Transcript Rosher G.Uncommon causes of severe abdominal pain

Uncommon causes of
severe abdominal pain
Guy Rosner M.D.
Department of Gastroenterology and Liver Diseases
Tel-Aviv Sourasky Medical Center
Case #1
• 23 years old male of Ashkenazi origin
• Healthy in general except recurrent
episodes of severe abdominal pain
• No family history of inherited diseases
Case #1
• Monthly bouts of severe diffuse abdominal pain
lasting 12-48 hours and resolving spontaneously
• First episode occurred at age 12 leading to
emergency laparotomy with no overt abnormality
detected except for mild edema of the small
bowel
• Vomiting, watery diarrhea and swelling of both
hands occurred concurrently
Case #1
• Physical examination was unremarkable
• Laboratory tests were all within normal limits
(including tests to exclude porphyria, FMF, and
celiac disease)
• Abdominal imaging (repeated CT scans) shows
diffuse thickening of the duodenal and small bowel
wall (in one episode small bowel intussusception
was suspected)
Case #1
Procedure of choice?
Blood analysis for C3, C4, C1 esterase inhibitor
levels
C3 - 87.4 mg/dl (normal < 80)
C4 - 2.4 mg/dl (normal >15)
C1 esterase inhibitor – 538 mg/dl (302-414) but
reduced activity on functional assays
Case #1 - Diagnosis
C1 esterase inhibitor deficiency
(Hereditary Angioedema)
Case #2
• 29 years old male, healthy in general
• 2 weeks of severe colicky upper abdominal pain
• Episodes of pain begin at night and last till the
morning and are accompanied by hiccups
• No fever, nausea, vomiting or diarrhea
Case #2
• Physical examination – upper abdominal tenderness
• Normal laboratory tests
• Imaging:
– Normal abdominal X-ray
– Normal abdominal ultrasound
• Gastroscopy – mild chronic carditis
• No improvement while taking PPI’s
Case #2 – Abdominal CT scan
Diagnosis ?
Case #2 - Diagnosis
segmental omental infarction
Case #3
• 25 years old female, healthy in general
• Intermittent episodes of severe colicky upper abdominal
pain with nausea and vomiting during the last couple of
weeks
• Pain relives while patient lies in “fetal position”
• Physical examination was unremarkable except for an
asthenic habitus (BMI 17)
• Normal lab tests, normal abdominal ultrasound
Case #3 – Upper GI study
• Dilatation of the 2nd-3rd duodenum
• Temporary obstruction of barium passage through the 3rd part of the
duodenum
Case #3 – Abdominal CT angiography
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Mild dilatation of the proximal part of the duodenum (2.5 cm)
Suspected stenosis of 3rd part of the duodenum (area between the SMA and
Aorta)
The angle between the SMA and Aorta is less than 40° (normal 40° - 60° )
The Aortomesenteric distance is only 4mm (normal 10-20mm)
Case #3 - Diagnosis
Superior Mesenteric Artery (SMA)
Syndrome
Discussion
3 infrequent causes of severe abdominal pain
• Hereditary angioedema
• Segmental omental infarction
• SMA syndrome
Hereditary angioedema
Background
• C1 esterase inhibitor (C1 INH) deficiency
• Subcutaneous and submucosal swellings in any
part of the skin / respiratory tract /GI tract
• The cardinal symptom is severe crampy
abdominal pain which can occur many years
before the characteristic skin edema*
*
Bork et al. Am J Gastro 2006
Hereditary angioedema
Background
• Circulatory collapse occurs in 4%*
• Symptoms usually appear early in life and are
normally accompanied by a family history
*
Bork et al. Am J Gastro 2006
Hereditary angioedema
Background
• Prevalence of 1/50,000
• Inherited in an autosomal dominant manner
• Spontaneous mutation rate of 25%
• No ethnic predilection
Hereditary angioedema
The complement system
Hereditary angioedema
Immunology
• C1 INH is the main regulator of the activation in the
classical complement pathway
• C1 INH also regulates the activation of kallikrein, plasmin,
and factors XI, XIIa
• When C1 INH is deficient
– inappropriate activation of the classical pathway
– Increase in kallikrein and bradykinin levels
Increase in vascular permeability and massive
local uncontrolled edema
Hereditary angioedema
Clinical Prospective
Type I
• 85% of all patients
• Deficiency of C1 INH
• Low plasma levels of
C1 INH (5%-30%)
Type II
• Normal to high plasma
C1 INH levels
• The C1 INH protein is
dysfunctional
An acquired form of angioedema is recognized, which is
much less prevalent, affects older patients and is associated
with lymphoproliferative disease or autoimmunity
Hereditary angioedema
Diagnosis
• Angioedema
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Recurrent
>24 h
Non-pruritic
Non-responsive to antihistamines
• No urticaria
• Unexplained abdominal pain (constriction by the
intestinal wall and mesenteric edema)
– Recurrent
– ‘Colicky’
• Family history
• Low C4 level
Hereditary angioedema
Laboratory
• Low serum C4 level (<30% of mean normal level)
