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Aims and Objectives: Although syndromic craniosynostosis is one of the causes for early closure of the anterior fontanelle, there is no literature on the incidence and causes of fontanelle closure in isolated single-suture craniosynostosis. The objective of this study was to review the incidence of fontanelle closure by a wormian bone in isolated, nonsyndromic sagittal craniosynostosis., Materials and Methods: Intraoperative records of 100 consecutive children under 1 year of age, operated for isolated sagittal synostosis over a 14-year period (1987- 2000), were reviewed to identify the presence of a wormian bone closing the anterior fontanelle., Results: The median age at surgery was 4.2 months with a range of 1.9-11.7 months. Intraoperatively, a wormian bone was seen replacing the anterior fontanelle in 4 cases giving an incidence of 4%., Conclusions: A wormian bone can occupy the anterior fontanelle in children with isolated sagittal craniosynostosis giving the appearance of a 'closed fontanelle'., (Copyright 2006 S. Karger AG, Basel)

Introduction: Mutism after posterior fossa tumour resection is generally said to be transient. Our experience suggested that speech did not usually normalise, and that mutism was associated with neurologic deficits that did not recover fully., Methods: Children with mutism after posterior fossa tumour resection, and alive more than 2 years post-operatively, were reviewed retrospectively. Charts were reviewed and parents contacted to ascertain details about mutism, associated neurologic deficits, and the most recent speech and neurologic status., Results: There were 7 children, with follow-up ranging from 2.5 to 13.1 years (mean 6.8 years). Tumours were midline, with 4 astrocytomas and 3 medulloblastomas. Mutism was noted immediately after post-operative extubation in all patients. Speech reappeared 1-15 weeks post-operatively, except for 1 patient, who remained mute at 2.5 years. Speech returned to normal in only 1 patient. Mutism was always accompanied by new or worsened cerebellar ataxia, which resolved incompletely in the long term. Sixth nerve palsies occurred in 3 and recovered incompletely. Seventh nerve paresis occurred in 2 and recovered completely., Conclusion: Mutism after posterior fossa tumour resection is associated with other neurologic deficits, particularly ataxia. Whereas speech usually returns, contrary to general opinion, speech rarely normalises. Other associated deficits rarely resolve completely. These findings have significant implications for counselling of family and patients., (Copyright 2003 S. Karger AG, Basel)

The relationship of surgeon experience, measured by operative volume, to the outcomes of ventricular shunt treatment of hydrocephalus in children is not clear. This paper explores this relationship based on first ventriculoperitoneal shunts (VPS) implanted in English-speaking Canada during the period from April 1989 to March 2001. Three thousand seven hundred and ninety-four first VPS insertions, performed by 254 surgeons, were reviewed. Surgical experience was represented by the number of shunt operations performed during the study period by each surgeon prior to the date of the operation. The 6-month shunt failure risk for less experienced surgeons was 38%, compared to 31% for more experienced surgeons. This difference decreased to 4% at 60 months and 3% at 120 months (p = 0.001). The infection rate for initial shunt insertions was 7% for patients treated by more experienced surgeons and 9.4% for those treated by less experienced surgeons (p = 0.006). A relationship between surgeon experience and shunt outcome that appears to be based on the operative experience that a surgeon brings to a procedure is in keeping with clinical experience. This observation has implications for public policy, service planning and surgical mentorship during the earlier years of a surgeon's career., (Copyright 2003 S. Karger AG, Basel)

Little is known about the long-term prognosis of patients with cervical myelocystoceles and meningoceles. In this study, we report the long-term follow-up (average 10 years and 8 months) of 8 patients with cervical myelocystoceles and meningoceles. Neurologic, orthopedic, urologic and psychosocial status was assessed on long-term follow-up. The results indicate that in patients with cervical myelocystoceles, neurologic deficits become noticeable in the first year of life as the infant matures. Motor deficit was common in these patients, whereas sensory and urologic deficits did not occur. We believe that this is related to underlying myelodysplasia. In addition, patients with cervical myelocystoceles tend to have significant orthopedic problems. In contrast, patients with cervical meningoceles do not have neurologic deficits, but do develop mild orthopedic problems., (Copyright 2000 S. Karger AG, Basel)

