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Graves&rsquo, orbitopathy (GO) is the most common extrathyroidal manifestation of Graves&rsquo, disease (GD). Our aim was to assess the efficacy and safety of Tocilizumab (TCZ) in GO refractory to conventional therapy. This was an open-label multicenter study of glucocorticoid-resistant GO treated with TCZ. The main outcomes were the best-corrected visual acuity (BVCA), Clinical Activity Score (CAS) and intraocular pressure (IOP). These outcome variables were assessed at baseline, 1st, 3rd, 6th and 12th month after TCZ therapy onset. The severity of GO was assessed according to the European Group on Graves&rsquo, Orbitopathy (EUGOGO). We studied 48 (38 women and 10 men) patients (95 eyes), mean age &plusmn, standard deviation 51 &plusmn, 11.8 years. Before TCZ and besides oral glucocorticoids, they had received IV methylprednisolone (n = 43), or selenium (n = 11). GO disease was moderate (n =29) or severe (n = 19) and dysthyroid optic neuropathy (DON) (n = 7). TCZ was used in monotherapy (n = 45) or combined (n = 3) at a dose of 8 mg/kg IV every four weeks (n = 43) or 162 mg/s.c. every week (n = 5). TCZ yielded a significant improvement in all of the main outcomes at the 1st month that was maintained at one year. Comparing the baseline with data at 1 year all of the variables improved, BCVA (0.78 &plusmn, 0.25 vs. 0.9 &plusmn, 0.16, p = 0.0001), CAS (4.64 &plusmn, 1.5 vs. 1.05 &plusmn, 1.27, p = 0.0001) and intraocular pressure (IOP) (19.05 &plusmn, 4.1 vs. 16.73 &plusmn, 3.4 mmHg, p = 0.007). After a mean follow-up of 16.1 &plusmn, 2.1 months, low disease activity (CAS &le, 3), was achieved in 88 eyes (92.6%) and TCZ was withdrawn in 29 cases due to low disease activity (n = 25) or inefficacy (n = 4). No serious adverse events were observed. In conclusion, TCZ is a useful and safe therapeutic option in refractory GO treatment.

Purpose: To examine extraocular rectus muscle tendons in patients with Graves' ophthalmopathy using optical coherence tomography (OCT). Methods: This was a cross-sectional observational study conducted with 55 healthy controls, 45 patients with clinically inactive Graves' ophthalmopathy, and 12 patients with clinically active Graves' ophthalmopathy. Scanning was performed at the 3- and 9-o'clock positions. The medial rectus tendon thickness was measured at 7.2 and 9.2 mm from the limbus and the lateral rectus tendon thickness was measured at 8.5 and 10.5 mm from the limbus. Results: The 9.2-mm medial rectus, 8.5-mm lateral rectus, and 10.5-mm lateral rectus tendons were thicker in the inactive Graves' ophthalmopathy group than the control group (240 ± 70, 231 ± 63, and 228 ± 54 µm vs 201 ± 71, 199 ± 53, and 200 ± 32 µm, respectively; P ≤ .011), whereas the 8.5-mm lateral rectus and 9.2-mm medial rectus tendons were thicker in patients with active Graves' ophthalmopathy than patients with inactive Graves' ophthalmopathy (274 ± 77 and 283 ± 68 µm vs 231 ± 63 and 240 ± 70 µm, respectively; P ≤ .048). A correlation was detected between lateral rectus and medial rectus tendon thicknesses and the Graves' ophthalmopathy clinical activity score ( R = 0.252, P = .035; and R = 0.291, P = .013, respectively). Conclusions: OCT emerged as an accurate method for measuring medial rectus and lateral rectus tendon thicknesses in patients with Graves' ophthalmopathy. The imaging tool was able to detect thicker horizontal rectus tendons in patients with inactive Graves' ophthalmopathy than in controls, and in patients with active compared to inactive disease. [ J Pediatr Ophthalmol Strabismus . 2018;55(6):356–362.]

