A complicated Chiari type 1 malformation and holocord syrinx as a likely cause for heel pain.

BACKGROUND: Chiari malformations are a rare group of rhomboencephalic abnormalities involving the brain, craniocervical junction and spine. They may manifest in a variety of clinical presentations which relate to the variable involvement of the cerebellum, brainstem, lower cranial nerves, spinal cord and altered CSF flow dynamics. METHOD: We report an unusual case of incidental diagnosis of a type I Chiari malformation with secondary cystic cerebellar tonsillar encephalomalacia and holocord syrinx following investigation of a 5YO girl presenting with heel swelling related to progressive neuropathic osteoarthropathy of the posterior calcaneal body and apophysis. RESULT: The child was treated with decompressive suboccipital craniectomy and C1 laminectomy and tonsillar resection. Cerebellar tonsillar gliosis and cystic degeneration were confirmed on histopathology. Referral for ongoing engagement with occupational and physical therapy. CONCLUSION: Most type I Chiari malformations in the paediatric population are incidental and asymptomatic. Neurological symptoms are typically mild and relate to altered CSF flow dynamics; however, we present a complex case of type I Chiari malformation with an unusual constellation of associated complications.

Traumatic pseudoaneurysm of the internal iliac artery following bone marrow aspiration in a child: An extremely rare complication of a very common procedure.

Presented here is a very rare and potentially life-threatening complication of a very common procedure. Vascular injury with traumatic pseudoaneurysm following BMAT in a paediatric patient has only been reported once in the literature to the best of our knowledge. Presented here is a second case, with pathognomonic imaging findings on CT that underwent successful coil embolisation.

Diffuse paediatric cerebellar glioma: two identical imaging phenotypes of an extremely rare entity with disparate pathology.

Although the posterior fossa is a common location for paediatric brain tumours [1], diffuse glioma isolated to the cerebellum is an extremely rare imaging entity. Only two cases of isolated diffuse paediatric cerebellar glioma have been reported in the English language to the best of our knowledge [2, 3], and only one of these cases had a similar imaging phenotype to our cases [3]. Although somewhat similar to Lhermitte-Duclos (dysplastic gangliocytoma of the cerebellum), the appearances are distinct from other neoplastic entities of the paediatric posterior fossa. Clinical presentation and neurological examination findings are vital however to help differentiate other diffuse pathologies involving the cerebellum such as rhombencephalitis. Presented here are two diffuse cerebellar gliomas in children under the age of 3 with near identical imaging phenotypes demonstrating differing histological and molecular genetic profiles.

Previously unreported variant collateral supply in an acute stroke patient with anomalous common and internal carotid artery anatomy undergoing mechanical thrombectomy.

Agenesis of the common carotid and internal carotid arteries are rare entities. Approximately 100 cases of internal carotid artery (ICA) agenesis are reported in the literature, and even fewer cases of common carotid agenesis. The collateral supply in such cases is well described and typically follows one of only a small number of variations. Lie classically described six major pathways of collateral supply in ICA agenesis (Congenital Anomalies of the Carotid Arteries. Excerpta Medica, Amsterdam, 1968; 35). Vasovic et al. in a comprehensive review of all published cases of common carotid agenesis have similarly described the typical variations in collateral supply seen in this setting. Neither described the anatomy presented here.

Outcomes of video-assisted thoracoscopic surgery lobectomy in septuagenarians.

BACKGROUND: Spread of technology and increased surveillance have led to more patients with lung cancers being identified than ever before. Increasingly, patients from the elderly population are referred for surgery; however, many studies do not focus on this patient group. We reviewed the outcomes of septuagenarians who underwent lobectomy via an open thoracotomy (OT) or video-assisted thoracoscopic surgery (VATS) approach to determine whether the VATS approach would result in superior post-operative outcomes. METHODS: Between January 2010 and June 2016, a total of 96 patients aged 70 years or older underwent a lobectomy for non-small cell lung carcinoma. Patients who underwent resection for metastatic disease, small cell lung cancer or neuroendocrine tumour were excluded. Demographic details, early and late post-operative outcomes including post-operative arrhythmia, myocardial infarction, respiratory failure, cerebrovascular events, infection, prolonged air leak, delirium, readmission and 30-day mortality were studied. Mean follow-up duration was 23 ± 19.1 months. RESULTS: Seventy-five patients underwent lobectomy via a VATS approach and 21 patients underwent lobectomy via an OT approach. There was no 30-day mortality and no difference in overall survival between the two techniques (P = 0.25). There was no significant difference between the two techniques with regard to post-operative stroke, myocardial infarction, atrial fibrillation, pneumonia, delirium or bronchopleural fistula. VATS patients had a significantly shorter mean hospital length of stay (VATS 4.7 days, OT 9.3 days, P = 0.005). CONCLUSION: Septuagenarians with non-small cell lung carcinoma can successfully undergo curative lung resection with a low incidence of post-operative complications.

Does Medicare-eligible high-risk breast cancer screening MRI target the right women?

