Upper limb motor function in young adults with spina bifida and hydrocephalus.

OBJECTIVE: The objective of the study was to measure upper limb motor function in young adults with spina bifida meningomyelocele (SBM) and typically developing age peers. METHOD: Participants were 26 young adults with SBM, with a Verbal or Performance IQ score of at least 70 on the Wechsler scales, and 27 age- and gender-matched controls. Four upper limb motor function tasks were performed under four different visual and cognitive challenge conditions. Motor independence was assessed by questionnaire. RESULTS: Fewer SBM than control participants obtained perfect posture and rebound scores. The SBM group performed less accurately and was more disrupted by cognitive challenge than controls on limb dysmetria tasks. The SBM group was slower than controls on the diadochokinesis task. Adaptive motor independence was related to one upper limb motor task, arm posture, and upper rather than lower spinal lesions were associated with less motor independence. CONCLUSIONS: Young adults with SBM have significant limitations in upper limb function and are more disrupted by some challenges while performing upper limb motor tasks. Within the group of young adults with SBM, upper spinal lesions compromise motor independence more than lower spinal lesions.

Spontaneous intraparenchymal hemorrhage in full-term neonates.

OBJECTIVE: Spontaneous intraparenchymal hemorrhage is extremely rare in full-term newborns. Reports to date have been limited to descriptions of individual cases, small groups within larger studies of intracranial hemorrhage, and one series of four patients. Structural lesions are rarely identified, and the majority of patients described have been managed without surgical intervention. METHODS: Analysis of a computerized database of pediatric neurosurgical patients from January 1960 to February 2000 identified full-term newborns younger than 3 months of age with nontraumatic intraparenchymal hemorrhages. Prenatal histories, labor and delivery histories, clinical presentations, imaging studies, management, and outcomes were reviewed. RESULTS: Eleven full-term newborns with spontaneous intraparenchymal hemorrhages were identified. The majority had normal prenatal courses. Most presented within the first 2 days of life (6 of 11 patients), and the most common presenting sign was seizure (7 of 11 patients). No cause was identified in 6 of 11 patients; the remainder were attributed to coagulopathy (n = 3), ruptured intracranial aneurysm (n = 1), or hemorrhagic infarction (n = 1). Eight patients underwent surgical hematoma evacuation on the basis of radiographic evidence of significant mass effect, evidence of signs of elevated intracranial pressure, or both. Three patients did not receive surgical intervention. There were no subsequent hemorrhages or deaths during a mean follow-up period of 4.5 years (range, 1-16 yr). Four patients had normal neurological outcomes, four had motor deficits (one of whom additionally demonstrated cognitive delay), and three had delayed speech. CONCLUSION: No cause is identified in most newborns with spontaneous intraparenchymal hemorrhage. Radiographic evidence of mass effect or signs of elevated intracranial pressure may necessitate surgical hematoma evacuation. Outcome varies widely and may be normal, even in patients with sizeable intraparenchymal hemorrhages.

Cerebellar arteriovenous malformations in children.

We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome.

How children with head injury represent real and deceptive emotion in short narratives.

Narratives are not only about events, but also about the emotions those events elicit. Understanding a narrative involves not just the affective valence of implied emotional states, but the formation of an explicit mental representation of those states. In turn, this representation provides a mechanism that particularizes emotion and modulates its display, which then allows emotional expression to be modified according to particular contexts. This includes understanding that a character may feel an emotion but inhibit its display or even express a deceptive emotion. We studied how 59 school-aged children with head injury and 87 normally-developing age-matched controls understand real and deceptive emotions in brief narratives. Children with head injury showed less sensitivity than controls to how emotions are expressed in narratives. While they understood the real emotions in the text, and could recall what provoked the emotion and the reason for concealing it, they were less able than controls to identify deceptive emotions. Within the head injury group, factors such as an earlier age at head injury and frontal lobe contusions were associated with poor understanding of deceptive emotions. The results are discussed in terms of the distinction between emotions as felt and emotions as a cognitive framework for understanding other people's actions and mental states. We conclude that children with head injury understand emotional communication, the spontaneous externalization of real affect, but not emotive communication, the conscious, strategic modification of affective signals to influence others through deceptive facial expressions.

