Predicting adult offending behavior for individuals who experienced a traumatic brain injury during childhood.

OBJECTIVES: To examine whether individuals who experienced a childhood traumatic brain injury (TBI) are at increased risk for subsequent offending behavior, to identify the emotional characteristics of adults who experienced childhood TBI, and to examine whether these predict offending behavior. PARTICIPANTS: Individuals from the Canterbury region who had experienced a childhood (<18 years of age) injury event and were now 18 years or older and more than 5 years postinjury (between 18 and 30 years of age). Three groups were formed: (1) moderate/severe TBI (n = 62); (2) mild TBI (n = 58); and (3) fracture control group (n = 38). SETTING: University of Canterbury. MAIN MEASURES: A semistructured interview assessed lifetime involvement in offending behavior; the Emotional Behavior Scale measured internalizing and externalizing behaviors, including malevolent aggression, social anxiety, and social self-esteem. RESULTS: Compared with controls, there was an increased risk of offending behavior (mild TBI: odds ratio = 8.7; moderate/severe TBI odds ratio = 20.4). Binary logistic regression analysis revealed that the strongest predictors of offending behavior were TBI status, higher levels of malevolent aggression, and age at injury. CONCLUSIONS: Individuals who experienced childhood TBI are at increased risk of offending behavior. Emotional behavior measures were useful predictors of offending behavior, offering opportunity for intervention.

Patterns of hearing loss following retrosigmoid excision of unilateral vestibular schwannoma.

Objectives To determine the pattern of auditory responses, time-course of hearing deterioration, and possible site of lesion following retrosigmoid excision of unilateral vestibular schwannomas. Design Prospective, nonrandomized, observational pilot study. Setting Tertiary referral medical center. Main outcome measures Preoperative and postoperative pure-tone and speech audiometry, auditory brainstem response testing, and distortion product otoacoustic emissions were performed in 20 patients. Testing was conducted every 24 hours for the duration of hospitalization. Transtympanic electrocochleography was performed if delayed deterioration of auditory responses was documented. Results Of the 20 patients, 7 had no discernible cochlear nerve at the end of the procedure. Of the 13 patients with an intact nerve, 6 retained hearing, 3 with evidence of reduced neural function. Of the 7 who lost hearing despite an intact nerve, 5 lost at least cochlear and possibly also neural function, and 1 had reduced neural function but retained cochlear function. There were two examples of delayed deterioration of cochlear nerve responses. Conclusions Hearing loss following retrosigmoid removal of vestibular schwannomas most often involves loss of at least cochlear function, possibly in addition to neural damage. In a smaller number of cases anacusis or hearing deterioration can be attributed to purely neural trauma.

Spinal cord stimulation for intractable chronic upper abdominal pain: a case report of the first patient in New Zealand.

We present the first patient in New Zealand to undergo Spinal Cord Stimulation (SCS) for intractable upper abdominal pain. The patient was a 53-year-old man with a 20-year history of debilitating upper abdominal pain associated with chronic pancreatitis secondary to pancreatic divisum. Prior to the SCS, he was prescribed 680 mg of morphine sulphate equi-analgesia a day. Despite the intense analgesia, he still suffered monthly attacks of upper abdominal pain requiring hospitalisation. Nine months after implanting a Spinal Cord Stimulator, the monthly attacks ceased, his background pain was effectively controlled and the need for opioids decreased to 510 mg of morphine sulphate equi-analgesia a day.

The CDKN2A G500 allele is more frequent in GBM patients with no defined telomere maintenance mechanism tumors and is associated with poorer survival.

