Spinal canal stenosis in children with achondroplasia: the role of augmentation laminoplasty-a 15-year single institution experience.

PURPOSE: Achondroplasia typically results in compressive spinal canal stenosis in one-third of children, but rare under the age of 15 years. Laminectomy is the mainstay of treatment but this leads to instability and progressive deformity requiring complex fixation. In order to reduce that risk, we developed a novel modified augmented laminoplasty that increases spinal canal diameter while preserving the posterior column stability. METHODS: All laminoplasty cases for spinal compressive achondroplasia from 2006 to 2020 were included. Ten augmentation laminoplasty procedures were performed in 7 children with regular clinical and radiological post-operative follow-up. Kyphotic deformity and clinical outcomes (neurological and urological) were evaluated. RESULTS: At presentation, clinical features included radiculopathy, neurogenic claudication or acute cauda equina compression with sphincter dysfunction. The average age at initial surgery was 11.2 (range 5-16) with a mean follow-up of 5 (range 2-8) years. All patients demonstrated improvement in neurogenic claudication symptoms after surgery; however, bladder dysfunction persisted in some children. In one child, cervical and lumbar augmentation laminoplasties were performed for concomitant disease. Augmentation laminoplasty effectively prevented deformity progression over time in all cases except one where a further revision laminoplasty with extension was required for screw loosening. Despite this, progressive symptomatic kyphotic deformity led to a 360° fixation. Minor complications included one dural breach (repaired intraoperatively) and one superficial wound infection. CONCLUSION: Augmentation laminoplasty is a viable surgical option following laminectomy in achondroplasia patients as an alternative to surgical fixation. Fixation can be reserved for cases where there is progressive deformity and kyphosis.

Paediatric intracranial aneurysms: a British institutional review.

INTRODUCTION: Paediatric intracranial aneurysms are rare, with a differing natural history and thought to account for only up to 7% of all intracranial aneurysms. There is much uncertainty that surrounds the prevalence of unruptured intracranial aneurysms and it is estimated to be anywhere between 2 and 90 per 1000. This is the largest British single-centre analysis of paediatric intracranial aneurysms. We present the patient course from their initial presentations to the outcome of treatment and evaluate a serial assessment of adequacy of aneurysmal obliteration radiologically. RESULTS: Twenty-two paediatric cases were identified that required treatment. The median age of presentation was 11.3 years (mean 9.9, range 0 to 15.9), 68% (15/22) were male and 77% (17/22) were ruptured on presentation. The majority of aneurysms were located at the anterior circulation (77% (17/22)). The overall median aneurysm size (n = 21) was 7.4 mm (mean 5 mm, range 2.5-19 mm). Twenty patients survived the acute phase and 80% (16/20) underwent coil embolisation and the other patients' surgical clipping. The overall outcomes were available for the 20 patients; on discharge, 90% (18/20) had a favourable clinical outcome (GOS score of 3-5). Treatment-specific clinical favourable outcomes were 88% (14/16) for coil embolisation against 100% (4/4) after surgical clipping. Of the two patients that died in the acute phase, one had sickle cell anaemia. Aneurysm aetiology was unknown in all other cases. None of the patients had a family history of aneurysms. CONCLUSION: Paediatric intracranial aneurysms while rare should be considered a differential diagnosis of children presenting with unexplained loss of consciousness with or without focal neurological deficit and/or headache. There is a two to one preponderance for males with a larger proportion of aneurysms within the posterior circulation (25%). Coil embolisation is the preferred method of securing a paediatric intracranial aneurysm.