Malignant transformation of a lipomyelocele into a rhabdomyosarcoma?

SUMMARY: We report the unusual transformation of a lipomyelocele to a rhabdomyosarcoma in a 3-year-old boy. A lipomyelocele is not considered pre-malignant, but the possibility of developing such a tumor may represent another reason for close neurological follow-up in patients with spinal dysraphism.

Hereditary cerebral cavernous angiomas: presentation as idiopathic familial epilepsy.

In cavernous angiomas of the brain, abnormally dilated blood vessels are tightly clustered together with no intervening neural parenchyma. Commonly recognised as a sporadic finding, these lesions may also be inherited in an autosomal dominant fashion. This disorder was recognised as a common entity with the advent of MRI. As lesions were often not detected on CT imaging, symptomatic patients presenting with seizures were mistakenly diagnosed with an idiopathic seizure disorder. Seizures are the commonest presenting symptom followed by haemorrhage and focal neurologic deficit. We present another cause of familial epilepsy that also emphasises the importance of MRI as the modality of choice in investigating seizures in children.

Posterior fossa tumours in childhood: evaluation of presenting clinical features.

A retrospective review was performed of the age profile and clinical features at presentation of 79 children with posterior fossa tumours. The mean age at presentation in this series (6.6 years) is consistent with a decreasing trend over the past 70 years. Headaches, ataxia and torticollis emerge as significant symptoms worthy of further investigation whilst abdominal pain and constipation might herald the presence of a posterior fossa tumour on rare occasions.

Herpes simplex encephalitis occurring after chemotherapy, surgery, and stereotactic radiotherapy for medulloblastoma.

Reactivation of latent herpes simplex virus (HSV) in the trigeminal ganglion most commonly gives rise to recurrent herpes labialis and rarely to herpes simplex encephalitis. The mechanisms underlying reactivation of latent trigeminal HSV are complex. Here we report the case history of a 25-year-old woman who developed a fatal, bilateral necrotizing destructive temporal lobe lesion following surgical removal of a cerebellar medulloblastoma and combined radiotherapy and chemotherapy for recurrent tumor. Neuropathologic examination of the brain revealed minimal inflammatory changes, but immunohistochemistry was positive for HSV protein, and HSV deoxyribonucleic acid (DNA) was recovered from formalin-fixed paraffin-embedded brain tissue. The temporal proximity of the surgery, chemotherapy, and radiotherapy to the onset of disease suggests that these factors may have acted as triggers that precipitated conversion of latent HSV to overt HSV.

Self-inflicted head trauma using a captive bolt pistol: report of three cases.

Three cases of self-inflicted head trauma using a captive-bolt pistol are described. This is a rarely reported phenomenon and presents with an unique pattern of brain injury.

Psoas abscess secondary to discitis: a case report of conservative management.

We report a case of secondary psoas abscess in a 37-year-old man with a 3-week history of severe low backache managed conservatively without surgical drainage. Apart from bilaterally restricted straight leg raising (<70 degrees), his neurologic examination was within normal limits. Magnetic resonance imaging showed discitis of the L3-L4 space and a left-sided secondary psoas abscess. Aspiration biopsy of the abscess material under radiologic control isolated Staphylococcus aureus, which responded to appropriate antibiotic therapy with complete resolution. A high index of suspicion is necessary for diagnosis of psoas abscess, which should be considered in patients with pyrexia and backache with a neurologic examination that is otherwise normal. We discuss the recommendations for surgical and nonsurgical approaches.

Posterior plagiocephaly: proactive conservative management.

The pathology, diagnosis and management of posterior plagiocephaly remains highly controversial. While the rationale for surgical management of true lambdoid synostosis is undisputed, opinions vary greatly on how to manage severe, unresolving, non-synostotic cases. We reviewed 39 cases of posterior plagiocephaly, 37 of which were treated conservatively. Of these, 34 patients had a significant improvement over the following year with sleep posture modification and/or physiotherapy. While only eight cases returned to complete normality, the remainder had deformities that were deemed mild by both mothers and surgeons, and did not merit surgery. Defining recalcitrant cases remains elusive as standard imaging is often unhelpful. While 3-D CT offers a much more accurate diagnosis of true lambdoid synostosis with bony union and allows objective assessment of the deformity, serial scans involve radiation doses that are difficult to justify. Clinical follow-up is the only reliable method at present.