Management and outcomes of posterior fossa subdural hematomas in neonates.

OBJECTIVE: To review and analyze a contemporary series of 15 neonates who were treated for posterior fossa subdural hematomas (PFSDHs) during the era of computed tomography and magnetic resonance imaging. METHODS: A retrospective chart review identified all neonates with PFSDHs for whom neurosurgical consultations were obtained for treatment planning. RESULTS: There were nine male and six female patients. The mean gestational age was 39 weeks. Nine of the 15 mothers of the patients were primiparous. Instrument-assisted delivery (forceps and/or vacuum extractor) was undertaken for seven patients. The mean birth weight of the infants was 3165 g (range, 2160-3930 g). The mean 5-minute Apgar score was 7.5. Symptoms of PFSDH developed within the first 24 hours of life in 13 neonates. The predominant symptoms and signs were failure to thrive, irritability, seizures, apnea, and bradycardia. Lumbar punctures to rule out central nervous system sepsis were performed in six neonates. Hemograms revealed that six neonates were anemic with low hemoglobins, five had low platelets, and four had abnormal prothrombin and/or partial thromboplastin times at the time of diagnosis. Computed tomography established the diagnosis of PFSDH in all cases. Magnetic resonance imaging was performed for two neonates. The median time to diagnosis by imaging studies was 10 hours after birth. Surgical evacuation of the PFSDHs was performed in eight neonates. Seven neonates were followed conservatively with serial imaging studies. There was no mortality in either treatment group. Follow-up ranged from 2 to 10 years, with a mean of 4.5 years. Functional outcome assessment revealed that seven neonates were neurodevelopmentally normal, three were mildly delayed, two were moderately delayed, and three were profoundly delayed. In addition to traumatic causes of the PFSDHs, three neonates were observed to have coagulation disturbances at birth and one was observed at follow-up to have a posterior fossa medulloblastoma that had bled at birth. CONCLUSION: PFSDHs are rare but important lesions to diagnose early in the neonatal period. Surgery can be life-saving when performed in a timely manner for signs and symptoms of brain stem dysfunction. A search for an underlying cause predisposing to a PFSDH may, on occasion, reveal a coagulation disturbance or a neoplasm that will require additional therapeutic considerations.

Long-term survival of an infant with 'anaplastic' astrocytoma.

Anaplastic astrocytomas are intermediate in differentiation between astrocytoma and glioblastoma multiforme. Survival with anaplastic astrocytoma is favorably affected by extensive anaplasia, maximal resection and presentation in early life. We report a 2-month-old infant who had a tumor of astrocytic lineage with anaplastic features of necrosis, nuclear atypia and mitotic activity. Following subtotal resection the child is alive but has a radiologically visible tumor.

Endovascular management of paediatric intracranial aneurysms.

SUMMARY: The purpose of this paper is to discuss the radiological and clinical features of paediatric intracranial aneurysms including endovascular techniques currently being used for their therapy. Paediatric patients who presented with the diagnosis of intracranial aneurysm between August 1982 and February 1997 were included. The data were collected retrospectively. Clinical features included patient's age, sex, presentation, treatment and outcome. Radiologic features included location, size and number of aneurysms present. 21 patients (13 females and 8 males) ranging in age from 9 months to 16 years had intracranial aneurysms. Seventy six percent of the aneurysms were located in the anterior circulation and 24% in the posterior circulation. Seven patients (33%) had giant aneurysms and three patients (14%) had multiple aneurysms. Endovascular treatment was performed in six patients (29%) and neurosurgical treatment in eight patients (38%). Endovascular treatment included the use of coils and/or balloon occlusion. Of the remaining patients, two (9.5%) died prior to any surgical or radiological intervention. A further five patients (24%) were treated conservatively. Intracranial aneurysms in children demonstrate clinical and radiologic features that differ significantly from those seen in adults. There is a higher percentage of ICA termination aneurysms, giant aneurysms and posttraumatic aneurysms. However, aneurysms in children, like those in adults, can and have been successfully treated utilizing endovascular as well as neurosurgical techniques.

