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Biomedical subjects

R D Hayward

Publications and source records attributed to R D Hayward.

At least 55 records · Page 3Linked to original sources
PubMed1996

Split cervical spinal cord with Klippel-Feil syndrome: seven cases.

We report seven cases of rare high cervical split spinal cord associated with extensive vertebral fusions (Klippel-Feil anomaly). In light of previous embryological theories and recent research findings we attempt to explain the origin of split cord and vertebral fusions. Two distinctly separate mechanisms are suggested for the development of split cords observed in our cases: a midline lesion bisecting the neuroepithelium and the notochordal plate could be responsible for complete splitting of the cervical cord with anterior bony defect while a localized disturbance of cervical neural tube closure would account for cases with partial dorsal splitting of the cord with posterior vertebral defect. Vertebral fusion anomalies are likely to be associated with disturbance of Pax-1 gene expression in the developing vertebral column. We confirm with our cases the frequent association of failure of normal segmentation and split cord in the cervical region. Clinically, only three patients had neurological deficit which was mild and has remained stable, and they had no radiological evidence of tethering; the minimal disproportionate growth of the cord and spine and the rarity of a bony spur in the cervical region are the likely reasons. A conservative policy was therefore pursued in these cases with careful long-term follow-up.

Adolescent↗
PubMed1996

Cervical spine in Pfeiffer's syndrome.

Studies of cervical spine anomalies in patients with Crouzon's and Apert's syndromes have shown an increased incidence of fusions in comparison with that in the normal population. Currently, only small series of patients with Pfeiffer's syndrome who exhibit abnormalities have been published. The objective was to assess the incidence and pattern of radiological cervical spine abnormalities in patients with Pfeiffer's syndrome. All cervical spine radiographs of 22 patients with a confirmed diagnosis of Pfeiffer's syndrome treated at Great Ormond Street Hospital during the last 10 years were studied. All of the radiographs were reviewed by the craniofacial team along with a pediatric radiologist with experience in the assessment of skeletal dysplasias. Radiological abnormalities included hypoplasia of the neural arches, hemivertebrae, and a "butterfly" vertebra as well as vertebral fusion. Evidence of vertebral fusion was present in 16 (73%) of cases. Fusion of both the vertebral bodies and the posterior elements were noted. C2-C3 was the level most commonly involved, although fusion was noted at all levels within the cervical spine. Block fusions involving multiple vertebrae were noted. Analysis of sequential radiographs in 11 patients revealed evidence of progression in eight patients. These results reveal an incidence of anomalies that is higher than previously reported. The older age of the patients in our study demonstrates the progressive nature of the cervical fusions in Pfeiffer's syndrome.

Acrocephalosyndactylia↗
PubMed1996

The effectiveness of papilledema as an indicator of raised intracranial pressure in children with craniosynostosis.

Craniosynostosis management partially depends on the detection and treatment of elevated intracranial pressure (ICP). Examination for papilledema is considered to be the most reliable screening method for identifying raised ICP, but its effectiveness has not been defined. One hundred and twenty-two children with craniosynostosis who underwent funduscopic examinations and then Camino ICP monitoring were studied. All eye examinations were performed by an ophthalmologist after pharmacological pupillary dilation. Fifteen patients (12%) had papilledema. Subsequent ICP monitoring showed that the median ICP was 12.7 mm Hg, with 41 patients (34%) having elevated ICPs (> 15 mm Hg). Those with papilledema had higher ICPs (17.5 +/- 3.2 versus 12.7 +/- 5.5 mm Hg), were older (5.9 +/- 4.7 versus 1.9 +/- 2.6 years), and were more likely to have craniofacial syndromes (73 versus 41%) than those without papilledema (P < 0.05). Patients with both elevated ICPs and papilledema were older (5.9 +/- 4.7 versus 1.6 +/- 1.4 years) and more likely to have multiple-suture synostosis (92 versus 61%) than those with elevated ICPs and no papilledema (P < 0.05). The presence of papilledema was a specific (98%) indicator of raised ICP, but its sensitivity was age-dependent. It was 100% sensitive in children older than 8 years, but it indicated elevated ICP in only 22% of younger patients. These results suggest that ICP monitoring to document elevated ICP is unnecessary in children older than 8 years who have detailed ophthalmological examinations. In the younger child, the presence of papilledema reliably indicates elevated ICP but its absence does not rule out elevated ICP; formal ICP measurement has a greater role in detecting elevated ICP in these patients.

