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Pediatric Duodenal Atresia Treatment & Management

  • Author: Frederick Merrill Karrer, MD, FACS; Chief Editor: Carmen Cuffari, MD  more...
 
Updated: Nov 11, 2014
 

Medical Therapy

No medical therapies are available for the definitive treatment of duodenal atresia or stenosis; all treatment is surgical. Adequate intravenous (IV) hydration, total parenteral nutrition, and gastric decompression are essential until the neonate has been stabilized for surgical repair.

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Surgical Therapy

Duodenal atresia and stenosis are treated surgically. In patients with duodenal obstruction, a duodenoduodenostomy is the most commonly performed procedure. A duodenojejunostomy is now uncommonly performed due to its higher risk of long-term complications. Duodenal repair may be performed via a right upper quadrant incision, an umbilical incision, or laparoscopically, depending on surgeon preference.[9, 10, 11, 12, 13]

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Preoperative Details

Little preoperative preparation is necessary if the diagnosis is secured within the first 24 hours. Placement of an orogastric (OG) tube and maintenance of intravenous (IV) hydration is mandatory in all infants with duodenal obstruction. If prolonged OG suction is necessary, IV fluid replacement of the gastric aspirate with one half normal saline with added potassium should be administered. Prior to proceeding with operative repair, the surgeon should ensure that both fluid and electrolyte derangements are adequately corrected. The surgeon should also perform a thorough examination of the infant, with special attention to cardiac and pulmonary function before undertaking duodenal repair.

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Intraoperative Details

As with all neonatal surgery, pay attention to preserving body temperature. Coordination with an anesthesiologist with specialized training in neonatal surgery is advantageous when possible because advances in pediatric anesthesia have contributed to improved overall survival of these infants. When ready to proceed, the abdomen is entered through a transverse skin incision begun 2 cm above the umbilicus from the midline and extending approximately 5 cm into the right upper quadrant (see the image below).

Incision for duodenal exposure. Incision for duodenal exposure.

Divide the abdominal musculature transversely using cautery. For adequate exposure, carefully retract the liver superiorly and pack Morison’s pouch with laparotomy pads.

Thoroughly explore the abdomen for evidence of other abnormalities. Then mobilize the duodenum using the Kocher maneuver and use an OG tube to determine the location of the obstruction without opening the stomach. The stomach and proximal duodenum are often thickened and dilated. When a significant gap is present between the proximal and distal ends, the distal duodenum must be adequately mobilized.

The authors prefer a duodenoduodenostomy for repair, when possible. This may be performed in either a side-to-side or a diamond-shaped fashion (authors' preference). For the side-to-side technique (see the image below), make parallel incisions in both the proximal and distal segments.

Side-to-side duodenoduodenostomy. Side-to-side duodenoduodenostomy.

With gentle pressure on the gallbladder, document the site of the ampulla of Vater. Examine the distal segment for other atresias or webs by passing a small red rubber catheter through the distal duodenotomy because 1-3% of patients with duodenal atresia have an additional distal small intestinal atresia.

When ready to proceed, the authors' preference is a single layer anastomosis with 4.0 or 5.0 PDS or Vicryl suture. Some surgeons prefer a 2-layer closure; the internal layer is completed with a running 4.0 Vicryl, and Lembert sutures of 5.0 silk are used for the outer layer. The authors prefer a diamond-shaped repair (see the image below), whereby the duodenotomies are created differently.

Diamond-shaped duodenoduodenostomy. Diamond-shaped duodenoduodenostomy.

Make a transverse incision in the caudal end of the proximal duodenum and a longitudinal incision of the same length in the distal segment. Stay sutures on the proximal segment are often helpful prior to proceeding with the anastomosis. The latter can be accomplished in a single layer (authors' preference) or double layer as described above. When completed, the duodenoduodenostomy assumes the shape of a diamond.

In patients with a duodenal web, the surgeon can identify the site of the web's origin by passing the OG tube through the pylorus into the duodenum and noting the indentation of the duodenal wall caused by tenting of the web. A duodenotomy can be performed along the site of this indentation. Again, prior to repair, examine the distal duodenum for a second defect. Thereafter, the surgeon must identify the ampulla and note its relationship to the web because the medial portion of most of these defects is located close to the ampulla. Accordingly, excision of the web should proceed from the lateral duodenal wall, leaving the medial third of the web alone to avoid damaging the sphincter of Oddi or ampulla. Oversew the resection line with 4.0 Dexon and close the duodenotomy either longitudinally or transversely in one layer as described above.

