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Biomedical subjects

R D Hayward

Publications and source records attributed to R D Hayward.

At least 37 records · Page 2Linked to original sources
PubMed1999

Craniotomy-related complications in a paediatric neurosurgery unit--a prospective study.

A prospective study on four groups of children undergoing a craniotomy over a 6-month period was carried out in order to assess the incidence of complications. All complications were graded according to their severity and the requirement for extra general anaesthetics also recorded. From this information it is possible to calculate the resource implications of craniotomy-related complications in a paediatric neurosurgical unit, as well as point to those areas of clinical practice where improvements in technique are most needed.

Brain Diseases↗
PubMed1998

Maxillary distraction osteogenesis in Pfeiffer's syndrome: urgent ocular protection by gradual midfacial skeletal advancement.

Distraction osteogenesis is increasingly recognised as a potentially useful technique to achieve the co-ordinated augmentation of craniofacial skeletal and soft tissue. A case is presented where bilateral maxillary distraction was successfully used to advance the midface in the treatment of recurrent ocular dislocation, in a 10-month-old boy with Pfeiffer's syndrome.

Acrocephalosyndactylia↗
PubMed1998

The feet in Pfeiffer's syndrome.

Broad toes are the classic clinical finding occurring in the feet in Pfeiffer's syndrome patients, but few cases undergo formal radiological assessment. However, the feet in other craniosynostosis syndromes resulting from mutations of the fibroblast growth factor receptor 2 gene have anomalies at many other sites within the feet, which raised the possibility that there may also be a wider range of anomalies other than broad big toes in the feet of those with Pfeiffer's syndrome. The object of this study was to assess prospectively the incidence and pattern of clinical and radiographic anomalies of the feet of patients with a confirmed diagnosis of Pfeiffer's syndrome. Twenty-two Pfeiffer's syndrome patients were examined both clinically and radiologically for anomalies of the feet. Clinical examination revealed broad big toes in 12 patients and syndactyly in 2. Radiographs of the feet were evaluated by a radiologist with an interest in skeletal dysplasia as well as by members of the Craniofacial team. A wider range of radiological than clinical anomalies involving the phalanges, metatarsals, and tarsals was seen. The severity of the anomalies observed ranged from normal to those resembling the feet of Apert's syndrome patients. Only 4 patients had radiologically normal feet. These radiographic findings suggest that there is a range of foot anomalies more extensive than the broad big toes, which are classically associated with Pfeiffer's syndrome.

Acrocephalosyndactylia↗
PubMed1998

The elbow in syndromic craniosynostosis.

Craniosynostosis may occur in conjunction with limb and visceral anomalies in more than 100 syndromes and may include anomalies of the elbow. Apert's, Pfeiffer's, Crouzon's, and Saethre-Chotzen syndrome have been linked with anomalies of the elbow, but the incidence and severity of such anomalies is unknown. A prospective radiographic study was undertaken to establish the types, incidence, and severity of elbow anomalies in patients with either Apert's, Pfeiffer's, Crouzon's, or Saethre-Chotzen syndrome attending the Craniofacial Centre at Great Ormond Street Hospital during a 12-month period. This study showed that elbow anomalies were very common in Apert's and Pfeiffer's syndrome, but less so in Crouzon's syndrome. The elbows in all patients with Saethre-Chotzen syndrome were normal. A range of anomalies was seen, with overlap between the syndromes. The severest anomaly seen in children with Crouzon's, Pfeiffer's, and Apert's syndrome was complete synostosis, which may require surgical intervention in due course. The results of this study suggest that the incidence of elbow anomalies in Apert's, Pfeiffer's, and Crouzon's syndrome is higher than the current literature suggests. Synostosis can be so severe that orthopedic review as part of the management of these children may be beneficial and may become increasingly important as more of these children survive into adulthood.

Acrocephalosyndactylia↗
PubMed1997

The cervical spine in Crouzon syndrome.

