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We evaluated the accuracy of the quantitative and semiquantitative analysis in detecting regional atrophy patterns and differentiating mild cognitive impairment patients who remain stable (aMCI-S) from patients who develop Alzheimer’s disease (aMCI-AD) at clinical follow-up. Baseline magnetic resonance imaging was used for quantitative and semiquantitative analysis using visual rating scales. Visual rating scores were related to gray matter thicknesses or volume measures of some structures belonging to the same brain regions. Receiver operating characteristic (ROC) analysis was performed to assess measures’ accuracy in differentiating aMCI-S from aMCI-AD. Comparing aMCI-S and aMCI-AD patients, significant differences were found for specific rating scales, for cortical thickness belonging to the middle temporal lobe (MTL), anterior temporal (AT), and fronto-insular (FI) regions, for gray matter volumes belonging to MTL and AT regions. ROC curve analysis showed that middle temporal atrophy, AT, and FI visual scales showed better diagnostic accuracy than quantitative measures also when thickness measures were combined with hippocampal volumes. Semiquantitative evaluation, performed by trained observers, is a fast and reliable tool in differentiating, at the early stage of disease, aMCI patients that remain stable from those patients that may progress to AD since visual rating scales may be informative both about early hippocampal volume loss and cortical thickness reduction.

To evaluate the brain volumetric changes caused by BRAF gene mutation in non-epileptic CFC patients and the influence of the age of epilepsy onset on brain development in 2 cohorts of epileptic CFC patients. We enrolled CFC patients carrying BRAF gene mutations without epilepsy (4 patients) and with epilepsy (16 patients). CFC epileptic patients were divided into two cohorts based on the age of seizure onset: early-age onset (7 children) and late-age onset (9 adolescents). All three cohorts of patients underwent 3D FSPGR T1-weighted imaging to assess supratentorial and infratentorial brain volumes. Moreover, for each compartment, gray matter (GM), white matter (WM), and cerebrospinal fluid (CSF) volumes were measured. All measurements were compared with those of age-matched controls without neuroimaging abnormalities. All CFC patients showed supratentorial and infratentorial WM reduction and supratentorial ventricular enlargement (p

Evaluation of orbital proptosis and sutural synostosis pattern along the coronal ring in craniofaciosynostosis patients with or without fibroblastic growth factor receptor 2 (FGFR2) mutation. High-resolution computer tomography was used to assess, in children with or without FGFR2 mutation, the early synostotic involvement of the “major” and “minor” sutures/synchondroses of the coronal arch along with the following orbital parameters: interorbital angle, bone orbital cavity volume, globe volume, ventral globe volume, ventral globe index. Infants with FGFR2 mutation showed an increased number of closed minor sutures/synchondroses along the posterior coronal branch while both groups showed a comparable synostotic involvement of the minor sutures of the anterior coronal branch. FGFR2 infants with posterior coronal branch synostotic involvement showed a higher degree of proptosis due to both reduced bony cavity volume and increased globe volume (p

The aim of this study is to develop a prediction model for trismus (maximal interincisal distance equal to or less than 35 mm) based on a multivariable analysis of dosimetric and clinical factors.The maximum inter-incisal opening (MIO) of hean and neck cancer (HNC) patients who underwent radiotherapy (RT) ± concurrent chemotherapy with radical intent, was prospectively measured prior to RT (baseline) and 6 months post-RT. The outcome variable is trismus. The potential risk factors (clinical and dosimetric) were first screened by univariate analysis and then by multivariate analysis. At the end of this process, we used the features identified as relevant, to fit a logistic regression model and calculate the probability of observed trismus during the 6-month follow-up after RT.One hundred and four consecutive patients were included (mean age 63 years, range 25-87), 68 males, 36 females. In the univariate analysis, the MIO at baseline, as an independent variable, and several VA prediction nomogram was developed to assess trismus risk in planning process. An external validation of the model is required to apply it for current clinical use.

Diagnosis and management of sinonasal complications of dental diseases or treatment (SCDDT) may be challenging. We aimed to report our real-life experience in patients treated with endoscopic endonasal approach describing data about symptoms, etiology, extension of the disease and success rate. We evaluated retrospectively data about 262 patients diagnosed as SCDDT and managed from August 2015 to May 2022. In 44.65% cases, maxillary sinus complications were determined by a dental disorder; the remaining 55.34% of cases were iatrogenic. Patients were managed according to our multidisciplinary protocol including ENT, dental, and radiological evaluation. Treatments were planned with a personalized approach, based on the patient’s clinical characteristics; all patients were treated with an endonasal endoscopic mini-invasive conservative approach. Combined dental treatment was performed simultaneously in 152/262 (58%) of patients; in the remaining cases, it was postponed after surgery. The overall treatment success rate (symptom resolution and endoscopically observed maxillary sinus healing) was 96.5%. At 15 days after surgery, we observed a significant improvement in the quality of life. The mean post-operative Sinonasal outcome test-22 (SNOT-22) score was significantly lower compared to baseline (6 versus 43.4; p < 0.05). Our study showed that endoscopic sinus surgery can be a successful procedure for treatment of SCDDT, leading to fast resolution of sinonasal symptoms and improving the quality of life. Furthermore, the technique allows removal of migrated dental material or dental implants even in challenging cases.

