Low-lying spinal cord and tethered cord syndrome in children with anorectal malformations

<p><b>INTRODUCTION</b>Anorectal malformations (ARMs) and low-lying spinal cord (LLC) are commonly associated owing to their common embryonic origin. LLC may lead to tethered cord syndromes (TCS), requiring surgery. This study aimed to review the incidence of LLC in children with ARMs using ultrasonography (US) and magnetic resonance (MR) imaging, the incidence of TCS and the surgical outcomes of these patients after detethering.</p><p><b>METHODS</b>We conducted a retrospective study of children who underwent surgery for ARMs in 2002-2009 at KK Women's and Children's Hospital, Singapore.</p><p><b>RESULTS</b>Out of 101 (16.8%) ARM patients, 17 had LLC, of which 12 (70.6%) were high ARMs. 12 of the 17 (70.6%) patients had abnormal US and MR imaging findings. Five (29.4%) had normal US but abnormal MR imaging results; in these five patients, MR imaging was performed due to new symptoms and equivocal US findings. These 17 patients subsequently underwent surgical detethering. Three out of seven patients with TCS improved after surgery. None of the 17 patients had any complications.</p><p><b>CONCLUSION</b>LLC appeared to be associated with high ARMs, although this was not statistically significant. LLC should be investigated for whenever ARM is diagnosed, regardless of its type. Lumbar US is useful for first-line screening for LLC. Abnormal US or onset of new symptoms should subsequently be investigated with MR imaging. Equivocal US findings are also likely to benefit from further MR imaging. Surgery to detether LLC can improve outcome in TCS, while prophylactic detethering for asymptomatic patients with lipoma of the filum terminale has very low surgical risk.</p>

Epidemiology, management and treatment outcome of medulloblastoma in singapore

<p><b>INTRODUCTION</b>Medulloblastoma/primitive neuroectodermal tumour is the most common type of malignant brain tumour in children. Long-term survival rates have improved over the years with a combination of surgical, radiotherapeutic and chemotherapeutic treatment modalities in the developed world. This paper aims to analyse the epidemiology and outcome of medulloblastoma in Singapore and compare our results with those reported in the literature.</p><p><b>MATERIALS AND METHODS</b>A 9-year retrospective study was done using data reported to the Singapore Children's Cancer Registry from June 1997 to June 2005. Only 39 children up to the age of 15 years diagnosed histologically with medulloblastoma or primitive neuroectodermal tumour arising from the cerebellum were included in the study. Follow-up data were collected up to June 2006 and analysed using SPSS v 13.0 software.</p><p><b>RESULTS</b>Medulloblastoma/primitive neuroectodermal tumour was the most common type of brain tumour, accounting for 40.7% of all brain tumours diagnosed in children in Singapore. The 5-year event-free survival rate was 44.5%, while the 5- year overall survival rate was 51.5%. Nearly half (41%) of our patients had spinal metastasis at presentation and this was associated with a worse event-free survival (6.3% vs 71.9%, P = 0). Children under 36 months of age had a significantly poorer overall survival (28.8% vs 52.2%, P = 0.041).</p><p><b>CONCLUSIONS</b>The outcome of medulloblastoma in Singapore was inferior to reported figures in the literature. We need to close identified gaps in care, like standardising assessment and treatment protocols, in order to improve our results. Research into molecular and genetic characteristics may also throw light on whether the disease is inherently more aggressive in our population.</p>