Pattern, management and outcome of cervical spine injuries associated with head injuries in paediatric patients.

INTRODUCTION: This study examines the management and outcome of cervical spine injuries in children with head injuries, to assess the need for surgical treatment. MATERIAL AND METHODS: We performed a retrospective analysis (1995-2005) of 445 children admitted intubated and ventilated to the intensive care unit with head injuries. OUTCOME MEASURES: Frankel grade for spinal injuries and Glasgow Outcome Scale (GOS) for head injuries. RESULTS: Cervical spine injuries were detected in 11 patients (incidence 2.5%, mean age: 6.3 years, range: 21 months-15 years). The injuries were: C1/2 distraction: 2; C1/2 subluxation: 2; odontoid peg fracture with C1/2 dislocation and cord transection: 1; disruption of posterior longitudinal ligament at C2: 1; odontoid peg fracture with C2/3 distraction: 1; C2/3 subluxation: 1; C3 lamina fracture: 1; C3/4 facet fracture: 1; C6/7 fracture dislocation with cord transection: 1. One patient was managed operatively, ten patients nonoperatively, two with halo vests and eight with hard collars. There were three deaths (mortality 27%) associated with severe head injuries. At 6 months follow-up, two patients remained quadriplegic (Frankel Grade A), one of them ventilator dependent, one had residual motor function but of no practical use (Frankel Grade C), five had good spinal outcome (Frankel Grade E). Seven patients had good head injury outcomes (GOS 5), one remained disabled (GOS 3). CONCLUSION: Most children with cervical injury can be managed nonoperatively with good outcomes. Surgical management may be necessary in severe unstable injuries.

Incidence of vascular malformations in spontaneous intra-cerebral haemorrhage in children.

AIM: To assess the incidence of vascular malformations in children presenting with non-traumatic intra-cerebral haemorrhage and outline the need for radiological investigations. MATERIALS AND METHODS: Between 1993 and 2002, 26 children presented with spontaneous intra-cerebral haemorrhage at a mean age of 26 months (range 1-192 months). RESULTS: Twenty-two children had, as first treatment, surgical removal of the haematoma and any malformation found. One patient with no evidence of vascular malformation had conservative treatment, two had embolisation and one had stereotactic radiosurgery of arteriovenous malformations (AVMs). The diagnosis of vascular malformation was confirmed histologically and/or radiologically in 16 (61%) patients. Of these 16 patients, 7 were AVMs, 1 thrombosed middle cerebral artery (MCA) aneurysm, 1 cavernous angioma, 6 aggregates of abnormal vessels, 1 vein of Galen aneurysm. Pre-operatively, 12 patients had magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and 7 were positive for vascular malformation (1 false positive) with no false negatives. Digital subtraction angiography (DSA) was performed pre-operatively in 7 patients, 4 were positive with no false negatives. There were no re-bleeds at the follow-up period. Five (19%) patients died from the haemorrhage, 7 (27%) had severe neurological deficit and 14 (54%) had no neurological deficit. Residual malformation after surgery requiring additional treatment was found in 3 (16%) patients. All patients with confirmed malformations were followed-up with DSA. CONCLUSION: MRI/MRA has high sensitivity and specificity in identifying vascular malformations in children presenting acutely with spontaneous intra-cerebral haemorrhage. This may prove useful when pre-operative DSA is not promptly available. After acute clot evacuation, there is high incidence of residual malformation and such patients should be followed-up with DSA.

Diastematomyelia in children: treatment outcome and natural history of associated syringomyelia.

