Delayed interval delivery of the second twin: obstetric management, neonatal outcomes, and 2-year follow-up.

OBJECTIVES: To evaluate the obstetric management and neonatal outcomes in twin pregnancies with delayed delivery of the second twin, including follow-up. METHODS: This study is a review of four cases of delayed delivery of the second twin in our hospital from 2009 to 2012. The obstetric management of the cases from the expulsion of the first twin to the delivery of the second twin is analyzed. The neonatal outcomes including follow-up for 2 years were reviewed. RESULTS: The first twins were delivered between 15 and 25 weeks (average 21 weeks) and the second twins were delivered between 25 and 31 weeks (average 27 weeks). One first twin (25 %) survived, while three (75 %) second twins survived. Two out of the three second twins delivered after 28 weeks were in satisfactory condition. CONCLUSIONS: The delayed delivery of the second twins which occurred in the third trimester is associated with favorable outcome, however, the risks should not be ignored.

Síndrome de Berdon: diagnóstico intrauterino y evolución posnatal / Berdon syndrome: intrauterine diagnosis and postnatal outcome

El síndrome de Berdon es un síndrome muy poco frecuente de carácter congénito, caracterizado por megavejiga, microcolon e hipoperistalsis intestinal, cuyo pronóstico es infausto en la mayoría de las ocasiones y cuyo manejo supone un reto que requiere un abordaje multidisciplinar. Revisamos una serie de casos diagnosticados en nuestro centro entre 1997 y 2010, comentando el diagnóstico, manejo y evolución de estos pacientes(AU)

Berdon syndrome is a rare congenital syndrome characterized by megacystis, microcolon and intestinal hypoperistalsis. It has an ominous prognosis in most cases, and its management requires a multidisciplinary approach. We review a series of cases diagnosed in our center between 1997 and 2010, and discuss the diagnosis, management and outcomes of these patients(AU)

Evolución obstétrica de paciente afectada de síndrome de Ehlers-Danlos / Pregnancy outcome in Ehlers-Danlos Syndrome

El síndrome de Ehlers-Danlos (SED) es un grupo de enfermedades del tejido conectivo caracterizado principalmente por fragilidad tisular e hiperlaxitud articular. Es poco frecuente su asociación con el embarazo y algunos tipos cursan con complicaciones severas maternas. Presentamos nuestra experiencia con una paciente de 35 años afectada de SED tipo clásico, que presentó un embarazo sin complicaciones, que alcanzó las 38 semanas y, un aborto espontáneo en el primer trimestre de una gestación gemelar(AU)

Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders characterised by tissue fragility and joint hypermobility. Although it is rarely associated with pregnancy, some types may have severe maternal complications. We report our experience of a 35-year-old patient suffering from a classic EDS, who had a successful pregnancy at 38 weeks, and first trimester spontaneous miscarriage of a twin pregnancy(AU)

Mesenchymal hamartoma: prenatal and postnatal diagnosis by imaging.

We present a case of a twin pregnancy in which one fetus developed a rapidly growing unilateral intrathoracic tumor. While a cystic adenomatoid malformation was suspected in the ultrasound scan, the magnetic resonance scan suggested a pulmonary blastoma or a bronchioalveolar carcinoma. Postnatal chest radiography and contrast-enhanced computed tomography of the affected newborn were performed, and it was ruled out the possibility of malignant origin. Finally, the anatomopathologic exam revealed the presence of a mesenchymal hamartoma in the chest wall. Nevertheless, parents refused any treatment for the newborn.

Fetal arthrogryposis secondary to a giant maternal uterine leiomyoma.

Arthrogryposis multiplex congenital is a rare condition defined as contractures in multiple joints at birth due to disorders starting in fetal life. Its etiology is associated with many different conditions and in many instances remains unknown. The final common pathway to all of them is decreased fetal movement (fetal akinesia) due to an abnormal intrauterine environment. Causes of decreased fetal movements may be neuropathic abnormalities, abnormalities of connective tissue or muscle, intrauterine vascular compromise, maternal diseases, and space limitations within the uterus. When the cause of arthrogryposis is space limitations in uterus, the most common etiology is oligohydramnios. The same can result from intrauterine tumours as fibroids, although to our knowledge there are only two papers reporting cases of fetal deformities related to uterine leiomyomas. We describe a well-documented exceptional case of arthrogryposis associated with the presence of a large uterine fibroid. It could illustrate the importance of a careful and appropriate assessment of uterine fibroids before and in the course of a pregnancy considering that they can cause both serious maternal and fetal complications.

Effect of antiangiogenic treatment on peritoneal endometriosis-associated nerve fibers.

