Echocardiographic differential diagnosis of the cyanotic newborn.

Cyanosis in newborns can be caused by cyanotic heart defects, such as transposition of the great arteries, tetralogy of Fallot, pulmonary and tricuspid atresia, hypoplastic left heart syndrome, common arterial trunk, Ebstein's anomaly of the tricuspid valve, and total anomalous pulmonary venous return. The indicated cyanotic heart defects can be diagnosed or ruled out with three simple echocardiographic views: The parasternal long and short axis view and the apical or subcostal 4-chamber view. If these three views are normal, a cyanotic heart defect can be ruled out. In the case of a cyanotic heart defect, one or more views are pathological.

Sonographic and Doppler Sonographic Diagnosis of Posthemorrhagic Hydrocephalus.

Severe intracranial hemorrhages occur especially in very immature premature infants born with a gestational age under 28 weeks of gestation and a birth weight below 1000 g. Severe hemorrhages are often complicated by a post-hemorrhagic hydrocephalus (PHH). PHH can be caused by a blockage of the cerebro-spinal fluid pathways or by obliterative arachnoiditis of the posterior cranial fossa. Cerebral sonography can differentiate between both entities. In cases of obstruction of the cerebro-spinal fluid circulation the parts of the ventricular system infront of the obstruction are dilated. Color coded Doppler sonography can display the patency or obstruction of the physiologic constrictions of the ventricular system. Increased intracranial pressure can noninvasively be detected by spectral Doppler: The increase of the peak systolic flow velocity in the intracranial section of the internal carotid artery in comparison with the extra-cranial part is an early indication of a raised intracranial pressure. The decrease of the end-diastolic flow velocity during fontanel compression is indicative of abolished cranial compliance and increased intracranial pressure. In the case of raised intracranial pressure diastolic amplitudes and end-diastolic flow velocities are decreased and the resistive-indices are increased.

[Sonographic screening of basilar arteries reduces the risk of sudden infant death]. / Sonographisches Screening der Hirnbasisarterien zur Risikominimierung des plötzlichen Säuglingstodes.

Sudden infant death syndrome (SIDS) is the most frequent cause of death in the first year of life. The causes of SIDS remain unclear although multiple theories have been published in recent decades. However, some important risk factors associated with SIDS, such as prone sleeping have been validated. Over 85% of all SIDS victims were found in a prone position but it is unclear why the prone sleeping position is more dangerous than the supine sleeping position. A possible cause of SIDS is hypoperfusion of the brain stem during head rotation. Some infants show compression of the vertebral arteries at the craniocervical junction during head rotation, especially in the prone position and this may lead to a subsequent decrease of brain stem perfusion. If compression lasts for a longer time hypoperfusion of the brainstem and central apnea and bradycardia result, which can lead to SIDS. The decrease in brainstem perfusion occurs more often and is more pronounced in the prone position as the head is more rotated in the prone than in the supine position. Doppler sonographic flow measurements of the flow in the basilar artery through the open fontanel, allow the detection of patients at risk of position-dependent hypoperfusion of the brain. Flow measurements are obtained in a neutral position (head in midline) and during head rotation. In the vast majority of infants (98.7%) the flow in the basilar artery is independent of head rotation and body position. In rare cases (1.3%) flow velocities drop to below 50% of the initial value during head rotation. A pathological biphasic or even retrograde flow can be found during head rotation in only 0.3% of infants and these infants may have an increased risk for SIDS. To prevent SIDS head rotation which leads to an abnormal or pathological flow decrease during head rotation should be avoided. Additionally these infants should be monitored until blood flow in the basilar artery has returned to normal, which usually occurs during the first year of life. This approach reduced the incidence of SIDS in our patients from 1% to 0.04‰.

Doppler sonographic screening of the flow in the basilar artery during head rotation reduces the risk for sudden infant death.

