Meckel's diverticulum

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Meckel's diverticulum
Diverticule de Meckel.jpg
Schematic drawing of a Meckel's diverticulum with a part of the small intestine.
Specialty Medical genetics   OOjs UI icon edit-ltr-progressive.svg
Named after Johann Friedrich Meckel

A Meckel's diverticulum, a true congenital diverticulum, is a slight bulge in the small intestine present at birth and a vestigial remnant of the vitelline duct. It is the most common malformation of the gastrointestinal tract and is present in approximately 2% of the population, [1] with males more frequently experiencing symptoms.

Contents

Meckel's diverticulum was first explained by Fabricius Hildanus in the sixteenth century and later named after Johann Friedrich Meckel, who described the embryological origin of this type of diverticulum in 1809. [2] [3]

Signs and symptoms

The majority of people with a Meckel's diverticulum are asymptomatic. An asymptomatic Meckel's diverticulum is called a silent Meckel's diverticulum. [4] If symptoms do occur, they typically appear before the age of two years. [5] The most common presenting symptom is painless rectal bleeding such as melaena-like black offensive stools, followed by intestinal obstruction, volvulus and intussusception. Occasionally, Meckel's diverticulitis may present with all the features of acute appendicitis. [6] Also, severe pain in the epigastric region is experienced by the person along with bloating in the epigastric and umbilical regions. At times, the symptoms are so painful that they may cause sleepless nights with acute pain felt in the foregut region, specifically in the epigastric and umbilical regions.[ citation needed ] In some cases, bleeding occurs without warning and may stop spontaneously. The symptoms can be extremely painful, often mistaken as just stomach pain resulting from not eating or constipation.[ citation needed ] Rarely, a Meckel's diverticulum containing ectopic pancreatic tissue can present with abdominal pain and increased serum amylase levels, mimicking acute pancreatitis. [7]

Complications

The lifetime risk for a person with Meckel's diverticulum to develop certain complications is about 4–6%. Gastrointestinal bleeding, peritonitis or intestinal obstruction may occur in 15–30% of symptomatic people (Table 1). On rare occasions the diverticulum can herniate through the abdominal wall also known as a Littre hernia. Only 6.4% of all complications require surgical treatment, and untreated Meckel's diverticulum has a mortality rate of 2.5–15%. [8]

Table 1 – Complications of Meckel's Diverticulum: [9]

ComplicationsPercentage of symptomatic Meckel's Diverticulum (%)
Haemorrhage 20–30
Intestinal obstruction 20–25
Diverticulitis 10–20
Umbilical anomalies≤10
Neoplasm 0.5-2

Bleeding

Bleeding of the diverticulum is most common in young children, especially in males who are less than 2 years of age. [10] Symptoms may include bright red blood in stools (hematochezia), weakness, abdominal tenderness or pain, and even anaemia in some cases. [11]

Bleeding may be caused by:

  • Ectopic gastric or pancreatic mucosa:
  1. Where diverticulum contains embryonic remnants of mucosa of other tissue types.
  2. Secretion of gastric acid or alkaline pancreatic juice from the ectopic mucosa leads to ulceration in the adjacent ileal mucosa i.e. peptic or pancreatic ulcer. [12]
  3. Pain, bleeding or perforation of the bowel at the diverticulum may result.
  4. Mechanical stimulation may also cause erosion and ulceration.

The appearance of stools may indicate the nature of the bleeding:

  • Tarry stools: Alteration of blood produced by slow bowel transit due to minor bleeding in upper gastrointestinal tract
  • Bright red blood stools: Brisk bleeding
  • Stools with blood streak: Anal fissure
  • "Currant jelly" stools: Ischaemia of the intestine leads to copious mucus production and may indicate that one part of the bowel invaginates into another intussusception.

Diverticulitis

Inflammation of the diverticulum can mimic symptoms of appendicitis, i.e., periumbilical tenderness and intermittent crampy abdominal pain. Perforation of the inflamed diverticulum can result in peritonitis. Diverticulitis can also cause adhesions, leading to intestinal obstruction. [14]

Diverticulitis may result from:

  • Association with the mesodiverticular band attaching to the diverticulum tip where torsion has occurred, causing inflammation and ischaemia. [15]
  • Peptic ulceration resulting from ectopic gastric mucosa of the diverticulum
  • Following perforation by trauma or ingested foreign material e.g. stalk of vegetable, seeds or fish/chicken bone that become lodged in Meckel's diverticulum. [16]
  • Luminal obstruction due to tumors, enterolith, foreign body, causing stasis or bacterial infection. [17]
  • Association with acute appendicitis [6]

