Ganglionic Eminence Anomalies and Coexisting Cerebral Developmental Anomalies on Fetal MR Imaging: Multicenter-Based Review of 60 Cases.

BACKGROUND AND PURPOSE: The ganglionic eminences are transient fetal brain structures that produce a range of neuron types. Ganglionic eminence anomalies have been recognized on fetal MR imaging and anecdotally found in association with a number of neurodevelopmental anomalies. The aim of this exploratory study was to describe and analyze the associations between ganglionic eminence anomalies and coexisting neurodevelopmental anomalies. MATERIALS AND METHODS: This retrospective study includes cases of ganglionic eminence anomalies diagnosed on fetal MR imaging during a 20-year period from 7 centers in Italy and England. Inclusion criteria were cavitation or increased volume of ganglionic eminences on fetal MR imaging. The studies were analyzed for associated cerebral developmental anomalies: abnormal head size and ventriculomegaly, reduced opercularization or gyration, and abnormal transient layering of the developing brain mantle. The results were analyzed using χ2 and Fisher exact tests. RESULTS: Sixty fetuses met the inclusion criteria (21 females, 24 males, 15 sex unknown). Thirty-four had ganglionic eminence cavitations (29 bilateral and 5 unilateral), and 26 had increased volume of the ganglionic eminences (19 bilateral, 7 unilateral). Bilateral ganglionic eminence cavitations were associated with microcephaly (P = .01), reduced opercularization, (P < .001), reduced gyration (P < .001), and cerebellar anomalies (P = .01). Unilateral ganglionic eminence cavitations were not significantly associated with any particular feature. Bilateral increased volume of the ganglionic eminences showed an association with macrocephaly (P = .03). Unilateral increased volume was associated with macrocephaly (P = .002), abnormal transient layering (P = .001), unilateral polymicrogyria (P = .001), and hemimegalencephaly (P < .001). CONCLUSIONS: Ganglionic eminence anomalies are associated with specific neurodevelopmental anomalies with ganglionic eminence cavitations and increased ganglionic eminence volume apparently having different associated abnormalities.

Assessment of the Membranous Labyrinth in Infants Using a Heavily T2-weighted 3D FLAIR Sequence without Contrast Agent Administration.

BACKGROUND AND PURPOSE: Imaging is fundamental to assessing the acoustic pathway in infants with congenital deafness. We describe our depiction of the membranous labyrinth in infants using the heavily T2-weighted 3D FLAIR sequence without a contrast agent. MATERIALS AND METHODS: We retrospectively reviewed 10 infants (20 ears) (median term equivalent age: 2 weeks; IQR: 1-5 weeks) who had undergone brain MR imaging including a noncontrast heavily T2-weighted 3D FLAIR scan of the temporal bone. For each ear, 3 observers analyzed, in consensus, the saccule, the utricle, and the 3 ampullae, assessing the visibility (score 0, not appreciable; score 1, visible without well-defined boundaries; score 2, visible with well-defined boundaries) and morphology ("expected" or "unexpected" compared with adults). The heavily T2-weighted 3D FLAIR sequence was scored for overall quality (score 0, inadequate; score 1, adequate but with the presence of image degradation; score 2, adequate). RESULTS: Six (60%) MR examinations were considered adequate (score 1 or 2). The saccule was visible in 10 ears (83.3%) with an expected morphology in 9 ears (90%). In 1 ear of an infant with congenital deafness, the saccule showed an unexpected morphology. The utricle was visible as expected in 12 ears (100%). The lateral ampulla was visible in 5 ears (41.6%), the superior ampulla was visible in 6 ears (50.0%), and the posterior ampulla was visible in 6 ears (50.0%), always with expected morphology (100%). CONCLUSIONS: MR imaging can depict the membranous labyrinth in infants using heavily T2-weighted 3D FLAIR without an injected contrast agent, but the sequence acquisition time reduces its feasibility in infants undergoing MR studies during natural sleep.

Structural brain anomalies in Cri-du-Chat syndrome: MRI findings in 14 patients and possible genotype-phenotype correlations.

