The Geneva and Lausanne (French-speaking Switzerland) experience: in favor of the transsphenoidal approach when feasible

Background: The authors present their current attitude towards management of craniopharyngiomas in children. Radical surgery cannot be performed when one is not sure about its potential danger to the visual pathways and the hypothalamus. Methods: Most of the surgeries that have been performed in our institution via an intracranial approach were incomplete and followed by radiotherapy, in the last 10 years with stereotactic conformational radiotherapy. Discussion: Considering our past and present experience concerning the transphenoidal approach for treatment of craniopharyngiomas, we make a plea for the consideration of this approach in children whenever feasible (around 25% of the cases in children older than 5 years). Craniopharyngiomas that can be removed by this approach represent a milder form of the disease and the results concerning the visual, hypothalamic functions and quality of life are significantly better than that obtained via an intracranial approach. When the craniopharyngioma cannot be removed by this approach because of tight hypothalamic adherences, the technique of cystosphenoidostomy with a custom-made shunt plus adjuvant stereotactic conformational radiotherapy is an alternative for its total remova

A 10-year experience in paediatric spontaneous cerebral hemorrhage: which children with headache need more than a clinical examination?

INTRODUCTION: When a child is seen in a clinic with a headache, stroke is certainly not the first on the list of differential diagnoses. In western countries, stroke is typically associated with adults and the elderly. Although rare, haemorrhagic strokes are not exceptional in the paediatric population, as their incidence is around 1/100 000/year. Prompt diagnosis is essential, since delayed treatment may lead to disastrous prognosis in these children. MATERIALS AND METHODS: This is a retrospective review of paediatric cases with spontaneous cerebral haemorrhage that presented in two university hospitals in the last ten years. The experience of these primary and tertiary referral centres comprises 22 consecutive cases that are analysed according to aetiology, presenting symptoms, treatment and outcome. RESULTS: 77% of the children diagnosed with haemorrhagic stroke presented with headaches. 41% of them had a sudden onset, while 9% developed headaches over a period of hours to weeks. While 9% presented only with headaches, the majority had either subtle (diplopia, balance problems) or obvious (focal deficits, unilateral weakness and decreased level of consciousness) concomitant neurological signs. 55% had an arteriovenous malformation (AVM), 18% had an aneurysm and 14% had a cavernous malformation. In 14% the aetiology could not be determined. The majority of haemorrhages (82%) were supratentorial, while 18% bled into the posterior fossa. All children underwent an emergency cerebral CT scan followed by specific investigations. The treatment was dependent on the aetiology as well as the mass effect of the haematoma. In 23% an emergent evacuation of the haematoma was performed. Two children (9%) died, and 75% had a favourable clinical outcome. CONCLUSION: Headaches in children are a common problem, and a small minority may reveal an intracranial haemorrhage with poor prognosis if not treated promptly. Although characterisation of headaches is more difficult in a paediatric population, sudden, unusual or intense headaches should lead to imaging work-up. Any neurological finding, even one as subtle as hemianopsia or dysmetria, should alarm the physician and should be followed by emergency imaging investigation. If the cerebral CT reveals a haemorrhage, the child should be referred immediately to a neurosurgical referral centre without further investigation. The outcome is grim for children presenting in coma with fixed, dilated pupils. The long-term result overall for children after spontaneous intracranial haemorrhage is not dismal and depends critically on specialised management

Occipital extra- and intracranial lipoencephalocele associated with tectocerebellar dysraphia

Objects: Tectocerebellar dysraphism was first described by Padget and Lindenberg, together with occipital encephaloceles, cerebellar midline defects, tectal beaking, posterior kinked and displaced brainstem, and inverted cerebellum. We present a unique case of lipoencephalocele associated with tectocerebellar dysraphia and discuss the etiologies of both pathologies. The importance of the developmental comprehension of posterior fossa malformations is stressed. Case report: A 9-month-old girl in good health was referred for a huge occipital mass. On local examination, the patient had a rounded mass about 10×12cm in size, covered by alopecic skin, with a small eccentric dimple without any discharge. Magnetic resonance imaging revealed a lipoencephalocele with an intra- and an extracranial part, associated with tectocerebellar dysraphia with no aspect of inverted cerebellum as originally described in the tectocerebellar dysraphia. Resection of the extracranial part of the lipoma was performed for esthetic reasons. The intracranial part remained in situ. Conclusion: The association of lipoencephalocele and tectocerebellar dysraphia is extremely rare. The functional prognosis seems to be less severe for this combination than for the isolated tectocerebellar dysraphia. This association favors the dysraphic theory for the embryogenesis of the lipoma. It seems safe to restrict the surgery of this bizarre malformation to its external par