Among patients with isolated sagittal synostosis (ISS), the head shape varies considerably in relation to the severity of the abnormality. This study aimed to quantify skull base morphometry and intracranial volume to investigate their relationships with the severity of scaphocephaly. We studied 66 infants with ISS identifying three groups according to the morphological severity of cranial deformity (group I: mild deformity; group II: moderate deformity; group III: severe deformity), by combining two scaphocephaly severity indices as descriptors of the relation of three morphological measurements (length, width and height) We perform a quantitative analysis using high-resolution CT images calculating following parameters: cranial fossae dimensions, supratentorial (ICV) and infratentorial (PCFV) cranial volume, supratentorial (WBV) and infratentorial (PCFBV) brain volume, ICV/WBV, PCFV/PCFBV, supratentorial and infratentorial cerebrospinal fluid (CSF). In all subgroups, anterior and middle skull base lengths were increased, while posterior hemifossae lengths were unchanged. In mild subgroup, ICV/WBV was significantly different and ICV, WBV and CSF supratentorial volume increased (p

Background The 8th edition of tumor, node, metastasis (TNM) classification incorporates depth of invasion evaluation in the staging of oral cavity squamous cell carcinoma, since it is a predictor of nodal metastasis and an independent prognostic factor. Although the histopathological definition of depth of invasion is clear, an accurate method for its radiological assessment has not yet been validated. Purpose To investigate the role of MRI-derived depth of invasion evaluation in staging oral tongue squamous cell carcinoma and to assess the inter-reader agreement and the radiological–pathological correlation. Material and Methods We retrospectively reviewed 43 patients with oral tongue squamous cell carcinoma who underwent preoperative MRI. The MRI-derived depth of invasion was measured by two radiologists, each with a different degree of experience in head and neck imaging. The pathological depth of invasion was recorded from histopathological reports. The inter-reader and the radiological–histopathological correlations for the depth of invasion were evaluated with Bland–Altman plots, the intraclass correlation coefficients (ICC), and the paired samples test; agreements for T staging were assessed using the Kappa coefficient. Results Inter-reader reliability was excellent for the MRI-derived depth of invasion (ICC = 0.91), very good between MRI-derived depth of invasion and pathological depth of invasion (ICC = 0.89 for the experienced reader, 0.86 for the inexperienced reader). Both readers reached a good agreement regarding T staging (kappa value = 0.70). Furthermore, the agreement between radiological and pathological T staging was good (kappa value = 0.74 for the experienced reader, 0.60 for the inexperienced reader). Conclusion MRI-derived depth of invasion should be measured in the pretreatment assessment of oral tongue squamous cell carcinoma as it has an excellent inter-reader reliability and nearly excellent radiological–pathological correlation.

Background High-grade pineal region tumors are rare and heterogeneous types of primary central nervous system neoplasms; radiological differential diagnosis is challenging but it is important because it has a therapeutic relevance. Purpose To discriminate among high-grade pineal region tumors by combining apparent diffusion coefficient (ADC) volumetric values and qualitative features in order to predict their histology. Material and Methods Twenty-two patients with high-grade pineal region tumors were assessed by qualitative and quantitative analysis. Margins, T2-weighted signal intensity, contrast enhancement, hemorrhage, calcifications, different volumetric ADC fractions (ADCmean, ADCmax, ADCmin) were evaluated and were compared to the histopathologic findings (cell count and proliferation index). Results Our qualitative imaging data showed that only margins were different among different tumors and each tumor type showed peculiar age onset. ADCmean was found the best quantitative value to discriminate high-grade tumors of the pineal region. ADCmean correlated with proliferation index but not with cell count. ADCmean values were lower in tumors with higher proliferation rate and a significant difference in ADCmean values were found between germinomas and pineoblastomas, between germinomas and papillary tumors and between papillary tumors and pineoblastomas. Moreover, the cut-off value of 0.865 × 10–3 mm2/s for ADCmean (ADC mean threshold value) could differentiate germinoma from pineoblastomas with the best combination of sensitivity and specificity. Conclusion The ADCmean value measured on the whole tumor, reflecting tumor proliferative activity, may be a practical and non-invasive marker for predicting tumor histology in high-grade pineal region lesions and might be useful in preoperative assessment.

Polidocanol sclerotherapy of head and neck venous malformations (VMs) and lymphatic malformations (LMs) has been reported only in limited series. In this manuscript we evaluated the efficacy and safety of polidocanol sclerotherapy in a series of head and neck venous and lymphatic malformations.This retrospective observational study analysed data on 20 head and neck VMs and LMs that underwent to percutaneous or endoscopic intra-lesional 3% polidocanol microfoam sclerotherapy at our institution. Clinical response was ranked as excellent, moderate and poor based on volume reduction by MRI and resolution of symptoms.The median volume decreased from 19.3 mL to 5.8 mL after sclerotherapy (mean volume reduction: 72.98 ± 16.1%). An excellent-moderate response was observed in 94.4% of cases. We observed a mean volume reduction of 79.5 ± 16.1 in macrocystic LMs, of 76.1 ± 13.0% in VMs, of 60.5 ± 10.9% in mixed lymphatic ones and 42.5% in microcystic lymphatic ones.Polidocanol sclerotherapy appears to be an effective and safe treatment for venous and lymphatic head and neck malformations. We observed the best responses in macrocystic LMs and VMs, whereas mixed lymphatic ones showed a moderate response and microcystic lymphatic ones a poor response.La scleroterapia con polidocanolo nella gestione delle malformazioni venose e linfatiche del distretto testa-collo.La scleroterapia con polidocanolo (POL) nelle malformazioni venose (VM) e nelle malformazioni linfatiche (LM) della testa e del collo è stata descritta solo in casisitiche limitate. In questo lavoro abbiamo valutato l’efficacia e la sicurezza del POL in pazienti affetti da VM e LM della testa e del collo.Sono stati analizzati i dati di 20 pazienti affetti da VM e LM del testa-collo, sottoposti a scleroterapia con POL al 3%, iniettato nella lesione per via percutanea o endoscopica. La risposta è stata classificata come eccellente, moderata o scarsa in base alla riduzione del volume valutata mediante risonanza magnetica e alla risoluzione dei sintomi.Il volume mediano si è ridotto da 19,3 ml a 5,8 ml dopo il trattamento (riduzione volumetrica media: 72,98 ± 16,1%). Il 94,4% dei casi ha ottenuto una riposta eccellente-moderata. In particolare è stata osservata una riduzione volumetrica media del 79,5 ± 16,1% nelle LM macrocistiche, del 76,1 ± 13,0% nelle VM, del 60,5 ± 10,9% LM miste e del 42,5% nell’unica LM microcistica.La scleroterapia con POL sembra essere un trattamento efficace e sicuro: i risultati migliori sono stati ottenuti nelle LM macrocistiche e nelle VM, mentre le LM miste hanno mostrato una moderata risposta e le LM microcistiche una scarsa risposta.

