Divertikulum mekel

7/30/2019 Divertikulum mekel

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Background

Meckel diverticulum (also referred to as Meckel's Diverticulum) is the most commoncongenital abnormality of the small intestine; it is caused by an incomplete obliterationof the vitelline duct (ie, omphalomesenteric duct). Although originally described byFabricius 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 ofcomplications can require clinical attention. One type involves ectopic mucosal tissueand most often leading to GI bleeding in younger children. In the second type, thesequelae 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 thevitelline duct. The duct then undergoes progressive narrowing and usually disappearsby 7 weeks' gestation. When the duct fails to fully obliterate, different types of vitellineduct anomalies appear. Examples of such anomalies include (1) a persistent vitellineduct (appearing as a draining fistula at the umbilicus); (2) a fibrous band that connectsthe ileum to the inner surface of the umbilicus; (3) a patent vitelline sinus beneath theumbilicus; (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 ofthe layers normally found in the ileum.[2] The tip of the diverticulum is free in 75% ofcases and is attached to the anterior abdominal wall or another structure in theremainder 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 thevitelline artery), which arises from the ileal branch of the superior mesenteric artery.Usually, the artery terminates in the diverticulum; however, it has been reported tocontinue up to the abdominal wall in some cases. Rarely, these blood vessels persist inthe form of fibrous remnants that run between the Meckel diverticulum and theabdominal wall or small bowel mesentery.

Meckel diverticulum occurs on the antimesenteric border of the ileum, usually 40-60 cmproximal 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 istypically lined by ileal mucosa, but other tissue types are also found with varyingfrequency.

The heterotopic mucosa is most commonly gastric. This is important because pepticulceration 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 tissuewas found in 6%, both pancreatic tissue and gastric mucosa were found in 5%, jejunal


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

Epidemiology

Frequency

United StatesThe prevalence of Meckel diverticulum is usually noted to be approximately 2% of thepopulation,[3] but published series range from 0.2-4%.[4] Complications are only seen inabout 5% of those with the anomaly. In a comprehensive survey of 43 children'shospitals in the United States, 815 children had a Meckel diverticulectomy during a 2-year span. Slightly more than half (60%) were symptomatic and the remainder wereincidental in children who had laporotomy for a different reason.[50]

InternationalPrevalence figures similar to those found in the United States have been reported inEurope 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 conditionas an incidental finding during operations or autopsies, males are as much as 3-4 timesmore 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 for74% of the primary cases.[50]

Age

The classic presentation in children is considered to be painless rectal bleeding in atoddler younger than 2 years. One large series found that 53% had surgery before theirfourth birthday. However, the largest group (slightly more than 30%) were younger thanone 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 withobstruction, the same recent series of 815 children found that a primarydiverticulectomy was performed in 30% of the children (< 18 y) for obstruction while27% presented with bleeding and 19% had intussusception.[50]About one quarter did nothave a clear cut diagnosis. Although neonatal presentation of Meckel is rare, case


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reports have described perforation,intussusception, segmental ileal dilation, and ilealvolvulus in newborns. In one neonate, massive hematochezia was reported on day 6 . [5]

In adults, obstruction and inflammation are more common presentations than lower GIbleeding. Several population-based studies have reported a decreased incidence ofcomplications with increasing age, although other studies have not. Therefore, the issueof incidental diverticulectomy in older patients remains controversial.

Proceed toClinical Presentation

History

Most patients are asymptomatic. Meckel diverticulum is most frequently diagnosed asan incidental finding when a barium study or laparotomy is performed for otherabdominal conditions.

Symptomatic Meckel diverticulum is virtually synonymous with a complication. This isestimated 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 includedbowel 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 ismuch less common.[7, 8]

o Acute lower GI bleeding is secondary to hemorrhage from peptic ulceration. Suchulceration occurs when acid secreted by heterotopic gastric mucosa damagescontiguous 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 ofhematochezia. 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 withoutintussusception.[9]

Although intestinal obstruction in pediatrics is not considered very prevalent, someseries report it in 25-40% of pediatric complications. It is the most commoncomplication in adults. Obstruction can be the result of various mechanisms.[2]

o Omphalomesenteric band (most frequent cause)o Internal hernia through vitelline duct remnantso Volvulusoccurring around vitelline duct remnantso T-shaped prolapse of both efferent and afferent loops of intestine through a

persistent vitelline duct fistula at the umbilicus in a neonateo 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
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inflammation than the appendix because most diverticula have a wide mouth, havevery little lymphoid tissue, and are self-emptying.

o The clinical presentation includes abdominal pain in the periumbilical area thatradiates 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

oftuberculosisandCrohn diseasewithin the diverticulum have been reported. Less frequently, the Meckel diverticulum may develop benign tumors (eg,

leiomyomas, angiomas, neuromas, lipomas). About three quarters of the malignanttumors are carcinoids[51] but others include sarcoma,[10] carcinoidtumor,[11] adenocarcinomas [12] and Burkitt lymphoma[13] , as well as additional rarelesions.[51] Rarely, the diverticulum may perforate from a swallowed fish bone orsewing needle.