INTRODUCTION: Cri-du-Chat Syndrome (CdCS) is a genetic condition due to deletions showing different breakpoints encompassing a critical region on the short arm of chromosome 5, located between p15.2 and p15.3, first defined by Niebuhr in 1978. The classic phenotype includes a characteristic cry, peculiar facies, microcephaly, growth retardation, hypotonia, speech and psychomotor delay and intellectual disability. A wide spectrum of clinical manifestations can be attributed to differences in size and localization of the 5p deletion. Several critical regions related to some of the main features (such as cry, peculiar facies, developmental delay) have been identified. The aim of this study is to further define the genotype-phenotype correlations in CdCS with particular regards to the specific neuroradiological findings. PATIENTS AND METHODS: Fourteen patients with 5p deletions have been included in the present study. Neuroimaging studies were conducted using brain Magnetic Resonance Imaging (MRI). Genetic testing was performed by means of comparative genomic hybridization (CGH) array at 130 kb resolution. RESULTS: MRI analyses showed that isolated pontine hypoplasia is the most common finding, followed by vermian hypoplasia, ventricular anomalies, abnormal basal angle, widening of cavum sellae, increased signal of white matter, corpus callosum anomalies, and anomalies of cortical development. Chromosomal microarray analysis identified deletions ranging in size from 11,6 to 33,8 Mb on the short arm of chromosome 5. Then, we took into consideration the overlapping and non-overlapping deleted regions. The goal was to establish a correlation between the deleted segments and the neuroradiological features of our patients. CONCLUSIONS: Performing MRI on all the patients in our cohort, allowed us to expand the neuroradiological phenotype in CdCS. Moreover, possible critical regions associated to characteristic MRI findings have been identified.

[Prioritization or unlimited resources in orthopedics and trauma surgery?] / Priorisierung oder unbegrenzte Ressourcen in Orthopädie und Unfallchirurgie?

The development of the healthcare system in Germany is increasingly approaching human and economic limits. A social consensus and a political concept at which point priorities are promoted and for which services the money should be primarily spent, do not exist on the whole. As soon as it becomes clear that resources are limited and that is now, prioritization has to be introduced to avoid the alternative threat of rationing of treatment benefits. The goal of prioritization is to rationally and optimally use the existing but limited resources. Medical progress and the relationship to the demographic development are the variables in the future. The individual care of the patient, patients' needs and dependence on access to treatment are the foundations of ethical actions. They must be at the center of attention for doctors and nurses because, after all they are the patient's advocates in the complex healthcare system. At the same time, unjustified claims for entitlement must be rejected just as a preservation of vested rights. Efficiency and economic considerations in diagnostics and treatment are not mutually exclusive. The physician acts as a mediator between the claims of the patient to be treated, the individual realization and the existing resources in the healthcare system.

Peri-lead edema after deep brain stimulation surgery for Parkinson's disease: a prospective magnetic resonance imaging study.

BACKGROUND AND PURPOSE: The aim of this study was to define the prevalence and characteristics of peri-electrode edema in a prospective cohort of patients undergoing deep brain stimulation (DBS) surgery and to correlate it with clinical findings. METHODS: We performed brain magnetic resonance imaging (MRI) between 7 and 20 days after surgery in 19 consecutive patients undergoing DBS surgery for Parkinson's disease. The T2-weighted hyperintensity surrounding DBS leads was characterized and quantified. Any evidence of bleeding around the leads was also evaluated. Clinical and follow-up data were recorded. In a subgroup of patients, a follow-up MRI was performed 3-6 weeks after surgery. We also retrospectively reviewed the post-operative computed tomography scans of patients who underwent DBS at our center since 2013. RESULTS: Magnetic resonance imaging showed a peri-lead edematous reaction in all (100%) patients, which was unilateral in three patients (15.8%). In six patients (31.6%), we detected minor peri-lead hemorrhage. Edema completely resolved in eight out of 11 patients with a follow-up MRI and was markedly reduced in the others. Most patients were asymptomatic but six (31.6%) manifested various degrees of confusional state without motor symptoms. We found no significant correlation between edema volume, distribution and any clinical feature, including new post-operative neurological symptoms. The retrospective computed tomography analysis showed that peri-electrode hypodensity consistent with edema is absent at early post-operative imaging but is common at scans performed >3 days after surgery. CONCLUSIONS: Peri-electrode edema is a common, transient reaction to DBS lead placement and a convincing relation between edema and post-operative clinical status is lacking.

