Duodenal atresia

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Duodenal atresia
DuodAtres.png
Radiograph of abdomen with double-bubble sign from duodenal atresia
Specialty Pediatric gastroenterology, Pediatric surgery
Symptoms Antenatal period: polyhydramnios Neonatal period: bilious or non-bilious vomiting within first 36 hours of life, abdominal distension, lack of stooling
Usual onsetDuring embryological development
CausesUnknown
Risk factors Down syndrome
Differential diagnosis annular pancreas, duodenal stenosis
Treatment nasogastric suction, surgery (duodenoduodenostomy)

Duodenal atresia is the congenital absence or complete closure of a portion of the lumen of the duodenum. It causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies. Newborns present with bilious or non-bilous vomiting (depending on where in the duodenum the obstruction is) within the first 24 to 48 hours after birth, typically after their first oral feeding. Radiography shows a distended stomach and distended duodenum, which are separated by the pyloric valve, a finding described as the double-bubble sign.

Contents

Treatment includes suctioning out any fluid that is trapped in the stomach, providing fluids intravenously, and surgical repair of the intestinal closure.

Signs and symptoms

History and physical examination

During pregnancy, duodenal atresia is associated with increased amniotic fluid in the uterus, which is called polyhydramnios. [1] This increase in amniotic fluid is caused by the inability of the fetus to swallow the amniotic fluid and absorb it in their digestive tract.[ citation needed ]

After birth, duodenal atresia may cause abdominal distension, especially of the upper abdomen. Bilious or non-bilious vomiting, depending on the position of the atresia in relation to the Ampulla of Vater, commonly occurs within the first day of life.[ citation needed ]

Associated conditions

Approximately 20–40 percent of all infants with duodenal atresia have Down syndrome and 50% have a congenital cardiac anomaly. [2] Approximately 8% of infants with Down syndrome have duodenal atresia. [1]

Diagnosis

Imaging

Antenatal

Antenatal ultrasonography allows for earlier detection and diagnosis of duodenal atresia. The duodenum is not typically filled with fluid on imaging, however if fluid is visualized on ultrasound, this may suggest duodenal atresia as it causes obstruction and fluid may not pass distal to the atretic area. [3] Early diagnosis of duodenal atresia provides time for clinicians to provide prenatal counseling and prepare for postnatal management. [4]

Postnatal

The diagnosis of duodenal atresia is usually confirmed by radiography. An X-ray of the abdomen is the first step in evaluation. The x-ray should be obtained after placement of a nasogastric tube (feeding tube), evacuating the stomach and filling 40-50 ml of air [5] to demonstrate two large air filled spaces, the so-called "double bubble" sign. [6] [7] 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. [8] Since the closure of the duodenum is complete in duodenal atresia, no air is seen in the distal duodenum. Note that the double bubble sign is typically pathologic however it is not specific for duodenal atresia and may indicate other pathologies such as annular pancreas or midgut volvulus. [9] A limited upper gastrointestinal series using barium contrast can be used to differentiate between duodenal atresia and midgut volvulus. [10]

Causes

The cause of duodenal atresia is not known. Dr. Julius Tandler hypothesized in 1900 that the etiology of duodenal atresia was due to occlusion of the duodenal lumen and failure to re-canalize during embryological development. Research surrounding duodenal atresia in recent years has switched focus to the fibroblast growth factor pathway in mouse models as Tandler's original hypothesis was unable to account for the different types of duodenal atresia. [11]

Diagnosis

Classification

Duodenal atresia is classified into 3 types: [5]

Management

Early treatment includes removing fluids from the stomach via a nasogastric tube, and providing fluids intravenously. [13] The definitive treatment for duodenal atresia is surgery (duodenoduodenostomy or duodenojejunostomy), which may be performed openly or laparoscopically. [14] The surgery is required but not immediately urgent - a 24 to 48-hour delay is permissible to facilitate transport, further evaluation and fluid resuscitation. [13] The initial repair has a 5 percent morbidity and mortality rate. [15] Nasogastric suction is typically continued post-operatively and patients can be transitioned to small oral feeds once nasogastric tube output decreases significantly or stops completely. [5]

Complications

Prognosis is usually very good, although complications are more likely to occur when there are serious congenital anomalies. [13] Complications from surgical repair include Peptic ulcer disease, Gastroesophageal reflux disease, Cholecystitis, Esophagitis, Megaduodenum, Blind loop syndrome, and anastomotic leak. [10] Late complications may occur in about 12 percent of patients with duodenal atresia, and the mortality rate for these complications is 6 percent. [15]

Epidemiology

Duodenal atresia occurs in 1 in every 10,000 live births and is the most common intestinal atresia, constituting up to 60% of intestinal atresias. [16]

Related Research Articles

<span class="mw-page-title-main">Small intestine</span> Organ in the gastrointestinal tract

The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the pancreatic duct to aid in digestion. The small intestine is about 5.5 metres long and folds many times to fit in the abdomen. Although it is longer than the large intestine, it is called the small intestine because it is narrower in diameter.

