Background

Meckel diverticulum (also referred to as Meckel's Diverticulum) is the most common congenital abnormality of the small intestine; it is caused by an incomplete obliteration of the vitelline duct (ie, omphalomesenteric duct). Although originally described by Fabricius Hildanus in 1598, it is named after Johann Friedrich Meckel, who established its embryonic origin in 1809.[1] Despite the availability of modern imaging techniques, diagnosis is challenging. Although Meckel diverticulum is usually of no medical significance, two types of complications can require clinical attention. One type involves ectopic mucosal tissue and most often leading to GI bleeding in younger children. In the second type, the sequelae of the diverticulum involve an aberrant intra-abdominal structure.

Pathophysiology
Early in embryonic life, the fetal midgut receives its nutrition from the yolk sac via the vitelline duct. The duct then undergoes progressive narrowing and usually disappears by 7 weeks' gestation. When the duct fails to fully obliterate, different types of vitelline duct anomalies appear. Examples of such anomalies include (1) a persistent vitelline duct (appearing as a draining fistula at the umbilicus); (2) a fibrous band that connects the ileum to the inner surface of the umbilicus; (3) a patent vitelline sinus beneath the umbilicus; (4) an obliterated bowel portion; (5) a vitelline duct cyst; and, most commonly (97%) Meckel diverticulum, which is a blind-ending true diverticulum that contains all of the layers normally found in the ileum.[2] The tip of the diverticulum is free in 75% of cases and is attached to the anterior abdominal wall or another structure in the remainder of cases. Enterocystomas, umbilical sinuses, and omphaloileal fistulas are among the other congenital anomalies associated with Meckel diverticulum. The diverticulum is usually supplied by the omphalomesenteric artery (a remnant of the vitelline artery), which arises from the ileal branch of the superior mesenteric artery. Usually, the artery terminates in the diverticulum; however, it has been reported to continue up to the abdominal wall in some cases. Rarely, these blood vessels persist in the form of fibrous remnants that run between the Meckel diverticulum and the abdominal wall or small bowel mesentery. Meckel diverticulum occurs on the antimesenteric border of the ileum, usually 40-60 cm proximal to the ileocecal valve. On average, the diverticulum is 3 cm long and 2 cm wide. Slightly more than one half contain ectopic mucosa. Meckel diverticulum is typically lined by ileal mucosa, but other tissue types are also found with varying frequency. The heterotopic mucosa is most commonly gastric. This is important because peptic ulceration of this or adjacent mucosa can lead to painless bleeding, perforation, or both. In one study, heterotropic gastric mucosa was found in 62% of cases, pancreatic tissue was found in 6%, both pancreatic tissue and gastric mucosa were found in 5%, jejunal

mucosa was found in 2%, Brunner tissue was found in 2% and both gastric and duodenal mucosa were found in 2%.[2] Rarely, colonic, rectal, endometrial, and hepatobiliary tissues have been noted.

Epidemiology
Frequency
United States The prevalence of Meckel diverticulum is usually noted to be approximately 2% of the population,[3] but published series range from 0.2-4%.[4] Complications are only seen in about 5% of those with the anomaly. In a comprehensive survey of 43 children's hospitals in the United States, 815 children had a Meckel diverticulectomy during a 2year span. Slightly more than half (60%) were symptomatic and the remainder were incidental in children who had laporotomy for a different reason. [50] International Prevalence figures similar to those found in the United States have been reported in Europe and Asia.

Mortality/Morbidity
See Complications.

Race
No racial biases have been reported.

