Fears and anxiety in children with long-QT syndrome compared to children with asthma.

OBJECTIVE: To compare children with asthma to children with long-QT syndrome (LQTS) in terms of anxiety and medical fears. METHOD: Forty children (25 males/15 females) with asthma and their mothers participated, along with seven children with LQTS (four males/three females) and their mothers. RESULTS: Children with asthma had significantly more medical fears, fear of danger/death, and fear of minor injury and small animals compared to children with LQTS. Children with LQTS tended to have more fear of failure and criticism, and tended to keep their feelings to themselves and minimize their real feelings of anxiety. Children with LQTS had significantly more internalizing problems, and their mothers had significantly higher anxiety. CONCLUSION: Fear and uncertainty can be overwhelming in LQTS. Children with LQTS do not seem to be able to share their feelings openly. Examining the psychosocial adjustment of affected children may assist professionals to help families to cope more effectively.

QT intervals in metabolic dilated cardiomyopathy.

OBJECTIVES: The present study determined the prevalence of dilated cardiomyopathy together with prolonged corrected QT (QTc) intervals in children. The study also examined whether an association exists between prolonged QTc intervals and ventricular dysrhythmia in a patient cohort with dilated cardiomyopathy. BACKGROUND: The morbidity and mortality for pediatric patients with dilated cardiomyopathy remains high and is a clinical challenge. The patient population includes a significant number of Hutterite patients with metabolic disease associated with dilated cardiomyopathy. METHODS: Thirty-eight pediatric patients with dilated cardiomyopathy were reviewed for the presence of prolonged QTc and dysrhythmias. Eleven patients had a metabolic etiology for their dilated cardiomyopathy. RESULTS: Thirty-six per cent of the patient cohort had a long QTc interval. After 50 months of follow-up, the probability of survival for a child with a long QTc interval was approximately 50%. The probability of survival for a child with a normal QTc interval was 72%. Seventy per cent of the patients who died had a metabolic etiology for their dilated cardiomyopathy and a long QTc. CONCLUSIONS: Dilated cardiomyopathy may be associated with a prolonged QTc and may increase the patient's risk for sudden death. The presence of a metabolic etiology for dilated cardiomyopathy increases the risk of death.

Electrocardiogram interpretation and management in a pediatric emergency department.

OBJECTIVES: To determine the accuracy of electrocardiogram (ECG) interpretation by pediatric emergency physicians through comparison with a pediatric cardiologist and to determine the intrarater and interrater reliability for pediatric emergency physicians and cardiologists. METHODS: This was a prospective cohort study in which pediatric emergency physicians ordering an ECG completed a standardized questionnaire. The same emergency physician, a second emergency physician, and a pediatric cardiologist also completed the questionnaire for all ECGs at a later time. A randomly selected subset of ECGs was also interpreted by the same cardiologist and a second pediatric cardiologist. Major outcome variables were (1) whether the ECG was normal or abnormal, and if abnormal, (2) whether the abnormality represented a minor or major concern, and (3) whether the ECG warranted referral to a pediatric cardiologist. RESULTS: For pediatric emergency physicians, the intrarater and interrater kappa values were 0.56 and 0.24 for the presence of an abnormality, 0.49 and 0.36 for level of concern, and 0.63 and 0.25 for need of cardiology follow-up. For pediatric cardiologists, the intrarater and interrater kappa values were 0.82 and 0.92 for the presence of an abnormality, 0.71 and 1.00 for level of concern, and 0.82 and 0.91 for need of cardiology follow-up. A comparison of the initial emergency physician and cardiologist interpretations yielded kappa values of 0.42 for the presence of an abnormality, 0.16 for level of concern, and 0.31 for need of cardiology follow-up. CONCLUSIONS: When compared with interpretation by a pediatric cardiologist, ECG interpretation by pediatric emergency physicians was relatively inaccurate; intrarater and interrater agreement among emergency physicians was good and poor, respectively, and the intrarater and interrater agreement among pediatric cardiologists was excellent.

MDA, oxypurines, and nucleosides relate to reperfusion in short-term incomplete cerebral ischemia in the rat.

