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27 Colonic Ischemia (Also Known as Ischemic Colitis) 263
Fig. 27.5 Coronal CT image of the same patient demonstrates the peripheral location of the portal
venous gas (oval). Imaged portions of hepatic flexure and descending colon (arrows) show marked
colonic wall and haustral thickening giving the appearance of “thumbprinting”. Note adjacent fat
stranding (asterisk) involving the hepatic flexure. With permission from Dr. Abraham Dachman
and Dr. Justin Ramirez
characteristic finding on colonoscopy in patients with ischemic colitis (Fig. 27.6).
Other mucosal lesions including edema, erythema, pale regions and bluish hemorrhagic nodules. Frank bleeding may also be present. Biopsies demonstrate
mucosal and submucosal hemorrhage, edema, crypt destruction, and granulation
tissue.
Fig. 27.6 Colonoscopy shows submucosal hemorrhage and mucosal edema in a patient with
colonic ischemia
264 C. Lee and E. D. Ehrenpreis
Histologic Findings
Histologic findings include mucosal and submucosal hemorrhage, edema, crypt
destruction, and granulation tissue. These changes can mimic histologic findings of
Crohn disease. Ghost cells, preserved cellular outlines without cell content, can be
pathognomonic for infarction in CI.
Treatments
Medical
Treatments of CI vary according to the severity of the disease (Table 27.3).
Based on previous studies, severity of CI is generally divided into mild, moderate and severe forms. Patients with mild disease have typical symptoms of CI
Table 27.3 Ischemic colitis: classification of disease severity and management
Disease
severity
Mild Typical symptoms of CI with a segmental colitis not
Moderate Any patient with CI and up to 3 of the following
Severe Any patient with CI and more than 3 of the criteria for
Adapted from: Brandt, Lawrence. ACG Clinical Guideline: Epidemiology, Risk Factors, Patterns
of Presentation, Diagnosis, and Management of Colon Ischemia. AM J Gastroenterol 2015;
110:18–44. Copyright © Wolters Kluwer 2015
Criteria Treatment
isolated to the right colon and with none of the
commonly associated risk factors for poorer outcome
that are seen in moderate disease
factors:
Male gender
Hypotension (systolic BP <90 mm Hg)
Tachycardia (HR >100 beats/min)
Abdominal pain without rectal bleeding
BUN >20 mg/dl
Hgb <12 g/dl
LDH >350 U/I
Serum sodium <136 mEq/l (mmol/l)
WBC >15 cells/cmm (109/l)
Colonic mucosal ulceration identified
colonoscopically
moderate disease or any of the following:
Peritoneal signs on physical examination
Pneumatosis or portal venous gas on radiographic
imaging
Gangrene on colonoscopic examination
Pancolonic distribution or isolated right colonic
ischemia (IRCI) on imaging or colonoscopy
Observation
Supportive care
Correction of
cardiovascular
abnormalities
Broad-spectrum
antibiotic therapy
Surgical consultation
Emergent surgical
consultation
Transfer to intensive
care unit
Correction of
cardiovascular
abnormalities
Broad-spectrum
antibiotic therapy
27 Colonic Ischemia (Also Known as Ischemic Colitis) 265
without isolated right colonic disease. Moderate disease has other associated factors
(male patient, hypotension, tachycardia, abdominal pain without rectal bleeding,
blood urea nitrogen >20 mg/dl, Hgb <12 g/dL, LDH >350 U/l, serum sodium
<136 mEq/l, WBC >15 109/l or mucosal ulcerations. Severe CI is defined by
more than three of the above factors, peritoneal signs, pneumatosis on CT or
gangrene on colonoscopy.