Intestinal obstruction

Symptoms: Vomiting, abdominal pain and severe or complete constipation. [18]

  • The vitelline vessels remnant that connects the diverticulum to the umbilicus may form a fibrous or twisting band (volvulus), trapping the small intestine and causing obstruction. Localised periumbilical pain may be experienced in the right lower quadrant (like appendicitis). [17]
  • "Incarceration": when a Meckel's diverticulum is constricted in an inguinal hernia, forming a Littré hernia that obstructs the intestine. [19]
  • Chronic diverticulitis causing stricture
  • Strangulation of the diverticulum in the obturator foramen.
  • Tumors e.g. carcinoma: direct spread of an adenocarcinoma arising in the diverticulum may lead to obstruction
  • Lithiasis, stones that are formed in Meckel's diverticulum can:
  1. Extrude into the terminal ileum, leading to obstruction
  2. Induce local inflammation and intussusception. [17]
  • The diverticulum itself or tumour within it may cause intussusception. For example, from the ileum to the colon, causing obstruction. Symptoms of this include "currant jelly" stools and a palpable lump in the lower abdomen. [9] This occurs when the diverticulum inverts into the lumen of the ileum, due to either:
  1. An active peristaltic mechanism of the diverticulum that attempts to remove irritating factors
  2. A passive process such as the transit of food [11]

Umbilical anomalies

Anomalies between the diverticulum and umbilicus may include the presence of fibrous cord, cyst, fistula, or sinus, leading to: [14]

  • Infection or excoriation of periumbilical skin, resulting in a discharging sinus
  • Recurrent infection and healing of sinus
  • Abscess formation in the abdominal wall
  • Fibrous cord increases the risk of volvulus formation and internal herniation

Tumors

Neoplasms (tumors) in Meckel's diverticulum may cause bleeding, acute abdominal pain, gastrointestinal obstruction, perforation or intussusception. They may be benign or malignant. [14]

  • Benign tumors:
  1. Leiomyoma
  2. Lipoma
  3. Vascular and neuromuscular hamartoma
  • Malignant tumors:
  1. Carcinoids: most common, 44%
  2. Mesenchymal tumors: Leiomyosarcoma, peripheral nerve sheath and gastrointestinal stromal tumors, 35%
  3. Adenocarcinoma, 16%
  4. Desmoplastic small round cell tumor

Other complications

  • A diverticulum inside a Meckel's diverticulum (daughter diverticula)
  • Stones and phytobezoar (a bezoar of vegetable fibers) in Meckel's diverticulum
  • Vesicodiverticular fistula [9]

Pathophysiology

Meckel's diverticulum surgical specimen Meckel's Diverticulum AFIP.jpg
Meckel's diverticulum surgical specimen

The omphalomesenteric duct (omphaloenteric duct, vitelline duct, or yolk stalk) normally connects the embryonic midgut to the yolk sac ventrally, providing nutrients to the midgut during embryonic development. The vitelline duct narrows progressively and disappears between the 5th and 8th weeks of gestation.[ citation needed ]

In Meckel's diverticulum, the proximal part of vitelline duct fails to regress and involute, which remains as a remnant of variable length and location. [16] The solitary diverticulum lies on the antimesenteric border of the ileum (opposite to the mesenteric attachment) and extends into the umbilical cord of the embryo. [8] The left and right vitelline arteries originate from the primitive dorsal aorta, and travel with the vitelline duct. The right becomes the superior mesenteric artery that supplies a terminal branch to the diverticulum, while the left involutes. [17] Having its own blood supply, Meckel's diverticulum is susceptible to obstruction or infection.

Meckel's diverticulum is located in the distal ileum, usually within 60–100 cm (2 feet) of the ileocecal valve. This blind segment or small pouch is about 3–6 cm (2 inch) long and may have a greater lumen diameter than that of the ileum. [20] It runs antimesenterically and has its own blood supply. It is a remnant of the connection from the yolk sac to the small intestine present during embryonic development. It is a true diverticulum, consisting of all 3 layers of the bowel wall which are mucosa, submucosa and muscularis propria. [17]

As the vitelline duct is made up of pluripotent cell lining, Meckel's diverticulum may harbor abnormal tissues, containing embryonic remnants of other tissue types. Jejunal, duodenal mucosa or Brunner's tissue were each found in 2% of ectopic cases. Heterotopic rests of gastric mucosa and pancreatic tissue are seen in 60% and 6% of cases respectively. Heterotopic means the displacement of an organ from its normal anatomic location. [21] Inflammation of this Meckel's diverticulum may mimic appendicitis. Therefore, during appendectomy, ileum should be checked for the presence of Meckel's diverticulum, if it is found to be present it should be removed along with appendix.[ citation needed ]

