[Currarino syndrome: variability of imaging findings in 22 molecular-genetically identified (HLXB9 mutation) patients from five families]. / Currarino-Syndrom: Variabilität der bildgebenden Befunde bei 22 molekulargenetisch identifizierten (HLXB9-Mutation) Patienten aus fünf Familien.

PURPOSE: The imaging documents, obtained in connection with a primarily molecular genetic study on Currarino syndrome, should be evaluated with special respect to the constancy resp. the variability of findings in patients with proven HLBX9 mutations. METHODS: In five female non-related index patients with clinico-radiologically diagnosed Currarino syndrome and further 53 members of their families, changes of the homeobox gene HLXB9 had been analysed. Variable mutations of this gene were found in a total of 23 individuals including the five index patients. In 22 of them the preexisting radiological documents could be collected as well as further imaging (plain sacrococcygeal radiography and/or lumbosacral MRI at least) initiated. This was followed by a detailed evaluation of pathological findings in the os sacrum/coccyx as well as in the presacral, the intraspinal, the anorectal, and the urogenital region, finally. RESULTS: Imaging investigations revealed concomitant phenotypic abnormalities in all and even nine clinically asymptomatic individuals with proven HLXB9 mutations. A sacrococcygeal defect of varying intensity was depicted in every case. Complete Currarino triad (i. e. sacrococcygeal defect, presacral mass = anterior meningocele and/or tumor, anorectal malformation) was only found in the five index patients and three further relatives. In all other cases, one or more of the following anomalies were detected with variable combination and with decreasing frequency: anterior meningocele (12), presacral tumor (11), tethered cord (10), intraspinal lipoma (8), anorectal stenosis/atresia (8), syringocele (5), rectal fistula (3), urogenital (2). CONCLUSION: Currarino syndrome should be considered as a differential diagnosis in all patients with chronic constipation since early infancy and its imaging index finding, i. e. a sacrococcygeal defect, should be looked for with plain radiography, first. In positive cases or other phenotypic suspicious constellations molecular genetic analysis for HLBX9 mutations should be the next step. If positive again, this should be followed by complete adequate imaging in the patient as well as by plain sacrococcygeal radiography in, at least, symptomatic family members.

[Diagnostic imaging in invasive cervical carcinoma: MRI, CT, and ultrasonography]. / Bildgebende Diagnostik beim invasiven Zervixkarzinom: MRT, CT und Ultraschall.

Carcinoma of the cervix is the fourth most common cancer in women. Accurate staging of the disease is essential in selecting optimal therapy. The clinical staging based on the criteria of the FIGO is inaccurate. With MRI an excellent imaging of the tumour spread within the cervix can be achieved, also tumour extension to the parametria and infiltration of the neighbouring structures as bladder and rectum. MRI is the method of choice in the preoperative staging of cervical cancer. Computed tomography and sonography have lower staging value compared to the MRI. An advantage of CT, compared with MRI, is the high accuracy in investigations of patients with ureteral obstruction and hydronephrosis. Both methods MRI and CT are comparable in assessing pelvic and paraaortic lymph node metastases.

Effects of prosthetic valve placement on mitral annular dynamics and the left ventricular base.

Insertion of a rigid mitral prosthesis impairs the function of the mitral annulus and induces systolic narrowing of the left ventricular outflow tract (LVOT). To study this mechanism, we investigated dynamic changes in the left ventricular (LV) base, which consists of the mitral annulus and LVOT orifice. In seven patients with mechanical mitral valve prostheses and eight normal subjects, the image of the LV base was reconstructed three-dimensionally and its dynamic change during systole was studied. In the patients, the rigid prosthetic valve (=mitral annulus) tilted toward the left ventricle with a hinge point at the posterior mitral annulus during systole. The left ventricular base exhibited contraction, but the size of the prosthetic valve was constant. As a consequence, the prosthetic valve occupied more of the left ventricular base, which resulted in narrowing of the LVOT. In the normal subjects, the mitral annulus did not interfere with the region of the LVOT orifice during systole as the mitral annulus underwent both dorsiflexion and contraction. Thus, fixation of the mitral annulus induces an anti-physiologic motion of the annulus. Conscious preservation of annular flexibility in mitral valve surgery is important in avoiding potential dynamic LVOT obstruction.

CSF flow studies of intracranial cysts and cyst-like lesions achieved using reversed fast imaging with steady-state precession MR sequences.