INTRODUCTION: MRI is the most sensitive modality to screen for breast cancer, but it is expensive with somewhat limited access. Audit of screening performance should reflect appropriate population targeting. METHODS: An observational study on consecutively screened high-risk women, assessment of the contralateral breast staging a new cancer, or surveillance in women with prior breast cancer or high-risk lesion in Perth, Western Australia. All breast MRI studies from 1 January 2015 to 7 September 2018 were included. Studies were 3T comprising T2, DWI, ADC and T1-weighted +/- fat saturation +/- IV gadolinium, +/- subtraction. DCE was read on the dynamics or DynaCAD (Invivo, Gainesville, FL, USA). Fellowship-trained breast radiologists blindly double-read by consensus; additional reader/s arbitrated. The reference standard was the histopathology result or cancer registry notification for cancer diagnoses and benign biopsies, benign follow-up imaging or subsequent screening MRI. RESULTS: Of 993 MRI studies in 554 women, 870 eligible MRI were performed in 471 women, and 706 had a reference standard. Median age was 44 years (range 18-80). The majority of studies (65% 457/706) were screening Medicare rebate-eligible high familial risk; 26% for surveillance after a breast cancer or contralateral staging; 6% screened BRCA carriers. Eleven cancers were diagnosed, eight were MRI-detected. Only two of these were at high-risk screening MRI. Five were detected at staging contralateral ILC, after negative 2D mammography and ultrasound. Cancer prevalence was highest for staging contralateral ILC, at 600/10,000 MRI, for high-risk screening 77/10,000 MRI and surveillance 116/10,000 MRI. CONCLUSIONS: Cancers were predominantly detected in women undergoing preoperative staging of new invasive lobular carcinoma in the contralateral breast, rather than the Medicare rebate-eligible high-risk screening group.

A local case of fulminant primary amoebic meningoencephalitis due to Naegleria fowleri.

Primary amoebic meningoencephalitis is an extremely rare, predominantly fulminant central nervous system infection caused by the amoeba Naegleria fowleri, first described in Australia in 1965. Despite the ubiquitous presence of N. fowleri, as few as 300 cases of infection have since been reported worldwide, with a case fatality rate approaching 98%. A combination of low index of suspicion, non-specific clinical findings and largely ineffective treatment modalities make this rapidly progressive meningoencephalitis virtually impossible to treat. Early and aggressive treatment utilising intravenous and intrathecal routes by a multidisciplinary team of neurosurgeons, intensivists and microbiologists is required. Presented is a case of a 56-year-old man who presented to the Gold Coast University Hospital in Queensland, Australia, with rapidly progressive primary amoebic meningoencephalitis. He received maximal therapy and died of his disease while in hospital.

Three-year experience with interventional neuroradiology for management of cerebral aneurysms at a single Australian centre.

INTRODUCTION: Over the last decade interventional neuroradiology (NR) has become the mainstay of managing cerebral aneurysms. The aim of our study was to review our growing experiences with interventional NR and quantify morbidity and mortality associated with these procedures. METHODS: The electronic medical records of all patients admitted to the Gold Coast (University) Hospital Intensive Care Unit following subarachnoid haemorrhage (SAH) or elective interventional NR management of cerebral aneurysms between January 2012 and December 2014 were retrieved. Primary outcomes of interest were death, thromboembolic and haemorrhagic events during hospital admission. RESULTS: One hundred and fifty-two patients underwent interventional NR procedures for cerebral aneurysms. This consisted of 92 (60.5%) elective cases and 60 (39.5%) emergency cases following SAH. The all-cause mortality rate and the rate of thromboembolic and haemorrhagic events for the entire cohort were 5.9%, 11.2% and 7.2% respectively. Intra-procedural complications occurred in 6.6% of the entire cohort. Median length of follow-up was 448 days, with 91.6% of the entire cohort followed up. At follow-up, 64.1% of patients had no neurological deficits, 29% had mild non-specific deficits and 6.9% had significant disability. CONCLUSIONS: Interventional NR represents the primary treatment modality for all patients presenting to our service with cerebral aneurysm. Our results are encouraging and are comparable to published data in the international literature. Reducing the burden of thromboembolism in patients undergoing endovascular treatment of their aneurysmal disease is our main research focus currently, and we aim to improve outcomes for this patient group.

Surgical Management of Caseous Calcification of the Mitral Annulus.

Caseous calcification of the mitral annulus (CCMA) is a rare variant of mitral annular calcification; it can manifest with conduction abnormalities or systemic embolization. It typically involves the posterior mitral annulus, and surgery is indicated for severe mitral valve dysfunction, for embolic complications or when the diagnosis is not certain. We describe a structured approach to the surgical management of CCMA using bovine pericardium to repair the defect.

Veno-Arterial ECMO in the Setting of Post-Infarct Ventricular Septal Defect: A Bridge to Surgical Repair.

Extracorporeal membrane oxygenation (ECMO) is a complex rescue therapy utilised to provide circulatory and/or respiratory support to critically ill patients who have failed maximal conventional therapy. The use of ECMO in adult cardiac surgery is not routine, occurring in a minority of critically ill patients, typically postoperatively. Presented here are three cases of post-infarct ventricular septal defect with cardiogenic shock managed preoperatively with ECMO support as a bridge to definitive surgical closure. We present a review of ECMO in the adult cardiac surgical population and highlight the potential role of preoperative ECMO for cardiogenic shock in the setting of post-infarct ventricular septal defect (PI VSD) as a bridge to definitive closure.