Resumen Proposito Evaluar el grosor de los musculos extraoculares y la quemosis tras el tratamiento con tocilizumab en pacientes con oftalmopatia de Graves activa mediante tomografia de coherencia optica. Metodos Serie de 5 casos con oftalmopatia de Graves activa (escala de actividad clinica ≥4/10) tratados mediante 4 dosis de tocilizumab. Estos pacientes habian sido tratados previamente con corticoides sin mejoria. Se empleo una tomografia de coherencia optica de dominio espectral para determinar el grosor del recto lateral y del recto medial, y la quemosis antes y despues de 4 dosis de tocilizumab administradas mensualmente. Se realizo el escaneado a las 3 y 9 h (nasal y temporal). Resultados Se estudiaron 4 mujeres y un hombre con una edad mediana de 52 anos (rango: 38-73). La duracion mediana de la actividad fue de 17 meses (rango: 12-18). El grosor muscular mediano del recto medial y del recto lateral pretratamiento fue 249 μm (174-366) y 337 μm (142-443) respectivamente, siendo la quemosis mediana 409 μm (290-610). Tras el tratamiento con tocilizumab el grosor muscular disminuyo a 157 μm (88-187) y 197 μm (99-290) respectivamente (p = 0,043; Wilcoxon), y la quemosis a 59 μm (0-78). La escala de actividad clinica disminuyo de 5 (4-8) a 1 (0-3). Conclusiones Se observo una reduccion en el grosor muscular de los rectos horizontales y en la quemosis en pacientes con oftalmopatia de Graves tras el tratamiento con tocilizumab mediante tomografia de coherencia optica, por lo que esta tecnica podria ser util para la valoracion de la respuesta al tratamiento.

Background:Tocilizumab (TCZ) has shown promising results in the treatment of inflammatory ocular disease, especially in uveitis (1-3). Graves orbitopathy (GO) is the most common complication of Graves’ disease (GD). Conventional immunosuppressive drugs are not always effective or well tolerated. TCZ may be useful in the treatment of GO.Objectives:To assess the efficacy of TCZ in corticoid-resistant GO.Methods:Multicenter open study of corticoid-resistant GO. We measured clinical activity using the clinical activity score (CAS). CAS evaluates 10 different ocular items, ranging from 0 (no symptoms) to 10. We defined remission as the presence of CAS ≤ 3.Results:We studied 48 (95 eyes) patients (TABLE). Besides oral corticosteroids, they had received iv Methylprednisolone (n=43), methimazole (n=20), selenium (n=11) or radioactive iodine (n=5). According to the classification of severity (EUGOGO group), before of TCZ onset our patients had severe (n=19) or moderate (n=29) disease. TCZ was used in monotherapy (n=45) or combined with methotrexate (n=2) or azathioprine (n=1) at a dose of 8 mg/kg/iv/4 weeks (n=43) or 162 mg/sc/week (n=5). TCZ yielded rapid and maintained improvement and most patients achieved remission (FIGURE).TABLE:Number of patients/eyes affected, n/n48/96Age, mean (SD), years50.96 (11.78)Sex, men/women, n/n (%)10/38 (20.8 / 79.2)Regimen of TCZ therapyMonotherapy/combined treatment, n (%)45/3 (93.8/6.2)• AZA1 (2.1)• MTX2 (4.2)TCZ dosage, n (%)• 8 mg/kg/iv/4 weeks43 (89.6)• 162 mg/sc/week5 (10.4)Follow-up on TCZ therapy, mean (SD), months16.05±2.06• Remission, n (%)72 (75.8)• Discontinuation treatment, n (%)29 (60.4) • Remission25 (86.2) • Inefficacy4 (13.8) • Side effects0FIGURE:After a mean follow-up of 16.1±2.1 months, most patients experienced ocular improvement, with TCZ withdrawal in 29 cases due to remission (n=25) or inefficacy (n=4). Only 5 relevant adverse events were observed (neutropenia, external otitis, otitis media, costal osteitis and gingival hyperplasia). None of these events resulted in discontinuation of the treatment.Conclusion:TCZ appears to be an effective and safe treatment in corticoid-resistant GO.References:[1] Santos-Gómez M, et al. Clin Exp Rheumatol 2016; 34 Suppl 102(6):34-40[2] Calvo-Río V, et al. Clin Exp Rheumatol. 2014; 32 (4 Suppl 84): S54-7[3] Vegas-Revenga N et al. Am J Ophthalmol. 2019 Apr; 200:85-94.Disclosure of Interests:Lara Sanchez-Bilbao Grant/research support from: Pfizer, David Martinez-Lopez: None declared, Belén Atienza-Mateo: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Janssen and Celgene, Speakers bureau: Pfizer and Lilly, Vanesa Calvo-Río Grant/research support from: Abbvie, Lilly, UCB, MSD, Cellgene, Speakers bureau: Abbvie, Lilly, UCB, MSD, Cellgene, Rosalía Demetrio-Pablo: None declared, Monica Calderón-Goercke: None declared, D. Prieto-Peña: None declared, Iñigo González-Mazón: None declared, Elia Valls-Pascual Grant/research support from: Roche, Novartis, and AbbVie, Speakers bureau: AbbVie, Lilly, Pfizer, MSD, Novartis, Janssen, Bristol Myers Squibb, UCB Pharma, Beatriz Valls-Espinosa: None declared, Olga Maiz-Alonso: None declared, Ana Blanco Speakers bureau: Abbvie, Ignacio Torre-Salaberri: None declared, Verónica Rodriguez-Mendez: None declared, Ángel García-Aparicio: None declared, Raúl Veroz González: None declared, Vega Jovani: None declared, Diana Peiteado Grant/research support from: AbbVie, Lilly, MSD, and Roche, Speakers bureau: AbbVie, Roche, and MSD, Santos Castañeda: None declared, Margarita Sánchez-Orgaz: None declared, Eva Tomero Muriel: None declared, Francisco J. Toyos Sáenz de Miera: None declared, Valvanera Pinillos: None declared, Elena Aurrecoechea: None declared, Ángel Mora: None declared, Arantxa Conesa: None declared, Manuel Fernández: None declared, J. Antonio Troyano: None declared, Marcelino Revenga: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

