A pediatric neurologic assessment score may drive the eculizumab-based treatment of Escherichia coli-related hemolytic uremic syndrome with neurological involvement.

BACKGROUND: Thrombotic microangiopathy (TMA) is a clinical syndrome encompassing a large group of rare but severe disorders including thrombotic thrombocytopenic purpura (TTP) and both typical and atypical forms of hemolytic uremic syndrome (HUS). The key role of the complement system is well known in TTP and atypical HUS, but recent reports describe its involvement in the pathogenesis of HUS secondary to gastrointestinal infections due to Shiga toxin-producing Escherichia coli (STEC). METHODS: TMA mainly affects the kidney, but extra-renal complications are frequently described. The involvement of the central nervous system (CNS) represents often a life-threatening condition and it can result in serious long-term disability in HUS patients who overcome the acute phase of illness. In the present study, we retrospectively analyzed a pediatric cohort of a single tertiary pediatric hospital in Southern Italy, in which this complication occurred in 12/54 children (22% of cases), of whom five with severe neurological involvement had been successfully treated with eculizumab. RESULTS: The great clinical variability of brain injury in our cohort has led us to retrospectively build a "neurological score" useful to assess the clinical severity of neurologic involvement. Subjects with higher neurologic score due to the most severe CNS involvement resulted in the group of patients early treated with eculizumab, obtaining a good clinical response (four out five patients). In conclusion, the early treatment with eculizumab in children with severe neurological involvement during STEC-HUS was associated with complete regression of both acute kidney injury (AKI) and neurological lesions observed at magnetic resonance imaging (MRI). CONCLUSIONS: A "neurological score" may be a useful tool to drive the early treatment of CNS complications in STEC-HUS with eculizumab, although future perspective controlled studies are urgently needed to validate this therapeutic approach.

Endovascular Treatment of Large and Giant Carotid Aneurysms with Flow-Diverter Stents Alone or in Combination with Coils: A Multicenter Experience and Long-Term Follow-up.

BACKGROUND: Although flow diverters (FDs) have been widely accepted by neurointerventionists, their safety has yet to be fully defined. Stratification by aneurysm size and location is essential for correct evaluation of treatment outcomes and risks of flow diversion. OBJECTIVE: To compare neurological and anatomic outcomes and evaluate the risk of complications after endovascular treatment of large or giant internal carotid artery (ICA) aneurysms with FD/FDs alone or together with coil embolization. METHODS: We reviewed all patients with large or giant, ruptured or unruptured ICA aneurysms who underwent endovascular treatment with flow diversion alone (group A) or with concomitant coiling (group B) in 4 international institutions between 2010 and 2015. Anatomic outcome was evaluated using the Byrne scale on digital subtraction angiography and/or angioMRI and/or angioCT scans 3, 6, 12, and 24 months postoperatively. RESULTS: We collected 44 patients with large or giant ICA aneurysms. Four patients (9%) presented with aneurysmal subarachnoid hemorrhage (SAH). FD/FDs were used alone in 26 patients and in combination with coil embolization in the 18 remaining patients. The mortality rate due to procedure-related and/or neurological complications was 2.2%. Twelve months after the procedure, 88.6% (n = 39) of patients had a favorable neurological outcome. One year after the procedure, the aneurysm was completely occluded in 72.7% of patients: 61.5% (16/18) in group A and 88.9% (16/26) in group B. CONCLUSION: Clinical outcomes and rates of intraoperative and postoperative complications did not differ significantly between the groups. Better anatomic results using FD/FDs combined with coils were documented 6 months after the procedure; this option seems to provide a higher aneurysm occlusion rate and reduce the need for retreatment.

Postpartum ovarian vein thrombosis with simultaneous pyelocaliectasis: diagnosis and follow-up by MR imaging. Case report and literature review.

We describe the critical role of MR imaging in a case of postpartum ovarian vein thrombosis (OVT) with concomitant pyelocaliceal ectasia. MR imaging confirmed the diagnosis suspected on the basis of ultrasonography and computed tomography by demonstration of a subacute clot with high signal intensity within the right ovarian vein and its complete resolution after anticoagulant therapy. MR imaging is a useful noninvasive, accurate tool for the diagnosis and follow-up of this potentially life-threatening condition, providing information helpful for choosing a prompt medical treatment rather than a surgical therapy. To our knowledge, no previous case of OVT causing pyelocaliceal ectasia documented by MR imaging has been reported. However, even though our case is suggestive, a cause-effect relationship between OVT and hydronephrosis could not be demonstrated with certainty.