A memory aid is the rule of 2s: [22]

However, the exact values for the above criteria range from 0.2–5 (for example, prevalence is probably 0.2–4%).[ citation needed ]

It can also be present as an indirect hernia, typically on the right side, where it is known as a "Hernia of Littré ". A case report of strangulated umbilical hernia with Meckel's diverticulum has also been published in the literature. [23] Furthermore, it can be attached to the umbilical region by the vitelline ligament, with the possibility of vitelline cysts, or even a patent vitelline canal forming a vitelline fistula when the umbilical cord is cut. Torsions of intestine around the intestinal stalk may also occur, leading to obstruction, ischemia, and necrosis.

Diagnosis

Technetium-99m Pertechnetate Scan with a Meckel's Diverticulum. Meckel's Diverticulum by Technetium-99m Pertechnetate Scan.jpg
Technetium-99m Pertechnetate Scan with a Meckel's Diverticulum.

A technetium-99m (99mTc) pertechnetate scan, also called Meckel scan or nuclear scintigraphy scan, is the investigation of choice to diagnose Meckel's diverticula in children. This scan detects gastric mucosa; since approximately 50% of symptomatic Meckel's diverticula have ectopic gastric or pancreatic cells contained within them, [24] this is displayed as a spot on the scan distant from the stomach itself. In children, this scan is highly accurate and noninvasive, with 95% specificity and 85% sensitivity; [17] however, in adults the test is only 9% specific and 62% sensitive. [25] This scan is more accurate in children because gastric mucosa is found in 90% of bleeding diverticula; which is the most common symptom in children, not adults. [26]

Patients with these misplaced gastric cells may experience peptic ulcers as a consequence. Therefore, other tests such as colonoscopy and screenings for bleeding disorders should be performed, and angiography can assist in determining the location and severity of bleeding. Colonoscopy might be helpful to rule out other sources of bleeding but it is not used as an identification tool.[ citation needed ]

Angiography of a Meckel's diverticulum that presented with bleeding. Angiography of a Meckel's diverticulum that presented with bleeding.jpg
Angiography of a Meckel's diverticulum that presented with bleeding.

Angiography might identify brisk bleeding in patients with Meckel's diverticulum. [17]

Ultrasonography could demonstrate omphaloenteric duct remnants or cysts. [27] Computed tomography (CT scan) might be a useful tool to demonstrate a blind ended and inflamed structure in the mid-abdominal cavity, which is not an appendix. [17]

In asymptomatic patients, Meckel's diverticulum is often diagnosed as an incidental finding during laparoscopy or laparotomy.[ citation needed ]

Treatment

Treatment is surgical, potentially with a laparoscopic resection. [17] In patients with bleeding, strangulation of bowel, bowel perforation or bowel obstruction, treatment involves surgical resection of both the Meckel's diverticulum itself along with the adjacent bowel segment, and this procedure is called a "small bowel resection". [17] In patients without any of the aforementioned complications, treatment involves surgical resection of the Meckel's diverticulum only, and this procedure is called a simple diverticulectomy. [17]

With regards to asymptomatic Meckel's diverticulum, some recommend that a search for Meckel's diverticulum should be conducted in every case of appendectomy/laparotomy done for acute abdomen, and if found, Meckel's diverticulectomy or resection should be performed to avoid secondary complications arising from it. [28]

Epidemiology

Meckel's diverticulum occurs in about 2% of the population. [21] Prevalence in males is 3–5 times higher than in females. [20] Only 2% of cases are symptomatic, which usually presents among children at the age of 2. [8]

Most cases of Meckel's diverticulum are diagnosed when complications manifest or incidentally in unrelated conditions such as laparotomy, laparoscopy or contrast study of the small intestine. Classic presentation in adults includes intestinal obstruction and inflammation of the diverticulum (diverticulitis). Painless rectal bleeding most commonly occurs in toddlers. [5]

Inflammation in the ileal diverticulum has symptoms that mimic appendicitis, therefore its diagnosis is of clinical importance. Detailed knowledge of the pathophysiological properties is essential in dealing with the life-threatening complications of Meckel's diverticulum. [17]

Related Research Articles

<span class="mw-page-title-main">Gastrointestinal tract</span> Organ system within humans and other animals

The gastrointestinal tract is the tract or passageway of the digestive system that leads from the mouth to the anus. The GI tract contains all the major organs of the digestive system, in humans and other animals, including the esophagus, stomach, and intestines. Food taken in through the mouth is digested to extract nutrients and absorb energy, and the waste expelled at the anus as faeces. Gastrointestinal is an adjective meaning of or pertaining to the stomach and intestines.