Sex
Although no sex-based difference was reported in studies that evaluated this condition as an incidental finding during operations or autopsies, males are as much as 3-4 times more prone to complications than females. In a large series of cases from 2007-2008, Meckel diverticulectomy was 2.3 times more common in boys and boys accounted for 74% of the primary cases.[50]

Age
The classic presentation in children is considered to be painless rectal bleeding in a toddler younger than 2 years. One large series found that 53% had surgery before their fourth birthday. However, the largest group (slightly more than 30%) were younger than one year.[50] Although most other pediatric cases occur in patients aged 2-8 years, many continue to present with hematochezia. Although children usually present with hematochezia and adults usually present with obstruction, the same recent series of 815 children found that a primary diverticulectomy was performed in 30% of the children (< 18 y) for obstruction while 27% presented with bleeding and 19% had intussusception.[50] About one quarter did not have a clear cut diagnosis. Although neonatal presentation of Meckel is rare, case

reports have described perforation, intussusception, segmental ileal dilation, and ileal volvulus in newborns. In one neonate, massive hematochezia was reported on day 6. [5] In adults, obstruction and inflammation are more common presentations than lower GI bleeding. Several population-based studies have reported a decreased incidence of complications with increasing age, although other studies have not. Therefore, the issue of incidental diverticulectomy in older patients remains controversial. Proceed to Clinical Presentation

History
Most patients are asymptomatic. Meckel diverticulum is most frequently diagnosed as an incidental finding when a barium study or laparotomy is performed for other abdominal conditions.

Symptomatic Meckel diverticulum is virtually synonymous with a complication. This is estimated to occur in as many as 4-16% of patients.[2]Complications are the result of obstruction, ectopic tissue, or inflammation. In one study of 830 patients of all ages, complications included bowel obstruction (35%), hemorrhage (32%), diverticulitis (22%), umbilical fistula (10%), and other umbilical lesions (1%). In children, hematochezia is the most common presenting sign.[6] Bleeding in adults is much less common.[7, 8] o Acute lower GI bleeding is secondary to hemorrhage from peptic ulceration. Such ulceration occurs when acid secreted by heterotopic gastric mucosa damages contiguous vulnerable tissue, often times resulting in direct erosion of a vessel. Clinically, hemorrhage is usually noted to be substantial painless rectal bleeding. However, some patients may present only with pain preceding the onset of hematochezia. The pain can be quite significant and often delays the correct diagnosis. o Not all patients have abdominal pain; however, when present, it can be significant. A rare cause of abdominal pain from the Meckel diverticulum is inversion without intussusception.[9] Although intestinal obstruction in pediatrics is not considered very prevalent, some series report it in 25-40% of pediatric complications. It is the most common complication in adults. Obstruction can be the result of various mechanisms.[2] o Omphalomesenteric band (most frequent cause) o Internal hernia through vitelline duct remnants o Volvulus occurring around vitelline duct remnants o T-shaped prolapse of both efferent and afferent loops of intestine through a persistent vitelline duct fistula at the umbilicus in a neonate o Intussusception (when Meckel diverticulum itself acts as a lead point for an ileocolic or ileoileal intussusception) None of these mechanisms have clinical features that are pathognomonic, and the precise etiology is rarely known preoperatively. Like other diverticula in the body, Meckel diverticulum can become inflamed. Diverticulitis is usually seen in older patients. Meckel diverticulum is less prone to

inflammation than the appendix because most diverticula have a wide mouth, have very little lymphoid tissue, and are self-emptying. o The clinical presentation includes abdominal pain in the periumbilical area that radiates to the right lower quadrant. o Persistence of periumbilical pain or a history of bleeding per rectum may be helpful in distinguishing this entity from appendicitis. o Subacute or chronic inflammation of Meckel diverticulum is rare, but a few cases of tuberculosis and Crohn disease within the diverticulum have been reported. Less frequently, the Meckel diverticulum may develop benign tumors (eg, leiomyomas, angiomas, neuromas, lipomas). About three quarters of the malignant tumors are carcinoids[51] but others include sarcoma,[10] carcinoid tumor,[11] adenocarcinomas[12] and Burkitt lymphoma[13] , as well as additional rare lesions.[51] Rarely, the diverticulum may perforate from a swallowed fish bone or sewing needle.

