Bruce Clark

CT-determined intracranial volume for a normal population.

Intracranial volume comparisons of patients with craniosynostosis and normal have been contrary to expectations, leading to questioning of the validity of the current normal reference material. Computed tomography-determined intracranial volume is presented for a white normal population. Specifically, intracranial volumes for 157 subjects (82 female and 75 male) were measured from computed tomography data using the Cavalieri estimator: volume determination was based on measuring the area in each computed tomography section. Monomolecular and Gompertz models were applied to find curves of best fit to the intracranial volume as a function of the age. The best fit was obtained using the monomolecular model when the response variable was the logarithmically transformed intracranial volume, and the independent variable was the logarithm of the age from conception. For example, the mean (standard deviation) for male subjects at 1 year and 20 years were 1,125.6 (89.6) ml and 1,472.9 (117.2) ml, respectively, and for female subjects 1,024.9 (84.0) ml and 1,321.7 (108.3) ml, respectively. Although the shape and rate of increase of the female and male curves is similar, the female mean is 1.3 standard deviations below the male mean at 20 years. These curves were compared with the commonly referenced curves of Blinkov (1941), Lichtenberg (1960), and Dekaban (1977). Our male curve is substantially higher than these curves in the age range 8 months to 4 years. Our female curve, however, is approximately 1 standard deviation below Lichtenbergs curve from birth to 7 months. There are then only minor differences between our female curve and Lichtenbergs curve until his curve crosses ours at 41 months, where they significantly diverge from approximately 4.5 years. Our curves indicate that 95% of the final intracranial volume has been attained by 42 months for girls and 46 months for boys.

Evaluation of a Neuraminidase Detection Assay for the Rapid Detection of Influenza A and B Virus in Children

ABSTRACT A prototype version of a new diagnostic assay for influenza A and B (Zstat Flu™) based on detection of viral neuraminidase was evaluated and compared to culture in 196 clinical samples. Children with respiratory illnesses were prospectively evaluated at a pediatricians office and at a large childrens hospital using the neuraminidase assay and viral culture performed on respiratory secretions. Influenza virus was isolated from 51 samples and 83 were positive by the neuraminidase assay. When compared to culture the sensitivity of the assay was 96%, specificity was 77%, positive predictive value was 59%, and negative predictive value was 98%. Testing in the laboratory of pure cultures of bacteria and non-influenza viruses frequently found in the respiratory tract showed 0% cross-reactivity with the neuraminidase assay and 100% specificity for influenza virus in vitro. This new assay provided useful information for the preliminary diagnosis of influenza A and B infections and appears to be suitable for both point-of-care use in the physicians office and rapid diagnosis in a virology laboratory. The high sensitivity makes it particularly useful as a screening test for exclusion of influenza A and B infections. To confirm the diagnosis and exclude a false-positive result, as well as to determine the influenza virus type, a viral culture may be considered.

Clinicopathological and radiological features of two cases of intraventricular meningioma in childhood.

The clinical, radiological and pathological features of two cases of intraventricular meningioma in a 9-year-old boy and a 9-year-old girl are reported. Presenting features included headache, vomiting and somnolence with no localizing neurological signs on physical examination. Neither patient showed evidence of neurofibromatosis. CT scans were helpful in establishing the preoperative diagnoses with uniformly hyperdense, well-circumscribed lesions showing bright enhancement after contrast within the lateral and third ventricles respectively. Histological examination revealed mixed fibroblastic/angioblastic and fibroblastic patterns, with typical electron-microscopic and immunohistochemical features of meningioma. Successful surgical removal was achieved in both cases.

Central nervous system imaging in Crouzon's syndrome

Although the need to prevent the secondary effects of craniosynostosis on the central nervous system is fundamental to the practice of craniofacial surgery, the detailed structural anatomy of the central nervous system in the syndromal craniosynostoses has become the subject of recent interest. A clinical and radiographic review of a population of 59 patients with Crouzons syndrome determined the frequency of central nervous system deformities. Twelve percent of patients had evidence of decreased mental function. Ventriculomegaly on computed tomographic scan was present in 51% and found to be of three grades: mild, moderate, and severe (hydrocephalus). This was nonprogressive in 7 of the 11 patients with follow-up computed tomographic scans. Ten patients underwent surgical release to increase intracranial space; however, 6 of these patients showed no progression in ventricular size. Nonventricular anomalies were found less frequently (14%). Central nervous system findings show fewer nonventricular anomalies than in Aperts syndrome patients, with a corresponding higher mental function. The principal anomaly of ventriculomegaly is not directly related to suture defect and may represent a primary brain abnormality. Recommendations are made for the assessment and management of patients with Crouzons syndrome with reference to these areas.

