Neurodevelopmental outcome related to cerebral risk factors in children after neonatal arterial switch operation.

BACKGROUND: Neurodevelopmental outcome after neonatal arterial switch operation for complete transposition of the great arteries is an important topic needing prospective assessment. METHODS: A group of 33 unselected children (3.0 to 4.6 years) operated on as neonates with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass and a control group of 32 age-matched healthy children (3.0 to 4.8 years) underwent evaluation of socioeconomic and clinical neurological status and a standardized test comprising all areas of child development. Results of patients were related to those of the control group, to population norms, and to preoperative, perioperative, and postoperative cerebral risk factors. RESULTS: Clinical neurological status was normal in 26 patients (78.8%) and reduced in 7 (21.2%). Complete developmental score and the subscores for motor function, visual perception, learning and memory, cognitive function, language, and socioemotional functions were not different compared to population norms. Compared to the patients, the children of the control group scored higher on tests of complete development, cognition, and language, but also on socioeconomic status. Complete developmental score and the scores for motor, cognitive, and language functions were weakly inversely related to the duration of circulatory arrest, but not to the duration of bypass. Cerebral risk factors such as serum levels of the neuron-specific enolase, perinatal acidosis, perinatal asphyxia, peri- and postoperative cardiocirculatory insufficiency, or clinical seizures were not correlated to the test results. CONCLUSIONS: Neonatal arterial switch operation with combined circulatory arrest and low flow bypass is associated with neurological impairment, but not with reduced development as assessed by formal testing of motor, cognitive, language, and behavioral functions. Perioperative serum level of the neuron-specific enolase is not a valid marker for later developmental impairment.

Mid-term follow-up after multiple system organ failure following cardiac surgery in children.

Multiple system organ failure after cardiac surgery in children is a severe complication with unknown mid- and long-term sequelae. We therefore evaluated 11 children (aged 20-126 mo, median: 67 mo) having survived multiple system organ failure after cardiac operations for congenital cardiac defects in a cross-sectional follow-up study 12-76 mo (median: 32 mo) after surgery. Clinical and laboratory examinations included cardiac, pulmonary, renal, hepatic, neurological and psychological function tests. All patients had adequate cardiac function. Lung mechanics were abnormal in three children and glomerular renal function was abnormal in two patients. Slight elevation of gamma-glutamyl transpeptidase and coagulation factor deficiency was present in six and seven patients, respectively (five of whom had undergone the Fontan operation). Severe neurological sequelae such as diplegia (n = 1) and mental retardation (n = 1) were observed in two patients. In addition, five children presented delayed motor, graphomotor and/or speech development. Two children were found to have abnormal intelligence. We conclude that with the exception of neurological impairment, mid-term sequelae of multiple system organ failure after cardiac surgery in children are mild. However, longer follow-up using an appropriate control group is mandatory.

[Tuberous sclerosis and organic bipolar disorder in a 15-year-old adolescent]. / Tuberöse Sklerose und organische bipolare Störung bei einer 15jährigen Jugendlichen.

We present the case of a 15-year-old girl with tuberous sclerosis who developed rapid cycling bipolar disorder under treatment with the anticonsulvant oxcarbazepine. Because of first occurrence of grand mal-seizures combined treatment with Valproate became necessary, but failed to prevent relapses of bipolar disorder. Mood stabilization was noticed under additional treatment with lithium, which was complicated by an increase of serum potassium.

[Cerebral vein and sinus thrombosis--an important cause of benign intracranial pressure increase in childhood]. / Die Hirnvenen- und Sinusthrombose--eine beachtenswerte Ursache der benignen intrakraniellen Druckerhöhung im Kindesalter.

The syndrome of raised intracranial pressure without any space-occupying lesion (pseudotumor cerebri) is still of unknown etiology. To some degree, secondary causes can be found. With respect to the therapeutical possibilities, a clinically important cause is the thrombosis of a major cerebral sinus. In children it is sometimes associated with otitis media. However, clinical signs of mastoiditis can be missed because of previous antibiotic treatment. We observed 11 children with pseudotumor cerebri. Four children suffered from an occlusion of a lateral major sinus after otitis media. The treatment with heparin in the acute stage and later on with acetylsalicylic-acid led to complete recovery. Our results suggest that the thrombosis of a major cerebral sinus induced by an otitis media is a frequent cause of pseudotumor cerebri in children and should be excluded by MRI or angiography in any doubtful case. In contrast to the treatment of adults, long term anticoagulation is not necessary.