Multiparametric MR imaging of Parkinsonisms at 3 tesla: Its role in the differentiation of idiopathic Parkinson's disease versus atypical Parkinsonian disorders.

OBJECTIVES: The Nigrosome-1 and putaminal hypointensity depicted on susceptibility-weighted imaging (SWI), and midbrain atrophy assessed on T1-weighted are some of the most common radiological parameters to diagnose Parkinsonism at Magnetic Resonance (MR) imaging. Our aim is to assess the feasibility of these signs in the differentiation of Idiopathic Parkinson's disease (IPD) patients versus disease (DC) and healthy controls (HC) and in the assessment of the Atypical Progressive Parkinsonisms (APPs). METHODS: Presence or loss of the Nigrosome-1 was assessed retrospectively on multiple-echo SWI obtained on a 3 T scan by two neuroradiologists. Results were compared with the 123I-FP-CIT SPECT images. Morphologic diagnostic features suggestive of APPs such as midbrain atrophy and putaminal hypointensity were evaluated by qualitative scores. The midbrain and putaminal scores were summed (combined score) and then added to the Nigrosome-1 score (global score). RESULTS: The study included 126 patients with IPD (n = 56), APPs patients (n = 30; 18 PSP, 3 MSA-C, 9 MSA-P), 16 DC and 24 HC. Sensitivity and specificity of the Nigrosome-1 in discriminating IPD from controls were 96,43% and 85.00%, APPs from controls were 100% and 85%, IPD from APPs were 96,43% and 0% respectively. Combined score for midbrain atrophy and putaminal hypointensity resulted in the most accurate for distinguishing APPs from IPD with a value of ≥ 2 (AUC = 0.98). CONCLUSION: Nigrosome-1 is a valid tool to differentiate IPD-APPs from controls. The combined score of midbrain atrophy and putaminal hypointensity represents a valid diagnostic pointer in the differential diagnosis of APPs from IPD.

MR Imaging in Menière Disease: Is the Contact between the Vestibular Endolymphatic Space and the Oval Window a Reliable Biomarker?

BACKGROUND AND PURPOSE: No reliable MR imaging marker for the diagnosis of Menière disease has been reported. Our aim was to investigate whether the obliteration of the inferior portion of the vestibule and the contact with the stapes footplate by the vestibular endolymphatic space are reliable MR imaging markers in the diagnosis of Menière disease. MATERIALS AND METHODS: We retrospectively enrolled 49 patients, 24 affected by unilateral sudden hearing loss and 25 affected by definite Menière disease, who had undergone a 4-hour delayed 3D-FLAIR sequence. Two readers analyzed the MR images investigating whether the vestibular endolymphatic space bulged in the third inferior portion of the vestibule contacting the stapes footplate. This sign was defined as the vestibular endolymphatic space contacting the oval window. RESULTS: We analyzed 98 ears: 27 affected by Menière disease, 24 affected by sudden sensorineural hearing loss, and 47 that were healthy. The vestibular endolymphatic space contacting the oval window showed an almost perfect interobserver agreement (Cohen κ = 0.87; 95% CI, 0.69-1). The vestibular endolymphatic space contacting oval window showed the following: sensitivity = 81%, specificity = 96%, positive predictive value = 88%, and negative predictive value = 93% in differentiating Menière disease ears from other ears. The vestibular endolymphatic space contacting the oval window showed the following: sensitivity = 81%, specificity = 96%, positive predictive value = 96%, negative predictive value = 82% in differentiating Menière disease ears from sudden sensorineural hearing loss ears. CONCLUSIONS: The vestibular endolymphatic space contacting the oval window has high specificity and positive predictive value in differentiating Menière disease ears from other ears, thus resulting in a valid tool for ruling in Menière disease in patients with mimicking symptoms.

MR imaging of endolymphatic hydrops in Ménière's disease: not all that glitters is gold.