Objectives We aim to report a case of a fetal goiter with postpartum spontaneous resolution. Fetal goiter can be secondary to maternal treatment and range from clinically asymptomatic or cause alterations in the fetus, from impaired swallowing to difficulty in vaginal delivery and even perinatal asphyxia due to the mass effect. The need for intrauterine treatment remains controversial. Case presentation We present a case of fetal goiter with postpartum resolution. A 34-year-old multigravida presented to the emergency department with hiperemesis gravidarum at 10 weeks’ gestation. During evaluation for severe vomiting, Graves disease was diagnosed and treated with propylthiouracil. A routine ultrasound scan at 28 weeks gestation revealed a fetal anterior neck mass suggesting a fetal goiter. Cordocentesis showed fetal iatrogenic hypothyroidism. Conservative treatment was decided. Pregnancy concluded uneventful and the mass resolved spontaneously in the newborn. Conclusions The fetal thyroid gland is a structure that usually goes unnoticed during the process of prenatal diagnosis. In cases of maternal Graves diseases, fetal thyroid needs monitoring during pregnancy and conservative treatment is an option. Fetal goiter should be searched for secondary to thyroid alterations of the gravida, and in selected cases it can be managed without intrauterine treatment.

Objetivo: La detección precoz del riesgo de complicaciones de la gestación como preeclampsia, parto pretérmino, y aborto, permitiría evitar morbimortalidad y secuelas. Hemos estudiado la relación entre niveles bajos de PAPP-A y BhCG con malos resultados obstétricos en una población con alta prevalencia de obesidad. Material y métodos: Estudio retrospectivo de casos y controles anidado en una cohorte de pacientes que acudieron para tamizaje de aneuploidías el I trimestre. Los casos fueron las pacientes con MoM PAPPA y/o BhCG por debajo del percentil 5 y el grupo control una muestra aleatorizada de pacientes con marcadores normales. Se ajustó por obesidad, edad, paridad, tabaquismo, y técnicas de reproducción. Resultados: La cohorte estuvo formada por 9111 pacientes. Se obtuvieron 382 casos con MoM PAPP-A inferior al percentil 5 y 325 con MoM BhCG por debajo del percentil 5, y 50 casos con ambos marcadores por debajo del percentil 5. Se tomaron 1417 controles. La prevalencia de obesidad fue del 20,7% y de sobrepeso el 28,4%. Los niveles bajos de PAPP-A se relacionaron con abortos, preeclampsia, crecimiento intrauterino retardado, pequeños para la edad gestacional, parto pretérmino y diabetes gestacional. Los niveles de BhCG por debajo del percentil 5 se relacionaron con la enfermedad hipertensiva gestacional. Los niveles de ambos marcadores por debajo del percentil 5 tuvieron relación significativa con aborto, preeclampsia precoz y parto pretérmino. Conclusión: Los niveles bajos de PAPP-A y BhCG se relacionan con malos resultados obstétricos en una población de alta prevalencia de obesidad. Background: Early identification of pregnant women at risk of developing intrauterine growth restriction, preeclampsia, preterm birth, stillbirth, among other complications would allow more intensive surveillance to reduce the risk of severe disease. We aimed to study whether low levels of maternal serum markers PAPP-A and BHCG are associated with adverse pregnancy outcomes in an obese population. Methods: Cases were obtained from a cohort of 9111 patients who attended first trimester screening. We included women with PAPP-A and/or BHCG below the 5th percentile. A randomized group of women with serum markers above the 5th percentile was used as control group. Results were adjusted for age, parity, smoking status, BMI or reproductive techniques. Results: Prevalence of obesity was 20,7%. We found 382 women with PAPP-A below the 5th percentile, 325 with BHCG below the 5th percentile, 50 with both markers low, and recruited 1417 controls. The cases with low PAPP-A were significantly more likely to experience abortion, preeclampsia, low birth weight, preterm birth, or gestational diabetes. Low BHCG was significantly associated with gestational hypertension. Low BHCG and PAPP-A in the same patient correlated with abortion, early preeclampsia and preterm birth. Conclusions: Low levels of maternal serum markers correlate with adverse pregnancy outcomes in an obese population. We recommend to develop further calculators of obstetric risk to improve positive predictive value and to establish a maternal-fetal surveillance plan.