[Neonatal, neurologic and psychosocial findings in higher order multiple births. Follow-up for 3 to 10 years]. / Neonatologische, neurologische und psychosoziale Befunde bei höhergradigen Mehrlingen. Nachuntersuchungen über 3 bis 10 Jahre.

We examined the outcome of 9 triplet, 3 quadruplet, 1 quintuplet and 1 sixtuplet pregnancies delivered between 1979-1989 at the perinatal center of the RWTH Aachen. The course of pregnancy and neonatal period were retrospectively analysed. The follow-up program covered at least 3, up to a maximum of 10 years. 12 families could be interviewed concerning psychosocial effects. The neonatal mortality was 4%. Neonatal morbidity; hyaline membrane disease (n = 18), intraventricular hemorrhage (n = 9), pneumothorax (n = 7), patent ductus arteriosus (n = 7), bronchopulmonary dysplasia (n = 8). At the age of 2 years 63% of the children were considered to be normal on developmental assessment, 17% showed mild, 20% severe developmental delay. With 3 to 10 years 83% were normal, 17% severely handicapped. In total 20% of the children died or showed severe handicap. Higher order multiple pregnancies make great demands on the perinatal medicine and lead in spite of an improved prognosis to a remaining burden for the children and their parents.

[Stroke in the child: etiology, differential diagnosis, relevant diagnostic procedures and therapeutic possibilities]. / Der Schlaganfall beim Kind: Atiologie, Differentialdiagnose, sinnvolle Diagnostik und therapeutische Möglichkeiten.

Strokes in children occur in conjunction with arteriovenous malformations or with occlusive vascular diseases secondary to cardiac disease, intracranial infection, hematological and metabolic disorders. Recently several inborn errors of metabolism have been recognized as possible causes of strokelike events and should be considered in differential diagnosis. In a survey we describe our experience with stroke and strokelike episodes in childhood and discuss the approach selected for its management.

Clinical application of a noninvasive multi-electrode array EMG for the recording of single motor unit activity.

Using a recently developed noninvasive EMG recording technique with multi-electrode arrays we investigated the pattern and distribution of motor unit action potentials (MUAP) following maximal voluntary contraction of the musculus abductor pollicis brevis. An additional parameter, i.e. muscular conduction velocity (CV) in single motor units, was calculated from the multi-electrode array EMG recordings. From 63 healthy children of various age the normal EMG pattern and CV were derived and compared to the EMG of diagnosed patients known to suffer from Duchenne muscular dystrophy and from spinal muscular atrophy. In normal individuals the muscular CV in neonates was lowest at 1-2 m/s and gradually reached a plateau of 2.9-4 m/s from the age of 4 years onwards. The EMG in 31 children with Duchenne muscular dystrophy showed an abnormal pattern with low amplitude action potentials. In 30 out of 31 patients a significantly lower muscular CV was found. In 10 children with spinal muscular atrophy the EMG showed action potentials of abnormally large amplitude and a reduced recruitment of firing motor units. The muscular CV remained within the normal range. Compared to classical needle EMG the application of this new noninvasive EMG technique in children is painless and offers an easy-to-handle diagnostic tool to differentiate between neuromuscular diseases of denervating or of myopathic origin.

[Diagnosis of complications of ventriculo-peritoneal and ventriculo-atrial shunts]. / Diagnostik von Komplikationen ventrikuloperitonealer und ventrikuloatrialer Shunts.