Physical

Although most patients are asymptomatic, patients can present with various clinical

signs, including peritonitis or hypovolemic shock. The 3 most common symptomaticpresentations are GI bleeding, intestinal obstruction, and acute inflammation of thediverticulum.

Most often, painless rectal bleeding (hematochezia) occurs suddenly and tends to bemassive in younger patients.[14] Bleeding occurs without prior warning and usuallyspontaneously subsides.

o When a severe bleeding episode occurs, the patient can present in hemorrhagicshock. Tachycardia is an early clinical sign of hemorrhagic shock, but orthostatichypotension 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 rectalbleeding 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 currantjelly. 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 uponexamination.

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 withacute peritoneal signs and lower GI bleeding.
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Patients with diverticulitis present with either focal or diffuse abdominal tenderness.Usually, abdominal tenderness is more marked in the periumbilical region than thepain of appendicitis.

o Children may present with abdominal guarding and rebound tenderness, in additionto 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 tocompletely obliterate at 5-7 weeks' gestation, followed by one of the variouscomplications 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 patientwith 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 B12 or folatedeficiency. These can occur secondary to small bowel overgrowth if dilation and/or
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stasis related to the diverticulum is present. Low albumin and low ferritin levels maylead 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 preoperativediagnosis if the presenting symptom was GI bleeding, but only 11% of preoperativediagnoses were correct if other symptoms predominated.[17]

History and physical examination are of paramount importance for establishing a clinicaldiagnosis. Imaging studies are performed to confirm a clinical suspicion of Meckeldiverticulum.

Plain radiography of the abdomen is of limited value. It may reveal evidence ofnonbleeding 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 withair-fluid levels.

When a patient has GI bleeding suggestive of Meckel diverticulum, the diagnostic

evaluation should focus on Meckel scanning, a technetium-99m pertechnetatescintiscan (0.2mCi/kg in children and 10-20mCi in adults). The pertechnetate is takenup by gastric mucosa. Because bleeding from the Meckel diverticulum is related to acidinduced damage of mucosa adjacent to the parietal cell containing tissue, it is alwaysincluded early in the work-up.[18]

After intravenous injection of the isotope, the gamma camera is used to scan theabdomen. This procedure usually lasts approximately 30 minutes. Gastric mucosasecretes the radioactive isotope; thus, if the diverticulum contains this ectopic tissue, itis recognized as a hot spot.

The Meckel scan is the preferred procedure because it is noninvasive, involves lessradiation exposure, and is more accurate than an upper GI and small-bowel follow-

through 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 lesscommon presentation, the scan has a lower sensitivity (62.5%), a much lowerspecificity (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 occurwhenever ectopic gastric mucosa is present. Duodenal ulcer, small intestinal
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obstruction, some intestinal duplications, ureteric obstruction, aneurysm, andangiomas of the small intestine have yielded positive results. False negative resultscan occur when gastric mucosa is very slight or absent in the diverticulum, if necrosisof 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 oftechnetium-99m pertechnetate from ectopic gastric mucosa.[21]This helps to improvethe lesion to background ratio in enhancing a Meckel scan. Pentagastrin alsoenhances uptake of the isotope but also increases peristalsis, attenuating its value.Glucagon is used to decrease peristalsis, thus allowing the signal to be taken upduring 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 abarium study is indicated, it should never precede the technetium-99m scan becausebarium 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'sown RBCs with technetium-99m, reinjecting them into the patient, and then scanningthe abdomen for hot spots.[22, 23]

Selective arteriography may be helpful in patients in whom the results from scintigraphyand barium studies are negative. Usually, this occurs if the bleeding is either intermittentor has completely resolved.

When the rate of bleeding is greater than 1 mL/min, a superior mesenteric arteriogramcan be helpful, but interpretation may be difficult due to overlying blood vessels. Inthese cases, selective catheterization of the distal ileal arteries may be needed.

Demonstration of abnormal arterial branches, dense capillary staining, orextravasation of the contrast medium confirms the presence of a Meckel diverticulum.However, a well-developed arterial supply may not always be present in the Meckeldiverticulum; thus, these arteriographic signs are not very reliable.

Traditional small-bowel series using barium have been unreliable in the detection ofMeckel diverticulum. However, in patients who require barium study to primarily look forother conditions, enteroclysis is more sensitive in detecting Meckel diverticulum.

Enteroclysis involves using a continuous infusion of barium with adequatecompression of the ileal loops and intermittent fluoroscopy to detect Meckeldiverticulum.

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 theantimesenteric side of the distal ileum. If filling defects are visualized, the diverticulummay contain a tumor.

Characteristic radiologic signs for Meckel diverticulum include demonstration of atriradiate fold pattern or a mucosal triangular plateau. Occasionally, a gastric rugalpattern may also be found within the Meckel diverticulum.

A barium enema can be performed if intussusception is suspected. Some people havetried hydrostatic therapy to reduce intussusception, but this has not been found to beuseful.