OBJECTIVE: To quantify the long-term outcome of children with diastematomyelia and the implication of syringomyelia in the natural history. MATERIALS: Retrospective study of 17 children (nine girls and eight boys) with diastematomyelia operated during 1989-2004. Mean age at diagnosis was 3.4 years (range 5 days-12 years), mean follow-up was 5 years; 14 had excision of spur and cord untethering, 3 had excision of spur alone. RESULTS: The bony spur was in the lumbar region in 12 and thoracic in 5 patients. Syringomyelia was present in eight (47%), associated with bony spur between L1 and 4 (p=0.088), spina bifida occulta in ten (58.8%), and spinal lipoma in one (6%). Preoperative Necker Enfants Malades (NEM) scores were 17 in eight patients, 16 in four, 15 in three, 13 in one, and 12 in one patient. Presenting neurological deficits were motor in eight, sensory deficits in three, and anal incontinence in one patient. There was improvement of NEM scores postoperatively in five patients only, but still with residual deficit. Repeat spur excision and cord untethering was performed in three patients for neurological deterioration 1-4 years after first operation. On postoperative MRI scans syringomyelia remained unchanged in all eight patients. There were one skin infection, one transient motor deterioration, and one patient with sensory deficit after surgery. CONCLUSION: Prophylactic operations were associated with the best clinical outcome. Despite improvement, all patients with established preoperative deficit still had residual neurological deficits at their last follow-up. The associated syringomyelia remained unchanged after surgery, indicating that it does not contribute to the neurological syndrome.

Outcome of children with posterior fossa medulloblastoma: a single institution experience over the decade 1994-2003.

AIM: While the impact of radiotherapy in the management of medulloblastoma was recognised, the introduction of chemotherapy was investigated in clinical trials and shown to confer an additional advantage. We reviewed the outcome of a series of consecutive patients to assess the impact in a population-based clinical establishment. MATERIALS AND METHODS: A series of 38 children treated for medulloblastoma at Birmingham Children's Hospital between 1994 and 2003 was analysed. The effect of surgery, radiotherapy, chemotherapy and metastasis on survival was analysed. RESULTS: The overall 5-year survival rate was 61.4% for the 36 patients who had resective surgery, while 2 patients had biopsy only and died within a few months. There was no operative mortality. The incidence of hydrocephalus needing permanent shunting was higher in the first 3 years of life (p = 0.007, chi-square). The 5-year survival rate of patients with total and sub-total excision of medulloblastoma was 61.1% and 61.8%, respectively. The 5-year survival rate of patients older than 3 years was 73.4% and for patients under 3 years was 36.3% (p = 0.007, log rank). Metastases at presentation did not influence survival. All deaths occurred in the first 32 months. CONCLUSION: The contribution of chemotherapy in the improvement of the overall survival appears more evident in children younger than 3 years or presenting with metastases. The absence of significant difference in survival between patients with total or sub-total excision of medulloblastoma supports the view that total excision of medulloblastoma can be avoided when the risk for potential intra-operative damage and consequent neurological deficits is high.

Cranioplasty in children.

OBJECTIVE: The objective was to assess the outcome and complications associated with different cranioplasty implant materials in children. MATERIALS AND METHODS: A retrospective review was conducted of 28 consecutive cranioplasties carried out on 24 children between 1994 and 2001 (age range, 9 months to 15 years; minimum follow-up 18 months). The indications were: defect from previous craniectomy for trauma, tumour, infection or evacuation of haematoma (n=21), intradiploic dermoid cysts (n=2), growing fractures (n=4) and residual bony defect following craniofacial reconstruction (n=1). The materials used were: patient's craniectomised bone flap (n=16), split calvarial graft (n=8), acrylic (n=3) and titanium (n=1). All patients were assessed for bony fixation, cosmesis, wound healing and flap infection. RESULTS: There was no mortality and 18% morbidity (n=5: 3 infected flaps, 1 sterile wound dehiscence and 1 sterile wound discharge; overall infection rate 10%). Out of the 14 patients who had their own craniectomised bone flaps implanted initially, 3 became infected (2 in patients with bilateral defects) necessitating flap removal. Two of these were successfully re-implanted. No donor or recipient bone flap complications were seen in the 8 split calvarial grafts, wound discharge was seen in 1, requiring wound toilet. No complications were seen with acrylic or titanium cranioplasties. CONCLUSION: In this series, the use of the patients' own craniectomised flap had a low infection rate, and was mainly seen in patients who had bilateral flaps re-implanted soon after removal. There were no complications arising from the use of split calvarial and allograft material. Use of autologous implant material should be preferred whenever possible due to obvious resource and biological advantages, and can even be re-implanted if infected.

