Atypical teratoid rhabdoid tumours (ATRTs)-a 21-year institutional experience.

PURPOSE: Atypical teratoid/rhabdoid tumours (ATRTs) are malignant embryonal tumours of childhood that affect the central nervous system (CNS). We aim to determine which factors, including patient age, extent of resection (EOR), presence of distal metastasis and use of adjuvant therapies, affect overall survival in children with atypical teratoid/rhabdoid tumours (ATRTs) treated at this single centre. METHODS: Retrospective cohort review of patients with histological diagnosis of ATRT treated over 21 years (1999-2020) was conducted. Data on demographics, tumour location, presence of metastasis, use of adjuvant therapy, extent of resection (EOR), complications, neurological outcome post-surgery, and overall survival were collected. Kaplan-Meier survival analysis was performed. RESULTS: A total of 45 children (mean age 2 years) underwent 64 operations. 25 patients were <1 year of age. Gross-total resection (GTR) pre-adjuvant therapy was achieved in 15, near-total resection (NTR) in 15, subtotal resection (STR) in 9, and biopsy in 6 children. Most children had good neurological outcomes post-operatively (28/45 with GOS 5). Fourteen patients survived longer than 4 years. Survival analysis showed a significant difference in median survival in favour of GTR and localised disease. There was no significant difference in median survival between patients <1 year vs >1 year of age (p=0.84). CONCLUSION: We find that presence of metastasis was an important factor in poor survival in patients with ATRT. GTR, where possible, may confer significant survival benefit in ATRT. Children aged <1 year appear to have performed as well as those >1 year and therefore should still be considered for radical surgery.

Atlanto-axial rotatory fixation: a serious potential complication of paediatric ENT surgery that requires prompt diagnosis and treatment.

BACKGROUND: Atlanto-axial rotatory fixation is a persistent deformity of the C1-2 vertebral relationship caused by subluxation of the articular surfaces, and can occur after positioning for ENT procedures where the head is rotated - for example to access the ear or posterior triangle of the neck. If promptly recognised, it can usually be managed successfully with conservative methods, without long-lasting sequelae, but delayed or inappropriate management may lead to permanent neck deformity, neurological problems and pain. METHOD: Case review. CASE REPORT: Two children with atlanto-axial rotatory fixation following ENT surgery; one child was referred early and managed successfully, and one had delayed referral resulting in permanent severe positional deformity. CONCLUSION: Atlanto-axial rotatory fixation is easily missed; there are significant clinical and medicolegal implications if it is not promptly recognised. A suggested management algorithm is presented.

Central nervous system infection after Onyx embolisation of arterio-venous malformations in two paediatric patients.

BACKGROUND: Increasingly, Onyx is used for endovascular embolization of aneurysms and arterio-venous malformations. Although reports in the literature on the use of Onyx are favourable, there have been so far no reports on the central nervous system (CNS) infection rate after embolisation with Onyx and no recommendations as to the management of these infections. CASE REPORTS: We present two cases of paediatric patients who acquired CNS infection with Pseudomonas aeruginosa after Onyx embolisation of AVMs and describe their subsequent management. CONCLUSIONS: Presence of established infection after Onyx embolisation should be dealt with by removal of infected material, administration of appropriate antibiotic therapy and supportive treatment.