Physical
Although most patients are asymptomatic, patients can present with various clinical signs, including peritonitis or hypovolemic shock. The 3 most common symptomatic presentations are GI bleeding, intestinal obstruction, and acute inflammation of the diverticulum.

Most often, painless rectal bleeding (hematochezia) occurs suddenly and tends to be massive in younger patients.[14] Bleeding occurs without prior warning and usually spontaneously subsides. o When a severe bleeding episode occurs, the patient can present in hemorrhagic shock. Tachycardia is an early clinical sign of hemorrhagic shock, but orthostatic hypotension may actually precede this. o The color of the stool often provides physicians with a clue to determine the site of bleeding. This has been well addressed in a classic description of the types of rectal bleeding associated with Meckel diverticulum.[15] o Prevalence of different types of bleeding has been described as follows: Dark red (maroon) - 40% Bright red - 35% Bright red or dark red - 12% Dark red or tarry - 6% Tarry - 7% o When bleeding is rapid, stools are bright red or have an appearance like currant jelly. When slow bleeding occurs, the stools are black and tarry. o Most patients with intestinal obstruction present with abdominal pain, bilious vomiting, abdominal tenderness, distension, and hyperactive bowel sounds upon examination. o Patients may develop a palpable abdominal mass. o Occasionally, when patients do not present early or if the diagnosis is missed, the obstruction can progress to intestinal ischemia or infarction. The latter manifests with acute peritoneal signs and lower GI bleeding.

Patients with diverticulitis present with either focal or diffuse abdominal tenderness. Usually, abdominal tenderness is more marked in the periumbilical region than the pain of appendicitis. o Children may present with abdominal guarding and rebound tenderness, in addition to abdominal tenderness. o Abdominal distention and hypoactive bowel sounds are late findings. Rarely, Meckel diverticulum has been reported to become incarcerated (Littre hernia) in the inguinal,[16] femoral, or obturator hernial sacs or even incisional defects.

Causes
Meckel diverticulum is caused by the failure of the omphalomesenteric duct to completely obliterate at 5-7 weeks' gestation, followed by one of the various complications listed above.

Differential Diagnoses

Appendicitis Colitis Colonic Vascular Malformations Constipation Crohn Disease Gastroenteritis Gastrointestinal Duplications Henoch-Schoenlein Purpura Hirschsprung Disease Intestinal duplication Intestinal Polyposis Syndromes Intussusception Juvenile Polyps Necrotizing Enterocolitis Peptic Ulcer Disease Peutz-Jeghers Syndrome Postoperative Adhesions Ulcerative Colitis Urolithiasis Volvulus

Laboratory Studies
Routine laboratory findings, including CBC count, electrolyte levels, glucose test results, BUN levels, creatinine levels, and coagulation screen results, are not helpful in establishing the diagnosis of Meckel diverticulum but are necessary to manage a patient with GI bleeding along with a type and cross. Hemoglobin and hematocrit levels are low in the setting of anemia or bleeding. Patients with significant bleeding develop anemia. In one series, 58% of children had average hemoglobin levels of less than 8.8 g/dL. Ongoing bleeding from a Meckel diverticulum can cause iron deficiency anemia. However, megaloblastic anemia can also be seen due to vitamin B 12 or folate deficiency. These can occur secondary to small bowel overgrowth if dilation and/or

stasis related to the diverticulum is present. Low albumin and low ferritin levels may lead to a diagnosis of inflammatory bowel disease.

Imaging Studies

According to Mayo, "Meckel's Diverticulum is frequently suspected, often looked for, and seldom found." Preoperative diagnosis is difficult, especially if the presenting symptom is not GI bleeding. In one series, patients often had a correct preoperative diagnosis if the presenting symptom was GI bleeding, but only 11% of preoperative diagnoses were correct if other symptoms predominated.[17] History and physical examination are of paramount importance for establishing a clinical diagnosis. Imaging studies are performed to confirm a clinical suspicion of Meckel diverticulum. Plain radiography of the abdomen is of limited value. It may reveal evidence of nonbleeding complications, including enteroliths and signs of intestinal obstruction or

perforation, such as air or air-fluid levels (see the image below).