The processes of sutures and synchondroses formation, interdigitation and closure set the normal skull growth. Sutures and synchondroses represent growth sites in normal skull development. These articulations have distinctive characteristics due to their different embryological origins and histologies; sutural fusion occurs by either intramembranous ossification or chondroid bone formation whereas synchondrosis fusion occurs by endochondral ossification.

Evaluation of orbital proptosis and sutural synostosis pattern along the coronal ring in craniofaciosynostosis patients with or without fibroblastic growth factor receptor 2 (FGFR2) mutation.High-resolution computer tomography was used to assess, in children with or without FGFR2 mutation, the early synostotic involvement of the "major" and "minor" sutures/synchondroses of the coronal arch along with the following orbital parameters: interorbital angle, bone orbital cavity volume, globe volume, ventral globe volume, ventral globe index.Infants with FGFR2 mutation showed an increased number of closed minor sutures/synchondroses along the posterior coronal branch while both groups showed a comparable synostotic involvement of the minor sutures of the anterior coronal branch. FGFR2 infants with posterior coronal branch synostotic involvement showed a higher degree of proptosis due to both reduced bony cavity volume and increased globe volume (p0.05).Our data show that FGFR2 mutation together with posterior coronal branch synostotic involvement has a synergic effect in causing a more severe degree of orbital proptosis.

Two degrees of posterior cranial fossa (PCF) maldevelopment can be hypothesized in children with myelomeningocele (MMC). This paper investigates the PCF deformation by quantitative magnetic resonance imaging analysis in MMC subjects with and without Chiari 2 malformation (CM2).PCF bone volume (PCFV), PCF brain volume (PCFBV), lengths of PCF, ventriculomegaly, level, and extension of the dysraphism were analyzed by magnetic resonance image scanning of 51 newborns with MMC surgically repaired at birth (and 41 controls). The possible correlation among PCF hypoplasia, level/extension of the spinal dysraphism, and ventriculomegaly was assessed.In MMC and CM2, the dysraphism level was above L4 in 30 and below L4 in 10 subjects. PCFV/PCFBV ratio and supraocciput and exocciput lengths were significantly reduced; foramen magnum diameters, mammillo-pontine distance, and pons length were significantly increased (P 0.05). In isolated MMC, the dysraphism level was below L4 in all cases. PCFV/PCFBV ratio and supraocciput length were significantly reduced; pons length was significantly increased (P0.05). The lower the MMC level, the lower the incidence of CM2. A positive correlation was found between PCF hypoplasia and MMC level above L4 (P0.001), while a negative correlation was found among PCF hypoplasia and MMC extension (P = 0.006), PCF hypoplasia, and ventriculomegaly (P = 0.02).PCF hypoplasia has to be considered a dynamic maldevelopment process in the 2 cohorts rather than 2 separated entities. The level of MMC is the main but not the unique cause influencing the severity of PCF maldevelopment.

The aim of this study was to identify quantitative tools to classify the severity of trigonocephaly to guide surgical management and predict outcome. METHODS We reviewed high-resolution computed tomography images of 59 patients with metopic synostosis. We assessed the craniofacial sutural pattern as well as interfrontal and metopic angles, and we related the frontal angulation degree with the sutural pattern, the surgical management, and clinical outcome. RESULTS We identified 3 groups according to the severity of trigonocephaly. No difference was found between the sutural pattern of nasion complex and severity, whereas the closure of zygomatic maxillary sutures increased with the severity degree (P < 0.05). The operative management was related to the severity degree (P < 0.001) and to the reduced age (P = 0.009). CONCLUSIONS Interfrontal and metopic angles are complementary measurements to evaluate with high accuracy the degree of frontal angulation. In preoperative assessment, they may guide surgery decision in particular when the choice is not straightforward.

Craniosynostostic syndromes are due to multisuture synostoses and affect the entire craniofacial skeleton. This study analyzed the facial complex and airways to quantify the relationship between insufficient facial growth, airways obstruction, and the sutural pattern of the splanchnocranium and cranial fossae. Preoperative high-resolution CT images in 19 infants with syndromic craniosynostosis were quantitatively analyzed. Because all children showed involvement of minor sutures/synchondroses coursing in the posterior cranial fossa, they were divided into three groups according to the synostotic involvement of “minor” sutures/synchondroses coursing in anterior (ACF) and middle (MCF) cranial fossae: group 1 (ACF), group 2 (MCF), and group 3 (ACF-MCF). Analysis of the facial complex and airway was performed. Each group was compared with age-matched healthy subjects. Premature closure of skull base synchondroses of ACF and MCF was found only in groups MCF and ACF-MCF. Group MCF showed synostosis in the posterior branch of the coronal ring and reduced anterior hemifossae lengths while group ACF-MCF showed synostosis in the anterior branch of the coronal ring and reduced middle hemifossae lengths. No group showed reduced maxillary or mandibular volumes but group MCF showed synostosis of the zygomaticomaxillary sutures and maxillary retrusion. All groups showed reduced airway volume but group 2 had a higher degree of airway hypoplasia. The skull base synostotic process drives the changes in facial complex growth and airway obstruction. Premature closure of synchondroses/sutures in the posterior branch of the coronal ring causes insufficient facial growth, maxillary retrusion, and more severe airway reduction.