PURPOSE: Position-dependent hypoperfusion of the brain stem may be a risk factor of sudden infant death. MATERIALS AND METHODS: From 1998 to 2009 we performed Doppler sonographic flow measurements in the basilar artery of 18 194 newborns, 9322 boys and 8872 girls, in five different positions: the neutral position with the head in the midline and during head rotation to the left and right in a supine or prone position. The peak systolic and the time average flow velocity were measured from the flow profile. The flow velocities during head rotation were converted to % of the flow in the neutral position. A decrease in the velocities during head rotation below 50 % was thought to be abnormal. Biphasic flow, flow oscillating around the zero line or retrograde flow during rotation was considered to be pathological. Head rotations, which had caused abnormal and pathological flow, were avoided. The incidence of SIDS in our study group was evaluated and compared with the incidence in a control group of 3 519 newborns. RESULTS: In 17 929 newborns (98.54 %) the blood flow in the basilar artery was independent of head rotation and body position. In 204 newborns (1.12 %) we found an abnormal decrease under 50 %. Pathological flow alterations could be found in 61 patients (0.33 %). The overall incidence rate of SIDS in the study group was 0.055 ‰ (1:18 194). The incidence rate of SIDS in the control group was 1.14 ‰ (4:3519). The comparison of both groups showed a statistically significant (p < 0.0030) lower incidence rate in the study group. CONCLUSION: Hypoperfusion of the brain stem may be a significant risk factor of SIDS.

[Diagnosis of fibromatosis colli via color-coded duplex sonography in 13 infants]. / Diagnose der Fibromatosis colli mit der farbkodierten Duplexsonografie bei 13 Säuglingen.

AIM: Fibromatosis colli is the most common cause of congenital torticollis. Patients show a solid tumor in the region of the sternocleidomastoid muscle which has to be differentiated from other congenital space-occupying lesions in the cervical region. PATIENTS AND METHOD: 13 infants (7 boys, 6 girls) with a mean age of 28 +/- 21 days with fibromatosis colli were examined with a high-resolution linear transducer via color-coded duplex sonography. The location of the tumor within the sternocleidomastoid muscle, delineation from surrounding structures, the echogenicity, and the vascularization were estimated. 3 tumors were surgically removed, and the other 10 infants were treated conservatively with physiotherapy. RESULTS: The tumors were located on the right side in 10 patients and on the left side in 3 infants. In 10 patients the tumor was located in the caudal part, in 3 in the middle part of muscle. All tumors showed a good delineation from the surrounding structures. The volume of the tumor was 5.4 +/- 2.7 ml. 8 tumors showed inhomogeneous echogenicity, and 5 showed homogenous echogenicity. 9 tumors had echopoor, 3 echogenic, 1 isoechogenic internal echoes compared to the contralateral healthy muscle. 13 tumors showed increased perfusion (10 diffusely, 3 focally) via color-coded Doppler sonography. CONCLUSION: Diagnosis of fibromatosis colli can be affirmed sonographically. The tumors are commonly located in the distal 2 / 3 of the sternocleidomastoid muscle and show good delineation from surrounding structures. Typically an inhomogeneous, echopoor tumor with increased perfusion can be shown. Additional diagnostic imaging modalities are usually not necessary. The treatment of choice is physiotherapy. In special cases with a lack of improvement, surgery is necessary.

Sonographic diagnosis of an unusual case of multilocular cystic nephroma mimicking polycystic kidney disease.

The following is a report of the unusual case of a multilocular cystic nephroma in an 8-year-old boy who was transferred to our unit with a palpable abdominal tumor. The patient suffered from thoracic pain and night sweating. The laboratory values were normal. Abdominal sonography showed a huge kidney tumor on the right side consisting of numerous small cysts transversed by irregular septa of variable thickness. The cysts had a diameter of 1 -5 mm; larger cysts of more than 1 cm in diameter were not able to be shown. In the center of the tumor a normal renal parenchyma was able to be shown. The tumor arose like a mushroom from the kidney. Color Doppler sonography showed good vascularity of the normal renal parenchyma while the tumor had only a few internal vessels. The tumor was surgically removed. The histologic diagnosis was cystic nephroma. Unusual features of this tumor were the small size of the numerous cysts similar to polycystic kidney disease and the mushroom-like growth of the tumor.

Spinal sonography in infants with cutaneous birth markers in the lumbo-sacral region--an important sign of occult spinal dysrhaphism and tethered cord.

