Ultrasound-guided puncture of a Dandy-Walker cyst via the lateral and III ventricles.

Since the Dandy-Walker syndrome was first described by Dandy and Blackfan, Taggart and Walker, the many variants of posterior fossa anomalies, the appropriate management of these malformations and the clinical outcome have been the subjects of controversy. Surgery of the posterior fossa with membrane excision was initially the preferred method of treatment. Unfortunately, there was a high rate of complications, and many of the patients treated in this way still needed a shunting system. Ventricular-peritoneal and/or cysto-peritoneal shunting is commonly used to treat symptomatic posterior fossa cysts of Dandy-Walker malformations and hydrocephalus. Cysto-peritoneal shunt implantation only was associated with a high rate of complications, and most patients so treated needed a ventriculo-peritoneal shunt in addition. According to the literature, combined ventriculo-peritoneal and cysto-peritoneal shunting is needed for satisfactory decompression of Dandy-Walker cyst and hydrocephalus in between 16% and 92% of cases. We report on a young patient with a Dandy-Walker malformation who needed drainage of the posterior fossa and a ventricular shunt. We decided to drain the cyst and the supratentorial ventricles via a single, especially prepared, catheter with many perforations. The catheter was inserted under ultrasound guidance. The tube was inserted from the left lateral ventricle through the foramen of Monro into the III ventricle and downwards into the cyst. Intraoperatively, an immediate decrease in the size of the cyst and the supratentorial ventricles was observed. Postoperative MRI confirmed the exact position of the catheter and sufficient drainage of the posterior fossa cyst and the ventricles. Six months later the girl was seen in our outpatient department. Clinical examination showed no neurological deficit, and MRI demonstrated sufficient drainage of the ventricles and the Dandy-Walker malformation, and in addition hypoplasia of the corpus callosum.

[Prenatal diagnosis of hypoplastic left heart syndrome--indications for abortion or transplantation?]. / Pränatale Diagnostik eines hypoplastischen Linksherzsyndroms--Indikation zur Abruptio oder zur Transplantation?

Early diagnosis of fetal cardiac malformations in center of Perinatal Medicine is now possible with the use of modern high frequency ultrasound technics in combination with the 3-step-program of the DEGUM. The diagnosis of most cardiac malformations can early already be obtained in the 2nd trimester as for example the lethal hypoplastic left ventricle syndrome. We want to discuss the problem of prenatal diagnosis of the hypoplastic left heart syndrome and the possibilities of therapeutic procedures.

The problem created by myocardial structure in assessing function.

The clinical understanding of the dynamics of myocardial contraction is hampered by an over-simplified interpretation of the intramural pattern of force generation. This limits the anaesthetist's knowledge of the direct effects of commonly used anaesthetics. A discrimination between a negative inotropic effect and changes in pre- and afterload is usually impossible in clinical conditions. By using needle force probes, it is now possible to measure intramyocardial mesh tension in volumes as small as 3 mm3. Force mapping in 20 to 30 layers of the hearts of five normal dogs has shown that there are different patterns in the basal, middle and apical portions of the free wall of the left ventricle. An inhomogeneity is also observed when myocardial hypertrophy is produced by a 6-week period of aortic banding. However, this decreased the gradient in wall tension between the basal and the other portions of the ventricle. Inhomogeneities in wall tension increase the difficulty of assessing the contractile state of heart muscle. It may, however, be useful to compare local wall dynamics in the more isometrically-contracting basal segment with those in the middle portion which brings about most of the emptying of the ventricle. In the future, transoesophageal echocardiography may be used to measure variations in wall thickness which change the global loading conditions in the basal midwall compartments of the left ventricle.

Local myocardial and global ventricular function compared during positive inotropic medication.

The assessment of the pharmacological efficiency of cardiotonic drugs interferes with the complex force generation within the spatially netted myocardial meshwork. By multifocal local force and distance measurements we distinguished "afterloaded" from "unloading" force types, which essentially differ in their transient behaviour during changes in ventricular shape. Histologically we found the "afterloaded" force type in oblique fibre populations whereas the "unloading" force curve is generated in surface parallel fibres. Amrinon reduces pre- and afterload, thus inducing ventricular shrinkage. The resulting rearrangement of the intramural force pattern modifies the transmission of fibre tension to ventricular ejection in a way that contractility indices, derived from left ventricular pressure, become inapplicable. However, direct intracoronary drug injection, monitored by segmental force and distance measurements within the irrigated myocardial area, characterizes Amrinon as a potent positive inotropic drug.

[Traumatic macroglossia--a rare indication for tracheotomy]. / Die traumatische Makroglossie--eine seltene Indikation zur Tracheotomie.

Hemorrhage into the tongue caused by a trauma is able to produce dangerous hematomas. During an hypertensive crisis a 17 year old patient suffered from an ictus with a bite on his tongue. A macroglossia developed with shortness of breath, so that only a tracheotomy could grand a sufficient ventilation. The macroglossia decreased within 6 days, the nasal breathing was no longer prevented, so the tracheostoma could be closed. An operative intervention is necessary, if conservative therapy is without success referred to diminuation of the tongue.