Craniofacial dysmorphology varies significantly along a wide spectrum of severity in metopic cranial synostosis (MCS). This study aimed to quantify craniofacial changes, in MCS, to investigate their relationships with the severity of trigonocephaly. By combining the metopic ridge and interfrontal angles, we identified three groups of trigonocephaly severity (mild group n.14, moderate group n.19, severe group n.18). We perform a quantitative analysis using high-resolution CT images evaluating (1) cranial fossae dimensions; (2) vault indices and ratios: interparietal/ intercoronal (IPD/ICD), interparietal/intertemporal (IPD/ITD), cephalic index, vertico-longitudinal index; (3) orbito-facial distances (midfacial depth, maxillary height, upper facial index, orbital distances, globe protrusions), maxilla and orbital volumes; (4) supratentorial (ICV) and infratentorial (PCFV) cranial volumes and supratentorial (WBV) and infratentorial (PCFBV) brain volumes. In all groups, middle skull base lengths and upper midface index were increased. In moderate and severe groups: anterior hemifossa lengths were reduced, IPD/ICD and vertico-longitudinal index were changed; midfacial depth, anterior, mild, and lateral interorbital distances were reduced; globe protrusions were increased. The comparison between moderate and severe groups showed an increase of both globe protrusions and IPD/ICD. Among all groups, ICV and WBV were reduced in the severe group. This morpho-volumetric study provides new insights in understanding the craniofacial changes occurring in infants at different severity of trigonocephaly. The increase of globe protrusions and the reduction of supratentorial volumes found in the severe group reflect the severity of trigonocephaly; these findings might have a clinical and surgical relevance.

Objective We performed a quantitative hemispheres analysis in epileptic children with hemispheres’ asymmetry -due to unilateral dysplastic malformation- in order to recognize subtle volumetric changes of the contralateral and apparently unaffected hemisphere. Methods 13 children with Hemimegalencephaly (HME) and 20 with Hemimicrencephaly (Hme) were clustered in subgroups according to underlying hemispheric cortical dysplastic malformation and epilepsy pattern. 3D FSPGR T1weighted images were used to assess white and grey matter volumes for both hemispheres. Each volumetric parameter was compared with the average of an age-matched healthy control group. Results HME subgroups: HME with pachygyria and focal (HME-PG-F; n 6) or multifocal epilepsy (HME-PG-MF; n.7). In both subgroups affected hemisphere (AH) volume was increased and contralateral hemisphere (CH) showed white matter volume reduction; in HME-PG-MF grey matter volume of CH was more reduced than HME-PG-F. Hme subgroups: Hme with polimicrogyria and focal epilepsy (Hme-PMG-F; n.8), Hme with giant subcortical nodular heterotopia and focal (Hme-SCH-F; n.6) or multifocal epilepsy (Hme-SCH-MF; n.6). In all subgroups AH volume was reduced; the volume of CH was significantly increased in Hme-PMG-F and Hme-SCH-MF while it was not significantly increased in Hme-SCH-F compared to affected hemisphere. Conclusions In patients with hemispheres’ asymmetry, quantitative high-resolution MRI offers a more objective assessment of brain structures volume. The type of hemispheric dysplastic malformation together with the age of epilepsy onset and epileptic pattern may contribute to changes in contralateral and apparently unaffected hemisphere. Future studies are warranted to evaluate whether the early identification of these changes might help in planning future antiepileptic treatments.

This study aimed to develop a novel approach to assess the severity of skull dysmorphology in infants with isolated sagittal synostosis (ISS) and its relationship with the surgical results. We divided 66 infants with ISS into three groups by combining the scaphocephalic (SSI-A) and platycephalic (VLI) indices as descriptors of the relation between length, width, and height. We evaluated each skull for morphology as hyperdolichocephalic (

Objective : Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of acute or subacute onset characterized by varied neurological symptoms including headache, impaired visual acuity or visual field deficits, confusion, disorders of consciousness, seizures, and motor neurological deficits. Even if recognition of severe forms of PRES has improved, mainly due to magnetic resonance imaging, pathogenesis is still unclear and management of these patients remains challenging. Moreover, prognosis is unpredictable varying from complete recovery to death and factors related to prognosis are still lacking. We studied early magnetic resonance imaging characteristics and their relationships with prognosis. Patients and Methods We performed a retrospective analysis in patients with clinical and neuroradiological charateristics of PRES performing magnetic resonance of the brain within 2 days of symptoms onset. Results After reviewing site database of magnetic resonance imaging and clinical records compatible with PRES, 157 patients were selected. After imaging reviewing, 25 patients with clinical and neuroradiological diagnosis of PRES were enrolled, 22 (88%) females. Mean age of enrolled patients at presentation was 44.4 + 18.4 years (range, 21–84 years). Patients were classified according to neuroradiological characteristics such as ischemic lesions, distribution and severity of edema, hemorrhage and contrast enhancement. In our group 23 patients (92%) showed an almost complete recovery but 2 patients (8%) died during hospitalization. Outcome was significantly related with hypointensity on ADC (p = 0.002) and CE (p Conclusion Early MR features may be helpful in suggesting prognosis. Moreover, neuroimaging at the early stage of PRES may give new insights in pathophysiological mechanisms underlying brain damage and neurological impairment.

