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M. Hernandez et al.
Most choledochal cysts are diagnosed in childhood, yet nearly 25% can be detected during adulthood. Choledochal cysts are more common in females com­pared to males with the ratios being as high as 4:1 or 3:1 [56]. Choledochal cysts are classied according to location within the biliary tree and have been described pre­viously by Todani etal. Patients who have a choledochal cyst often will report non­specic and vague symptoms such as abdominal pain [57]. Biliary cystic dilation complications due to local compression can include pancreatitis, cholangitis, sec­ondary biliary cirrhosis, spontaneous rupture, and increase the risk of cholangiocarcinoma.
Choledochal cysts can be categorized into ve types [54, 57]. Type I represents about 50 to 80% of all choledochal cysts, type II 2%, type III 1 to 4%, type IV 15–30%, type V 20%. Type I choledochal cyst can be classied into three additional phenotypes. Type Ia is a cystic dilation of the entire extrahepatic biliary tree that does not involve the intrahepatic ducts. Often the gallbladder and cystic duct will arise from a dilated common bile duct. Type Ib represents a focal segmental dilation of the extrahepatic biliary tree. Type Ic represents a fusiform dilation of the entire extrahepatic biliary tree that involves an intrahepatic duct. Type II choledochal cysts are a saccular diverticulum on the common bile duct. Type III choledochal cysts are also called choledochoceles, and these represent a cystic dilation of the intramural portion of the distal common bile duct that often will bulge into the duodenum. Type IV includes types IVa and IVb. Type IVa is the second most common choledochal cyst and it is represented by an intrahepatic and extrahepatic dilation of the biliary ducts. Type IVb represents multiple dilations of only the extrahepatic biliary tree. Type V choledochal cyst, also known as Caroli’s disease, represents multiple dila­tions of the intrahepatic biliary ducts. This anatomic anomaly is often associated with congenital hepatic brosis which can present with cirrhosis and is termed Caroli’s syndrome [58].
The etiology of choledochal cysts is an ongoing debate with both congenital and acquired proposed mechanisms. The most common is Babbitt’s theory where cho­ledochal cysts are thought to be due to an aberrant pancreaticobiliary ductal junction [59]. This manifests as the pancreatic duct joining the bile duct 1–2cm proximal to the sphincter of Oddi. This length of a common channel can vary between 10 and 45mm. A long common channel allows pancreatic juice to reux into the biliary tree which increases intraluminal pressure and can in theory result in ductal dilation [60]. This is supported in that there is often a high amylase concentration within the bile samples of patients with choledochal cysts [60]. Further pancreatic enzyme reux can lead to inammation, epithelial breakdown, dysplasia, and subsequently malignancy. Despite this proposed mechanism, the long common channel is only observed in about 50–80% of patients with choledochal cysts. Another theory is due to obstruction of the distal common bile duct. Sphincter of Oddi dysfunction can predispose to the development of choledochal cysts.
Because most choledochal cysts present in childhood and about 25% will present in adulthood, it is important to recognize a set of classic symptoms. Often there is a triad which includes abdominal pain, palpable abdominal mass, and jaundice. This
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is seen altogether in about <20% of all cases. In children, 85% will have these two features of the triad whereas in adults only 25% will present with at least two fea­tures of the triad.
Due to chronic inammation and subsequent bile stasis, stones and infections can develop. This can manifest in ascending cholangitis and obstructive jaundice. The symptoms of this include abdominal pain and fever. Chronic inammation especially along the distal common bile duct can cause pancreatitis. Secondary bili­ary cirrhosis can be due to chronic biliary obstruction. There is an increased risk for malignancy in patients with choledochal cysts. The incidence of developing malig­nancy varies between 2% and 18% [61]. In a series of 38 adult patients this was reported to be as high as 21%. The incidence of malignancy increases with age [62]. Malignancy is often observed in the extrahepatic ducts and about 50–60% of patients, gallbladder in about 30–50% of patients, intrahepatic biliary ductal malig­nancies in about 2% of patients, and about <1% of patients with liver and pancreas malignancies [62].
Clinical investigation should be performed using imaging as well as liver func­tion tests, serum amylase and lipase levels, INR and complete blood count to evalu­ate for obstructive jaundice or pancreatitis kidney function and altered coagulation proles. Tumor biomarkers can be obtained including a CA 19–9in patients who have suspicion for malignancy.
