Experience of a reference center on ventriculo-gallbladder shunt as an alternative treatment for peritoneal failure in children.

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.

Congenital orbital teratoma: a rare case with intracranial extension.

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.

Intracranial Tumors in the First Year of Life.

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.

Identifying abusive head trauma and its mimics: diagnostic nuances.

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.

Hydrocephalus and occipital encephaloceles: presentation of a series and review of the literature.

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.

Reviewing the prognostic factors in myelomeningocele.

OBJECTIVE: The goal of this study was to analyze the factors that have an impact on morbidity and mortality in patients with myelomeningocele (MMC). METHODS: A retrospective cohort study was conducted to analyze factors associated with MMC that influence the morbidity and mortality of the disease. Data were collected from medical records of children who underwent the primary repair of MMC at the Fernandes Figueira Institute-Oswaldo Cruz Foundation (IFF-Fiocruz) between January 1995 and January 2015, with a minimum follow-up of 1 year. The following variables were analyzed: demographic characteristics (gestational age, sex, and birth weight); clinical features (head circumference at birth, anatomical and functional levels of MMC, hydrocephalus, symptomatic Chiari malformation type II, neurogenic bladder, and urinary tract infection [UTI]); and surgical details such as timing of repair of MMC, age at first shunt placement, shunt surgery modality (elective or emergency), concurrent surgery (correction of MMC and shunt insertion in the same surgical procedure), incidence and cause of shunt dysfunction, use of external ventricular drain, transfontanelle puncture, surgical wound complications prior to shunting, and endoscopic treatment of hydrocephalus. RESULTS: A total of 231 patients with MMC were included in the analysis. Patients were followed for periods ranging from 1 to 20 years, with a mean of 6.9 years. The frequency of shunt placement was observed mainly among patients with MMC at the highest spinal levels (p < 0.01). The main causes of morbidity and mortality in patients with MMC were shunt failures, diagnosed in 91 of 193 cases (47.2%) of hydrocephalus, and repeated UTIs, in 129 of 231 cases (55.8%) of MMC; these were the main causes of hospitalization and death. Head circumference ≥ 38 cm at birth was found to be a significant risk factor for shunt revision (p < 0.001; 95% CI 1.092-1.354). Also, the lumbar functional level of MMC was associated with less revision than upper levels (p < 0.014; 95% CI 0.143-0.805). There was a significant association between recurrent UTI and thoracic functional level. CONCLUSIONS: Macrocephaly at birth and higher levels of the defect have an impact on worse outcome and, therefore, are a challenge to the daily practice of pediatric neurosurgery.

Results and limits of posterior cranial vault expansion by osteotomy and internal distractors.

Background: Expanding the posterior cranial vault has become a common procedure in the treatment of complex craniosynostosis. Several techniques are available to remodel the posterior vault. Aim of this study was to analyze the posterior vault distraction osteogenesis. Methods: Between 2011 and 2014, 21 children (12 boys) were operated on for a posterior distraction of the cranial vault. The mean age was 8.6 months (minimum, 3 months; maximum, 15 years). Thirteen patients presented a craniofacial syndrome. Five had already been operated on (two anterior cranial surgery, two suboccipital decompression, and one craniotomy for sagittal synostosis). Raised intracranial pressure (ICP) was present in 6 patients. Seven patients had symptomatic cerebellar tonsils herniation (TH). Results: In 17 children, 2 lateral distractors were placed, in 3 a 3rd medial distractor was placed, and in 1 child 4 distractors were implanted. Volumetric analysis based on computed tomography showed a mean increase of volume of 13.9% 117 days later. After the distraction, symptoms related to raised ICP or TH were improved in all patients, however, radiologically TH was improved at the last follow-up in 54% of the cases. Conclusion: Posterior cranial vault distraction is an efficacious technique to enlarge the posterior skull vault and treat increased ICP. Moreover, it appears to be efficacious in treating TH-related symptoms.