Abstract
Background
A 28-year-old woman undergoing endoscopy was found to have a carpet-like distribution of innumerable 2-10 mm polyps within the visualized duodenum.
Summary
Our patient was undergoing esophagogastroduodenoscopy (EGD) as part of the routine evaluation for bariatric surgery. This presentation was initially thought to be a rare duodenal manifestation of familial adenomatous polyposis (FAP). Several polyps were biopsied, along with mucosal samples from her stomach and esophagus. The results demonstrated reactive lymphoid follicles within the duodenum, characteristic of nodular lymphoid hyperplasia (NLH). The patient was also found to demonstrate histologic evidence of H. pylori infection.
Conclusion
Nodular lymphoid hyperplasia is characterized by innumerable polyps composed of reactive lymphoid follicles within the duodenum. We report an instance of adult NLH associated with concurrent H. pylori infection. NLH rarely manifests in adults and has been suggested to precede mucosa-associated lymphoid tissue lymphoma. Surveillance by regularly visualizing the upper GI tract removes the Roux-en-Y as an approach for bariatric surgery.
Key Words
nodular lymphoid hyperplasia; familial adenomatous polyposis; duodenitis; Helicobacter pylori; mucosa-associated lymphoid tissue; germinal center hyperplasia
Abbreviations
NLH: Nodular lymphoid hyperplasia
CVID: Common variable immune deficiency
FAP: Familial adenomatous polyposis
EGD: Esophagogastroduodenoscopy
H. pylori: Helicobacter pylori
MALT: Mucosa-associated lymphoid tissue
Case Description
Among the various responsibilities of the gastrointestinal tract is the sampling of luminal contents and reaction to potential threats. In children, hypertrophy of mucosa-associated lymphoid tissue (MALT) after exposure to certain viral antigens may appear as numerous nodules along the duodenal mucosa. This is attributed to a relatively immature adaptive immune system and is seldom seen in adults.1 We reported a rare instance of nodular lymphoid hyperplasia (NLH) found incidentally on esophagogastroduodenoscopy (EGD) in an adult.
Our patient is a 28-year-old female with a history of class III morbid obesity (BMI 56), polycystic ovarian syndrome, iron deficiency anemia, and vitamin D insufficiency. She presented for EGD as part of the routine pre-operative evaluation for bariatric surgery. At every encounter, she denied acid reflux/heartburn, post/pre-prandial nausea, epigastric pain, and indigestion. We proceeded with endoscopy using the Olympus TJFQ180V duodenoscope.
After unremarkably visualizing the esophagus and stomach, the duodenum demonstrated a remarkable carpet-like distribution of 2-10 mm polyps (Figure 1). Biopsies were taken of the lesions, stomach, and distal esophagus. With familial adenomatous polyposis (FAP) as the working diagnosis, our patient was scheduled for a colonoscopy which proved unremarkable, demonstrating a grossly normal appearing colonic mucosa.
Figure 1. Initial EGD Visualizing Second Part of Duodenum With Many Sub-Centimeter Nodules. Published With Permission
The EGD biopsy/pathology report read:
- Duodenum: Chronic duodenitis with reactive lymphoid follicles;
- Stomach: Chronic active gastritis with organisms morphologically consistent with Helicobacter pylori;
- Distal Esophagus: Active esophagitis with focally up to 40 eosinophils per high power field.
The patient was diagnosed with Helicobacter pylori (H. pylori) gastritis and subsequently prescribed omeprazole, amoxicillin, metronidazole, and clarithromycin, a standard eradication therapy. An EGD performed a month later confirmed the successful eradication of H. pylori infection, but the duodenal mucosa retained its ominous morphology (Figure 2).
Figure 2. Follow-Up EGD Visualizing Second Part of Duodenum With Many Sub-Centimeter Nodules. Published With Permission
The Roux-en-Y gastric bypass (RYGB) procedure has proven superior to the sleeve gastrectomy in diabetic populations and for relieving reflux symptoms, and thus was the initial operation of choice for this patient.2 In the RYGB, the proximal 10% of the stomach is divided, leaving a 30-50 cc gastric pouch and a separated gastric remnant. The gastric pouch is then anastomosed to the jejunum (this is the Roux limb). The Roux limb, which is between 100 and 150 cm in length, is then anastomosed to the biliopancreatic limb, combining to form the common channel. In comparison, sleeve gastrectomy involves resecting a large crescent-shaped section of the stomach from the fundus to the antrum, preserving the gastroduodenal junction. The RYGBs resultant anatomic rearrangement prevents traditional endoscopic visualization of the biliopancreatic limb. Considering this, we decided to shift course to a sleeve gastrectomy as the potential progression of NLH to lymphoma warrants regular surveillance of the upper GI tract. She was scheduled for a repeat EGD a year after the operation.