Placode untethering in myelomeningocele patients can result in improvement and/or stabilization of neurological function, spinal curvature and pain. This paper reviews the outcome of untethering procedures in 24 patients to determine the frequency of subsequent functional deterioration. Decreased range of movement, joint stiffness and changes in muscle tone were the commonest indications for surgical intervention, occurring in 15 patients. Untethering resulted in improvement in 8 patients, stabilization of progression in 6 and continued deterioration in 1 patient. Two patients previously untroubled with spasticity became symptomatic within 3 months of the procedure. Changes in ambulation were present preoperatively in 9 patients. Stabilization was observed in 4 and improvement in 5. One of the patients, who had improved, deteriorated during the 1st year of follow-up. Alterations in bladder capacity and continence occurred in 7 patients. Improvement was seen in 2 patients, deterioration in 2 and no change in 3. Of the patients who improved, 1 subsequently deteriorated again within the 2nd postoperative year. Of those patients who had stable bladder function preoperatively, 6 subsequently deteriorated despite untethering. Pain was a less frequent symptom, occurring in 6 patients. Six patients became pain-free within 3 months of untethering. Two patients who did not have pain preoperatively had pain at the operative site persisting for up to 3 months postoperatively. Of the 20 patients having a single untethering procedure to date, 11 have further symptoms that can be attributed to retethering.

Objective: Prenatal ultrasonography can localize the level of the spinal cord malformation, allowing prediction of the potential postnatal neurological deficit and functional prognosis., Methods: This study has two evaluations: (a) a retrospective prenatal review of 26 fetuses with spinal dysraphism (1987-1991), and (b) a follow-up descriptive study of patients (1971-1981) who underwent closure of the spinal lesion and ventricular shunting in the neonatal period., Results: Prenatal ultrasound evaluation enabled the accurate definition of the last intact vertebral level which allows separation of fetuses into three functional groups (last intact level L2, L3-4, L5-sacral). Patterns of ambulation, urinary and bowel continence, and school performance vary according to level of spinal lesion and the neurological deficit. The need for ventricular shunts, the incidence of other spinal malformations and surgical interventions did not vary with the level of the spinal lesion., Conclusions: The functional outcome for patients with myelomeningocele is variable; however, distinct patterns emerge based on the level of spinal dysraphism and the resultant neurological deficit. By relating the level of the fetal spinal lesion to outcome data, more precise functional prognoses can be given to families.

Since 1969, the Meningomyelocele Clinic at British Columbia's Children's Hospital has been serving the needs of children with spina bifida. In 1994, the clinic provided urgent and routine multidisciplinary clinical assessments to 330 patients and families. The purpose of this study was to assess, from the patients' and families' perspective, the appropriateness of the frequency of routine clinic visits and the value of the health services provided. Sixty-five patients/families were interviewed and completed a questionnaire developed by the clinic. Current visit frequency and services provided appear to meet the patient/family needs. The medical and nursing contributions were seen as valuable resources for the patient/families resulting in most patient/families feeling well informed about spina bifida.

The requirements for radiographic imaging of the ventricles after a ventricular shunt procedure are not well defined. At British Columbia's Children's Hospital, the standard protocol included an ultrasound examination or CT scan at 6-8 weeks postoperatively, and a delayed CT scan at 6 months to 2 years, with additional scans only if the patient had symptoms of shunt malfunction. This study was performed to determine if the delayed scan could be omitted without compromising patient care. The study comprised 86 children with ventriculoperitoneal shunt operations, who had early CT scans or ultrasound examinations less than 20 weeks postoperatively, delayed postoperative CT scans between 20 weeks and 2 years, and who were asymptomatic at the time of these radiographic studies. In 39 of the 86 patients a change in ventricular size occurred between the early and delayed imaging studies, and in these patients the delayed scan was felt to be required. In patients in whom the early imaging study was done at more than 12 weeks postoperatively, and probably in patients with small ventricles preoperatively, there was no added information gained by doing a delayed scan. It is concluded that a radiographic examination of ventricular size at 12 weeks after a shunt operation may provide an adequate baseline study. If the early postoperative radiographic study is done less than 12 weeks after surgery, we would advise that in asymptomatic patients, with the exception of patients with very small ventricles preoperatively, a CT scan be repeated at a later date to obtain an appropriate baseline assessment of ventricular size for the future.