Adolescent↗
PubMed1996

The beaten copper cranium: a correlation between intracranial pressure, cranial radiographs, and computed tomographic scans in children with craniosynostosis.

OBJECTIVE: The beaten copper appearance of the cranium, as well as other cranial radiographic and computed tomographic findings in children with craniosynostosis, is often interpreted by clinicians as evidence of elevated intracranial pressure (ICP). However, a correlation between radiological findings and ICP measurements has not been previously demonstrated, and their usefulness in detecting elevated ICP has not been defined. METHODS: To address those issues, 123 children with craniosynostosis who had cranial radiographs and ICP monitoring were studied. To assess the specificity of certain radiological findings to patients with craniosynostosis, cranial radiographs of patients with craniosynostosis were compared to those of age- and sex-matched controls. In patients with craniosynostosis, findings on cranial radiographs were compared to computed tomographic scans of the brain. Radiographic findings were then correlated with ICP measurements obtained while the patient was sleeping, which was measured using a Camino fiberoptic ICP monitor (Camino Laboratories, San Diego, CA). All radiographs were independently analyzed by two radiologists who were blinded to clinical and ICP data. RESULTS: A diffuse beaten copper pattern, erosion of the dorsum sellar, and suture diastasis were seen more commonly in patients with craniosynostosis than in controls (P < 0.05), but the presence of the beaten copper pattern was no more common in children with craniosynostosis. ICP was greater when a diffuse beaten copper pattern, dorsum sellar erosion, suture diastasis, or narrowing of basal cisterns was present (P < 0.05). CONCLUSION: Although this study demonstrates that some cranial radiographic and computed tomographic findings do correlate with elevated ICP, the sensitivity of radiological methods for detecting elevated ICP is universally low and they are not recommended to screen for elevated ICP in children with craniosynostosis.

Child↗
PubMed1996

Growth and endocrine sequelae of craniopharyngioma.

The growth and endocrine sequelae of 75 children (33 girls and 42 boys) with craniopharyngioma, treated from 1973 to 1994, were studied by retrospective review and by follow up assessment in 66 survivors, with a mean time from initial surgery of 6.7 years (range 1.5 to 19.8 years). Although infrequently complained of, 71% of patients had symptoms to suggest an endocrinopathy at diagnosis. After surgery, multiple endocrinopathies were almost universal, such that 75% of children had panhypopituitarism at follow up. Hypoadrenal crises in association with intercurrent illness contributed significantly to morbidity and mortality, as did the metabolic consequences of concomitant antidiuretic hormone (ADH) insufficiency and absent thirst. Final height in 25 patients was significantly below genetic target height, particularly in the girls, with loss of height potential occurring during the pubertal years. The endocrine morbidity associated with craniopharyngioma and its treatment remains high but manageable with appropriate hormone replacement. However, the combination of ADH insufficiency and an impaired sense of thirst following aggressive surgery and severe hypothalamic injury remains one of the most complex management problems.

Adolescent↗
PubMed1996

Congenital cervical spinal fusion: a study in Apert syndrome.

