03gastrointestinal Emergencies - Principles and Practice of Pediatric

15/9/2015 Gastrointestinal Emergencies | Principles and Practice of Pediatric…
GASTROINTESTINAL EMERGENCIES
A very common complaint in cancer patients is abdominal pain, which can reflect issues as
simple as treatmentinduced nausea and constipation to an acute abdomen requiring
urgent medical care. The differential diagnosis of abdominal pain is lengthy in this
population, but reviewing the history and the concomitant symptoms (fever, diarrhea,
bleeding), as well as judicious imaging, can readily determine the cause. This section will
focus on GI emergencies, but symptomatic management can be found inChapter 43 .
Etiology and Pathogenesis
Table38.2 Differential Diagnosis of Abdominal Pain in Pediatric
Cancer Patients
A child with cancer can develop an acute abdomen for the same reasons as any other child,
data:text/html;charset=utf8,%3Cheader%20id%3D%22812dba18126443379f31e4fdf0001bb3%22%20style%3D%22margin%3A%200px%2015px%2015px%3… 1/11
but the differential diagnosis must be broadened to include issues related to the underlying
malignancy and its treatment. Abdominal processes that are localized in healthy children
may be generalized in the neutropenic or immunosuppressed child. GI emergencies in the
child with cancer arise because of hemorrhage, mechanical obstruction, perforation,
infection, and inflammation (Table 38.2 ). Unlike adults, most GI emergencies in
children are secondary to cancer treatment rather than the underlying cancer itself.
Hemorrhage may result from thrombocytopenia, coagulopathy, mucosal ulceration, or
abnormal vessels within the tumor. Obstruction results from compression of a GI lumen by
a tumor or abscess or from medicationinduced ileus. Perforation can result from
unresolved obstruction, localized ulceration, and segmental necrosis. Any part of the GI
tract may become inflamed, causing esophagitis, gastritis, enterocolitis, typhlitis, and
hepatitis. A patient’s inflammatory response may be blunted by neutropenia, and wound
healing may be slow because of the effects of malnutrition and chemotherapy, notably
corticosteroids, on fibroblast proliferation and collagen production. Massive acute hepatic
enlargement can result from tumor infiltration by neuroblastoma, hemophagocytic
lymphohistiocytosis (HLH), or leukemia or an acute hepatic hemorrhage by a liver primary
or metastases.30 Venoocclusive disease (VOD) can cause rapid liver failure, generally in
the post–bone marrow transplant (BMT) setting.31
Evaluation
Pain is the principal symptom of an acute abdominal process, no matter how compromised
the patient. It is important to determine the location, quality, and timing of pain in relation
to the cancer location, recent medications, and surgical history. Changes in vital signs,
including fever, hypertension or hypotension, and tachycardia; the presence of blood in
vomitus or stool; abdominal distention; and absence of flatus or stool are nonspecific
indications of an acute abdomen.
On observation, one should note whether the child lies still, is willing to move, or
winces with movement or motion of the bed. Examination may also reveal abdominal
distension, asymmetry, and surgical scars. Compressing the head of the stethoscope gently
into the abdomen during auscultation can reveal guarding or tenderness. Auscultation can
help differentiate ileus from obstruction. Inspection of the oropharyngeal and perirectal
mucosa may provide insight into the state of the esophageal or gastric mucosa. Pain with
defecation or detected on perineal examination may be the result of generalized serositis,
mucositis, or abscess.
Laboratory and Diagnostic Studies
Selective use of laboratory and radiographic studies may differentiate the child who
requires immediate surgical intervention from one who will respond to medical
management. Serial CBCs can help monitor hemorrhage and reveal neutropenia that
interferes with pus and abscess formation. Persistently positive blood cultures despite
treatment with appropriate antibiotics may indicate the presence of an abscess or necrotic
bowel. Electrolyte changes may reveal metabolic deficits from fluid shifts before they are
clinically apparent. Abdominal paracentesis, diagnostic lavage, and laparoscopy have been
replaced by noninvasive imaging modalities.9,32
Diagnostic radiologic studies begin with supine, erect, and left lateral decubitus
abdominal radiographs. Obstruction (especially high smallbowel obstruction),
pneumatosis intestinalis, and intraabdominal free air can be diagnosed by these studies.