A 66-year-old woman with breast cancer presented with a painless mass in the left orbit. MRI revealed a well-defined intraconal mass in the temporal quadrant of the orbit. Fifteen months later, a further MRI indicated the mass had grown, displacing the left optic nerve and making contact with the lateral rectus muscle, suggesting its possible intramuscular origin. Despite the clinical and radiological characteristics of the lesion and its slow growth, a PET/CT study was developed because of the history of malignant disease. No metabolic activity of the mass or malignant lesion in other locations was observed. After surgical excision, histopathological examination revealed an abundant myxoid matrix with few spindle-shaped cells and no signs of malignancy. The cells were immunopositive for CD34, positive for S-100 protein, and negative for EMA, actin, and CD57. A diagnosis was made of a nerve sheath myxoma. The orbital location of these tumors is extremely rare.

Congenital malformations occur in up to 10% of monochorionic twins and are usually discordant. Body wall defect (BWD) is a rare entity of very low prevalence, defined as a combination of various malformations, including fetal body mutilations of thoracic and/or abdominal organs that can be caused by amniotic rupture early in embryonic development. Sirenomelia is a form of caudal regression syndrome that presents with lower limb fusion, among other anomalies. In this report, we will present a second trimester monochorionic diamniotic pregnancy, with BWD-abdominoschisis in one fetus and sirenomelia in the other one. Amniotic bands could not be found. Of particular importance in respect to both malformations is the timing of the insult that could have caused both anomalies.

Increased nuchal and perinuchal translucency in euploid fetuses can be due to a wide range of congenital malformations, metabolopathies or syndromes. We present a case of transient increased perinuchal fluid accumulation in a euploid female fetus that showed no anomalies in the morphological ultrasound scan on the 20th week of gestation. The lymphatic fluid accumulation resolved spontaneously but hydrothorax developed at week 29 of pregnancy. The hydrops recurred despite the placement of a pleuro-amniotic shunt, and the gestation ended in premature delivery and early neonatal death due to massive hydrops. Necropsy revealed pulmonary lymphangiomatosis. This disease had not been previously related to increased perinuchal translucency in a euploid fetus.

Pseudotrisomy 13 syndrome is determined by the combination of three findings: holoprosencephaly, postaxial polydactyly, and a normal karyotype. We report two cases of a prenatal diagnosis of pseudotrisomy 13 syndrome and one case of a suspected hydrolethalus syndrome, another disorder with a similar phenotype and karyotype. Thorough literature search yields limited information, and the genetic cause of this syndrome remains unclear; however, it is thought to be monogenic and inherited as an autosomal recessive disorder. Given the poor prognosis and the easily recognizable malformations associated with this disease, it is important to perform an early diagnosis.

A female fetus of a dizygotic pregnancy presented with a retrovesical cystic mass at 16 weeks. Severe and recurrent fetal ascitis developed at 25 weeks. Pre-eclampsia, probably due to mirror syndrome, precipitated fetal extraction, which led to severe prematurity complications and neonatal death. Necropsy showed: cloacal anomaly, anal atresia, ambiguous genitalia and bicornuate uterus.

Organochlorines (OCs) tend to accumulate in human tissues and can be measured in amniotic fluid (AF). The detection of OCs in AF samples reflects intrauterine exposure of human beings to these persistent organic pollutants. The present study was performed to evaluate the level of contamination of AF by OCs in 100 pregnant women from Tenerife Island (Canary Islands, Spain). Gas chromatography/mass spectrometry (GC/MS) was used to identify and quantify the analytes, including 7 polychlorobiphenyl (PCB) congeners and 18 OC pesticides and metabolites. The majority of the AF samples (67%) showed some detectable OC-residue, hexachlorobenzene (HCB) being the most frequently detected compound (66% of the samples) and at the highest concentration (median 0.023 ng/ml). Lindane was also detected in 28% of the samples. Inverse associations were found between previous lactation and hexachlorocyclohexane isomers (HCH) and cyclodienes in the group of younger women (p = 0.037 and p = 0.027, respectively). Unexpectedly, serum values of HCB (r = -0.414; p = 0.04), gamma-HCH (r = -0.294; p = 0.035), and SigmaOCs (r = -0.350; p = 0.014) were negatively related to age. Even more, women with detectable levels of HCH isomers were younger (33.9 +/- 4.9 years) than women with undetectable levels of them (36.1 +/- 4.9 years; p = 0.035). We conclude that approximately one in two fetuses in the Canary Islands is exposed to OCs in utero, and that, therefore, the exposure of young women from these Islands to some HCH isomers persists nowadays. Because prenatal exposure to these chemicals may be a causative factor in adverse health trends, further studies are required to enhance preventive measures.