BACKGROUND AND PURPOSE: Differentiating between intracranial cysts or cyst-like structures and communicating or noncommunicating cysts is often not possible with cranial CT or nonfunctional MR imaging. We evaluated a retrospective ECG-gated fast imaging with steady-state precession (PSIF) MR sequence with optional cine mode to differentiate cystic masses from enlarged CSF spaces and to determine the accuracy of detecting communication between cysts and neighboring CSF spaces. METHODS: Fourteen patients with intracranial cystic masses underwent CSF flow studies with an ungated and a retrospective ECG-gated cine-mode PSIF sequence in addition to spin-echo imaging. Findings were evaluated retrospectively by using a five-point rating scale and without knowledge of clinical or other imaging findings. Results were compared with intraoperative findings or with results of intrathecal contrast studies. RESULTS: Eighteen arachnoid cysts and one enlarged cisterna magna were diagnosed. Improved differentiation between cysts and enlarged CSF spaces was obtained with cine-mode PSIF imaging in six lesions (six patients). Increased diagnostic certainty as to communication between cysts and CSF spaces was obtained in 18 cysts (13 patients). Diagnoses were verified by membranectomy in five lesions, by CT cisternography in five lesions, and indirectly by shunting in one cystic lesion. In one case, MR diagnosis was not confirmed by CT cisternography. CONCLUSION: Cine-mode MR imaging with a retrospective ECG-gated flow-sensitive PSIF sequence contributed to the certainty of communication between arachnoid cysts and neighboring CSF spaces with an accuracy of 90%, using surgical findings or intrathecal contrast studies as reference. Differentiation between intracranial cysts and enlargement of CSF spaces and other cystic masses was improved in 25% of cases.

Pre-operative staging of cervical cancer: comparison of magnetic resonance imaging (MRI) and computed tomography (CT) with histologic results.

Thirty-two patients with histologically confirmed cervical carcinoma were preoperatively investigated using MRI; in addition, a CAT-scan was performed on 15 of these patients. The diagnostic results using both modalities were compared with the histological findings (after hysterectomy according to Wertheim-Meigs, including lymph node dissection in the pelvic and, in part, in para-aortal regions). Determination of tumour volume was possible with high accuracy using MRI. Accuracy in assessing the parametria was 86%, vagina 90%, bladder and rectum 97%. The shortcoming of MRI is still the detection of infiltrated lymph nodes. The accuracy of 69% achieved for lymph nodes is equal to results with computed tomography. The general accuracy for our patients in staging was 81% for MRI versus 47% for CT. MRI-based diagnosis enables us to determine a correct tumour staging preoperatively, and is therefore very helpful in planning an adequate therapy. If MRI were used more widely it would contribute to simplification and shortening of the preoperative diagnostic procedure in patients with cervical carcinoma.

The effects of the use of piezoelectric motors in a 1.5-Tesla high-field magnetic resonance imaging system (MRI).

PURPOSE: This paper presents the results of an experimental investigation with two different rotatory piezomotors in a closed 1.5 Tesla high-field MRI. The focus of the investigation was on testing the functionality of these motors within the MRI and to determining the image interference they caused. MATERIALS AND METHODS: To obtain a differentiated estimate of the interference the motors were tested in both the passive (turned off, i.e. without current flow) and active (turned on, i.e. with current flow) state during MRI scanning. Three different types of sequences were used for the test: Spin-Echo (SE), Gradient-Echo (GE) and Echo-Planar Imaging (EPI). A plastic container filled with a gadolinium-manganese solution was used for representation of the artefacts. The motors investigated were placed parallel to the container at predetermined distances during the experiment. RESULTS AND CONCLUSIONS: The results show that the motors investigated suffered no functional limitations in the magnetic field of the MRI but, depending on the type of motor, the measurement distance and the state of the motor, the motors had different effects on the sequence images. A motor in the off-state placed immediately next to the object to be measured mainly causes artefacts because of its material properties. If, on the other hand, the piezomotor is in the on-state images with strong noise result when the motor is immediately next to the object being measured. The images regain their normal quality when the motor is approximately at a distance of 1 m from the object being investigated. Driving the motor inside the MRI, therefore, is only to be recommended during the pauses in scanning: this delivers artefact-free images if minimal, motor-specific distances are kept to. With regard to the three different types of sequences it was determined that the SE sequence was the least sensitive and the EPI sequence the most sensitive to disturbance. The GE sequence showed only minimal differences to the SE sequence with regard to signal-to-noise ratios. Since it requires considerably shorter scan-times it can be considered to be the most effective type of sequence under these conditions.