Choices in the 1990s for the management of pediatric cerebral arteriovenous malformations.

A retrospective 45-year analysis of the management of 160 children with intracranial arteriovenous malformations at The Hospital for Sick Children, Toronto, reveals substantially improving outcomes which relate to more efficient diagnoses and treatments. 80% of children will declare their malformation by means of spontaneous intracranial hemorrhage. For those children who present with hemorrhage or epilepsy, 80% will require an operation. The overall mortality rate has declined to 12% since 1975 and that for the cerebellar lesions from 67 to 42%. 53% of the patients operated upon will be neurologically normal. Endovascular embolization of a child's AVM is a customized, partial solution for a limited number of children. Stereotactic radiosurgery will be used increasingly to obliterate those small lesions in children which are unassociated with hemorrhage or are the residua of an operation. As many as 10% of children (15/160) with diagnosed AVMs cannot be helped with operative or other interventional therapies. The recognition of the pediatric stroke syndromes, the early triage and diagnosis of a child's cerebral hemorrhage, the operative and anaesthetic technologies and the adjunct therapies - choices of the nineties - have resulted in a 66% decline in the overall mortality from this vascular lesion as well as greater assuredness that for most the lesion can be permanently obliterated.

Supratentorial primitive neuroectodermal tumors in children.

A retrospective review of 36 children diagnosed with a supratentorial primitive neuroectodermal tumor (PNET) at the Hospital for Sick Children was performed for the period 1970-1995. All children but one received their initial treatment at our institution. There were 18 males and 18 females and the median age at diagnosis was 35 months. Twenty-two PNETs were lobar, 3 were deep in the hemisphere, and 10 were located in the pineal region. One child presented with intracranial leptomeningeal disseminated disease. The tumors were mostly undifferentiated although 22 had some evidence of differentiation along one or more neuroepithelial lines. Five children had a biopsy, 24 had subtotal resection, and 7 had gross total resection. Twenty-six children had adjuvant radiotherapy and 13 had chemotherapy. At last follow-up 30 patients were dead and 6 were alive. The median survival was 23 months and the 2, 3, and 5 year survivals were 50%, 34%, and 18% respectively. All of the survivors received craniospinal radiation and 4 received chemotherapy. There was a statistically significantly worse survival in young children. There was a trend to better survival in children treated since 1984, and in children undergoing gross total resection. Because of the extremely poor survival, we recommended that all children undergo gross total resection followed by chemotherapy. For children older than 3 years of age craniospinal radiation should also be given.

The detection and management of intracranial hypertension after initial suture release and decompression for craniofacial dysostosis syndromes.

We performed a retrospective study of 107 consecutive patients with syndromic forms of craniosynostosis (craniofacial dysostosis) seen by the craniofacial team at the Hospital for Sick Children between 1986 and 1992. There were 51 patients with Crouzon's syndrome, 33 with Apert's syndrome, 8 with Pfeiffer syndrome, 11 with Saethre-Chotzen syndrome, and 4 with kleeblättschadel anomaly. Six patients developed raised intracranial pressure (ICP) after initial suture release and decompression (Apert's syndrome, three patients; Pfeiffer syndrome, one patient; Saethre-Chotzen syndrome, two patients). Raised ICP was considered in those children who returned with a bulging fontanelle, progressive frontal bone protrusion, intermittent headaches, irritability, and vomiting. The diagnosis of raised ICP was based on papilledema (four patients), progressive macrocephaly (one patient), and ICP monitoring (one patient). No child in this group had hydrocephalus requiring cerebrospinal fluid diversion. Once raised ICP was detected in these children, a second operation was immediately performed to reduce the ICP with the intention of expanding the volume of the cranial cavity. The second procedures included: anterior cranial vault and upper orbital reshaping (four patients), posterior cranial vault reshaping (one patient), and total cranial vault reshaping (one patient). There were no perioperative complications in these patients, although one patient showed subsequent recurrence of raised ICP requiring further cranial vault re-expansion. At follow-up, ranging from 3 to 7 years, all six patients were asymptomatic without evidence of raised ICP. In our series, raised ICP occurred in 6% of the children with a craniofacial dysostosis syndrome after initial suture release and decompression.(ABSTRACT TRUNCATED AT 250 WORDS)

The editors' labours: separating the wheat from the chaff.