Acute subdural hematomas in the full-term neonate are usually thought to be a manifestation of birth trauma. Most commonly, these hematomas are associated with a tentorial tear, and less frequently are secondary to damage to the occipital sinus accompanying occipital osteodiastasis, or to rupture of bridging superficial cerebral convexity veins. We report 6 cases of acute subdural hematoma associated with cerebral infarction (SDH/CI) in term neonates, an entity which has not been well-documented previously. This combination accounted for half the cases of neonatal acute subdural hematomas referred to a pediatric neurosurgical service. All children presented within 72 h of birth, usually after an uneventful delivery, and typically with seizures (5/6), apneic spells (3/6) and a full or bulging fontanel (5/6). Although the patients were referred to the neurosurgical servive for management of a convexity subdural hematoma, the CT scans showed a large associated cerebral infarct, which was hemorrhagic in 5 of the 6 cases, most commonly in the distribution of the middle cerebral artery. Craniotomy was performed in 4 patients, needle aspiration of the subdural hematoma in 1, and no surgical intervention in 1. The outcome in 3 patients, who have been followed for more than 1 year, has been good, with some mild focal neurologic deficits, but no seizure activity. The pathogenesis of this entity is unclear, but the acute subdural hematoma in these cases is probably secondary to the cerebral infarction. Recognition of SDH/CI as a specific entity in term neonates has significant management and possibly medicolegal implications.

The classification and embryogenesis of cystic cervical dysraphic lesions are discussed in the light of the authors' experience and review of the literature. It is felt that these lesions are best described as meningoceles or myelocystoceles, and the use of the term 'myelomeningocele' may be more confusing than clarifying. The authors hypothesize that the cervical meningocele and the myelocystocele are part of a spectrum of the same underlying developmental abnormality, namely limited dorsal myeloschisis, with the eventual abnormality depending on the presence or absence of associated hydromyelia.

The morbidity associated with gross total removal of pediatric posterior fossa tumors is well recognized although it is rarely isolated from other factors that comprise the management morbidity for these tumors. This study reviews (1) the operative and postoperative complications in 105 patients and (2) the neurological morbidity in a subset of 91 patients undergoing gross total removal of their tumor between 1982 and 1992. Gross total removal was achieved in 102 patients with a single procedure. Two patients with residual tumor underwent early repeat craniotomy for excision and 1 is being followed without repeat resection. Intra- and postoperative complications occurred in 33 patients and included hematoma requiring craniotomy (3), gastrointestinal hemorrhage (2), hydrocephalus requiring shunt placement (9), wound problems (4), and pseudomeningocele formation requiring additional treatment (5). Delayed onset hydrocephalus requiring shunting occurred in 2 patients and spinal deformity in 4 patients. Worsening of preoperative deficit (new cranial nerve palsies, worsening ataxia, bulbar dysfunction including apnea, mutism and seizures) occurred in 41% of patients operated on for primitive neuroectodermal tumors (PNET) (14/34), 53% of ependymomas (10/19), and 30% of astrocytomas (15/50). No patient who had a choroid plexus tumor was worsened by the procedure. Complete recovery of new postoperative deficits occurred in 14% of PNET (2/14), 50% of ependymoma (5/10) and 47% of astrocytoma (7/15), most often within 6 months of the procedure. Residual neurological morbidity, due to persistence of preoperative symptoms or due to deficits that occurred as the result of the surgical procedure, was assessed in a subgroup of 91 patients followed for an average of 48 months (2-147 months). This assessment did not include morbidity due to adjuvant therapy. Sixty-two percent of patients continued to exhibit abnormal cerebellar or bulbar signs. Forty-three percent of the total population exhibited limitation in function due to residual deficit. Only 38% of patients were both functionally normal and had a normal neurological examination at last follow-up.