In a large retrospective study, the presence of peritoneal signs on examination and
pneumatosis intestinalis on radiograph were highly associated with the presence of an
acute surgical process in children receiving chemotherapy.33 A CXR may reveal pneumonia
as the etiology of upper quadrant abdominal pain. Direct examination with endoscopy or
colonoscopy has replaced contrast studies in the evaluation of the GI tract in both
compromised and healthy children.9,32 Abdominal US, CT, positron emission tomography
(PET), and magnetic resonance imaging (MRI) may all be used to locate and characterize
mass lesions, peritoneal fluid, and abnormal bowel wall thickening.
Gastrointestinal Hemorrhage
Upper Gastrointestinal Hemorrhage
Hematemesis, melena, and occult fecal blood often consists of blood swallowed from
epistaxis or mucositis/esophagitis, but occasionally may signify upper GI hemorrhage.
Children with cancer taking highdose or prolonged corticosteroids, those with increased
intracranial pressure (ICP), and those who underwent highdose chemotherapy are prone
to develop esophagitis, gastritis, and even gastric ulcers. Prophylaxis with oral antacids,
H2 blockers, or protonpump inhibitors (PPIs) should be considered for all atrisk children.
Chemotherapyinduced emesis can cause MalloryWeiss tears, which are mucosal
lacerations at the gastroesophageal junction resulting from large gradients between the
intragastric and intrathoracic pressure during emesis.34,35,36
Medical management of acute bleeding consists of initiating intravenous PPIs such as
omeprazole or pantoprazole, and correction of thrombocytopenia and coagulation
abnormalities.34,36 Selflimited hematemesis or coffeeground emesis can be treated
expectantly, whereas brisk bleeding often requires volume resuscitation with fluids and
packed red blood cells and correction of coagulopathy with platelets and fresh frozen
plasma (FFP). Passage of a largebore nasogastric tube and gastric lavage can help
differentiate an active bleed (bright red blood) from coffeeground gastric contents.
Continued bright red blood despite repeated lavage is highly suggestive of severe
bleeding.34,36 Endoscopy is indicated for uncontrolled or recurrent bleeding, and
hemostasis may be achieved with electrocautery. Surgery for persistent bleeding must be
individualized but most often entails resection and anastamosis or oversewing of an ulcer.
Esophageal Varices
Esophageal varices are very rare in pediatric patients and can develop as a consequence of
longterm portal hypertension, fibrosis, cholangitis, VOD, and cirrhosis. Variceal bleeding
is brisk, and patients may have associated hematemesis and melena. A CBC, PT (INR),
aPTT, and blood type and cross match should be obtained immediately. Initial
management includes elevation of the head of the bed to 30 to 45 degrees, judicious
volume expansion with normal saline or Ringer’s lactate, and correction of anemia.
