The role of early intra-operative MRI in partial resection of optic pathway/hypothalamic gliomas in children.

INTRODUCTION: Optic pathway/hypothalamic gliomas (OPHGs) are generally benign but situated in an exquisitely sensitive brain region. They follow an unpredictable course and are usually impossible to resect completely. We present a case series of 10 patients who underwent surgery for OPHGs with the aid of intra-operative MRI (ioMRI). The impact of ioMRI on OPHG resection is presented, and a role for ioMRI in partial resection is discussed. METHODS: Ten patients with OPHGs managed surgically utilising ioMRI at Alder Hey Children's Hospital between 2010 and 2013 were retrospectively identified. Demographic and relevant clinical data were obtained. MRI was used to estimate tumour volume pre-operatively and post-resection. If ioMRI demonstrated that further resection was possible, second-look surgery, at the discretion of the operating surgeon, was performed, followed by post-operative imaging to establish the final status of resection. Tumour volume was estimated for each MR image using the MRIcron software package. RESULTS: Control of tumour progression was achieved in all patients. Seven patients had, on table, second-look surgery with significant further tumour resection following ioMRI without any surgically related mortality or morbidity. The median additional quantity of tumour removed following second-look surgery, as a percentage of the initial total volume, was 27.79% (range 11.2-59.2%). The final tumour volume remaining with second-look surgery was 23.96 vs. 33.21% without (p = 0.1). CONCLUSIONS: OPHGs are technically difficult to resect due to their eloquent location, making them suitable for debulking resection only. IoMRI allows surgical goals to be reassessed intra-operatively following primary resection. Second-look surgery can be performed if possible and necessary and allows significant quantities of extra tumour to be resected safely. Although the clinical significance of additional tumour resection is not yet clear, we suggest that ioMRI is a safe and useful additional tool, to be combined with advanced neuronavigation techniques for partial tumour resection.

Endoscopic Third Ventriculostomy in Children with Myelomeningocele: A Case Series.

Hydrocephalus develops in up to 80-90% of children with myelomeningocele (MM) after closure of the defect. Traditionally, ventriculoperitoneal shunts have been used to manage hydrocephalus in these patients. A role for endoscopic third ventriculostomy (ETV) in MM has provoked much debate, principally due to anatomical variants described, which may complicate the procedure. We present 7 cases of children with MM and hydrocephalus undergoing a total of 10 ETV procedures. All patients demonstrated clinical improvement (in acute/subacute cases) or stabilization (in chronic cases). Three patients requiring a second ETV have shown clinical stability and renewed radiological evidence of functioning ventriculostomies in follow-up since reintervention. ETV can be used, albeit cautiously, in selected cases of hydrocephalus associated with MM. However, the frequency with which anatomical variation is encountered and the difficulty of the assessment of success make the procedure more challenging than usual.

MRI findings of intracranial anomalies associated with cephalocele--a case series.

INTRODUCTION: Cephalocele is a relatively rare cranial dysraphism characterised by herniation of intracranial structures through the skull. Surgical management is primarily necessary where a risk of infection through communication of the lesion with the intracranial space exists, a risk of rupture, or for cosmetic purposes. Cephalocele is often associated with venous anomalies such as vertical embryonic positioning of the straight sinus, splitting of the superior sagittal sinus, vein of Galen elongation, along with tenting of the tentorium [Morioka et al. Childs Nerv Syst 25:309-315, 2009] PATIENTS: Here, we report four cases of cephalocele with pre-operative MRI imaging retrospectively studied, demonstrating associated venous anomalies. Three of these patients went on to have uncomplicated, corrective surgery, while one was managed conservatively. RESULTS: All four cases demonstrated the main venous drainage going through a persistent falcine sinus to drain into the superior sagittal sinus. Upward tenting of the tentorium was observed in three cases (cases 1, 3 and 4). Two of our cases demonstrated other venous anomalies frequently reported in the literature, namely splitting of the superior sagittal sinus and absence of the transverse sinus (case 1) and communication of the cephalocele with the superior sagittal sinus and absence of the straight sinus (case 2). CONCLUSION: The association between cephalocele and venous anomalies suggests that pre-operative MRI should be mandatory for a full evaluation of a suspicious midline cranial lesion in order to evaluate the safety of corrective surgery.