Prognostic markers for glioblastoma multiforme (GBM) are important for patient management. Recent advances have identified prognostic markers for GBMs that use telomerase or the alternative lengthening of telomeres (ALT) mechanism for telomere maintenance. Approximately 40% of GBMs have no defined telomere maintenance mechanism (NDTMM), with a mixed survival for affected individuals. This study examined genetic variants in the cyclin-dependent kinase inhibitor 2A (CDKN2A) gene that encodes the p16(INK4a) and p14(ARF) tumor suppressors, and the isocitrate dehydrogenase 1 (IDH1) gene as potential markers of survival for 40 individuals with NDTMM GBMs (telomerase negative and ALT negative by standard assays), 50 individuals with telomerase, and 17 individuals with ALT positive tumors. The analysis of CDKN2A showed NDTMM GBMs had an increased minor allele frequency for the C500G (rs11515) polymorphism compared to those with telomerase and ALT positive GBMs (p = 0.002). Patients with the G500 allele had reduced survival that was independent of age, extent of surgery, and treatment. In the NDTMM group G500 allele carriers had increased loss of CDKN2A gene dosage compared to C500 homozygotes. An analysis of IDH1 mutations showed the R132H mutation was associated with ALT positive tumors, and was largely absent in NDTMM and telomerase positive tumors. In the ALT positive tumors cohort, IDH1 mutations were associated with a younger age for the affected individual. In conclusion, the G500 CDKN2A allele was associated with NDTMM GBMs from older individuals with poorer survival. Mutations in IDH1 were not associated with NDTMM GBMs, and instead were a marker for ALT positive tumors in younger individuals.

Occipital expansion without osteotomies in Apert syndrome.

INTRODUCTION: Cranial expansion has been the mainstay of initial management of children with Apert syndrome. Surgical timing is a balance between the risk of relapse if performed too early and the possibility of raised intracranial pressure and ossification defects if performed later. Primary occipital expansion has been proposed as a method to delay the timing of frontal surgery. We have applied the principal of spring expansion of patent sutures to expand the posterior cranial fossa without a cranial osteotomy. PATIENTS AND METHODS: All new Apert syndrome patients seen during the 3-year period December 2004-December 2007 underwent initial occipital expansion without osteotomy using spring expansion of the patent lambdoid suture. RESULTS: Four new Apert syndrome patients underwent posterior spring expansion of the patent lambdoid suture. Good occipital expansion was achieved in all cases. Standard frontal advancement was performed 5-18 months later. No relapse after frontal advancement has been seen after mean follow-up of 41 months. CONCLUSIONS: Spring expansion of the patent lambdoid suture is an alternative technique to expand the posterior cranial fossa. Compared to current techniques it has very low morbidity. Occipital expansion is thought to treat several of the mechanisms responsible for raised intracranial pressure in Apert syndrome. When performed at 6 months of age it has enabled us to delay the time at which we would normally perform frontal advancement surgery until a time when the surgical result is likely to be more stable.

Evolution of surgery for sagittal synostosis: the role of new technologies.

BACKGROUND: Techniques for sagittal synostosis correction continue to evolve to improve outcomes and minimize morbidity. The techniques now used by our craniofacial service are spring-assisted cranioplasty for younger children (generally up to 9 months of age) and biparietal barrel staving with cross-struts using bioabsorbable plates for older children. We evaluate the evolution of, and rationale for, our current methods of treatment. METHOD: All patients who underwent surgery for craniosynostosis over the period 1982 to 2007 were retrospectively reviewed. Patients with sagittal synostosis were analyzed according to the type of operative procedure performed. Blood loss, operative time, and hospital stay were compared between techniques using analysis of variance. RESULTS: Seventy-one patients had a cranial vault remodeling procedure for craniosynostosis. Thirty-four patients had sagittal synostosis with scaphocephaly. During the period 1982 to 2000, 13 patients had a strip craniectomy, and 5 patients had a barrel stave and morcellation procedure. Since 2001, the cross-strut technique using bioabsorbable plates has been used in 7 patients. In 2005, the spring cranioplasty technique was introduced and has been used in 9 patients. There were no deaths and no serious complications. One patient treated with springs had a second spring procedure performed to further increase biparietal width. Spring-assisted cranioplasty had a significantly shorter operating time than other techniques (P < 0.01). CONCLUSION: The availability of both absorbable plates and expansile cranial springs has revolutionized the techniques our unit uses for scaphocephaly correction. Our early experience with these techniques has shown that the techniques are reliable and give good cranial shape and form with minimal treatment morbidity.

Pontomedullary ganglioglioma: a rare tumour in an unusual location.

