Biomedical subjects
Publications and source records attributed to K H Deeg.
Congenital absence of the penis is a very rare malformation. The incidence is about 1:30 million persons or 1:16,000 necropsies. The cause of the malformation is unknown. Frequent associated malformations of the lungs, the colon and the urinary tract are determinating the prognosis. The therapy of choice in case of isolated penile agenesis is primary orchidectomy, later vaginoplasty and hormonal feminisation. We report our experience with two cases.
15 infants (gestational age 47 +/- 11 weeks; weight 3180 +/- 1082 g) with angiocardiographically proven coarctation of the aorta and 53 healthy infants (gestational age 39 +/- 8 weeks; weight 2830 +/- 1418 g) were investigated by echocardiography and pulsed doppler sonography. In all children pulsed doppler recordings were obtained in the anterior cerebral artery (prestenotic reference vessel) and the truncus coeliacus (poststenotic reference vessel). Nine variables were measured from each doppler signal: The maximal systolic velocity, the endsystolic and enddiastolic velocity, the pulsatility-index, the acceleration slope (peak rate of acceleration), the deceleration slope (peak rate of deceleration), the antegrade flow time and the acceleration and deceleration time. The healthy infants showed a pulsatile flow profile in the anterior cerebral artery and the truncus coeliacus. In normal subjects the maximal systolic velocity and the acceleration slope in the truncus coeliacus were always higher than in the anterior cerebral artery. There was no difference in the endsystolic and enddiastolic velocity, the pulsatility-index, the flow times and the deceleration slope. In children with coarctation of the aorta a decreased and nonpulsatile flow in the truncus coeliacus could be shown. The following variables were significantly lower than in the healthy control group: The maximal systolic velocity, the pulsatility-index, the acceleration and deceleration slope and the antegrade flow time and acceleration time. In the anterior cerebral arteries of children with coarctation the maximal systolic and enddiastolic velocity were significantly higher than in the healthy control group. Pulsed doppler recordings were obtained in 9 patients after surgical correction: The maximal systolic and endsystolic velocity, the pulsatility-index, the acceleration and deceleration slope in the truncus coeliacus increased significantly although these values were lower than in the healthy control group. The flow parameters in the anterior cerebral arteries postoperatively showed no significant difference from the preoperative values. Pulsed dopplersonography of the flow in the anterior cerebral artery and the truncus coeliacus is a good method for diagnosis of coarctation of the aorta and postoperative controls.
A cyanotic newborn is described to illustrate and discuss the rare transient tricuspid insufficiency together with functional pulmonary atresia. Etiologically the manifold causes of perinatal asphyxia are to be taken into consideration. The clinical appearance corresponds to type A of transient myocardial dysfunction according to the classification of Rowe together with respiratory distress syndrome and heart failure. Pathogenetically the disease is based on the different histological consequences of myocardial ischaemia of the right ventricle in connection with a delayed fall of the pulmonary arterial resistance. Fetal connections lead to a pronounced right-to-left-shunt. The diagnosis is primarily established by the way of clinical and echocardiographic investigation and pulsed doppler examination. The most important differential diagnosis are in Ebstein-anomaly, obstruction and atresia of the right ventricular outflow tract. Therapy is conservative. Its aim is to improve myocardial performance and to lower right ventricular afterload. Outcome of patients with neonatal tricuspid insufficiency is uncertain and depends on the severity of myocardial ischaemia. In our patient the course has been uneventful after initial difficulties.
A paralysis of the left vocal cord was seen by laryngoscopy in a 3-days-old boy with inspiratory stridor. A ductus aneurysm was established by angiocardiography, which was believed to be the cause of the vocal cord paralysis. Within 8 days after the angiocardiography the aneurysm became smaller and the stridor disappeared. Twelfth months later the control-angiocardiography showed the total obliteration of the ductal aneurysm.
In 384 healthy prematurely born infants, full-term newborns and older infants (conceptional age: 26 to 60 weeks; weight at investigation: 720 g to 5800 g) the flow in the anterior cerebral arteries was measured by pulsed doppler-sonography. All infants were investigated with a 5 MHz probe (ATL Mark 600 and Acuson 128). The following parameters were measured from the flow profile: The maximal systolic velocity, the end-systolic and end-diastolic velocity as well as the mean flow velocity, the pulsatility-index and the resistance-index. For all parameters the relationship to the conceptional age and weight were analysed. All flow velocities increased with increasing conceptional age: There was an exponential increase of the flow velocities with the conceptional age, whereas the relationship between the weight and the flow velocities was linear. In contrast to this neither the pulsatility-index nor the resistance-index changed with increasing conceptional age and weight. As flow velocities in the anterior cerebral arteries increase with increasing conceptional age and weight both parameters must be taken into consideration when pathologic flow velocities are measured.
The mesoblastic nephroma is the most common renal tumor among newborn and young infants. The tumor is benign, and metastatic growth has only been described in individual cases. Three patients suffering from histologically proven mesoblastic nephroma were examined with an ultrasonic device. Sonographically, the mesoblastic nephroma is manifest as solid and homogeneous tumor of a fine to medium coarse basic texture and of a medium echogenity which may show individual small cystic areas. The tumor cannot be delimited sharply from the healthy renal tissue although uneventful tissue which often caps the kidney is proved. A secure differential diagnosis against a Wilms' tumor is not possible if metastases cannot be detected sonographically or by means of an excretory urogram. The echopattern and the relation of the mesoblastic nephroma to the kidney in combination with an excretory urogram should, however, make the delimitation of a neuroblastoma possible.
