Overview
Definition:
Duodenal atresia is a congenital obstruction of the duodenum, typically occurring distal to the ampulla of Vater, characterized by a complete or near-complete blockage of the intestinal lumen
Diamond duodenoduodenostomy is a common surgical technique used to bypass this obstruction by creating a side-to-side anastomosis between the proximal and distal duodenal segments in a diamond shape.
Epidemiology:
Duodenal atresia is the second most common congenital intestinal obstruction, occurring in approximately 1 in 5,000 to 10,000 live births
It is strongly associated with Down syndrome (trisomy 21), with up to 30% of affected infants having this chromosomal abnormality
Other associated anomalies include annular pancreas, malrotation, and cardiac defects.
Clinical Significance:
Prompt diagnosis and surgical intervention are critical for survival
Delayed diagnosis can lead to severe dehydration, electrolyte imbalances, malnutrition, and increased risk of sepsis and death
Understanding the management of duodenal atresia is a core competency for pediatric surgeons and essential knowledge for all surgical residents preparing for DNB and NEET SS examinations.
Clinical Presentation
Symptoms:
Non-bilious vomiting shortly after birth, often projectile
Abdominal distension, typically in the upper abdomen
Failure to pass meconium within the first 24-48 hours of life
Poor feeding and failure to thrive
Jaundice may be present in some cases.
Signs:
Visible peristalsis of the stomach (gastric waves)
Palpable epigastric mass
Tympanitic abdomen with increased upper abdominal girth
Dehydration with decreased skin turgor and sunken fontanelles
Sepsis may be evident in severe cases.
Diagnostic Criteria:
Diagnosis is typically made based on a combination of clinical presentation and characteristic findings on imaging
Prenatal ultrasound may detect polyhydramnios and a dilated stomach bubble
Postnatal diagnosis relies on abdominal radiography and upper gastrointestinal contrast studies.
Diagnostic Approach
History Taking:
Detailed maternal history regarding prenatal ultrasound findings
Detailed infant history of feeding, vomiting (character, timing, volume), and stooling pattern
Assess for any associated congenital anomalies or family history
Red flags include non-bilious projectile vomiting within hours of birth and failure to pass meconium.
Physical Examination:
Thorough abdominal examination, noting distension, visible peristalsis, and any masses
Assess hydration status and vital signs
Examine for other congenital anomalies, particularly facial features suggestive of Down syndrome, cardiac murmurs, or limb abnormalities.
Investigations:
Abdominal radiography: may show a dilated stomach and proximal duodenum with an absence of distal gas ("double bubble sign")
Upper gastrointestinal contrast study (UGI series): essential to confirm the level and type of atresia (e.g., web, diaphragm, or complete discontinuity) and assess for associated malrotation
Chromosomal analysis: indicated due to the high association with trisomy 21
Echocardiography and other imaging: to screen for associated anomalies.
Differential Diagnosis:
Pyloric stenosis (bilious vomiting, usually later onset, olive-like mass)
Malrotation with duodenal obstruction (bilious vomiting, usually associated with volvulus)
Jejunal atresia (usually more distal obstruction, bilious vomiting, significant abdominal distension)
Gastric outlet obstruction
Congenital duodenal diaphragm or web.
Management
Initial Management:
Immediate resuscitation with intravenous fluids to correct dehydration and electrolyte imbalances
Nasogastric (NG) tube decompression to drain gastric contents and reduce distension
Broad-spectrum antibiotics initiated if sepsis is suspected
Correction of any coagulopathy.
Surgical Management:
Surgical repair is indicated for all cases of confirmed duodenal atresia
The preferred procedure is typically a diamond duodenoduodenostomy or a similar side-to-side anastomosis
This involves creating a window between the dilated proximal duodenum and the decompressed distal duodenum
The goal is to restore gastrointestinal continuity and allow passage of contents
Some cases of duodenal web or diaphragm may be managed with less extensive resection and reconstruction.
Operative Technique Diamond Duodenoduodenostomy:
A transverse incision is made in the anterior wall of the dilated proximal duodenum and a corresponding incision in the distal duodenum
These incisions are extended obliquely to create a diamond-shaped opening
The mucosa of both segments is then approximated and sutured using absorbable or non-absorbable sutures, creating a wide, patent anastomosis
Care is taken to ensure adequate mobilization and avoid tension on the anastomosis
The integrity of the anastomosis is tested with saline irrigation.
Postoperative Care:
Continued NG tube decompression until bowel motility returns (indicated by bowel sounds and passage of flatus/stool)
Gradual reintroduction of enteral feeds, starting with clear fluids and advancing slowly
Monitor for signs of anastomotic leak, sepsis, or obstruction
Strict fluid and electrolyte monitoring
Pain management
Early mobilization if appropriate
Nutritional support may be required via parenteral nutrition until full enteral feeds are tolerated.
Complications
Early Complications:
Anastomotic leak: the most serious complication, leading to peritonitis and sepsis
Wound infection
Sepsis
Recurrent obstruction due to stenosis at the anastomosis or intussusception
Ileus
Bleeding at the anastomosis.
Late Complications:
Stricture formation at the anastomosis
Malabsorption
Chronic abdominal pain
Intermittent intestinal obstruction
Gastroparesis.
Prevention Strategies:
Meticulous surgical technique with tension-free anastomosis
Adequate decompression of the proximal bowel
Careful handling of tissues
Judicious use of sutures
Aggressive postoperative monitoring for early detection of leaks
Early mobilization and gradual feeding advancement.
Prognosis
Factors Affecting Prognosis:
The presence and severity of associated anomalies (especially Down syndrome and cardiac defects)
Gestational age and birth weight
Presence of prematurity
Promptness of diagnosis and treatment
Development of complications such as anastomotic leak or sepsis.
Outcomes:
With prompt diagnosis and surgical repair, the prognosis is generally good
Survival rates are high, especially in infants without significant associated anomalies
Infants with Down syndrome may have a slightly higher morbidity and mortality due to co-existing conditions
Long-term outcome depends on the successful management of any associated conditions and the absence of significant anastomotic complications.
Follow Up:
Regular follow-up in a pediatric surgery clinic is essential
This includes monitoring for growth and development, assessing nutritional status, and screening for any signs of recurrent obstruction or malabsorption
Long-term follow-up may involve gastroenterologists
Parents should be educated on warning signs requiring immediate medical attention.
Key Points
Exam Focus:
The "double bubble" sign on plain radiography is pathognomonic for duodenal atresia/obstruction
High association with Down syndrome
Diamond duodenoduodenostomy is a common surgical repair technique
Non-bilious projectile vomiting is a hallmark symptom
NG tube decompression is crucial pre-operatively.
Clinical Pearls:
Always consider malrotation in any infant with duodenal obstruction, even if the initial UGI series is negative, as volvulus can occur later
Thorough postnatal assessment for associated anomalies is critical
Postoperative vigilance for anastomotic leak cannot be overstated.
Common Mistakes:
Delaying surgical intervention due to misdiagnosis or parental concerns
Inadequate decompression of the proximal bowel pre-operatively
Performing an anastomosis under tension
Failing to adequately screen for or manage associated anomalies
Inappropriate or delayed reintroduction of feeds post-operatively.