Non-immune hydrops fetalis secondary to congenital chylothorax with diffuse interstitial lung disease: a diagnostic conundrum.

A Chinese male infant was born at 35 weeks weighing 2935 g to a mother with polyhydramnios and prenatal hydrops fetalis. He developed marked respiratory distress secondary to bilateral congenital chylothorax and required pleural drainage, high frequency oscillation and inhaled nitric oxide therapy. He was extubated to non-invasive ventilation by day 14. There was no bacterial or intrauterine infection, haematologic, chromosomal or cardiac disorder. He was exclusively fed medium-chain triglyceride formula. High-resolution CT showed diffuse interstitial lung disease. He received a dexamethasone course for chronic lung disease to facilitate supplemental oxygen weaning. A multidisciplinary team comprising neonatology, pulmonology, haematology, interventional radiology and thoracic surgery considered congenital pulmonary lymphangiectasia as the most likely diagnosis and advised open lung biopsy, lymphangiography or scintigraphy for diagnostic confirmation should symptoms of chylothorax recur. Fortunately, he was weaned off oxygen at 5 months of life, and tolerated human milk challenge at 6 months of life and grew well.

Neonatal Arterial Tortuosity and Adult Aortic Aneurysm-Is There a Missing Link?-A Case Report.

We report a novel case of a full term newborn with non-immune fetal hydrops and arterial tortuosity mimicking a double aortic arch, and cranial fractures in the immediate neonatal period. The infant had no classic features of neonatal arterial tortuosity syndrome or Loeys Dietz syndrome apart from bilateral inguinal hernia. He also had skeletal manifestations in the form of fractures in the neonatal period without any trauma during birth and without clinical evidence of Osteogenesis Imperfecta. A heterozygous missense variant of uncertain significance was detected in MYH11 gene which is increasingly recognized to be belonging to the familial/hereditary thoracic aneurysm and aortic dissection group of disorders. Fetal hydrops as an association with arterial tortuosity has not been reported in the literature. We hypothesize the possible mechanism behind developing fetal hydrops in this case and discuss the genetic and phenotypic heterogeneity of the Familial Thoracic Aortic Aneurysm and Dissection (FTAAD) group of conditions highlighting the unique phenotypic and genotypic presentations. We recommend a high index of suspicion and vigilance in the early detection of such potentially lethal conditions with sequelae also in adulthood.

Perfluorobutane contrast-enhanced ultrasonography: a new standard for ultrasonography-guided thermal ablation of sonographically occult liver tumours?

INTRODUCTION: Image-guided thermal ablation, preferably with ultrasonography (US), is increasingly used for treatment of small liver tumours. Perfluorobutane-contrast-enhanced US (pCEUS) is a promising tool that may allow for targeting of tumours that are otherwise imperceptible on greyscale US. Although pCEUS has been reported to be effective, the literature has been limited outside of Japan and South Korea. We aimed to provide data that supports the use of pCEUS in the thermal ablation of sonographically occult liver tumours. METHODS: We conducted a retrospective single-centre study of 35 consecutive patients who underwent pCEUS-guided ablation of 48 liver tumours with a median size of 1.2 cm. Periprocedural, one-month post-treatment and relevant follow-up imaging studies were reviewed. Electronic records were also obtained, with long-term follow-up data of 12-28 months being available for 32 patients. RESULTS: 36 (75%) tumours that were imperceptible on greyscale US became visible with pCEUS. Overall, complete tumour ablation at one month was 89%. 1 (3%) patient developed a major complication following treatment, while 6 (17%) had minor post-treatment complaints. The local tumour progression rate was 17%, with a median time of 14 months. CONCLUSION: pCEUS has a role in US-guided thermal ablation of liver tumours, offering a high technical success rate that is comparable to reported data. Additional benefits may include improved procedural time and freedom from ionising radiation.

Outcome and Distal Access Patency in Subintimal Arterial Flossing with Antegrade-Retrograde Intervention for Chronic Total Occlusions in Lower Extremity Critical Limb Ischemia.