The occurrence and pattern of cervical spinal fusions have been assessed in 59 cases of Apert syndrome (acrocephalosyndactyly type 1). Radiological evidence of vertebral fusion either in progress or completed was observed in 37 (63%) of the cases. Fusion was limited to a single vertebral level in 18 cases and multiple levels, involving either contiguous or skipped levels in the remaining 19+ C3-4 and C5-6 were the levels most commonly involved. This distribution of fusions is different from other instances of congenital spinal fusion including those associated with other varieties of craniosynostosis. There was a significant association between age at the time of radiograph and the presence of spinal fusions (p < 0.001, Wilcoxon 2-sample test). Analysis of sequential radiographs in 17 patients revealed evidence of progressive fusion in 10. Small size of the vertebral body and reduced intervertebral disc space were indicators of subsequent bony fusion. The fusions seen in Apert syndrome thus appear to be progressive, occurring at the site of subtle congenital vertebral anomalies and may not be apparent as a congenital feature. The implications for the aetiology of so-called "congenital' spinal fusions in Apert syndrome and other situations are discussed.

Acrocephalosyndactylia↗
PubMed1996

Obesity in childhood craniopharyngioma: relation to post-operative hypothalamic damage shown by magnetic resonance imaging.

OBJECTIVE: To quantify the extent of hypothalamic damage after surgery for craniopharyngioma using magnetic resonance imaging (MRI) and to relate the findings to changes in body mass index (BMI). PATIENTS: Sixty-three survivors (36 males, 27 females) of childhood cramopharyngioma were treated surgically between 1973 and early 1994. METHODS: Cranial MRI was performed at a structured follow-up assessment 1.5-19.2 yr after the initial surgery. Hypothalamic damage was scored as 0 (no visible damage), 1 (intermediate), or 2 (severe). RESULTS: After surgery there was an increase in BMI standard deviation (SD) from diagnosis to study assessment in all but 7 patients. However, patients with MRI scores of 2 (n = 17) had a significantly greater increase in median BMI SD score at follow-up (+5.5 SD score), compared with +2.5 SD score and +1.1 SD score for patients with MRI scores of 1 or 0, respectively. Of the 17 cases with MRI scores of 2, 10 had a history of extreme weight loss or weight gain at presentation; preoperative neuroimaging demonstrated extensive hypothalamic infiltration by tumor in these cases. CONCLUSION: MRI gives sufficient anatomical definition to allow assessment of the extent of hypothalamic damage and, thereby, prediction of the patients most at risk for severe post-operative weight gain.

Adolescent↗
PubMed1996

Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted?

Seventy-five children treated for craniopharyngioma between 1973 and 1994 were studied to demonstrate which pre- and intraoperative factors were indicative of a poor outcome as defined by a quantitative assessment of morbidity. This involved a retrospective review of 65 patients and a prospective study of 10 patients focused on clinical details and cranial imaging and a follow-up "study assessment" of 66 survivors performed over the last 2 years. As part of the assessment, 63 patients underwent magnetic resonance imaging with a three-dimensional volume acquisition sequence 1.5 to 19.2 years after initial surgery. Predictors of high morbidity included severe hydrocephalus, intraoperative adverse events, and young age ( < or = 5 years) at presentation. Predictors of increased hypothalamic morbidity included symptoms of hypothalamic disturbance already established at diagnosis, greater height ( > or = 3.5 cm) of the tumor in the midline, and attempts to remove adherent tumor from the region of the hypothalamus at operation. Large tumor size, young age, and severe hydrocephalus were predictors of tumor recurrence, whereas complete tumor resection (as determined by postoperative neuroimaging) and radiotherapy given electively after subtotal excision were less likely to be associated with recurrent disease. Based on these findings, the authors propose an individualized, more flexible treatment approach whereby surgical strategies may be modified to provide long-term tumor control with the lowest morbidity.

Adolescent↗
PubMed1996

Use of an adjustable, transportable, radiolucent spinal immobilization device in the comprehensive management of cervical spine instability. Technical note.