Introduction and hypothesis: We studied ten different three-dimensional ultrasound (3D-US) and three-dimensional Power-Doppler angiography (3D-PDA) variables to assess female pelvic floor status and endopelvic vascularization. We hypothesize that 3D-US is a reliable method to determine and evaluate changes occurring in the pelvic floor during normal pregnancy and puerperium, and can be used by any urogynecology specialist with US-imaging experience. We aimed to validate this method for clinical application. Materials and methods: We performed a prospective study of 162 nulliparous pregnant women evaluated at weeks 12, 28 and 36, and at 48 hours postpartum and three months post-delivery. At each of the five visits, we performed transperineal 3D-US of the pelvic floor and 3D-PDA to assess periurethral vascularization. Results: Quantification of the vaginal anterior vaginal wall anchors (AWA) is an anthropometric parameter sensitive to changes in pregnancy and puerperium. Significant differences in AWA were observed according to delivery mode, being lower in vaginal deliveries than cesarean sections and even lower in prolonged labor deliveries and those in which episiotomy was performed. Posterior uretrovesical angle (UVA) increased during pregnancy, partially recovering after delivery although less so in those with epidural analgesia. Urethral sphincter volume (USV) decreased significantly during labor and postpartum. Periurethral vascularization increased during pregnancy and decreased significantly after delivery. Levator hiatus area (LHA) increased during pregnancy and decreased after delivery. Patients who received epidural analgesia and oxytocin showed better LHA recovery. Prolonged labor and greater fetal head circumference increased LHA. The anorectal angle (ARA) increased during pregnancy and decreased postpartum. Conclusion: All 3D-US variables and their changes during pregnancy, childbirth and the postpartum period are measurable using the Virtual Computer-Aided Analysis (VOCAL method) with automatic threshold, which is more reliable and faster for measuring USV. AWA as an important new biometric parameter should be included in the study of the pelvic floor.

Resumen Presentamos el diagnostico prenatal de un caso de trisomia parcial del cromosoma 1 (1q25-qter) por translocacion paterna en una paciente de 31 anos, a la que se realizo amniocentesis para estudio cromosomico a las 16 semanas de gestacion por la existencia de datos dismorfologicos ecograficos. Se efectua un estudio necropsico del feto y las anomalias encontradas se comparan con las referidas en otros casos de trisomia parcial del cromosoma 1.

To describe the prevalence of epiphora and associated factors after uncomplicated phacoemulsification.Prospective and descriptive design. A group of 125 patients was asked about tearing one to two weeks after cataract surgery. A comprehensive examination was performed on the patients who answered affirmatively. All patients were re-interviewed by phone after three months and those who complained of epiphora were explored again.Two weeks after surgery, prevalence of epiphora was 10.4%, but only 3.2% appeared after surgery. In patients with preoperative epiphora, the most frequently associated factors were ocular surface alterations (89%) and unctual and canalicular stenosis (56%). In patients with "new onset" epiphora (4 patients), these factors were: blepharitis (2/4), low tear break-up time (2/4), high tear meniscus (2/4), partial nasolacrimal duct obstruction (2/4), and unctual stenosis (1/4). Three months after surgery, the prevalence of epiphora was 13%, but only 7% appeared after surgery (6 patients). Of those 6 patients, 4 were re-examined: nasolacrimal duct obstruction (2/4), ocular surface problems (3/4), and unctual stenosis (1/4).Presence of epiphora two weeks after phacoemulsification was infrequent, despite being present in a high proportion of patients before surgery. There was not a noticeable increase in epiphora at three months. The most frequently related factors were mixed disorders of the ocular surface and lacrimal system, without an important incidence of unctual and canalicular stenosis. We consider that our perception about complaints from patients after phacoemulsification may be biased due to those who had previously suffered from epiphora.