The effects of premature fusion of one coronal suture cause skull and orbital alterations in term of side-to-side asymmetry. This study aimed to quantify the cranio-orbital complex changes related to the severity of skull base dysmorphology in patients with unicoronal synostosis. Twenty-four infants affected by unicoronal synostosis were subdivided in three subgroups according to the severity of skull base deformity and their high-resolution CT images were quantitatively analyzed (groups IIa, IIb, III). Dimensions of cranial fossae, intracranial volume (ICV), ICV synostotic and ICV non synostotic side, whole brain volume (WBV), orbital volumes (OV), ICV/WBV, ICVsynostotic/ICVnon-synostotic, and OVsynostotic/OVnon-synostotic were evaluated. Asymmetry and reduction in the growth of the anterior and middle fossae were found in all groups while asymmetry of the posterior cranial fossa was found only in IIb and III groups. In all groups, ICV, WBV, and ICV/WBV were not significantly different while ICVsynostotic/ICVnon-synostotic and OVsynostotic/OVnon-synostotic resulted significant difference (p

Background: Sensitivity of dipyridamole stress echocardiography (SE) is often lower than required. The aim of the present work is the evaluation of the association of dipyridamole and exercise echocardiography. Methods: From June 2007 to January 2011, 259 consecutive patients referred to Camposampiero Echocardiography Laboratory underwent SE. Stress protocol started with dipyridamole infusion of 0.84 mg/kg over 6 min. In patients without a new dyssynergy after dipyridamole, SE was carried on with supine exercise. If endocardial border detection was suboptimal, ultrasound contrast agent was used. Coronary angiography was performed in positive patients. The events recorded during the follow-up were cardiac death, nonfatal myocardial infarction (hard events), and coronary revascularization. Results: Of 259 patients, 74 had a positive result: 37 were positive after infusion of dipyridamole, and 37 became positive during exercise. All 74 positive patients underwent coronary angiography: 67 had significant coronary artery disease (36 positive with dipyridamole, and 31 positive with exercise), and 7 had not significant artery disease. In positive patients, the coronary revascularizations were 40. Furthermore, 3 of the 185 negative patients underwent coronary revascularization. During follow-up of 20 ± 10 months, 6 (8.1%) hard events occurred in positive patients. No hard event was observed in negative patients. Conclusions: Dipyridamole SE with the addition of exercise can be proposed as a strategy to carry on the ischemic cascade and to identify the patients who elude the dipyridamole alone SE. A negative result is suggestive of a very good prognosis, free from hard events at 20 ± 10 months.

We propose an magnetic resonance imaging (MRI)-based quantitative morphovolumetric approach to the posterior cranial fossa (PCF) and craniocervical junction (CCJ) changes in achondroplastic patients investigating possible associations with ventriculomegaly and medullary compression. We analyzed MRI of 13 achondroplastic children not treated by surgery. 3D FSPGR T1-weighted images were used to analyze (1) PCF synchondroses; (2) PCF volume (PCFV), PCF brain volume (PCFBV), PCFV/PCFBV ratio, cerebellar volume, cerebrospinal fluid (CSF) spaces volume, and IV ventricle volume; (3) PCF (clivus, supraocciput, exocciput lengths, tentorial angle) and CCJ (AP and LL diameters of foramen magnum (FM)) morphometry; (4) measurements of FM and jugular foramina (JF) areas; and (5) supratentorial ventricular volume. All patients showed synostosis of spheno-occipital synchondroses, eight showed synostosis of intra-occipital synchondroses, nine showed CCJ impingement on the cervical cord but only three had cervical myelopathy. Compared to controls, clivus and exocciput lengths, LL and AP diameters of FM, FM area and JF area were significantly reduced, supraocciput length, tentorial angle, PCFV, PCFBV, cerebellar volume, supratentorial ventricular system volume were significantly increased. A correlation was found between clivus length and supratentorial ventricular volume, premature closure of intra-occipital synchondroses and FM area while a trend was found between FM area and supraocciput length. Our analysis demonstrates a relationship between the shortening of the clivus and the ventriculomegaly. On the other hand the premature closure of PCF synchondroses, the shape, and the growth direction of supraocciput bone contribute to reduce the FM area, causing in some patients medullary compression.

Pentoxifylline (PTX) is extensively metabolized in the body, and all its 3 plasma metabolites (M1, M4, M5) are pharmacologically active. The authors evaluated the pharmacokinetics of PTX and its metabolites in 20 patients with chronic heart failure (CHF). Eleven had moderate and 9 severe CHF. The time courses of PTX, M1, M4, and M5 plasma levels were determined after oral administration of a sustained-release 600-mg tablet of PTX, and for each compound, AUC, maximal plasma concentration (C(max)), and time to C(max) (T(peak)) were calculated. Compared with patients with moderate CHF, those with severe CHF showed a significant delay in T(peak) of PTX (3.9 vs 1.6 hours) and M5 (5.6 vs 3.6 hours), a 59% significant increase in M5 AUC, and a 56% nonsignificant increase in PTX AUC. In the whole population, the AUCs of PTX, M4, and M5 were inversely correlated with markers of liver function, whereas the AUCs of M4 and M5 were inversely correlated with the creatinine clearance. In view of the kinetic features of slow-release formulations (flip-flop phenomenon), the delay in T(peak) of PTX in patients with severe CHF compared with moderate CHF should be ascribed to a reduced elimination rate.

