Fetal epidural hematoma secondary to a mild blunt prenatal trauma: case report and review of the literature.

Fetal intracranial hemorrhage affects 1 in every 10,000 pregnancies. In most cases, the etiology of the bleeding is multifactorial, and they can be either related to the mother or the fetus. Blunt prenatal trauma was occasionally associated with these hemorrhages, nevertheless, reports of hematomas secondary to mild traumas are rare. Within the prenatal intracranial bleedings, the most frequent are the subarachnoid hematoma and intraparenchymal, scarcely ever the epidural hematoma. Treating these bleedings is challenging due to the ongoing pregnancy. Thus, the prognosis is often reserved, with a mortality rate of 43% and 25% of neurological sequelae. Here, we report a singular case of a fetal epidural hematoma secondary to a mild blunt trauma at the third trimester with a good outcome.

Hydrocephalus secondary to dengue encephalitis in an infant: case report.

BACKGROUND: Dengue is the leading cause of mosquito-borne viral infection. It is responsible for high morbidity and mortality in children living in endemic areas. Nowadays, neurological complications are progressively referred to and include a broad spectrum of symptoms. It can be secondary to metabolic alterations, direct invasion by the virus, and enhanced autoimmune response. CASE PRESENTATION: Here, we report a rare case of hydrocephalus secondary to dengue encephalitis. A 13-day-old boy was diagnosed with dengue. He evolved with seizures and impaired consciousness being diagnosed with encephalitis. After clinical treatment, he was discharged fully recovered. Three months later, he presented with signs and symptoms of intracranial hypertension. Brain MRI revealed hydrocephalus secondary to acquired aqueduct stenosis. The patient underwent an endoscopic third ventriculocisternostomy. CONCLUSION: Dengue infection is a differential diagnosis for acute febrile neurological impairment in children from endemic areas. Follow-up should be offered after dengue encephalitis to detect possible late-onset complications, as hydrocephalus.

Anorexia as the first clinical manifestation of von Hippel-Lindau syndrome.

Hemangioblastomas (HBs) of the brain may present without neurological symptoms over a long period of time due to their benignity and slow growth. We herein present the case of a female patient who developed a HB of the fourth ventricle presenting only with severe weight loss and anorexia. The patient was screened for mutations in all 3 exons of the VHL gene using Sanger sequencing, and was found to have a nonsense mutation in the VHL gene (single-nucleotide change causing a premature stop codon: c.481C>T; p.Arg161*), causing formation of a truncated protein, consistent with von Hippel-Lindau syndrome (VHLs). The patient was first misdiagnosed with anorexia nervosa (AN) due to the lack of other symptoms. Molecular diagnosis allows further investigation of other VHLs-related tumors and timely, appropriate treatment. However, misdiagnosing anorexia nervosa may lead to poor prognosis and even death; thus, differential diagnosis is crucial in all such cases. The present case report provides evidence that fourth ventricular lesions may affect food intake control and satiety, and highlights the importance of accurate molecular diagnosis.

Pediatric intrinsic brainstem lesions: clinical, imaging, histological characterization, and predictors of survival.

PURPOSE: Brainstem lesions comprise 10-20% of all pediatric brain tumors. Over the past years, the benefits of stereotactic biopsy versus the use alone of MRI features to guide treatment have been controversial. METHODS: Retrospective study with pediatric patients submitted to stereotactic brainstem biopsies between 2008 and 2018. Demographic, clinical, imaging, and surgical characteristics were recorded, as well as the histological diagnosis, complications, and survival. Predictors of survival were evaluated through Cox regression models after multivariate adjustment. RESULTS: Twenty-six patients (mean age of 8.8 ± 4.3 years and 14 female). Diagnosis was reached on 84.6% (95% CI 65.1-95.6%) of the patients. Glioma was diagnosed on 20 cases (11 high-grade and 9 low-grade lesions). There was no association between age and gender and the dichotomized histological diagnosis. Contrast enhancement, diffuse distribution, invasion of adjacent structures, and remote injury were present on 62.5%, 75.0%, 62.5%, and 25.0% of the cases. Hydrocephalus at admission was present on almost half of the patients (46.2%). Only radiological invasion of adjacent structures had a possible association with high-grade lesions (p = 0.057). Surgical trajectory was trans-cerebellar in most of the cases (79.9%). There were no major complications and only two minor/transitory complications. Poorer survival was independently associated with high-grade lesions (HR 32.14, 95% CI 1.40-735.98, p = 0.030) and contrast enhancement at MRI (HR 36.54, 95% CI 1.40-952.26, p = 0.031). CONCLUSIONS: Stereotactic biopsy was safe and allows successful tissue sampling for a definite diagnosis. Poorer survival was independently associated with high-grade and contrast-enhancing lesions.