Most cases of CI are mild and self-limited. If hospitalized, patients can be treated
with supportive measures with intravenous hydration, electroly te correction and
bowel rest. There is currently no strong evidence to support the administration of
antibiotics in patients with mild CI, although they may be indicated in moderate to
severe disease. Animal models do demonstrate that antibiotics decrease bacterial
translocation during acute ischemia and reperfusion injury. Currently, the choice of
antimicrobial agents and optimal duration of thera py have not been established. Of
interest, some antibiotics are also associated with the development of CI. In severe
cases of CI, surgical intervention is required as mortality from necrotic bowel can
be as high as 100%. Acute indications for immediate surgery include peritoneal
signs, massive bleeding, fulminant colitis, and porta l venous gas and pneumatosis
intestinalis on imaging. In addition, failure to respond to treatment within 2 –
3 weeks with continued symptoms or a protein-losing colopathy may require
segmental colectomy. The type of surgical procedure depends on the affected region
of colon. Risk stratification should be considered prior to surgical intervention as
mortality post operation for CI may be as high as 37–47%.
Complications
CI is usually a self-limited condition which requires supportive treatment in most
cases. However, severe ischemia can cause inflammation which can deve lop into a
stricture or segmental ulcerating colitis. Ischemic strictures can be asymptomatic or
cause obstructive symptoms. For patients with symptoms, a repeat colonoscopy
may be required to visualize strictures or segmental colitis. Some strictures resolve
in one to two years but symptomatic strictures usually require segmental bowel
resection. Recurrent episodes of colonic ischemia associated with segmental colitis
may also require elective segmental resection. Unfavorable outcomes were more
frequent in isolated right colon ischemia compared with other regions of the colon
(40.9 vs. 10.3%).
Suggested Readings
1. Alpern MB, Glazer GM, Francis IR. Ischemic or infarcted bowel: CT findings. Radiology.
1988;166:149.
2. Bailey RW, Bulkley GB, Hamilton SR, Morris JB, Smith GW. Pathogenesis of nonocclusive
ischemic colitis. Ann Surg. 1986;203(6):590–9.
266 C. Lee and E. D. Ehrenpreis
3. Salk A, Ehrenpreis ED. Ischemic colitis with Type I interferons used in the treatment of
hepatitis C and multiple sclerosis: an evaluation from the food and drug administration adverse
event reporting system and review of the literature. Ann Pharmacother. 2013;47:537–42.
4. Brandt L. ACG clinical guideline: epidemiology, risk factors, patterns of presentation,
diagnosis, and management of colon ischemia. AM J Gastroenterol. 2015;110:18–44.
5. Kontogianni A, Delakidis S, Basioukas P, Tsantoulas D, Tamvakis N, Demonakou M.
Ischemic colitis: clinical, endoscopic and histologic spectrum of 254 cases. Ann Gastroenterol. 2003;16.
6. Longstreth GF, Yao JF. Diseases and drugs that increase risk of acute large bowel ischemia.
Clin Gastroenterol Hepatol. 2010;8:49.
7. Luo CC, Shih HH, Chiu CH, et al. Translocation of coagulase-negative bacterial
staphylococci in rats following intestinal ischemia-reperfusion injury. Biol Neonate.
2004;85:151–4.
8. Midian-Singh R, Polen A, Durishin C, et al. Ischemic colitis revisited: a prospective study
identifying hypercoagulability as a risk factor. South Med J. 2004;97:120–3.
9. Mitsudo S, Brandt LJ. Pathology of intestinal ischemia. Surg Clin North Am. 1992;72:43.
10. Montoro M, Brandt L, Santolaria S, et al. Clinical patterns and outcomes of ischaemic colitis:
results of the working group for the study of ischaemic colitis in Spain (CIE study). Scand J
Gastroenterol. 2011;46:2:236–46.
11. Mosele M, Cardin F, Inelmen EM, et al. Ischemic colitis in the elderly: pre-dictors of the
disease and prognostic factors to negative outcome. Scand J Gastroenterol. 2010;45:428–33.
12. Smerud MJ, Johnson CD, Stephens DH. Diagnosis of bowel infarction: a comparison of plain
films and CT scans in 23 cases. AJR Am J Roentgenol. 1990;154:99.
13. Theodoropoulou A, Koutroubakis IE. Ischemic colitis: clinical practice in diagnosis and
treatment. World J Gastroenterol. 2008;14:7302.