Short-term incomplete cerebral ischemia (5 min) was induced in the rat by the bilateral clamping of the common carotid arteries. Reperfusion was obtained by removing carotid clamping and was carried out for the following 10 min. Animals were sacrificed either at the end of ischemia or reperfusion. Controls were represented by a group of sham-operated rats. Peripheral venous blood samples were withdrawn from the femoral vein from rats subjected to cerebral reperfusion 5 min before ischemia, at the end of ischemia, and 10 min after reperfusion. Neutralized perchloric acid extracts of brain tissue were analyzed by a highly sensitive high-performance liquid chromatography (HPLC) method for the direct determination of malondialdehyde, oxypurines, nucleosides, nicotinic coenzymes, and high-energy phosphates. In addition, plasma concentrations of malondialdehyde, hypoxanthine, xanthine, inosine, uric acid, and adenosine were determined by the same HPLC technique. Incomplete cerebral ischemia induced the appearance of a significant amount (8.05 nmol/g w.w.; SD = 2.82) of cerebral malondialdehyde (which was undetectable in control animals) and a decrease of ascorbic acid. A further 6.6-fold increase of malondialdehyde (53.30 nmol/g w.w.; SD = 17.77) and a 18.5% decrease of ascorbic acid occurred after 10 min of reperfusion. Plasma malondialdehyde, which was present in minimal amount before ischemia (0.050 mumol/L; SD = 0.015), significantly increased after 5 min of ischemia (0.277 mumol/L; SD = 0.056) and was strikingly augmented after 10 min of reperfusion (0.682 mumol/L; SD = 0.094). A similar trend was observed for xanthine, uric acid, inosine, and adenosine, while hypoxanthine reached its maximal concentration after 5 min of incomplete ischemia, being significantly decreased after reperfusion. From the data obtained, it can be concluded that tissue concentrations of malondialdehyde and ascorbic acid, and plasma levels of malondialdehyde, oxypurines, and nucleosides, reflect both the oxygen radical-mediated tissue injury and the depression of energy metabolism, thus representing early biochemical markers of short-term incomplete brain ischemia and reperfusion in the rat. In particular, these results suggest the possibility of using the variation of malondialdehyde, oxypurines, and nucleosides in peripheral blood as a potential biochemical indicator of reperfusion damage occurring to postischemic tissues.

Ipsilateral motor activation in patients with cerebral gliomas.

OBJECTIVE: The aim of this study is to provide neurophysiologic evidence of ipsilateral hemispheric activation in patients affected by intracerebral gliomas via the use of transcranial magnetic stimulation. BACKGROUND: The mechanisms involved in such ipsilateral activation have yet to be established, but they may involve preexisting routes that either are suppressed or undetected in the normal brain. Ipsilateral pathways may act in reserve, activated by the impairment of contralateral control. This hypothesis is suggested by the fact that the considerable size of the tumors in our patients is not matched by a proportionate loss of motor performance in the limbs contralateral to the affected hemisphere. However, it remains possible that ipsilateral motor-evoked potentials (iMEPs) may reflect reorganizational changes without significant functional effects. METHODS: The effects of such activation were investigated using both focal and nonfocal coils stimulating cortical motor areas, with MEPs recorded from both left and right thenar muscles. Fifteen healthy control subjects and seven patients were examined. RESULTS: iMEPs were generally absent in normal subjects, but in contrast they were obtained in the patients by stimulating the healthy hemisphere using both round and figure-of-eight coils. Distinct from contralateral MEPs, iMEPs are obtained with higher thresholds (range, 60 to 80% of stimulator output) and display longer latencies (20.9 msec versus 19.4 msec). CONCLUSIONS: Taken in conjunction with recent research using functional imaging brain exploration and a variety of clinical, anatomic, and neurophysiologic studies, our results reflect a growing awareness of ipsilateral motor control and its potential compensatory role when contralateral routes are damaged.

Birthday of peripheral nervous system surgery: the contribution of Gabriele Ferrara (1543-1627).

SURGERY OF THE peripheral nerves has only recently achieved brilliant results thanks to technological advances in the development of neurosurgical instrumentation. In past centuries, few surgeons made relevant contributions to this topic and improvement was slow and difficult. Avicenna, Guglielmo da Saliceto, and Guido Lanfranchi reported some attempts to suture nerves directly, but Gabriele Ferrara was the first to give a lucid and succinct description of suturing of the stumps of a transected nerve. He described applying gentle traction on the retracted stumps, suturing using a special needle with an eye, after immersion in a decoction of red wine, rosemary, and roses (alcoholic disinfection?), and, finally, insulating the sutured segment with a mixture of oils. We are celebrating the 400th anniversary of the first edition of his fundamental work (1596-1996), and we truly esteem this surgeon, a pioneer of peripheral nerve surgery wo primarily contributed to its progress.

The contribution of Davide Giordano (1864-1954) to pituitary surgery: the transglabellar-nasal approach.

