gastrointestinale Manifestationen Sklerodermie
Sklerodermie
mit Beteiligung des Ösophagus: Während im kranialen Abschnitt des Ösophagus noch eine weitgehend normale Peristaltik vorhanden ist, ist der Ösophagus in den unteren zwei Dritteln dilatiert und deutlich hypomotil. Das Kontrastmittel fällt passiv durch das "Glasrohr". Die Passage durch die Cardia erfolgt aber weitgehend regelrecht, was die Abgrenzung zur Achalasie erlaubt.
Sklerodermie
mit Beteilung des Ösophagus: Im unteren Ösophagus (glatte Muskulatur!) deutliche Wandstarre mit passivem Durchfallen des Kontrastmittels / des Bolus (Glasrohr-Ösophagus).
Imaging of
intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Intestinal vasculitis and benign intestinal pneumatosis in a 52-year-old male with a history of known Scleroderma presenting with abdominal pain. Physical examination revealed generalized abdominal tenderness without rebound or guarding. Upright CXR (A) was done in the ER. Pneumoperitoneum (thick black arrows) was noted at CXR in the absence of signs or symptoms of peritonitis. Due to this discrepancy, abdominal CT scan (B, C) was performed and confirmed pneumoperitoneum (thin black arrow) along with extensive intestinal pneumatosis in the transverse colon (black arrowheads) and jejunum (thick white arrows). The patient underwent conservative treatment. He was discharged one week later with improved signs/symptoms. After three weeks, MRE was obtained and displayed no pneumoperitoneum. Axial T2-W image (D) demonstrates segmental jejunal mural thickening (white dotted oval) with hypointense appearance. Coronal T2-W image (E) shows bowel dilation and the classic hidebound sign (black dotted oval) in the ileum related to known scleroderma. The T2 hypointense segment of thick jejunum is also seen (curved arrows), suggestive of chronic mural thickening perhaps due to fibrosis
Scleroderma
• Scleroderma with interstitial fibrosis - Ganzer Fall bei Radiopaedia
Scleroderma
• Scleroderma - small bowel involvement - Ganzer Fall bei Radiopaedia
Scleroderma
(gastrointestinal manifestations) • Scleroderma with esophageal cancer and peptic stricture - Ganzer Fall bei Radiopaedia
Scleroderma
(gastrointestinal manifestations) • Duodenal involvement of scleroderma - Ganzer Fall bei Radiopaedia
Scleroderma
(gastrointestinal manifestations) • Esophageal dysmotility - systemic sclerosis - Ganzer Fall bei Radiopaedia
Scleroderma
(gastrointestinal manifestations) • Scleroderma - esophagus and small bowel involvement - Ganzer Fall bei Radiopaedia
Scleroderma
(gastrointestinal manifestations) • Scleroderma - esophagus and small bowel involvement - Ganzer Fall bei Radiopaedia
Imaging of
intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Colonic pseudo-obstruction in a 35-year-old female with known scleroderma presenting with constipation, nausea, vomiting, and abdominal distension. CT scan with IV/oral contrast was done. Axial CT image (A) demonstrates dilation of the distal esophagus (thick white arrows), a common finding in scleroderma. Axial CT image (B) reveals marked diffuse dilatation of colon (*) without obvious wall thickening or abrupt transition point, suggestive for colonic pseudo-obstruction (Ogilvie syndrome)
Imaging of
intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data. Small bowel obstruction in a 31-year-old female with known scleroderma presenting with generalized abdominal pain, nausea, and vomiting without previous Hx of surgery. Axial view of CTE shows extensive cutaneous and subcutaneous calcifications (thick white arrows). Short segment benign-looking stricture (white arrowhead) is seen at the distal ileum in the pelvis, leading to upstream small bowel dilatation (white asterisks). Following segmental resection and anastomosis, this was confirmed to be a fibrotic stricture. Collapsed ileum distal to stricture is also depicted (thick black arrow)
Gastrointestinal manifestations of scleroderma can occur in up to 90% of patients with scleroderma with the most common site of gastrointestinal involvement being the esophagus. After skin changes and Raynaud phenomenon, gastrointestinal changes are the third most common manifestation of scleroderma.
As the clinical presentation, radiographic appearances and differential diagnosis vary with the location of involvement these are discussed sequentially by region.
For a general discussion of scleroderma, please refer to the parent article: scleroderma.
Pathology
Smooth muscle atrophy and fibrosis are thought to be the chief underlying mechanism which leads to luminal dilatation, reduced motility and reduced sphincter tone.
Esophagus
The esophagus is affected in 80% of scleroderma cases. Symptoms include heartburn and dysphagia.
Radiographic features
- dilatation of distal two-thirds of the esophagus
- deficient esophageal emptying in a recumbent position
- apparent shortening of length due to fibrosis
- dysmotility of the lower esophagus (normal peristalsis above aortic arch)
- gastro-esophageal reflux due to reduced sphincter tone
- air-fluid level in the esophagus when supine (CT)
Complications
- aspiration
- esophagitis
- mucosal erosion
- fusiform stricture ~4-5 cm above the gastro-esophageal junction
- progression to Barrett esophagus (~40%)
- higher risk of development of esophageal cancer (adenocarcinoma)
Differential diagnoses
The differential diagnosis includes other causes of a dilated esophagus (see achalasia pattern) and includes:
- achalasia: distal segment narrowing is less than 3.5 cm
- central and peripheral neuropathy
- esophageal malignancy
- esophageal stricture
Stomach
Gastric involvement is relatively uncommon but can result in delayed gastric emptying with or without gastric dilatation. Gastric vascular antral ectasia (dilated submucosal capillaries), often known as watermelon stomach, may also occur.
Small bowel
The small bowel is affected in more than 60% of scleroderma patients, the duodenum most frequently. Patients may be asymptomatic or may present with bloating or malabsorption due to bacterial overgrowth.
Radiographic features
- luminal dilatation (can be massive)
- reduced peristalsis / delayed contrast transit
- mucosal folds appear relatively normal despite dilatation
- hidebound bowel sign (crowding of valvulae conniventes): thought to be pathognomonic of scleroderma
- accordion sign: well seen evenly spaced mucosal folds in duodenum
- sacculation (often on the mesenteric border)
Differential diagnoses
- sprue: segmentation, flocculation, hypersecretion
- small bowel obstruction
Large bowel
The large bowel is affected in ~40% of patients and may cause constipation or diarrhea. Reduced anal sphincter tone can result in fecal incontinence.
Radiographic features
- pseudosacculation
- loss of haustration
- colonic dilatation
- reduced colonic transit time
Differential diagnosis
See also
- musculoskeletal manifestations of scleroderma
- pulmonary manifestations of scleroderma
- cardiac manifestations of scleroderma
- hepatobiliary manifestations of scleroderma
- renal manifestations of scleroderma
Siehe auch:
- Sklerodermie Ösophagus
- Achalasie
- Dünndarmileus
- Pseudoachalasie
- Systemische Sklerodermie
- Wandstarre unterer Ösophagus
- Ösophagusstrikur
- Sprue
- stack of coins
- Pneumatosis intestinalis bei Sklerodermie
- oesophageal cancer (adenocarcinoma)
- pseudo-obstruction
- Morbus Hirschsprung beim Erwachsenen
und weiter:
Assoziationen und Differentialdiagnosen zu gastrointestinale Manifestationen Sklerodermie:
