Glutaric aciduria type I: ultrasonographic demonstration of early signs.

BACKGROUND: Glutaric aciduria type I (GA-I) is a rare inherited metabolic disease with increased excretion of glutaric acid and its metabolites. Diagnosis is often delayed until the onset of irreversible neurological deficits. MATERIAL AND METHODS: We reviewed the clinical and imaging (US, CT and MRI) findings in six patients with proven GA-I and with emphasis on the early US findings. Coronal and sagittal US images of the brain were obtained through the anterior fontanelle in all patients. CT was obtained in three patients and MRI was obtained in two. RESULTS: Macrocephaly was found in all patients, being present in three children at birth or developing rapidly within the first weeks of life. US showed, in all patients, bilateral symmetrical cyst-like dilatation of the sylvian fissures. Progressive fronto-temporal atrophy developed within the first months. CT and MRI demonstrated fronto-temporal atrophy with lack of opercularisation in all cases and basal ganglia or periventricular hypodensities in three patients. CONCLUSIONS: In patients with macrocephaly at birth or rapidly developing within the first weeks of life, US should be performed as the primary imaging modality. Cyst-like bilateral widening of the sylvian fissures is the first sign of GA-I, followed by progressive fronto-temporal and ventricular enlargement. These patients should be screened for GA-I in order to initiate treatment in the asymptomatic stage.

Ureteral fibroma: an unusual clinical symptomatology.

The authors describe a case of ureteral fibroma. The aetiology, clinical symptomatology and diagnosis are discussed, and differentiation between benign and malignant lesions is emphasized.

Computerized tomography of colonic diverticulitis.

In this review article, the value of computerized tomography (CT) vs. contrast enema (CE) in patients with acute diverticulitis is discussed. Acute diverticulitis is a frequent differential diagnosis in abdominal disease. Approximately 15 to 30% of all patients with diverticulosis develop diverticulitis, whereby approximately 21% of these must undergo surgery. The clinical as well as the radiographical differential diagnosis between diverticulosis and diverticulitis often poses a great problem. While the contrast enema demonstrates the changes of the intestinal lumen, the true extent of the inflammatory process cannot be estimated. Numerous studies have shown that CT is preferable in demonstrating the extent of pericolic inflammation, which is often underestimated with the contrast enema. Within the range of computer-tomographical symptoms, infiltration and colonic wall thickening show a high sensitivity. These CT symptoms are not pathognomonic. The differential diagnosis of the findings with CT is discussed.

[Neonatal form of a nonketotic hyperglycinemia in consanguinous parents]. / Neonatale Form einer nicht-ketotischen Hyperglycinämie bei blutsverwandten Eltern.

This is a report of the neonatal form of nonketotic hyperglycinemia with rapid progressing neurological symptoms, respiratory distress and seizures. The EEG pattern is characteristic, the ratio of cerebrospinal fluid to plasma glycine abnormally high. The result of cranial computerized tomography demonstrates also in our patient that nonketotic hyperglycinemia joins a growing list of inborn errors of metabolism associated with brain malformations. The consanguinity of parents supports the known autosomal recessive form of inheritance.

[Ultrasound image of the gallbladder fossa after cholecystectomy in the immediate postoperative period]. / Das sonographische Bild des Gallenblasenbettes nach Cholezystektomie in der unmittelbar postoperativen Periode.

70 patients clinically classified as n.a.d. were sonographically examined for evaluation of the gallbladder fossa within two weeks after cholecystectomy. Four marked signs were noted. In 35 patients (50%) we found homogeneous echogenic formations of triangular, band-like, or oval shape in the bed of the gallbladder. In 25 cases (35.7%) inhomogeneous structures were seen. In 6 cases (8.5%) cystic structures resembling the normal gallbladder were seen which were interpreted as representing seromas or liquified haematomas. In 4 patients (5.7%) the bed of the gallbladder was entirely normal. Whether the gallbladder bed was closed by sutures or not, had no statistically significant effect on ultrasound appearance.

[Idiopathic facial paralysis and magnetic resonance tomography (MRT)]. / Idiopathische Fazialisparese und Magnetresonanztomographie (MRT).