Brain abnormalities in myelomeningocele patients.

BACKGROUND: Myelomeningocele (MMC) is often related to hydrocephalus and Chiari malformation (CM) type 2; however, other brain abnormalities have been reported in this population. In order to better understand and quantify other forebrain abnormalities, we analyzed magnetic resonance imaging (MRI) of MMC patients treated in utero or postnatal. METHODS: Between January 2014 and March 2017, 59 MMC were treated in our hospital. Thirty-seven patients (32 postnatal and 5 intrautero repair) had brain MRI and were enrolled at the study. MRI was analyzed by two experienced neuroradiologists to identify the supra and infratentorial brain abnormalities. RESULTS: A wide range of brain abnormalities was consistently identified in MMC patients. As expected, the most common were hydrocephalus (94.5%) and CM type II (89.1%). Of note, we found high incidence of corpus callosum abnormalities (86.4%), mostly represented by dysplasia (46%). CONCLUSIONS: The data are consistent with the concept that brain abnormalities related to MMC can be both infratentorial and supratentorial, cortical, and subcortical. More studies are needed to correlate these forebrain abnormalities to long-term functional outcome and their prognostic value for these patients.

Surgical approaches for the lateral mesencephalic sulcus.

OBJECTIVE: The brainstem is a compact, delicate structure. The surgeon must have good anatomical knowledge of the safe entry points to safely resect intrinsic lesions. Lesions located at the lateral midbrain surface are better approached through the lateral mesencephalic sulcus (LMS). The goal of this study was to compare the surgical exposure to the LMS provided by the subtemporal (ST) approach and the paramedian and extreme-lateral variants of the supracerebellar infratentorial (SCIT) approach. METHODS: These 3 approaches were used in 10 cadaveric heads. The authors performed measurements of predetermined points by using a neuronavigation system. Areas of microsurgical exposure and angles of the approaches were determined. Statistical analysis was performed to identify significant differences in the respective exposures. RESULTS: The surgical exposure was similar for the different approaches-369.8 ± 70.1 mm2 for the ST; 341.2 ± 71.2 mm2 for the SCIT paramedian variant; and 312.0 ± 79.3 mm2 for the SCIT extreme-lateral variant (p = 0.13). However, the vertical angular exposure was 16.3° ± 3.6° for the ST, 19.4° ± 3.4° for the SCIT paramedian variant, and 25.1° ± 3.3° for the SCIT extreme-lateral variant craniotomy (p < 0.001). The horizontal angular exposure was 45.2° ± 6.3° for the ST, 35.6° ± 2.9° for the SCIT paramedian variant, and 45.5° ± 6.6° for the SCIT extreme-lateral variant opening, presenting no difference between the ST and extreme-lateral variant (p = 0.92), but both were superior to the paramedian variant (p < 0.001). Data are expressed as the mean ± SD. CONCLUSIONS: The extreme-lateral SCIT approach had the smaller area of surgical exposure; however, these differences were not statistically significant. The extreme-lateral SCIT approach presented a wider vertical and horizontal angle to the LMS compared to the other craniotomies. Also, it provides a 90° trajectory to the sulcus that facilitates the intraoperative microsurgical technique.

Evaluation of Postoperative Deficits following Motor Cortex Tumor Resection using Small Craniotomy.