From the author's standpoint, the editor is viewed as 'one who separates the wheat from the chaff and then prints the chaff'! Since its inception, Pediatric Neurosurgery has tried to provide the reader with the guarantee that only the 'wheat' is published. This paper will examine the policies of the journal's Editorial Board and in particular outline the review process for submitted manuscripts. In addition to certain operational items, the paper will consider how the 'peer' in 'peer review' is identified, the grading instruments for paper acceptance, the likelihood that the readers and peer reviewers agree on manuscript quality, and how authors should view failure. The Society is firmly committed to our journal. Our individual responsibilities begin with paper preparation and then its presentation at the annual meeting. Thereafter, it is important for the membership to appreciate that their participation, as either author and/or reviewer, is critical for the continued success of our journal.

Encephaloceles of the anterior cranial fossa.

From a total of 114 encephaloceles treated surgically at The Hospital for Sick Children in the 15 years to 1994, the case records of 17 patients with sincipital and 5 patients with basal defects were reviewed retrospectively. The condition was evident at birth in 64% of patients, while the remainder presented with either cerebrospinal fluid (CSF) rhinorrhea, nasal obstruction, or feeding difficulty. Hypertelorism affected 73% of patients. All encephaloceles were repaired transcranially, at a mean age of 2 years, usually by means of an intradural pericranial graft. Five children with gross hypertelorism underwent orbital translocation at the time of encephalocele repair. Of those not corrected, primary and secondary hypertelorism regressed in most instances where the encephalocele was treated before the age of 2 years. There were no deaths. The only case of CSF rhinorrhea occurred in a patient with a basal defect, in whom intradural repair was not possible because of adherence of diencephalic structures to the sac wall. Hypertelorism recurred in 1 patient after orbital translocation, requiring recorrection 2 years later. One patient with untreated secondary hypertelorism failed to regress after the encephalocele was excised at the age of 4 months. Developmental outcome was normal in 59% of children, whilst 18% have mild mental or physical disability, and 23% are severely impaired. A child with a sincipital or basal defect and mild hypertelorism should have the encephalocele treated in early childhood to allow the facial skeleton to remodel with growth. When an encephalocele is accompanied by gross hypertelorism or a facial cleft, one-stage correction can be undertaken safely in early childhood with minimal mortality and acceptable morbidity.

Benign cerebellar astrocytoma in childhood: experience at the Hospital for Sick Children 1980-1992.

Between 1 January 1980, and 31 December 1992, 66 patients with cerebellar astrocytoma were diagnosed and treated at The Hospital for Sick Children in Toronto. These patients ranged in age between 1 and 18 years, with a mean age of 7.3 years. Thirty-eight patients were male and 28 were female. Total removal was possible in 61 patients and no recurrence occurred in these 61 patients. In the 5 patients in whom an incomplete resection was carried out, regrowth occurred between 1 and 8 years following resection.

Cerebral arteriovenous malformations in children.