In this report the authors describe a device that consists of a transportable, radiolucent board that couples to a standard halo head ring. The board provides continuous cervical spine immobilization during all phases of acute medical treatment of cervical spine instability, including closed reduction, transport, radiographic imaging, and operative procedures. By combining the advantages of several existing systems, this immobilization device facilitates and improves the safety of comprehensive acute management of cervical spinal instability by eliminating the need for patient transfer from stretcher to radiography machine to operating table. Its radiolucent construction and its compatibility with standard operating tables allow unencumbered surgical access and ample room for biplanar fluoroscopy, thereby also facilitating operative procedures, particularly the placement of internal spinal fixation.

Cervical Vertebrae↗
PubMed1996

Use of intracranial pressure monitoring in the management of childhood hydrocephalus and shunt-related problems.

Although the clinical and radiological diagnosis of hydrocephalus in children is usually straightforward, there exists a minority of patients in whom the decision to shunt can be extremely difficult. Similarly, although the diagnosis of shunt malfunction usually presents little difficulty in the context of an acute blockage, a child can present with a confusing and unpredictable constellation of symptoms that might be caused by conditions separate from shunt malfunction. Continuous intraparenchymal intracranial pressure (ICP) monitoring was used to assess 41 patients with hydrocephalus, either as part of the initial diagnostic evaluation of ventriculomegaly (18 patients) or in the assessment of presumed shunt malfunction (23 patients). In 9 of 18 patients with ventriculomegaly, the ICP was within normal limits and surgical insertion of shunts was avoided. Of the 23 patients being assessed for shunt malfunction, the change in ICP profile indicated a siphoning or overdrainage process in 13. In no patient was there significant attendant morbidity, and the process was well tolerated and simple to perform. Clinical and radiological criteria alone can afford insufficient information in the initial evaluation and the subsequent management of the child with hydrocephalus. ICP monitoring provides a safe means of investigating such patients and provides valuable information upon which to base surgical management.

Adolescent↗
PubMed1996

Use of a commercial computer program for continuous audit of surgical complications.

Audit is essential for good medical practice, but is time-consuming, and each unit has been left to devise their own techniques. One method is described using a widely available computer package that allows the results to be charted and updated automatically. Any deterioration in performance is thus readily apparent. This method has been particularly useful for auditing shunt infections, but could easily be adapted for other uses.

Cerebrospinal Fluid Shunts↗
PubMed1995

The use of autologous skull bone grafts for posterior fusion of the upper cervical spine in children.

STUDY DESIGN: Traditionally bone grafts used in posterior cervical fusion have been harvested from the iliac crest, rib, tibia, or fibula. Their use is not without significant morbidity, and it is often difficult to harvest suitable quantities of good quality bone in children. We have used autologous calvarial bone secured by sublaminar wires in seven patients with congenital spinal anomalies to achieve craniocervical stabilization and fusion. OBJECTIVES: To detail our experiences with this new method of harvesting autologous bone grafts from the patient's skull for occipitocervical fusion in a pediatric practice. SUMMARY OF BACKGROUND DATA: Calvarial bone has been used extensively in craniofacial reconstructive work with good long-term results and with no significant bone resorption. The use of membranous bone is supported by several basic science studies reported in the plastic surgery literature that claim a superiority over endochondral bone regarding fusion. In the present study, autologous calvarial bone has been used in the treatment of seven cases of congenital upper cervical spine instability associated with neurologic deficit. This method circumvents many of the problems attached to the use of traditional donor sites and provides good quality bone in large quantity that can be specifically tailored to match the contours of the craniocervical junction. METHOD: We used autologous calvarial bone secured by sublaminar wires in seven children with congenital spinal anomalies to achieve craniocervical stabilization and fusion. The ensuing calvarial defect was repaired using split-thickness parietal bone with the bone graft, which was ultimately used for the cervical fusion and acted as a template to obtain a mirror image and exact match to achieve good cosmetic results. The patients were immobilized after surgery in a halo orthosis for 3 months. RESULTS: Satisfactory results (100% fusion) were achieved in all seven cases with no attendant morbidity related to the method of graft procurement. CONCLUSIONS: We recommend this technique as a safe and effective alternative to the more traditional means of graft procurement previously used in cases of craniocervical instability in children.

