Intestinal Duplications or Enteric Duplication cyst signifies one of the many congenital but uncommon abnormalities of the gastrointestinal tract. It is a rare cause of critical abdominal pain and remains a serious concern for the pediatricians, as most of the cases are detected in the pediatric population. Intestinal Duplications or Enteric Duplication are a specific tubular structures that remain attached to the intestines and share similar blood supply like that of intestines; their lining just look like that of the GI tract. The most common occurrence of duplication is found in the jejunum and ileum followed by the colon, stomach, duodenum, and esophagus. Intestinal Duplications or Enteric Duplication usually observed in the 1st or 2nd year of life. In many instances, duplications can be asymptomatic or can cause obstructive symptoms leading to chronic pain, or feeling of abdominal mass. In most of the cases, if no other physical problems are obvious, surgery is the only option with complete resection of the duplicated part.
Types of Intestinal Duplication or Enteric Duplication
Intestinal Duplications or Enteric Duplication can be differentiated into the following types –
- Cervical Duplications: It signifies the duplications of esophagus.
- Thoracic and Thoracoabdominal Duplications: These duplications are detected in the thoracic or thoracoabdominal regions. In majority of the cases, a second or third duplication cyst below the diaphragm is also detected.
- Gastric Duplications: These duplications are generally cystic in nature and mostly found on the greater curvature and don’t possess any connection with the stomach.
- Small Bowel Duplications: Small bowel duplication is associated with all three small bowel subtypes, viz. duodenal, jejunal, and ileal. Among these three, Jejunal duplications comprise 50% of small bowel duplications, followed by almost 40% of ileal and 10% of duodenal duplications.
- Large Bowel Duplications: These may be of two types viz. Cystic colonic duplications and tubular colonic duplications.
- Cystic Colonic Duplications: These are isolated types of duplications or may have an external fistula to the skin, urinary tract, or normal colon.
- Tubular Colonic Duplications: Tubular duplication of the colon is normally linked with duplication of the anus, vagina, and penis.
Apart from these two types of duplications, multiple short-segment duplications of the colon may also be present.
- Rectal Duplications: These types of duplications are detected in rectal space.
Symptoms of Intestinal Duplication or Enteric Duplication
Symptoms of this condition depend on the location of Intestinal Duplications or Enteric Duplication. Here are the common symptoms of Intestinal Duplications or Enteric Duplication depending on the location of the problem –
- Patients with cervical duplication cysts are usually asymptomatic but sometimes can develop symptoms like dysphagia or chest pain due to mild to severe compression of surrounding structures. Patients with this issue may also face breathing problems.
- In case of thoracic or thoracoabdominal duplication cysts, patients may complain of chest pain, dysphagia, shortness of breath and abdominal pain etc.
- Patients with gastric duplication cysts can be asymptomatic but in some cases they may develop symptoms like diffuse abdominal pain, weight loss, epigastric pain, vomiting, gastric outlet obstruction, and formation of ulcerated antral mass etc.
- Patients with small bowel duplications complain of abdominal pain. The duplication cysts may also lead to vomiting and melena.
- Colonic duplication cysts can be asymptomatic, but in many occasions mild to severe abdominal pain may occur along with obstruction, and bleeding. The lesions may be asymptomatic, but the gastrointestinal bleeding can occur if ectopic gastric mucosa ulcerates and erodes into adjacent organs or vessels.
Epidemiology of Intestinal Duplication or Enteric Duplication
Intestinal Duplications or Enteric Duplication are detected in 1 of every 4500 autopsies, mostly in American and European white male child. Though the problem can be traced at any age; till to-date, 80% of cases are detected in infants before they attain the age of 2. Relative incidences of the various types of intestinal duplication are as follows:
- Cervical Duplications: These types of duplication cyst are rarest of the rare case and the most unusual enteric duplication. Till date only 10 such cases are reported.
- Thoracic and Thoracoabdominal Duplications: This type of enteric duplication normally makes up 4% of all enteric duplications.
- Gastric Duplications: These account for 7-8% of all reported intestinal duplications worldwide.
- Small Bowel Duplications: These are the most frequently reported duplications that account for 45% of total cases reported worldwide.
- Large Bowel Duplications: These may be either cystic or tubular types. They represent 20% of total duplications as per the world report available.
Diagnosis of Intestinal Duplication or Enteric Duplication
If the duplication cyst is doubted in the esophageal region, a chest x-ray is needed. If the esophageal duplication is present, the x-ray will show accumulation of some mass in the posterior chest. In other cases, USG including Endoscopic USG (EUS), CT or MRI scan may be needed to ensure the presence of duplication cysts. In pediatric cases, if there is a history of regular bleeding in the stool is reported, a Technetium scan is undertaken.
Treatment of Intestinal Duplication or Enteric Duplication
Here are the commonly undertaken treatment procedures in different types of Intestinal Duplications or Enteric Duplication –
- Treatment for Cervical Duplication Cysts: In most of the symptomatic cases, the surgical removal method is commonly undertaken. In asymptomatic cases, the cyst may lead to ulceration; hence surgery can give permanent relief. In some asymptomatic cases, laparoscopic surgery of esophageal cyst is also undertaken successfully.
- Treatment for Gastric Duplication Cysts: There have been too few cases reported of gastric cancer arising from gastric duplication cysts to date. Even though, there remains a minimal possibility of cancer out of gastric duplication cysts, many experts decide to undertake surgery for these lesions in asymptomatic patients. On the other hand, other experts prefer traditional treatment procedures since malignant transformation of these lesions is very remote.
- Treatment for Thoracic and Thoracoabdominal Duplication Cysts: With regards to treatment in case of Thoracic and thoracoabdominal duplications, surgery is the treatment of choice in all symptomatic cases. In asymptomatic cases too, surgical resections are suggested due to the rare development of malignancy, but many other experts prefer to observe these lesions.
- Treatment for Small Bowel Duplication Cysts: Duodenal cysts are typically treated with surgical resection. In some instances, endoscopic treatment has been undertaken where the duplication is in close proximity to the major duodenal papillae. In case of ileal and jejunal duplications, even if these are asymptomatic, surgeries are undertaken.
- Treatment for Large Bowel Duplication Cysts: Regarding the large bowel duplication cysts, experts undertake surgeries due to potential threat of perforations, bleedings, and obstructions etc. In some extreme situations, malignant degeneration is also possible.
Prognosis of Intestinal Duplication or Enteric Duplication
The consequences of surgical and traditional management of Intestinal Duplications or Enteric Duplication are quite satisfactory. Metaplasia that commonly occurs in any untreated Intestinal Duplications or Enteric Duplication can be successfully prevented, depending on the position of such duplication, through appropriate surgical interference. The severity and types of abnormalities accompanying in the form of Intestinal Duplications or Enteric Duplication play important roles in determining the extent of the disease and mortality rate.
Intestinal Duplications or Enteric Duplication are rare congenital lesions in people. Some patients with Intestinal Duplications or Enteric Duplication may remain asymptomatic while others can show symptoms like abdominal pain, bleeding, and chest pain etc. Diagnostic procedures undertaken for detecting the location and condition of the cysts can spot the positions and state of these duplication cysts quite accurately. Once the finding of a duplication cyst is established beyond doubt, treatment depends on the occurrence of symptoms. In symptomatic cases, surgical resection is obvious but in asymptomatic cases surgical resections may not be required and depends on the doctors judgment of the condition.