Histological parameters as predictors of prognosis in childhood brain tumors.

Using histological parameters with high recognition reliability, we retrospectively analyzed all newly diagnosed patients under the age of 16 years (n = 100) with brain and spinal cord tumors presenting to the National Neuroscience Centres of the Richmond and Beaumont Hospitals, Dublin, Ireland, between 1985 and 1990, allowing analysis of 5-year survival in all cases. Tumor histology was reviewed by two neuropathologists blinded to previous histological diagnosis and to the site of lesion. We found that certain histological features such as very low cell density and microcyst formation had a positive effect on prognosis. Mitoses and pleomorphism had a negative effect on prognosis, whereas necrosis and meningeal involvement had no effect on prognosis. It is suggested that identification of reliably recognized histological features rather than assignation of tumors to particular diagnostic categories may be a more reliable predictor of tumor behavior in the pediatric age-group.

Embolic bacterial aneurysm of the basilar artery: case report.

A patient with basilar artery rupture caused by a septic embolus originating from a mitral valve vegetation is reported. The pathogenesis, investigation and management of infected cerebral aneurysms are reviewed.

Titanium mesh and bone dust calvarial patch during cranioplasty.

A technique of filling calvarial defects using bone dust reinforced with titanium Micro Mesh (Leibinger) was investigated using plain x-ray films and computed tomography (CT) in seven consecutive patients (age range, 7-21 years). The aim of our study was to assess whether, in the presence of the titanium, bone dust harvested with a power burr promotes persistent ossification that is comparable with adjacent bone. The mesh was localized by standard skull plain x-ray films, and orthogonal CT scans were obtained at between 9 and 18 months post-operatively. An ultrahigh-resolution algorithm was used to detect neoossification on either side of the Micro Mesh (1-mm collimation, 330-mA and 120 KV(p) at the center of 400 HU, and window width of 2000 Hu). The mesh induced minimal streak artifact. Virtually no new bone formation was seen. It appears that bone dust was completely reabsorbed in this patient group even in the presence of semi-rigid fixation.

Acute near-fatal parainfectious cerebellar swelling with favourable outcome.

The clinical course, neuro-imaging features and neuropathologic findings in a child with para-infectious acute cerebellar swelling are described. Reversible transtentorial and transforaminal herniations occurred and required emergency posterior fossa decompression with external ventricular drainage. Neuropathologic examination of a cerebellar biopsy demonstrated a subacute pathogen-free cerebellitis. Following neurosurgical intervention and steroid therapy, symptoms and signs resolved and the patient is well 3 years later. Acute para-infectious cerebellar swelling is potentially fatal unless recognised and treated early in its evolution.

"Tethered cord syndrome"--recent clinical experience.

Thirty-three consecutive patients treated surgically for "tethered cord syndrome" over a 3-three year period were reviewed. The main presenting complaints were back and leg pain, progressive lower limb and spinal deformity, and neurological deficits. Untethering of the cord was achieved in 32 patients, in whom the filum terminale was divided in 17. There were no serious complications; one patient had a CSF leak which required surgical repair. The mean postoperative follow-up was 15 months and 20 of the patients reported improvement, the rest were unchanged. There was no neurological deterioration in any of the patients. Our series suggests that cord release in patients with "tethered cord syndrome" improves or arrests the progression of neurological deterioration.

Cloverleaf skull anomaly with extreme orbitostenosis.

A neonate presented with cloverleaf skull anomaly and severe proptosis requiring urgent cranioplasty to attain eyelid closure. Despite this, she experienced exposure keratitis and corneal perforations. A ventriculoperitoneal shunt was performed subsequently to relieve hydrocephalus, but respiratory problems eventually led to her death at 6 months. This case highlights the complexity of the problems encountered in the cloverleaf skull anomaly, and a brief review discussing its management is included. Despite improvements in treatment of this condition, the overall prognosis remains poor.