<span class="mw-page-title-main">Small intestine</span> Organ in the gastrointestinal tract

The small intestine or small bowel is an organ in the gastrointestinal tract where most of the absorption of nutrients from food takes place. It lies between the stomach and large intestine, and receives bile and pancreatic juice through the pancreatic duct to aid in digestion. The small intestine is about 5.5 metres long and folds many times to fit in the abdomen. Although it is longer than the large intestine, it is called the small intestine because it is narrower in diameter.

<span class="mw-page-title-main">Bowel obstruction</span> Medical condition

Bowel obstruction, also known as intestinal obstruction, is a mechanical or functional obstruction of the intestines which prevents the normal movement of the products of digestion. Either the small bowel or large bowel may be affected. Signs and symptoms include abdominal pain, vomiting, bloating and not passing gas. Mechanical obstruction is the cause of about 5 to 15% of cases of severe abdominal pain of sudden onset requiring admission to hospital.

<span class="mw-page-title-main">Abdominal pain</span> Stomach aches

Abdominal pain, also known as a stomach ache, is a symptom associated with both non-serious and serious medical issues. Since the abdomen contains most of the body's vital organs, it can be an indicator of a wide variety of diseases. Given that, approaching the examination of a person and planning of a differential diagnosis is extremely important.

<span class="mw-page-title-main">Diverticulosis</span> Condition of the wall of the intestine

Diverticulosis is the condition of having multiple pouches (diverticula) in the colon that are not inflamed. These are outpockets of the colonic mucosa and submucosa through weaknesses of muscle layers in the colon wall. Diverticula do not cause symptoms in most people. Diverticular disease occurs when diverticula become clinically inflamed, a condition known as diverticulitis.

<span class="mw-page-title-main">Gastrointestinal disease</span> Medical condition

Gastrointestinal diseases refer to diseases involving the gastrointestinal tract, namely the esophagus, stomach, small intestine, large intestine and rectum, and the accessory organs of digestion, the liver, gallbladder, and pancreas.

<span class="mw-page-title-main">Intussusception (medical disorder)</span> Medical condition

Intussusception is a medical condition in which a part of the intestine folds into the section immediately ahead of it. It typically involves the small bowel and less commonly the large bowel. Symptoms include abdominal pain which may come and go, vomiting, abdominal bloating, and bloody stool. It often results in a small bowel obstruction. Other complications may include peritonitis or bowel perforation.

<span class="mw-page-title-main">Volvulus</span> Twisting of part of the intestine, causing a bowel obstruction

A volvulus is when a loop of intestine twists around itself and the mesentery that supports it, resulting in a bowel obstruction. Symptoms include abdominal pain, abdominal bloating, vomiting, constipation, and bloody stool. Onset of symptoms may be rapid or more gradual. The mesentery may become so tightly twisted that blood flow to part of the intestine is cut off, resulting in ischemic bowel. In this situation there may be fever or significant pain when the abdomen is touched.

<span class="mw-page-title-main">Diverticular disease</span> Medical condition

Diverticular disease is when problems occur due to diverticulosis, a benign condition defined by the formation of pouches (diverticula) from the weak spots in the wall of the large intestine. This disease spectrum includes diverticulitis, symptomatic uncomplicated diverticular disease (SUDD), and segmental colitis associated with diverticulosis (SCAD). The most common symptoms across the disease spectrum are abdominal pain and bowel habit changes such as diarrhea or constipation. Otherwise, diverticulitis presents with systemic symptoms such as fever and elevated white blood cell count whereas SUDD and SCAD don’t. Treatment ranges from conservative bowel rest to medications such as antibiotics, antispasmodics, acetaminophen, mesalamine, rifaximin, and corticosteroids depending on the specific conditions.

<span class="mw-page-title-main">Gastrointestinal perforation</span> Medical condition

Gastrointestinal perforation, also known as gastrointestinal rupture, is a hole in the wall of the gastrointestinal tract. The gastrointestinal tract is composed of hollow digestive organs leading from the mouth to the anus. Symptoms of gastrointestinal perforation commonly include severe abdominal pain, nausea, and vomiting. Complications include a painful inflammation of the inner lining of the abdominal wall and sepsis.