1H and 31P NMR characterisation of a double breast coil for spectroscopic measurements and imaging.

For the first time a double turn breast coil has been described which can be used for 1H imaging, 1H spectroscopy and 31P spectroscopy. The paper describes basic technical features of the coil, coil design, B1 field/excitation field distribution for 1H and 31P, sensitivity, and feasibility for 31P spectroscopic in vivo studies. The main advantage of the double frequency tuneable coil is that 1H imaging for tumor localization and 31P spectroscopy for response control can be done without an additional repositioning of the patient.

[Malignant lymphoma: magnetic resonance tomography findings in residual supradiaphragmatic space-occupying lesions]. / Malignes Lymphom: Magnetresonanztomographische Befunde bei residuellen supradiaphragmalen raumfordernden Läsionen.

PURPOSE: Evaluation of MR imaging in patients with Hodgkin's lymphoma and high grade non-Hodgkin's-lymphoma and mediastinal residual mass after first line chemotherapy. MATERIALS AND METHODS: MR imaging (1.5 T) was performed in 36 patients (Hodgkin's lymphoma n = 26, NHL n = 10) after first line chemotherapy). Twenty patients had inactive residual mass, 16 patients had residual lymphoproliferative lesions. T1- and T2-weighted spin echo images were visually analysed by a score index (range 1-5) as well as quantification of enhancement by signal-intensity-ratios SImax/SIplain). RESULTS: For the differentiation between residual lymphoproliferative activity and inactive residual mass, the highest accuracy was obtained for the signal intensity of residual mass on T2-w-SE compared to pectoralis muscle (94% sensitivity, 80% specificity, likelihood ratios: 4.0 [LR+]; 0.3 [LR-]). The cut-off value of the SI ratio was calculated retrospectively at 1.96 (p > 0.05). CONCLUSIONS: Differentiation between inactive (fibrotic) and lymphoproliferative (active) residual mediastinal mass is possible by MR imaging using as parameter the size reduction after therapy and the signal intensity on T2-w-SE in comparison to pectoralis muscle. Thus study suggests an additional value using the SI ratio for the differentiation.

Gadofluorine 8: initial experience with a new contrast medium for interstitial MR lymphography.

RATIONALE AND OBJECTIVES: Differential diagnosis of malignant and beign lymph nodes is still a problem in lymphographic imaging modalities. Plain magnetic resonance imaging (MRI) and computed tomography (CT) are inadequate for detecting metastases in normal-sized lymph nodes and for differentiating enlarged nodes. Therefore it is important to have a contrast agent that accumulates in healthy lymphatic tissue but does not accumulate in metastatic deposits. METHODS: The lymphographic contrast agent Gadofluorine 8 (Schering AG, Berlin, Germany) is a lipophilic but water-soluble gadolinium complex. Lymphographic effects were investigated in guinea pigs, dogs, and tumor-bearing rabbits after interstitial (subcutaneous or intracutaneous) injection. MR imaging was performed using T1-weighted gradient-echo sequences until 120 min after administration. RESULTS: After interstitial injection Gadofluorine 8 accumulates in regional lymph nodes, resulting in a pronounced increase in signal intensity in the lymph nodes. Differentiation between normal and metastatic lymph nodes was achieved. CONCLUSIONS: Gadofluorine 8 is an innovative contrast agent that can distinguish between normal and tumorous lymph nodes in interstitial MR lymphography.

Staging and monitoring of malignant lymphoma of the bone: comparison of 67Ga scintigraphy and MRI.

UNLABELLED: The aim of this study was two-fold: to compare 67Ga scintigraphy with MRI (a) for the staging of malignant lymphoma of the bone and (b) with regard to accuracy in detecting residual disease after first-line chemotherapy for restaging. METHODS: Twenty-one patients with 36 malignant osseous lesions were examined, including 7 patients with primary or multifocal osseous lymphoma and 14 patients with malignant lymphoma and simultaneous or secondary involvement of the bone. After first-line therapy, MRI and 67Ga scintigraphy were performed on 13 patients. The remission status based on all clinical and radiological findings during the follow-up was used as the gold standard. RESULTS: The osseous lesions were located on the axial skeleton in 64% of patients and on the appendicular skeleton in 36%. 67Ga scintigraphy detected 77% of the osseous lesions examined by MRI. For restaging after first-line therapy, MRI had a sensitivity of 90% and a specificity of 80% when dynamic MRI information was included. There were several false-positive results as a result of the pathologic increase in signal intensity ratios of reactive hematopoietic regions after chemotherapy. For 67Ga scintigraphy, a sensitivity of 70% and a specificity of 93% were calculated. CONCLUSION: These data show that monitoring malignant lymphoma of the bone still presents diagnostic problems. Given the high sensitivity of MRI and the high specificity of 67Ga scintigraphy but the limited specificity of MRI and sensitivity of 67Ga scintigraphy, both methods are valuable but should be used as complementary diagnostic tools.

