RESUMO
INTRODUCTION: Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm. CLINICAL PRESENTATION: Congenital orbital teratoma commonly presents as unilateral proptosis, with rapid growth, leading to exposure keratopathy. DIAGNOSIS: Prenatal ultrasound may detect the orbital mass, computed tomography (CT) scans, and magnetic resonance (MR) imaging are better in demonstrating multilocular cystic and solid mass, without bone erosion. Laboratory tests should include alfa-fetoprotein (AFP) and B-human chorionic gonadotropin (B-HCG), and histopathologically, it contains all three germ cell layers components. The management is surgical removal of the lesion, the mature teratoma has a benign behavior, and the immature has a poor prognostic. We describe a rare case of congenital orbital teratoma with intracranial extension of the lesion, in which was treated with orbital exenteration. After surgery, AFP levels decreased, the middle face displacement has improved and development milestones were appropriate.
Assuntos
Neoplasias Orbitárias , Teratoma , Humanos , Teratoma/cirurgia , Teratoma/congênito , Teratoma/diagnóstico por imagem , Teratoma/patologia , Neoplasias Orbitárias/cirurgia , Neoplasias Orbitárias/congênito , Neoplasias Orbitárias/diagnóstico por imagem , Neoplasias Orbitárias/patologia , Imageamento por Ressonância Magnética , alfa-Fetoproteínas/metabolismo , Tomografia Computadorizada por Raios X , Feminino , Masculino , Recém-NascidoRESUMO
PURPOSE: Ventriculo-peritoneal shunt is the gold standard for non-obstructive hydrocephalus. Despite advances in material, infection prevention, and valve technologies, failure can still occur. The aim of this article is to present a comprehensive study based on the experience of a reference center in pediatric neurosurgery in Rio de Janeiro with the use of the ventriculo-gallbladder shunt as an alternative to peritoneal failure. METHODS: A retrospective study was conducted from January 2018 to December 2023 of patients diagnosed with cerebrospinal fluid shunt dysfunction due to peritoneal failure and submitted to ventriculo-gallbladder shunt as an alternative in a reference center of Rio de Janeiro. RESULTS: From 2018 to 2023, 18 peritoneal failures were diagnosed. Among them, 10 patients (55.5%) were selected for ventriculo-gallbladder shunt (VGS). Different causes were responsible for the hydrocephalus in these patients. VGS was placed at a mean age of 35.4 months. Four patients had temporary complications: 2 self-limited diarrheas in the first month and 2 shunt infections. After the resolution of the infection, a new VGS was placed successfully. The average follow-up was 18.8 months (follow-up 9-68 months) without further issues. CONCLUSION: VGS is a viable option for patients facing peritoneal failure. This paper provides valuable insights into the surgical technique and outcomes associated with this alternative.
Assuntos
Derivações do Líquido Cefalorraquidiano , Vesícula Biliar , Hidrocefalia , Humanos , Pré-Escolar , Hidrocefalia/cirurgia , Estudos Retrospectivos , Feminino , Masculino , Lactente , Criança , Vesícula Biliar/cirurgia , Derivações do Líquido Cefalorraquidiano/métodos , Derivações do Líquido Cefalorraquidiano/efeitos adversos , Derivação Ventriculoperitoneal/métodos , Derivação Ventriculoperitoneal/efeitos adversosRESUMO
Intracranial tumors in the first year of life are rare and, in this age group, are the second most common type of pediatric cancer after leukemias. As the more common solid tumor in neonates and infants, they present some peculiarities such as the high incidence of malignancies. Routine ultrasonography made easier to detect intrauterine tumors, but diagnosis can be delayed due to the lack or scarcity of recognizable symptoms. These neoplasms are often very large and highly vascular. Their removal is challenging, and there is a higher rate of morbidity and mortality than seen in older children, adolescents, and adults. They also differ from older children with respect to location, histological features, clinical behavior, and management. Pediatric low-grade gliomas represent 30% of the tumors in this age group and comprise circumscribed and diffuse tumors. They are followed by medulloblastoma and ependymoma. Other non-medulloblastoma embryonal neoplasms, former known as PNETS, are also commonly diagnosed in neonates and infants. Teratomas have an expressive incidence in newborns but decline gradually until the end of the first year of life. Immunohistochemical, molecular, and genomic advances are impacting the understanding and targeting of the treatment of some tumors, but, despite all these advances, the extent of resection remains the most important factor in the prognosis and survival of almost all types of tumors. The outcome is difficult to estimate, and 5-year survival ranges from one-quarter to three-quarters of the patients.