Ventricular asymmetry is common after ventricular shunting, and occasionally isolation of the contralateral ventricle necessitates a second shunt. The following study was performed to determine whether contralateral placement of the ventricular catheter via ultrasound-guided perforation of the septum pellucidum would decrease the incidence of postshunting ventricular asymmetry which might predispose to symptomatic isolation of a lateral ventricle. Fifty-eight children with hydrocephalus and an open fontanel, who were having a ventriculo-peritoneal shunt via a parieto-occipital approach, were randomized to have the ventricular catheter tip placed into either the ipsilateral or the contralateral frontal horn. Ventricular asymmetry occurred in 23% of the patients with contralateral versus 54% with ipsilateral placement (chi 2 = 5.53; p = 0.019). In 7 patients with contralateral placement, in whom a special catheter with two sets of holes was used, such that holes were located in both ventricles, no shunt-related ventricular asymmetry occurred. In 2 children with ipsilateral placement, a second shunt was required for a symptomatic isolated lateral ventricle. Perforation of the septum pellucidum with placement of the ventricular catheter in the contralateral ventricle, using a specially designed catheter with two sets of holes, may decrease the incidence of postshunting ventricular asymmetry and symptomatic isolation of a lateral ventricle.

Fifty consecutive children are described with spastic cerebral palsy treated with selective functional lumbar and sacral rhizotomy and followed for a minimum of 6 months. In all patients, spasticity improved postoperatively, but this was not necessarily accompanied by a functional improvement. Eighteen children who could not walk preoperatively were able to do so after rhizotomy. All 17 children who could walk preoperatively could do so following surgery, and in 15, gait was improved. Complications included transient urinary dysfunction in 4 children and sensory loss in 1. The operative procedure evolved with time: the technique of replacement laminotomy was refined; the electrophysiologic basis for selection of nerve rootlets changed after studies of nonspastic controls; smaller percentages of the L3 and L4 roots were sectioned in an attempt to prevent postoperative weakness of quadriceps, and there was a trend in the most recent patients to cut a smaller portion of all the posterior roots.

The technique for freeing adherent ventricular catheters is described in which a Bugbee wire, insulated except for the tip, is used to deliver a monopolar coagulating current to the tissue adherent to the drainage holes of the catheter. This may have some advantage over the use of an uninsulated metal cannula for delivery of a unipolar diathermy cutting current. The technique described has permitted effective and safe removal of adherent ventricular catheters.

A male child with type I (no neck) Klippel-Feil syndrome presented at birth with a transient and partial cord injury. Investigations failed to reveal spinal instability or foramen magnum compression. Subsequent deterioration led to investigations that demonstrated neuroschisis. A cervical meningocoele manque with cord tethering was found at exploration and untethering reversed the deficit. The surgical pathology of the cervical cord is described.

We describe a 15-month-old girl who presented with an acute hemiplegia. The sequence of events appears to have been clinically silent subarachnoid hemorrhage, vasospasm, infarction and a second lethal hemorrhage 3 months later. The old infarction was seen on computed tomography during her second illness. Autopsy confirmed the presence of a recent rupture of an intracranial aneurysm and old hemorrhage. In addition there was an unusual fibroblastic proliferation in the aneurysm wall. This case demonstrates that clinically silent subarachnoid hemorrhage, vasospasm and infarction can occur as complications of aneurysms, even in very young children.

Apnea is a known complication of the Chiari II malformation presenting in infancy. Obstructive apnea secondary to bilateral abductor palsy or laryngomalacia and centrally mediated expiratory apnea with cyanosis can occur. Observations of 9 patients suggest that these forms of apnea may represent stages in a continuum of brain stem dysfunction due to the combined effects of the hindbrain malformation and its compression, hydrocephalus and progressive arachnoiditis. Obstructive apnea in some patients may be reversed by optimal control of hydrocephalus with or without cervical decompression. These patients may also develop episodes of cyanotic expiratory apnea of central origin (PEAC). This form of apnea does not respond to surgical or medical treatment and may show progressive worsening over time. Five of 6 patients with this form of apneic spell died suddenly, 2 of these died despite full recuscitative efforts. It is recommended that reports of treatment address results for both forms of apnea.