Caso Clínico: Meningohidroencefalocele Gigante / Case Report: Giant Meningohydroencephalocele

INTRODUCCIÓN: Los encefaloceles son una anomalía congénita rara, que afecta a 1 de cada 5 000 nacidos vivos. Un meningohidroencefalocele es la herniación de meninges, ventrículos y parte del encéfalo a través de un defecto congénito en el cráneo. El pronóstico de los recién nacidos con encefalocele depende de varios factores. La corta edad y las anomalías congénitas asociadas en estos pacientes plantean desafíos en el diagnóstico y manejo. CASO CLÍNICO: Recién nacido masculino, nacido a las 37 semanas de gestación, por cesárea de emergencia por parto en fase latente, presentado una masa de gran tamaño, sobre la región parieto-occipital, de bordes lobulados, irregulares, cubierta en parte por cuero cabelludo, y en otras zonas de un tono rojo violáceo. El neonato fue poco reactivo, con tono disminuido, llanto ausente. EVOLUCIÓN: Tras valoración del caso por especialistas, debido al importante defecto de la calota craneal y las anomalías estructurales cerebrales, el paciente no fue candidato para tratamiento quirúrgico. Neonato fue dado de alta con expectativa de vida reducida, para recibir cuidados en el hogar. CONCLUSIÓN: El meningohidroencefalocele gigante es un defecto del tubo neural poco frecuente. El manejo de las encefaloceles puede ser complicado y debe ser individualizado y dependerá de las estructuras anatómicas comprometidas. Si bien en este caso el pronóstico fue malo, siempre es necesario el trabajo multidisciplinario para un adecuado manejo.

BACKGROUND: Encephaloceles are a rare congenital anomaly, affecting 1 in 5 000 live births.A meningohydroencephalocele is the herniation of the meninges, ventricles, and part of the brain through a congenital defect in the skull. The prognosis of newborns depends on several factors. The young age and the associated congenital anomalies in these patients contribute to the diagnostic and treatment challenge. CASE REPORT: Male newborn, born at 37 weeks of gestation, by emergency C-section due to latent labor, presenting a large mass over the parieto-occipital region, with lobed, irregular edges, partially covered by the scalp, and in other areas a purplish red tone. The newborn was no very reactive, had decreased tone, absent crying. EVOLUTION: After specialist's evaluation, due to the significant defect in the cranial shell and the structural brain abnormalities, the patient was not a candidate for surgical treatment. The newborn was discharged with a reduced life expectancy, to receive care at home. CONCLUSION: giant meningohydroencephalocele is a rare neural tube defect. The management of encephaloceles can be complicated and must be individualized, and will depend on the anatomical structures involved. Although in this case the prognosis was bad, multidisciplinary work is always necessary for proper management.

Adult Nasoethmoidal Encephalocele Corrected by Supraorbitary Approach: Case Report and Literature Review

Encephalocele is a protrusion of the central nervous system elements through a defect in the dura mater and in the cranium. The prevalence of encephalocele ranges from 0.08 to 0.5 per 1,000 births. The posterior encephaloceles are more common in North America and Europe, while frontal defect is frequently found in Asia. The present paper describes a 26-year-old male patient presenting with cerebrospinal fluid leak and meningitis symptoms. He was diagnosed with congenital nasoethmoidal encephalocele and treated surgically using a supraorbital approach without complications.

Large Vertex Epidural Hematoma: Case Report and Review of Surgical Approaches

Vertex epidural hematomas (VEHs) are a special clinical entity due to their clinical presentation, vascular etiology and options of surgical approach. The clinical suspicion involves recognizing the mechanism of the injury and the correct visualization of the hematoma in computed tomography (CT) coronal sequences. In the present article, we describe a case of a very large (146 mL) VEH with central brain herniation, and provide a technical note on the surgical planning and treatment. A 34-year-old male patient was admitted to the hospital after an injury on the left superior parietal region. The Glasgow coma scale score was 6, and the left pupil of the patient was dilated. The CT scan showed a large epidural hematoma on the vertex between the coronal e lambdoid sutures, and a fracture over the sagittal suture. During the surgery, multiple burr holes were made laterally to the sagittal suture, and after inspection and no visualization of bleeding in the superior sagittal sinus (SSS), we performed a standard biparietal craniotomy. The patient was discharged three days after the surgery without any deficits. Currently, with the improvement in imaging modalities,more cases of VEH are being identified. Identifying the etiology prior to the craniotomy is challenging in severe cases. Tears in the SSS can bleed profusely, and they demand strategies during the craniotomy.With multiple burr holes parallel to the sagittal suture, we can visualize whether there is bleeding in the SSS and design a craniotomy with or without a central osseous bridge to anchor the dura. Neurosurgeons must be prepared to plan a surgical strategy in cases of large VEHs. Due to its rare frequency and bleeding risks, VEHs are considered challenging.

