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A case of cerebrospinal fluid leakage from a meningoencephalic herniation of the temporal bone is presented. Computed tomography and magnetic resonance imaging were useful for a diagnosis of meningoencephalic herniation. After surgical treatment, no recurrence was observed during a 6-month follow-up period.
We present the case of a patient seen in the Emergency Department (ED) at the height of enteroviral meningitis season with the chief complaint of the worst headache of his life. He was subsequently found to have pneumococcal meningitis as the result of an encephalocele located within the left ethmoid sinus. The key features of the patient's past medical history, the steps to diagnosis, and a discussion of this exceedingly rare entity are detailed.
An 8-year-old Japanese boy had Sakoda complex (basal encephalomeningocele, agenesis of the corpus callosum, and cleft lip and/or palate) associated with bilateral anophthalmia, dysgenesis of the cerebral cortex, severe mental retardation, and intractable epilepsy as core symptoms and hemiparesis, microcephalus, short stature, and hemivertebra. Tada and Nakamura described the first case of the Sakoda complex associated with bilateral anophthalmia, cortical dysgenesis, neonatal-onset seizures, and severe mental retardation. Fourteen patients with the Sakoda complex with or without ocular dysplasia were reviewed. It is proposed that these cases belong to a clinical entity that is distinguishable from the remaining 12 patients because of bilateral anophthalmia, cortical dysgenesis, and its resulting severe mental retardation and intractable epilepsy. There is a possibility that these two cases are one severe end of certain spectrum disorders in which certain common gene(s) might be implicated.
Thirteen infants with congenital brachial plexus palsy (eight with upper, five with upper and lower) were monitored by magnetic resonance imaging (the first performed between 7 and 41 days of age and the second at 3 months of age), electromyography (the first performed between 27 and 50 days and the second at 3 months), and the muscle scoring system of the Hospital for Sick Children (at 3, 6, and 9 months of age). The findings were evaluated with respect to the clinical status of the patients at 12 months of age. Magnetic resonance imaging, which could be performed readily even in the neonatal period, revealed pseudomeningoceles in two of the five patients with a poor prognosis (in all planes even in the early days after birth) and in two of the eight patients with a good prognosis (more easily visible at 3 months of age). Electromyography implied root avulsion in three of five patients with a poor prognosis. Electromyography can be of great value for patients with a poor prognosis and root avulsion but may underestimate the severity. The muscle scoring system (Hospital for Sick Children) was determined to be the most predictive method for prognosis.
Fibrous dysplasia is an uncommon benign disorder of unknown aetiology. It most likely represents a disorder of normal bone development. As it may occasionally involve the temporal bone, and encroach on the middle cranial fossa, it is of interest to the neurosurgeon. Fibrous dysplasia of the cystic variety is very uncommon. We report a case of a large cystic fibrous dysplasia involving predominantly the squamous temporal bone. The radiology and histology are discussed, and its differentiation from other lesions.
One-stage correction of fronto-ethmoidal meningo-encephaloceles and related stigmata, via an orbito-cranial approach, is recommended. A bifrontal craniotomy is only required when simultaneous correction of hypertelorism is to be undertaken. A combined intra- and extracranial approach is essential. The possibility of a high relapse rate for repaired fronto-ethmoidal meningo-encephaloceles, together with the possibility of prolonged postsurgical cerebrospinal fluid leakage, meningitis and other complications is invited when either a transcranial bifrontal craniotomy surgical approach, or an extracranial approach via the facial lesion, is undertaken alone. Modification of existing craniofacial surgical approaches in order to avoid a frontal craniotomy, allowed for good repair of the encephalocele together with significant benefits in terms of simplification of the surgical procedure, operating time, blood loss, frontal lobe retraction and complications.
A patient is presented who was born with a large swelling on the left side of his face. This was removed by a paediatric surgeon at six weeks of age, and the histology was that of an encephalocele. Based on our findings at a later procedure to correct the child's facial asymmetry resulting from the congenital deformity, we concluded that this was a spheno-maxillary meningo-encephalocele. We have been able to find only one other such meningo-encephalocele reported in the literature.