STUDY DESIGN: All cervical spine radiographs of 44 patients with Crouzon syndrome treated at Great Ormond Street Hospital during the past 10 years were studied. OBJECTIVES: To assess the incidence and pattern of cervical spine abnormalities of patients with a confirmed diagnosis of Crouzon syndrome, but particularly regarding progressive fusion. SUMMARY OF BACKGROUND DATA: Previous studies into the cervical spine anomalies in those with Crouzon syndrome have shown an increased incidence of congenital abnormalities compared with the normal population. There is some suggestion from previous studies that cervical spine fusions are progressive in nature. METHODS: All radiographs were reviewed by the craniofacial team, along with a single pediatric radiologist with experience in assessment of skeletal dysplasias. RESULTS: Radiologic abnormalities included "butterfly" vertebrae and fusions of the bodies and the posterior elements. Evidence of fusion was present in eight of 44 (18%) of patients. C2-C3 and C5-C6 were almost equally affected. Block fusions involving multiple vertebrae were seen. Analysis of sequential studies in 16 patients showed evidence of progression in five. CONCLUSIONS: These results reveal an incidence of fusions that is lower than reported previously. There is radiologic evidence from serial studies that the fusions are progressive, and because these patients are children, the fusion process may not be complete, which may account for the lower incidence of fusions than in previous studies. The pattern of fusions is different from that in earlier studies, which may be a result of the method of diagnosis because this population is less likely to include atypical forms of other syndromes (which have a higher incidence of cervical fusions, particularly at C2-C3). Regarding other congenital anomalies, it appears that butterfly vertebrae are especially prevalent in association with Crouzon syndrome.

Cervical Vertebrae↗
PubMed1997

Hand anomalies in Crouzon syndrome.

OBJECTIVE: To review the hand radiographs of patients with Crouzon syndrome, to look for extracranial manifestations of the condition at this site. DESIGN: The hand radiographs of those with Crouzon syndrome attending the Craniofacial Service at Great Ormond Street between 1985 and 1996 were reviewed. RESULTS: Thirty-three patients underwent a total of 34 radiographs, one patient having had a serial study. This revealed a range of minor anomalies. Most striking was the presence of carpal fusions in four cases, a feature which has not previously been reported. CONCLUSION: The hands of Crouzon syndrome may have anomalies including carpal fusions, and these results are evidence that the condition may produce subtle effects on the hands.

Adolescent↗
PubMed1997

Anomalous venous drainage in a case of non-syndromic craniosynostosis.

The authors describe the clinical and radiological findings in a case of non-syndromic craniosynostosis affecting multiple sutures, in which the intracranial venous drainage was grossly anomalous. Investigation by magnetic resonance imaging and angiography revealed that almost all of the intracranial venous blood was draining from the dural sinuses transosseoussly via enlarged emissary veins to the external jugular veins and the vertebral veins. Although present, both internal jugular veins were small. This discovery represented a contraindication for the vault expansion surgery that had been recommended as treatment for the patient's raised intracranial pressure, and it has important implications for the management of all types of craniosynostosis.

Cerebral Angiography↗
PubMed1997

Split cord malformations of the lumbar region. A model for the neurosurgical management of all types of 'occult' spinal dysraphism?

From a group of 84 patients with split cord malformations presenting to our Department between 1976 and 1990, we have selected 47 cases in whom the split cord was confined to the lower dorsal-lumbar region and in whom there were no other dysraphic features such as meningocele, lipoma or dermoid cyst. We have studied these cases of 'pure split cord malformation' in an attempt to decipher its natural history and the effects of surgical procedures designed to untether the spinal cord. We conclude that the 'neuro-orthopaedic syndrome' (which includes lower limb asymmetry, talipes and modest sensory and motor problems) is, in most cases, an inevitable consequence of the abnormal functional anatomy of the split cord and is not due to any mechanical effects of tethering of the spinal cord. Its emergence as a child grows (assuming that it was not obvious at birth) is not influenced by surgery. True neurological deterioration (defined as the loss of a previously established neurological or urological function) is a rarer event. It may occur at any age--including well into adulthood--but in children the average age at presentation is 6.8 years (range 2 years 9 months to 11 years). It is due to the mechanical effects of tethering of the spinal cord and is more likely to be arrested than improved by surgery. 'True' deterioration did not occur in any of our cases who had undergone prophylactic untethering of the spinal cord and we have concluded, therefore, that surgery has a role in the prevention of late neurological problems affecting the lower limbs and bladder. Surgery to untether the spinal cord will not, however, have any affect upon the emergence of the neuro-orthopaedic syndrome.

Adolescent↗
PubMed1997

Aetiology of herniation of the hindbrain in craniosynostosis. An investigation incorporating intracranial pressure monitoring and magnetic resonance imaging.