Gangliogliomas are rare low-grade tumours composed of neoplastic glial and neuronal cell elements. Although they may arise within any part of the neuraxis, involvement of the brainstem is rarely encountered. The distinct nature of gangliogliomas means that they have a comparatively better prognosis than other tumours in this location following subtotal resection. We present the case of an infant with a pontomedullary ganglioglioma and discuss the relevant literature.

Correction of nonsynostotic scaphocephaly without cranial osteotomy: spring expansion of the sagittal suture.

BACKGROUND: Scaphocephaly is usually due to sagittal synostosis. Scaphocephaly may also be seen in the presence of a nonsynostosed sagittal suture. In this situation traditional surgery is controversial due to the altered risk-benefit profile. This paper reports the first known series of patients with nonsynostotic scaphocephaly treated using spring assisted expansion of the sagittal suture. METHODS: All patients referred to our craniofacial program over the period February 2005-February 2008 were retrospectively reviewed. Eleven patients were seen with nonsynostotic scaphocephaly. Seven patients underwent spring expansion of the sagittal suture without osteotomy. RESULTS: Four female and three male patients had spring expansion of a patent sagittal suture. Four patients were born prematurely. Two patients had Beckwith-Weidemann syndrome. The ages ranged from 6 to 26 months (mean 12 months). The average preoperative cranial index was 66 (range 63-67). This improved to 76 at the time of spring removal (range 73-78). Springs were kept in situ for an average of 7.25 months. The mean blood loss was 7 ml and the mean operative time 36 min. CONCLUSION: Spring cranioplasty for sagittal synostosis is ideally performed before 6 months of age however in nonsynostotic scaphocephaly older children can be considered due to the absence of frontal bossing. Significant aesthetic improvement was achieved in all cases and normalization of the cranial index was achieved in 86% of cases with minimal morbidity and no significant complications. This technique is an alternative for nonsynostotic cases that were previously either untreated or undergone major remodelling surgery.

Association of mutant TP53 with alternative lengthening of telomeres and favorable prognosis in glioma.

The molecular basis for alternative lengthening of telomeres (ALT), a prognostic marker for glioma patients, remains unknown. We examined TP53 status in relation to telomere maintenance mechanism (TMM) in 108 patients with glioblastoma multiforme and two patients with anaplastic astrocytoma from New Zealand and United Kingdom. Tumor samples were analyzed with respect to telomerase activity, telomere length, and ALT-associated promyelocytic leukemia nuclear bodies to determine their TMM. TP53 mutation was analyzed by direct sequencing of coding exons 2 to 11. We found an association between TP53 mutation and ALT mechanism and between wild-type TP53 and telomerase and absence of a known TMM (P < 0.0001). We suggest that TP53 deficiency plays a permissive role in the activation of ALT.

Facial nerve function and hearing preservation after retrosigmoid excision of vestibular schwannoma: Christchurch Hospital experience with 97 patients.

BACKGROUND: Between January 1988 and December 2002, 97 patients underwent surgery for excision of vestibular schwannoma via the retrosigmoid approach at Christchurch Hospital. METHODS: A retrospective review was undertaken of the clinical notes with emphasis on facial nerve function and hearing preservation postoperatively. RESULTS: Of patients with small and medium-sized tumours, 81% had good facial nerve function at 1 year (House-Brackmann grade 1 and grade 2), 16% had moderate function (grade 3 and grade 4) and 3% had poor function (grade 5). Of patients with large tumours, 22% had good facial function (grade 1 and grade 2), 37% had moderate function (grade 3 and grade 4) and 41% had poor function (grade 5 and grade 6). Useful postoperative hearing was preserved in 21% of the 47 patients with tumours <3 cm and useful preoperative hearing. In the last 5 years the authors have been operating in conjunction with an ear, nose and throat surgeon (PAB) trained in base-of-skull surgery. Over this period, useful hearing was preserved in 32% of patients with small and medium-sized tumours and useful preoperative hearing. CONCLUSIONS: Tumour size was an important predictor of the postoperative facial and cochlear nerve function. The multidisciplinary approach to these tumours offers better results. These results compare well with other published series.

Excision of high and mid cervical spinal cord arteriovenous malformations by anterior operation.