The value of imaging (cranial CT, cranial and abdominal sonography, plain film surveys) was examined retrospectively in 28 shunted children in whom 82 instances of suspected dysfunction arose. There were 23 obstructions, 12 dislocations, 1 disconnection, 6 infections, 3 overdrainages and 5 slit ventricle syndromes. Impaired absorption with ascites, a peritoneal liquor cyst and a seroma occurred in the peritoneal part of the shunt. Progressive dilatation of the ventricle system shown by CCT (89%) or ultrasound was the most sensitive sign of high-pressure hydrocephalus. Periventricular hypodensity and flattening of the gyri (15%) were found less often. An examination strategy in suspected shunt dysfunction is suggested on the basis of these findings.

[Infarct of the basal ganglia in childhood--clinico-neuroradiologic findings and differential diagnosis]. / Stammganglieninfarkte im Kindesalter--klinische-neuroradiologische Befunde und Differentialdiagnose.

Basal ganglionic infarctions during childhood are comparable in size and favourable prognosis with lacunary infarctions in adults. Considering their etiology they are comparable with capsular infarctions in adults. In four children with basal ganglionic infarctions and acute motoric hemiparesis, a quick and complete regression of clinical signs and CT-findings was observed. With regard to the differential diagnosis of basal ganglionic infarction we additionally report of a female patient with initially unilateral lesion who had silently developed the hemiparesis. In this case the clinical course and the neuroradiological follow-up findings suggested Leigh disease.

Hypoxic ischaemic encephalopathy: correlation between ultrasound and computed tomography.

Hypoxic ischaemic encephalopathy (HIE) is a major cause of motor and mental retardation. There ist no doubt that the haemorrhagic form of HIE can be detected by cerebral sonography, but it is of great interest to recognize non-haemorrhagic HIE as well. We demonstrate the follow-up of three patients with different types of non-haemorrhagic HIE: Periventricular leukomalacia in a critical ill premature infant 35 weeks gestational age, multicystic encephalopathy in a term newborn with severe perinatal asphyxia and circumscript ischaemic leukomalacia in a five month old infant with near miss event for sudden infant death. Correlation between ultrasound and computed tomography proved that non-haemorrhagic HIE produces global or circumscript high echogenicity in the first week after the hypoxic event whereas computed tomography shows pathologic hypodensity in the same areas. Cerebral sonography is a very helpful and harmless method for the difficult diagnosis HIE. In the case of periventricular leukomalacia, CT scan gives no further information and can be avoided. Concerning HIE of term newborns and small infants, CT scan remains necessary, to evaluate the extension of cerebral injury.

Orbital venous congestion in childhood. Diagnostic and therapeutical implications.

A case study of 2 patients with venous congestion of the orbit due to different etiologies is presented. Both the children demonstrated orbital pain, proptosis, chemosis and conjunctival injection. In one case early diagnosis left to complete recovery by conservative treatment in a patient with a pyogenic cavernous sinus thrombosis. In the other case a traumatic carotid sinus cavernous fistula could be treated successfully using transvasculary navigated detachable balloons.

[Cortical abnormalities of the brain. Morphologic and clinical findings]. / Rindenfehlbildungen des Gehirns. Morphologische und klinische Befunde.

Ten children at the age of 1-16 with various types of cortical dysplasias are demonstrated by means of computerized tomography. Clinical features of focal subcortical mass of heterotopic gray matter (focal pachygyria) are contralateral partial seizures (n = 5). Pachygyria commonly is associated with severe psychomotor retardation and epilepsy (n = 3). In two cases nodular heterotopias protruding the ventricular walls are accompanied by epilepsy only.

[Endogenous foreign body meningitis due to a spinal epidermoid]. / Endogene Fremdkörpermeningitis bei spinalem Epidermoid.

We present a two year old child with recurrent aseptic meningitis due to a spinal epidermoid in the subdural space. A dermal sinus had been operated earlier in this child. The connection between dermal sinus and spinal epidermoid is demonstrated. Computed tomography (CT) examination should be used as a screening in all patients with congenital dermal sinus with special reference to visualizing anatomical details.

[How effective is the treatment schedule in children with infantile spasms?]. / Wie begründet ist die Stufentherapie bei Kindern mit BNS-Leiden?