Percivall Pott and the miners of Cornwall.

Percivall Pott (1714-1788) was an astute and prolific mid-eighteenth century English surgeon with a strong interest in head injury. He was partial to the use of the trephine for all skull fractures and favoured early exploratory trephination when an intracranial collection could not be ruled out. If haemorrhage or abscess was beneath the dura, it was incised. Later in eighteenth century England and France, trephination fell out of favour. This was especially so in the crowded hospitals where putrid conditions were common. Surprisingly, on the remote southwestern English peninsula of Cornwall, where compound depressed skull fractures in miners were a frequent occurrence, Pott's principles prevailed unchanged. Generations of surgeons trephined early in suspected cranial fractures, without waiting for symptoms of compression or irritation. A large percentage of patients recovered. Those who died generally had diffuse injury, brain laceration, or a fractured base. The Atlantic breezes were believed beneficial to recovery. The operation of early 'preventive' trephination continued in the Cornish mining districts through the 1870s, when antiseptic methods were gradually adopted as elsewhere in England. Head injuries in miners became less common as mining reforms were introduced and head protection improved.

Cerebral abscess associated with a pulmonary arteriovenous malformation in a patient suffering from alpha thalassaemia.

We report the case of a young girl suffering from alpha thalassaemia and presenting with a brain abscess caused by a silent pulmonary arteriovenous malformation. To our knowledge this is the first such case associated with alpha thalassaemia. Diagnostic investigations and treatment options are discussed.

Spinal sagittal malalignment following surgery for primary intramedullary tumours in children.

BACKGROUND/OBJECTIVE: As prior studies analysed predictive factors for various post-laminectomy spinal deformities in mixed spinal regions, age groups or pathologies, their validity and conclusions were unclear. The objective of this study was to determine predictive factors for worsened cervical or thoracic spinal sagittal alignment following laminectomy or laminotomy for primary intramedullary spinal cord tumours in children. METHODS: In this retrospective study, patients treated between 1980 and 1998 were reviewed. Changes in spinal alignment at the last follow-up compared to the pre-operative state were studied. Factors analysed were age, pre-operative spinal alignment, procedure types (laminectomy or laminoplasty), number of laminae operated, surgery of C2 or T1 laminae, histological grade, presence of post-operative neurological deficit and post-operative radiotherapy. RESULTS: There were 27 patients. The mean age was 5.6 years (range 1.3-14.0 years), and the mean duration of follow-up was 3.7 years (range 0.075-9.9 years). In the cervical-cervicothoracic surgical group (n = 12), alignment worsened post-operatively in 3 patients. The number of laminae operated upon had a statistically significant impact on the development of post-operative kyphosis (p = 0.07). In the thoracic-thoracolumbar surgical group (n = 15), alignment worsened in 9 patients. Procedure types were statistically significantly different, with laminectomy associated with an increased risk of post-operative kyphosis (p = 0.01). All 5 patients who had spinal fusion for worsened post-operative alignment were in the thoracic-thoracolumbar group; no patients in the cervical-cervicothoracic group required spinal fusion (p = 0.047). Other predictive factors did not reach statistical significance (p > 0.05). CONCLUSIONS: Worsened spinal sagittal alignment following laminectomy or laminoplasty and the need for spinal fusion is more common in the thoracic-thoracolumbar region than in the cervical-cervicothoracic region. In the cervical-cervicothoracic region, operation on a greater number of laminae tends to increase the risk of worsened alignment. In the thoracic-thoracolumbar region, laminectomy is associated with worsened alignment, while laminoplasty reduces this risk; also, pre-operative kyphotic deformity tends to increase the risk of worsened alignment post-operatively.

Outcome of surgical treatment for subdural fluid collections in infants.