Symptomatic cervical spinal arteriovenous malformations (AVMs) located on the anterior aspect of the spinal cord are rare and surgical removal of these AVMs presents considerable challenges and risks. Surgical techniques to date have usually been by posterior approach and lateral dissection around the cord or via midline myelotomy, both approaches involving cord manipulation and retraction and in the latter, dissection through the spinal cord. We present two teenage patients with symptomatic anteriorly placed mid to high cervical spinal AVMs and associated aneurysm in which excision of the AVMs and aneurysm was performed by an anterior approach using vertebrectomy/corpectomy. The first case had a small perimedullary glomus-type AVM with an aneurysm on the anterior aspect of the cord at the C3/4 level; excision was performed using a single level vertebrectomy/corpectomy, the patient remaining neurologically intact. The second case had a medium-sized juvenile AVM with an aneurysm, both perimedullary and intramedullary, centred at the C5/6 level; excision was performed using a two-level vertebrectomy/corpectomy with no deterioration in the marked pre-operative tetraparesis, which at long-term follow up had improved and stabilised. Anterior approaches have been recently described for treatment of anteriorly placed cervical arteriovenous fistulas (AVFs) and an intramedullary haemangioblastoma, but not as yet for spinal AVMs. These are the first two reported cases of anteriorly situated cervical AVMs successfully removed surgically by an anterior approach and with good neurological outcomes.

Intraosseous lipoma of the body of the sphenoid bone.

A case of intraosseous lipoma arising from the body of the sphenoid bone with intrasellar and suprasellar components is reported. This is the first report of this uncommon tumour occurring in an atypical intracranial site, producing the locally invasive and pressure effects of visual failure and hypopituitarism. The diagnosis was based on computed tomographic (CT) and magnetic resonance (MR) imaging with histological confirmation following frontal craniotomy.

The neuro-cardio-endocrine response to acute subarachnoid haemorrhage.

OBJECTIVE: Whereas cardiac hormones increase after subarachnoid haemorrhage (SAH), and may contribute to sodium wastage and hyponatraemia, there is controversy concerning the relative roles of atrial natriuretic peptide (ANP) vs. brain natriuretic peptide (BNP) and the factors initiating their secretion. Noting previous work linking stress hormone responses with cardiac injury after SAH, we have studied responses in stress hormones, markers of cardiac injury and the temporal changes in ANP and BNP and related them to changes in sodium status post ictus and during recovery from acute SAH. DESIGN, PATIENTS, MEASUREMENTS: Eighteen patients with verified SAH of variable severity were studied in a single unit for a 14-day period post ictus under controlled conditions of sodium and fluid intake. All received a standardized protocol of daily dexamethasone and nimodipine throughout the study. Severity was graded using criteria of Hess and Hunt at admission. Stress hormones (AVP, catecholamines and admission plasma cortisol), markers of cardiac injury (ECG and daily plasma troponin T) and cardiac hormones (ANP and BNP) were measured daily and related to severity, plasma sodium and renin-aldosterone activity. Hormone levels (ANP, BNP and endothelin) in cerebrospinal fluid (CSF) were also measured in nine patients. RESULTS: Intense neurohormonal activation (AVP, cortisol and catecholamines) at admission was associated with increased levels of both plasma ANP and BNP whereas levels in CSF were unaffected. In individual patients plasma levels of ANP and BNP were strongly correlated (P < 0.001). Cardiac events (abnormal ECG and/or elevated troponin) occurred in six of seven patients graded severe but neither stress hormones nor cardiac peptides differed significantly in patients with mild (n = 11) vs. severe (n = 7) SAH. During the course of a progressive fall in plasma sodium concentration (P = 0.001), there was a delayed activation of renin-aldosterone which was inversely correlated with declining levels of plasma ANP/BNP (P < 0.002). CONCLUSIONS: Excessive secretion of both ANP and BNP occurs in all patients after acute subarachnoid haemorrhage and is unrelated to severity, stress hormone activation or markers of cardiac injury. Inhibition of renin-aldosterone by cardiac hormones may impair renal sodium conservation and contribute to developing hyponatraemia. In the absence of evidence for activation of natriuretic peptides within the brain, the prompt and consistent increase in both ANP and BNP strongly supports the view that the heart is the source of increased natriuretic peptide secretion after acute subarachnoid haemorrhage.