Congenital skull indentation: a case report and review of the literature.

Congenital depressions of the skull are rare in Western countries. The majority relate to obstetric trauma at delivery. We present a case of a congenital depression of a neonate's skull not relating to obstetric trauma. The child had an ovoid indentation behind the right coronal suture in the temperoparietal region. This skull depression was thought to relate to the position of her right hand in utero. We report her management, neuro-imaging and outcome on follow-up. A literature review is given in brief.

Infant subcortical cystic leucomalacia: a distinct pathological entity resulting from impaired fluid handling.

BACKGROUND: In the first months of life the subcortical white matter appears prone to fluid accumulation and cystic change. This condition has generally been considered to be due to hypoxic-ischaemic injury (HII) and is grouped with other forms of white matter disease including periventricular leucomalacia (PVL). AIMS: Our aim was to describe the sequential tissue changes in the formation of subcortical cystic leucomalacia in the infant brain and to delineate this from other forms of white matter disease in order to better understand its pathogenesis and aetiology. STUDY DESIGN: Standard samples of the frontal lobe, including subcortical white matter, were stained to demonstrate the cellular processes responsible for subcortical cyst formation in infants who had died from global hypoxic-ischaemic injury (HII) and brain swelling. Cases were of infants who had survived for known periods after collapse in order to determine the time course of the pathological changes. SUBJECTS: 20 infants under 5 months of age with global HII and no other primary brain pathology, and who had survived for between 2h and 13 days after collapse. OUTCOME MEASURES: The description of the sequential changes leading to subcortical cyst formation in infants after severe global HII. RESULTS: With increasing time of survival after global HII the subcortical white matter became more oedematous. Subcortical cysts were seen after one day but were most common in infants surviving more than 5 days. Cysts were not associated with cellular responses and seemed to be the result of fluid accumulation. The pathology was quite distinct from PVL which is due to tissue necrosis. CONCLUSIONS: Subcortical leucomalacia results from fluid accumulation and not necrosis. Predisposition to fluid accumulation may be age-related and due to impairment of fluid handling pathways which remain immature in this age group. Potential mechanisms are discussed.

An unusual cause of head drops.

The presence of vertical ocular motor apraxia should prompt a careful assessment to detect an underlying neurometabolic condition. But congenital vertical ocular motor apraxia is a rare entity that may be attributed to perinatal stroke, hypoxia or kernicterus. We report a case of a 5-year old girl with congenital vertical ocular motor apraxia. Her magnetic resonance imaging (MRI) brain showed symmetrical high signal in the inferomedial thalamic nuclei in the distribution of the vessel of Percheron, a rare posterior cerebral artery variant. Awareness of this rare cause of childhood stroke may obviate unnecessary investigation.

Substantial observer variability in the differentiation between primary intracerebral hemorrhage and hemorrhagic transformation of infarction on CT brain imaging.

BACKGROUND AND PURPOSE: CT remains the most commonly used imaging technique in acute stroke but is often delayed after minor stroke. Interobserver reliability in distinguishing hemorrhagic transformation of infarction from intracerebral hemorrhage may depend on delays to CT but has not been reported previously despite the clinical importance of this distinction. METHODS: Initial CT scans with intraparenchymal hematoma from the first 1000 patients with stroke in the Oxford Vascular Study were independently categorized as intracerebral hemorrhage or hemorrhagic transformation of infarction by 5 neuroradiologists, both blinded and unblinded to clinical history. Thirty scans were reviewed twice. Agreement was quantified by the kappa statistic. RESULTS: Seventy-eight scans showed intraparenchymal hematoma. Blinded pairwise interrater agreements for a diagnosis of intracerebral hemorrhage ranged from kappa=0.15 to 0.48 with poor overall agreement (kappa=0.35; 95% CI, 0.15 to 0.54) even after unblinding (kappa=0.41; 0.21 to 0.60). Blinded intrarater agreements ranged from kappa=0.21 to 0.92. Lack of consensus after unblinding was greatest in patients scanned >or=24 hours after stroke onset (67% versus 25%, P=0.001) and in minor stroke (National Institutes of Health Stroke Scale or=24 hours after minor stroke and in 48% of all 30-day stroke survivors in whom reliable diagnosis would be expected to influence long-term management. CONCLUSIONS: Reliability of diagnosis of intraparenchymal hematoma on CT brain scan in minor stroke is poor, particularly if scanning is delayed. Immediate brain imaging is justified in patients with minor stroke.