AIM: Cutaneous markers in the lumbo-sacral region are indicators of occult spinal dysrhaphism and tethered cord. By means of spinal sonography, anatomical abnormalities of the spinal cord can be shown in the neonatal period. PATIENTS: We report on 6 infants with lumbo-sacral cutaneous abnormalities who were investigated with a high resolution linear transducer (> 7.5 MHz) and a computer sonographic unit (Sequoia, Acuson). The investigations were performed between the first day of life and the ninth week (m: 26 days). The following cutaneous markers could be found: Asymmetrical gluteal crease (4); dermal sinus (2), hairy tuft (1); pigmented naevus (1); cutaneous appendage (1); haemangioma (1); unilateral peroneal paralysis with hypotrophic correspondic leg (1). RESULTS: Sonographic evaluation showed the following abnormalities: Tethered cord (6); diastematomyelia (2); tight filum terminale (2); spinal lipoma (3); lipomyelomeningocele (2), myelocystocele and hydromyelia (1). In all infants, sonographic diagnosis could be confirmed by MR imaging and intraoperatively. Surgical correction was performed at the age of 2 to 12 months (m: 7.7 months). CONCLUSION: All infants with cutaneous markers in the lumbo-sacral region should be investigated by spinal sonography as long as the vertebral arches are not completely ossified. Sonography of the spinal cord may detect occult spinal dysrhaphism and tethered cord and prevent neurological damage by early surgical correction at the end of the first year of life.

Spinal sonography in newborns and infants - part II: spinal dysraphism and tethered cord.

UNLABELLED: Patients with cutaneous markers in the lumbo-sacral region as well as infants with bladder and bowel dysfunction, orthopedic anomalies and progressive neurological dysfunction are at risk for spinal dysraphism and tethered cord. Three types of spinal dysraphism can be distinguished: Type I - open spinal dysraphisms with a non-skin covered back mass; type II - closed spinal dysraphisms with a skin covered back mass; type III - occult spinal dysraphisms without a back mass. All spinal dysraphisms can be associated with a tethered cord, characterized by a low position of the conus medullaris below L3. Type I dysraphisms are meningomyeloceles and myeloceles, which are associated with CHIARI-II malformations characterized by the low position of the cerebellar vermis within the foramen magnum. Type II dysraphisms are lipomyeloceles, lipomyelomeningoceles, posterior meningoceles and myelocystoceles. Lipomeningoceles and lipomyelomeningoceles are characterized by a subcutaneous echogenic mass which communicates with the spinal canal and may cause tethered cord. Posterior meningoceles are, dorsal cystic space occupying lesions without internal neural tissue. Myelocystoceles are characterized by a cystic dorsal mass which communicates with a dilated central canal characteristic of syringo-hydromyelia. Type III dysraphisms without a back mass are frequently associated with cutaneous markers in the lumbo-sacral region. Sonographically dermal sinus tracts, diastematomyelia, tight filum and lipoma of the filum terminale and the caudal regression syndrome have to be distinguished. Dermal sinuses are characterized by an echogenic tract from the skin to the spinal canal, often associated with a spinal dermoid. Diastematomyelia is characterized by a complete or partial duplication of the spinal cord which can only be shown on axial images. Tight filum terminale or lipoma of the filum terminale is characterized by a thick echogenic filum with a diameter of more than 2 mm, and a conus below L3. CONCLUSION: All different forms of spinal dysraphisms and tethered cord can be diagnosed sonographically in the neonatal period as long as the spinal arches are not completely ossified.

Spinal sonography in newborns and infants--Part I: method, normal anatomy and indications.

Spinal sonography can be performed in newborns and young infants as long as the vertebral arches are not completely ossified. With high resolution linear transducers (>10 MHz), excellent detailed images of the spine may be obtained from the base of the skull to the caudal end of the thecal sac. Sagittal and axial sections are performed routinely. Beside the spinal cord, the dorsal and ventral nerve roots and the cauda equina can be shown. The medullary conus normally ends above the level of L2/L3. Lower positions are suspective of tethered cord. M-mode sonographic examinations reveal oscillations of the cord due to respiration and the pulse cycle. Colour Doppler sonography displays the epidural venous plexus as well as the central branches of the anterior spinal artery. Normal variants are transient widening of the central canal, terminal ventricle and asymmetric nerve roots. Indications for spinal sonography are midline cutaneous markers in the lumbosacral region, subcutaneous masses, foot abnormalities, anorectal and genitourinary malformations and neurological abnormalities of the lower extremities. All these clinical symptoms are suspicious of spina bifida occulta and tethered cord which should be ruled out by spinal sonography.