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Abdominal CT scanning is usually not helpful because differentiating Meckeldiverticulum from the small-bowel loops is difficult; however, a blind-ending fluid-filledand/or gas-filled structure in continuity with small bowel may be visualized. CT scanningmay also reveal an enterolith, intussusception, or diverticulitis. CT enterographyadvancements have increased the sensitivity in the diagnosis of Meckel diverticulum.[18]

Ultrasonography has been used in some cases of Meckel diverticulum. Ultrasonographytends to be helpful if the patient presents with anatomic rather than mucosalcomplications.

Wireless capsule endoscopy has been successfully used to identify Meckel diverticulumin young children.[24] In adults, this same technique has been used to identify an invertedMeckel diverticulum that presented as GI bleeding.[52]

Histologic Findings

In one study, heterotropic gastric mucosa was found in 62% of cases, pancreatic tissuewas found in 6%, both pancreatic tissue and gastric mucosa were found in 5%, jejunalmucosa was found in 2%, Brunner tissue was found in 2%, and both gastric andduodenal mucosa were found in 2%.[2]

Although some reports have associated Helicobacter pyloriwith ectopic gastric mucosain Meckel diverticulum, a small series of 21 consecutive patients from Turkey usingpolymerase chain reaction (PCR) failed to identify 23S ribosomal RNA sequences fromthe 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 clinicalpresentation 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 Meckeldiverticulum. The surgery team should be consulted to discuss the possible need forlaparoscopy and/or laparotomy.

Surgical CareIf the patient is bleeding but is hemodynamically stable, a Meckel scan is warranted. Onthe other hand, the presence of peritoneal signs or hemodynamic instability demandsurgent surgical intervention. Signs of small bowel obstruction also require surgicalintervention.[25]

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


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o Excision is carried out by performing a wedge resection of adjacent ileum andanastomosis, with the use of a stapling device. Adjacent ileum is included in theresection 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 alsobe accomplished through laparoscopy, using an endoscopically designed

autostapling device.

[27, 28, 29]

A large series of national trends in the surgicalmanagement of Meckel diverticulum found that one fourth of cases are now treatedlaparoscopically. This group was older (6.4 y 5.1 y vs 5.1 y 5.3 y) and hadshorter length of stay and trended toward lower total hospital charges.[50]

o In some cases of Meckel diverticulum, a primitive persistent right vitelline arteryoriginating from the mesentery has been found during operation. When present, theartery is found to supply the Meckel diverticulum; therefore, it must be identified andligated 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 withincreasing age. These authors concluded that assuming a 6% mortality rate fromMeckel diverticulum complications, 400 asymptomatic diverticula would have to beexcised to save one patient.[30]

o Another faction favors prophylactic removal of a diverticulum, which is a simpleoperation. This view is supported by data that demonstrate that managing acomplication of Meckel diverticulum is associated with high morbidity and mortalityrates. Others feel the only exception to universal excision is if the diverticulum is sobroad based or so short that stapled excision cannot be performed technically.Fortunately, patients are less likely to develop complications in both of thesesituations.

o One recent small series suggested that only patients younger than 50 years clearlybenefitted from removal if discovered unintentionally.[31]

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 Meckeldiverticulitis, strangulation, perforation, or signs of small bowel obstruction orsepsis are present.

Antibiotics Class Summary

Empiric antimicrobial therapy must be comprehensive and should cover all likelypathogens in the clinical setting.

View full drug information
http://reference.medscape.com/drug/ampi-omnipen-ampicillin-342475#1http://reference.medscape.com/drug/ampi-omnipen-ampicillin-342475#1http://reference.medscape.com/drug/ampi-omnipen-ampicillin-342475#1


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Ampicillin (Omnipen, Marcillin)

Interferes with bacterial cell wall synthesis during active replication, causing

bactericidal activity against susceptible organisms. View full drug information

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 chaininitiation at the bacterial ribosome, where it preferentially binds to the 50Sribosomal subunit, causing bacterial replication inhibition.


Gentamicin (Gentacidin, Garamycin)

If used in combination with an antianaerobic agent, such as clindamycin or

metronidazole, provides broad gram-negative and anaerobic coverage. Dosingregimens are numerous and adjusted on the basis of creatinine clearance andchanges in distribution volume.


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. Inhibitsbacterial cell wall synthesis by binding to 1 of the penicillin-binding proteins;inhibits final transpeptidation step of peptidoglycan synthesis, resulting in cell walldeath.

Antibiotics have proven effective in decreasing rate of postoperative woundinfection and improving outcome in patients with intraperitoneal infection andsepticemia.

Complications

Because the diagnosis of Meckel diverticulum can be quite elusive, a high index ofsuspicion is warranted to correctly and expeditiously diagnose this condition.Complicated Meckel diverticulum can lead to significant morbidity and mortality, mostoften because of a delay in diagnosis. For example, a higher frequency of intestinalinfarction has been encountered in patients who present with complete intestinalobstruction. 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 ofpostoperative complications in this cohort was found to be 7%. If the Meckeldiverticulum is removed as an incidental finding, the risk of mortality and morbidity andlong-term complications are much less (1%, 2%, and 2%, respectively).

As many as 5% of complicated Meckel diverticulum contain malignant tissue.

Prognosis
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See Complications.

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