ABSTRACT
A case is described of a full-term newborn with hydroanencephalia due to congenital toxoplasmosis. Prenatal diagnosis had been made. The infection was supposedly acquired in the early stages of the pregnancy, the mother presented positive serology for active infection. The newborn presented elevated specific IgM. Fetal and newborn MRI was compatible with hydrancencephalia. The newborn presented macrocephalia, cataracts, abnormal archaic reflexes, hypotonia, thermoregulation abnormalities, and altered spinal fluid. Due to the severity and rarity of this clinical picture, and an absence of a national detection program, the possibility of offering mothers the possibility of detecting this illness is highlighted.
Se presenta el caso de un recién nacido de término con hidranencefalia por toxoplasmosis congénita con diagnóstico prenatal, el cual habría adquirido su infección en forma precoz durante el embarazo. Destaca madre con serología positiva para infección activa. Recién Nacido (RN) con IgM específica elevada. Resonancia Magnética fetal y del neonato compatible con hidranencefalia. RN con macrocefalia, catarata, reflejos arcaicos anormales, hipotonía, alteración del la termorregulación y LCR alterado. Frente a la gravedad y lo inusual del cuadro clínico y considerando la ausencia de un programa nacional de tamizaje se destaca la importancia de ofrecer a la madre embarazada la posibilidad de detección precoz y prevención de esta enfermedad frente a las severas complicaciones neurológicas de esta infección congénita.
Subject(s)
Humans , Male , Female , Pregnancy , Infant, Newborn , Hydranencephaly/etiology , Toxoplasmosis, Congenital/complications , Fatal Outcome , Hydranencephaly/diagnosis , Infectious Disease Transmission, Vertical , Pregnancy Complications, Infectious , Prenatal Diagnosis , Toxoplasmosis, Congenital/diagnosis , Toxoplasmosis/complicationsABSTRACT
La hidranencefalia pertenece a las encefalopatías del sistema nervioso central. Es un defecto grave de la perfusión cerebral que produce una destrucción tardía de los territorios irrigados por ambas arterias carótidas internas, con preservación de los territorios irrigados por las arterias vertebro basilares. El conocimiento de esta encefalopatía por el odontopediatra permitirá tratar adecuadamente al paciente y realizar un tratamiento exitoso, mejorando su calidad de vida. Se presenta el caso clínico de un niño al que durante el quinto mes de vida intrauterina le fue realizada una ecografía donde se diagnosticó la encefalopatía.
Subject(s)
Humans , Male , Child, Preschool , Dental Care for Disabled/methods , Hydranencephaly/diagnosis , Hydranencephaly/pathology , Hydranencephaly/rehabilitation , Diagnosis, Differential , Preventive Dentistry/methods , Mouth Rehabilitation/methodsABSTRACT
OBJECTIVE: To prospectively evaluate the results of endoscopic choroid plexus cauterization (ECPC) and ventriculoperitoneal shunts (VPSs) in infants with hydranencephaly or near hydranencephaly. METHODS: We prospectively collected clinical data from all untreated hydranencephalic and near hydranencephalic children from October 2006 to March 2008. All patients treated were randomly divided into 2 groups, ECPC or VPS, and submitted to either endoscopic choroid plexus cauterization or ventriculoperitoneal shunt placement. RESULTS: Seventeen patients were entered into the study. ECPC was completed in 9 patients; the procedure successfully controlled excessive head circumference and signs of increased intracranial pressure in 8 of these patients (88.8%). One endoscopic procedure in a hydranencephalic child failed after 7 months, resulting in VPS placement. Thus, of the 10 patients randomized to ECPC, 8 were treated successfully by ECPC (80%), and 2 went on to have a VPS. There were no complications related to this method of treatment. Seven children were randomized to the VPS group; and of these, 2 patients (28.5%) required shunt revisions during follow-up. There were no complications related to shunt placement. There was no difference in the success rate between patients randomized to ECPC and VPS, but the ECPC was more economical. CONCLUSION: ECPC is an acceptable alternative to VPS for treatment of hydranencephaly and near hydranencephaly. It is a single, definitive, safe, effective, and economical treatment that may avoid the complications of shunting.
Subject(s)
Choroid Plexus/surgery , Endoscopy/methods , Hydranencephaly/pathology , Hydranencephaly/surgery , Ventriculoperitoneal Shunt/methods , Adult , Cerebral Ventricles/surgery , Female , Follow-Up Studies , Humans , Hydranencephaly/diagnosis , Male , Prospective Studies , Retrospective Studies , Time Factors , Treatment OutcomeABSTRACT
Existe gran similitud entre hidranencefalia e hidrocefalia congénita severa, tanto clínicamente como en estudios de imagen. En la hidranencefalia, habitualmente el electroencefalograma no muestra actividad eléctrica y los potenciales evocados auditivos del tallo cerebral son normales. En el presente artículo se informa el caso de un niño con hidranencefalia asociada a oclusión bilateral de las carótidas internas y se discuten aspectos clínicos de reuroimagen, así como potenciales auditivos del tallo cerebral, perfil del sueño y actividad electroencefalográfica paroxística subclínica
Subject(s)
Infant , Humans , Male , Hydranencephaly/diagnosis , Electroencephalography , Carotid Stenosis , Sleep/physiologyABSTRACT
Os autores relatam sete casos de hidranencefalia verificados em uma populaçäo de 62 pacientes com diagnóstico presuntivo de hidrocefalia. Chamam a atençäo para a alta proporçäo desta patologia, dentre os pacientes analisados, bem como, as disparidades dos achados clínicos, a extensäo das malformaçöes e as controvérsias existentes na literatura
Subject(s)
Infant, Newborn , Infant , Humans , Male , Female , Hydranencephaly/diagnosisABSTRACT
Se comunican cinco casos de diagnóstico antenatal de hidranencefalia por ultrasonografía. Los cinco recién nacidos son estudiados por ultrasonografía, tomografía axial computarizada, electroencefalograma, y un caso fallecido, por anatomía patológica. En los cuatro sobrevivientes se realizó examen neurológico la primera y cuarta semana de vida, y posteriormente cada dos meses. Se efectua análisis clínico y patológico de estos pacientes, comentando las etiologías probables y su pronóstico. Algunos elementos del diagnóstico diferencial antenatal y posnatal entre hidrocefalia, hidranencefalia y porencefalia se enfatizan, considerando nuestra experiencia y la literatura al respecto
Subject(s)
Infant, Newborn , Humans , Male , Female , Hydranencephaly/diagnosis , Prenatal Diagnosis , Ultrasonography , Follow-Up StudiesABSTRACT
The study of six patients with hydranencephaly is reported. Clinical and necroscopic findings have been performed in correlation to routine laboratory examination. The Authors have concluded that transilumination is an important procedure for early diagnostic of these cases, especially when the patients have a milder or incomplete form of the syndrome. The necroscopic findings suggest that hydranencephaly may be the result of encephaloclastic lesions (inflammatory, mechanical or vascular) that, either before or after birth, lead to complete destruction of the brain, with sparing of sub-tentorial structures.