In patients with an annular pancreas, pancreatic tissue should not be divided for fear of pancreatic fistula. Instead, a diamond-type or side-to-side duodenoduodenostomy is recommended. Patients who present with associated malrotation should undergo a Ladd procedure at the time of duodenal repair. Although gastrostomy tubes were often used in the past, complications associated with their placement and long-term problems with gastroesophageal reflux (following gastrostomy) have prompted the authors to avoid these adjuncts, except in cases where gastrostomy is likely to be needed in the future (ie, an infant with trisomy 21 and complex congenital heart disease).[14]

If possible, the authors prefer placement a small, transanastomotic feeding tube (5F silastic nasojejunal feeding tube) across the anastomosis to facilitate postoperative enteral feeding. The authors also always leave an OG tube in place for gastric decompression. One should consider placing a peripheral intravenous central catheter (PICC) or central intravenous catheter at the time of operation because of the expected prolonged ileus and the need for parenteral nutrition.

Many patients have a very dilated proximal duodenum at the time of initial repair. In patients with an extensively floppy and distended duodenum (megaduodenum) with persistent symptoms of obstruction, an antimesenteric tapering duodenoplasty can be used to address duodenal dysmotility. An autostapling device is the most common method to resect excess duodenal tissue. Alternatively, resection with a 2-layer closure or plication with interrupted sutures over a dilator can be used. In most cases, the proximal dilatation of the duodenum resolves with time after a successful duodenoduodenostomy. A small number of infants develop megaduodenum later in life. However, the authors do not recommend duodenoplasty at the initial operation.[15, 16]

Close the abdominal wound in layers. Close the peritoneum and posterior fascia separately from the anterior fascia, using 4.0 PDS or Vicryl suture. Close the skin with a running subcuticular suture of 5.0 Vicryl.

An umbilical approach for the treatment of pyloric stenosis was described in 1986; recently, this incision has been applied to other intra-abdominal anomalies such as duodenal atresia and stenosis. A semicircular umbilical incision is made in a skin fold and the peritoneum is entered in the midline. The pliability of the neonatal abdominal wall allows retraction to expose the right upper quadrant. Repair of the duodenal anomaly proceeds in a similar fashion as described above.[17, 18]

For the laparoscopic approach, neonatal laparoscopic instruments (3 mm) and trocars are used. The patient is placed supine at the end of the operating table. The operating surgeon stands at the patient's feet. The abdomen is insufflated through a 5-mm umbilical port. Two other ports, one 3 mm and one 5 mm are placed in the right lower quadrant and left mid quadrant, respectively. The left mid-quadrant port is placed for the introduction of suture. At times, a fourth port is placed in the right upper quadrant to retract the liver. After the duodenum is Kocherized, the site of the obstruction typically becomes easily visible. A standard diamond anastomosis is then performed using interrupted sutures or u-clips (see the image below).[19, 9]

Upper GI contrast study following laparoscopic duo Upper GI contrast study following laparoscopic duodenal atresia repair. No leak is present, and an open anastomosis is shown. The white arrow highlights the anastomosis, which was performed using u-clips.

As with the open repair, stay sutures are placed at each corner to facilitate the anastomosis. The distal bowel is then examined to identify another distal atretic segment or suggestion of a web. Once completed, the ports are removed and the sites are closed with absorbable suture.

One retrospective case series compared the right upper quadrant incision to a laparoscopic repair for duodenal atresia or stenosis.[20] Fourteen patients were in the open group, and 15 patients were in the laparoscopic cohort. No anastomotic leaks were reported in either group. Patients that underwent a laparoscopic repair were advanced to full feeding quicker (9 d vs 17 d) and were discharged from the hospital sooner (13 d vs 20 d) compared with patients who underwent open repair.

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Postoperative Details

Nutrition should be provided by intravenous alimentation or via a transanastomotic feeding tube. Maintain low intermittent suction on an OG tube until stool is passed and drainage from the OG is less than 1 mL/kg/h and is clear. Feeding can then be advanced slowly by mouth.

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Follow-up

See infants 2 weeks following discharge from the neonatal intensive care unit to assess wound healing and ensure adequacy of nutrition and gastrointestinal function. Thereafter, see infants on a yearly basis to assess for the long-term complications of duodenal repair and to ensure that current practices are not contributing to long-term morbidity.