We reviewed retrospectively the management of 47 infants with subdural collections at the Birmingham Children's Hospital. Of those patients who had needle aspiration as their initial treatment 42% required some form of further treatment and 25% were complicated by infection of the subdural collection. Of those patients treated with burr hole evacuation or with observation alone, 78 and 73%, respectively, required no further procedures. However, 17% of those who had burr holes as their initial treatment developed post-operative infected collections. Of those who had subdural-peritoneal shunt as initial or subsequent treatment, none required further procedures to treat the subdural collections. No infections were noted following shunting. There was no statistically significant difference in overall clinical outcome at last follow-up between the different treatment groups. We conclude that needle aspirations should be avoided due to high infection risk, and that observation alone can be sufficient in the absence of intracranial hypertension, as the condition may be self-limiting. In cases requiring surgery, it should be borne in mind that burr hole evacuation has a higher infection risk, whereas subdural shunting has the additional need for removal of the device if the parents and surgeon decide accordingly.

Thoraco-lumbar duplication of the spine. Case report and embryology review.

OBJECT: We present a case of an asymptomatic and neurologically normal 6-year-old girl who was noted to have a gross spinal abnormality. METHODS: Neuroimaging demonstrates gross thoraco-lumbar spine duplication, which is at the severe end of the split cord malformation (SCM) group of congenital abnormalities. Despite the position of the neural structures, the clinical condition has so far not suggested that any surgical intervention is indicated, though the girl's later growth spurts may still unmask features of a tethered spinal cord. CONCLUSIONS: We present a brief review of the literature dealing with theories of embryogenesis relating to SCM, the common clinical and radiological features, and finally the surgical options available.

The treatment of infantile hydrocephalus: "differential-pressure" or "flow-control" valves. A pilot study.

The choice of shunt valve in the treatment of hydrocephalus in children remains controversial. We embarked on a pilot study to determine the differences in outcome between differential-pressure and flow-regulating valves. Prospective data collected on 50 consecutive first-time shunt insertions, performed between June 1993 to June 1996, was analysed. Children with tumour-related hydrocephalus and Dandy-Walker malformations as well as children who had external ventricular drainage prior to definitive shunt insertion were excluded from the study. The defining event was the first complication necessitating surgery, including obstruction, over-drainage and infection. Of the 50 children (31 males), 23 had differential pressure (medium-pressure) and 27 had Delta (performance level 2) valves inserted. The mean age at shunt insertion was 26.4 months. The mean follow-up was 53.8 months. The overall cumulative shunt survival at 5 years was 58.6% for the differential pressure and 58.7% for the Delta valves. The mean shunt life was 37.1 months for the differential pressure group and 34.6 months for the Delta group. This difference was not statistically significant (P=0.72, t-test). Both valves had a similar outcome with respect to obstruction (including proximal, valve, distal). The main differences between the two valves were with respect to the incidence of over-drainage and infection. Amongst the differential pressure valves, there were 4 instances of overdrainage (3 slit-ventricle syndrome, 1 bilateral subdural collection)--all occurring within the first 36 months. The Delta valve group had only one instance of over-drainage (bilateral subdural collection). There were no infections in the differential pressure valve group, whereas 3 of the Delta valve shunts got infected, all within the first month. Whereas both shunt types seemed to have a similar overall survival, there was a relatively higher incidence of over-drainage amongst the differential pressure valves. The Delta valves, on the other hand, had higher rates of infection. Similar studies with larger numbers could suggest whether the choice of shunt type will ultimately have to be a compromise accepting one or the other complication.

Intracranial volume change in childhood.