Diagnostic workup using imaging should be performed to assist with operative planning and complete excision of all choledochal cysts. Abdominal ultrasound is often a rst step. Sensitivity of ultrasound is about 70–97% [63]. Ultrasounds can also be used for postoperative surveillance [64]. In addition to an ultrasound, con­trasted MRI and MRCP can be used to delineate biliary anatomy [65]. HIDA scans can be used and have a sensitivity of 100% for type I choledochal cyst, meanwhile the sensitivity diminishes for the other types [66]. Computed tomography is highly accurate and can be used to help delineate arterial and vascular anatomy assisting in surgical planning [65]. CT scans can also be utilized to estimate cyst wall thicken­ing which can be seen in malignancy. ERCP is extremely sensitive and diagnostic for choledochal cysts; the sensitivity decreases in patients who have developed con­siderable inammation and scarring over time. ERCP is an invasive technique which can potentially cause cholangitis and pancreatitis [67]. MRCP is still consid­ered the gold standard for diagnosis as the sensitivity is as high as 90 to 100% MRCP also avoids ionizing radiation and is noninvasive compared to ERCP [65]. MRCP can also be used with MRI imaging to evaluate surrounding vascular anat­omy, presence of biliary sludge or stones and malignancies.
Management of choledochal cysts has changed considerably over time. Several decades ago, initial management consisted of a cyst enterostomy. However, this was associated with recurrence of symptoms and a high risk of malignancy within the remaining in situ cyst wall several decades later. There was an observed malignancy rate of nearly 30% in patients who had previously undergone a cyst enterostomy for the management of a choledochal cyst [62]. Complete excision of the biliary cyst was then recommended and reconstructed or diverted biliary tree was subsequently
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needed. Biliary diversion can be performed using a hepaticoduodenostomy or hepaticojejunostomy.
Complete excision of the cyst followed by Roux-en-Y hepaticojejunostomy is the standard of choice for the treatment of most choledochal cysts. This procedure can be performed via the open, laparoscopic, or robotic approach. Long-term sur­veillance and outcomes are dependent on complete cyst excision and appropriate surveillance.
Duodenal Lymphoma
Duodenal follicular lymphomas are often found incidentally [68]. Often upper endoscopic surveillance is performed for another reason and patients who have low stage follicular lymphomas often do not have overt clinical symptoms [68]. If the patient presents with symptoms it is often vague with abdominal pain. The mean age at diagnosis is 65years [68]. There is no difference between male and female. Most commonly the second portion of the duodenum is involved.
At the time of endoscopy, a duodenal follicular lymphoma can be found as either a solitary or multiple nodular masses [69]. These can present as a polypoid lesion, and they are usually between 1 and 5mm in size. As the masses grow, they can become ulcerated which can make endoscopic sampling easier. Multifocal disease is not uncommon with lesions located elsewhere in the jejunum or ileum [70]. On histologic evaluation, most follicular lymphomas of the duodenum are low grade [71]. On microscopy, these appear as well-circumscribed germinal centers with no visible macrophages and without mantle cell zones. Sheets of lymphoid cells that contain irregular nuclei are often present, and the lymphoma will involve the mucosa and submucosa [70]. The immunophenotype is similar to other low-grade follicular lymphomas and these will express CD20, CD10, BCL–6, BCL–2 and lack expres­sion for CD5, CD23, CD43, BCL–1, and T-cell markers [72]. Often these lympho­mas will have a low Ki-67 rate [72].
The classic genetic aberration often found is a translocation (14; 18)(q. 32; q. 21) for the genes of the immunoglobulin heavy chain and B-cell leukemia lymphoma [72]. Duodenal follicular lymphoma has a pathogenesis that is often related to repeat inammation and antigen stimulation and shares some similarities with mucosa-associated lymphoid tissue (MALT) lymphomas [73]. Differential diagno­sis includes MALT lymphoma, chronic lymphocytic leukemia, mantle cell lym­phoma, and gastrointestinal involvement by systemic follicular lymphoma [74].
Duodenal follicular lymphomas often have a good prognosis with median sur­vival exceeding 12years [75]. The only treatment for this is chemotherapy. Patients who have limited stage follicular lymphomas within the duodenum can also be treated with radiation therapy [76, 77]. This is associated with a good overall out­come as relapse impacts subsequent outcomes. Even though treatment with radio­therapy to the duodenal lymphoma is quite successful most patients will not undergo treatment initially [78]. There is an option to watch and wait which has also been
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demonstrated to be effective as well. Other approaches for early stage disease con­sist of rituximab with chemotherapy, rituximab alone, combined modality therapies, or a variety of other systemic therapies [78].