Intracystic gadolinium-enhanced MRI in the evaluation of residual giant-cystic craniopharyngiomas in children: report of four cases.

Residual giant-cystic craniopharyngiomas are amenable to intracavitary bleomycin treatment. Radiologic identification of potential cyst leaks is of paramount for treatment decisions. This report describes our experience in the use of intracystic Gadolinium (Gd)-enhanced MR imaging to determine potential communications between the tumoral cysts and other intra-axial spaces in 4 pediatric patients with residual giant-cystic craniopharyngiomas in whom intracavitary bleomycin treatment was planned after the injection of .1-.2 mL of gadopentetate dimeglumine (Gd-DTPA). In three cases no leaks were found. In one case, whose previous water-soluble iodinated contrast-enhanced CT cystography was negative for leaks, intracystic Gd-enhanced MR showed intraventricular Gd enhancement. We conclude that MR imaging after intracystic administration of Gd-based contrast paramagnetic agents is useful in the detection of potential leaks in cases of giant residual craniopharyngiomas.

Fascitis craneal: caso clínico y revisión de la literatura / No disponible

La fascitis craneal es una lesión miofibroblástica benigna infrecuente, de rápido crecimiento, localizada en el cráneo y de aparición preferente en niños durante el primer año de vida. Histológicamente es similar a la fascitis nodular o a la fascitis seudosarcomatosa. Puede mimetizar patologías más agresivas, como histiocitosis o sarcomas, por su rápido crecimiento de forma nodular en el tejido subcutáneo. La resección completa se considera curativa y tiene bajo riesgo de metástasis o de malignización. En el presente trabajo se presenta el caso de un varón de 4 años con fascitis en tejido celular subcutáneo craneal con erosión de la tabla externa y reacción perióstica circundante, respetando las meninges. El objetivo de este trabajo es destacar la ausencia de necesidad de tratamiento adyuvante con radioterapia o quimioterapia. Se lleva a cabo, además, una revisión de la literatura (AU)

[Cranial fasciitis: a case report and review of the literature]. / Fascitis craneal: caso clínico y revisión de la literatura.

Cranial fasciitis is an uncommon, rapidly-growing, benign, non-tumoural, myofibroblastic lesion of the skull, found mainly among young children in their first year of life. It is histologically similar to nodular fasciitis and pseudosarcomatous fasciitis. It may mimic more aggressive pathologies, such as sarcomatosis or histiocytosis, due to its rapid, nodular growth in subcutaneous tissue. Complete resection is considered curative and, therefore, entails a low risk of metastases or malignant recurrences. We present the clinical, radiological and pathological findings in a 4-year-old boy with cranial fasciitis in the deep, subcutaneous, soft tissue, with erosion of the outer table of the cranium, which also produced periosteal reaction, while respecting the inner table and meninges. The objective of this article is to highlight the absence of radiotherapeutic or chemotherapeutic adjuvant treatment. In addition, an exhaustive review of the literature is also presented.

Foreign body granuloma secondary to ventriculo-peritoneal shunt: a rare scenario with a new insight.

Ventriculo-peritoneal shunts are used extensively for the management of hydrocephalus and frequently present with complications such as shunt blockage and infection. Cerebrospinal fluid (CSF) eosinophilia and allergic responses to the shunt itself are rare, poorly understood but increasingly recognised complications. Here, the authors describe a child who required multiple shunt revision surgeries due to extensive scalp tenderness overlying the shunt tubing and persistent severe headaches despite having a normal working shunt and no CSF infection or eosinophilia. Histological investigation of excised tissue during the last shunt revision demonstrated fibrosis with scar tissue and chronic inflammatory infiltrate with foreign body giant cells and few abortive granulomata. This was felt to represent a foreign body reaction to the shunt. A hypoallergenic 'extracted' shunt was trialled (extracted Delta® valve and extracted ventricular and peritoneal catheters; Medtronic) and the child has had no further shunt revisions and is currently asymptomatic 1 year after the insertion.