Objetivo: Estudiar los resultados perinatales de fetos diagnosticados con flujo umbilical ausente en diástole en el Hospital Universitario de Canarias, España. Métodos: Estudio retrospectivo de gestantes con Doppler fetal umbilical con flujo diastólico ausente entre 2004 y 2011, excluyendo embarazos gemelares. Las variables estudiadas fueron: edad y enfermedades maternas, edad gestacional al diagnóstico y parto, vía de parto, Apgar y estado del recién nacido. Resultados: Se recogieron 57 casos. Hubo un 43% de gestantes hipertensas y 19% de diabéticas. La edad gestacional media al diagnóstico fue de 30+4 semanas. En el 89% de los casos se observó redistribución del flujo y en el 21% el Doppler umbilical fue reverso. El 28% se acompañó de oligoamnios y en el 26% el peso fetal estimado inferior al percentil 3. Hubo 3 muertes fetales. En el 80% de los casos la vía de parto fue por cesárea. La supervivencia fue del 83% con una tasa de mortalidad perinatal del 17%. En el seguimiento entre 1 y 6 años encontramos un 35% de niños sin secuelas. Conclusiones: En nuestra serie, el Doppler con flujo umbilical ausente en diástole se relaciona con alto riesgo de morbimortalidad perinatal. El 65% de los niños tendrán secuelas de algún tipo en el seguimiento entre 1 a 6 años. El momento óptimo para la extracción fetal debe ser individualizado y resuelto junto con el consejo pediátrico y participación de los padres. Objective: To determine the perinatal outcome in fetal growth restriction were umbilical artery Doppler end diastolic flow was absent. Methods: A retrospective study performed at the Canary Islands University Hospital. All consecutive cases between 2004 and 2011 were included. We excluded twin pregnancies. Data was abstracted for maternal age, gestational age at diagnosis and delivery, mode of delivery, Apgar and perinatal adverse outcomes. Results: Fifty seven patients were included. Forty three percent had a pregnancy complicated by hypertension and 19% diabetes. Mean gestational age at diagnosis was 30+4 weeks. We found 89% and 21% absent umbilical artery end diastolic flow and reverse flow respectively. Oligohydram-nios was seen in 28% of the cases. Fetal weight was below the third percentile in 26% of the cases. Fetal demise occurred in 3 cases. Mode of delivery was cesarean section in 80% of the cases. We found a rate of 17% perinatal mortality. At 1 to 6 years follow up 35% of the children had no sequelae. Conclusion: Absent end diastolic umbilical flow is associated with a high risk of perinatal mortality. Up to 65% of the children will show some type of sequelae. The optimal gestational date for fetal extraction should be individualized after multidisciplinary counseling.

The aims of this study were to present and discuss ultrasound findings of prenatal fetal cholelithiasis in two cases with different etiology and evolution. Case 1: a pregnant woman from sub-Saharan Africa, suffering from Lyme disease, was treated with ceftriaxone sodium. Six weeks later, biliary sludge associated with polyhydramnios was detected in the fetus and the fetal growth percentile was 14. Emergency caesarean was performed at 36 weeks of gestation due to fetal distress. Biliary sludge persists in the two-and-a-half-year-old child. Case 2: the fetus of a Caucasian woman with normal pregnancy showed multiple cholelithiasis associated with polyhydramnios at 31 weeks of gestation. At 39 weeks and 4 days, cesarean section was performed due to lack of dilation. The biliary disease resolved spontaneously at seven months of age, with no associated abnormalities. In conclusion, prenatal diagnosis of cholelithiasis is straightforward, but prognosis cannot be defined yet. Serious complications do not arise in 70% of cases, but severe diseases may ensue in 20%. Persistence of cholelithiasis after one year of age results in cholelithiasis in childhood and beyond. Biliary sludge is associated with worse prognosis than cholelithiasis when it appears before 28 weeks of gestation.

Objectives: We used three-dimensional ultrasound with trans-abdominal Power-Doppler (3D-US PD) to determine placental vascular evolution in normal pregnancies (NP) and in insulin-dependent Gestational Diabetes Pregnancies (GDP). Study design: We obtained 473 measurements from 43 NP at 20 weeks -40 weeks gestation and 122 measurements from 70 insulin-dependent GDP at 22 weeks -40 weeks. Standardization was achieved recording three successive placental vascular tree volumes, measuring the spherical volume between the chorionic and basal plates always under or near funicular insertion. Parameters analysed were: mean intensity of blood flow (Flow Index, FI); percentage of volume occupied by vessels (Vascularisation Index, VI); and intensity of blood flow in the volume occupied by vessels (Vascularisation-flow index, VFI). Results: In NP, FI increased throughout early pregnancy and decreased before delivery and correlated with fetal parameters. In contrast, in GDP, FI was high from the onset, better correlated with placental parameters, and had a diagnostic cutoff value of 45, which was only found in 7% of NP at the end of the pregnancy. VI was variable and had a low diagnostic value, being related to placental parameters; VI values were significantly lower in NP ( 17.4 ± 7.4%) than in GDP (21 ± 12%) with a diagnostic cutoff point at 31%. In NP, FI-peak was at 32 weeks, two weeks after the VI-peak, while VFI showed no significant differences. Conclusions: The results showed that placental blood flow (FI) was related to fetal circulation, while the percentage of vessels per volume (VI) was related to maternal circulation. 3D-US PD indicated a diagnosis of GDP for FI > 45 and VI > 30%. We also discuss the values of change in VI that predict changes in fetal FI.