The treatment of cerebral arteriovenous malformations (AVM) or vascular anomalies are challenging neurosurgical procedures for an anaesthetist. Large AVMs are uncommon in children. Only 18% of AVMs become symptomatic before the age of 15 yr. This series reviews the experience at this institution during the period of 1982 to 1992. The symptoms at the time of presentation are varied and include haemorrhage (50%), seizures and hydrocephalus (36%) or congestive cardiac failure (18%). Symptoms of congestive heart failure predominate in the newborn whilst neurological symptoms, such as stroke, seizures or hydrocephalus occur more commonly in infants and older children. Approximately one third of AVMs in childhood present acutely. Radiological investigations, e.g., CT scan, MRI and cerebral angiography are essential to identify the precise location of the lesion. Therapeutic intervention in the acute presentation may involve craniotomy for evacuation of haematoma and treatment of increased intracranial pressure (ICP). Control of seizures and congestive heart failure may take priority and allow time to plan the elective procedures of embolization and surgical excision of the AVM. Operative intervention is hazardous and peroperative complications can be expected in more than 50% of patients. The morbidity and mortality associated with cerebral AVM are high, especially in infants who present in the neonatal period with congestive cardiac failure. The overall mortality in this series was 20%. Children presenting with intracranial arteriovenous malformations require a multidisciplinary approach. The successful management of anaesthesia either for embolization or surgical resection necessitates an understanding of the disciplines of paediatric and neuroanaesthesia. Special care and specific attention to detail may contribute to reduce the high morbidity and mortality encountered in these compromised children.

Development of anaplastic changes in low-grade astrocytomas of childhood.

The authors present their experience with six children who developed anaplastic astrocytomas after receiving treatment for low-grade astrocytomas. Five children were from a series of 55 children with optic chiasmatic-hypothalamic gliomas who have been studied since 1976. The sixth child initially had a low-grade astrocytoma of the thalamus. The mean age of the children at initial presentation was 5.3 years. Five children were treated with surgery and radiation therapy; one child with a chiasmatic-hypothalamic glioma received radiation therapy alone. The amount of external radiation therapy used in all children was 50-52.5 Gy delivered in standard fractionations over approximately 6 weeks to include the volume of the original tumor plus a margin of 2 cm. The time to anaplastic transformation varied between 2 and 10 years (mean, 6.4 years). At tumor recurrence, the children had seizures or symptoms and signs of raised intracranial pressure. The location of the second tumor in all patients was either at the primary site or within the field of radiation therapy. Five of the six children underwent a second craniotomy and subtotal resection of their malignant gliomas. One child had positive cerebrospinal fluid cytology and multiple intraspinal metastatic tumor nodules detected by magnetic resonance imaging. On histopathological examination, four children had anaplastic astrocytoma, and two had glioblastoma multiforme. Four of the six children have died of their anaplastic astrocytomas (mean time from diagnosis of anaplastic astrocytoma to death, 10 months). Two children underwent chemotherapy and spinal irradiation for their anaplastic astrocytomas, and are currently alive and undergoing treatment. The possible mechanisms by which anaplastic tumors have developed in children treated previously for low-grade astrocytomas is discussed. The data suggest that radiation therapy may have played an integral role in the genesis of anaplastic astrocytomas in these children.

Frameless stereotaxy in children.

Frameless stereotaxy provides stereotactic localization without a standard stereotactic frame. Other reference points on the patient are linked to radiographic images by means of a three-dimensional (3D) digitizer. We report the use of a frameless stereotactic system to assist in 53 procedures in 52 children using the ISG Viewing Wand system (ISG Technologies, Missasauga, Ont.). This device uses surface landmarks as well as random surface points as the reference system. The three-dimensional digitizer consists of a six-jointed articulated arm to which pointers, a rigid ventriculoscope, or stereotactic biopsy probe guide can be attached. Image reconstruction is performed on standard CT or MRI (3D volume acquisition) images, with typically 3-mm slice thickness. The tip of the probe can be displayed on 3D surfaced objects which can be cut away to reveal reformatted gray scale displayed on the cut surface, or in a triorthogonal view where simultaneous reformatted coronal and sagital views at the level of the tip of the probe are displayed. Cases for which the viewing wand was used included craniotomies for tumor (30), vascular malformation (6), epilepsy surgery (5), other lesions (3), rigid ventriculoscopy (9), and stereotactic biopsy (2). The accuracy of the system was measured by placing 20 fiducial markers on a plastic head of which 3-mm thickness CT images had been made. The average error using 4 fiducials as reference points was 1.5 mm when the fiducials were distributed around the circumference of the head. Using random surface points of 10, 20 and 40 in number gave average errors of 2.3, 1.6, 1.5 mm, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)