Abstract
Spontaneous rupture of extrahepatic choledochal cysts is very rare. Neonatal cases
generally present with biliary ascites, and older children with acute abdomen. Although
the cause is unclear, accumulation of protein plugs in an anomalous pancreaticobiliary
junction, irritation of the cyst wall due to reflux of pancreatic juice, and weakness
due to a developmental error such as common channel syndrome have all been suggested
to play contributing roles. The treatment of choice is complete excision of the cyst
and hepatico-jejunostomy. In newborns who are in a poor condition, the primary treatment
should be simple peritoneal drainage.
Résumé
La rupture spontanée d'un kyste extra hépatique du cholédoque est très rare. Les cas
néonataux se présentent généralement avec une ascite biliaire et les enfants plus
grands avec un abdomen aigu. Bien que la cause ne soit pas claire, l'accumulation
de bouchons protéiques dans une jonction pancréatico-biliaire anormale, l'irritation
de la paroi du kyste, le reflux de liquide pancréatique, la faiblesse due à une anomalie
du développement comme un canal commun ont été suggérés comme jouant un rôle. Le traitement
de choix est l'excision complète du kyste et une anastomose hépatico-jéjunale. Chez
les nouveau-nés qui sont en condition médiocre, le traitement primaire peut être un
simple drainage péritonéal.
Resumen
La rotura espontánea del quiste del colédoco extrahepático es muy rara. Los casos
neonatales se presentan generalmente con ascitis biliosa y en los niños mayores con
abdomen agudo. Sin embargo la causa es poco clara pudiendo ser la acumulación de tapones
de proteínas en una unión bilio-pancreática común anómala, la irritación de la pared
del quiste debida a reflujo de jugo pancreático o la debilidad debida a un defecto
de desarrollo como en el síndrome del canal común. El tratamiento de elección es la
excisión completa del quiste y la hepático-yeyunostomía. En recién nacidos que están
en mal estado el tratamiento primario debe ser simplemente el drenaje peritoneal.
Zusammenfassung
Die spontane Ruptur einer extrahepatischen Choledochuszyste ist sehr selten. Neugeborene
werden im Allgemeinen mit einem leberbedingten Aszites vorgestellt, ältere Kinder
mit einem akuten Abdomen. Obwohl die Ursache unklar ist, wird angenommen, dass eine
Obstruktion durch Proteinzylinder in einer pathologischen pancreatiko-biliären Einmündung
in das Duodenum, eine Entzündung der Zystenwand mit Reflux von Pankreassaft in die
Zysten, angeborene Wandinstabilitäten oder ein sogenanntes Common-Channel-Syndrom
als Ursache infrage kommen. Die Behandlung der Wahl ist die komplette Exstirpation
der Zyste und die Anlage einer Hepatikojejunostomie. Bei Neugeborenen in sehr schlechtem
Allgemeinzustand genügt auch als überbrückende Maßnahme die Anlage einer Peritonealdrainage.
Key words
Choledochal cyst - spontaneous perforation - bile peritonitis
Mots-clés
Kyste du cholédoque - perforation spontanée - péritonite biliaire
Palabras clave
Quiste del colédoco - perforación espontánea - peritonitis biliar
Schlüsselwörter
Choledochuszyste - spontane Perforation - Common-Channel-Syndrom
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M.D. Irfan Serdar Arda
Department of Pediatric Surgery
Baskent University Faculty of Medicine
Fevzi Çakmak caddesi 10. sokak No: 45
06490 Bahçelievler Ankara
Turkey
Email: serdara@baskent-ank.edu.tr
