Duodenal Atresia

Duodenoduodenostomy REF: Bailey and love 24^th ed p. 1198

The most common method of treatment of duodenal atresia is duodenoduodenostomy or duodenojejunostomy.

Duodenal atresia REF: Bailey and love 24^T0 ed p. 1199, Nelson’s pediatrics 17^th ed p. 1234

Double bubble sign is seen in

• Ladd’s bands, malrotation
• Annular pancreas
• Duodenal atresia, stenosis, web, duplication, obstruction

Down syndrome REF: Sabiston 18′^h ed chapter 71

Duodenal atresia (DA) is thought to occur as a result of failure of vacuolization of the duodenum from its solid cord stage. The range of anatomic variants includes duodenal stenosis, mucosal web with intact muscular wall (so-called windsock deformity); two ends separated by a fibrous cord, or complete separation with a gap within the duodenum.

Associated Anomalies: DA is associated with several conditions, including prematurity, Down syndrome, maternal polyhydramnios, malrotation, annular pancreas, and biliary atresia. Other  anomalies, such as cardiac, renal, esophageal, and anorectal anomalies, are also common. In most cases, the duodenal obstruction is distal to the ampulla of Vater, and infants present with bilious emesis in the neonatal period. In patients with a mucosal web, the symptoms of postprandial emesis may occur later in life.

Diagnosis: The classic plain abdominal radiograph of DA is termed the double-bubble sign (air-filled stomach and duodenal bulb, In cases in which there is no distal air, the diagnosis is secured, and no further studies are necessary. On the other hand, if distal air is present, an upper gastrointestinal contrast study is performed fairly rapidly. This study is important not only to confirm the diagnosis of duodenal stenosis or atresia but also to exclude midgut volvulus, which would constitute a surgical emergency.

Treatment: The management of DA is by surgical bypass of the duodenal obstruction as either a side-to-side or proximal transverse—to—distal longitudinal (diamond-shaped) duodenoduodenostomy.

Duodenal atresia [Ref: Nelson 18/e p1559; Journal- Radiology. 2001;220:463-464.]

• “The ultrasound hallmark of duodenal atresia is the ‘double bubble sign’. This consists of two adjacent. fluid-filled cavities in the upper abdomen: a lateral and usually somewhat larger cavity representing the dilated stomach, and a medial cavity representing the distended proximal duodenum between the pylorus and the stenosis.”- Ultrasound in obstetrics and gynecology By Eberhard Merz, F. Bahlmann 2/e p298

Double bubble sign:

• The double bubble sign is a finding observed on radiographs of newborns and infants. It is reproducible with a variety of other imaging modalities, including upper gastrointestinal studies and prenatal sonography. The finding depicts two air-filled structures in the upper abdomen, with little or no air distally. Horizontal-beam radiographs will demonstrate air and fluid levels in both bubbles.
• The first bubble (proximal and left sided) is – stomach
• The second bubble ( to the right of the midline) is – duodenum
• The detection of the double bubble sign leads to suspicion of a number of structural anomalies, all of which cause duodenal obstruction.

Causes:

• The specific duodenal structural anomaly causing the obstruction determines the appearance, prominence, and presence of air distal to the obstruction. Duodenal atresia produces complete obstruction, Partially obstructing anomalies, such as duodenal web or duodenal stenosis, allow passage of some air distal to the obstruction and a less dramatic double bubble.

Duodenal atresia 

Duodenal atresia

Duodenal atresia

In Pyloric obstruction there will be only single air fluid level. For multiple air fluid levels on X ray obstruction should be distal to duodenal bulb.

Ref: Walker’s Pediatric Gastrointestinal Disease: Physiology, Diagnosis and Management By Ronald E. Kleinman, 2008, Page 122

Duodenal atresia is due to failure of recanalization of the duodenal lumen during weeks 3–7 of embryologic development.

Newborn will have bilious vomiting in the first 24 hours of life.

“Double-bubble” sign and absence of gas distal to the duodenum can be seen on plain films.

In duodenal atresia, postnatal abdominal plain radiographs show gaseous distention of the stomach and proximal duodenum (the “double-bubble” radiologic sign).

With protracted vomiting, there is less air in the stomach and less abdominal distention. 

Ans. is ‘c’ i.e., Duodenal atresia

o Most common cause of neonatal intestinal obstruction                   Meconium plug syndrome.

o Second most common cause of neonatal intestinal obstruction       Duodenal atresia.

Important causes of intestinal obstruction in newborn

• Meconium plug syndrome            o Duodenal atresia              o Jujunoileal atresia
• Malrotation of gut                       o Meconium ileus

Meconium plug syndrome —> when meconium plug causes obstruction of colon. It is seen in Hirschsprung disease, maternal DM, maternal preeclampsia, prematurity, sepsis, hypothyroidism.

Meconium Ileus —> when meconium plug obstruct ileum. It is seen in cystic fibrosis.

Note ‑

o Intussusception is the most common cause of intestinal obstruction between 3 months and 6 year of age (not in neonate).

Ans. is ‘b i.e., Intestinal atresia & `c’ i.e., Duodenal atresia

• Most common cause of Intestinal obstruction in Down’s syndrome is duodenal atresia.
• Duodenal atresia is a type of intestinal atresia.

