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Meningiomas with haemorrhagic onset

SummaryClassically meningiomas present and evolve with a progressive course. Meningiomas manifest themselves mainly in middle and old age when the incidence of strokes is higher. The authors report three instances of meningioma with acute haemorrhagic onset. The clinical picture of our cases consisted of subarachnoid bleeding, subdural heamatoma and intracerebral haemorrhage respectively. The diagnostic difficulties encountered when assessing these patients are discussed. The current literature regarding meningioma associated with haemorrhage is reviewed. One of our cases seems to be the fisrt reported case of subarachnoid haemorrhage originating from a meningioma of the petrous bone.

Macrocephaly, dystonia, and bilateral temporal arachnoid cysts: glutaric aciduria type 1.

Two siblings presented with macrocephaly, psychomotor delay, and progressive dystonia. The initial diagnosis was of hydrocephalus and bilateral temporal cerebrospinal fluid collections. Following ventriculoperitoneal shunting, the patients showed only modest neurological improvement. Metabolic investigations performed later in the course of the disease disclosed increased levels of glutaric acid in the urine and decreased levels of serum carnitine, which were confirmatory of glutaric aciduria type 1. The association of macrocephaly, dystonia, and bilateral temporal arachnoid cysts, shown either by computed tomography or magnetic resonance imaging, seems to be diagnostic of glutaric aciduria type 1. The authors report these two cases as they think they might be of interest to neurosurgeons.

Pediatric Creutzfeldt-Jakob disease: probable transmission by a dural graft

A 10-year-old boy underwent a posterior fossa craniectomy for removal of a grade 2 cerebellar astrocytoma. Dural closure was achieved by the placement of a dural graft. Eight years later the patient developed dementia and myoclonus. Electroencephalography demonstrated generalized slow activity that evolved into a pattern of periodic tripahsic waves. Computed tomography scan and magnetic resonance imaging were unremarkable. Brain biopsy confirmed spongiform encephalopathy of the Creutzfeldt-Jakob type. In the light of previous reports of four similar occurrences, and of our own experience with two further cases of this disease, we believe that the cadaveric dura was the source of transmission of Creutzfeld-Jakob disease in our patient. The authors remark the importance of the awareness of this late complication of dural substitutes, both for the diagnosis of possible future cases and for taking preventive measures to stop the spread of the disease.

Atlanto-Axial Rotatory Subluxation in Children: Early Management

Summary. Atlanto-axial rotatory subluxation (AARS) is an uncommon condition involving dislocation and abnormal fixation of the atlas on the axis. AARS is difficult to diagnose and if improperly treated it may lead to permanent neck deformity. We report a retrospective series of patients diagnosed with AARS seen during the last five years. The children presented with neck pain, head tilt, and reduction in neck mobility. In three of the cases the condition was secondary to cervical trauma and in the fourth it was associated with otitis (Grisel syndrome). The diagnosis was established by three-dimensional computerised tomography. In three cases, the atlanto-axial fixation was cured after cervical immobilisation and physiotherapy. A posterior cervical fusion was required in the remaining case. Early detection and intensive conservative treatment constitutes the mainstay of management of AARS. Surgery is reserved for cases with irreducible or recurrent subluxation.

Ventriculopleural shunting with new technology valves

Abstract Ventriculoperitoneal shunting constitutes the standard procedure for draining cerebrospinal fluid (CSF) in children with hydrocephalus. Ventriculoatrial and ventriculopleural shunting are alternative methods of CSF drainage, which have gained less acceptance. Ventriculopleural shunts are seldom used owing to justified fears of pneumothorax and symptomatic effusions of CSF. The addition of an antisiphon device to standard shunt systems seems to have prevented CSF pleural effusion. From 1988 to 1998, we treated each of six hydrocephalic children with a ventriculopleural shunt. In five cases we used new-technology valves designed to prevent the effects of siphoning with current differential pressure valves. Peritoneal adhesions, recent peritonitis, ascites, and obstruction of a previous ventriculoatrial shunt were the indications for pleural shunting. After a mean follow-up period of 2.5 years all shunts were functioning adequately. Only one patient showed transient symptoms of CSF overdrainage, which were corrected by up-grading the valve setting with the magnet. A late death was unrelated to the pleural shunting procedure. The use of valves of a new design designed to prevent overdrainage seems to account for the satisfactory outcomes observed in this series. We suggest that ventriculopleural shunting should be considered as the preferred alternative to peritoneal drainage in children with intra-abdominal adhesions or with a history of recent peritoneal infection.

Posttraumatic intradiploic arachnoid cyst of the posterior fossa

Abstract We report the case of a 5-year-old girl with an enlarging suboccipital mass, a posttraumatic intraosseous arachnoid cyst. Diagnostic work-up revealed that the lesion consisted of an intradiploic arachnoid cyst and an extra-axial occipital pouch that communicated by way of an osseous and dural defect. Surgical repair was undertaken with good results. A search of the current literature has shown only seven previous reports of leptomeningeal cysts situated at the occipital bones, most of them the result of an antecedent skull fracture. A pathogenetic hypothesis is presented comparing the growth of arachnoid intraosseous cysts and the development of meningocencephaloceles.

Development of a middle fossa arachnoid cyst A theory on its pathogenesis

Abstract The progression of congenital arachnoid cysts has seldom been documented. We report the case of a child who was diagnosed with arrested hydrocephalus at the age of 13 months. Neuroimaging studies performed when the girl was 22 months old showed the appearance of an arachnoid cyst in the right middle fossa, while the previously enlarged ventricles seemed to have decreased in size. To the best of our knowledge, the paradoxical expansion of an arachnoid pouch following a reduction in the size of the ventricular system has not previously been documented. We advance the hypothesis that the development of some cases of arachnoid cyst might be pathogenically related to impaired CSF dynamics associated with pre-existing hydrocephalus. We also briefly review the pertinent literature on the formation and evolution of congenital cerebral arachnoid cysts.

Aplasia cutis congenita of the scalp.

Abstract Case report. We report the case of a newborn baby who presented with scalp aplasia cutis congenita and an underlying skull defect. Discussion.We discuss the diverse options for the management of this condition and the feasibility of early surgical repair in the light of the current literature. We also comment on the possible role of benzodiazepines in the genesis of this lesion.

Raised intracranial pressure in minimal forms of craniosynostosis

Abstract Most cases of craniosynostosis are diagnosed during early infancy, but occasionally craniosynostosis evolves with minimal cranial involvement and goes unnoticed until late childhood. Seemingly these mild forms of craniosynostosis cause few, if any, symptoms of neurological involvement. We describe the cases of a 9-year-old girl and a 6-year-old boy who presented with evident signs of raised intracranial pressure (ICP), together with a negligible skull deformity. We have termed these cases as occult craniosynostosis. Differential diagnosis in our patients was established against known causes of benign intracranial hypertension. Bilateral expanding craniotomies afforded total relief from the symptoms and signs of raised ICP. Neurosurgeons treating children with symptoms and signs of benign intracranial hypertension should be aware of the possibility of minimal forms of craniosynostosis evolving with marked manifestations of raised ICP.

Bilateral Temporal Arachnoid Cysts in Neurofibromatosis

The occurrence of bilateral temporal arachnoid cysts has been considered as a rare event. Unilateral arachnoid pouches have been reported in a few instances associated with neurofibromatosis. The authors describe a 5-year-old girl with bilateral temporal arachnoid cysts who presented obvious stigmata of von Recklinghausens disease. To the best of our knowledge, this is the first time that this association has been described in the literature. (J Child Neurol 1993;8:383-385).