<span class="mw-page-title-main">Bowel obstruction</span> Medical condition

Bowel obstruction, also known as intestinal obstruction, is a mechanical or functional obstruction of the intestines which prevents the normal movement of the products of digestion. Either the small bowel or large bowel may be affected. Signs and symptoms include abdominal pain, vomiting, bloating and not passing gas. Mechanical obstruction is the cause of about 5 to 15% of cases of severe abdominal pain of sudden onset requiring admission to hospital.

<span class="mw-page-title-main">Pyloric stenosis</span> Medical condition

Pyloric stenosis is a narrowing of the opening from the stomach to the first part of the small intestine. Symptoms include projectile vomiting without the presence of bile. This most often occurs after the baby is fed. The typical age that symptoms become obvious is two to twelve weeks old.

<span class="mw-page-title-main">Omphalocele</span> Rare abdominal wall defect in which internal organs remain outside of the abdomen in a sac

Omphalocele or omphalocoele also called exomphalos, is a rare abdominal wall defect. Beginning at the 6th week of development, rapid elongation of the gut and increased liver size reduces intra abdominal space, which pushes intestinal loops out of the abdominal cavity. Around 10th week, the intestine returns to the abdominal cavity and the process is completed by the 12th week. Persistence of intestine or the presence of other abdominal viscera in the umbilical cord results in an omphalocele.

<span class="mw-page-title-main">Tracheoesophageal fistula</span> Medical condition

A tracheoesophageal fistula is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy.

<span class="mw-page-title-main">Ileus</span> Medical condition

Ileus is a disruption of the normal propulsive ability of the intestine. It can be caused by lack of peristalsis or by mechanical obstruction. The word 'ileus' is from Ancient Greek eileós. The term 'subileus' refers to a partial obstruction.

Colic in horses is defined as abdominal pain, but it is a clinical symptom rather than a diagnosis. The term colic can encompass all forms of gastrointestinal conditions which cause pain as well as other causes of abdominal pain not involving the gastrointestinal tract. The most common forms of colic are gastrointestinal in nature and are most often related to colonic disturbance. There are a variety of different causes of colic, some of which can prove fatal without surgical intervention. Colic surgery is usually an expensive procedure as it is major abdominal surgery, often with intensive aftercare. Among domesticated horses, colic is the leading cause of premature death. The incidence of colic in the general horse population has been estimated between 4 and 10 percent over the course of the average lifespan. Clinical signs of colic generally require treatment by a veterinarian. The conditions that cause colic can become life-threatening in a short period of time.

<span class="mw-page-title-main">Intussusception (medical disorder)</span> Medical condition

Intussusception is a medical condition in which a part of the intestine folds into the section immediately ahead of it. It typically involves the small bowel and less commonly the large bowel. Symptoms include abdominal pain which may come and go, vomiting, abdominal bloating, and bloody stool. It often results in a small bowel obstruction. Other complications may include peritonitis or bowel perforation.

<span class="mw-page-title-main">Volvulus</span> Twisting of part of the intestine, causing a bowel obstruction

A volvulus is when a loop of intestine twists around itself and the mesentery that supports it, resulting in a bowel obstruction. Symptoms include abdominal pain, abdominal bloating, vomiting, constipation, and bloody stool. Onset of symptoms may be rapid or more gradual. The mesentery may become so tightly twisted that blood flow to part of the intestine is cut off, resulting in ischemic bowel. In this situation there may be fever or significant pain when the abdomen is touched.

Intestinal malrotation is a congenital anomaly of rotation of the midgut. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Malrotation can lead to a dangerous complication called volvulus. Malrotation can refer to a spectrum of abnormal intestinal positioning, often including:

<span class="mw-page-title-main">Annular pancreas</span> Medical condition

Annular pancreas is a rare condition in which the second part of the duodenum is surrounded by a ring of pancreatic tissue continuous with the head of the pancreas. This portion of the pancreas can constrict the duodenum and block or impair the flow of food to the rest of the intestines. It is estimated to occur in 1 out of 12,000 to 15,000 newborns. The ambiguity arises from the fact that not all cases are symptomatic.