<span class="mw-page-title-main">Blood in stool</span> Medical condition

Blood in stool or rectal bleeding looks different depending on how early it enters the digestive tract—and thus how much digestive action it has been exposed to—and how much there is. The term can refer either to melena, with a black appearance, typically originating from upper gastrointestinal bleeding; or to hematochezia, with a red color, typically originating from lower gastrointestinal bleeding. Evaluation of the blood found in stool depends on its characteristics, in terms of color, quantity and other features, which can point to its source, however, more serious conditions can present with a mixed picture, or with the form of bleeding that is found in another section of the tract. The term "blood in stool" is usually only used to describe visible blood, and not fecal occult blood, which is found only after physical examination and chemical laboratory testing.

<span class="mw-page-title-main">Vitelline duct</span>

In the human embryo, the vitelline duct, also known as the vitellointestinal duct, the yolk stalk, the omphaloenteric duct, or the omphalomesenteric duct, is a long narrow tube that joins the yolk sac to the midgut lumen of the developing fetus. It appears at the end of the fourth week, when the yolk sac presents the appearance of a small pear-shaped vesicle.

<span class="mw-page-title-main">Distal intestinal obstruction syndrome</span> Medical condition

Distal intestinal obstruction syndrome (DIOS) involves obstruction of the distal part of the small intestines by thickened intestinal content and occurs in about 20% of mainly adult individuals with cystic fibrosis. DIOS was previously known as meconium ileus equivalent, a name which highlights its similarity to the intestinal obstruction seen in newborn infants with cystic fibrosis. DIOS tends to occur in older individuals with pancreatic insufficiency. Individuals with DIOS may be predisposed to bowel obstruction, though it is a separate entity than true constipation.

<span class="mw-page-title-main">Bowel resection</span> Surgical procedure in which a part of an intestine is removed

A bowel resection or enterectomy is a surgical procedure in which a part of an intestine (bowel) is removed, from either the small intestine or large intestine. Often the word enterectomy is reserved for the sense of small bowel resection, in distinction from colectomy, which covers the sense of large bowel resection. Bowel resection may be performed to treat gastrointestinal cancer, bowel ischemia, necrosis, or obstruction due to scar tissue, volvulus, and hernias. Some patients require ileostomy or colostomy after this procedure as alternative means of excretion. Complications of the procedure may include anastomotic leak or dehiscence, hernias, or adhesions causing partial or complete bowel obstruction. Depending on which part and how much of the intestines are removed, there may be digestive and metabolic challenges afterward, such as short bowel syndrome.

<span class="mw-page-title-main">Hemosuccus pancreaticus</span> Medical condition

Hemosuccus pancreaticus is a rare cause of hemorrhage in the gastrointestinal tract. It is caused by a bleeding source in the pancreas, pancreatic duct, or structures adjacent to the pancreas, such as the splenic artery, that bleed into the pancreatic duct, which is connected with the bowel at the duodenum, the first part of the small intestine. Patients with hemosuccus may develop symptoms of gastrointestinal hemorrhage, such as blood in the stools, maroon stools, or melena, which is a dark, tarry stool caused by digestion of red blood cells. They may also develop abdominal pain. It is associated with pancreatitis, pancreatic cancer and aneurysms of the splenic artery. Hemosuccus may be identified with endoscopy (esophagogastroduodenoscopy), where fresh blood may be seen from the pancreatic duct. Alternatively, angiography may be used to inject the celiac axis to determine the blood vessel that is bleeding. This may also be used to treat hemosuccus, as embolization of the end vessel may terminate the hemorrhage. However, a distal pancreatectomy—surgery to remove of the tail of the pancreas—may be required to stop the hemorrhage.

<span class="mw-page-title-main">Epiploic appendagitis</span> Medical condition

Epiploic appendagitis (EA) is an uncommon, benign, self-limiting inflammatory process of the epiploic appendices. Other, older terms for the process include appendicitis epiploica and appendagitis, but these terms are used less now in order to avoid confusion with acute appendicitis.

Abdominal guarding is the tensing of the abdominal wall muscles to guard inflamed organs within the abdomen from the pain of pressure upon them. The tensing is detected when the abdominal wall is pressed. Abdominal guarding is also known as 'défense musculaire'.

Jejunoileal bypass (JIB) was a surgical weight-loss procedure performed for the relief of morbid obesity from the 1950s through the 1970s in which all but 30 cm (12 in) to 45 cm (18 in) of the small bowel were detached and set to the side.

Hinchey Classification is used to describe perforations of the colon due to diverticulitis. The classification was developed by Dr. E John Hinchey (1934–present), a general surgeon at the Montreal General Hospital and professor of surgery at McGill University.

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