Assuntos
Neoplasias Encefálicas , Neoplasias Cerebelares , Glioma , Meduloblastoma , Neoplasias Embrionárias de Células Germinativas , Adolescente , Humanos , Criança , Recém-Nascido , Neoplasias Encefálicas/diagnóstico por imagem , Meduloblastoma/tratamento farmacológicoRESUMO
BACKGROUND: Abusive head trauma (AHT) is the most serious injury inflicted to the nervous system of neonate an infant with a high incidence of disabilities. The authors present two cases in which the initial manifestations and neurologic status were misinterpreted and stress that clinical presentation and imaging can be variable and confuse the examiner. DISCUSSION: Subdural hemorrhage (SDH) in this age group raises high suspicion of non-accidental trauma but have been reported in other situations such as several bleeding disorders. Although rare, hematological diseases should be considered when other data of maltreatment are lacking. CONCLUSION: Differential diagnosis is important to avoid underdiagnosing AHT and to prevent morbidity if a pre-existing hematological disease is misdiagnosed.
Assuntos
Maus-Tratos Infantis , Traumatismos Craniocerebrais , Lactente , Criança , Recém-Nascido , Humanos , Maus-Tratos Infantis/diagnóstico , Traumatismos Craniocerebrais/diagnóstico por imagem , Traumatismos Craniocerebrais/etiologia , Hematoma Subdural/diagnóstico por imagem , Hematoma Subdural/etiologia , Tomografia Computadorizada por Raios X/métodos , IncidênciaRESUMO
INTRODUCTION: Encephaloceles are rare congenital malformations of the central nervous system in which brain tissue is extruded from a defect in the skull. Hydrocephalus can occur in 60 to 90% of patients with posterior encephaloceles when compared to other types of this malformation. This article aims to present a series of posterior encephaloceles and its association with hydrocephalus as well as promote a review of the pertinent literature. MATERIAL AND METHODS: A retrospective study of our series based on hospital charts of 50 patients with posterior encephaloceles was performed. Data on sex, location of encephalocele, presence of associated malformations, presence of neural tissue within the malformation, presence of hydrocephalus and microcephaly were recorded. RESULTS: There were 29 females and 21 males. There were 25 (50%) supratorcular, 8 (16%) torcular, and 17 (34%) infratorcular lesions. Mean age of encephalocele primary repair was 8 days (range 2-120 days). Hydrocephalus was diagnosed in 25 (50%) of the cases. Ventriculoperitoneal shunt was inserted in 24 patients. The mean age at VP shunt insertion was 1.3 months (range 0.3-9 months). Endoscopic third ventriculostomy was successfully performed in one patient. Dandy-Walker malformation and ventriculomegaly prior to encephalocele surgical correction were positively associated with hydrocephalus (p values 0.05 and 0.01, respectively). Chiari III malformation was found in 2 cases, both requiring CSF shunt for treatment of hydrocephalus and are stable in follow-up. Microcephaly was present in 9 cases. The known mortality rate was 8%. CONCLUSIONS: Hydrocephalus is common in patients with posterior encephaloceles, being more frequent in the supratorcular type, especially when associated to Dandy-Walker, Chiari III malformation, and pre-existing ventriculomegaly. The severity of giant encephaloceles, when associated to torcular types and microcephaly, is a limiting factor for development of hydrocephalus, due both to the rapid evolution of natural history and the structural changes in microcephaly.