Introduction Surgical treatment of brain tumors in eloquent areas has always been considered a major challenge because removal-related cortical damage can cause serious functional impairment. However, few studies have investigated the association between small craniotomies and the higher risk of incidence of motor deficits and prolonged recovery time. Here, we analyzed neurologic deficits and the prognostic variables after surgery guided by navigation for motor cortex tumors under general anesthesia. Methods This was a prospective study that included 47 patients with tumors in the precentral gyrus. All surgeries were performed with neuronavigation and cortical mapping, with direct electrical stimulation of the motor cortex. We evaluated the prognostic evolution of patients with pre- and postoperative Karnofsky Performance Scale using the Eastern Cooperative Oncology Group scale. Results Complete resection was verified in all 18 cases of metastasis, 13 patients with glioblastoma multiforme, and 5 patients with low-grade gliomas. An analysis of the motor deficits revealed that 11 patients experienced worsening of the deficit on the first day after surgery. Only four patients developed new deficits in the immediate postoperative period, and these improved after 3 weeks. After 3 months, only two patients had deficits that were worse those experienced prior to surgery; both patients had glioblastoma multiforme. Conclusion In our series, motor deficits prior to surgery were the most important factors associated with persistent postoperative deficits. Small craniotomy with navigation associated with intraoperative brain mapping allowed a safe resection and motor preservation in patients with motor cortex brain tumor.

Supracerebellar Infratentorial Variant Approaches to the Intercollicular Safe Entry Zone.

OBJECTIVE: To describe and compare surgical exposure through microsurgical cadaveric dissection of the intercollicular region afforded by the median, paramedian, and extreme-lateral supracerebellar infratentorial (SCIT) approaches. METHODS: Ten cadaveric heads were dissected using SCIT variant approaches. A neuronavigation system was used to determine tridimensional coordinates for the intercollicular zone in each route. The areas of surgical and angular exposure were evaluated and determined by software analysis for each specimen. RESULTS: The median surgical exposure was similar for the different craniotomies: 282.9 ± 72.4 mm2 for the median, 341.2 ± 71.2 mm2 for the paramedian, and 312.0 ± 79.3 mm2 for the extreme-lateral (P = 0.33). The vertical angular exposure to the center of the intercollicular safe entry zone was also similar between the approaches (P = 0.92). On the other hand, the horizontal angular exposure was significantly wider for the median approach (P < 0.001). CONCLUSIONS: All the SCIT approaches warrant a safe route to the quadrigeminal plate. Among the different variants, the median approach had the smallest median surgical area exposure but presented superior results to access the intercollicular safe entry zone.

Traumatic Lumbosacral Spondyloptosis in a Pediatric Patient: Case Report and Literature Review.

A 4-year-old girl was admitted to the emergency department after having been buried beneath a wall. A computed tomography scan revealed anterior grade V L5-S1 spondylolisthesis, and magnetic resonance imaging showed a traumatic rupture of the fibrous annulus of the L5-S1 intervertebral disc and lesion of the anterior longitudinal and yellow ligaments. The patient underwent anterior and posterior fixation. Four months later she was able to walk independently, despite a persistent left foot drop. Additionally, we conducted a literature review on lumbosacral spondyloptosis in the pediatric population published between 1990 and 2017. We found 16 cases, 86.6% of which were male, with a mean patient age of 16 ± 5.05 years. Most patients underwent spine instrumentation. Based on the data reviewed, the neurological status at admission might be a valid predictor of outcome. Pedicle screws are a safe and reliable procedure for stable fixation of the spine in these cases. The removal of screws is discouraged.