[Rupture of a coronary artery aneurysm in mucocutaneous lymph node syndrome (Kawasaki syndrome). Demonstration of the coronary aneurysms by 2-dimensional echocardiography]. / Ruptur eines Coronararterien-Aneurysmas bei mukokutanem Lymphknotensyndrom (Kawasaki-Syndrom). Darstellung der Koronaraneurysmen durch 2-dimensionale Echokardiographie.

A further case of mucocutaneous lymphnode syndrome (MCLS) in infancy with multiple aneurysms both of the coronary arteries and systemic arteries is presented. Aged four months the male infant died of rupture of an aneurysm of the right coronary artery causing a hemopercardium. The aneurysms both of the coronary and systemic arteries were diagnosed intra vitam by 2-dimensional echocardiography.

Architecture of the myocardium in computed tomography.

To determine whether current concepts of myocardial organization were in fact the result of artifacts introduced by the dissection technique, a method comprising air injection of the coronary arteries (which gently separated the myocardial fibers) and computerized tomography was devised. Viewing the tomographs cinematographically confirmed the concept that the heart is formed by a double looping of myocardial fiber bands was correct. Examination of abnormal hearts, eg, one with Duchenne muscular dystrophy, shows that pathologic tissue disarrangements could also be identified.

[Tomo- and thermographic structure analysis of the myocardium]. / Tomo- und thermographische Strukturanalyse des Myokardkörpers.

The myocardial body consists of striated muscle fibers which branch densely to form a spiral substructure. Its functional significance is still subject to discussion. Recently, a model of this looping fiber system has been prepared, starting and inserting at the root of the aorta and of the pulmonary artery. Its arrangement fits well with the component lay out of the coronary and the conductive system. Since it is theoretically possible to produce a macroscopic preparation corresponding to any fascicular substructure in densely woven tissue, we used noninvasive means to ascertain whether, during life, the loop structure within the myocardial body is a dynamic arrangement of fiber trajectories, of which postmortem investigations are able to provide only a static impression. Computer tomography and thermography using coronary gas perfusion showed that the heart muscle contains spiral preferential fiber arrangements circling the ventricular cavities.

[Catheter tip force probe: a paraclinical development for per- and postoperative monitoring in heart surgery]. / Katheterspitzenkraftaufnehmer: Eine kliniknahe Entwicklung zur per- und postoperativen Uberwachung in der Herzchirurgie.

Local intramyocardial "one-point" force measurement is well adapted to the myocardial architecture. The dense three-dimensional muscle network does not permit direct measurement of longitudinal tension in a particular bundle of muscle fibers. However, measurements on the skeletal muscle have shown changes in tendon stress to be strictly proportional to changes in local force measured simultaneously at different sites on the gastrocnemial muscle. In the heart in situ the response to inotropic and pre- and afterloads changes is extremely sensitive. However, in comparison to left ventricular pressure, local force measurements at different sites and in different ventricular layers show distinct force gradients and phase dislocation, indicating a wide range of inhomogeneities in ventricular dynamics. The method is suitable for clinical application in cardiac surgery: the probe may be implanted directly on the exposed heart or during ventricular catheterization using a pointed catheter tip force probe.

[Diverticulum of the brachiocephalic trunk--angiographic image and embryologic significance]. / Das Divertikel des Truncus brachiocephalicus--Angiographische Darstellung und embryologische Deutung.

A diverticulum of the brachiocephalic trunk is described in 5 children with unilateral absence of the pulmonary artery, in a child with tetralogy of Fallot, and in an other child with Bland-White-Garland syndrome. The diverticulum is a remnant of a contralateral ductus arteriosus, which cLosed after birth and represents the origin of the distal part of the sixth aortic arch.

Coronary rheothermia: a quasi non-invasive method for controlling bypass patency.

A method based on the principle of thermodilution was developed for a quasi non-invasive permeability control of aortocoronary bypass. An epivascularly-attached thermistor records the cooling of the bypass wall when, following the intravenous injection of 5 to 10 ml of a NaCl solution at 4 degrees C, a bolus of cooled blood passes through the bypass. During cardiosurgical intervention, the thermistor is attached to the venous bridge by one or 2 sutures. The efferent cable goes through the thorax wall and is coupled to a subcutaneously implanted telemetric amplifier unit. The influence of the vessel wall on the perivascular temperature signal, as compared to the intravascular one, was studied in acute and chronic animal experiments. In acute experiments the perivascular peak of temperature was found to be lower than the intravascular one. Continuous measurements over 9 days showed variations in the perivascular signals which must have been due to changes in the thermal capacity of the tissue coupled to the thermistor as well as to changes in resistance caused by a variable extent of scarred area and by the varying water content of the wound bed. These variables will continue to keep rheothermia within the limitations of a method with primarily binary results (= bypass: open or closed). Given stable coupling conditions after full development of the scar around the thermistor, the signal falsification by the then constantly coupled tissue capacity becomes calculable such as to obtain semiquantitative results which, theoretically should vary predominantly with cardiac output.