastrointestinal abnormalities associated with Down syndromes such as anal atresia, Hirschsprung disease, diaphragmatic hernia, and omphalocele.[8]

Prognosis

Prognosis after successful surgical treatment of duodenal atresia is excellent.

Complications

Complications are chiefly associated with surgery and include the following:

  • Megaduodenum 
  • Blind loop syndrome
  • Cholecystitis
  • Esophagitis
  • Peptic ulcer disease
  • GERD
  • Pancreatitis

Pearls and Other Issues

In utero, duodenal atresia, a proximal gastrointestinal tract obstruction, causes polyhydramnios by interfering with the gastrointestinal absorption of amniotic fluid swallowed by the fetus distal to the level of intestinal obstruction. Polyhydramnios is defined as abnormally large amounts of amniotic fluid in the gestational sac during pregnancy. Antenatal ultrasound is diagnostic for polyhydramnios when the amniotic fluid index is greater than 25 cm or when the total amniotic fluid is estimated to be greater than 1500 to 2000 ml. In duodenal atresia, amniotic fluid that is swallowed by the fetus is prevented from moving distally to be absorbed by the fetal gastrointestinal tract and transferred to the maternal circulation through the placenta.  Rather it is refluxed back into the amniotic fluid. Eighty percent of cases of polyhydramnios are due to maternal or idiopathic causes. Maternal causes include congestive heart failure and gestational diabetes. Gestational diabetes can cause fetal hyperglycemia and fetal polyuria.  Fetal causes include congenital anomalies that interfere with swallowing and intestinal obstruction including duodenal atresia. Other anomalies causing polyhydramnios include esophageal atresia, head and neck masses, thoracic masses, and supratentorial disorders that disrupt swallowing.  Polyhydramnios increases the risk for cord prolapse, placental abruption, and preterm delivery.

Enhancing Healthcare Team Outcomes

Duodenal atresia is a relatively common intestinal disorder which often presents soon after delivery, hence it is best managed by a multidisciplinary team that includes a pediatric surgeon, obstetrician, neonatal nurse, dietitian, pediatrician, intensivist and a geneticist. The definitive management of this condition is surgery, but the procedure is often associated with complications that prolong hospital stay. Over the past three decades, the mortality rates of duodenal atresia have significantly dropped averaging 2-5%. Mortality rates are not directly related to the surgery but to the other associated organ anomalies like complex congenital cardiac defects. However, survival continues to improve with better NICU care, nutritional support and improved pediatric anesthesia. Today long-term survival of most infants (more than 80%) with duodenal atresia is the norm. The biggest controversy for the future is the use of endoscopy to excise the duodenal web. This technique remains experimental. The other controversy surrounds the time to feed the patient after surgery. Experts recommend a fluoroscopic evaluation to check for a leak before initiating feeding. 


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Duodenal Atresia And Stenosis - Questions

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A newborn is found to have a double-bubble on an abdominal radiograph. What is the next step in management?



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A newborn develops bilious vomiting. Abdominal x-rays reveal a double-bubble sign. Fluid and electrolyte balance has been established. What is the most appropriate treatment?



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A double-bubble sign is seen on abdominal radiographs obtained at birth. This can account for what complication suffered by the mother during pregnancy?



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Which radiographic sign is associated with duodenal obstruction?



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A newborn is found to have a double-bubble on an abdominal x-ray. What is the next step in this newborn's management?

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What is the best treatment for a patient with duodenal atresia?



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An infant with Down syndrome presents with persistent bilious vomiting. Which of the following is the most likely diagnosis?



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Which of the following conditions commonly is seen with duodenal atresia?



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A newborn in the newborn nursery has persistent bilious vomiting. What is the next best step in management?

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Duodenal atresia commonly is found in which of the following disorders?



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Which of the following is the best initial investigation for diagnosing duodenal atresia?

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An infant presents with bilious vomiting within 24 hours of birth. On physical examination, the abdomen is not markedly distended. What is the most likely diagnosis?



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Within 24 hours after birth, a neonate has bilious vomiting when the mother tried to breastfeed. The vomiting is forceful and the infant is no longer able to feed. The mother states that the pregnancy was uneventful and the vaginal delivery was not complicated. His abdominal x-ray is shown below. After a quick exam, the provider suspects that the infant may have a genetic defect. Auscultation reveals that the infant has a systolic ejection murmur at the upper left sternal border and a low pitched early diastolic rumble at the lower end of the sternal border. The second heart sound appears to be split. What heart defect may be responsible for his findings on auscultation?

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The pediatrics team is consulted to investigate a 1 day old male in the nursery. The patient is constantly throwing up feeds and his abdomen is quite distended. Upon physical examination, there is a single palmar crease and low set ears. An xray of the abdomen shows a classic "double-bubble-sign". What is the most common cause of this condition?



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Duodenal Atresia And Stenosis - References

References

Morris JK,Springett AL,Greenlees R,Loane M,Addor MC,Arriola L,Barisic I,Bergman JEH,Csaky-Szunyogh M,Dias C,Draper ES,Garne E,Gatt M,Khoshnood B,Klungsoyr K,Lynch C,McDonnell R,Nelen V,Neville AJ,O'Mahony M,Pierini A,Queisser-Luft A,Randrianaivo H,Rankin J,Rissmann A,Kurinczuk J,Tucker D,Verellen-Dumoulin C,Wellesley D,Dolk H, Trends in congenital anomalies in Europe from 1980 to 2012. PloS one. 2018     [PubMed]
Oh C,Lee S,Lee SK,Seo JM, Laparoscopic duodenoduodenostomy with parallel anastomosis for duodenal atresia. Surgical endoscopy. 2017 Jun     [PubMed]
Srisajjakul S,Prapaisilp P,Bangchokdee S, Imaging spectrum of nonneoplastic duodenal diseases. Clinical imaging. 2016 Nov - Dec     [PubMed]
Sheorain VK,Cohen HL,Boulden TF, Classical wind sock sign of duodenal web on upper gastrointestinal contrast study. Journal of paediatrics and child health. 2013 May     [PubMed]
Prasad TR,Bajpai M, Intestinal atresia. Indian journal of pediatrics. 2000 Sep     [PubMed]
Gilet AG,Dunkin J,Cohen HL, Pylorospasm (simulating hypertrophic pyloric stenosis) with secondary gastroesophageal reflux. Ultrasound quarterly. 2008 Jun     [PubMed]
Cohen HL,Blumer SL,Zucconi WB, The sonographic double-track sign: not pathognomonic for hypertrophic pyloric stenosis; can be seen in pylorospasm. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. 2004 May     [PubMed]
Koberlein G,DiSantis D, The "double bubble" sign. Abdominal radiology (New York). 2016 Feb     [PubMed]
Blumer SL,Zucconi WB,Cohen HL,Scriven RJ,Lee TK, The vomiting neonate: a review of the ACR appropriateness criteria and ultrasound's role in the workup of such patients. Ultrasound quarterly. 2004 Sep     [PubMed]
Cohen HL,Moore WH, History of emergency ultrasound. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine. 2004 Apr     [PubMed]
Latzman JM,Levin TL,Nafday SM, Duodenal atresia: not always a double bubble. Pediatric radiology. 2014 Aug     [PubMed]

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