BACKGROUND Cardiac allograft vasculopathy (CAV) is the major cause of graft failure and death in cardiac transplant recipients after transplantation. We aimed to evaluate the clinical and angiographic outcomes of patients with evidence of CAV who were treated percutaneously with drug-eluting stents (DES-PCI). METHODS Between December 2002 and September 2005, we reviewed the baseline procedural characteristics and the clinical outcome of consecutive cardiac transplanted recipients with evidence of CAV treated with DES-PCI, eligible for at least 9 months of follow-up. RESULTS Fifteen consecutive patients received a total of 18 coronary DESs. Total treated lesions were 20; 12 (60%) involved the left anterior descending artery. CAV was focal in nine patients, diffuse in five patients, and mixed in only one patient. Mean time interval from transplantation to DES implantation was 126 +/- 44 months. Angiographic and procedural success was 100%. There were no adverse in-hospital events. During follow-up (17.9 +/- 8.4 months), two patients died of noncardiac cause. Six patients required a new percutaneous intervention for a repeat revascularization rate of 40%: one patient underwent target lesion revascularization because of in-DES restenosis, one patient had target vessel revascularization because of a new coronary lesion proximal to the stent. Finally, four more patients underwent repeat DES-PCI in a coronary vessel other than the treated one. CONCLUSIONS Although DES implantation seems to be associated with a high procedural success rate and a low restenosis rate, the repeat revascularization rate due to CAV progression remains in nearly half of our study patients.

This study aimed to explain the functional role of lambdoid arch sutures in the development of cerebellar tonsillar herniation. Posterior cranial fossa (PCF) changes were investigated in infants with premature synostosis of the major and minor sutures of the lambdoid arch without premature synostosis of the PCF synchondroses. Morphometric and volumetric PCF measurements were performed on preoperative high-resolution CT studies in 12 infants with multisutural craniosynostosis involving the lambdoid arch and compared with those of 12 age-matched healthy subjects. All 12 patients had hypoplasia of PCF bone structures and normal volumes of the PCF and neural structures. PCF hypoplasia was related to exocciput length in infants with isolated involvement of major sutures, while it was related to posterior skull base hemifossae in infants with isolated involvement of minor lambdoid arch sutures. Foramen magnum AP diameter was reduced in babies with major suture involvement and tonsillar herniation, while foramen magnum AP and LL diameters were reduced in babies with minor suture involvement without tonsillar herniation. Right and left jugular foramen (JF) areas differed in all infants; however, the area of the smaller JF was significantly reduced only in infants with involvement of minor lambdoid arch sutures. Hypoplasia of PCF bone structures due to sutural synostosis of the lambdoid arch is a required predisposing but not sufficient factor for the development of cerebellar tonsillar herniation through the foramen magnum. Normal PCF volume and foramen magnum anatomy may partly explain the development of cerebellar tonsil herniation in infants with lambdoid arch synostosis.

This study aimed to assess changes in the posterior cranial fossa (PCF) to shed light on the mechanism of cerebellar herniation in children with Costello syndrome (CS) and posterior fossa crowding. We performed a morphovolumetric PCF analysis on brain magnetic resonance imaging (MRI) in seven children with CS (mean age 31 ± 16 months) comparing the MRI scans with those of seven age-matched healthy subjects. PCF volume (PCFV), PCF brain volume (PCFBV) and cerebellar volume (CeV) were assessed on axial T2-weighted MRI. Morphometric parameters (diameters of the foramen magnum, tentorial angle, basiocciput, supraocciput, basisphenoid and exocciput lengths) were measured on sagittal T1-weighted MRI. The volume of the cerebrospinal fluid (CSF) spaces was calculated as PCFV minus PCFBV. Five out of seven CS children showed tonsillar herniation in the upper cervical canal; no child had hydrocephalus but three out of seven children showed ventriculomegaly. In addition, the PCFV/PCFBV ratio, PCFV, CSF spaces volume, basiocciput, basisphenoid and exocciput lengths and latero-lateral and antero-posterior diameters of the foramen magnum were significantly reduced, whereas no significant changes were found in supraocciput length, PCFBV, CeV or hindbrain volume The volumetric reduction of the PCF due to bony posterior fossa hypoplasia is a predisposing factor for developing cerebellar tonsillar herniation through the foramen magnum in children with CS. The altered anatomy of the foramen magnum and upward expansion of the PCF secondary to an increased tentorial slope serves to explain the possible mechanism of cerebellar herniation in patients with CS.

Intramural left atrial dissection and hematoma as a complication of a coronary stenting procedure is a very rare entity. We report the case of a 73-year-old man who underwent percutaneous coronary angioplasty for a severe stenosis of the left circumflex coronary artery, complicated by a left atrial intramural hematoma, and was successfully treated with via a minimally invasive port-access surgical approach.

We describe the angiographic characteristics of coronary artery spasm observed in 12 out of 247 (4.9%) patients who underwent 808 coronary angiographies after heart transplantation. Coronary artery spasm was diagnosed when localized and reversible narrowing of the coronary lumen was identified. After coronary artery spasm identification all patients were followed-up clinically for a mean period of 5.1 years. Coronary artery spasm was documented 1-3 years after heart transplant. Coronary artery spasm affected 1 main coronary artery in 10 patients and 2 in 2 patients; in 3 patients 1 or more secondary branches were also affected. The right coronary artery was affected by coronary artery spasm in 8 patients and the anterior descending coronary artery in 6 patients. In 6 patients coronary artery spasm was mechanically induced by the catheter tip. The degree of luminal narrowing due to coronary artery spasm ranged from mild to almost complete occlusion. Coronary artery spasm appeared as a single tubular smooth and concentric stenosis in 8 patients, was discrete in 2 patients and multiple on the same vessel in 2 patients. In 1 patient coronary artery spasm was erroneously interpreted as an organic lesion and percutaneous transluminal coronary angioplasty was planned. During follow-up 3 patients out of 4 who had shown multiple coronary artery spasm died and 2 patients developed critical organic stenosis. In conclusion coronary artery spasm after heart transplant is less rare than commonly believed. Although it usually has a peculiar appearance, it can be misinterpreted as an organic lesion. Multiple coronary artery spasm appears to carry a poor prognosis.

