A Massive Intestinal Vaso-occlusive Crisis or “Girdle Syndrome” in a 6-year-old Boy Observed as a First Manifestation of Sickle Cell Disease

Abstract

Sickle cell disease is a chronic hematologic disease with variable but often severe systemic symptoms. In this report, we describe a 6-year-old boy presenting with acute bowel pseudo-obstruction. During this episode, previously undiagnosed sickle cell disease was discovered upon peripheral blood smear analysis. The condition was therefore interpreted as a massive intestinal vaso-occlusive crisis or “girdle syndrome”. Conservative treatment with hydration therapy, analgesia and a manual partial exchange transfusion was initiated. The patient fully recovered within 5 days.

Conclusion: Girdle syndrome is a rare but severe adverse event associated with sickle cell disease that must be considered as differential diagnosis in patients with sickle cell disease.

Zusammenfassung

Die Sichelzellkrankheit ist eine chronische Erkrankung mit häufig sehr schweren Symptomen. Wir berichten einen 6 Jahre alten Jungen, der sich mit einer akuten Ileussymptomatik vorstellte. Im Rahmen der initialen Diagnostik konnte zudem eine Sichelzellkrankheit im peripheren Blutausstrich erstdiagnostiziert werden. Daher bestand der Verdacht, dass der Junge an einem intestinalen vaso-okklusivem Syndrom leidet, auch bekannt als „Girdle Syndrom“. Wir begannen eine konservative Therapie mit Hydrierung, Analgesie und einer manuellen partiellen Austauschtransfusion. Nach 5 Tagen erholte sich der Patient vollständig.

Schlussfolgerung: Girdle-Syndrom ist eine seltene aber schwerwiegende Symptomatik der Sichelzellkrankheit und muss als Differenzialdiagnose bei Patienten mit Sichelzellkrankheit bedacht werden.

Key words

sickle cell disease - girdle syndrome - bowel pseudo-obstruction - adverse event

Schlüsselwörter

Sichelzellkrankheit - Girdle-Syndrom - paralytischer Ileus - Symptomatik

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