Anteroposterior view of abdominal radiograph showing multiple dilated loops of a small bowel with air-fluid levels.

When a patient has GI bleeding suggestive of Meckel diverticulum, the diagnostic evaluation should focus on Meckel scanning, a technetium-99m pertechnetate scintiscan (0.2mCi/kg in children and 10-20mCi in adults). The pertechnetate is taken up by gastric mucosa. Because bleeding from the Meckel diverticulum is related to acid induced damage of mucosa adjacent to the parietal cell containing tissue, it is always included early in the work-up.[18] After intravenous injection of the isotope, the gamma camera is used to scan the abdomen. This procedure usually lasts approximately 30 minutes. Gastric mucosa secretes the radioactive isotope; thus, if the diverticulum contains this ectopic tissue, it is recognized as a hot spot. The Meckel scan is the preferred procedure because it is noninvasive, involves less radiation exposure, and is more accurate than an upper GI and small-bowel followthrough study. In children the Meckel scan has a reported sensitivity of 80-90%, a specificity of 95% and an accuracy of 90%. However, in adults where GI bleeding is a much less common presentation, the scan has a lower sensitivity (62.5%), a much lower specificity (9%), and a lower accuracy (46%).[19] Because the Meckel scan is specific for gastric mucosa (ie, in the stomach or ectopic) and not specifically diagnostic of Meckel diverticulum, false positive results occur whenever ectopic gastric mucosa is present. Duodenal ulcer, small intestinal

obstruction, some intestinal duplications, ureteric obstruction, aneurysm, and angiomas of the small intestine have yielded positive results. False negative results can occur when gastric mucosa is very slight or absent in the diverticulum, if necrosis of the diverticulum has occurred, or if the Meckel is superimposed on the bladder. [20] Accuracy of the scan may be enhanced with administration of cimetidine, glucagon, and pentagastrin. Cimetidine enhances the uptake and blocks the secretion of technetium-99m pertechnetate from ectopic gastric mucosa.[21]This helps to improve the lesion to background ratio in enhancing a Meckel scan. Pentagastrin also enhances uptake of the isotope but also increases peristalsis, attenuating its value. Glucagon is used to decrease peristalsis, thus allowing the signal to be taken up during a longer exposure time. One strategy uses both pentagastrin and glucagon. With newer imaging technology, false-positive and false-negative rates have declined. Barium studies have largely been replaced by other imaging techniques; however, if a barium study is indicated, it should never precede the technetium-99m scan because barium may obscure the hot spot. A bleeding scan can be performed to identify the source if the patient is bleeding at 0.1ml/min or more. This scan involves removing and labeling some of the patient's own RBCs with technetium-99m, reinjecting them into the patient, and then scanning the abdomen for hot spots.[22, 23] Selective arteriography may be helpful in patients in whom the results from scintigraphy and barium studies are negative. Usually, this occurs if the bleeding is either intermittent or has completely resolved. When the rate of bleeding is greater than 1 mL/min, a superior mesenteric arteriogram can be helpful, but interpretation may be difficult due to overlying blood vessels. In these cases, selective catheterization of the distal ileal arteries may be needed. Demonstration of abnormal arterial branches, dense capillary staining, or extravasation of the contrast medium confirms the presence of a Meckel diverticulum. However, a well-developed arterial supply may not always be present in the Meckel diverticulum; thus, these arteriographic signs are not very reliable. Traditional small-bowel series using barium have been unreliable in the detection of Meckel diverticulum. However, in patients who require barium study to primarily look for other conditions, enteroclysis is more sensitive in detecting Meckel diverticulum. Enteroclysis involves using a continuous infusion of barium with adequate compression of the ileal loops and intermittent fluoroscopy to detect Meckel diverticulum. If the barium mixture is too dense and the fold pattern cannot be visualized, carboxymethylcellulose sodium can be used as the contrast medium. On barium studies, Meckel diverticulum may appear as a blind-ending pouch on the antimesenteric side of the distal ileum. If filling defects are visualized, the diverticulum may contain a tumor. Characteristic radiologic signs for Meckel diverticulum include demonstration of a triradiate fold pattern or a mucosal triangular plateau. Occasionally, a gastric rugal pattern may also be found within the Meckel diverticulum. A barium enema can be performed if intussusception is suspected. Some people have tried hydrostatic therapy to reduce intussusception, but this has not been found to be useful.