A 53-year-old man with a high-risk cardiovascular profile presented to the emergency department for a subacute myocardial infarction with signs and symptoms suggesting impending myocardial rupture. The case seemed to be resolved by an early successful surgical intervention. At 6-month follow-up, after repeated cycles of cardiac rehabilitation, the echocardiography revealed a pseudo-aneurysm of the apex and a left-to-right shunt through an inter-ventricular septum defect. The patient needed a new surgical intervention to repair this unexpected complication.

OBJECTIVE: To describe a case of acute urinary retention due to bladder hypotonia during ranolazine treatment. CASE SUMMARY: An 81-year-old male with multiple cardiovascular diseases was hospitalized for worsening angina and heart failure symptoms. Ranolazine 375 mg twice daily was started, in addition to ongoing therapy (clopidogrel 75 mg once daily, diltiazem 60 mg 3 times daily, isosorbide mononitrate 40 mg 3 times daily, carvedilol 6.25 mg twice daily, rosuvastatin 20 mg once daily, enoxaparin 5000 IU once daily, pentoxifylline 600 mg twice daily, pantoprazole 40 mg twice daily, enalapril 20 mg twice daily, furosemide 150 mg once daily, and spironolactone 37 mg once daily). Two months later, the ranolazine dose was increased to 500 mg twice daily; shortly thereafter, acute urinary retention occurred and persisted despite institution of α-lytic (alfuzosin) and antiandrogenic (dutasteride) therapy. A urodynamic study revealed that urinary retention was caused by severe hypocontractility of the detrusor muscle. Ranolazine was withdrawn and, within 2 days, the patient recovered his ability to void spontaneously; a second urodynamic study confirmed that detrusor contractility was substantially improved. Drug rechallenge was not performed due to the patient's clinical condition. Nevertheless, a phenotyping test to assess the activity of the cytochrome isoenzymes CYP3A4 and CYP2D6 (responsible for ranolazine metabolism) was performed, with dextromethorphan used as the probe drug. The urinary metabolic ratios indicated relatively low activity for CYP3A4 and intermediate activity for CYP2D6. DISCUSSION: The causal role of ranolazine in our case of bladder hypotonia is probable according to the Naranjo criteria. The mechanism of bladder dysfunction is tentatively ascribed to blockage of late sodium current in smooth muscle cells. Although drug plasma concentrations were not measured, they were probably elevated, since the metabolic activity of CYP3A4 was at the lower end of the reference range. Enzyme inhibition produced by diltiazem may have contributed to decreasing CYP3A4 activity. CONCLUSIONS: Acute urinary retention in elderly men taking ranolazine may be due to drug-induced bladder hypotonia.

Pentoxifylline (PTX) is extensively metabolized in the body, and all its 3 plasma metabolites (M1, M4, M5) are pharmacologically active. The authors evaluated the pharmacokinetics of PTX and its metabolites in 20 patients with chronic heart failure (CHF). Eleven had moderate and 9 severe CHF. The time courses of PTX, M1, M4, and M5 plasma levels were determined after oral administration of a sustained-release 600-mg tablet of PTX, and for each compound, AUC, maximal plasma concentration (C(max)), and time to C(max) (T(peak)) were calculated. Compared with patients with moderate CHF, those with severe CHF showed a significant delay in T(peak) of PTX (3.9 vs 1.6 hours) and M5 (5.6 vs 3.6 hours), a 59% significant increase in M5 AUC, and a 56% nonsignificant increase in PTX AUC. In the whole population, the AUCs of PTX, M4, and M5 were inversely correlated with markers of liver function, whereas the AUCs of M4 and M5 were inversely correlated with the creatinine clearance. In view of the kinetic features of slow-release formulations (flip-flop phenomenon), the delay in T(peak) of PTX in patients with severe CHF compared with moderate CHF should be ascribed to a reduced elimination rate.

The treatment of elderly patients with coronary artery disease (CAD) is challenging because this population is complex and greatly expanding. Drug-eluting stents (DES) generally improve the outcome in high-risk cases. We evaluated the clinical impact of different first-generation DES, i.e., sirolimus-eluting stents (SES) and paclitaxel-eluting stents (PES), in this context. A prospective, nonrandomized, single-center, allcomers registry consecutively enrolling all patients aged ≥75 years eligible for percutaneous coronary intervention (PCI) with DES was carried out. Only one type of DES was implanted per protocol for each patient. Two groups were identified according to the type of implanted stent, i.e., SES and PES. The primary end point encompassed major adverse cardiac events (MACE), including death, myocardial infarction, and target lesion revascularization (TLR). The secondary end point encompassed the rate of definite/probable stent thrombosis and target vessel revascularization (TVR). From June 2004 to May 2008, 151 patients were enrolled. Among them, 112 (74.2%) received SES and 39 (25.8%) received PES. Baseline clinical characteristics were similar, while few angiographic features (ostial location, stent diameter, proximal reference vessel diameter) showed minor differences. At the median follow-up of 22.6 months, primary and secondary end points did not significantly differ in terms of MACE (SES 12.5% vs PES 20.5%, P = 0.3), death (SES 5.4% vs PES 7.7%, P = 0.7), myocardial infarction (SES 4.5% vs PES 10.3%, P = 0.2), TLR (SES 2.7% vs PES 2.6%, P = 1.0), stent thrombosis (SES 1.8% vs PES 5.1%, P = 0.3), and TVR (SES 1.8% vs PES 0%, P = 0.6). In this real-world population of elderly patients treated by DES–PCI for CAD, the overall efficacy and safety have been excellent in both DES, and the choice between SES and PES did not influence the clinical outcome.