Ménière's disease (MD) is a chronic condition characterised by fluctuating hearing loss, intermittent vertigo, tinnitus and aural fullness. Its anatomical and pathological counterpart is represented by endolymphatic hydrops (EH). Recent development and progress in magnetic resonance (MR) imaging techniques has enabled visualisation of EH in living human subjects using a 3 Tesla (T) scanner and gadolinium-based contrast-agent (GBCA) via intravenous (IV) or intra-tympanic (IT) administration. Data emerging from the literature about MR imaging of EH in MD patients are limited, and we therefore reviewed the most common MR imaging findings in the study of the endolymphatic space in both MD and non-MD patients.

Flat Panel Angiography in the Cross-Sectional Imaging of the Temporal Bone: Assessment of Image Quality and Radiation Dose Compared with a 64-Section Multisection CT Scanner.

BACKGROUND AND PURPOSE: Cross-sectional imaging of the temporal bone is challenging because of the complexity and small dimensions of the anatomic structures. We evaluated the role of flat panel angiography in the cross-sectional imaging of the temporal bone by comparing its image quality and radiation dose with a 64-section multisection CT scanner. MATERIALS AND METHODS: We retrospectively collected 29 multisection CT and 29 flat panel angiography images of normal whole-head temporal bones. Image quality was assessed by 2 neuroradiologists, who rated the visualization of 30 anatomic structures with a 3-point ordinal scale. The radiation dose was assessed with an anthropomorphic phantom. RESULTS: Flat panel angiography showed better image quality than multisection CT in depicting the anterior and posterior crura of the stapes, the footplate of the stapes, the stapedius muscle, and the anterior ligament of the malleus (P < .05). In contrast, multisection CT showed better image quality than flat panel angiography in assessing the tympanic membrane, the bone marrow of the malleus and incus, the tendon of the tensor tympani, the interscalar septum, and the modiolus of the cochlea (P < .05). Flat panel angiography had a significantly higher overall image quality rating than multisection CT (P = .035). A reduction of the effective dose of approximately 40% was demonstrated for flat panel angiography compared with multisection CT. CONCLUSIONS: Flat panel angiography shows strengths and weaknesses compared with multisection CT. It is more susceptible to artifacts, but due to the higher spatial resolution, it shows equal or higher image quality in assessing some bony structures of diagnostic interest. The lower radiation dose is an additional advantage of flat panel angiography.

MR Imaging in Sudden Sensorineural Hearing Loss. Time to Talk.

Sudden sensorineural hearing loss is defined as acute hearing loss of the sensorineural type of at least 30 dB over 3 contiguous frequencies that occurs within a 72-hour period. Although many different causative factors have been proposed, sudden sensorineural hearing loss is still considered "idiopathic" in 71%-85% of cases, and treatments are empiric, not based on etiology. MR imaging implemented with a 3D FLAIR sequence has provided new insights into the etiology of sudden sensorineural hearing loss. Herein, we review the current management trends for patients with sudden sensorineural hearing loss, from the initial clinical diagnosis to therapeutic strategies and diagnostic work-up. We focused primarily on MR imaging assessment and discuss the relevance that MR imaging findings might have for patient management, pointing out different perspectives for future clinical research.

[Reliability of venous blood gas analysis and radionuclide angiography in post-traumatic dystrophy]. / Reliabilität der venösen Blutgasanalyse und Radionuklidangiographie bei Posttraumatischer Dystrophie.