Adolescent↗
PubMed1995

Intracranial ependymomas in childhood: a report of 24 cases followed for 5 years.

The management and outcome of 24 patients with intracranial ependymomas admitted to our institution between January 1979 and May 1988 have been analysed. Four patients out of the ten with infratentorial ependymomas who underwent a visible total resection were alive and free of disease at 5 years, whereas all those who had had an incomplete resection were dead. The prognosis in the supratentorial group was worse; only one patient out of seven was alive and free of disease at 5 years. Nine patients (six infratentorial and three supratentorial ependymomas) were investigated with myelography for occult spinal metastases, and apart from one who had a direct extension of an infratentorial tumour into the upper cervical spine, none had detectable metastases. Eight patients with infratentorial tumours underwent a ventriculo-peritoneal shunt insertion, and in three of them this followed a visible total resection of the tumour. Comparison of our results with those from other series suggests that any improvement in outlook is related primarily to an increased number of children surviving their initial surgical treatment, rather than to better methods of postoperative tumour control.

Cerebral Ventricle Neoplasms↗
PubMed1995

Apert syndrome results from localized mutations of FGFR2 and is allelic with Crouzon syndrome.

Apert syndrome is a distinctive human malformation comprising craniosynostosis and severe syndactyly of the hands and feet. We have identified specific missense substitutions involving adjacent amino acids (Ser252Trp and Pro253Arg) in the linker between the second and third extracellular immunoglobulin (Ig) domains of fibroblast growth factor receptor 2 (FGFR2) in all 40 unrelated cases of Apert syndrome studied. Crouzon syndrome, characterized by craniosynostosis but normal limbs, was previously shown to result from allelic mutations of the third Ig domain of FGFR2. The contrasting effects of these mutations provide a genetic resource for dissecting the complex effects of signal transduction through FGFRs in cranial and limb morphogenesis.

Acrocephalosyndactylia↗
PubMed1995

Medulloblastoma: factors influencing the educational potential of surviving children.

Twenty-five children with a histological diagnosis of medulloblastoma operated upon in our unit and who had then been treated with identical radiotherapy regimes were studied in an attempt to determine the academic potential of children who survive for more than two years following their initial presentation. Fourteen children were attending a mainstream school at which they required no special help or remedial classes and were in the appropriate year for their age while eleven required special help with their schooling. Seven of these attended a mainstream school, but required remedial classes for help with reading, writing and arithmetic while two were in a class a year below what would have normally been expected for their age group. Two children attended a special school for children with learning difficulties. These differences in academic potential can be related to the age of the child at presentation and the presence or absence of postoperative complications.

Cerebellar Neoplasms↗
PubMed1995

Intracranial pressure in single-suture craniosynostosis.

The indications for surgical correction of craniosynostosis in which there is involvement of only one of the cranial vault sutures have traditionally been based upon the cosmetic merits of the deformity alone. Whilst it is now appreciated that intracranial hypertension is commonly associated with the more complex forms of craniosynostosis, this aspect has not previously been addressed in detail among cases of single-suture craniosynostosis. This retrospective study reports our experience of overnight subdural intracranial pressure monitoring in 74 children with premature closure of a single cranial suture. A single coronal suture was involved in 37 patients, the sagittal suture in 25 and the metopic suture in 12. Intracranial pressure was raised in 13 (17%), borderline in 28 (38%) and normal in 33 (45%). Elevated intracranial pressure was seen more commonly where a midline suture was involved (sagittal or metopic) than when a single coronal suture was fused. We conclude that intracranial hypertension occurs in a significant proportion of children with single-suture craniosynostosis and suggest that this factor should be borne in mind during the initial assessment of these children so as to enable timely intervention where required and appropriate counselling of parents.

Child↗