This report describes the fundamental contribution made by Davide Giordano, proposing the transglabellar surgical approach in a period in which transfacial and transbasal operative approaches to the pituitary gland were considered inadvisable because of their risk. His idea was to gain access through bilateral paranasal and frontal skin incisions, allowing removal of the ethmoid bone and the anterior wall of the sphenoidal cube. With the anterior and inferior aspects of the sella turcica thus exposed, bone is removed and the gland is exposed by incision of the dura mater. The technique proposed by Giordano is undoubtedly a forerunner of the transsphenoidal route to the pituitary gland. The importance of his contribution was confirmed by Cushing, who reported his first use of the approach of Giordano in 1909 in a patient with a pituitary adenoma. The efforts of Giordano clearly inspired surgeons of his era to perform this operation clinically, giving impetus to the further development of neurosurgery.

Successful radical removal of an intracranial meningioma in 1835 by Professor Pecchioli of Siena.

In 1835, Pecchioli, Professor of Surgery and Operating Medicine at the University of Siena, removed a "fungus of the dura mater" (meningioma). The lesion was a large ulcerated cranial outgrowth at the level of the right sinciput, which at operation proved to originate from the dura mater and to be eroding the bone. Surgery was radical. The patient recovered and attended for follow-up review several times in the course of 30 months, always fit and with no sign of recurrence. This procedure was later selected for the competition for the chair of Surgery at the University of Paris in 1840. In 1847, Pecchioli summarized his surgical material spanning 16 years' activity, including neurosurgical operations.

Lipoma of the spinal cord. Case report.

A case of intradural spinal lipoma has been reported. Its pathological and clinical features are compared with those of 129 other cases collected from the literature.

Antonio Pacchioni (1665-1726): early studies of the dura mater.

The clustering of arachnoid villi along the sagittal sinus forms what is known as "Pacchioni granulations." These structures were first described in 1705 by Antonio Pacchioni, an Italian scientist. Pacchioni was born in Reggio Emilia, Italy, in 1665, and there he received his degree in medicine. Later he moved to Rome where he built a successful career dedicated to medical practice, research, and teaching. He became a friend of some of the leading scientists of his age: Lancisi, Malpighi, and Morgagni, among others. He devoted himself to elucidating the structure and function of dura mater, and in his studies often used the new technique of maceration of anatomical specimens in various fluids. Among Pacchioni's written works, the Dissertatio Epistolaris de Glandulis Conglobatis Durae Meningis Humanae (1705) deserves the greatest consideration as it contains the first description of arachnoid granulations. He compared dura to cardiac muscle and attributed to its "glandulae" (glands) the faculty of secreting lymph for lubrication of the sliding movements between meninges and brain during contractions. Three centuries after Pacchioni's death in Rome in 1726, the fine structure of arachnoid villi has not been fully elucidated; moreover, many questions related to mechanisms underlying cerebrospinal fluid absorption remain unanswered.

Evolution of a primary intrasellar germinomatous teratoma into a choriocarcinoma. Case report.

A case of intrasellar teratoma with a germinal structure in a 10-year-old girl is described. A few months after intracranial surgery the tumor differentiated into a choriocarcinoma and finally spread to multiple cerebral, pulmonary, and renal metastases. In the course of choriocarcinomatous evolution, very high urinary levels of luteinizing gonadotropin (HCG) developed, but there was no clinical or anatomical evidence of precocious puberty.

Celebrating the centennial (1894-1994): Leonardo Gigli and his wire saw.

In spite of the recent introduction of craniotomes in neurosurgical practice, the simple but brilliant wire saw invented by Leonardo Gigli still holds an important place in neurosurgical instrumentation. Born in Florence in 1863, Gigli was forced by circumstances to leave Italy soon after getting his medical degree. He first spent 2 years attending the most celebrated obstetrical clinics in Paris and London and then, in 1892, moved to Breslau where he worked with Fritsch and Mikulicz. During this successful and rewarding period, Gigli proposed the lateralized pubiotomy (Gigli's operation) for safe delivery in cases of maternal pelvic deformities and, inspired by the sight of a jagged knife during a country banquet, conceived his wire saw to simplify the procedure. In 1894, at Professor Obalinski's suggestion, he successfully tested a modified saw type with a whalebone guide for the preparation of osteoplastic cranial flaps. In spite of his great popularity and the high esteem in which he was held abroad, Gigli's aims were systematically belittled in Italy, where he never qualified for a university teaching position. He died in 1908, at age 44. Although the once celebrated Gigli's operation has merely historical interest today, the favorable features of his wire saw make it a safe and efficient tool in the hands of twentieth-century neurosurgeons worldwide.

