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Biomedical subjects

R Hayward

Publications and source records attributed to R Hayward.

At least 19 recordsLinked to original sources

Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management.

In the management of craniosynostosis subdural intracranial pressure (ICP) monitoring has proved a useful and safe means of identifying those children with raised ICP who are at risk from its long-term sequelae and who would benefit from early surgical intervention. Overnight subdural ICP recordings have been obtained in 136 unoperated cases of craniosynostosis. Fifteen patients were studied both before and after cranial vault remodelling procedures. ICP was raised (> 15 mmHg) in 35%, borderline (10-15 mmHg) in 37% and normal (< 10 mmHg) in 27% of cases. Raised ICP was present in 28/53 of the syndromic craniofacial dysostosis cases and in 20/83 non-syndromic craniosynostosis cases investigated (P < 0.001). Raised mean ICP and periodic plateaux of sustained ICP during sleep were particularly associated with the syndromic cases. Of the 15 patients studied following cranial vault surgery, 9 showed a reduction in ICP, 3 were unchanged and 3 had higher ICP postoperatively. The results of ICP monitoring can contribute significantly to formulating a rational and staged surgical management plan incorporating the need to normalise ICP and correct the frequently severe functional and cosmetic consequences of these disorders.

Adolescent

The adoption of preventive care practice guidelines by primary care physicians: do actions match intentions?

OBJECTIVE: To measure primary care physicians' familiarity with, attitudes toward, and confidence in preventive care practice guidelines for the elderly and to determine whether their attitudes are associated with implementation of guidelines into clinical practice. DESIGN: A self-administered survey of physicians employed by a health maintenance organization (HMO) and of patients cared for by those physicians. Medical records were also reviewed to assess compliance with practice guidelines. SETTING: An HMO in Southern California. PARTICIPANTS: Forty-eight primary care physicians completed the survey (100% response rate). The medical records of 3,249 randomly selected elderly patients (65 to 75 years old) were studied. Of these patients, 2,799 completed a preventive care survey (response rate 86.1%). MEASUREMENT AND RESULTS: Most HMO primary care physicians agreed or strongly agreed that guidelines will improve quality of medical care (88%) and that guidelines have caused them to change their care of patients (73%). Although the physicians' general attitudes about guidelines did not often correlate with their use of preventive care guidelines, the physicians who stated that practice guidelines had changed their practices were more likely to offer their patients clinical breast examinations (75.9% vs 67.2%, p = 0.04) and to counsel their patients to exercise (70% vs 58%, p = 0.01) than were the physicians who did not. There was a significant association between physicians' support for and adoption of specific practice guidelines regarding mammography (r = 0.34, p = 0.02) and immunizations against influenza (r = 0.42, p < 0.005), pneumococcal pneumonia (r = 0.47, p < 0.001), and tetanus (r = 0.31, p = 0.03). CONCLUSIONS: Physicians employed by an HMO were familiar with and hopeful about the role of guidelines for improving patient care. Physicians' attitudes toward specific preventive care guidelines and admission that guidelines had caused them to change their practice did at times, but not always, correlate with their implementation of guidelines into clinical practice.

Adult

Identical mutations in the FGFR2 gene cause both Pfeiffer and Crouzon syndrome phenotypes.

Mutations in the fibroblast growth factor receptor 2 (FGFR2) gene have been identified in Crouzon syndrome, an autosomal dominant condition causing premature fusion of the cranial sutures (craniosynostosis). A mutation in FGFR1 has been established in several families with Pfeiffer syndrome, where craniosynostosis is associated with specific digital abnormalities. We now report point mutations in FGFR2 in seven sporadic Pfeiffer syndrome patients. Six of the seven Pfeiffer syndrome patients share two missense mutations, which have also been reported in Crouzon syndrome. The Crouzon and Pfeiffer phenotypes usually breed true within families and the finding of identical mutations in unrelated individuals giving different phenotypes is a highly unexpected observation.

Acrocephalosyndactylia

New autosomal dominant form of spondyloepiphyseal dysplasia presenting with atlanto-axial instability.

We present a family with a radiologically distinct new form of autosomal dominant spondyloepiphyseal dysplasia, presenting with cervical instability and attendant neurological compromise and emphasise the radiological characteristics which delineate this condition. Cervical vertebral abnormalities, including malformation of the odontoid process, have been observed in some forms of spondyloepiphyseal dysplasia, but rarely lead to neurological sequelae, in contrast to the pedigree we describe.

Atlanto-Axial Joint

Relationship between intracranial pressure and intracranial volume in craniosynostosis.

Premature fusion of cranial sutures in craniosynostosis has been thought to lead to craniostenosis, which in turn may lead to increased intracranial pressures. In 41 consecutive patients with craniosynostosis, intracranial pressure and intracranial volume were measured. Of the 41 patients, 38 (92.6%) had raised intracranial pressure but only 4 (9.7%) had a decreased skull volume. In the present study, there is no correlation between intracranial volume and intracranial pressure. This study confirms that the measurement of intracranial volume, a non invasive procedure, cannot be used to assess intracranial pressure and to avoid an invasive procedure.

Aging

Complications in paediatric craniofacial surgery: an initial four year experience.