Intraspinal tumours in children--clinical presentation.

Intraspinal tumours in children are rare, the estimated average ratio of spinal to intracranial tumour in the paediatric population is 1:10. We reviewed our experience of paediatric spinal tumours over the period 1992-96. Nineteen patients presented during this time, 12 males and 7 females with the mean age of 7.8 years. The main presenting symptoms were pain, limb weakness, ataxia, sensory disturbance and spinal curvature abnormalities with a mean duration of 10 months. There was a wide variety of tumour types. All underwent a laminectomy with 8 having total tumour excision, 5 partial excision, and 6 had biopsies only. Five patients had extradural lesions and fourteen were intra-dural, four of which were extramedullary and six were intramedullary tumours. There were no major complications of surgery and only one patient had a CSF leak which was repaired. The average hospital stay was 15 days. Seven patients underwent radiotherapy and four had chemotherapy. Four patients are disease free and seven are symptom free after a mean follow-up of 2 years. Four patients died in this series with extensive diffuse tumours.

The Dandy-Walker syndrome--a 10-year experience of its management and outcome.

Hydroephalus of childhood may be associated with intracranial haemorrhage, infection, aqueductal stenosis, Arnold-Chiari malformation (spina bifida), tumours or more uncommonly with the Dandy-Walker syndrome (DWS). We present our experience with the management of this condition over a 10-year period. Twelve children with a definite diagnosis of DWS are reviewed with regard to the age at diagnosis, investigations, treatment, associated problems and overall outcome. Two patients died; there was a high incidence of mental retardation (65%) and associated problems. All but one child had ventriculo-peritoneal shunting and two had cysto-peritoneal shunts in addition to their V-P shunts. There were no familial cases; one child had a chromosomal abnormality. Although the long-term outcome is dismal in most cases, about 35% of the children have a reasonable outcome and hence the condition warrants energetic treatment.

Primary leptomeningeal melanoma: an unusually aggressive tumor in childhood.

Primary malignant melanoma of the leptomeninges of the central nervous system is a rare and aggressive tumor in children. We report our experience from 1964 to 1990 with this tumor in eight children. The mean age at diagnosis was 4.9 years (range, 1.3 to 13 yr). Five children presented with signs and symptoms of raised intracranial pressure from hydrocephalus secondary to tumoral obliteration of the basal cisterns, but the time from the initial symptomatology to diagnosis was frequently delayed. Three patients in this series had hairy nevi in association with their leptomeningeal melanoma. Cerebrospinal fluid (CSF) analysis typically showed raised opening pressures, decreased glucose, and increased protein concentrations. Malignant melanoma cells were found in the CSF in three patients. Confirmatory radiographic examinations included air encephalography, myelography, and computed tomographic and magnetic resonance scanning. Four patients were treated with lumboperitoneal shunts, and one patient was treated with a ventriculoperitoneal shunt for hydrocephalus. Two patients underwent craniotomies and subtotal excisions of their tumors. In seven patients, a definitive diagnosis of leptomeningeal melanoma was made by pathological examination of tissues sent at surgery or at post mortem. In one case, the diagnosis was established by a detailed cytological analysis of the CSF. Four children died of fulminant disease and tumor spread before treatment could be instituted. The four children who received treatment had a combination of radiation therapy and chemotherapy. One child received intrathecal methotrexate. The two children with the longest survivals (2 and 3 yr, respectively) received cisplatinum and dimethyltriazenoimidazole carboxamide in addition to craniospinal irradiation.(ABSTRACT TRUNCATED AT 250 WORDS)

Presentation and diagnosis of brain tumours.

The clinical presentation of brain tumours depends largely upon the biological activity, the age of the patient and the site. The presentation may be with raised intracranial pressure and/or the gradual development of focal neurological signs. The increasing availability of computed tomography and magnetic resonance imaging is changing the way patients with suspected tumours should be investigated.