We investigated 15 patients with unilateral facial paralysis using gadolinium(Gd)-DTPA (diethylenetriamine pentaacetic acid) enhanced (magnetic resonance imaging (MRI). Eleven were idiopathic and 1 each was due to basal skill trauma, Lyme's disease, Foville's syndrome and herpes zoster oticus. Ten of 11 Bell's palsies showed a significant enhancement of the facial nerve on the paralysed side. In all cases enhancement was shown in the labyrinthine segment, in 9 in the meatal segment, in 8 in the mastoid segment and in 7 in the tympanic segment also. Follow-up Gd-enhanced MRI investigation was performed 3-11 months later in 8 patients. In 1 case with incomplete return of function after 3 months MRI enhancement was decreased. We could not find any correlation between the intensity of the Gd enhancement and the course, severity or outcome of Bell's palsy, or stapedius reflex audiometry. The mechanisms and aetiology of the Gd enhancement in Bell's palsy seem to be non-specific phenomena which are also found in post-traumatic facial lesions, for instance. Nevertheless, the ability to image the facial nerve in Bell's palsy provides a new means of examination in this disorder. In our opinion Gd-enhanced MRI is recommended in cases of recurrent or "atypical" Bell's palsy and in cases with total loss of electrical excitability, to exclude tumours. It is further suggested that MRI may provide valuable information concerning areas which may require surgical exploration or decompression.

[MRT of bladder carcinoma: tumor staging and gadolinium contrast behavior]. / MRT des Harnblasenkarzinoms: Tumorstaging und Gadoliniumkontrastverhalten.

33 patients with tumours of the urinary bladder were studied via MR, both with and without the paramagnetic contrast medium Gd-DTPA. Results were compared with the final pathological classification after TUER and bimanual palpation. The signal intensity ratio of tumour tissue/fat and tumour/muscle were calculated on T1 weighted images and after GD-DTPA and examined for their statistical significance. The increase in signal intensity of the tumours was statistically significant (Wilcoxon test p less than 0.01). There was no advantage for the T2 weighted images compared with Gd-contrasted enhanced T1 weighted images. MRI staging was correct in 28 out of 33 cases (accuracy 84.8%). Because of the relatively short acquisition time of T1 weighted images and the specific tumour enhancement, administration of Gd-DTPA proved to be of help in the staging of the carcinoma of the bladder. Two small tumours and regressive changes in central areas of tumours were recognised after Gd-DTPA.

[The significance of computed tomography and ultrasound in the diagnosis of puerperal ovarian vein thrombophlebitis]. / Die Bedeutung von Computertomographie und Ultraschall in der Diagnostik der puerperalen Ovarialvenenthrombophlebitis (POVT).

The diagnosis of puerperal ovarian venous thrombophlebitis (POVT) is problematic, not only because of the low and variable incidence (1:600-6000 births), but also because there are no characteristic clinical symptoms. So far, therefore in 80% of cases it has been diagnosed by exploratory laparatomy. Early non-invasive diagnostic procedures must be aimed at, because surgical intervention involves a high rate of complications and uncomplicated cases can be treated by conservative methods (broad-spectrum antibiotics and anti-coagulants). Several case reports refer to the possibility of recognizing POVT by real-time ultrasound (US) and computer tomography (CT). Two cases are described to illustrate this option for preoperative diagnosis by CT and US. A tubular, annular structure with ring enhancement after application of dye along the anatomical course of the ovarian vein seems to be characteristic for POVT.

[Differential diagnosis of the "fat leg"--a case report]. / Zur Differentialdiagnose des "Dicken Beins"--ein Fallbericht.

This is a report about a patient, who came to our hospital because of a painful thickening of her left calf. She was treated with Heparin 48,000 IU/24 hours. A crural vein thrombosis was suspected. Instead of improvement of the symptoms the diameter of the leg grew until the state of a complete Compartment-syndrome. After exact inspection (Sonography, Computertomogramm) we found a hematom of the calf, caused by a rupture of the tibial part of the gastrocnemius muscle. Fasciotomies and evacuation of the blood brought a restitutio ad integrum. Diagnostic methods and faults are discussed.

Bilateral seminal vesicle abscesses.