A i.e. Duodenal atresia

A i.e. Duodenal atresia

Answer is ‘b’ i.e. Duodenal atresia

• Antenatal double-bubble appearance on ultrasound is seen in duodenal obstruction, the two bubbles being the fluid filled stomach and the proximal duodenum.

Causes of duodenal obstruction in the newborn are : duodenal atresia duodenal stenosis

– duodenal web annular pancreas

– duodenal duplication cyst malrotation and midgut volvulus

• Duodenal obstruction is usually associated with polyhdyramnios.
• In 85% of infants with duodenal obstruction, the opening of bile duct is proximal to the site of obstruction. In these cases the vomiting is bilious.
• The surgical treatment of choice for duodenal atresia, stenosis or annular pancreas is duodenoduodenostomy.

Ans. is ‘a’, ‘b’ & ‘c’ i.e. Duodenal atresia, Duodenal stenosis and Volvulus 

Ans is ‘b’ ie Duodenal atresia 

• Congenital atresia and stenosis are the commonest cause.
• The incidence of atresia varies with anatomical site:

• Vomitting is bile stained or not, depends on the atresia proximal or distal to ampulla of vater. In 85% of cases the atresia is distal to the ampulla, therefore the vomitting is bilious.
• Approximately 1/3 of newborns with duodenal atresia have associated Down’s syndrome*.
• The ‘double bubble* seen on the air contrast upper GI series is characterstic of duodenal atresia.
• Treatment of choice is – Duodenoduodenostomy*

Ans. is ‘d’ i.e., Duodeno duodenostomy 

Ans is b i.e. Duodenal atresia 

“The ultrasound hallmark of duodenal atresia is the ‘double bubble sign’. This consists of two adjacent, fluid-filled cavities in the upper abdomen: a lateral and usually somewhat larger cavity representing the dilated stomach, and a medial cavity representing the distended proximal duodenum between the pylorus and the stenosis.”

Double bubble sign:

• The double bubble sign is a finding observed on radiographs of newborns and infants. It is reproducible with a variety of other imaging modalities, including upper gastrointestinal studies and prenatal sonography. The finding depicts two air-filled structures in the upper abdomen, with little or no air distally. Horizontal-beam radiographs will demonstrate air and fluid levels in both bubbles.
• The first bubble (proximal and left sided) is – stomach
• The second bubble ( to the right of the midline) is – duodenum
• The detection of the double bubble sign leads to suspicion of a number of structural anomalies, all of which cause duodenal obstruction.

Causes:

• The specific duodenal structural anomaly causing the obstruction determines the appearance, prominence, and presence of air distal to the obstruction. Duodenal atresia produces complete obstruction, Partially obstructing anomalies, such as duodenal web or duodenal stenosis, allow passage of some air distal to the obstruction and a less dramatic double bubble.

Ans. D: Duodenal Atresia

Plain films are useful in assessing the level of the obstruction.

Air-fluid levels in the stomach and duodenum (double-bubble sign), an important finding of acute duodenal obstruction, can also be seen on plain films.

Ans. Duodenal atresia

Ans. Duodenal atresia

Ans:D.)All of the above.

The Image Shows Double Bubble Sign.

The double bubble sign

It is seen in infants and represents dilatation of the proximal duodenum and stomach. It is seen in both radiographs and ultrasound, and can be identified antenatally .
Causes include

• congenital obstruction
  • duodenal web
  • duodenal atresia
  • duodenal stenosis
  • annular pancreas
• midgut volvulus
• external compression of the dudenum
  • choledochal cyst
  • mesenteric duplication cyst
  • intramural duodenal haematoma
  • preduodenal portal vein
  • retroperitoneal tumour
  • superior mesenteric artery syndrome

Ans:B.)Duodenal atresia

“Double bubble” sign is seen in the image .
-Thin arrow: dialated stomach
-Thick arrow: dialated proximal duodenum

Double bubble sign

• It is seen in infants and represents dilatation of the proximal duodenum and stomach.
• It is seen in both radiographs and ultrasound, and can be identified antenatally .
• Causes include :
  • congenital obstruction
    • duodenal web
    • duodenal atresia
    • duodenal stenosis
    • annular pancreas
  • midgut volvulus
  • external compression of the dudenum
    • choledochal cyst
    • mesenteric duplication cyst
    • intramural duodenal haematoma
    • preduodenal portal vein
    • retroperitoneal tumour

Duodenal Atresia

• It is thought to represent a failure of recanalisation of the bowel lumen that is a solid tube early in fetal life (~11-12 weeks).
• Approximately 20–40 percent of all infants with duodenal atresia have Down syndrome.
• During pregnancy, duodenal atresia is associated with increased amniotic fluid in the uterus, which is called polyhydramnios.
• After birth, duodenal atresia may cause abdominal distension, especially of the upper abdomen.
  • Bilious vomiting commonly occurs within the first day of life.
• The diagnosis of duodenal atresia is usually confirmed by radiography.
  • An X-ray of the abdomen shows two large air filled spaces, the so-called “double bubble” sign. The air is trapped in the stomach and proximal duodenum, which are separated by the pyloric sphincter, creating the appearance of two bubbles visible on x-ray.
• Treatment
  • Early treatment includes removing fluids from the stomach via a nasogastric tube, and providing fluids intravenously. The definitive treatment for duodenal atresia is surgery (duodenoduodenostomy), which may be performed openly or laparoscopically.