The occurrence and extent of herniation of the hindbrain has been evaluated in a population of children with craniosynostosis by means of magnetic resonance imaging of the craniocervical junction. The role of intracranial pressure (ICP), posterior fossa size and hydrocephalus in the development of this deformity has also been assessed. Magnetic resonance imaging (Siemens Magnetom 1.5T) was reviewed in 27 cases of craniosynostosis in whom there had been no previous cranial vault surgery. The position of the cerebellar tonsils in relation to the plane of the foramen magnum was measured and an index of the size of the posterior fossa relative to the rest of the cranial vault was also calculated for each case. The presence of hydrocephalus (requiring a cerebrospinal fluid diversion procedure) was documented. In 22 of these cases overnight, subdural ICP monitoring using the Camino fibre optic device had also been performed. Herniation of the hindbrain below the plane of the foramen magnum occurred in 10 of 27 cases (37%). The level of ICP showed a significant correlation with the extent of hindbrain herniation (p < 0.001) as did small posterior fossa size (p = 0.0035). Hydrocephalus was present in 4 patients, all of whom had hindbrain herniation. The extent of hindbrain herniation did not correlate with age (p = 0.48). We propose that herniation of the hindbrain in craniosynostosis is a consequence of brain deformation occurring in response to the physical forces imposed by a combination of the anatomical deformity at the skull base and intracranial hypertension rather than a primary malformation of brain development as commonly supposed.

Arnold-Chiari Malformation↗
PubMed1997

Consequences of cranial vault expansion surgery for craniosynostosis.

Remodelling the cranial vault in an attempt to increase the intracranial volume and thus control intracranial hypertension, whilst at the same time improving the patient's appearance, has been the mainstay of surgery for syndromic craniosynostosis. We report a case of craniosynostosis in whom cranial vault expansion was followed by the development of hind-brain herniation and hydrocephalus. This prompted a review of our other cases of craniosynostosis who had been evaluated by magnetic resonance imaging following surgery in order to assess the frequency of hind-brain herniation and hydrocephalus in these children. Magnetic resonance imaging had been performed in the postoperative evaluation of 34 cases of craniosynostosis who had undergone procedures intended to increase the intracranial volume. The position of the cerebellar tonsils and the presence or otherwise of hydrocephalus was recorded for all cases. The effectiveness of surgery in treating raised intracranial pressure (ICP) was evaluated by means of postoperative ICP monitoring and had been performed in 22 cases. Herniation of the hind-brain below the level of the foramen magnum was observed in 18 cases (53%). Hydrocephalus, requiring the insertion of a ventriculoperitoneal shunt, was present in 14 cases (41%) and had developed after the cranial vault procedure in 9. The mean sleeping ICP measured postoperatively was normal (<10 mm Hg) in 5, borderline (10-15) in 7, and raised (>15 mm Hg) in 10 cases. Cranial vault expansion in complex craniosynostosis may fail to address the underlying aetiology of intracranial hypertension. Furthermore, both hydrocephalus and hind-brain herniation may develop following such surgery. Neither the increase in intracranial volume afforded by cranial vault expansion nor the shunting of hydrocephalus precludes the persistence of abnormal ICP. These findings are discussed in the light of possible mechanisms, in addition to cephalocranial disproportion responsible for intracranial hypertension in complex craniosynostosis. The implications for the surgical management of complex craniosynostosis are reviewed.

Acrocephalosyndactylia↗
PubMed1997

The long-term outlook for hydrocephalus in childhood. A ten-year cohort study of 155 patients.

Despite the fact that ventriculoperitoneal shunt insertion is the most commonly performed surgical operation in the pediatric neurosurgeon's repertoire, there is a surprising paucity of long-term outcome studies for these patients detailing either the complication rate over a predetermined time period or more importantly their intellectual outcome. The aims of this study, therefore, were to determine the 10-year outcome in a cohort of 155 children with shunted hydrocephalus, both in terms of the number and time sequence of shunt complications and also the long-term academic (schooling) outcome of these individuals. This was a cohort study of 155 hydrocephalic children who underwent first-time ventriculoperitoneal shunt insertion between the years 1978 and 1983, who were then followed up on an annual outpatient basis for a period of 10 years or until death. Their academic records and the surgical morbidity and mortality encountered over the 10-year study period were used as the main outcome measures. For those children surviving until schoolage, 59% were able to attend a normal school. The academic outlook for those children with hydrocephalus secondary to infection (postmeningitic) or intraventricular hemorrhage was less favorable with 52 and 60% requiring special schooling compared to those children with congenital hydrocephalus (29%; p = 0.036). 44% (68/155) of patients in this cohort did not require a shunt revision. The commonest reasons for shunt revision were blockage (49%) and infection (19%) which predominantly occurred within the first year of their original shunt procedure. Overall the infection rate was 12% (44/380 procedures). Furthermore an increased incidence of shunt infection was noted in those under 6 months old (p = 0.040). There was an 11 % mortality during the 10-year follow-up period for those with nontumor-related hydrocephalus.