We report a case of a seminal vesicle abscess successfully treated by percutaneous perineal drainage. Magnetic resonance imaging was helpful in clarifying the differential diagnosis. Seminal vesicle abscess is a rare pathological entity; since 1958, only 7 cases have been reported in the literature. Because of its nonspecific clinical symptoms, clinical diagnosis is often ambiguous. An overview of the literature, as well as a discussion of the diagnosis and the treatment of the seminal vesicle abscess will be presented.

[Ultrasound, computed tomography and magnetic resonance tomography in a child with macrocephaly and glutaric aciduria type I]. / Ultraschall, Computertomographie und Magnetresonanztomographie bei einem Kind mit Makrozephalie und Glutarazidurie Typ I.

The rare case of glutaric aciduria type I (GA Type I) is described. Its characteristics are discussed and compared with cases in the literature. This disease is basically due to a lack of glutaryl-CoA-dehydrogenase with increased excretion of glutaric acid. Most authors describe frontotemporal cerebral atrophy. In the majority of cases macrocephaly is also present. This sign was also seen in our case and was the reason for performing an ultrasound examination, CT and MR. Ultrasound and CT showed a large insular cistern with incomplete formation of the opercula and frontal atrophy. In addition MR revealed hyperintensity of the basal ganglia and the periventricular white matter. To our knowledge this is the first publication of radiological findings in GA Type I in the German language.

[Can ultrasound detect skeletal metastases?]. / Gelingt sonographisch der Nachweis von Skelettmetastasen?

78 radiologically certified skeletal metastases--osteolytic as well as osteoplastic--were examined via sonography. A defect of the cortex could be seen in 82%, a periostal reaction in 28.2% and a concomitant soft tissue tumorous formation in 79.4%. In proving a soft part component sonography is superior to the conventional x-ray. Our study shows that osseous metastases can be verified by sonography.

[Computed tomography of colonic diverticulitis]. / Computertomographie der Kolondivertikulitis.

33 patients with clinically suspected diverticulitis of the colon were studied prospectively by CT. The predictive value of symptoms, such as thickening of the colonic wall (86.6%), inflammatory changes of the pericolic fatty tissue (87.5%), the presence of diverticula (73.3%) and abscess formation (100%), were examined separately and their significance was evaluated. Our study was performed mainly on clinically less severe cases of diverticulitis. True positive results by CT were reached in 20/21 cases (sensitivity = 95.2%), true negative findings in 9/12 (specificity = 75.0%). The results of CT examinations were compared with those of contrast enemas (n = 24) and/or endoscopy (n = 6). The number of cases was too low to achieve statistic significance; the relatively high percentage of questionably positive results shows the difficulties inherent in these methods. Our study shows that CT is a good means to demonstrate even less severe forms of colonic diverticulitis with sufficient reliability.

[Size relations of the spleen and lymph nodes in normal and pathologic processes]. / Grössenverhältnisse von Milzen und Lymphknoten bei normalen und krankhaften Zuständen.

63 spleens and 79 lymph node specimens, both normal and diseased, were measured in three planes. The ratio of length to width and to thickness was established to determine if characteristic changes in proportion - in connection with the expansion of the respective organs - are evident. The healthy spleens did not differ markedly from the diseased ones, whereas striking differences were obvious among the diseased lymph nodes. Lymph nodes involved in malignant disease appeared globular, whereas those involved in inflammatory disease showed a longish and flattened shape.

[What does ultrasound study contribute in acute appendicitis (using dosed compression)?]. / Was bringt die Ultraschalluntersuchung der akuten Appendizitis (bei dosierter Kompression)?

The value of ultrasound in diagnosing acute appendicitis has been examined in a prospective study of 103 patients. Only those patients with an uncertain diagnosis of acute appendicitis were included in the study. We used a 5 or 7.5 MHz linear array transducer applying graded compression. An acutely inflammatory appendix appeared as a tubular structure with a diameter of 3 to 12 mm. The sensitivity of the examination in our material was 82%, the specificity 97%. The main reason for the relatively low sensitivity were cases with a retrocaecal position of the appendix. Ultrasound can be recommended as a highly specific but less sensitive imaging method for the diagnosis of acute appendicitis in clinically equivocal cases.