Dermoid cyst with cerebellar meningoencephalocele at different locations accompanied by posterior fossa abnormalities: case report

ABSTRACT CONTEXT: Dermoid cysts are well-defined cysts containing sebaceous glands and dermal structures. In the literature, dermoid cysts and associated closure defects have been described in the same locations. CASE REPORT: In this case, a dermoid cyst was found at the base of the mouth with a coexisting closure defect in the occipital calvarium. Additional abnormalities were also observed, including posterior myeloschisis, right cerebellar dysgenesis, vermian hypogenesis and posterior fusion of the second and third vertebrae. The finding of a dermoid cyst located at the base of the mouth is discussed here, with additional imaging findings. CONCLUSION: Dermoid cysts in the head and neck region may be accompanied by posterior fossa abnormalities.

Tomografía computada y resonancia magnética de variantes normales/congénitas de apariencia quística y presentación frecuente en el encéfalo / CT and MRI of Normal/Congenital Variables with Cystic Appearance and Frequent Presence in the Brain

La presentación de una imagen de apariencia quística durante el estudio del encéfalo constituye un hallazgo incidental cada vez más frecuente, pudiendo encontrarse en el espacio extra o intraaxial. Las mismas pueden ser de naturaleza congénita o adquirida, benigna o maligna, ocupantes de espacio con desplazamiento de la línea media o simplemente presentarse sin efecto compresivo alguno. De localización supra o infratentorial, esas imágenes constituyen un desafío diagnóstico, siendo imprescindible su reconocimiento para no solicitar estudios o tratamientos innecesarios. Valoraremos las imágenes de apariencia quística más frecuentes empleando tomografía computada o imágenes de resonancia magnética.

Presence of a cystic formation in brain examination is frequently an incidental finding. They can be intra or extra-axial in location, congenital or acquired, benign or malignant with or without mass effect. Intracranial cysts can be a diagnostic challenge and we should know them to avoid unnecessary exams or treatment. We will analyze the most common cystic formations seen in computed tomography and magnetic resonance.

Encefalocele frontonasal: reporte de un caso con diagnóstico prenatal / Frontonasal encephalocele: report of a case with prenatal diagnosis

El encefalocele es un defecto congénito mayor que se caracteriza por la herniación del tejido nervioso y las meninges a través de un defecto de la bóveda craneal. Esto se debe a alteraciones del cierre del tubo neural ocurridas en la cuarta semana de gestación. El diagnóstico prenatal es posible realizarlo a través de ecografía 2D y 3D. Se presenta el caso de un neonato con encefalocele anterior, entidad poco frecuente dentro de los defectos del cierre del tubo neural. Se realizó el diagnóstico presuntivo prenatal por ecografía 2D y 3D, y se confirmó al nacimiento por los hallazgos clínicos y escanografía(AU)

The encephalocele is a major birth defect characterized by herniation of neural tissue and meninges through a defect in the skull, due to alterations of neural tube closure occurring in fourth week of pregnancy. Prenatal diagnosis is possible through 2D and 3D ultrasound. We report a case of anterior encephalocele, a rare disease among the defects of neural tube closure, with diagnosis by 3D and 2D ultrasonography, which was confirmed at birth by clinical findings and scan(AU)

[Herpes simplex virus encephalitis during immunosuppressive treatment of ulcerative colitis]

Today, with the advent of new medications, treatment of Ulcerative colitis [UC] has been markedly improved. Immunosuppressive drugs used in therapy predispose patients to opportunistic infections. A 22-year-old woman was admitted to emergency department due to acute exacerbation of UC and decreased level of consciousness. She was under treatment with 5-aminosalicylate, prednisolone and azathioprine. In neurological evaluation, patient had cerebral herniation. Non-contrast CT scan revealed multiple hemorrhagic areas in both frontal lobes. Right frontal craniotomy was performed emergently. Histopathologic evaluation of brain tissue was reported as "Herpes simplex encephalitis". Polymerase chain reaction [PCR] assay was also positive for HSV DNA. Immunosuppressive drugs such as azathioprine have a pivotal role in the treatment of resistant and/or severe cases of UC. Prevalence of infectious complications was reported to be 7.4%, 1.8% of which were severe [including herpes zoster encephalitis] .Our review of literature indicates that no case of herpes simplex encephalitis following immunosuppressive therapy for UC has been reported

Limb-body wall complex, report of 2 cases with their quintessence in prenatal diagnosis.

Limb-body wall complex is a complicated fetal malformation with the essential features of: 1) exencephaly/encephalocele with facial clefts, 2) thoraco- and/or abdominoschisis, and 3) limb defect. The diagnosis was based on two of three of the above features. We report 2 cases of limb-body wall complex. The first case had thoraco-abdominal and limb anomalies while the other had abdominal wall, limb and neuro-facial anomalies. Both cases were diagnosed prenatally by ultrasonography. They were terminated by medical induction. Chromosome studies were evaluated for academic purposes. Autopsies were done to confirm diagnosis. Aspects of their varieties of clinical features, differences in differential diagnosis, and pitfalls in prenatal diagnosis were discussed.

Meckel-Gruber syndrome associated with short limbed dwarfism.

Antenatal detection of Meckel-Gruber Syndrome associated with short-limbed devarfism is described here. This association has not been previously reported.