Platelets should be transfused for thrombocytopenia and FFP given if there is evidence of a
coagulopathy or if multiple units of red blood cells are required. It is recommended that
patients receive broadspectrum antibiotics, as bleeding varices may be a sign of sepsis in a
patient with portal hypertension and to reduce infectious complications.36 Lavage can be
initiated as described above. Urgent management is indicated for persistence of bright red
blood in the lavage fluid. Somatostatin (Octreotide) therapy should be initiated and
continued for several days as it decreases portal pressure. If bleeding persists, endoscopy
with endoscopic variceal band ligation or sclerotherapy is indicated, preferably performed
within hours of the onset of bleeding. Refractory bleeding is an indication for a
transjugular intrahepatic portosystemic shunt procedure. Balloon tamponade, under direct
observation with a SengstakenBlakemore tube or Linton tube, may control refractory
bleeding but is associated with considerable morbidity and mortality and should not be
used for more than 24 hours.36
Lower Gastrointestinal Hemorrhage
Infections with Clostridium difficile, cryptosporidium, and fungi, and
typhlitis/neutropenic enterocolitis are associated with bloody stools. Typhlitis is the most
common cause of lower GI bleeding in adults with acute leukemia and is discussed in more
detail below.35Intussusception due to tumor, surgery, or an abscess can cause pain,
intermittent bleeding, or currant jelly stools.37 Most major lower GI hemorrhage in the
pediatric cancer patient is multifactorial, for example, C. difficile enterocolitis in a child
with thrombocytopenia and coagulopathy. Hemorrhoids and anal fissures can cause mild
bleeding, often reported as blood in the toilet bowl or on the toilet paper. Management is
directed at each of the underlying causes.
Gastrointestinal Obstruction
Figure38.4 Contrast study of a 3yearold girl 6 days after removal of a
Wilms tumor shows evidence of complete smallbowel obstruction. The study
illustrates a filling defect from smallbowel intussusception.
Although bowel obstruction from primary cancer is rare in children, it can be seen with de
novo or refractory sarcoma (especially desmoplastic round cell sarcoma), abdominal
lymphoma, bulky renal/adrenal tumors, ovarian and colon carcinoma, and presacral
teratoma. Obstruction from smallbowel intussusception (Fig. 38.4 ) is the presentation
of 17% to 25% of abdominal Burkitt lymphomas.37 The differential diagnosis of bowel
obstruction in the child who has had adjuvant therapy or previous abdominal surgery
includes obstipation or paralytic ileus induced by vinca alkaloids or narcotics, adhesions or
strictures, and intussusception.9,32 Abdominal compartment syndrome, defined as elevated
intraabdominal pressure (≥20 mm Hg) associated with respiratory compromise and renal
insufficiency, can be seen in patients with bulky abdominal tumors at diagnosis or as a
postoperative complication.38
History, physical examination, and plain radiographs that document obstipation can
help differentiate narcotic or vincristinerelated ileus from a surgical abdomen. CT scan
with oral contrast can delineate soft tissue masses, air in the bowel wall, and sometimes
reduce intussusception.32Smallbowel obstruction is initially managed by making a patient
NPO and placing a nasogastric tube for decompression. Evaluation for the underlying
etiology should proceed as described in laboratory and diagnostic studies. Intussusception
is often diagnosed and temporarily reduced by aircontrast or barium enema, but surgical
reduction is generally necessary for intussusception caused by leadingedge tumors.37,39
Gastrointestinal Perforation
Perforation may be the outcome of unresolved obstruction, ulcers, or gastritis
unresponsive to medical therapy, infections such as typhlitis, or erosion by the primary
tumor.9,32 In the case of abdominal Burkitt lymphoma, perforation may occur at
presentation, during therapy, or after clinical improvement in association with necrosis of
the underlying tumor.40 Upright abdominal radiographs may reveal air under the
diaphragm, tracking into the liver, or along the flank on a decubitus view. CT scan may also
show free air but is better at delineating pneumatosis intestinalis, infectious abscess,
extravasation of oral contrast, and an obstructing soft tissue lesion.9,32 Perforations are a
surgical emergency and are managed either by resection of the affected area and secondary
closure or by primary reanastomosis, along with broadspectrum antibiotics.9,32,40
Gastrointestinal Infection and Inflammation
Typhlitis, also referred to as neutropenic enterocolitis, is a necrotizing colitis typically
localized to the terminal ileum and cecum, but can occur anywhere in the colon and
occasionally in the small bowel. Traditionally, it was defined as the triad of neutropenia,
abdominal pain, and fever, primarily seen in patients with acute leukemia. Improved
imaging, revealing bowel wall thickening and pneumatosis, has enabled diagnosis of
typhlitis in both neutropenic and nonneutropenic patients, and in children with solid
tumors. Symptoms in order of frequency include abdominal pain (90%), fever (84%),
diarrhea (72%), and, less frequently, nausea/vomiting, and GI bleed. The incidence of
typhlitis in children with cancer is reported at 1.4% to 11.6%, and 65% to 75% of cases are
in patients with hematologic malignancies.41,42,43,44
Figure38.5 CT scan showing a poorly defined soft tissue mass and
indicating a phlegmon (arrowheads) and thickened cecal wall (curved arrow) in
a patient with typhlitis.(Courtesy of James S. Meyer, MD, Children’s Hospital of
Philadelphia.)