Signal-enhanced color Doppler sonography of deep venous thrombosis in the lower limbs and pelvis.

Detection of Doppler signal tends to be more difficult in peripheral veins owing to low flow velocity. This can be caused by nonoccluding thrombosis, post-thrombotic wall changes, or a deep anatomic location of pelvic veins. The last-mentioned frequently is accompanied by interference by bowel gas. In addition, inappropriate insonation angles adversely affect the outcome of color-coded Doppler interrogation. The purpose of the present study was to evaluate the effectiveness of signal-enhanced color Doppler sonography on peripheral veins in 31 patients clinically suspected of having deep vein thrombosis. As a result of diagnostic uncertainty, additional enhanced studies were performed on 43 venous segments. The enhancement led to a decrease in false-positive results (from four patients to one patient) and false-negative results (from four patients to two patients) compared to unenhanced studies. Evaluation of the deeply located pelvic veins profited the most through signal enhanced Doppler sonography.

[Cervix carcinoma: staging, therapy, after-care. Experiences with magnetic resonance tomography of cervix carcinoma based on recent literature 1993 to 1997]. / Zervixkarzinom: Staging, Therapie, Nachsorge. Die Erfahrungen in der Magnetresonanztomographie des Zervixkarzinoms anhand der neuen Literatur von 1993 bis 1997.

The investigation of cervix carcinoma with magnetic resonance tomography (MRT) is still controversially discussed with regard to its diagnostic value as well as for planning radiation therapy. The purpose of this article is to present and discuss papers published between 1993 and 1997 in this field with respect to the technique used, the contrast media applied and its clinical value. A literature search using three different databases (Medline, Embase, Cancerlit) identified 39 publications, which were then analysed. Despite the partially suboptimal presentation of results in these papers MRT proved superior to other imaging modalities. Due to better demarcation of cervix carcinoma with MRT, it was possible to calculate tumor value as well as to correctly judge the infiltrative character. This allows for a more precise treatment and staging of the patient's prognosis. In the future, MRT might be useful in diagnosing recurrence at relatively early stage. Unfortunately lymphatic nodes can only be insufficiently verified using MRT.

The relationship between the mitral annulus and left ventricular outflow tract.

Mitral annular inflexibility due to rigid prostheses (ring or valve) has long been considered to contribute to the mechanism of dynamic left ventricular outflow tract (LVOT) obstruction after mitral repair or replacement. In clarifying the geometric relationship between LVOT orifice and mitral valve annulus (MVA) in eight normal subjects, the authors have endeavored to show how that a rigid mitral prosthesis might obstruct the LVOT based on the assumption that any rigid prosthesis necessarily follows the motion of the posterior half of the MVA (MVApost) in the course of every heart beat. During systole, the relationship between the MVApost and the approximated plane of the LVOT orifice was constant. However, with the respect to the relationship between the LVOT orifice and the approximated plane of the MVApost (PI-MVApost), the intersection between the two shifted toward the apex during systole. Assuming the prosthesis is aligned on the MVApost with the same orientation as the PI-MvApost, this shift implies a reduction in the effective size of the LVOT orifice due to the prosthesis. The calculated obstruction rate was 24.9% (0 ms), 30.9% (100 ms), 35.5% (200 ms), and 45.4% (300 ms). These results indicate the importance of maintaining the flexibility of the MVA after mitral valve surgery.

Dynamic change in the left ventricular base with or without a rigid mitral valve prosthesis.