PURPOSE: To report the outcome and distal access patency of the Subintimal Arterial Flossing with Antegrade-Retrograde Intervention (SAFARI) technique for chronic total occlusion (CTO) in critical limb ischemia (CLI). MATERIALS AND METHODS: From January 2009 to June 2015, 220 SAFARI procedures were performed for 200 limbs in 191 patients (108 males [56.5%]; median age, 70 years old; range, 36 to 97 years old) with CLI (9.4% were Fontaine classification 3; and 90.6% were Fontaine classification 4). Distal access was obtained from the distal superficial femoral artery (n = 6), popliteal artery (n = 49), anterior tibial artery (n = 56), dorsalis pedis (n = 51), peroneal artery (n = 12), posterior tibial artery (n = 45), and lateral plantar artery (n = 1). Distal access hemostasis was obtained with internal balloon tamponade in 71.4% (n = 157). Outcome measurements were technical success, freedom from major amputation and complications. Preprocedural angiograms of clinically driven repeat interventions were reviewed in 73 cases for distal access patency. RESULTS: Technical success was achieved in 80.5% (n = 177). Reasons for technical failure include inability to obtain distal access (n = 3), cross the occlusion retrogradely (n = 16), re-enter the true lumen (n = 9), and achieve antegrade blood flow after the procedure (n = 15). Freedom from major amputation for technically successful procedures was 84.7%, 82.9%, and 81.9% at 6, 12, and 24 months, respectively. There were 3 cases of distal access bleeding with 1case that required coil embolization. The distal access remained patent in 80.8% of observable cases with repeated endovascular intervention. CONCLUSIONS: Distal retrograde arterial access (SAFARI) technique is safe and effective in the treatment of CTOs in the context of CLI, after failure of antegrade revascularization.

Primary Ciliary Dyskinesia and Situs Ambiguus: A Rare Association.

Primary ciliary dyskinesia (PCD) is a rare disorder with impaired ciliary function resulting in a spectrum of clinical manifestations of varying severity. PCD affects approximately one in every 20,000 individuals with a reported prevalence between 1:4000 and 1:50,000. Due to its nonspecific clinical features, the condition is usually diagnosed late in its course, unless situs inversus (SI) or organ laterality defects are discovered at imaging. A small subset of patients with PCD display associated organ laterality defects, different from the classical SI totalis. We present here, the clinical and imaging findings in a young female with PCD along with left-sided isomerism, a variant of heterotaxy syndromes.

Reducing radiation hazard opportunities in neonatal unit: quality improvement in radiation safety practices.

AIM: Guided by the ALARA - "As Low As Reasonably Achievable" principle in radiation safety, a quality improvement project to optimise the bedside diagnostic imaging process to the best standards of care was conducted over a six month period. The goal was too reduce the radiation hazard opportunities in the neonatal intensive care unit by at least 75% from the existing level at Q2/2015, within 6 months. METHODS: The existing bedside imaging process was critically analysed and the following quality improvement initiatives were implemented namely, mandatory lead protective gear to healthcare staff, gonadal shield for neonates, guidelines for optimal collimation of X-ray beam and optimal positioning of neonates. Radiation dosimetry results, regular staff awareness sessions and strong collaboration between neonatologists, radiologists, radiographers and neonatal nurses helped to ensure compliance to the revised imaging process. Radiation hazard opportunities were measured by analysing all radiographs done during the period under baby exposure and healthcare staff exposure categories. SUMMARY OF RESULTS: Radiation hazard opportunities were reduced by 100% to healthcare staff and 75% to neonates, and the overall reduction was 83%. The rate of discordance between radiograph request forms and images taken was measured as a surrogate marker for compliance to the project initiatives and it declined by 77%. Mandatory orientation of staff to the revised policy on the standardised diagnostic imaging process, regular radiation awareness talks and staff feedback sessions are among several measures taken to sustain the project.

Lipoma arborescens.

Lipoma arborescens is a chronic, slowly progressive intra-articular lesion characterised by villous lipomatous proliferation of the synovium, usually involving the suprapatellar pouch of the knee joint. It is an uncommon cause of intra-articular masses that presents as slowly progressive painless swelling of the joint, which persists for many years and is accompanied by intermittent effusions. We highlight this condition to raise awareness of its clinical spectrum and imaging features, so that early diagnosis and appropriate treatment can be given, and misinterpretation of this condition as other more complex intra-articular masses is avoided. This pictorial essay aims to provide a brief yet comprehensive review of the clinical features, distribution, morphological types and imaging characteristics of lipoma arborescens, including its common differential diagnoses and management.