Typhlitis usually occurs within 2 weeks of cytotoxic chemotherapy or steroids and tends
to occur as the ANC is nadiring.41,42,43 Other risk factors for typhlitis include age >10 years,
being postBMT, and visible mucositis. The cause of typhlitis is unclear but is believed to
result from a combination of mucositis, bacterial overgrowth leading to bowel wall edema,
and impaired vascular blood flow to the intestinal wall. This can lead to worsening
ulceration, necrosis, and perforation.41,42,43,45 Gramnegative bacteria, such
as Pseudomonas species,Escherichia coli, and Clostridium species, are the most common
bacterial pathogens, butStaphylococcus, Streptococcus, and Enterococcusspecies have
been identified.41,45,46,47,48 Fungus, including Candida and Aspergillus species, has been
identified as less frequent pathogens.43,44Radiographic studies may demonstrate
thickening of the bowel wall (>0.3 mm), free air, or pneumatosis intestinalis.41 In a
retrospective review of 24 children with typhlitis, Sloas et al.45 found that CT scan (Fig.
38.5 ) and US were more sensitive than plain radiographs; falsenegative rates were 15%
for CT, 23% for US, and 48% for plain radiographs. US is reported to be equivalent to CT in
detecting typhlitis and appendicitis and eliminates radiation exposure.41 Patients with
bowel wall thickening greater than 1 cm on US are more likely to have a longer duration of
symptoms and a worse outcome.
In the past, the mortality rate from typhlitis ranged from 20% to almost 100% with
either surgical or medical treatment, but now stands at 0% to 8.3%, primarily with medical
treatment alone.41,42,45 Surgical intervention in typhlitis should be reserved for persistent
GI bleeding despite resolution of thrombocytopenia and correction of clotting
abnormalities, evidence of free air or perforation, necrotic bowel causing sepsis,
obstruction, or failure of medical management.43,44In two recent reports, only 4% to 8% of
patients with typhlitis ultimately required a surgical intervention.41,42 Pneumatosis and
localized peritoneal signs are not sufficient to warrant surgical exploration in the absence
of one or more of the above indications. Using these criteria, most patients can be managed
medically with broadspectrum antibiotics to cover gramnegative pathogens, GI
anaerobes (metronidazole or a carbapenem), and fungi.
Typhlitis and appendicitis have very similar presentations in children with cancer.
Appendicitis also presents with abdominal pain, but typhlitis is more frequently associated
with fever, diarrhea, and prolonged neutropenia.49 Incidence rates of appendicitis range
from 0.5% to 1.5% and is primarily seen in children with leukemia or lymphoma.49 Despite
the sensitive imaging modalities of US and CT, onethird of oncology patients with
appendicitis have a delay in diagnosis due to blunted inflammatory response and a broad
differential of abdominal pain.49,50 Case reports exist of appendicitis being treated
successfully with conservative medical management alone.51
Patients who do not have typhlitis but have signs and symptoms of enterocolitis may
have transverse colitis, intussusception, or antibioticrelated pseudomembranous or
clostridial enterocolitis. C. difficile colitis is now the major cause of nosocomial diarrhea in
the United States and Western Europe.48Although use of second and thirdgeneration
cephalosporins, clindamycin, ampicillin, and amoxicillin is associated with the highest risk
of C. difficile colitis, even a single dose of almost any antibiotic can predispose a patient
to C. difficilecolitis. The specific therapy for C. difficile colitis is oral metronidazole or oral
vancomycin.48 Infection with C. septicum is also a diagnostic consideration in neutropenic
patients, and may present without significant fever, but can progress rapidly.52 Any
suspected abdominal infection in neutropenic patients merits urgent antibiotic coverage.