Abdominal CT scanning is usually not helpful because differentiating Meckel diverticulum from the small-bowel loops is difficult; however, a blind-ending fluid-filled and/or gas-filled structure in continuity with small bowel may be visualized. CT scanning may also reveal an enterolith, intussusception, or diverticulitis. CT enterography advancements have increased the sensitivity in the diagnosis of Meckel diverticulum. [18] Ultrasonography has been used in some cases of Meckel diverticulum. Ultrasonography tends to be helpful if the patient presents with anatomic rather than mucosal complications. Wireless capsule endoscopy has been successfully used to identify Meckel diverticulum in young children.[24] In adults, this same technique has been used to identify an inverted Meckel diverticulum that presented as GI bleeding.[52]

Histologic Findings

In one study, heterotropic gastric mucosa was found in 62% of cases, pancreatic tissue was found in 6%, both pancreatic tissue and gastric mucosa were found in 5%, jejunal mucosa was found in 2%, Brunner tissue was found in 2%, and both gastric and duodenal mucosa were found in 2%.[2] Although some reports have associated Helicobacter pylori with ectopic gastric mucosa in Meckel diverticulum, a small series of 21 consecutive patients from Turkey using polymerase chain reaction (PCR) failed to identify 23S ribosomal RNA sequences from the organism even in the 12 surgical specimens with heterotopic gastric mucosa. [53]

Medical Care
The emergency department evaluation and treatment of patients depends on the clinical presentation of Meckel diverticulum.

Because most symptomatic patients are acutely ill, establish an intravenous line immediately, start crystalloid fluids, and keep the patient on nothing by mouth (NPO) status. Obtain the blood investigations suggested above with a type and cross match. If significant bleeding occurs, perform a transfusion of packed red cells. A patient who presents with intestinal obstruction usually requires nasogastric decompression; also perform plain radiography of the abdomen. When a child presents with bleeding, specifically a dark tarry stool, perform a gastric lavage to rule out upper GI bleeding. If the gastric lavage is negative for bleeding, consider an upper endoscopy and flexible sigmoidoscopy. Meckel scan results may be negative despite a high clinical suspicion of Meckel diverticulum. The surgery team should be consulted to discuss the possible need for laparoscopy and/or laparotomy.

Surgical Care
If the patient is bleeding but is hemodynamically stable, a Meckel scan is warranted. On the other hand, the presence of peritoneal signs or hemodynamic instability demands urgent surgical intervention. Signs of small bowel obstruction also require surgical intervention.[25]

Definitive treatment of a complication, such as a bleeding Meckel diverticulum, is the excision of the diverticulum along with the adjacent ileal segment.