Conclusion

Duodenal lesions span the spectrum of benign, premalignant, and malignant etiolo­gies. Their presentation is often as obstruction, bleeding, or pain and their treatment is primarily endoscopic or surgical resection when appropriate.

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Chapter 12
Endoscopic Duodenal Resection
CatherineVozzo andAshleyFaulx

Introduction

Endoscopic duodenal resection of lesions and polyps is a complex topic. This is due to the variety of pathology encountered in the duodenum, their proximity to the ampulla, and the unique anatomical considerations within the duodenum including its robust blood supply, thin muscle layer, and scope positioning. In addition, the management of lesions will vary depending on the presence or absence of polyposis syndromes [1]. Careful consideration and thought must be given to each case prior to scheduling a patient for endoscopic resection.

Pre-procedural Considerations

Pre-procedural discussion will include thorough discussion of risks, benets, and alternatives to the procedure. The patient’s comorbidities, surgical history, and med­ications should be reviewed prior to the procedure. Anticoagulant and antiplatelet agents should be held according to society guidelines based on risk of bleeding [2]. The sedation plan should be discussed with the patient and anesthesiologist (if applicable).
C. Vozzo · A. Faulx (*) Division of Gastroenterology, University Hospitals of Cleveland, Case Western Reserve University School of Medicine, Cleveland, OH, USA e-mail: catherine.vozzo@uhhospitals.org; Ashley.faulx@uhhospitals.org
Switzerland AG 2025 E. P. Ceppa et al. (eds.), The SAGES Manual of Evolving Techniques in Pancreatic Surgery, https://doi.org/10.1007/978-3-031-78409-5_12
189© The Author(s), under exclusive license to Springer Nature
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Indications
Careful endoscopic evaluation should take place prior to resection of any lesion. The lesions may appear mucosal or subepithelial. The lesion’s proximity to the ampulla should be noted because endoscopic resection technique is vastly different if the lesion is considered ampullary or non-ampullary. Finally, the patient’s medi­cal history should be evaluated for genetic polyposis syndromes as the management of a sporadic duodenal polyp will differ from a patient with familial adenomatous polyposis or MUTYH-associated polyposis. As discussed in Chap. 11, some duode­nal lesions may harbor malignant potential, whereas others are benign. Lesions should be considered for resection if they are pre-malignant or if they are symptom­atic, such as bleeding or obstruction [1]. Lesions with malignant potential include gastrointestinal stromal tumors, carcinoids, solitary Peutz-Jeghers polyps, leiomyo­mas, and adenomas. Benign lesions include lipomas, gastric metaplasia, inamma­tory polyps, and Brunner’s glands or hamartomas (Fig.12.1) [1, 3].
Table 12.1 outlines a suggested broad approach for various polyps encountered in the duodenum. However, the remainder of this chapter will primarily focus on resection of ampullary and non-ampullary adenomas.
Endoscopic Assessment ofNon-ampullary Adenomas
Detection and characterization of duodenal polyps can be challenging because the duodenum is a xed structure in the retroperitoneum. Distal attachment of a cap to the endoscope may improve stability, allow for better visualization of the papilla, and identify polyps in between folds [4]. It may be necessary to utilize a
Fig. 12.1 Previously bleeding duodenal polyp in second portion of the duodenum, marked with clip and tattoo, pathology ultimately positive for Brunner’s gland hamartoma
12 Endoscopic Duodenal Resection
Table 12.1 Approach by polyp type
Polyp type Resection approach
Non-ampullary adenomas
Ampullary adenomas Endoscopic retrograde cholangiopancreatography and endoscopic
Brunner’s gland hamartoma
Gastric heterotopia No intervention required. Consider H pylori testing Inammatory broid
polyp Lipoma Cold snare polypectomy only if symptomatic Carcinoid If no evidence of invasion of the muscularis layer and 1cm or less in
Gastrointestinal stromal tumors
Solitary Peutz­Jeghers polyp
Table modied from Culver and Mcintyre [3]
<6mm in size: Cold snare polypectomy >6mm in size: Endoscopic mucosal resection (EMR) technique
ultrasound to assess extension with endoscopic papillectomy Cold snare polypectomy or EMR only if symptomatic
Cold snare polypectomy or EMR only if symptomatic
size can be removed via EMR Endoscopic ultrasound-guided FNB for diagnostic purposes, but in most
cases endoscopic resection is not considered (refer to GI surgeon) Cold snare polypectomy
191
Table 12.2 Paris classication (Major variants of type 0 neoplasia)
0-Ip Protruded, pedunculated 0-Is Protruded, sessile 0-IIa Slightly elevated 0-IIb Flat 0-IIc Supercial, shallow, depression 0-III Excavated (ulcer)
Table adapted from Ref. [6]
side- viewing endoscope to clearly outline the location of the polyp in relation to the papilla and should be photo documented prior to intervention [5]. The polyp’s mac­roscopic appearance is typically milk white or reddish mucosa. Once the polyp is identied, tumor size should be estimated with an open biopsy forceps or snare. Inspection using high-denition white light endoscopy and denition should be pro­vided according to the Paris classication (Table12.2) [7]. Endoscopic example of a non-ampullary duodenal adenoma is seen in Fig.12.2.
Histologic assessment varies depending on the region of the world in which you are practicing. When magnifying endoscopy with narrow-band imaging is available, biopsy of the lesion may not be necessary. This technique, not available in the United States, may distinguish neoplastic from non-neoplastic lesions in addition to low- and high-grade dysplasia [8, 9]. Features concerning for advanced histology include the size of the adenoma larger than 1cm, the degree of involved mucosal circumference, and relation to the ampulla. Features concerning for submucosal invasion are Paris IIc, surface ulceration, and non-lifting after submucosal injection (Fig.12.3). The role of EUS in small duodenal adenomas is minimal but may impact the management of polyps <2cm, or in cases where metastases are noted [5].