Imaging of the skin, subcutis, and galea aponeurotica.

An understanding of the anatomy of the layers of the scalp helps to interpret the radiology and therefore differentiate between various pathologic processes. This article describes the anatomy of the scalp and highlights the common, and some less common, pathologies that occur. Computed tomography, magnetic resonance imaging, and ultrasound can all help to categorize a lesion in the scalp.

Imaging of the calvarium.

The skull vault consists of a multitude of flat bones held together by the cranial sutures. Radiologists encounter a vast array of calvarial pathologies that tend to cause abnormalities in thickness, abnormalities in density, focal defects, or an excess of soft tissue or bone tissue. Further anomalies related to the cranial sutures and fontanelles occur in the dynamic pediatric skull. The imaging features of the host of conditions resulting in these commonly detected calvarial abnormalities are reviewed and illustrated.

Craniosynostosis: a radiological and surgical perspective.

Craniosynostosis is the premature fusion of the cranial sutures. It is identified early in life either due to cosmetic deformity or due to more serious functional complications. Isolated forms of craniosynostosis are much more common and less challenging in their management than their syndromic counterpart. Modern multislice computed tomography and 3D reconstructions have revolutionized surgical planning and follow-up. We review the radiological features of the craniosynostoses, complications, and associated conditions that may alter patient management. Common surgical techniques and postoperative appearances are illustrated in the article.

Implications of a vertex bulge following modified strip craniectomy for sagittal synostosis.

BACKGROUND: Modified strip craniectomy is a common treatment for early isolated sagittal synostosis. The authors assessed the significance of the development of a progressive vertex bulge following strip craniectomy as a predictor of raised intracranial pressure or multiple suture synostosis. METHODS: All cases of sagittal synostosis treated by modified strip craniectomy (removal of the sagittal suture with lateral barrel staving) at the authors' institution were reviewed. Eighty-nine patients with isolated sagittal synostosis were treated by modified strip craniectomy, usually before 6 months of age, between 1995 and 2005. Seven patients were noted to have developed a progressive vertex bulge. The vertex bulge was noted an average of 8 months postoperatively (range, 2 to 25 months). The clinical records of these seven patients were evaluated with regard to their clinical course, radiologic investigations, genetics testing, intracranial pressure monitoring, and the need for further surgery. RESULTS: Computed tomographic scanning demonstrated new synostosis involving other calvarial sutures in five patients. Five patients underwent intracranial pressure monitoring, and this was elevated in four patients. One patient required a ventriculoperitoneal shunt for hydrocephalus. All patients underwent genetic screening, and two were found to have fibroblast growth factor receptor (FGFR) mutations (one FGFR2 and one FGFR3 mutation). All patients required reoperation (calvarial remodeling) for either raised intracranial pressure, deteriorating head shape, or both. CONCLUSIONS: A progressive vertex bulge after modified strip craniectomy is a sign of possible raised intracranial pressure, the development of progressive multiple suture synostosis, or both. It is an indication for genetic testing for FGFR mutations.

The clinical syndrome associated with lumbar spinal stenosis.

Lumbar spinal stenosis is well defined in patho-anatomical terms but its clinical features are heterogeneous. We carried out a comprehensive retrospective review of the clinical features, radiological changes and outcome of 75 patients with radiologically diagnosed lumbar spinal stenosis in order to define its clinical spectrum. The presenting complaints were of weakness, numbness/tingling, radicular pain and neurogenic claudication in almost equal proportions. The commonest symptom was numbness or tingling of the legs. Neurogenic claudication eventually occurred in only 61%. Ninety-three per cent showed abnormalities on neurological examination, but these were generally mild with reduced ankle jerks being commonest. Imaging of the lumbar spine showed that moderate to severe central spinal stenosis correlated with complaints of weakness and abnormal motor power on clinical examination. Patients were reviewed at a mean of 4 years after diagnosis and 65% had undergone surgical decompression; this was not a prospective comparison of different treatment modalities. Overall, a third of patients felt that their symptoms had improved while a quarter felt that they had worsened. More than half had satisfactory neurological function at the time of review. Thirty-nine per cent of those treated surgically, and 25% of those managed conservatively, reported improved symptoms. A poorer functional status at review correlated with complaints of motor weakness and associated comorbid disease. Degenerative lumbar stenosis is a clinically heterogeneous neurological disorder of the lower limbs in the elderly with variable longer-term outcome. A high index of suspicion is required and neuroimaging should be obtained to confirm the diagnosis.