To evaluate the narrowing of the left ventricular outflow tract (LVOT) during systole caused by a rigid mitral prosthesis, the geometric relationship between the prosthesis (or the mitral annulus) and the left ventricular base (LVB) was studied in five patients with mechanical mitral valve prostheses and eight normal subjects. The images of the mitral valve annulus (MVA) and the LVOT orifice reconstructed in three dimensions were projected on the plane of the LV base. Calculating the areas of these projected images (i.e., those for MVA [Sm], LVOT orifice [So], the LVB [Sb; Sb = Sm + So]), the MVA-LVB ratio (Sm/Sb) was determined. In the normal subject, the MVA-LVB ratio was nearly constant during systole (59 +/- 5% at 0 msec and 62 +/- 7% at 300 msec, respectively), whereas in the patients with prostheses, the ratio increased from 61 +/- 4% (0 msec) to 69 +/- 4% (300 msec). The increase in MVA-LVB ratio reduces the proportionate share of LVOT orifice in relation to the total LVB. The ideal mitral valve prosthesis should be flexible at the annulus to attain good performance in LVB dynamics.

Imaging of the orifice of the left ventricular outflow tract: technique and initial results.

The mechanism of left ventricular outflow tract (LVOT) obstruction in the patient after mitral valve replacement or repair was examined with the aid of 2D echocardiography. For the interpretation of the spatial relationship between the aortic root and mitral annulus, however, the 2D display is sometimes inadequate since it may not simultaneously capture these structures in each center. We developed a method to clarify this relationship in 3D based on magnetic resonance images. We defined the office of the left ventricular outflow tract (LVOT orifice), consisting of, in turn, a muscular region, i.e., edge of the interventricular septum, and an annular region, i.e., the annulus of the anterior mitral leaflet. In this paper we present image data obtained from one of eight normal subjects examined and compare this with data of one of two patients who preoperatively suffered degenerative mitral regurgitation and subsequently underwent chordal-preserving mitral valve replacement, in which anterior chordae were reattached to the anterior annulus. In the normal subject, the mitral annulus exhibited a flexible change in shape during the systole, maintaining sufficient LVOT orifice size. In the patient, the prosthetic valve exhibited translational motion during systole, resulting in dynamic LVOT obstruction. This phenomenon was also observed in one other case. Furthermore, the lateral view of the LVOT orifice revealed a projection of the prosthetic valve into the LVOT, causing mechanical LVOT obstruction. The finding that translational motion of the prosthetic valve with an inflexible mitral annulus results in dynamic LVOT obstruction may support the hypothesis that annular rigidity causes dynamic LVOT obstruction after mitral valve repair with a rigid prosthetic ring.

Mitral annular flexibility.

An analysis of three-dimensional movement of the mitral valve annulus (MVA) may address the question of geometrical change after mitral valve repair to preserve mitral annular function. Conventionally, annular contraction has been studied for this purpose. We investigated this geometrical change occurring in the anterior half of the MVA and discuss its clinical significance. Three-dimensional images of the MVA during systole were reconstructed from magnetic resonance images of eight normal subjects. The posterior half of the MVA exhibited translational motion. We assume that this portion, exhibiting translational motion as well as contraction, purely follows the motion of the left ventricular contraction. Compensating for the discrepancy between the motion of the aortic root and that of the posterior half of the MVA, the anterior half exhibited a flexible change in shape during systole, thus maintaining a sufficient left ventricular outflow tract (LVOT). The increase in the extent of displacement of the anterior MVA from the posterior half of the MVA during systole, which was 3.6 +/- 1.0 mm (mean +/- SD), indicates the annular flexibility. The preservation of annular flexibility may prevent LVOT obstruction. Further geometrical analysis of patients after mitral repair will clarify annular function as presented in this article.

The inflexible mitral annulus after valve prosthesis. Inherent risk of dynamic left ventricular outflow tract obstruction.

Although chordal preserving mitral valve replacement is beneficial to cardiac function, the loss of flexibility of the annulus and consequent translational motion of the valve prosthesis during systole may cause potential left ventricular outflow tract (LVOT) obstruction after surgery. The extent of the flexibility of the mitral valve annulus (MVA) necessary for the prosthetic valve to prevent potential LVOT obstruction was determined. The three dimensional images of the MVA at 0, 100, 200, and 300 msec delay from the electrocardiogram R wave were reconstructed from cine-mode magnetic resonance images in eight normal subjects. In the lateral view of the MVA, the dorsal flexion angle (DFA) was defined. This angle implies the extent of the flexion of the anterior half of the MVA in relation to the posterior half. The data (mean +/- SD) for the DFA were 31.7 +/- 5.4 degrees (0 msec), 36.4 +/- 4.5 degrees (100 msec), 39.0 +/- 3.8 degrees (200 msec), and 43.6 +/- 2.6 degrees (300 msec), whereas the systolic increase in DFA was 11.9 +/- 3.2 degrees. The flexibility observed in normal mitral annuli is relevant to prosthetic mitral valves.