Transmaxillosphenoidal approach to tumors invading the medial compartment of the cavernous sinus.

A transmaxillosphenoidal approach was used to remove sellar tumors invading the cavernous sinus. This procedure, a widening of the standard transsphenoidal approach to the sella turcica, uses the sublabial or transnasal route in which the medial wall of the maxillary sinus is laterally dislocated. This method provides good exposure of the prominences of bone above the carotid artery which lies on the posterolateral wall of the sphenoid sinus. This bone area is the key to opening the cavernous sinus inferomedially and removing lesions within its medial compartment. The inferomedial approach takes an entirely extracerebral route so that tumors invading the cavernous sinus through its medial wall are approached inferomedially following the direction of tumor growth. It also allows direct visualization of the intracavernous carotid artery during tumor removal, thus sparing the cranial nerves, which run on the opposite side. Adequate surgical exposure of a pituitary adenoma is achieved with a custom-made sphenoidal retractor with asymmetric blades, the shorter blade holding aside the thin medial wall of the maxillary sinus. Between October, 1989, and July, 1993, 11 patients with tumors invading the cavernous sinus underwent surgery via this approach; 10 had pituitary adenomas and one had a craniopharyngioma. Eight tumors were treated by primary operation: four tumors were totally and four subtotally (> 80%) removed; one tumor already operated on elsewhere was totally removed; and of two tumors already operated on and irradiated, one was subtotally removed and the other only partially (approximately 40%) removed owing to marked postirradiation scarring. None of the patients suffered permanent cranial nerve deficit and all but one showed marked clinical improvement.

Steroid receptors in the pathogenesis of chronic subdural hematoma.

Chronic subdural hematoma (CSDH), located between the dura mater and the arachnoid and usually characterized by a well-vascularized external capsule (HEM), has a higher incidence in male patients with elevated urinary estrogens than in female patients. In an attempt to increase our understanding of the physiopathogenesis of CSDH, total estrogen receptor (ER) was measured in HEM specimens from four male patients by a sodium thiocyanate exchange assay and cytosol progesterone receptor (PRc) in three specimens by a dextran-coated charcoal adsorption assay. Although no nuclear ER could be detected, ERc and PRc were found in all three specimens examined. The presence of ER in a mesenchymal tissue like HEM could suggest that, in addition to inducing vascular changes, estrogens might act directly on HEM through a receptor-mediated mechanism more pronounced in men than in women, whose vascular network is adapted to high estrogen values.

Hormones and tumours in central nervous system (CNS): steroid receptors in primary spinal cord tumours.

On the basis of the studies reported on steroid receptors in brain tumours, cytoplasmic and nuclear estrogen (ER) and progesterone (PR) receptors have been examined in forty primary spinal cord tumours: fifteen neurinomas, three neurofibromas, nine meningiomas, nine ependymomas, two astrocytomas, one oligodendroglioma and one hemangiopericytoma with the exchange method in the presence of sodium thiocyanate for ER and using the synthetic progestin R5020 for PR. Regardless the type of the tumour, ER have been detected with a higher incidence in male than in female patients (78% versus 59%). PR had the same incidence in male and in female patients. The neurinoma was the oncotype more constantly provided with steroid receptors: nuclear ER, in fact, has been found with an incidence of 75% in male and of 43% in female patients. This oncotype is usually provided in both sexes with PR. Ependymomas is spinal tumour with the highest incidence of cytosol ER both in male and in female patients. On the basis of the above results reported it can be assumed that hormonal factors might be involved in the occurrence as well as in the growth of spinal cord tumours. Therefore it can be hypothesized that hormonal treatment might favourably be used as an adjuvant therapy in some selected patients with receptor positive spinal tumours.

Congenital right ventricular diverticulum in association with disseminated neonatal hemangiomatosis.

The case of a term infant with disseminated neonatal hemangiomatosis and congenital right ventricular diverticulum is reported. Visceral hemangiomas were present in the liver and kidneys and the patient had lower gastrointestinal bleeding that resolved. The right ventricular diverticulum remained clinically silent. The association of right ventricular diverticula and disseminated neonatal hemangiomatosis has not been previously documented in the literature.

The crochetage pattern in electrocardiograms of pediatric atrial septal defect patients.