OBJECT: The goal of this study was to construct a model of normal changes in intracranial volume occurring throughout childhood from age 7 days to 15 years. METHODS: Using the technique of segmentation on magnetic resonance images obtained in healthy children, intracranial volume was measured and plotted against age. CONCLUSIONS: Intracranial volume in the first few months of life is on average 900 cm3 in males and 600 cm3 in females. By the age of 15 years, it increases up to 1500 cm3 in males and 1300 cm3 in females, increased by factors of 1.6 and 2.1, respectively. By the time the child reaches 2 years of age, intracranial volume has reached 77% (1150 cm3 in males and 1000 cm3 in females) and, by 5 years, 90% (1350 cm3 in males and 1200 cm3 in females) of the volume observed at age 15 years. The change in intracranial volume that occurs with age is not linear, but there seems to be a segmental pattern. Three main periods can be distinguished, each lasting approximately 5 years (0-5, 5-10, and 10-15 years), during which the growth of intracranial volume is linear. Throughout childhood, males have higher intracranial volumes than females, with a similar growth pattern.

Intracranial volume change in craniosynostosis.

OBJECT: There is still controversy regarding the optimum time to perform surgery for craniosynostosis. Some recommend surgery soon after birth and others delay until the age of 12 months. Intracranial pressure has been measured in an attempt to provide a scientific rationale, but many questions remain unanswered. To date, little attention has been given to intracranial volume and its changes during the first few years of life in children with craniosynostosis. The authors' goal was to focus on intracranial volume during this period and to compare measurements obtained in patients with craniosynostosis with measurements obtained in healthy individuals. METHODS: Using the technique of segmentation, the intracranial volume of 84 children with various forms of craniosynostosis was measured on preoperative computerized tomography scans. The change in average volume that occurs with increasing age was calculated and compared with a model of normal intracranial volume growth. The age at presentation for children with craniosynostosis was 1 to 39 months; 76% of the patients were younger than 12 months. In eight patients in whom only one cranial expansion procedure was performed, postoperative intracranial volumes were measured as well. Several interesting observations emerged. 1) There was little difference in head growth between boys and girls with craniosynostosis during the first few months of life. After the age of 12 months, however, the difference in intracranial volume normally seen between the two genders was observed in the craniosynostosis group as well. 2) Excluding children with complex pansynostosis, who have smaller heads, children with all other types of craniosynostosis have similar head growth after the 1st year of life, with no difference between the number of and type of suture affected. Children with Apert's syndrome develop greater than normal intracranial volumes after the 1st year of life. 3) Although children with craniosynostosis are born with a smaller intracranial volume, by the age of 6 months volume has reached normal levels, and from that point on volume follows the pattern of normal head growth. 4) Children who presented after the age of 6 months and later developed recurrent craniosynostosis after initial successful treatment had a small intracranial volume at their initial presentation. 5) Of the patients whose postoperative intracranial volumes were measured, all but one had preoperative volumes at or above normal values, and their postoperative volumes were considerably higher than normal for their age. These children all followed a growth curve parallel to that of healthy children but at higher volume value. One patient with a smaller-than-normal initial intracranial volume was surgically treated at a very young age and, despite cranial expansion surgery, postoperative volume did not reach normal levels. It is postulated that this was due to the fact that the operation was performed at a time when craniosynostosis was still active. CONCLUSIONS: The results of this study indicate that the underlying mechanism leading to craniosynostosis and constriction of head volume "exhausts" its effect during the first few months of life. Measurement of intracranial volume in clinical practice could be used to "fine tune" the optimum time for surgery. In late-presenting children, this may be useful in predicting possible recurrence.

Pediatric oncology and hematology in Birmingham, England.

The hematology and oncology service at Birmingham Children's Hospital was established in the late 1960s and now is one of the largest in the United Kingdom. It provides comprehensive care for the entire range of childhood malignancies, coagulation disorders, and hemoglobinopathies and other hematological disorders, and undertakes bone marrow transplant and megatherapy/peripheral blood stem cell procedures. Research includes clinical trials of treatments of childhood cancers; molecular biology studies on leukemia, Hodgkin's disease, neuroblastoma, and sarconas; childhood cancer epidemiology, and geographical and racial incidence; and treatment of hemophilia and molecular investigation of coagulation disorders. These activities involve collaboration with local, national, and international research groups.

Skull base growth in childhood.