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Complications

Despite improvements in early mortality rates, as many as 22% of children may incur late complications. Late complications include blind-loop syndrome, megaduodenum with altered duodenal motility, gastritis with duodenal-gastric reflux, peptic ulcer, esophagitis and gastroesophageal reflux, pancreatitis, and cholecystitis. Blind-loop syndrome can be corrected by conversion to a duodenoduodenostomy. Megaduodenum with abnormal duodenal motility can be addressed by performing a tapering duodenoplasty. Today, these issues may be addressed at the time of initial operation by performing the duodenoduodenostomy along with duodenoplasty when necessary.[21, 22, 23]

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Outcome and Prognosis

The overall mortality rate for infants with duodenal atresia was 33% in a large series published in 1967. Today, the early mortality rate associated with this condition has declined to approximately 3% in most series. Most deaths occurring in association with duodenal atresia are attributed to the presence of multiple associated anomalies (usually complex cardiac defects). Improvement in survival rates is most likely a result of advances in neonatal care such as high-frequency ventilation, surfactant supplementation, nutritional support, pediatric anesthesia, and sophisticated cardiac surgery. Long-term survival is excellent at rates reported between 86% and 90%.

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Future and Controversies

Endoscopic excision of a duodenal web is possible but is not widely practiced and is of questionable efficacy because of the precision required to avoid damaging the ampulla when excising the membrane. Laparoscopic duodenoduodenostomy is a reasonable undertaking when the surgeon is comfortable with advanced minimally invasive techniques in infants. The ideal candidate for a laparoscopic intervention is an infant of reasonable size (>2.5 kg) without significant congenital cardiac disease. The latter may preclude maintenance of a pneumoperitoneum, which is necessary to complete the procedure safely.

Timing for the initiation of oral feeds has been traditionally based on return of bowel function (ie, passage of stool, decreased orogastric [OG] tube aspirate volume and change from bilious to clear gastric fluid). However, routine postoperative fluoroscopic evaluation for duodenal leak after laparoscopic repair has contributed to earlier initiation of oral feeds and quicker discharge from the hospital. Routine use of fluoroscopic evaluation, regardless of the technique of duodenal repair, may reduce the need for parenteral nutrition and decrease the length of hospital stays.[20]

The value of gastrostomy at the time of duodenal repair remains controversial. In 1969, some 80% of pediatric surgeons surveyed routinely used gastrostomy. Some purport large-caliber gastrostomy offers better gastric drainage when compared to an OG tube, but the authors have had little difficulty in achieving adequate gastric decompression with OG tubes. The authors believe that a gastrostomy tube adds no advantage in postoperative management, and it may contribute to late development of gastroesophageal reflux.

The authors use a transanastomotic feeding tube when it can be easily accomplished at the time of surgery. If the tube does not easily pass into the upper jejunum, attempts to place it are abandoned. A transanastomotic tube protects the anastomosis in the early postoperative period when reinstituting feeding and has low risk of causing postoperative complications. Previous worries of anastomotic complications following transanastomotic tube placement are no longer justified. The slender silicone character of newer tubes remains supple in the lumen of the bowel unlike earlier polythene and plastic tubes, which hardened in situ. Nevertheless, pediatric surgeons remain divided in their use of transanastomotic tubes.

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Contributor Information and Disclosures
Author

Frederick Merrill Karrer, MD, FACS Professor of Surgery and Pediatrics, Head, Division of Pediatric Surgery, University of Colorado School of Medicine; The Dr David R and Kiku Akers Chair in Pediatric Surgery, Surgical Director, Pediatric Transplantation, The Children’s Hospital

Frederick Merrill Karrer, MD, FACS is a member of the following medical societies: American Academy of Pediatrics, American Association for the Study of Liver Diseases, Children's Oncology Group, International Liver Transplantation Society, Transplantation Society, International Society of Paediatric Surgical Oncology, Pacific Association of Pediatric Surgery, International Pediatric Transplant Association, Colorado Medical Society, Society of Critical Care Medicine, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society of Transplant Surgeons, Western Surgical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Casey M Calkins, MD Associate Professor of Surgery, Division of Pediatric Surgery, Medical College of Wisconsin; Consulting Staff, Department of Pediatric Surgery, Children's Hospital of Wisconsin

Casey M Calkins, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association

Disclosure: Nothing to disclose.

D Dean Potter, MD Fellow in Pediatric Surgery, The Children's Hospital

D Dean Potter, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, Minnesota Medical Association, International Pediatric Endosurgery Group

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching.

Additional Contributors

Jayant Deodhar, MD Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

References
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Complete duodenal obstruction.
Incomplete duodenal obstruction (duodenal stenosis).
Incision for duodenal exposure.
Side-to-side duodenoduodenostomy.
Diamond-shaped duodenoduodenostomy.
Upper GI contrast study following laparoscopic duodenal atresia repair. No leak is present, and an open anastomosis is shown. The white arrow highlights the anastomosis, which was performed using u-clips.
 
 
 
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