14. Washington C, Carmichael JC. Management of ischemic colitis. Clin Colon Rectal Surg.
(2012);25(4):228–35. PMC. Web. 6 Oct. 2018.
Mesenteric Lymphangioma
Eli D. Ehrenpreis and Siddhartha Guru
Definition
Lymphangiomas are very rare, benign lesions that are a result of an abnormality of
thin-walled lymphatic structures. Lymphangiomas are characterized by an increase
in the number or size of lymphatic channels and lymphatic spaces, or the occurrence of large, partially organized cystic lesions. A very small number of cases
involve the portion of the mesentery that is proximal to the small intestine. They
appear as mass-like or cystic lesions on abdominal imaging.
Epidemiology
28
Lymphangiomas are rare lesions with fewer than 900 cases reported in the medical
literature. Of these, only 72 cases involving the mesentery adjacent to the small
intestine have been described. Lymphangiomas are most commonly found in the
soft tissues of the head and neck. These are most often diagnosed in children, with
50% occurring at birth and 90% before the age of 2. A 3:1 male to female ration has
been suggested for adult forms of mesenteric lymphangioma.
E. D. Ehrenpreis (&) S. Guru
Department of Medicine, Advocate Lutheran General Hospital, 1775 Dempster St, 6 South,
Park Ridge, IL 60068, USA
e-mail: e2bioconsultants@gmail.com
© Springer Nature Switzerland AG 2021
E. D. Ehrenpreis et al. (eds.), The Mesenteric Organ in Health and Disease,
https://doi.org/10.1007/978-3-030-71963-0_28
267
268 E. D. Ehrenpreis and S. Guru
Patients at Risk
Pediatric cases of lymphangioma are congenital, with no stated relationship to other
congenital disorders. Abdo minal trauma, abdominal surgery, lymphatic obstruction
and radiation injury appear to be risk factors for the development of the acquired
form of mesenteric lymphangioma in adults.
Pathophysiology
Congenital lymphangiomas arise from abnormal embryonic development of lymphatic tissue. Lymphangioma occurrence may result blockage or arrest of normal
growth of the primitive lymph channels, failure of the primitive lymphatic sac to
reach the venous system, or inappropriate location of lymphatic vessels during
embryogenesis. In adult patients, lymphangiomas may occur after abdominal surgery trauma, inflammation, or radiation injury.
Clinical Presentation
A variety of presentations of mesenteric lymphangioma have been described,
ranging from asymptomatic patients to an acute abdomen and bowel obstruction
from volvulus or intussusception. In a recent ly published series of six patients with
mesenteric lymphangiomas, all were diagnosed after acute presentations, but one
patient had a two-year history of abdominal bloating. Patients may develop
symptoms from a mass effect from compression of adjacent small bowel or other
organs. Fever, nausea, diarrhea and gastrointestinal bleeding have also been
described.
Physical Findings
In the head and neck, lymphangiomas present as painless, soft, and compressible
masses that have a slow rate of growth. In patients with mesenteric lymphangiomas,
abdominal tenderness and a palpable mass may be present on examination. Signs
and symptoms of intestinal obstruction can be seen in cases with volvulus or
intussusception. The acute abdomen presentation of lymphangioma can be confused with appendicitis and a myriad of other causes.
28 Mesenteric Lymphangioma 269
Laboratory
Complications of mesenteric lymphangiomas may be manifested by anemia,
leukocytosis, and a variety of biochemical abnormalities.
Histology
Lymphangiomas may be divided into three histologic subtypes: capillary (simple),
cavernous and cystic. The capillary lymphangioma are composed of small thin
walled lymphatic vessels and are usually located in the skin. Cavernous lymphangiomas are connected to nearby lymphatic spaces and are composed of various
sized lymphatic vessels and lymphoid stroma. Cystic lymphangiomas are characterized by the formation of large cystic lymphatic spaces with collagen and smooth
muscle bundles lined by endothelial layers. These cystic lymphangiomas with lack
connections to adjacent normal lymphatics and major lymphatic vessels of the small
intestine.