A fatal incident during combined anticonvulsive therapy with ACTH and Dexamethason prompted us to reevaluate our treatment results in 19 children suffering from infantile spasms. We followed Doose's recommendation using Clonazepam as a means of first choice. Failing that, than Dexamethason and Primidone was used. A combination of ACTH, Dexamethason and Primidone was administered to patients failing both treatment courses. Our results question the necessity, the tolerance limit and the efficacy of this treatment schedule. Therefore we propose a prospective multicentric study to reinvestigate the therapeutic guideliness for infantile spasms.

Predominant sensory Guillain-Barré syndrome in hereditary sensory neuropathy--case report of a three-year-old boy.

We observed a three-year-old boy with increasing ataxia over a period of two weeks, which cleared up completely within four months. The electroneurographic features and their course in the child and the father allow us to suspect the additional existence of a hereditary sensory neuropathy. The authors put forward the hypothesis that the genetic disturbance of the sensory nerves is responsible for this clinical variant of Guillain-Barré syndrome.

[The value of computer tomographic examination in children with congenital tetra- and diplegia]. / Die Wertigkeit computertomographischer Untersuchungen bei Kindern mit konnatalen Tetra- und Diplegien.

31 children suffering from congenital spastic di- or quadriplegia, born between 1963-1976, were re-examined neurologically and admitted to CT. The mental level was obtained by noting the educational placement of the child. 14 patients showed normal CT patterns. In 17 patients a volume loss of the cerebral white matter with ventricular enlargement was present, but there were no local cortical defects. From similar changes on the ventricles in CT four different patterns can be ascertained. Belonging to one of these CT patterns permits no statement about the neurological and mental defect in an individual case. But massive ventricular enlargements e vacuo go hand in hand with serious neurological and mental defects.

Microprocessor-based long term cardiorespirography. II. Status evaluation in term and premature newborns.

In 1965 URBACH et al. and RUDOLPH et al. [35, 39] described a loss of heart rate variability in severely ill neonates. In this study we investigated the correlation between instantaneous heart rate patterns and status diagnosis. We used a microprocessor-based cardiorespirography system. Seventy five newborn infants (51 prematures and 24 term neonates) were studied for about 12 hours each. Twenty nine patients had a second record after the first investigation. Parameters were: Type of frequency and oscillation, long time variability (LTV), short time variability (STV) and the newly introduced P-value (maximal difference between two successive R-peaks in five minutes). We found clear differences between the study groups. With increasing severity of illness mean values ("group mean values") of long time variability, short time variability and P-value decreased. Fixed heart rate became predominant. The most pronounced loss of heart rate variability was seen in infants with severe intracranial bleeding, thus offering a tentative diagnosis. For statistical analysis long time variability and the silent oscillation type have been proved as best parameters for this diagnosis. Severely decreased heart rate variations also have been seen in infants with acute renal failure--possibly because of brain edema--, after application of muscle relaxants, repeated doses of sedatives, and after prolonged anesthesia. Otherwise, the heart rate variability was probably dependent on age and gestational age in prematures and newborn infants without intracranial bleeding. It is possible to use microprocessor-based long time cardiorespirography as a simple screening method for the diagnosis of neonatal intracerebral bleeding. In future experiences transcutaneous measurements of oxygen tension should be included.

[The problems of the decrease in periventricular density due to hypoxia in computer tomograms of new-born infants]. / Zur Problematik der hypoxiebedingten periventrikulären Dichteminderung im Computertomogramm von Neonaten.

200 premature and mature neonates with clinical evidence of hypoxia or traumatic encephalopathy were examined by cranial computerized tomography (CT) during the first two weeks postnatally. The findings were compared with those in a "control group" consisting of 14 neonates with extraneural malformations. Cerebral hemorrhages were easily identified by their high density, in CT images. Bilateral areas of lowered density within the periventricular white matter, however, could not always be attributed to irreversible tissue damage prior to about 14 days after the hypoxic event in term newborns, and in preterm infants even later. During this early period of life an apparent decrease of periventricular density was regularly observed that must not be mistaken for a pathological change. In two cases persistent periventricular areas of decreased density were caused by neuropathologically verified foci of incomplete leucomalacia.