<span class="mw-page-title-main">Foregut</span> Anterior part of the gastrointestinal tract

The foregut is the anterior part of the alimentary canal, from the distal esophagus to the first half of the duodenum, at the entrance of the bile duct. Beyond the stomach, the foregut is attached to the abdominal walls by mesentery. The foregut arises from the endoderm, developing from the folding primitive gut, and is developmentally distinct from the midgut and hindgut. Although the term “foregut” is typically used in reference to the anterior section of the primitive gut, components of the adult gut can also be described with this designation. Pain in the epigastric region, just below the intersection of the ribs, typically refers to structures in the adult foregut.

<span class="mw-page-title-main">Intestinal atresia</span> Medical condition

Intestinal atresia is any congenital malformation of the structure of the intestine that causes bowel obstruction. The malformation can be a narrowing (stenosis), absence or malrotation of a portion of the intestine. These defects can either occur in the small or large intestine.

<span class="mw-page-title-main">Gastric outlet obstruction</span> Medical condition

Gastric outlet obstruction (GOO) is a medical condition where there is an obstruction at the level of the pylorus, which is the outlet of the stomach. Individuals with gastric outlet obstruction will often have recurrent vomiting of food that has accumulated in the stomach, but which cannot pass into the small intestine due to the obstruction. The stomach often dilates to accommodate food intake and secretions. Causes of gastric outlet obstruction include both benign causes, as well as malignant causes, such as gastric cancer.

Gastric volvulus or volvulus of stomach is a twisting of all or part of the stomach by more than 180 degrees with obstruction of the flow of material through the stomach, variable loss of blood supply and possible tissue death. The twisting can occur around the long axis of the stomach: this is called organoaxial or around the axis perpendicular to this, called mesenteroaxial. Obstruction is more likely in organoaxial twisting than with mesenteroaxial while the latter is more associated with ischemia. About one third of the cases are associated with a hiatus hernia. Treatment is surgical.

<span class="mw-page-title-main">Subglottic stenosis</span> Medical condition

Subglottic stenosis is a congenital or acquired narrowing of the subglottic airway. It can be congenital, acquired, iatrogenic, or very rarely, idiopathic. It is defined as the narrowing of the portion of the airway that lies between the vocal cords and the lower part of the cricoid cartilage. In a normal infant, the subglottic airway is 4.5-5.5 millimeters wide, while in a premature infant, the normal width is 3.5 millimeters. Subglottic stenosis is defined as a diameter of under 4 millimeters in an infant. Acquired cases are more common than congenital cases due to prolonged intubation being introduced in the 1960s. It is most frequently caused by certain medical procedures or external trauma, although infections and systemic diseases can also cause it.

Hematocolpos is a medical condition in which the vagina is pooled with menstrual blood due to multiple factors leading to the blockage of menstrual blood flow. The medical definition of hematocolpos is 'an accumulation of blood within the vagina'. It is often caused by the combination of menstruation with an imperforate hymen. It is sometimes seen in Robinow syndrome, uterus didelphys, or other vaginal anomalies.

Megaduodenum is a congenital or acquired dilation and elongation of the duodenum with hypertrophy of all layers that presents as a feeling of gastric fullness, abdominal pain, belching, heartburn, and nausea with vomiting sometimes of food eaten 24 hours prior.

Neonatal bowel obstruction (NBO) or neonatal intestinal obstruction is the most common surgical emergency in the neonatal period. It may occur due to a variety of conditions and has an excellent outcome based on timely diagnosis and appropriate intervention.

<span class="mw-page-title-main">Double bubble (radiology)</span>

In radiology, the double bubble sign is a feature of pediatric imaging seen on radiographs or prenatal ultrasound in which two air filled bubbles are seen in the abdomen, representing two discontiguous loops of bowel in a proximal, or 'high,' small bowel obstruction. The finding is typically pathologic, and implies either duodenal atresia, duodenal web, annular pancreas, and on occasion midgut volvulus, a distinction that requires close clinical correlation and, in most cases, surgical intervention.

