A205 CONGENITAL ABSENCE OF THE PORTAL VEIN: DOES IT REALLY EXIST?
Notice bibliographique
Résumé
Congenital portosystemic shunts are rare vascular malformation that can lead to pulmonary hypertension, encephalopathy and liver tumors. They were classified as type I(end-to-side portocaval fistula with no visible portal flow in the liver) and type II(side-to-side portocaval fistula)shunts.Type I shunts are often referred to a congenital absence of the portal vein(CAPV), and are still considered an indication for liver transplantation, whereas surgical or percutaneous closure is usually feasible for type II shunts. Through a case report and a review of all published patients, we show that what is initially diagnosed as CAPV may conceal a hypoplasic portal vein that can successfully be closed by surgical ligation. A 2-year-old girl was referred for liver transplantation in the context of recent diagnosis of CAPV. At presentation she was asymptomatic and showed a hypertrophic left liver lobe, without any complications. Her blood tests were normal excepted for moderately elevated serum ammonia levels. MRI confirmed the diagnosis of a type Ib portosystemic shunt(with superior mesenteric and splenic veins joining to form a short portal trunk ending into the inferior vena cava). Percutaneous venogram confirmed the absence of the portal vein. Nevertheless, a second direct catheterization of the shunt with temporary shunt occlusion allowed us to visualize an hypoplasic portal vein arising from the posterior face of the shunt. Pressure was measured at 12mmHg in standard conditions and 36mmHg upon temporary occlusion. We decided for a two-step occlusion. A partial banding was carried out without significant complications(normal liver tests, minimal transient ascites). The shunt was permeable, with a measurable portal flow, at follow-up Doppler ultrasound. 2 months later, moderate elevation of liver enzymes(3xULN) and mild ascites were detected, but resolved spontaneously within a few weeks. The shunt was not detected anymore, and the Doppler study showed a normal portal flow. A percutaneous venogram confirmed the total closure of the shunt and the permeability of the portal vein. The child is asymptomatic at 6-month follow-up. 202 cases of extrahepatic shunts were reported since 1979, of which 134 were described as CAPV. 38 patients(19%) had percutaneous or surgical shunt closure(12 &26, respectively), whereas 25 patients(12%) received a liver transplantation. Among all transplanted patients, only 4% had a preoperative percutaneous venogram with temporary shunt occlusion. Overall, the case reported here exemplifies what emerges from published literature: a precise evaluation of the shunt with percutanous venogram and temporary occlusion is warranted in all patients with suspected CAPV. It allows detecting otherwise invisible hypoplasic portal veins that allow tore establish a physiological hepatic circulation and avoid liver transplantation. None
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Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,008 |
| Méta-épidémiologie (sens strict) | 0,001 | 0,001 |
| Méta-épidémiologie (sens large) | 0,001 | 0,001 |
| Bibliométrie | 0,002 | 0,001 |
| Études des sciences et des technologies | 0,001 | 0,003 |
| Communication savante | 0,002 | 0,005 |
| Science ouverte | 0,001 | 0,001 |
| Intégrité de la recherche | 0,006 | 0,003 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,003 | 0,001 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».