Cerebral Hemorrhage↗
PubMed1997

The cervical spine in Saethre-Chotzen syndrome.

Twenty patients with a diagnosis of Saethre-Chotzen syndrome had their cervical spine radiographs reviewed. Radiologic abnormalities including vertebral fusion were present in 9 of the 20 patients. Fusion of both the vertebral bodies and the posterior elements was noted, although the latter site was more common. C2-3 was the level most commonly involved, although other levels were recorded. Analysis of sequential radiographs in nine patients revealed evidence of progression in seven patients. In those studies in children aged under 2 years, only 1 of 18 films showed evidence of fusion, while in those over 2 years of age, 10 of 12 showed evidence of fusion. These results reveal that the incidence of cervical anomalies in Saethre-Chotzen syndrome is greater than that in the general population. There is both direct and indirect evidence that the vertebral fusions are progressive during childhood.

Blepharoptosis↗
PubMed1997

Medulloblastoma: is the 5-year survival rate improving? A review of 80 cases from a single institution.

A series of 80 cases of medulloblastomas in children undergoing operation and postoperatively followed between 1980 and 1990 at Great Ormond Street Hospital for Children (GOSH) has been reviewed and compared to an earlier series reported from the same institution by McIntosh. The overall 5-year survival rate for the present series was 50%, although three patients died after surviving 5 years. The operative mortality rate was 5%. Survival analysis revealed that the presence or absence of spinal metastases and the necessity for some form of cerebrospinal fluid diversion within 30 days of the operation independently significantly affected survival in this series. Those patients with no spinal metastasis and total tumor removal had a 5-year survival rate of 73%, making this the most favorable subgroup in the series. Patient age and gender, duration of symptoms, Chang T stages, tumor volume, extent of resection, and postoperative chemotherapy were not significant variables. Although these results are better than those reported in the earlier GOSH series, they are not significantly different from the results of the second 5-year cohort of patients described in that article. Radiotherapy remains the greatest advance in treatment, although it is hoped that further improvement will result from the various chemotherapy protocols now being studied and from increasing knowledge of the biological behavior of these tumors.

Adolescent↗
PubMed1996

Ventriculo-peritoneal shunt requirement in children with posterior fossa tumours: an 11-year audit.

The authors reviewed 175 paediatric patients with posterior fossa tumours treated at the Hospital for Sick Children at Great Ormond Street, London, between 1983 and 1993, in an attempt to determine the requirement for shunt procedures in these children. The age of the patient population ranged from 4 months to 14 years 6 months. The factors evaluated included age at diagnosis, tumour type, extent of surgical resection, and the presence of CSF leak and/or infection. Shunt placement was required postoperatively in 18.9% of patients. Age of the patient was felt to be a significant factor in the need for shunt placement. Tumour type and completeness of tumour excision were also felt to be significant factors affecting the need for post-operative shunt insertion.

Adolescent↗
PubMed1996

Does the child with shunted hydrocephalus require long-term neurosurgical follow-up?

A prospective study was initiated to investigate parents' and doctors' attitudes towards how the follow-up of children treated with shunts for hydrocephalus should be undertaken. Twenty-three out of 85 (27%) of families whose children had been treated more than 6 months previously failed to attend for their clinic appointment compared with 15% of those attending for other (non-hydrocephalus-related) problems. The possible reasons for this discrepancy have been analysed together with the results of a questionnaire filled in by both the parents and the clinic doctors at the time of the appointment. Although both parties felt that there was a sizeable group of patients for whom continuing neurosurgical follow-up was no longer required there was little concordance in identifying who these patients actually were. Also taken into account were the economic factors which influence both parental and medical decision-making. In the light of our study, new ways of managing the follow-up of these children have been formulated.

Adolescent↗