We report about a four weeks old male infant who developed jaundice with cholangitis and fever caused by cholelithiasis which could be confirmed by sonographic examination. Familiar history revealed several relatives with gallstones.
33 premature infants (age: 32 +/- 3 weeks; birth weight 1,268 +/- 535 gs) with the clinical signs of patent ductus arteriosus Botalli (PDA) and a control group of 96 healthy infants (age: 37 +/- 4 weeks; birth weight 2 348 +/- 944 gs) were investigated. Pulsed doppler recordings were obtained in the anterior cerebral arteries (ACA) and compared with the flow pattern in the truncus coeliacus (TC). In all children the maximal systolic velocity (Vs), the endsystolic (Ves) and the enddiastolic velocity (Ved) and the pulsatility-index (PI) were measured. The 96 healthy premature born infants showed the following velocities: Vs: 41 +/- 12 cm X sec-1; Ves: 19 +/- 7 cm X sec-1; Ved: 10 +/- 4 cm X sec-1. The pulsatility-index was 0.74 +/- 0.08. In children with PDA all velocities were significantly lower than in the healthy control group: Vs: 31 +/- 10 cm X sec-1; Ves: 7 +/- 6 cm X sec-1; Ved: -1 +/- 5 cm X sec-1. Ved was more decreased than Vs resulting in a significant increase in PI (1.04 +/- 0.14). 22 infants with surgically proven large PDA (age: 31 +/- 3 weeks; birth weight: 1,160 +/- 467 gs) showed significant lower velocities (Vs: 34 +/- 8 cm X sec-1; Ves: 4 +/- 4 cm X sec-1; Ved: -4 +/- 4 cm X sec-1) in comparison with the healthy control group and the 11 children with small PDA (age: 33 +/- 4 weeks; birth weight: 1,494 +/- 621 gs).(ABSTRACT TRUNCATED AT 250 WORDS)
Using the open fontanelle as an acoustic window brain tumours were diagnosed by gray scale ultrasonography in 3 infants aged 1 day to 5 months. The tumours were characterized by their echo dense structure and their good delimination from the surrounding brain. In 2 children the tumour was localized infratentorially (medulloblastoma and unclassified neuroectodermal tumour) and had caused an occlusive hydrocephalus. Both children died aged 3 and 5 months in central nervous dysregulation. One child suffered from plexus papilloma which had caused a hypersecretory hydrocephalus. After resection of the tumour the hydrocephalus decreased without any further treatment. Comparison with axial computed tomography and autopsy findings showed, that gray scale ultrasonography is equally efficient in diagnosing brain tumours and associated hydrocephalus.
In a premature infant bilateral renal venous thrombosis and associated thrombosis of the inferior vena cava were diagnosed at the age of 6 weeks, using a 2-dimensional ultrasound sector scanner with integrated pulsed doppler ultrasound of the renal vessels. In contrast to 30 healthy children, who showed continuous forward flow in the renal arteries throughout systole and diastole, diastolic flow was decreased or even retrograde in renal venous thrombosis (RVT) according to the severity of thrombosis. Doppler recordings in renal veins normally showed continuous retrograde flow patterns, whereas in RVT venous flow was decreased or absent. Pulsed doppler ultrasound is a useful and noninvasive method for recording renal flow patterns, especially in renovascular disease.
Rapunzel syndrome is a rare cause of intestinal trichobezoar. Especially adolescent girls with uncharacteristical abdominal symptoms as well as weight loss and trichophagia are affected. The symptoms are caused by large amounts of swallowed hair within the stomach which can extend far into the intestinuum. Beside conventional radiology and sonography the diagnostic procedure of choice is endoscopy. If endoscopic removal of the bezoar fails surgical intervention is mandatory. As many of these patients show emotional disorders psychiatric evaluation and treatment has to be done to prevent recurrence. We report on a 12 year old girl with a huge trichobezoar which completely filled the stomach and extended far into the small bowel. As the bezoar could not completely be removed by endoscopy the girl had to be treated by surgery.
Most intracranial bleedings in hemophiliacs occur in patients more than 6 months of age. In the neonatal period, this complication is rare and almost always observed in the first week of life. Based on a review of the literature, intracranial hemorrhage is an exceedingly rare occurrence in infants with hemophilia aged 2 weeks-6 months. We report on a male infant with hemophilia A who was referred to our hospital on day 18 because of pallor and jaundice. The neurological examination was normal. A cerebral ultrasound showed a left sided subdural hematoma with a shift of the midline structures to the right. Packed red blood cells (10 ml/kg) and factor VIII replacement (250 IU) were rapidly instituted and a craniotomy with evacuation of the hematoma was performed. The postoperative course was uneventful. On discharge, the neurological examination was considered normal.
Within a six-year period (1988-1993), we diagnosed and treated 143 cases of fetal hydrocephalus. We investigated the relationship between the degree of dilatation of the cerebral ventricles, the thickness of the pallium, and blood flow velocities in cerebral arteries. The assumption that progressive dilatation of the fetal ventricles leads to increasing resistance of the cerebral blood flow and disrupted cerebral perfusion was confirmed. Applying the postnatal pediatric principle of indication for shunt implantation based on Doppler sonographic diagnosis, delivery was instituted immediately in cases with pathologically increased pulsatility indices (PI). In all cases, this indication and the need for immediate shunt implantation were confirmed by postnatal pediatric ultrasonography. Cerebral Doppler velocimetry enables timely identification of fetuses at risk for pressure- or ischemia-induced cerebral tissue damage. The timing of delivery and subsequent neurosurgical therapy can thus be optimized.