Antecedentes: La ventriculomegalia fetal es un signo ecográfico muy sensible para las alteraciones del sistema nervioso central fetal. La morbilidad asociada a la misma depende principalmente de la etiología y de la presencia de otras malformaciones asociadas. Si es aislada y no progresiva se puede preveer un resultado posnatal favorable hasta en el 70% de los casos aunque hay una gran disparidad de cifras entre distintos centros y autores. Objetivo: Establecer la relación entre ventriculomegalia y pronóstico perinatal. Método: Se han revisado 60 casos diagnosticados mediante ecografía prenatal en el Hospital Universitario de Canarias siguiendo los criterios ecográficos de ventriculomegalia (astas posteriores mayores de 10 mm). Se realizaron cariotipos y serología materna y en líquido amniótico para completar el estudio. Resultados: Hubo un total de 23 interrupciones voluntarias de la gestación y 28 recién nacidos sin secuelas (47%). Un 70% de las ventriculomegalias aisladas correspondieron a recién nacidos sin secuelas. El 10% de las ventriculomegalias aparecieron en fetos con aneuploidías. Conclusión: El pronóstico fue favorable en los casos de ventriculomegalia no progresiva y aislada. La medida de la ventriculomegalia no predice el resultado. La variabilidad de resultados en la literatura obliga a cada centro a disponer de sus protocolos y datos para aconsejar a la pareja que ha de tomar una decisión sobre el futuro de su embarazo. Background: Fetal ventriculomegaly is the most sensitive sonographic sign for central nervous system anomalies. Neonatal morbidity and mortality depends on the etiology of the ventriculomegaly and the presence of other malformations. Isolated non-progressive ventriculomegaly is associated with 70% favorable results, with a great disparity among studies. Aims: To establish the relationships between ventriculomegaly and perinatal outcome. Methods: The authors reviewed 60 cases of prenatally diagnosed fetal ventriculomegaly in the Canary Islands University Hospital. An ultrasonographic measurement of the lateral ventricles of 10 mm or more was considered as ventriculomegaly. Fetal and maternal serology was performed as well as fetal karyotyping. Results: 23 patients decided to terminate the pregnancy due to unfavorable prognosis. In 28 cases outcome was favorable. 70% of isolated ventriculomegaly corresponded to healthy newborns. 10% of the fetuses had aneuploidies. Conclusion: Prognosis is favorable if ventriculomegaly is isolated and non-progressive. Measurement of the lateral ventricle does not predict outcome. Disparity of results in literature makes it necessary for each Hospital to rely on their own data and guidelines to assess parents on the fate of the pregnancy.

Adnexal masses are common among peri- and post-menopausal women. Although ovarian cancer is a significant cause of mortality in menopausal women, large population-based studies demonstrate that the majority of adnexal masses are benign. Despite this, the appearance of an adnexal mass is a concern for the patient and an insight exercise for physicians. In most cases, an adnexal enlargement is an incidental finding, generally corresponding to a benign cyst and easily diagnosed by conventional ultrasound. Exceptionally an ovarian tumour may be malignant and should be treated as early as possible. When conventional ultrasound renders complex morphology other diagnostic tools must be used such as: colour Doppler and functional tumour vessel properties, serum CA 125 levels, nuclear magnetic resonance imaging and in some cases laparoscopy. Several new tumour markers are being studied for clinical application, although there are presently no clear recommendations. Adnexal masses with benign morphological and functional properties must be periodically monitored as an alternative to surgery since malignant transformation is exceptional.

Diagnostico de un caso de enfermedad familiar a partir de un hidrops fetal. Gestante con 2 abortos previos ocurridos al inicio del segundo trimestre, ambos fetos masculinos. Se detecta en la semana 12 un higroma quistico en el contexto de un hidrops fetal. Estudio serologico (TORCH y parvovirus B12), con resultado negativo y biopsia corial con cariotipo 46 XY. La anamnesis y exploracion detallada hacen sospechar una enfermedad ligada al cromosoma X, dado que la paciente y una hermana estan afectas de una genodermatosis desde la infancia: Incontinentia Pigmenti.

A case of in utero spondylothoracic dysostoses is diagnosed during a routine third trimester ultrasound scan in a low-risk patient. Ultrasound showed spine deformation, hemivertebrae and crab-like ribs. The newborn had no other associated anomalies except inguinal hernia, and karyotype was normal. Bidimensional and 3-4D ultrasound imaging as well as radiograph of the fetus and photos of the newborn are presented and discussed. Sporadic cases are difficult to diagnose and classify, as they are probably due to genetic heterogenicity. Sporadic cases of hemivertebrae are rare and not always lethal. Fetal ultrasound scan findings can help predict prognosis, enabling the parents’ informed decision making.

