Case report: tuberous sclerosis and persistent hyperplastic primary vitreous.

BACKGROUND: Persistent hyperplastic primary vitreous (PHPV) in a patient with tuberous sclerosis (TS) has been described in one previous case report in 1999. Otherwise, there is no literature around this potential association. We describe a case of an infant with TS and PHPV. CASE PRESENTATION: An 11-month old male was under investigation for leukocoria, microphthalmia and suspected PHPV after being seen in ophthalmology clinic. He presented to hospital with seizures and was diagnosed with TS on imaging. Imaging also showed the known microphthalmia and a mass associated with the lens. Subsequent paediatric ophthalmology review and examination under anaesthesia confirmed microphthalmia, PHPV and a retrolental mass which was thought to represent total retinal detachment or a retinal hamartoma within a retinal detachment. CONCLUSIONS: This is the second case report of PHPV in a patient with TS. The previous case report postulated that the atypical location of the retinal hamartoma was secondary to the abnormal globe development in PHPV.

Ethnic Disparity in the Incidence and Outcome of Biliary Atresia in New Zealand.

To determine incidence and outcome of biliary atresia (BA) between ethnic groups in New Zealand (NZ), a retrospective review was undertaken of children with BA born between 2002 and 2014. Prioritized ethnicity was used to determine ethnicity and was compared to population data. Uni- and multivariate analyses were undertaken to determine demographic and biochemical factors associated with outcome. Overall incidence was 1 in 9181 (Maori 1 in 5285; European 1 in 16,228; P < 0.0001). Overall and transplant-free survival rates at 1, 2, and 5 years were 92%, 86%, 82% and 70%, 49%, 30% respectively with Maori having improved transplant-free survival (P < 0.05) despite European children undergoing Kasai earlier (49 vs 63 days). BA is more common in NZ than Europe and North America, which is attributable to a higher incidence in Maori but overall outcome is poorer. Maori have improved transplant-free survival compared to NZ European children but the reason is unknown.

Dual-energy CT for occult pelvic fractures: An audit and roc analysis.

INTRODUCTION: There is an increasing incidence of hip and pelvic fractures with an ageing population. Accurate and timely diagnosis is important in the emergency setting. While magnetic resonance imaging (MRI) is the gold standard, it is a limited resource. Dual energy CT (DECT) is comparable to MRI in detection of bone marrow oedema. Our hospital was the first centre in our country to introduce DECT for occult pelvic fractures. We aimed to describe its utility in occult pelvic fractures since commencement. METHODS: Retrospective study of consecutive pelvic bone CT (conventional or DECT) performed to look for an occult fracture over a 10-month period. Sensitivity and specificity calculated based on clinical and imaging follow-up. ROC study performed where three observers visually interpreted pelvic radiographs, conventional CT and DECT and scored their confidence for an acute fracture from 1 to 5. The null hypothesis was that DECT would not improve observer performance compared with conventional CT. RESULTS: DECT studies were performed on 178 patients of whom 84 (47%) had acute fractures. Sensitivity on audit was 99% and specificity was 100%. ROC analysis showed that, for all observers, the area under curve increased from radiograph to conventional CT to DECT. The difference between conventional CT and DECT was statistically significant for all observers where metal implants were not present. CONCLUSION: DECT improves accuracy compared to conventional CT in the diagnosis of occult pelvic fractures and should be used for this indication when available.

The impact of antibiotic pre-treatment on diagnostic yield of CT-guided biopsy for spondylodiscitis: A multi-centre retrospective study and meta-analysis.

INTRODUCTION: Patients with suspected spondylodiscitis often undergo CT-guided biopsy to identify a causative microbiological organism. Antibiotic pre-treatment has been postulated as a cause for a negative biopsy, although previous clinical studies have been heterogenous with a meta-analysis suggesting no effect. The aim of this study was to assess the impact of antibiotic pre-treatment on microbiological yield. METHODS: Retrospective review of consecutive adult patients undergoing CT-guided biopsy for suspected spondylodiscitis in two tertiary centres between 2010 and 2016. Demographic, procedural and clinical data were collected. Antibiotic pre-treatment was ascertained from patient drug charts. RESULTS: Over the 6-year period, 104 biopsies in 104 patients were included. 51% had a positive microbiological yield at CT-guided biopsy, with the most common isolated organism being Staphylococcus aureus (10.6%). Over two thirds of patients (69.3%) were off antibiotics at time of biopsy. There was no significant difference in microbiological yield in those patients on versus off antibiotics (48.2% vs 54.2%, P = 0.55). 10.6% patients had a final diagnosis of Mycobacterium tuberculosis spondylodiscitis, and this organism was significantly associated with a positive microbiological yield (90.9% vs 46.2%, P = 0.01). There was an inverse association between the presence of fever and sepsis with positive microbiological yield. CONCLUSIONS: CT-guided biopsy in suspected spondylodiscitis obtains a positive microbiological yield in about half of patients. This was significantly higher in patients diagnosed with tuberculosis spondylodiscitis, but there was no significant difference with antibiotic pre-treatment. Therefore, antibiotic pre-treatment should not preclude clinicians from pursuing a microbiological sample through CT-guided biopsy.