• When C4 level is low proceed with measuring C1
INH level
– In type I: C1 INH level is low (immunochemistry)
– In type II: C1 INH level is normal or increased but the
protein is dysfunctional
• Repeat the test in order to exclude false positive
results
Hereditary angioedema
Treatment
• Primary prevention
– Identify triggers (infections, HP eradication, avoidance of
estrogens and ACE inhibitors)
• Long-term prophylaxis – reduce frequency and
severity of attacks
– Antifibrinolyric agents (tranexamic acid, EACA)
• Inhibit plasminogen activation with ‘sparing’ of C1 INH usage
– Synthetic attenuated androgens (danazol 400 mg/day,
stanozolol)
• Increase the biosynthesis of C1 INH protein
• Emergency care (acute attacks)
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C1 INH concetrate (500-1500 U)
Attenuated androgens (danazol up to 1 g/day)
Tranexamic acid 1gx4 in 48 hr
FFP (2 units) – only when C1 INH concentrate is not available
Hereditary angioedema
Back to case presentation
• Treatment with danazol 200 mg twice daily
started
• No episodes of abdominal pain have been
reported since then (4 months)
Segmental omental infarction
Segmental omental infarction
Backgroung
• Pathology:
– Acute / chronic inflammation of the omentum with
vascular thrombosis, fat necrosis and fibroblastic
reaction
• Pathogenesis:
– Omental torsion (usually due to anatomical
malformation)
– Venous thrombosis of vessels supplying the omentum
– Congenital vascular anomaly of the greater omentum
– Obesity as a risk factor (relative omental ischemia?)
Segmental omental infarction
Etiology
– Primary
• without a recognized predisposing abnormality
– Secondary to
• torsion
• hypercoagulable states
• vasculitis
• pancreatitis
• postprandial vascular congestion
• anomalus arterial supply to the omentum
• kinking of the veins secondary to increased intraabdominal pressure
Segmental omental infarction
Clinical presentation
– Localized pain with peritoneal signs
– Absence of other GI symptoms
– Normal lab tests
Segmental omental infarction
Diagnosis
Restricted mass with dense fat tissue and
hyperdense streaks, usually located at the right
side of the omentum
Segmental omental infarction
Treatment
• This condition is considered to be benign, self-limiting,
with spontaneous resolution
• Conservative treatment – observation with analgesics
• There is some risk for abscess or adhesion formation
due to the necrotic intrabdominal mass
• Role of evaluation for hypercoagulable states is not
established
Segmental omental infarction
Back to case presentation
With conservative treatment symptoms resolved
in a couple of weeks
Superior Mesenteric Artery (SMA)
Syndrome
Superior Mesenteric Artery (SMA)
Syndrome
• An uncommon entity characterized by
compression of the transverse (third) portion of
the duodenum against the aorta by the SMA
• Exact frequency is not known, but is suspected in
0.013%-0.3% of upper GI series
• Females > males
• Usually occurs in adolescents
SMA Syndrome - Pathophysiology
• Normal angle range (between the SMA and abdominal aorta) is
40o-60o
• Narrowing of that angle to 6o-25o can cause entrapment and
compression of the third part of the duodenum
• The aortomesenteric distance in SMA syndrome is decreased to
2-8 mm (normal 10-20mm)
SMA Syndrome
• The clinical presentation is that of chronic /
intermittent / acute complete or partial duodenal
obstruction
• The symptoms are typically relieved in the left
lateral decubitus / knee-to-chest position
• Asthenic habitus is common (80% of cases)
SMA Syndrome - Causes
• Constitutional factors
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Thin body build
Severe lumbar lordosis
Visceroptosis and abdominal wall laxity
Depletion of the mesenteric fat (i.e. weight loss)
• Severe injuries with prologed bed rest
• Dietary disorders
– Anorexia nervosa
– Malabsorption
• Unusual
– Familial SMA syndrome
– Recurrent SMA syndrome
– Traumatic aneurysm of the SMA after a stab wound
SMA Syndrome - Imaging
• Upper GI study
– Characteristic dilatation of the first and second
parts of the duodenum with abrupt cutoff in
the third part with normal mucosal folds
• CT scan
– Provides diagnostic information (Aorta-SMA
angle and distance and duodenal distention)
SMA Syndrome - Treatment
• Remove the precipitating factor
• Conservative treatment:
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Adequate nutrition
GI decompression
Proper positioning of the patient after eating
Metoclopramide
• In cases of rapid and severe weight loss:
– Enteral feeding tube
– TPN
• Surgical intervention:
– Duodenojejunostomy is successful in 90%
– Laparoscopic lysis of the ligament of Treitz and mobilization of
the duodenum
SMA Syndrome
Back to case presentation
With proper diet the patient gained
weight and symptoms resolved
Thank you!