A case of carotid artery stenting with an AngioGuard cerebral protection device is described in a patient with tight internal carotid stenosis. Deployment of AngioGuard induced sudden severe vasospasm, with subsequent minor stroke. According to our observations, AngioGuard may cause a vasospastic response with adverse neurologic effects.

Coronary artery fistulas are occasionally found in patients who undergo a coronary angiography and they may involve any epicardial coronary artery; the natural history in asymptomatic adult patients is unknown. Besides the invasive diagnosis with cardiac catheterization, it is possible to detect significant coronary fistulas also with different non-invasive methods, but they need the presence of shunt of enough size. Therapeutic options can be surgical or percutaneous. The demonstration of a communication between the coronary and distal tract of the pulmonary artery is difficult to explain with the embryogenic theory. We report the case of a patient with aortic stenosis and a shunt between the distal tract of the left pulmonary artery and the circumflex coronary artery.

The aim of the study was to compare the contractile response to ryanodine of human heart preparations taken from right and left ventricles of patients affected by idiopathic (IDCM) and secondary (SCM) end-stage dilated cardiomyopathies. Right and left ventricle myocardial strips were obtained from hearts of patients undergoing orthotopic heart transplantation and suspended in an oxygenated bath (T = 35 degrees C; stimulation frequency = 0.5 Hz). After an equilibration period, a cumulative dose-response curve for contractility (peak tension) was obtained with ryanodine (0.5, 1, 2, 4, 8, 16, 32, 64 microM). Basal contractility was not significantly different between right and left ventricles or between IDCM and SCM preparations. Ryanodine reduced peak myocardial tension but failed to completely suppress it, even at concentrations which achieved maximum effect. Ryanodine effect still persisted after a 45'-60' washout. The concentration-effect curves from IDCM right ventricle, IDCM left ventricle, SCM right ventricle and SCM left ventricle were compared: IDCM left ventricle was less sensitive to ryanodine than IDCM right ventricle and SCM left ventricle, while no difference was detectable between SCM left ventricle and SCM right ventricle. Thus, the overall sensitivity ranking was: IDCM left ventricleIDCM right ventricle = SCM right ventricle = SCM left ventricle. IDCM left ventricle showed, in addition, a biphasic response with a shift from negative to positive inotropic effect at concentrations higher than approximately 10 microM. These findings indicate that the cardio-depressant effect of ryanodine, a drug which interferes with intracellular Ca release from the sarcoplasmic reticulum, differs quantitatively and qualitatively in IDCM left ventricle from both IDCM right ventricle and SCM left ventricle. This suggests that some specific alteration in the intracellular Ca signalling in IDCM exists and, from a methodological point of view, stresses the need for a "bi-ventricular" approach to studying biochemical and functional abnormalities of advanced congestive heart failure.

Prognostic features in endomyocardial biopsy (EMB)-proven myocarditis remain poorly defined. Purpose: We assessed role of gender and of serum anti-heart (AHA), anti-intercalated disk (AIDA), anti-endothelial (AECA) and anti-nuclear autoantibodies (ANA) at diagnosis as possible predictors of death or heart transplantation (HTx). Methods: Our prospective cohort studied 250 consecutive myocarditis patients (117 with active, 102 borderline lymphocytic, 10 giant cell, 21 other histology types), 87 female, aged 37 ± 24 years, follow-up 57 ± 49 months. Polymerase chain reaction (PCR) was used to detect viral genomes on EMB. AHA (organ-specific, partially organ-specific or cross-reactive types) and AECA, AIDA, ANA were detected by indirect immunofluorescence on human heart and skeletal muscle. Univariate and multivariable Cox regression analyses for death or HTx status were used. Results: At last follow-up in May 2012, 179 patients were alive, 38 were dead or transplanted, 33 were lost to follow-up. In 20% of patients viral PCR was positive. Frequencies of positive antibody tests were as follows: AHA 55%, AIDA 17%, ANA 17%, AECA 10%. Actuarial survival at 6 years was lower in females (72% vs 87%, P=0.02). Females compared to males had higher frequency of family history of heart disease (45% vs 26%, p=0.003), extra-cardiac autoimmune disease (p=0.008), presentation with heart failure (p=0.01), higher NYHA class (p=0.03), higher frequency (p=0.009) and higher titer ANA (p=0.03). Univariate predictors of death/HTx in the whole cohort were: longer symptom duration, giant cell myocarditis, NYHA II-IV, presentation with ventricular dysfunction/symptomatic heart failure, echocardiographic and hemodynamic indexes of biventricular dysfunction, AECA, ANA. Independent predictors were female gender (p=0.01), young age (p=0.04), high titre ANA (p=0.001), high titre organ-specific AHA (p=0.02), lower echocardiographic LV ejection fraction at diagnosis (p=0.000). Conclusions: In EMB-proven myocarditis, an autoimmune pathogenesis, identified by high titer organ-specific AHA and ANA, is associated with a dismal prognosis, particularly in young females. This may reflect the well-known predilection of autoimmune disease for the female gender.