BACKGROUND: The diagnosis "post-traumatic dystrophy" (PTD) was first defined with clinical and paraclinical criteria by Scola et al. in 2013. OBJECTIVES: The objectivity and reliability of the paraclinical criteria (venous blood gas analysis [vBGA], radionuclide angiography [RNA]), and recommendations for therapy should be assessed in a prospective study. MATERIALS AND METHODS: In five patients with clinical signs of post-traumatic nonbacterial inflammation of the hand, both diagnosis and a 3­week hospital treatment were carried out in accordance with the publication mentioned above. The primary traumata (four fractures and one soft-tissue injury) were located in either the hand or the forearm. Unsuccessful outpatient treatment always led to hospital admission. One patient with severe osteopenia in the hand skeleton was treated with bisphosphonates for 6 months. RESULTS: All patients fulfilled the clinical and paraclinical criteria for the diagnosis of PTD. On admission, an elevated venous partial pressure of oxygen was found by vBGA in the affected hand (∆pO2 mean 22 ± 3 mm Hg) and a hyperperfusion due to arteriovenous shunts was measured using RNA (mean 75 ± 47%). The symptomatic treatment was extremely well tolerated; by the time of discharge, all patients achieved full functioning of the hand with minor loss of strength (venous ∆pO2 mean 5 ± 3 mm Hg). The osteopenia in the one patient treated with bisphosphonates showed recalcification after 6 months. CONCLUSION: The reliability of clinical and paraclinical criteria for PTD were confirmed. vBGA and RNA seem to be good parameters for confirming the diagnosis of PTD. "Rubor," a symptom traditionally interpreted as "hyperemia," contradicts the paraclinical findings and leads to the assumption that the cause of this post-traumatic syndrome is microvascular dysfunction.

[Bone resorption in posttraumatic dystrophy. Root cause analysis based on the literature]. / Knochenresorption bei posttraumatischer Dystrophie. Ursachenanalyse anhand der Literatur.

BACKGROUND: In posttraumatic dystrophy the resorption of cancellous bone remains an unsolved phenomenon. OBJECTIVES: The possible effects of arteriovenous (av) anastomoses, hypoxia and acidosis in posttraumatic dystrophy on cancellous bone structures should be elucidated. MATERIAL AND METHODS: In posttraumatic dystrophy of the hand after distal radius fracture, hand fractures and elective hand surgery [20] the results suggest that persistent av anastomoses could be the reason for this syndrome. Possible pathways for the occurrence of cancellous bone resorption are discussed in context with the literature. RESULTS AND CONCLUSION: Intraosseous av anastomoses with acidosis and hypoxia of the tissues are probably responsible for the excessive activity of osteoclasts in acute posttraumatic dystrophy. Even enhancements in the late static phase of the three phase bone scan (TPBS) are in agreement with this hypothesis. In cancellous bone these enhancements are induced by the bone seeking tracers. The nomenclature for these tracer molecules is in line with the recommendations of International Union of Pure and Applied Chemistry (IUPAC) as methylene bisphosphonate and hydroxymethylene bisphosphonate. From this, therapeutic recommendations for posttraumatic dystrophy can be derived. The term diphosphonates should be changed to bisphosphonates.

[Posttraumatic dystrophy. Diagnosis and therapy after distal radius fractures and hand injuries]. / Die posttraumatische Dystrophie. Diagnostik und Therapie nach distalen Radiusfrakturen und Handverletzungen.

BACKGROUND: Posttraumatic dystrophy manifests as signs and symptoms of endothelial dysfunction in the microcirculation with pronounced involvement of arteriovenous (AV) anastomosis. PATIENTS AND METHODS: Blood gas analysis was performed in 28 patients with persistent painful swelling 6-14 weeks after distal radius fracture or hand injury. The patients showed higher levels of venous oxygenation on the affected side in comparison to the contralateral arm. Furthermore, an increased perfusion and an AV shunt situation were proven by radionuclide angiography. These findings correlate with insufficient oxygen utilization by the tissue (dystrophy = capillary malperfusion). Pathophysiologically, complex processes must be assumed which require a multimodal therapy and treatment focuses on open AV shunts. RESULTS: The disastrous consequences of hand dystrophy (loss of function due to fibrosis) could be prevented in all patients. Statistical analyses between affected and normal hands showed significant differences. CONCLUSION: Posttraumatic dystrophy needs early detection for successful therapy and should not be identified under the umbrella term "complex regional pain syndrome" (CRPS).

White matter damage in frontotemporal lobar degeneration spectrum.