Aims We present a new method of repairing gastroschisis, where the disproportion between the size of the eviscerated intestine and the hypoplastic abdominal cavity could dangerously elevate abdominal pressure if primary closure of the abdominal wall is attempted. Methods We used umbilical cord graft strengthened by a polypropylene mesh to achieve definitive closure of the abdominal wall in a single operation. Results This technique provides a mesothelial surface in contact with the intestines, strengthened by a fibrous reaction that prevents future herniation. Five-year follow-up in four patients showed no umbilical hernias and overall excellent results. Conclusions The technique described is useful and easily applicable in children with abdominal wall defects when primary anatomic closure is not feasible. It also prevents future herniation through the anatomical defect of the abdominal wall.

Objectives 3D-Ultrasound reconstruction, routinely available since 1994, has brought new technical capabilities such as virtual sonography that can be tele-consulted. Our experience is summarized in the present paper. Methods During one year, 73 3D-US volumetric images coming from 34 patients were acquired and 68 were consulted at distance. Acquisitions were carried out through an existing 2D device adding a magnetic tracking system on the US-probe. Probe positioning and video output was introduced into a PC running software that allows the generation of 2D-orthogonal and 3D volume images, as well as tele-consultation. Several image analysis techniques for 3D-reconstruction were evaluated. Results Final volumes were small (1.5 Mb) and required about 4+/-2 min to be transmitted over one ISDN channel (64 Kbs). Good correlation (k = 0.7) was found between local and distant diagnoses. In 30%, images were considered of low quality and in 29% of good quality; diagnosis could be done with confidence in all except 7 cases. Virtual sonography, by means of oblique cuts in all space directions, improved distant diagnostic confidence. Limitations were linked to incomplete sampling due to the short acquisition time periods (26 s) and difficulties on hand-free probe movement. 3D reconstructions were time consuming (20 min to 4 h) and of limited indication. Conclusions 3D reconstruction could reduce multiple explorations due to image constrains such as suboptimal fetal positioning, among others. Virtual sonography was important to reach confidence on distant diagnosis; it was also considered a tool for off-line local review of non-trained sonographer acquisitions.

BACKGROUND: Three-dimensional ultrasound images allow virtual sonography even at a distance. However, the size of final 3-D files limits their transmission through slow networks such as the Internet. OBJECTIVE: To analyze compression techniques that transform ultrasound images into small 3-D volumes that can be transmitted through the Internet without loss of relevant medical information. METHODS: Samples were selected from ultrasound examinations performed during, 1999-2000, in the Obstetrics and Gynecology Department at the University Hospital in La Laguna, Canary Islands, Spain. The conventional ultrasound video output was recorded at 25 fps (frames per second) on a PC, producing 100- to 120-MB files (for from 500 to 550 frames). Processing to obtain 3-D images progressively reduced file size. RESULTS: The original frames passed through different compression stages: selecting the region of interest, rendering techniques, and compression for storage. Final 3-D volumes reached 1:25 compression rates (1.5- to 2-MB files). Those volumes need 7 to 8 minutes to be transmitted through the Internet at a mean data throughput of 6.6 Kbytes per second. At the receiving site, virtual sonography is possible using orthogonal projections or oblique cuts. CONCLUSIONS: Modern volume-rendering techniques allowed distant virtual sonography through the Internet. This is the result of their efficient data compression that maintains its attractiveness as a main criterion for distant diagnosis. [J Med Internet Res 2001;3(2):e21]

ss Open Acce Poster presentation without discussion Dopamine β-hydroxylase and p73 expression during aging and ventricular dilation in the cerebrospinal fluid and circumventricular organs of SHR rats Emilia Ma Carmona-Calero*1,2, Ibrahim Gonzalez-Marrero1,2, Paloma Fernandez-Rodriguez2, Herminia Perez-Gonzalez1, Juan M Gonzalez-Toledo1, Leandro Castaneyra-Ruiz2, Agustin CastaneyraRuiz1, Juan M Troyano1, Dionisio M Garcia1 and Agustin CastaneyraPerdomo1,2

Background Hydrocephalus is a disorder characterized by brain ventricular dilation and excess of cerebrospinal fluid (CSF), in many cases its etiopathic origin is acquired as: post-hemorrhage, meningitis and tumours. But, there is no attributable cause in 45% of hydrocephalus cases and foetal hydrocephalus due to aqueductal stenosis is one of the two main groups of non acquired or congenital hydrocephalus, however whether narrowing of the aqueduct of Sylvius is cause or consequence of hydrocephalus is discussed by different authors. Anyhow, most investigators describe an alteration in the SCO and certain circumventricular structures in the hydrocephalus, that can even occur before hydrocephalus is present and of course before the aqueduct stenosis. The objective of the present work is to investigate probable changes in the protean composition of the CSF in perinatal hydrocephalus and to analyze, in those liquids, the presence of soluble proteins that are immunoreactive or antibodies against Reissner fibre proteins and human foetal SCO extracts