107 children undergoing transcranial craniofacial surgery in a paediatric hospital have been reviewed to assess the incidence and type of complications which arose. This represents the first 4 years' experience of the craniofacial team. There were no deaths or permanent adverse sequelae of surgery. A total of 53 complications were seen in 42 patients. In 9.3% of patients they were potentially life-threatening, serious in 12.1% and of a minor nature in 28%. The more serious complications were related either to haemorrhage and/or vasovagal shock at operation or to infection post-operatively. Infants undergoing monoblock frontofacial advancements and those with tracheostomies were at particular risk.

Adolescent

Pituitary adenoma presenting as the Foster-Kennedy syndrome.

A 27-year-old man presented to the casualty department with visual failure. Clinically he demonstrated the Foster-Kennedy syndrome. Computed tomography revealed a large space-occupying lesion which was subsequently shown to be a pituitary adenoma. The literature is reviewed and possible mechanisms of the Foster-Kennedy syndrome are discussed.

Adult

Magnetic resonance imaging in the assessment of craniosynostosis.

Modern interest in craniosynostosis began when clinicians, regardless of their individual specialty but united in their interest in the problems raised by patients with this condition, first realised that if treatment was confined simply to opening up the fused sutures of the skull vault, success in terms of both function and the restoration of a more normal appearance was likely to be limited. Indeed, the grouping together of such specialists into formal craniofacial units was tacit recognition of this, as was the acceptance that many affected children had evidence of clinical problems that could not be explained on simple mechanical grounds alone. It is not surprising, therefore, that the advent of any new method of investigation has been welcomed by craniofacial surgeons eager to learn anything that might lead to an improvement in management, particularly of the more complex syndromes.

Brain

The epicardial electrogram: a quantitative assessment during balloon angioplasty incorporating monophasic action potential recordings.

An electrogram was recorded from the angioplasty catheter guide wire when coronary blood flow was interrupted in 20 patients undergoing percutaneous transluminal coronary angioplasty. Monophasic action potentials were recorded from the right ventricular septum together with the routine electrocardiogram. The patients were studied during angioplasty for lesions in the left anterior descending (12), circumflex (3), and right coronary arteries (6). ST elevation in the electrogram recorded in the left anterior descending and circumflex systems was usually more obvious than that in the electrocardiogram. Signals obtained from the right coronary artery were of very low amplitude and registered only minimal ST changes. The ST elevation developed in the electrogram during insertion of the catheter before inflation of the balloon in 11 of the 15 patients undergoing angioplasty of the left system. In eight of the patients showing pre-inflation ST elevation the ST shift lessened after successive inflations. Monophasic action potential recordings were obtained during 45 balloon inflations in 19 patients. In those patients undergoing angioplasty for lesions of the circumflex coronary artery the monophasic action potential showed no change during balloon inflation. In patients undergoing angioplasty for the right coronary artery the mean normalised duration at 60 seconds' occlusion was 99.6 (1.5)% of control. Of a total of 25 occlusions in the patients undergoing angioplasty for the left anterior descending coronary artery 19 showed shortening of less than 5%, five showed shortening between 5 and 10%, and one showed a shortening of 16.4% in the monophasic action potential. The QT interval was satisfactorily measured in the electrogram during 36 balloon inflations, and in 24 of these it was also measured in the electrocardiogram. QT changes in the electrogram tended to be the opposite of those in the electrocardiogram. When changes in RR interval were minimal (less than 20 ms) during the balloon inflation 14 of 17 electrograms showed QT prolongation but only one of 12 electrocardiograms showed prolongation. Conversely one of 17 electrograms showed shortening compared with eight of 12 electrocardiograms. There was angiographic evidence of the development of collaterals in six of 15 patients undergoing angioplasty of the left system. ST segment elevation in both the electrogram and electrocardiogram was less pronounced in these patients than in those without evidence of the development of collaterals. ST segment changes recorded from the angioplasty guide wire provide a more sensitive index of ischaemia than the surface 12 lead electrocardiogram, and fall in ST segments on balloon deflation is a prognostic index of a good angiographic result in the left anterior descending and circumflex arteries, but not in the right coronary artery.

Action Potentials

Balloon closure of a surgical aorto-atrial communication.

Surgical repair of an extensive dissection of the proximal aorta (Shumway type A or DeBakey type I) was complicated by persistent haemorrhage from the surgical suture lines and via the false lumen. This was controlled by closing the aortic adventitia round the repaired aorta and by creating an anastomosis between the subadventitial space and the right atrial appendage. Though the haemorrhage was contained, the left to right (aorto-atrial) shunt led to a severe low output state, which was corrected by percutaneous closure of the fistula with a detachable balloon. A year after operation computed axial tomographic scanning showed the balloon in place though the para-aortic space persisted and communicated freely with the aorta.

Aged

Intellectual development in Apert's syndrome: a long term follow up of 29 patients.

Twenty-nine patients with Apert's syndrome were ascertained through hospital records. The mean age was 19.3 years (range eight to 35 years). Further information was obtained on their intelligence, education, and employment records. Fourteen patients (48%) had a normal or borderline IQ (greater than 70), nine patients (31%) were mildly mentally retarded (IQ 50 to 70), four patients (14%) were moderately retarded (IQ 35 to 49), and two patients (7%) were severely retarded (IQ less than 35). Early craniectomy did not appear to improve intellectual outcome. Six of the seven school leavers with normal or borderline intelligence were in full time employment or vocational training.

Acrocephalosyndactylia