Double Myelomeningocele Repair by Fetal Surgery with a Single Micro-Hysterotomy.

INTRODUCTION: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases. CASE PRESENTATION: We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting. CONCLUSION: This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.

Estado de salud del primer molar permanente en niños de 6-11 años. Sancti Spíritus. 2011 / Health situation of the first permanent molar in children from 6-11 years old. Sancti Spíritus. 2011

Fundamento: los niños en edad escolar son la población más susceptible y de alto riesgo a padecer caries dental, causa principal de pérdida del primer molar permanente. Objetivo: identificar la situación de salud del primer molar permanente en la población de 6-11 años de la escuela primaria “Remigio Díaz Quintanilla” del municipio de Sancti Spíritus. Metodología: se realizó un estudio observacional descriptivo transversal en el período comprendido septiembre a diciembre de 2011. La población constituida por 622 y la muestra 526 niños. Se utilizó la entrevista estructurada y la observación, las variables: sexo, higiene bucal y nivel de información. Resultados: la caries del primer molar permanente fue frecuente en el grupo de 10-11 años en casi la mitad de los pacientes; en ambos sexos la higiene bucal fue regular, con predominio del sexo masculino y la tercera parte de los infantes tuvo un nivel de información insuficiente sobre salud bucal. Conclusiones: a pesar de los esfuerzos realizados para preservar la salud del primer molar permanente persiste la afectación por caries en este molar así como una higiene bucal regular por lo que se hace necesario diseñar un sistema de acciones educativas para contribuir a la salud del primer molar permanente.

Background: school children is the most susceptible population and of high risk to suffer from dental caries as a principal cause of first permanent molar loss. Objective: to identify the health situation of the first permanent molar in the population from 6-11 years old at “Remigio Díaz Quintanilla primary school” in Sancti Spiritus. Methodology: a transversal descriptive observational study was carried out in the period from September to December 2011. The population was composed by of 622 children and the simple taken was of 526 children. The observation and the structured interview were used; the variables were sex, oral health and level of information. Results: caries of the first permanent molar was frequent in almost half of the patients of the group from 10-11 years, in both sexes the oral hygiene was fair, but males were more preponderant, and the third part of the children had an insufficient level of information about oral health. Conclusions: despite the efforts carried out to preserve the health of the first permanent molar, the affectation persists due to caries of this molar as well as a fair oral hygiene so it is necessary to design an action system to contribute to the health of the first permanent molar.

Orquitis idiopática granulomatosa. Estudio anatomopatológico de un caso / Idiopathic granulomatous orchitis. Pathologic study of one case

OBJETIVO: Presentar un caso de orquitis granulomatosa idiopática, patología extremadamente infrecuente, en un paciente de 76 años. MÉTODOS/RESULTADOS: Se recibe testículo derecho con diagnóstico clínico y ecográfico de tumor testicular. En el estudio anatomopatológico se observa la ausencia de celularidad neoplásica y se aprecia hallazgos morfológicos compatibles con orquitis granulomatosa idiopática. CONCLUSIONES: La orquitis idiopática granulomatosa es una entidad de etiología desconocida, ecográfica y clínicamente no distinguible de una neoplasia testicular, llegándose al diagnóstico tras la orquiectomía

OBJECTIVE: To report one case of idiopathic granulomatous orchitis, an extremely rare disease, in a 76-year-old patient. METHODS/RESULTS: The pathology department received a testicle with the clinical/radiological diagnosis of testicular tumor. The pathologic study showed absence of neoplasias and presence of morphological findings compatible with idiopathic granulomatous orchitis. CONCLUSIONS: The idiopathic granulomatous orchitis is an entity of unknown etiology, clinically or ultrasonographically not distinguishable from testicular neoplasias, the diagnosis of which is made after orchiectom

Sarcoma de estroma endometrial. Estudio clinicopatológico e inmunofenotípico de 5 casos / Endometrial stromal sarcoma. Clinicopathologic and immunophenotypic study of 5 cases

Introducción: El sarcoma de estroma endometrial uterino es un tumor muy poco frecuente con una incidencia de 0,4 -3,4 por 100.000 mujeres, comprende menos del 1% de tumores malignos ginecológicos y el 2-5% de tumores malignos uterinos, siendo el tercer sarcoma en frecuencia después del carcinosarcoma y el leiomiosarcoma. Material y métodos: Presentamos cinco casos de sarcoma de estroma endometrial diagnosticados en un periodo de siete años con la evolución clínica de las pacientes. Resultados: Immunohistoquímicamente, estos tumores expresaban vimentina, receptores hormonales, CD10 y p53 (sarcomas de alto grado), siendo los marcadores musculares, la CK, el c-kit y ALK negativos. De estos cinco sarcomas dos de ellos eran de alto grado y tres de bajo grado (uno con diferenciación miogénica y otro combinado con áreas tipo «cordones sexuales», áreas miogénicas y áreas estromales puras). Discusión: Asimismo se plantean una serie de diagnósticos diferenciales con metodología práctica para poder caracterizar estos tumores

Introduction: Endometrial stromal sarcoma (ESS) is an infrequent neoplasm with an incidence of 0,4-3,4 cases/100.000 woman. This tumor represents less than 1% of the gynecologic malignancies as well as the 2,5% of the uterine malignant tumors being the third malignancy after carcinosarcoma and leiomyosarcoma. Patients and methods: We present five cases of endometrial sarcoma, diagnosed in a period of seven years. This study includes a complete immunohistochemical analysis as well as the follow-up of the patients. Results: Immunohistochemically, these tumors expressed vimentin, hormone receptors, CD10 and p53 (high grade sarcomas), whereas muscle markers, CK, c-Kit and ALK resulted negative. Two of the ESS were high grade tumors whereas the other three were considered low grade tumors (one with myogenic differentiation and the other combining sex cord areas, myogenic foci and pure stromal areas). Discussion: We discuss their differential diagnosis with other uterine malignant tumors