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Z Gastroenterol 2012; 50(10): 1100-1103
DOI: 10.1055/s-0032-1312921
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© Georg Thieme Verlag KG Stuttgart · New York

Raising Awareness about Chronic Intestinal Pseudo-Obstruction (CIPO): A Case Report Showing CIPO as Initial Manifestation of Atypical Seronegative Systemic Sclerosis

Mehr Beachtung für die chronische intestinale Pseudoobstruktion (CIPO): Ein Fallbericht mit CIPO als initiale Manifestation einer atypischen seronegativen Sklerodermie
J. Jäkel
1   Institute of Pathology, University Hospital of RWTH Aachen, Aachen, Germany
,
J. W. Heise
2   Department of Surgery, Bethlehem Hospital, academic teaching hospital of RWTH University Aachen, Stolberg (Rhineland), Germany
,
N. Gassler
1   Institute of Pathology, University Hospital of RWTH Aachen, Aachen, Germany
,
C. G. Dietrich
3   Department of Internal Medicine, Bethlehem Hospital, academic teaching hospital of RWTH University Aachen, Stolberg (Rhineland), Germany
› Author Affiliations
Further Information

Publication History

16 January 2012

21 May 2012

Publication Date:
11 October 2012 (online)

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Abstract

Only few case studies address pseudo-obstruction, a disorder – which often frustrates clinicians and patients due to an unclear diagnosis and limited therapeutic options. Thus, the aim of this paper is to investigate a relevant case concerning a patient presenting with symptoms of acquired chronic intestinal pseudo-obstruction (CIPO). After one year of extensive diagnostic tests and unsuccessful treatment with prokinetics, the patient underwent a subtotal ileocolectomy. The histology of the intestinal specimen revealed continuous atrophy and fibrosis mainly within the circular, inner muscle layer of muscularis propria of the ileum and colon. Even though serum markers were lacking, a subsequent skin biopsy showed signs of scleroderma supporting an initial diagnosis of intestinal involvement in systemic sclerosis. Despite treatment with steroids and methotrexate, the increasingly emaciated patient died. In conclusion, there is a bias against the publishing of pseudo-obstruction studies, in particular, due to the obscure underlying causes. To raise awareness of this problem, we call for clinicians to systematically generate comprehensive data about patients presenting these symptoms.

Zusammenfassung

Die chronische intestinale Pseudoobstruktion stellt sich oft als differenzialdiagnostisch schwierig einzuordnende und therapeutisch schlecht zu behandelnde Erkrankung dar. In diesem Fallbericht wird ein Patient mit erworbener schwerer intestinaler Pseudoobstruktion vorgestellt, bei welchem trotz umfangreicher Untersuchungen und probatorischer Prokinetikatherapie keine Besserung erreicht werden konnte, sodass letztlich eine subtotale Ileokolektomie durchgeführt wurde. Histologisch zeigte sich eine ausgedehnte Atrophie und Fibrose der Muscularis propria. Obwohl Serummarker für die Erkrankung wiederholt unauffällig waren, unterstützte eine Hautbiopsie die Diagnose einer Sklerodermie mit vorrangig intestinaler Beteiligung. Weder Steroide noch Methotrexat führten zu einer Besserung der ausgeprägten Symptomatik und trotz hochkalorischer parenteraler Ernährung kam es zu einer schweren Kachexie und schließlich zum Tod des Patienten. Aufgrund des häufig fehlenden Nachweises einer eindeutigen Krankheitsursache und schwieriger differenzialdiagnostischer Erwägungen ist von einem Mangel an Fallberichten auszugehen. Zum besseren Verständnis der chronischen Pseudoobstruktion und der zugrunde liegenden Ursachen ist eine umfängliche Erfassung dieser Fälle wünschenswert.

Key words

malassimilation - pseudo-obstruction - sclerodermia - systemic sclerosis - CIPO - chronic intestinal pseudo-obstruction

Schlüsselwörter

Malassimilation - Pseudoobstruktion - Sklerodermie - systemische Sklerose - CIPO - chronische intestinale Pseudoobstruktion
 

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