OBJECTIVE: To investigate the effect of antiangiogenic treatment on experimental endometriotic lesion nerve fibers. DESIGN: Heterologous mouse model of endometriosis. SETTING: University Institute IVI, University Hospital La Fe. ANIMAL(S): Ovariectomized nude mice (n = 16) receiving human endometrial fragments from oocyte donors (n = 4). INTERVENTION(S): Endometrium fragments stuck in the peritoneum of 5-week-old female nude mice treated with vehicle (n = 8) and antiangiogenic agent cabergoline (n = 8; Cb(2,) 0.05 mg/kg/day) for 14 days. MAIN OUTCOME MEASURE(S): Immunofluorescence analysis of von-Willebrand factor (vWF) and vascular smooth muscle cells (αSMA) for evaluating the number of immature blood vessels (IBV) and microvascular density (MVD); immunochemical analysis of protein-gene product 9.5 (PGP 9.5) to assess nerve fibers density (NFD), and blue toluidine staining to confirm presence of mast cells and macrophages in endometriotic lesions. RESULT(S): All the results were quantified by morphometric techniques. The IBV, NFD, and number of macrophages and mast cells were statistically significantly decreased in the Cb2-treated group when compared with controls. CONCLUSION(S): Antiangiogenic treatment statistically significantly diminishes new blood vessel formation after macrophage, mast cell, and nerve fiber reduction, providing a rationale to test antiangiogenic agents as a novel therapeutic approach to severe pelvic pain associated with human peritoneal endometriosis.

Pregnancy outcomes after kidney transplantation-immunosuppressive therapy comparison.

OBJECTIVE: To assess maternal, neonatal and graft outcomes after pregnancy in patients with kidney transplantation, and to compare the immunosuppressive therapies used. METHODS: Review of 29 pregnancies in 23 patients with kidney transplantation, managed at La Fe University Hospital, Valencia. Immunosuppressive therapies with Cyclosporine-A, Tacrolimus, Mycophenolate mofetil and Azathioprine were compared. RESULTS: No statistical differences were found in perinatal or maternal complications, with respect to the immunosuppressive therapy used. There were no differences between therapy and graft survival. Maternal complications occurred in 25 out of 28 deliveries. The most common were anemia (75%) and hypertension (53.6%). Of the 29 pregnancies, 26 were live deliveries, two were stillbirths and one was a miscarriage. The median birth weight of newborns was 2650 g (900-4350 g). From the 28 deliveries, maternal complications were reported in 25 patients. Perinatal complications were recorded in 55.6% of the patients, with prematurity being the most common (44.4%) type. One malformation was reported, this was a cleft palate in a 25 year old patient who was treated with mycophenolate mofetil. CONCLUSION: Pregnancies in patients with kidney transplantation should be considered high-risk pregnancies because of the higher rate of maternal and perinatal complications. Immunosuppressive therapies have not shown differences in maternal or perinatal outcomes.

Manejo quirúrgico de la endometriosis vésico-ureteral y sus complicaciones / Surgical management of deep endometriosis

La endometriosis ureteral es una infrecuente localización de endometriosis profunda, que puede condicionar una grave disminución de la función renal de forma silenciosa. Se presenta el caso de una paciente con fibrosis peritoneal secundaria a endometriosis profunda, cuya inespecífica sintomatologia conllevó un retraso diagnóstico, permitiendo el desarrollo de hidronefrosis. Es necesario descartar la presencia de endometriosis profunda en mujeres en edad fértil con hidronefrosis de etiología desconocida.

Deep endometriosis rarely entails ureteral involvement. It may be responsible of asymptomatic loss of renal function. A 35-year-old woman, gravida 1, para 1, was managed for peritoneal fibrosis due to deep infiltrating endometriosis. The nonspecific symptoms let a delayed diagnosis and a subsequent hydronephrosis. It must be excluded the existence of deep endometriosis in women of childbearing age with hydronephrosis of unknown etiology.

Mastectomía por fibroadenoma gigante juvenil recidivante / Mastectomy due to recurrent juvenile giant fibroadenoma

Los fibroadenomas son las lesiones mamarias más frecuentes durante la adolescencia. Se habla de «fibroadenoma gigante» cuando alcanza un tamaño mayor de 5cm o un peso superior a 500g. Su tratamiento consiste en la tumorectomía, siendo necesaria excepcionalmente la mastectomía. En ocasiones resulta complicada su diferenciación histológica con el tumor filodes, lesión mamaria benigna muy recidivante que raramente evoluciona como maligna. Presentamos el caso de una paciente diagnosticada de fibroadenoma gigante a los 17 años y sometida a cinco intervenciones por sucesivas recidivas tumorales, con resultado final de la realización de una mastectomía. En dos ocasiones la lesión fue catalogada como tumor filodes borderline. Consideramos de interés su presentación por su excepcional evolución y como ejemplo de la dificultad de las indicaciones quirúrgicas en determinadas lesiones mamarias benignas de difícil diagnóstico anatomopatológico (AU)

Fibroadenomas are the most common breast lesions in adolescents. The term “giant fibroadenoma” is used when the tumor reaches a size of more than 5cm or weighs more than 500g. Treatment consists of tumorectomy or, in a very few cases, mastectomy. The histological differential diagnosis between fibroadenoma and phyllodes tumor, a highly recurrent and usually benign lesion, is sometimes difficult. We present the case of a 17-year-old patient diagnosed with a giant fibroadenoma, who underwent five interventions due to multiple tumoral recurrences, with the final result of a mastectomy. The lesion was twice diagnosed as a borderline phyllodes tumor. The interest of this case lies in its exceptional clinical course. This case also illustrates the difficulty of surgical indication in some benign breast lesions with difficult histological diagnosis (AU)