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Clinical case: Duplication of the duodenum
This article is based on a case report published in the Journal "Case Reports in Surgery" in 2015, by Caroline C. Jadlowiec, Beata E. Lobel, Namita Akolkar, Michael D. Bourque, Thomas J. Devers, and David W. McFadden.
It has been modified and reviewed by Joel A. Vilensky PhD, Carlos A. Suárez-Quian PhD, Aykut Üren, MD.
- Objectives
- Case description
- Anatomical and embryological considerations
- Objective explanations
- Sources
Objectives
After reviewing this case you should be able to describe or do the following:
- How the patient’s symptoms and signs are associated with her diagnosis.
- The significance of bile pooling in the duodenum.
- The anatomical distinctions among the four parts of the duodenum.
- Why the surgeon did an end-to-side anastomosis rather than an end-to-end anastomosis.
- How imaging could be used to differentiate between a cystic and tubular duplication.
- Label on the cadaver image attached here the relevant parts of the GI tract to this case and describe using this image the surgical procedure.
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Case description
History
A 59-year-old female was seen for evaluation following several months of:
- increasing postprandial abdominal pain
- early satiety
- reflux
- unexpected weight loss
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Investigations
The patient’s laboratory evaluation was unremarkable, and she did not have any prior medical or surgical history that could account for her complaints. An upper GI radiographic exam suggested an abnormality involving the duodenum because the duodenal C-loop appeared to be markedly dilated (Figure 2-B and Figure 3 compared to normal - Figure 2-A).
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Differential diagnosis
Initially, the patient’s physicians considered two clinical diagnoses:
- Duodenal dilation was occurring secondary to a stricture, or an extrinsic compression in the fourth portion of the duodenum or at the duodenal-jejunal junction.
- The patient had GI duplication of some kind.
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Diagnosis
In order to determine if one of these diagnoses was correct an upper endoscopic exam was performed (Figure 5). This exam revealed that there were three downstream orifices just distal to the Ampulla of Vater (hepatopancreatic ampulla) suggesting that duodenal duplication was the correct diagnosis.
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Management
There was bile pooling in the duodenum. A decision was then reached to proceed with surgical exploration.
During the surgery, the duodenum was freed from its attachment to the posterior abdominal wall (remember the most of the duodenum is in retroperitoneum) and it became apparent that the duplication extended superiorly in front of the body of the pancreas.
Examination of the duodenum intraoperatively revealed that two of the orifices were blind with only one connection to the jejunum. Thus, the duplicated portions of the duodenum were fully mobilized and resected (Figure 6).
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A proximal transection was made just distal to the ampulla with a distal resection occurring at the entrance to the jejunum. A hand-sewn end-to-side duodenojejunostomy was then done to a slightly more downstream part of the jejunum (Figures 7+1). The surgical exploration confirmed duplication of the third and fourth portions of the duodenum.
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Anatomical and embryological considerations
Duplications of the gastrointestinal (GI) tract are uncommon congenital anomalies that occur in either cystic or tubular form. Characteristics shared by all enteric duplications include their close attachment to the GI tract, epithelial mucosal lining, and a well-developed visceral muscle layer. Abdominal GI duplications are hypothesized to result from recanalization errors of the neonatal solid GI tract. All enteric duplications are believed to occur with an incidence of about 1 per 4000–5000 live births. Relative to other alimentary tract duplications, duodenal duplications are comparatively rare (about 6%).
Objective explanations
Objectives
- How the patient’s symptoms and signs are associated with her diagnosis.
- The significance of bile pooling in the duodenum.
- The anatomical distinctions among the four parts of the duodenum.
- Why the surgeon did an end-to-side anastomosis rather than an end-to-end anastomosis.
- How imaging could be used to differentiate between a cystic and tubular duplication.
Links between signs, symptoms and diagnosis
The duodenal duplication present in this patient was undoubtedly present at birth, but apparently for the patient’s life until her 59th year it either did not cause any GI problems or the problems were not severe enough for the patient to seek intensive medical attention. We can presume that at this age her one true channel became partially or completely occluded resulting in the dilation of the proximal parts of the duodenum causing reflux, early satiety, pain, and weight loss.
In essence, the partially occluded true channel now acted similarly to a bowel obstruction causing dilation proximally; in this type of obstruction it would seem that the stomach might also be expected to become dilated. Although this may occur, the dilation is limited because belching/burping allows the release of gases from the stomach.
Significance of bile pooling
Bile pooling in the duodenum reflects both the occluded duodenal channel and the lack of effective peristalsis to propel the bile into the jejunum.
Anatomical parts of the duodenum
First part
The first part of the duodenum (superior part) is at vertebral level L1 and is joined to the pylorus. This part of the duodenum is mobile and has a mesentery, part of the lesser omentum, which is connected to the liver (hepatoduodenal ligament). The second part of the lesser omentum connects the lesser curvature of the stomach to the liver (hepatogastric ligament). The first part of the duodenum terminates when the duodenum turns inferiorly at the superior duodenal flexure.
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Second part
The second or descending part begins at that superior flexure and ends at the inferior flexure where the duodenum turns medially. It is at the level of the L2 vertebral body. The combined pancreatic and bile ducts enter the second part of the duodenum at the hepatopancreatic ampulla (Ampulla of Vater). This part of the duodenum, the descending part, is retroperitoneal and represents the end of the embryological foregut and beginning of the midgut.
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Third part
The third or horizontal (inferior) part is at the L3 level, begins at the inferior flexure and passes transversely to the left, posterior to the superior mesenteric artery and vein. This part also passes anterior to the inferior vena cava and aorta. It is this third part that can be compressed between the superior mesenteric artery and the aorta producing superior mesenteric artery syndrome.
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Fourth part
The last part, the fourth (ascending) part of the duodenum ascends joining with the jejunum at the duodenojejunal flexure. The duodenojejunal flexure is attached to the posterior abdominal wall by the suspensory muscle of the duodenum (ligament of Treitz). Although the fourth part, similar to the second and third parts of the duodenum, is retroperitoneal, the duodenojejunal flexure is intraperitoneal.
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Reasons for end-to-side anastomosis
GI surgeons may use end-to-end or end-to-side anastomosis when sewing one part of GI tract to another. Although end-to-end would seem to be “cleaner”, end-to-side is advantageous in situations in which the two parts of the GI tract are markedly different in size. That was the case here because the dilated duodenum was much larger than the normal-sized jejunum. Of course the blind end of the jejunum needed to be sewed closed, and presumably, peristalsis would prevent food from being stalled in the blind end (Figure 7+1).
Differentiating between a cystic and tubular duplication using imaging
Cystic duodenal duplications are typically fluid-filled and sometimes contain gallstones, bile, or pancreatic fluid. Ultrasound of cystic duplications should reveal an anechoic fluid-filled, double-walled cyst composed of an inner hyperechoic layer of mucosa - submucosa and an outer hypoechoic rim of smooth muscle.
However, ultrasound imaging of abdominal structures is often unclear in adults unless they are rather thin. For CT imaging, oral contrast can be useful because a cystic duplication will not fill and not show communication with the remainder of the gastrointestinal tract but rather may show compression on adjacent structures, whereas a tubular duplication would be expected to fill and communicate with the rest of the GI tract.
Clinical case: Duplication of the duodenum: want to learn more about it?
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