ABSTRACT
Abstract: Optic Neuritis (ON) is a condition caused by the inflammation of the optic nerve, causing diminished visual acuity and ocular pain. It is tightly related to Multiple Sclerosis (MS), often being the first demyelinating event. There is a 31% risk of recurrence during the first 10 years after the diagnosis, and 48% of the patients end up being diagnosed with Multiple Sclerosis1. Imaging studies like brain MRI (Magnetic Resonance Imaging) have a critical role in the diagnosis and prognosis of ON, as well as in the recognition of MS.2 The patient is a 11-year-old girl with recurrent ON and past medical history of Acute Disseminated Encephalomyelitis (ADEM) when she was 5 years old. ON diagnosis was based on clinical findings as well as on ophtalmologic, electrophysiologic and imaging studies. The recurrent episodes of ON improved after the use of high dose steroids. Recurrences were observed after titration of the dose, but remission was achieved after adjustment of treatment. During one of the recurrent episodes, blood work was performed to evaluate possible underlying infectious, demyelinating or autoinmmune process. Anti-MOG antibodies were found positive. The patient at the moment is not presenting with any other criteria suggesting MS or Optic Neuromyelitis, but long term follow up is adviced. Key words: Optic neuritis (ON), multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), magnetic resonance imaging (MRI), cerebrospinal fluid (CSF), right eye (RE); visual acuity (VA), optic neuromyelitis (ONM)
Subject(s)
Humans , Optic Neuritis , Encephalomyelitis, Acute Disseminated , Magnetic Resonance Imaging , Cerebrospinal Fluid , Antibodies , Multiple SclerosisABSTRACT
El síndrome de transfusión feto fetal es una complicación grave de las gestaciones gemelares monocoriales. Su etiología se debe a la transfusión crónica de sangre del feto donante, al feto receptor, a través de las anastomosis vasculares entre los territorios placentarios de ambos. El cuadro clínico característico se evidencia en ambos fetos, receptor y donante, lo cual es consecuencia directa de las alteraciones en la volemia que padece cada uno. En el feto receptor se observa un polihidramnios relacionado con la poliuria derivada de un estado de hipervolemia constante, que acaba evolucionando hacia una insuficiencia cardiaca congestiva. En el feto donante el cuadro es inverso, caracterizado por oligoamnios, oliguria, crecimiento intrauterino retardado e hipovolemia, con tasas de mortalidad sin tratamiento de entre el 80-100% de los casos, que varían en función del grado de transfusión. El diagnóstico se basa en el estudio ecográfico exhaustivo de ambos fetos, procurando un diagnóstico precoz y un estadiaje correcto, ya que el pronóstico se verá muy influenciado por la actuación temprana. El tratamiento de elección es la coagulación láser de las anastomosis vasculares por fetoscopia, que se realiza idealmente entre la semana 18 y 26 de embarazo. El pronóstico es variable, en función de la disponibilidad de terapia fetal y de la edad gestacional en el momento del diagnóstico, siendo, en general, de muy mal pronóstico sin tratamiento efectivo(AU)
Twin-twin transfusion syndrome (TTTS) is one of the most serious complications of monochorionic multiple gestations. The etiology is due to a chronic blood transfusion from de donor twin to the recipient twin trough vascular anastomoses between both placenta territories. Main clinical presentation is present in both donor and recipient fetuses, as a direct consequence of a blood volume alteration in each one. The recipient twin shows polyhydramnios related to polyuria resulting from a state of constant fluid overload, finally evolving into congestive heart failure. In the donor twin, the clinical presentation is opposite and shows oligoamnios, oliguria, intrauterine retarded growth and hypovolemia. Untreated mortality rates are between 80-100 % of all cases, which may vary depending on the severity of the transfusion. Diagnoses is based on exhausting ultrasound examination of both fetuses, securing early diagnosis and correct staging, since prognosis will be greatly influenced by early actions. Fetoscopic laser ablation is generally the definitive treatment between 16 and 26 weeks of gestation. The prognosis is variable, depending on the availability of fetal therapy and gestational age at diagnosis. It is generally very poor prognosis without effective treatment(AU)
Subject(s)
Fetofetal Transfusion/diagnosisABSTRACT
El síndrome de transfusión feto fetal es una complicación grave de las gestaciones gemelares monocoriales. Su etiología se debe a la transfusión crónica de sangre del feto donante, al feto receptor; a través de las anastomosis vasculares entre los territorios placentarios de ambos. El cuadro clínico característico se evidencia en ambos fetos: receptor y donante, lo cual es consecuencia directa de las alteraciones en la volemia que padece cada uno. En el feto receptor, se observa un polihidramnios relacionado con la poliuria derivada de un estado de hipervolemia constante, que evoluciona hacia una insuficiencia cardiaca congestiva. En el feto donante, el cuadro es inverso; caracterizado por oligoamnios, oliguria, crecimiento intrauterino retardado e hipovolemia, con tasas de mortalidad sin tratamiento de entre 80-100 por ciento de los casos, que varían en función del grado de transfusión. El diagnóstico se basa en el estudio ecográfico exhaustivo de ambos fetos, procurando un diagnóstico precoz y un estadiaje correcto, ya que el pronóstico se verá muy influenciado por la actuación temprana. El tratamiento de elección es la coagulación láser de las anastomosis vasculares por fetoscopia, idealmente entre la semana 18 y 26 de embarazo. El pronóstico es variable, en función de la disponibilidad de terapia fetal y de la edad gestacional en el momento del diagnóstico; al ser, en general, de muy mal pronóstico sin tratamiento efectivo(AU)
Twin-twin transfusion syndrome is one of the most serious complications of monochorionic multiple gestations. Its etiology is due to a chronic blood transfusion from de donor twin to the recipient twin through the vascular anastomoses between the placental territories of both fetuses. The characteristic clinical presentation appears in both donor and recipient fetuses, as a direct consequence of altered volemia in each one. Polyhydramnios related to polyuria resulting from a state of constant hypervolemia is observed in the recipient twin, finally evolving into congestive heart failure. The clinical presentation in the donor twin is reversed and characterized by oligoamnios, oliguria, retarded intrauterine growth and hypovolemia, with untreated mortality rates ranging 80-100 percent of all cases, which may vary depending on the severity of the transfusion. The diagnosis is based on exhaustive echographic examination of both fetuses to make an early diagnosis and correct staging, since the prognosis depends very much on early action. The treatment of choice is fetoscopic laser coagulation of vascular anastomoses between 18 and 26 weeks of gestation. The prognosis is variable, depending on the availability of fetal therapy and the gestational age at diagnosis, being prognosis generally very poor without an effective treatment(AU)