Persistence of right umbilical vein: a singular case.

BACKGROUND: Persistent right umbilical vein (PRUV) is one of the most common prenatally detected venous anomaly. In the intrahepatic variant (I-PRUV) the right umbilical vein fuses with right portal vein and through the ductus venous drains into inferior vena cava, while in the uncommon extrahepatic variant (E-PRUV), the vein bypasses the liver completely. E-PRUV has a worse prognosis compared to I-PRUV, due either to severe hemodynamic effects or to the frequent association with other severe fetal malformations. CASE: Here we report a case of E-PRUV with good outcome. Prenatal fetal ultrasonography (US) performed at 33 weeks of gestation in 28-year old woman, highlights the presence of E-PRUV with right UV draining in inferior vena cava. In the male neonate born at 35 weeks of gestation by C-section, the Apgar Score was 95`- 1010 and no other associated malformations and hemodynamic decompensation were found. Postnatal abdominal US showed the presence of enlarged paraumbilical veins. CONCLUSIONS: The association of E-PRUV draining into the inferior vena cava with shunt through paraumbilical veins, could have preserved offspring by severe cardiac overload, positively affecting prognosis.

Comparative study of obstetric antiphospholipid syndrome (OAPS) and non-criteria obstetric APS (NC-OAPS): report of 1640 cases from the EUROAPS registry.

OBJECTIVES: To compare clinical features, laboratory data and fetal-maternal outcomes between 1000 women with obstetric APS (OAPS) and 640 with aPL-related obstetric complications not fulfilling Sydney criteria (non-criteria OAPS, NC-OAPS). METHODS: This was a retrospective and prospective multicentre study from the European Registry on Obstetric Antiphospholipid Syndrome. RESULTS: A total of 1650 women with 5251 episodes, 3601 of which were historical and 1650 latest episodes, were included. Altogether, 1000 cases (OAPS group) fulfilled the Sydney classification criteria and 650 (NC-OAPS group) did not. Ten NC-OAPS cases were excluded for presenting thrombosis during follow-up. All cases were classified as category I (triple positivity or double positivity for aPL) or category II (simple positivity). Overall, aPL laboratory categories showed significant differences: 29.20% in OAPS vs 17.96% in NC-OAPS (P < 0.0001) for category I, and 70.8% in OAPS vs 82% in NC-OAPS (P < 0.0001) for category II. Significant differences were observed when current obstetric complications were compared (P < 0.001). However, major differences between groups were not observed in treatment rates, livebirths and thrombotic complications. In the NC-OAPS group, 176/640 (27.5%) did not fulfil Sydney clinical criteria (subgroup A), 175/640 (27.34%) had a low titre and/or non-persistent aPL positivity but did meet the clinical criteria (subgroup B) and 289/640 (45.15%) had a high aPL titre but did not fulfil Sydney clinical criteria (subgroup C). CONCLUSION: Significant clinical and laboratory differences were found between groups. Fetal-maternal outcomes were similar in both groups when treated. These results suggest that we could improve our clinical practice with better understanding of NC-OAPS patients.

Comparative study between obstetric antiphospholipid syndrome and obstetric morbidity related with antiphospholipid antibodies / Estudio comparativo entre síndrome antifisfolipídico obstétrico y morbilidad obstétrica relacionada con anticuerpos antifosfolípido

Background and objectives: To compare clinical, laboratory, treatment and live birth rate data between women with aPL-related obstetric complications (OMAPS) not fulfilling the Sydney criteria and women fulfilling them (OAPS). Materials and methods: Retrospective and prospective multicentre study. Data comparison between groups from The European Registry on Antiphospholipid Syndrome included within the framework of the European Forum on Antiphospholipid Antibody projects. Results: 338 women were analysed: 247 fulfilled the Sydney criteria (OAPS group) and 91 did not (OMAPS group). In the OMAPS group, 24/91 (26.37%) fulfilled laboratory Sydney criteria (subgroup A) and 67/91 (74.63%) had a low titre and/or non-persistent aPL-positivity (subgroup B). Overall, aPL laboratory categories in OAPS vs. OMAPS showed significant differences: 34% vs. 11% (p<0.0001) for category I, 66% vs. 89% (p<0.0001) for category II. No differences were observed when current obstetric complications were compared (p=0.481). 86.20% of OAPS women were treated vs. 75.82% of OMAPS (p=0.0224), particularly regarding the LDA+LMWH schedule (p=0.006). No differences between groups were observed in live births, gestational, puerperal arterial and/or venous thrombosis. Conclusions: Significant differences were found among aPL categories between groups. Treatment rates were higher in OAPS. Both OAPS and OMAPS groups had similarly good foetal-maternal outcomes when treated. The proposal to modify OAPS classification criteria, mostly laboratory requirements, is reinforced by these results

Fundamento Y objetivos: Comparar características clínicas, analíticas, tratamiento y tasa de hijos vivos entre gestantes con Síndrome Antifosfolípido Obstétrico (SAFO) y gestantes con morbilidad obstétrica relacionada con el síndrome que no cumplen los criterios de clasificación actuales. Material Y métodos: Estudio observacional retrospectivo y prospectivo multicéntrico: datos de once hospitales terciarios europeos recogidos en el European Registry on Antiphospholipid Syndrome. Resultados: Se analizaron 338 mujeres: 247 cumplían criterios de Sydney para SAFO (grupo OAPS), y 91 no (grupo OMAPS). En el grupo OMAPS, 24/91(26.37%) cumplían criterios analíticos, pero no clínicos para SAFO (subgrupo A) y 67/91(74.63%) presentaban títulos medio-bajos o títulos positivos no persistentes de anticuerpos antifosfolípido, con o sin cumplir criterios clínicos (subgrupo B). Se observaron diferencias significativas entre los 2 grupos en cuanto a las categorías analíticas: 34% vs. 11% (p<0.0001) para la categoría I y 66% vs. 89% (p<0.0001) para la categoría II, OAPS vs OMAPS, respectivamente. No se observaron diferencias significativas en cuanto a las complicaciones obstétricas (p=0.481). El 86.20% del grupo OAPS recibió tratamiento vs.el 75.82% del grupo OMAPS (p=0.0224). No se observaron diferencias en la tasa de hijos vivos, ni en la tasa de trombosis arterial y/o venosa gestacional y/o puerperal. Conclusiones: Ambos grupos fueron muy homogéneos, excepto en cuanto a la distribución de las categorías analíticas y en la tasa de tratamiento. Ambos grupos mostraron buenos resultados al ser tratados. Los resultados respaldan la opinión de muchos expertos de tener que revisar los criterios de clasificación actuales del Síndrome Antifosfolípido Obstétrico