Perirectal Abscess
Anorectal pain, tenderness, and discomfort with bowel movements may indicate a
perirectal abscess or fistula. Perirectal abscesses occur in patients with neutropenia,
especially those with acute myelogenous leukemia (AML).44 In a neutropenic patient, the
only physical finding may be tender, brawny woody edema with a dense cellulitic reaction.
Most abscesses are caused by a mixture of aerobes (including staphylococci, E. coli,
Pseudomonas, and streptococci) and fecal anaerobes. Initial therapy includes antibiotics to
cover aerobic and anaerobic gramnegative and grampositive bacteria. Sitz baths may
relieve pain. If the abscess or induration is well circumscribed or progresses, the lesion
may need to be incised and drained, but early aggressive medical management often
eliminates the need for surgical intervention, particularly while the patient is neutropenic.
Gastrointestinal Organ Dysfunction
Gastrointestinal Organ Dysfunction
Cholecystitis and Biliary Obstruction
Inflammation of the liver and biliary tract can present as localized right upper quadrant
pain or jaundice. The most common cause of hyperbilirubinemia, often asymptomatic, is
temporary hepatic toxicity from chemotherapy. Acute cholecystitis, particularly acute
acalculous cholecystitis, occurs in children who are septic, stressed, and volume depleted.
Biliary obstruction as a result of primary tumor is rare: lymphoma and neuroblastoma
occasionally block biliary flow, and rhabdomyosarcoma of the common duct occurs.
Ultrasonography or CT scan can differentiate calculous from acalculous cholecystitis.
Hydration, broadspectrum antibiotics, and nasogastric decompression usually treat the
acute cholecystitis. Antibiotics should cover gramnegative bacilli and, in the neutropenic
patient, fungi. Endoscopic retrograde cholangiopancreatography or combined endoscopic
cutaneous decompression is successful in most patients, and stent placement may provide
effective palliation for up to 6 months.
Acute Massive Hepatomegaly
Massive hepatomegaly may complicate stage IVS neuroblastoma or HLH in the neonate
and may be fatal. Unless lifethreatening respiratory embarrassment is present, supportive
care and observation are sufficient until the disease is treated. If hepatomegaly
compromises respiratory function, therapeutic options include standard chemotherapy
and radiation for neuroblastoma, or appropriate HLHdirected therapy.30 Radiation
therapy involves lateral portals with the child supine and fields encompassing most of the
liver, but sparing the ovaries and kidneys, and extending from the dome to the posterior
portion of the right hepatic lobe. A midplane dose of 150 cGy given on 3 successive days
has been sufficient to ameliorate lifethreatening hepatomegaly resistant to
chemotherapy.30
Venoocclusive Disease
VOD, also referred to as hepatic sinusoidal obstruction syndrome, consists of rapid and
often massive hepatic enlargement with resultant right upper quadrant pain, liver
tenderness, jaundice, weight gain, and ascites. VOD is most frequently a complication of
cytoreductive therapy for BMT and can range from mild to severe.31,53 Preexisting hepatic
disease, radiation, allogeneic transplant, use of busulfan with our without CPM, and
younger age have all been identified as risk factors for developing VOD in BMT.31,53 Mild
tomoderate reversible VOD has also occurred following vincristine and actinomycinD
with or without CPM therapy for rhabdomyosarcoma and Wilms tumor.54 Those younger
than 36 months of age were found to be at greatest risk for the development of hepatopathy
after treatment with vincristine, actinomycinD, and CPM for rhabdomyosarcoma, with an
incidence of 5%.54 Withholding vincristine or actinomycinD generally leads to