INTRODUCTION: Hydrocephalus, cerebrospinal fluid (CSF) leak, pseudomeningocele and CSF infection are potential complications related to surgical treatment of posterior fossa tumors. The objectives of this study were to review the incidence of such complications and to identify contributing factors related to them. MATERIAL AND METHODS: This study is based on a retrospective review of the medical records of 71 consecutive patients who underwent posterior fossa surgery for a tumor between the period January 1997 and December 2001. Postoperative hydrocephalus was defined as enlargement of the ventricles and the subsequent clinical worsening requiring surgical treatment. Criteria for CSF leakage were:observed leak of CSF through the wound, rhinorrhea or otorrhea. Pseudomeningocele was determined when there was a large epidural CSF collection diagnosed in the postoperative period or by magnetic resonance imaging performed at least three months after posterior fossa surgery. Finally, CSF infection was defined on clinical ground and positive biochemical examination, but not necessarily positive cultures. RESULTS: The series included 84 operations for resection of posterior fossa tumors on 71 patients. There were CSF related complications in 31% (26/84)with the following detailed incidence: 9.5% (8/84) postoperative hydrocephalus; 14.3% (12/84) CSF leak, 7.1% (6/84) pseudomeningocele; 8.3% (7/84) CSF infection. The mortality rate is 5.9% (5/84). The tumor size was the only statistically significant factor associated with the occurrence of CSF related complications (mean 39.43 mm, SD 18.51 mm vs.29.80 mm, SD 14.12 mm, p=0.015). In the subgroup of patients, in which hydrocephalus was managed preoperatively, the election of an external ventricular drain vs.other strategies (subcutaneous reservoir, definite shunt or endoscopic third ventriculostomy) was associated with a higher occurrence of CSF related complications (p=0.006). The mortality rate was associated with age (mean 63.60 years, SD 5.86 years vs.49.18 years, SD 16.39 years; p=0.002). The occurrence of CSF related complications also influenced mortality (p=0.030), particularly postoperative hydrocephalus (p< 0.001). Inpatient hospital stay was longer in the subgroup of patients who developed CSF related complications (p=0.002). CONCLUSIONS: Tumor size was the only factor associated with the development of CSF related complications after surgery for posterior fossa tumors. In the subgroup of patients in which hydrocephalus was surgically treated preoperatively, the election of an external ventricular drain compared to other surgical modalities was associated with a higher rate of CSF related complications. The development of such complications, particularly hydrocephalus, was related with mortality.
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We report a case of an anterolateral cervical meningocoele presenting as a neck mass in a patient with neurofibromatosis 1. This lesion was diagnosed with the aid of computerized tomography and radionuclide scanning. The presentation, behaviour and treatment options of this uncommon lesion are briefly discussed.
Meningo-encephalocoeles of the skull base may present as spontaneous cerebrospinal fluid rhinorrhoea or acute meningitis. Previous approaches to midline skull base lesions have been either intracranial, via a craniotomy, or by transfacial or endoscopic extracranial approaches. This paper presents an alternative approach to lateral sphenoid sinus encephalocoeles through a Le Fort I osteotomy approach.
Despite the complex embryological development of the nose and surrounding structures, significant developmental nasal anomalies are rare. We therefore present our experience in the management of such anomalies (excluding choanal atresia) over the past 10 years. Fifty cases were surgically treated; two nasopharyngeal cysts, four hairy polyps, two meningoencephalocoeles, seven gliomata, 20 dermoids, two capillary haemangiomata, four fibromas, one fibromyxyoma, one mucocoele, one granuloma, one lipoma, two nasal aplasias, two nasal clefts and one nasal web. All cases presented with nasal obstruction and/or as a nasal mass. Computerized tomography and magnetic resonance imaging aided diagnosis determining the extent of intracranial involvement. Various surgical techniques were used ranging from simple excision to the lateral rhinotomy and Goodman's external rhinoplasty approach. Only five patients (10 per cent) suffered complications; two CSF leaks and three recurrences, all treated successfully.
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BACKGROUND AND PURPOSE: Walker-Warburg syndrome is a congenital autosomal recessive oculocerebral disorder characterised by hydrocephalus, brain agyria, microphthalmos and retinal dysplasia with or without meningoencephalocele. We describe an unusual finding of congenital unilateral glaucoma and buphthalmos in one eye and microphthalmos in the fellow eye of two neonates with Walker-Warburg syndrome. PATIENTS: Two neonates with Walker-Warburg syndrome and unusual findings of buphthalmos in one eye and a microphthalmic fellow eye are presented. RESULTS: Histological examination of the buphthalmic eyes revealed the presence of mesenchymal tissue in the anterior angle covered by endothelium. No anterior chamber angle was identified in the microphthalmic fellow eye and the iris was adherent to the corneal periphery. CONCLUSIONS: Congenital buphthalmos may also appear in Walker-Warburg syndrome. The buphthalmos may result from later embryonal ocular developmental arrest than that of the microphthalmic eye.
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