White matter (WM) tract damage was assessed in patients with the behavioral variant frontotemporal dementia (bvFTD) and the 3 primary progressive aphasia (PPA) variants and compared with the corresponding brain atrophy patterns. Thirteen bvFTD and 20 PPA patients were studied. Tract-based spatial statistics and voxel-based morphometry were used. Patients with bvFTD showed widespread diffusion tensor magnetic resonance imaging (DT MRI) abnormalities affecting most of the WM bilaterally. In PPA patients, WM damage was more focal and varied across the 3 syndromes: left frontotemporoparietal in nonfluent, left frontotemporal in semantic, and left frontoparietal in logopenic patients. In each syndrome, DT MRI changes extended beyond the topography of gray matter loss. Left uncinate damage was the best predictor of frontotemporal lobar degeneration diagnosis versus controls. DT MRI measures of the anterior corpus callosum and left superior longitudinal fasciculus differentiated bvFTD from nonfluent cases. The best predictors of semantic PPA compared with both bvFTD and nonfluent cases were diffusivity abnormalities of the left uncinate and inferior longitudinal fasciculus. This study provides insights into the similarities and differences of WM damage in bvFTD and PPA variants. DT MRI metrics hold promise to serve as early markers of WM integrity loss that only at a later stage may be detectable by volumetric measures.

[Dislocated supracondylar humerus fracture in the child. Surgical technique and outcome with dorsal approach]. / Die dislozierte suprakondyläre Humerusfraktur des Kindes. Operationstechnik und Ergebnisse mit dem dorsolateralen Zugang.

Anatomical reduction and stabilization of displaced supracondylar humeral fractures in children is necessary to obtain good results. For most cases percutaneous crossed pinning is recommended. Sometimes open reduction is necessary but even in these cases neurological complications and varus deformities have been reported. So the technique of open pinning was modified. From 1995 to 1998 22 children were treated by a dorsolateral approach. The fracture was stabilized by crossed pinning: The proximal K-wire is drilled 10 degrees ascending to the dorsal humerus through the medial pillar into the ventral part of the medial epicondyle, after shortening it is not bent. The distal K-wire stabilizes the lateral pillar, after shortening its end is bent down. Immobilization for 3-4 weeks, mobilization is done by the patient. The implants are removed 2 weeks later. The follow up in 21 out of 22 patients (8-57 months, mean 35 months) according to Flynn's criteria showed 16 excellent, 4 good and 1 fair result. The fair result was due to valgus deformity. One patient has been reoperated due to displacement of K-wire. Neither iatrogenic nerve lesions nor varus deformities nor infections did occur. The dorsolateral approach combined with the above mentioned technique of pinning shows excellent and good results.

[Morphological changes in arterial ruptures]. / Morphologische Veränderungen bei Arterienrupturen.

The historical opinion that the intima layer of a ruptured artery of muscular type could stop bleeding by "rolling in" should be controlled experimentally. Five segments of human femoral/popliteal artery (3-4 cm long) were overstretched until complete rupture occurred. Furthermore a longitudinally split and a partially oblique incised segment were ruptured. As morphological finding a sandclock deformity was observed in the region of rupture. This phenomenon was induced by adventitia layer, which closed the ends of ruptured media layer like a Chinese finger trap. In the longitudinally split segment a transverse rupture of the media layer could be observed, while the fibers of adventitia layer were pulled out when the traction was continued. Neither macroscopical nor microscopical signs could be found for "rolling in" of intima or media layer. The reason for spontaneous hemostasis after arterial rupture is more likely the activation of platelets by collagenous fibers of adventitia layer than "rolling in" of intima or media layer. If there is no finger trap mechanism of adventitia layer like in shot- or stab wounds a massive blood loss must be expected.

The Gregorio Marañón Hospital experience with vertical partial laryngectomies.

We present a retrospective study of 551 patients treated with conservative surgery for glottic carcinoma at the Gregorio Marañón Hospital between 1962 and 1996. In all, 12% of cases were locally advanced carcinomas. In early-stage carcinomas there were no statistical differences in 5-year survival between those treated by endoscopic laser resection, vertical hemilaryngectomy and radiotherapy. However, tumor recurrence after primary radiotherapy was higher (27%) than with conservative surgery (12%), while the voice preservation rate was significantly higher with surgery (83%) than with radiotherapy (72%). With locally advanced cancer, irradiated patients (to 60 Gy) had a 50% probability of recurrence with a very low chance for salvage by total laryngectomy (5-year survival rate, 38.5%). In contrast, partial laryngectomy could be performed on carefully selected patients, and the results for these patients were comparable to those for smaller lesions (with a 5-year survival rate of 81%).