While studying skull base changes in craniosynostosis, it became apparent that there is a lack of reference studies quantifying the changes of three-dimensional (3D) parameters of the normal skull base throughout childhood. Using advanced 3D visualisation techniques, 34 points of the skull base were identified on MRI scans of 66 normal children, aged 1 month to 15 years. Several distances and angles between the various landmarks were measured in an attempt to quantify the growth of skull fossae with age. Two main growth periods were observed: before and after the first 5 years of life. Most change occurred in the first period. Anatomical regional differences were identified between the two sexes. During the first 5 years of life, the anterior fossa showed rapid growth rate with respect to its anterior projection in males, whereas in the females there was a more concentric growth pattern. The body of the sphenoid bone and the middle fossa showed a rapid growth rate in both sexes which was greater in the females. The posterior fossa showed a concentric pattern of growth in both sexes with a greater growth rate in the females. These findings provide new insight into the pattern of growth of the various parts of the skull base and can be used for comparative study of deformities that affect such growth.

Skull base growth in craniosynostosis.

Although considerable scientific work has been published on the role of the skull base in craniosynostosis, the changes with age throughout childhood have not been fully outlined. The realisation that little attention has been paid to the posterior skull in craniosynostosis, resulted in renewed interest in skull base growth. The availability of computer-based image analysis provides a new accurate method of study in three dimensions. Using three-dimensional visualisation techniques, 34 points of the skull base were identified on CT scans of 50 children with craniosynostosis of various types, aged from 1 month to 5 years. Several distances and angles between the various landmarks were measured in an attempt to quantify the growth of skull fossae with age. Comparisons were made with normal controls. In children with craniosynostosis, the anterior fossa was overdeveloped in the males, whereas in the females remained underdeveloped throughout the first 2 years of life. The body of the sphenoid showed moderate underdevelopment in the first 2 years in both sexes, the effect being more prominent in the males. The middle fossae showed overdevelopment in both sexes in the first 2 years of life. The posterior fossa was underdeveloped in both sexes in the first 2 years of life, the effect being more prominent in the females. Craniosynostosis seems to affect both sexes to a similar degree, but there are regional differences in the growth pattern. Better understanding of the normal growth pattern of the skull base and the effect of craniosynostosis upon it may assist our approach to surgical treatment and in particular the role of anterior and posterior skull expansive surgery.

Computer simulation of a neurosurgical operation: craniotomy for hypothalamic hamartoma.

Although magnetic resonance imaging has revolutionised the management of intracranial lesions with improved visualisation of anatomical structures, it only produces two-dimensional images, from which the clinician has to extrapolate a three-dimensional interpretation. Several approaches can be used to create 3D images; the discipline of image segmentation has encompassed a number of these techniques. Such techniques allow the clinician to delineate areas of interest. The resulting computer-generated outlines can be reconstructed in a three-dimensional arrangement. Although a plethora of "generic" segmentation techniques exist, we have developed a refined form, dependent on general and particular properties of the anatomical structures under investigation. High-contrast structures such as the ventricles and external surface of the head are found by using a localised adaptive thresholding technique. Less definable structures, with poor or nonexistent signal change across neighbouring structures, such as brain stem or pituitary, are found by applying an "energy minimisation"-based technique. To demonstrate the techniques we used the example of an 8-year-old boy with uncontrolled gelastic seizures due to a hypothalamic hamartoma, who is being considered for surgery. We were able to demonstrate the anatomical relationships between the hypothalamic hamartoma and adjacent structures such as optic chiasm, brain stem and ventricular system. We were subsequently able to create a video, reproducing the stages of craniotomy for excision of this tumour. By creating true 3D objects, we were able at any stage of the simulation to visualise structures situated contralaterally to the approaching surgical dissector. These 3D representations of the structures can be either invisible or opaque, in order to afford 3D localisation as the "virtual" surgical dissection proceeds. The clinical application of such techniques will enable surgeons to improve their understanding of anatomical relations of intracranial lesions and has obvious implications in image-guided surgery.