symptomatic improvement. Gemtuzumab therapy for AML is associated with a 15% to 24%
incidence of VOD, primarily developing days to weeks into BMT.55
In both the Children’s Oncology Group (COG) and United Kingdom pediatric ALL trials
that randomized 6mercaptopurine with thioguanine in maintenance, thioguanine was
associated with a significantly increased incidence of VOD during treatment, with a male
predominance.56,57 The rate of VOD in the thioguaninetreated patients was 10% to 15%
and higher if patients with evidence of portal hypertension such as splenomegaly and
thrombocytopenia were included. Although symptoms resolved in most patients within
weeks of stopping antimetabolites, more than 10% of affected patients had persistent
splenomegaly and thrombocytopenia, as well as evidence of varices, abnormal liver
biopsies, and portal hypertension requiring close followup by a gastroenterologist.56,57,58
Treatment of VOD is primarily supportive in the nontransplant setting.54 Historically,
children who developed irreversible VOD postBMT had a mortality rate approaching
100%, but use of defibrotide (a fibrinolytic, antithrombotic, and antiischemic agent) has
been associated with complete response rates between 29% and 46% and a 42% to 62%
survival at 100 days postBMT.31,53,59,60 Newer data also support the use of prophylactic
defibrotide (6.25 mg/kg) IV 4 times daily in pediatric patients at high risk of developing
VOD in BMT.31
Pancreatitis
When vomiting and abdominal pain occur in a child receiving asparaginase or
corticosteroids or in a child with ICP, pancreatitis should be included in the differential
diagnosis.61,62 The reported incidence of asparaginaseassociated pancreatitis ranges from
1% to 18%, tending to occur early in ALL/NHL therapy. Studies have shown that
pancreatitis is associated with concomitant steroid use and older age.61,62 An elevated
pancreatic amylase and lipase usually make the diagnosis, and imaging may only be
necessary for severe or persistent pain. An US or CT scan with contrast may show a
phlegmon as a diffuse homogenous area, and pancreatic pseudocysts may appear as
loculations of various densities within the pancreas.50
The majority of patients with clinically symptomatic pancreatitis require inpatient
admission, complete bowel rest, and intravenous narcotics for pain
management.63 Parenteral nutrition may be necessary. Placement of a nasogastric tube is
indicated for intractable emesis or an ileus. Antibiotics should be started for evidence of
pancreatic necrosis, abscess, or phlegmon, and should include gramnegative and
anaerobic coverage. Octreotide, a synthetic somatostatin analog, may ameliorate the
clinical course of pancreatitis in children.64 Most cases of pancreatitis are selflimited and
data:text/html;charset=utf8,%3Cheader%20id%3D%22812dba18126443379f31e4fdf0001bb3%22%20style%3D%22margin%3A%200px%2015px%2015px%… 10/11
can be treated medically. Indications for surgical drainage of a pancreatic abscess or
pseudocyst depend on the child’s condition and the evolution of the process on sequential
US or CT examinations. Necrotizing pancreatitis and large pseudocysts causing severe pain
or obstruction often require urgent surgical drainage. Percutaneous CTguided drainage by
an interventional radiologist may also be effective.65
data:text/html;charset=utf8,%3Cheader%20id%3D%22812dba18126443379f31e4fdf0001bb3%22%20style%3D%22margin%3A%200px%2015px%2015px%… 11/11

03gastrointestinal Emergencies - Principles and Practice of Pediatric

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

03gastrointestinal Emergencies - Principles and Practice of Pediatric

Uploaded by

Copyright:

Available Formats

15/9/2015 Gastrointestinal Emergencies | Principles and Practice of Pediatric…

You might also like