Meningeal solitary fibrous tumour in a child

Introduction: Meningeal solitary fibrous tumour is a relatively recent pathological entity that has rarely been described in children. With radiological techniques, it cannot be distinguished from meningiomas, and the diagnosis has to be confirmed histologically. Case report: We discuss the possible histogenesis of this tumour and the need for recognizing this lesion as a separate entity. We report the case of a 12-year-old boy who developed a meningeal solitary fibrous tumour; the main clinical symptoms were progressive headaches for a long period and recent transient hemiparesis. Conclusion: This child presents an uneventful evolution without additional therapy 3.5years after surger

Frontosphenoidal synostosis: a rare cause of unilateral anterior plagiocephaly

Introduction: When a child walks in the clinic with a unilateral frontal flattening, it is usually associated in our minds with unilateral coronal synostosis. While the latter might be the most common cause of anterior plagiocephaly, it is not the only one. A patent coronal suture will force us to consider other etiologies, such as deformational plagiocephaly, or synostosis of another suture. To understand the mechanisms underlying this malformation, the development and growth of the skull base must be considered. Materials and methods: There have been few reports in the literature of isolated frontosphenoidal suture fusion, and we would like to report a series of five cases, as the recognition of this entity is important for its treatment. Conclusion: Frontosphenoidal synostosis must be searched in the absence of a coronal synostosis in a child with anterior unilateral plagiocephaly, and treated surgicall

Third ventriculostomy vs ventriculoperitoneal shunt in pediatric obstructive hydrocephalus: results from a Swiss series and literature review

Introduction: Few series compare endoscopic third ventriculostomies (ETV) and ventriculoperitoneal shunts (VPS). To avoid the complications after a shunt insertion, there is an increased tendency to perform a third ventriculostomy. We reviewed all pediatric patients operated in the French-speaking part of Switzerland for a newly diagnosed obstructive hydrocephalus since 1992 and compared the outcome of patients who benefited from ETV to the outcome of patients who benefited from VPS. There were 24 ETV and 31 VPS. Discussion: At 5years of follow-up, the failure rate of ETV was 26%, as compared to 42% for the VPS group. This trend is also found in the pediatric series published since 1990 (27 peer-reviewed articles analyzed). Conclusion: In accordance to this trend, although a statistical difference cannot be assessed, we believe that ETV should be the procedure of choice in pediatric obstructive hydrocephalu

Introduction of a urodynamic score to detect pre- and postoperative neurological deficits in children with a primary tethered cord

Object: An increasing number of asymptomatic children are diagnosed with occult spinal dysraphism, raising the question of their optimal management. Urodynamic study (UDS) is the most reliable method of detecting neuro-urological abnormalities in these children. The rate of postoperative retethering ranges from 10 to 20% and is not always immediately clinically significant. The aim of this prospective study was to develop a reliable method that could be used in the preoperative assessment and postoperative follow-up of children with a tethered cord syndrome (TCS). Methods: From 1989 to 1997, 15 children underwent spinal cord untethering for TCS. Preoperatively, patients were assessed with MRI and UDS. Postoperative UDS were repeated at 6- to 12-month intervals. Four UDS parameters were identified, graded, and added to obtain a UDS score. A group of 38 children without dysraphic condition was used as control and allowed the calculation of a normal score. Conclusions: There was a statistically significant difference in the preoperative UDS scores between the control group and the study group (p<0.001). Postoperatively, there was a statistically significant improvement (p<0.001) in UDS scores. UDS score is a reliable tool for identifying and quantifying neuro-urological disorders in patients with TCS. Postoperatively, this score was useful in the early diagnosis of spinal cord retetherin