References

  1. 1 2 "Duodenal Atresia". Archived from the original on 2012-08-05. Retrieved 2007-12-03.
  2. ↑ Bethell, George Stephen; Long, Anna-May; Knight, Marian; Hall, Nigel J.; BAPS-CASS (2019-06-22). "Congenital duodenal obstruction in the UK: a population-based study". Archives of Disease in Childhood. Fetal and Neonatal Edition. 105 (2): fetalneonatal-2019-317085. doi: 10.1136/archdischild-2019-317085 . ISSN   1468-2052. PMC   7063389 . PMID   31229958.
  3. ↑ Blumer, Steven L.; Zucconi, William B.; Cohen, Harris L.; Scriven, Richard J.; Lee, Thomas K. (2004). "The vomiting neonate: a review of the ACR appropriateness criteria and ultrasound's role in the workup of such patients". Ultrasound Quarterly. 20 (3): 79–89. doi:10.1097/00013644-200409000-00002. ISSN   0894-8771. PMID   15322385. S2CID   40208130.
  4. ↑ Shawis, Rang; Antao, Brice (2006). "Prenatal bowel dilatation and the subsequent postnatal management". Early Human Development. 82 (5): 297–303. doi:10.1016/j.earlhumdev.2006.02.005. ISSN   0378-3782. PMID   16626900.
  5. 1 2 3 4 5 6 Gharpure, Vivek (January 2014). "Duodenal atresia". Journal of Neonatal Surgery. 3 (1): 14. doi:10.47338/jns.v3.78. ISSN   2226-0439. PMC   4420424 . PMID   26023485.
  6. ↑ Poki HO, Holland AJ, Pitkin J (2005). "Double bubble, double trouble". Pediatr. Surg. Int. 21 (6): 428–31. doi:10.1007/s00383-005-1448-z. PMID   15912365. S2CID   21589667.
  7. ↑ Traubici J (August 2001). "The double bubble sign". Radiology. 220 (2): 463–4. doi:10.1148/radiology.220.2.r01au11463. PMID   11477252.
  8. ↑ Rizi, Farid Rajaee. "Duodenal atresia | Radiology Case | Radiopaedia.org". Radiopaedia. doi:10.53347/rid-156737 . Retrieved 2022-12-01.
  9. ↑ Papandreou, E.; Baltogiannis, N.; Cigliano, B.; Savanelli, A.; Settimi, A.; Keramidas, D. (2004). "Annular pancreas combined with distal stenosis. A report of four cases and review of the literature". La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics. 26 (4): 256–259. ISSN   0391-5387. PMID   16366413.
  10. 1 2 Sigmon, David F.; Eovaldi, Benjamin J.; Cohen, Harris L. (2021), "Duodenal Atresia And Stenosis", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID   29261981 , retrieved 2021-11-07
  11. ↑ Jones, Matthew L. M.; Sarila, Gulcan; Chapuis, Pierre; Hutson, John M.; King, Sebastian K.; Teague, Warwick J. (2020). "The Role of Fibroblast Growth Factor 10 Signaling in Duodenal Atresia". Frontiers in Pharmacology. 11: 250. doi: 10.3389/fphar.2020.00250 . ISSN   1663-9812. PMC   7076179 . PMID   32210824.
  12. ↑ Borkar, NitinkumarBhajandas; Tiwari, Charu; Singh, Sunita; Mane, Sonali; Sinha, Chandrasen (2023). "Delayed presentation of duodenal atresia". African Journal of Paediatric Surgery. 20 (2): 97. doi:10.4103/ajps.ajps_66_21. ISSN   0189-6725.
  13. 1 2 3 Kimura, Kim; Loening-Baucke, V (May 1, 2000). "Bilious vomiting in the newborn: rapid diagnosis of intestinal obstruction". American Family Physician. 61 (9): 2791–8. PMID   10821158 . Retrieved 16 September 2012.
  14. ↑ Spilde, Troy L; St Peter, SD; Keckler, SJ; Holcomb GW, 3rd; Snyder, CL; Ostlie, DJ (June 2008). "Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series". Journal of Pediatric Surgery. 43 (6): 1002–5. doi:10.1016/j.jpedsurg.2008.02.021. PMID   18558173.
  15. 1 2 Escobar, Mauricio A; Ladd, AP; Grosfeld, JL; West, KW; Rescorla, FJ; Scherer LR, 3rd; Engum, SA; Rouse, TM; Billmire, DF (June 2004). "Duodenal atresia and stenosis: long-term follow-up over 30 years". Journal of Pediatric Surgery. 39 (6): 867–71, discussion 867–71. doi:10.1016/j.jpedsurg.2004.02.025. PMID   15185215 . Retrieved 16 September 2012.
  16. ↑ Best, Kate E.; Tennant, Peter W. G.; Addor, Marie-Claude; Bianchi, Fabrizio; Boyd, Patricia; Calzolari, Elisa; Dias, Carlos Matias; Doray, Berenice; Draper, Elizabeth; Garne, Ester; Gatt, Miriam (September 2012). "Epidemiology of small intestinal atresia in Europe: a register-based study". Archives of Disease in Childhood. Fetal and Neonatal Edition. 97 (5): F353–358. doi:10.1136/fetalneonatal-2011-300631. ISSN   1468-2052. PMID   22933095. S2CID   20852328.
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