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Pyloric Duplication and Associated Information

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Pyloric duplication is a rare congenital anomaly of the alimentary canal, and its clinical presentation usually imitates those of hypertrophic pyloric stenosis.

Medically reviewed by

Dr. Ghulam Fareed

Published At October 16, 2023
Reviewed AtOctober 16, 2023

Introduction:

Enteric duplications are not very common among congenital anomalies. The anomaly of stomach duplications is involved in only 3.6 to 3.9 % of gastrointestinal duplications. The duplication involving the pylorus is extremely rare. A firm attachment can be seen to at least one point of the alimentary tract with a well-evolved coat of smooth muscle and mucous membrane. Generally, an abdominal mass with vomiting is mainly discovered during the initial 12 months of life.

What Is Pyloric Duplication?

A pyloric duplication cyst is not a common congenital anomaly. It is an extremely rare anomaly of the alimentary canal. The clinical presentation usually imitates those of hypertrophic pyloric stenosis. Gastrointestinal duplications can be seen in 1 of every 4000 to 5000 autopsies. Gastrointestinal duplication is a rare congenital lesion that develops anywhere in the alimentary tract and may be present in the infant as an abdominal mass. A pyloric duplication is different from intra-abdominal cystic lesions with the presence of a normal gastrointestinal mucosal lining. The symptoms are generally gastric outlet obstruction and a palpable mass. This palpable mass is difficult to differentiate clinically from infantile hypertrophic pyloric stenosis. Infantile hypertrophic pyloric stenosis, as the name suggests, occurs in young infants. It is a disorder characterized by the hypertrophy of the pylorus, which may eventually lead to complete obstruction of the gastric outlet, which causes forceful vomiting. Enteric duplications may occur at any part of the gastrointestinal tract. Still, these types of duplications are commonly found in the ileum and could be called the rarest along the pyloric channel.

What Are Gastrointestinal Duplications?

Gastrointestinal duplications are rare to be seen types of congenital malformations. They vary at an extensive level in terms of presentation, size, location, and symptoms. The condition of gastrointestinal duplication has the following characteristics:

  • A properly developed coat of smooth muscle is present.

  • A portion of the alimentary tract is represented by an epithelial lining.

  • To some portion of the gastrointestinal tract. Duplications are generally intimately attached.

The reason behind the gastrointestinal tract duplications is yet not known. Various theories have come forth, but none have been accepted by everyone. The hypothesis is that the initial developmental abnormality can be seen in the gastrulation stage, resulting in a split notochord. At the early stage of embryogenesis, the notochord stays open, and the fusion of the yolk sac's endoderm and the notochord's ectoderm happens. A tube known as the neurenteric canal connects the yolk sac and the amnion. As a section of the development of the split notochord, an endodermal-ectodermal adhesion between the cord has been put forth to result in the continuation of an endo-mesenchymal tract between the yolk sac and amnion. The endo-mesenchymal tract that is formed is the cause behind the anomalies of the gastrointestinal tract system. But, this theory does not suit the example of every duplication, and other theories have been proposed too. A few of the duplications seen of the foregut and hindgut can result from partial twinning. Partial twinning is a group of rare congenital abnormalities of cloacal-derived structures such as intestinal, reproductive, and some other parts of the human body. Due to persistent embryologic diverticula, some other duplications, such as of the ileum, may occur. Aberrant luminal recanalizations can be the reason behind some portions of the intestinal tract having a solid stage during development. In the end, it can be said that intrauterine environmental factors like trauma or hypoxia during any vascular accident are the causative factors for the duplications at any part of the gastrointestinal tract.

What Is Pylorus?

Pylorus is elaborated as a conical constriction in the gastrointestinal tract that specifies the end of the stomach and the beginning of the small intestine. The primary function of the pylorus is to act as a barrier between the stomach and the small intestine, as it prevents the intestinal contents from re-entering the stomach when the small intestine contraction happens. It also limits the entry of large food particles or undigested material into the intestine. The gastric juice is secreted by the mucous membrane lining, which constitutes the internal surface of the pylorus. Right beneath the lining of the mucous membrane, circular muscle tissue allows the pyloric sphincter to open and close in order to permit food to pass or be retained. The pyloric sphincter maintains itself in an open or relaxed state around 2/3rd of the time. It permits small quantities of food to pass into the duodenum, the upper portion of the small intestine. When the duodenum gets full or starts to get filled, the pressure increases, and the pyloric sphincter contracts and closes. Muscular contractions, also known as peristaltic waves in the duodenum, push food deeper into the intestine. The relatively narrow pyloric sphincter allows only small amounts of well-emulsified food to pass through, even when it is open. The term pylorus is referred to the pyloric sphincter and can also be applied to the part of the stomach that is situated right above the pyloric sphincter.

What Are the Clinical Findings in the Patients With Pyloric Duplications?

The clinical findings in the cases suffering from pyloric duplications are given in the following points:

  • Abdominal pain of recurrent nature.

  • Vomiting and nausea.

  • Melena is known as blackened stool often seen while or with gastrointestinal bleeding.

  • Abdominal distension.

  • Recurrent pancreatitis.

  • Loss of weight

  • Gastric outlet obstruction

  • Loss of appetite.

  • Other digestive struggles.

Conclusion:

Pyloric duplication in pediatric patients is uncommon and cannot be explained with specific clinical manifestations. It is difficult to diagnose and confirm the condition. A thorough investigation should be considered for a few cases with non-specific clinical symptoms and auxiliary examinations. Often the diagnosis requires exploration with surgery in combination with a proper pathological examination. An examination with a computed tomography scan and ultrasounds should be done. This disease may eventually lead to serious complications, so surgery should be performed as soon as possible after the diagnosis is confirmed. Generally, resection through surgery is the only method to manage and treat duplications. It was observed that in adult patients, this disease has the potential to get transformed into a malignancy. Hence, surgical intervention is needed in those cases as soon as possible.

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Dr. Ghulam Fareed
Dr. Ghulam Fareed

Medical Gastroenterology

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