Immunohistochemical testing with D2-40 on cystic wall endothelium confirms the
presence of lymphatic endothelial cells. Differentiation from Lymphangiomyoma and
multicystic mesothelioma is performed by testing for HMB-45 immunoreactivity and
staining for calretinin. Lymphangiomyoma has HMB-45 immunoreactivity and
mesothelioma will stain positive for calretinin, while both of these are negative in
lymphangioma. Cell that line the cystic walls of lymphangiomas may be
immunoreactive for factor VIII-related antigen.
Imaging
Abdominal radiography may be used to diagnose bowel obstruction, volvulus and
intussusception. Abdominal ultrasound and CT are the most commonly imaging
studies used to diagnose mesenteric lymphangioma and to differentiate it from other
disorders. Nonetheless, due to the rarity of mesenteric lymphangiomas, they are
often misdiagnosed when seen on standard imaging studies. Ultrasound may reveal
a multiloculated cystic mass or masses with thin internal septae. On CT scan,
mesenteric lymphangiomas present as unilocular or multilocular masses with
contrast enhancement of the cyst walls and septae. Cystic lesions are most often
homogeneous, but appear inhomogeneous if they contain proteinaceous fluid, blood
or fat components. Lymphatic vessels may be seen coursing between intralesional
locules (Figs. 28.1, 28.2, 28.3, 28.4 and 28.5).
Retroperitoneal lymphangiomas ae very uncommon and may present as nonspecific masses or cystic lesions within the retroperitoneal space (Figs. 28.4 and 28.5).
270 E. D. Ehrenpreis and S. Guru
Fig. 28.1 Coronal CT image from a pediatric patient demonstrates mesenteric lymphangioma. An
expansile intraperitoneal cystic lesion with thin enhancing septations (arrows) is seen. Note the
displaced gas filled bowel at the periphery of the lesion
Fig. 28.2 CT image from the same patient demonstrates discrete, large intraperitoneal cystic
structures displacing adjacent organs. Subtle increased attenuation within an anterior cystic
structure (asterisk) suggests a more complex fluid component such as protein or blood products
MRI may demonstrate hemorrhagic lesions with increased signal intensity on the
T2 images. Imaging studies cannot in themselves be used to diagnosis mesenteric
lymphangioma as their appearance may mimic other cystic lesions such as
teratomas.
28 Mesenteric Lymphangioma 271
Fig. 28.3 Sagittal CT image
better demonstrates the
significant mass effect, nearly
filling the entire
Fig. 28.4 Axial CT image
from a patient showing a
retroperitoneal lymphatic
malformation in an adult
(oval)
Diagnosis
The diagnosis of mesenteric lymphangioma is diagnosed on histological examination and staining of tissue from excised lesions, (see Histology section above).
272 E. D. Ehrenpreis and S. Guru
Fig. 28.5 Axial T2-weighted MR image from the same patient demonstrates the septated, cystic
nature of the lesion. When discovered incidentally, as in this patient, the lesion is often
asymptomatic with little or no mass effect on adjacent structures. With permission from
Dr. Abraham Dachman and Dr. Justin Ramirez
Immunohistochemical testing with D2-40 on cystic wall endothelium is done to
determine the presence of lymphatic endothelial cells. Which would be positive in
both lymphangiomyoma and multicystic mesothelioma hence are further differentiated by testing using HMB-45 immunoreactivity and staining for calretinin. While
lymphangiomyoma will be positive with HMB-45 immunoreactivity and
mesothelioma will stain positive for calretinin. They are also immune reactive for
factor VIII related antigen.
Treatment
Medical
Although surgery is the mainstay of treatment for lymphangiomas, broad spectrum
antibiotics with symptomatic treatment with acetaminophen may be used as adjunctive
therapies. Intralesional injection therapy with OK-432, a lyophilized biological
preparation containing cells of Streptococcus Pyogenes su-strain treated with Benzylpenicillin has demonstrated efficacy in children with head and neck lesions.
Surgery
Surgical excision is the treatment of choice for mesenteric lymphangioma. The
rationale for surgery is that lesions can increase in size and impact adjacent organs.
Complications of unresected mesenteric lymphangiomas include bowel obstruction,