High Intra-Abdominal Pressure Secondary to Obesity as a Determining Factor for Ventriculoperitoneal Shunt Malfunction

The ventriculoperitoneal shunt (VPS) is an established treatment for hydrocephalus. The functioning of the system requires a pressure difference between the cranial and abdominal cavities. The VPS can be particularly problematic in patients with increased intra-abdominal pressure (IAP). We report the case of a 16-year-old girl with VPS since she was 2 months old due to hydrocephalus secondary to myelomeningocele. The patient had been asymptomatic ever since, but she sought the emergency service with intermittent headache and vomiting. A non-enhanced brain tomography, a shunt trajectory X-ray and an abdominal ultrasound revealed no cause of system malfunction. In view of the persistent clinical picture, a revision of the shunt was performed, which revealed adequate intraoperative functioning. She returned with the same symptoms two weeks after surgery. The patient was obese (body mass index [BMI]: 48). We hypothesized intermittent valve malfunction due to increased intra-abdominal pressure. She underwent a ventriculoatrial shunt, without intercurrences. In the postoperative period, the patient presented transient tachycardia and was asymptomatic at the 6-month follow-up. Obesity should be considered an important variable for the inadequate functioning of the VPS due to increased IAP and catheter dystocia to the extraperitoneal cavity. Studies have already correlated the IAP with the BMI, which reaches between 8 mm Hg and 12 mm Hg in obese individuals. Therefore, the BMI can be considered during the selection of valve pressure in systems with non-adjustable valves to prevent insufficient drainage. The recognition of obesity as a cause of VPS malfunction is fundamental to avoid unnecessary surgeries and intermittent malfunction of the system.

A derivação ventriculoperitoneal (DVP) é um tratamento estabelecido para a hidrocefalia; contudo, algumas variáveis podem influenciar na eficácia desta modalidade. O funcionamento do sistema requer uma diferença de pressão entre as cavidades craniana e abdominal. A DVP pode ser particularmente problemática em pacientes com aumento da pressão intra-abdominal (PIA). Neste artigo, relatamos o caso de uma paciente do sexo feminino, de 16 anos, portadora de DVP desde os 2 meses de idade por hidrocefalia secundária a mielomeningocele. Desde então assintomática, procurou o pronto-socorro com queixa de cefaleia e vômitos intermitentes. Uma tomografia de crânio sem contraste, um raio X (RX) do trajeto do cateter distal, e uma ultrassonografia (USG) abdominal não evidenciaram a causa do mau funcionamento do sistema. Diante do quadro persistente, realizou-se uma revisão da derivação, que mostrou funcionamento adequado no período intraoperatório. A paciente retornou com os mesmos sintomas duas semanas após a cirurgia. A paciente era obesa (índice de massa corporal [IMC]: 48). Aventou-se possível funcionamento intermitente da válvula pelo aumento da PIA. A paciente foi submetida a uma derivação ventrículo-atrial, que foi realizada sem intercorrências. No pós-operatório, ela apresentou quadro transitório de taquicardia, e não apresentou sintomas no acompanhamento feito depois de 6 meses. A obesidade deve ser considerada uma variável importante para o funcionamento inadequado da DVP, pelo aumento da PIA e pela associação com distocia do cateter para a cavidade extraperitoneal. Estudos já correlacionaram a PIA com o IMC, que pode atingir entre8 mm Hg e 12 mm Hg em obesos. Logo, o IMC pode ser considerado na seleção da pressão da válvula em sistemas com válvulas não ajustáveis, para prevenir a drenagem insuficiente. O reconhecimento da obesidade de risco para o mau funcionamento da DVP é fundamental para evitar cirurgias desnecessárias e o mau funcionamento intermitente do sistema.

Post-traumatic carotid-cavernous fistula in a pediatric patient: a case-based literature review.

BACKGROUND: Carotid-cavernous fistula (CCF) is a shunt between the carotid artery and the cavernous sinus. Traumatic CCFs are diagnosed in 0.2% of head traumas being only 4.6% of the pediatric population. Classified by Barrow in 1985, type A CCF is the most frequent, occurring in 75% of cases. Type A is characterized by direct and high-flow CCF that generally can occur as a result of traumatic injury or rupture of an intracavernous aneurysm. CASE PRESENTATION: The subject was an 8-year-old boy with penetrating trauma to his left eye. During the initial evaluation, a computed tomography (CT) scan was unremarkable, and after relief of symptoms, the patient was discharged. Seven days later, he developed grade I proptosis, conjunctival chemosis, ophthalmoplegia (III, IV, and VI cranial nerve palsies), and left-sided ptosis and mydriasis. Arteriography confirmed a post-traumatic CCF, and the patient was treated with an endovascular detachable balloon. CONCLUSION: CCF should be suspected in craniofacial traumas with ocular symptoms. The presence of a skull base fracture on CT is a poor predictor of CCF associated with head trauma. Early diagnosis and treatment can prevent permanent neurological deficits and unfavorable outcomes.