Spinal Anomalies in Pfeiffer Syndrome

Review of the spinal radiographs of a consecutive series of 11 patients with Pfeiffer syndrome presenting to the Australian Craniofacial Unit was performed. The prevalence of cervical spine fusions was high, and the pattern of fusion complex. Isolated anomalies were evident at lower levels, including two cases of sacrococcygeal eversion. Spinal anomalies occur more frequently in the more severely involved cases of Pfeiffer syndrome emphasizing the generalized dysostotic nature of this condition.

Congenital ossifying fibroma (osteofibrous dysplasia) of the tibia―a case report

Ossifying fibromas of the long bones of the leg are benign lesions occurring in the pediatric age group identical in histological appearance to the similarly named tumor of the jaw in adults. Most frequently presentation occurs after minor trauma with symptoms of a swelling of the tibia or fibula which may be painful. Pathological fracture or limp are also occasional presentations. Congenital cases are extremely rare. We describe an otherwise normal male neonate who presented at birth with a bowed right lower leg. The limb was 1 cm shorter than the other side, with tibia vara and a firm mass situated anteriorly. X-ray showed a mixed lytic and sclerotic lesion in the proximal metaphysis of the tibia. Biopsy showed collagenous stroma containing spindle cells and irregular trabeculae of woven bone rimmed by plump osteoblasts. As the appearances were typical of an ossifying fibroma (osteofibrous dysplasia) no surgical treatment was given. The patient was well with no growth of the tumor and with radiological evidence of healing at 1 year follow up. This case is presented to draw attention to the clinicopathological features of this unusual lesion which must be considered in the differential diagnosis of congenital lesions of the tibia.

Cervical spine in Treacher Collins syndrome.

Abstract Treacher Collins syndrome is a congenital syndrome with characteristic craniofacial malformations, which are well described in the literature. However, the presence of cervical spine dysmorphology in this syndrome has been minimally described. This study reviews cervical spine radiographs of 40 patients with Treacher Collins syndrome. In this sample, 7 of 40 patients displayed cervical spine anomalies, with 3 of these patients displaying multiple cervical spine anomalies. The patterns of spinal anomalies were variable, suggesting that the underlying genetic mutation has variable expressivity in cervical spine development as it does elsewhere in the craniofacial skeleton.

Fronto-facio-nasal dysplasia.

Fronto-facio-nasal dysplasia is a rare cause of facial clefts. The syndrome is characterized by paramedian facial clefts which involve the nose and palpebral fissures resulting in defects of the alae nasi and blepharophimosis, lagophthalmos, and S-shaped palpebral fissures. In addition affected children have ocular malformations such as epibulbar dermoids and colobomata of the iris or optic disk and may have a posterior encephalocele; these features distinguish this condition from fronto-nasal dysplasia and early amnion rupture sequence. We describe a child with unilateral features. Unilateral craniofacial clefts are usually assumed to have a low recurrence risk. However, fronto-facio-nasal dysplasia is an autosomal recessive condition and must be considered in any child with paramedian facial clefts.

The infant skull in Pfeiffer's syndrome.

A review of Pfeiffers syndrome patients presenting in infancy identifies characteristic patterns of onset and progression of premature sutural fusion. Classic Pfeiffers syndrome manifests symmetrical bicoronal synostosis; all other sutures are normal. The remaining patients, with a more extreme phenotypic expression, have superimposed on bicoronal synostosis progressive involvement of other cranial sutures, frequent hydrocephalus and craniolacunae, suggesting craniostenosis and intracranial hypertension. Although similar in clinical features and outcome, these patients have been subgrouped according to the presence or absence of a cloverleaf skull anomaly.

Choroid plexus papillomas in childhood

Chroid plexus papillomas account for only 0.4-0.6% of intracranial tumours at all ages and less than 5% in childhood. Two paediatric cases are reported to demonstrate the variability in clinical presentation, location and neurological outcome of this benign tumour. One case has been followed for 13 years.