Discussion
NLH is a condition with unknown etiology marked by the appearance of many sub-centimeter nodules distributed along the GI tract (most commonly the small intestine). While it may appear at any age, it is more common in children and very rare in adults. NLH alone does not have well-defined symptomatology and often is an incidental finding on EGD. The chronic inflammatory state present in the duodenum may cause chronic diarrhea, intestinal bleeding, and abdominal pain.3
There is little literature concerning this condition in adults, but etiology has been suggested as divisible into either immune deficiency or immune hyperstimulation. The immune deficiency state in question is one of an adaptive immunity defect. Common variable immune deficiency (CVID) has often been present in reported instances of NLH.4 In CVID, there is a maturational defect of the plasma cells that occupy germinal centers within lymphoid tissue. Those germinal centers within the mucosa of the duodenum then begin to hypertrophy as plasma cell precursors accumulate in an attempt to compensate for functional inadequacy. This ultimately may result in the hyperplastic nodular lymphatic tissue characteristic of NLH.
Conversely, some suggest repetitive stimulation of an adequate GI immune system leads to reactive hyperplasia of lymphatic follicles. This theory is supported by the apparent association between NLH and H. pylori infections. Khuroo et al. identified a relatively large cohort of individuals (40 patients; 23 male, 17 female) displaying NLH and prospectively followed them for five years.5 EGD identified patients with NLH during workup for epigastric pain, vomiting, and weight loss. Remarkably every patient was concurrently infected with H. pylori.
Further strengthening the connection, a significant resolution in the appearance of NLH followed the effective eradication of the infection. Their patients with resistant infections did not show substantial change. Curiously, iron deficiency anemia was a secondary finding in their entire cohort, as it was in our patient.
Malignancy is the greatest concern upon discovery of NLH as the appearance has been linked to lymphoma and may be a pre-malignant lesion disorder, transitioning into MALT lymphoma.3 There is little data to support the rate of transition nor an established protocol for monitoring these patients. Our bariatric procedure recommendation was adjusted to allow easy duodenal visualization and surveillance of her small intestine, given the sparsity of consensus.
Conclusion
NLH presents as the remarkable appearance of many sub-centimeter lymphatic nodules projecting into the lumen of the duodenum, sparing the bulb and often extending no further than its distal border. The condition may follow infection in children, but it is exceedingly rare in adults. Though not well characterized, literature regarding the condition has suggested a relation with or progression to lymphoma. The findings influenced our choice of bariatric procedure to facilitate future monitoring anatomically.
Lessons Learned
Addressing weight to the implications of unknown factors is an exercise of experience and education demonstrated in the break from algorithmic medicine that is a physician's judgment call. A fluid approach to a rigid outcome is key when accounting for rare findings in clinical decision-making, underscoring the importance of individualized medicine.
Authors
Idrissi FRa; Serrano Lab; DuCoin Cab
Author Affiliations
- University of South Florida Morsani College of Medicine, Tampa, FL 33612
- Tampa General Hospital Department of Surgery, Tampa, FL 33606
Corresponding Author
Fahd Rahmouni Idrissi
8603 North 29th Street
Tampa, Florida 33604
Phone: (727) 488-5824
Email: rahmouni@usf.edu
Disclosure Statement
The authors have no conflicts of interest to disclose.
Funding/Support
The authors have no relevant financial relationships or in-kind support to disclose.
Received: September 25, 2020
Revision received: November 28, 2020
Accepted: December 7, 2020
References
- Albuquerque A. Nodular lymphoid hyperplasia in the gastrointestinal tract in adult patients: A review. World J Gastrointest Endosc. 2014;6(11):534-540. doi:10.4253/wjge.v6.i11.534
- Li JF, Lai DD, Ni B, Sun KX. Comparison of laparoscopic Roux-en-Y gastric bypass with laparoscopic sleeve gastrectomy for morbid obesity or type 2 diabetes mellitus: a meta-analysis of randomized controlled trials. Can J Surg. 2013;56(6):E158-E164. doi:10.1503/cjs.026912
- Elkholy S, Mogawer S, Farag A. Nodular Lymphoid Hyperplasia of the Gastrointestinal Tract : a comprehensive review. Acta Gastroenterol Belg. 2017;80(3):405-410.
- Choi JH, Han DS, Kim J, Yi K, Oh YH, Kim Y. Diffuse Nodular Lymphoid Hyperplasia of the Intestine Caused by Common Variable Immunodeficiency and Refractory Giardiasis. Intern Med. 2017;56(3):283-287. doi:10.2169/internalmedicine.56.7305
- Khuroo MS, Khuroo NS, Khuroo MS. Diffuse duodenal nodular lymphoid hyperplasia: a large cohort of patients etiologically related to Helicobacter pylori infection. BMC Gastroenterol. 2011;11:36. Published 2011 Apr 11. doi:10.1186/1471-230X-11-36