Cholangiocarcinoma Seeding along a Ventriculoperitoneal Shunt Catheter: A Rare Initial Manifestation

Cholangiocarcinoma accounts for less than 2% of all malignant neoplasms. Its cutaneous metastases are extremely rare, accounting for 0.0002% of all cases. The ventriculoperitoneal (VP) shunt has been considered a potential route for tumor dissemination. This type of tumor proliferation has been well documented in patients with intra-abdominal metastases secondary to brain tumors and, less frequently, brain metastases secondary to intra-abdominal tumors. In spite of that, there are few reports of cutaneous metastases along the VP shunt catheter trajectory. We present the case of a cholangiocarcinoma cutaneous metastasis at the VP shunt trajectory as the first clinical sign of this type of tumor. The patient had undergone the shunt insertion 4 years earlier due to a hydrocephaly secondary to a subarachnoid hemorrhage. After diagnosis, she was referred to chemotherapy and oncology follow-up. In the literature, 30 cases of cutaneous metastasis of cholangiocarcinoma were reported. Dissemination has been described involving percutaneous biliary drainage and distant sites. However, the case presented in this study alerts us about the possibility of cutaneous metastasis of intra-abdominal tumors in the trajectory of VP shunt catheters.

O colangiocarcinoma representa menos de 2% das neoplasias malignas. Sua metástase cutânea é extremamente rara, representando 0.0002% dos casos. A derivação ventrículo-peritoneal (DVP) tem sido considerada uma via potencial para a disseminação dos tumores. Essa propagação tumoral já foi bem descrita em pacientes com tumores do sistema nervoso central e metástases intra-abdominais e, menos frequentemente, em metástases cerebrais secundárias a tumores intra-abdominais. Apesar disso, a presença de metástases cutâneas ao longo da trajetória do cateter de DVP é pouco relatada. Apresentamos o caso de uma paciente portadora de DVP há quatro anos devido a uma hidrocefalia secundária à hemorragia subaracnoide e que apresentou metástases cutâneas de colangiocarcinoma na trajetória do cateter de derivação como o primeiro sinal clínico do tumor. Após o diagnóstico, ela foi encaminhada para quimioterapia e acompanhamento com oncologia. Na literatura, foram relatados 30 casos de metástases cutâneas de colangiocarcinoma. Em geral, a disseminação pode envolver locais distantes ou cateteres de drenagem biliar percutânea. No entanto, o caso descrito neste estudo nos alerta sobre a possibilidade de metástases cutâneas de tumores intra-abdominais na trajetória de cateteres de DVP.

Hydrocephalus: a rare initial manifestation of sporadic intramedullary hemangioblastoma : Intramedullary hemangioblastoma presenting as hydrocephalus.

BACKGROUND: Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. CASE PRESENTATION: A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. CONCLUSION: Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

Cerebellopontine angle empyema after lumbar disc herniation surgery.

BACKGROUND: Lumbar discectomy is still one of the most common spinal surgeries performed today. Nevertheless, there are few publications considering severe complications. CASE DESCRIPTION: We report a case with severe complication, without any previous report, a brain empyema after cerebrospinal fluid leak with good outcome afterword. A 45 years old man, returned six days after surgery, presenting cerebrospinal fluid leak, which after clinical and laboratory deterioration was reoperated. In the 14th postoperative facing worsening level of consciousness was diagnosed empyema in cerebellopontine angle and hydrocephalus underwent emergency surgery. Evolved with hemiplegia in the first postoperative attributed to vasculitis, completely recovered after 5 days. CONCLUSIONS: Although infrequent, lumbar discectomy is subject to severe complications, which as the case presented, can be dramatic. In surgeries must be careful to avoid cerebrospinal fluid leak e infectious complications.