Cerebral infarct and meningitis secondary to Lemierre's syndrome.

The case is reported of a young man with Fusobacterium necrophorum septicemia who developed cavernous sinus thrombosis, meningitis, carotid artery stenosis and stroke. This article presents the only reported case of arterial stroke in Lemierre's syndrome. Clinical presentation, diagnostic difficulty and treatment are discussed.

Cranial bacterial infection.

Early diagnosis of cranial sepsis is mandatory if morbidity is to be avoided. In the case of structural integrity of the skull, haematogenous spread or extension from adjacent structures, especially the sinuses, are the most common sources of infection. Infections may be limited to compartments by the meninges or spread diffusely. Focal disease includes brain abscess as well as subdural and extradural empyaema. A history or signs of sinus disease should always be sought. Tuberculosis, lyme disease and listeriosis may present specific pathological findings. A series of cases is presented to illustrate the role of imaging in infective disease and to draw attention to diagnostic and management points of which radiologists should be aware.

Subdural haematoma and non-accidental head injury in children.

PATIENTS AND METHODS: In this retrospective study, 36 children referred to paediatric neurology and neurosurgery during April 1995-June 1998 with a diagnosis of subdural haematoma (SDH) were studied. Nine were accidental secondary to witnessed trauma and 4 were iatrogenic. Non-accidental head injury (NAHI) was suspected in the remaining 23 children. RESULTS: After a full clinical, radiological and social assessment, NAHI was diagnosed in 14, lateral sinus thrombosis in 1, 2 were accepted as accidental and 6 remained unexplained. In the NAHI group (n=14), 12 were between 4 and 16 weeks of age, 12 (85%) had retinal haemorrhages and skeletal surveys showed evidence of additional injury in 8. Computerised tomography (CT) brain scans showed bilateral SDH in 11, and 6 had inter-hemispheric bleeding along with loss of grey-white differentiation. Eleven had magnetic resonance imaging (MRI), which yielded additional information in 7. Seven required intensive care, and 2 died. Twelve had surgical aspiration. In the group with no satisfactory explanation for SDH ( n=6); 5 had neonatal problems, all except 1 were older than 5 months of age and not as ill with bilateral, old SDH. All but 1 had skeletal surveys, which were normal, and eye examination showed no retinal haemorrhages. A social services enquiry was non-contributory. CONCLUSIONS: SDH is frequently traumatic whether accidental or non-accidental. SDH due to NAHI tends to present before 4 months of age with an inconsistent history; the patients are more seriously ill and have other findings, such as fractures and retinal haemorrhages. A small subgroup of patients was identified who had isolated, old SDH and in whom full investigation remained inconclusive. A consistent, comprehensive approach needs to be maintained in all cases with the essential backup of detailed neuro-imaging including MRI.

Spontaneous intracranial hypotension due to thoracic disc herniation. Case report.

Spontaneous intracranial hypotension is frequently idiopathic. The authors report on a patient presenting with symptomatic intracranial hypotension caused by a transdural calcified thoracic disc herniation. Cranial magnetic resonance (MR) imaging revealed classic signs of intracranial hypotension, and a combination of spinal MR and computerized tomography myelography confirmed a mid-thoracic transdural calcified herniated disc as the cause. The patient was treated with an epidural blood patch and burr hole drainage of the subdural effusion on two occasions. Postoperatively the headache resolved and there was no neurological deficit. Thoracic disc herniation may be a cause of spontaneous intracranial hypotension.

Selective hypertrophy of the cauda equina nerve roots.

Two patients are described with a previously unreported hypertrophic radiculopathy of the cauda equina with preservation of peripheral nerve function. In one removal of an associated bronchial carcinoid tumour led to marked improvement in the neurological condition suggesting a paraneoplastic cause. The second patient later developed an external ophthalmoplegia but the underlying aetiology remains obscure.