The different features of congenital neuroblastomas: report about the sonographic diagnosis of three cases.

We report on three infants with congenital neuroblastoma. Two clinically asymptomatic infants showed a suprarenal right-sided mass, one infant had a mediastinal tumour with tracheal compression causing immediate postnatal dyspnoea. Diagnosis was established by ultrasonography showing hypoechoic tumours without significant calcifications. The two suprarenal masses were characterised by small cystic areas. Colour coded Doppler sonography revealed perfusion of the tumours distinguishing the suprarenal masses from adrenal haemorrhage. The right-sided mediastinal neuroblastoma showed infiltration of the intervertebral foramina and spinal canal without spinal cord compression. All tumours were surgically removed with favourable clinical outcome.

[Trans-fontanellar Doppler sonography of the intracranial veins in infants. Part II--pathology]. / Transfontanelläre Doppler-Sonographie der Hirnvenen im Säuglingsalter Teil II--Pathologische Befunde.

Colour coded Doppler sonography can be very helpful for the diagnosis of haemorrhagic infarction of the brain parenchyma, cerebral venous thrombosis and arterio-venous malformations of the brain. Intracranial haemorrhages into the brain parenchyma are caused by the compression of the subependymal and terminal veins which impede the venous drainage from the white matter. Haemorrhage of the basal ganglia is highly suspicious of cerebral venous thrombosis especially of the deep venous drainage. Unilateral occlusion of only one internal cerebral vein causes unilateral haemorrhage, bilateral thrombosis of both internal cerebral veins as well as occlusion of the great vein of Galens or the straight sinus causes bilateral haemorrhage of the basal ganglia as well as ventricular haemorrhage and haemorrhagic infarction of the white matter. The most common arterio-venous malformation of the brain is AV-malformation of Galens's vein. Sonographically, a pulsating cystic structure behind the 3rd ventricle can be shown. Colour coded Doppler sonography demonstrates the vascular nature of the cyst. Doppler sonography can show the feeding arteries, most frequently the posterior choroidal arteries and the venous drainage by the straight sinus.

[Carotid artery dissection with cerebral infarction after soft tissue trauma in the pharyngeal region]. / Karotisdissektion mit Mediainfarkt nach Weichteiltrauma im Bereich des Pharynx.

We report the case of an 8-year-old boy who suffered an internal carotid artery dissection as a result of an enoral soft tissue trauma. After an initial interval with minor symptoms he developed a cerebral infarction in the vascular region of the left middle cerebral artery. The patient was treated with decompression hemicraniectomy while therapy was monitored with intracranial pressure (ICP) measurements.

[Trans-fontanellar Doppler sonography of the intracranial veins in infants--part I--Normal values]. / Transfontanelläre Doppler-Sonographie der Hirnvenen im Säuglingsalter--Teil I--Normalbefunde.

Doppler sonographic flow measurements in the intracranial veins of infants are not often applied. For the understanding of intracranial pathology such as vascular malformations, intracranial hemorrhagic infarction and venous thrombosis, a thorough understanding of the anatomical position and of the normal flow profiles and flow velocities in intracranial veins is essential. We describe the normal anatomic course of the cerebral veins and their flow profiles and flow velocities in infants. In sagittal sections, the following veins can be shown by colour coded Doppler sonography and measured by pulsed Doppler sonography: The superior and inferior sagittal sinus, and the straight sinus as well as the internal cerebral vein, the vein of Galen and the thalamostriatic veins. In coronal sections, the medullary and subependymal veins, the terminal veins, the internal cerebral veins, the great vein of Galen, the superior sagittal, straight and transverse sinus can be demonstrated and measured.