Excision is carried out by performing a wedge resection of adjacent ileum and anastomosis, with the use of a stapling device. Adjacent ileum is included in the resection because ulcers frequently develop in the adjacent part of the ileum. [26] o Successful resection of a Meckel diverticulum, even in children and infants, can also be accomplished through laparoscopy, using an endoscopically designed autostapling device.[27, 28, 29] A large series of national trends in the surgical management of Meckel diverticulum found that one fourth of cases are now treated laparoscopically. This group was older (6.4 y 5.1 y vs 5.1 y 5.3 y) and had shorter length of stay and trended toward lower total hospital charges. [50] o In some cases of Meckel diverticulum, a primitive persistent right vitelline artery originating from the mesentery has been found during operation. When present, the artery is found to supply the Meckel diverticulum; therefore, it must be identified and ligated during the operation. Management of Meckel diverticulum in asymptomatic patients is controversial. o In the past, if a Meckel diverticulum was encountered in a patient undergoing abdominal surgery for some other intra-abdominal condition, many surgeons recommended its removal. o This practice was questioned when a large series described an overall 4.2% likelihood of complications in Meckel diverticulum and a decreasing risk with increasing age. These authors concluded that assuming a 6% mortality rate from Meckel diverticulum complications, 400 asymptomatic diverticula would have to be excised to save one patient.[30] o Another faction favors prophylactic removal of a diverticulum, which is a simple operation. This view is supported by data that demonstrate that managing a complication of Meckel diverticulum is associated with high morbidity and mortality rates. Others feel the only exception to universal excision is if the diverticulum is so broad based or so short that stapled excision cannot be performed technically. Fortunately, patients are less likely to develop complications in both of these situations. o One recent small series suggested that only patients younger than 50 years clearly benefitted from removal if discovered unintentionally.[31]
o

Consultations

Radiologist Surgeon Gastroenterologist

Medication Summary
In addition to the definitive therapy, urgently administer a regimen of antibiotics (eg, ampicillin, gentamicin, and clindamycin or cefotetan) whenever acute Meckel diverticulitis, strangulation, perforation, or signs of small bowel obstruction or sepsis are present.

Antibiotics
Class Summary Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the clinical setting.

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Ampicillin (Omnipen, Marcillin)

Interferes with bacterial cell wall synthesis during active replication, causing bactericidal activity against susceptible organisms.

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Clindamycin (Cleocin)
Useful treatment for serious skin and soft tissue infections caused by most staphylococci strains. Also effective against entericaerobic and anaerobic flora, except enterococci. Inhibits bacterial protein synthesis by inhibiting peptide chain initiation at the bacterial ribosome, where it preferentially binds to the 50S ribosomal subunit, causing bacterial replication inhibition.

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Gentamicin (Gentacidin, Garamycin)

If used in combination with an antianaerobic agent, such as clindamycin or metronidazole, provides broad gram-negative and anaerobic coverage. Dosing regimens are numerous and adjusted on the basis of creatinine clearance and changes in distribution volume.

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Cefotetan (Cefotan)
Second-generation cephalosporin used as single-drug therapy to provide broad gram-negative coverage and anaerobic coverage. Half-life is 3.5 h. Inhibits bacterial cell wall synthesis by binding to 1 of the penicillin-binding proteins; inhibits final transpeptidation step of peptidoglycan synthesis, resulting in cell wall death. Antibiotics have proven effective in decreasing rate of postoperative wound infection and improving outcome in patients with intraperitoneal infection and septicemia.

Complications

Because the diagnosis of Meckel diverticulum can be quite elusive, a high index of suspicion is warranted to correctly and expeditiously diagnose this condition. Complicated Meckel diverticulum can lead to significant morbidity and mortality, most often because of a delay in diagnosis. For example, a higher frequency of intestinal infarction has been encountered in patients who present with complete intestinal obstruction. Causes of mortality include strangulation, perforation, and exsanguination because of delay in resuscitation. Once a complication arises and surgery is required, the operative mortality and morbidity rates have both been estimated at 12%. The cumulative long-term risk of postoperative complications in this cohort was found to be 7%. If the Meckel diverticulum is removed as an incidental finding, the risk of mortality and morbidity and long-term complications are much less (1%, 2%, and 2%, respectively). As many as 5% of complicated Meckel diverticulum contain malignant tissue.

Prognosis

See Complications.