OBJECTIVES: To test the sensitivity and specificity of the crochetage pattern (a notch near the apex of the R wave in electrocardiographic inferior limb leads) in the pediatric electrocardiogram for detecting patients with a secundum atrial septal defect. PATIENTS AND METHODS: Electrocardiograms from 82 consecutive preoperative pediatric patients with a secundum atrial septal defect confirmed by two-dimensional echocardiography were reviewed for evidence of right ventricular hypertrophy and the crochetage pattern. These electrocardiograms were compared with 244 consecutive preoperative controls consisting of patients with echocardiographically proven patent foramen ovale, ventricular septal defect, pulmonary stenosis, tetralogy of Fallot and patients with normal echocardiogram studies. RESULTS: The electrocardiographic crochetage pattern was observed in 31.7% of preoperative patients with a secundum atrial septal defect in at least one inferior limb lead. The specificity of the crochetage pattern for the detection of a secundum atrial septal defect was high when present in all three inferior limb leads (greater than 92%). The crochetage pattern in at least one lead in secundum atrial septal defects shows no association with incomplete right bundle branch block (c2(1)=0.80, not significant), and thus these two findings together do not improve the detection of an atrial septal defect. CONCLUSIONS: The sensitivity and specificity of the electrocardiographic crochetage patterns in at least one inferior limb lead in echocardiographically proven secundum atrial septal defects are 31.7% and 86.1%, respectively. The electrocardiographic crochetage or notching pattern in inferior limb leads has a high specificity for atrial septal defects in the pediatric population.

Opening Pandora's box: parental anxiety and the assessment of childhood murmurs.

BACKGROUND: Most family physicians and pediatricians recognize the induction of parental anxiety when a newly diagnosed childhood murmur is discussed with a child's parent. OBJECTIVES: To assess parental anxiety before and after consultation with a pediatric cardiologist for assessment of a childhood murmur. The study investigated the relationship between anxiety and parental understanding of murmurs, and assessed the possible role of the primary physician's initial diagnosis of the child's murmur on the anxiety level of parents. SUBJECTS AND METHODS: One hundred ninety-five consecutive parents who presented with their child to the Alberta Children's Hospital Cardiology Clinic, Calgary, Alberta, were recruited. The children were referred for an initial consultation with a pediatric cardiologist for assessment of a heart murmur noted previously by the referring physicians. Before consultation, the patients were divided into three murmur groups for diagnosis: innocent, pathological and unknown. Anxiety questionnaires, as well as questionnaires assessing basic parent understanding of childhood murmurs, were administered to one of the parents pre- and postconsultation. RESULTS: The initial diagnosis given to the caregiver by the primary care physician as the reason for referral did not seem to affect the level of parental anxiety within groups. As expected, postconsultation anxiety levels were significantly higher in parents whose children received a pathological diagnosis from the pediatric cardiologist. A significant drop in parental anxiety post-consultation was seen in all parent groups, including parents of a child diagnosed with a significant cardiac problem or pathology requiring further investigation and/or cardiac follow-up. There was a consistent correlation between higher scores on parental understanding and lower levels of parental anxiety. CONCLUSION: The present study confirms that the finding of a childhood murmur by the family doctor or pediatrician induces significant parental anxiety, even when the presumptive diagnosis is that of an 'innocent' murmur. Pediatric cardiology consultation decreases parental anxiety, even in parents whose child has been given a diagnosis of pathology. The correlation between poor understanding of murmurs and high parental anxiety suggests that further study is required on the role of the referring physician in augmenting parental understanding of the term 'heart murmur' and the referral process.

The vertebral artery: developmental pathology.

The vertebral artery (VA), whose embryogenesis is unique, different from that of any other vessel, is characterised by a great variety of malformations and anomalies. Some of the formers are truly pathological (that is symptomatic); the latter are just either anatomic or angiographic by chance findings. All of them should be kept in mind by the surgeon approaching the deep cervical and cranio-spinal regions, as well as by the interventional radiologist. Width and length anomalies of the VA, tortuosity and kinking, course anomalies, duplication and fenestration, persistence of primitive arteries, anomalies of collateral branches are discussed in the light of a literature review. Other pathologies of the cervical VA (spontaneous aneurysms and arteriovenous fistulae) associated exclusively with genetic diseases as neurofibromatosis type 1 (NF1) and fibro-muscular dysplasia (FMD) are also mentioned.

Extracranial CSF shunting for infantile non tumoral hydrocephalus--a retrospective analysis of 360 cases.

A series of 360 consecutive extracranial CSF shunts performed between June 1960 and June 1976 for infantile non tumoral hydrocephalus is reviewed. The surgical methods used, the reasons for re-operation, the time distribution of post-operative complications, the causes of death and the long term results are analysed and discussed for each of the four groups: tetraventricular communicating hydrocephalus, triventricular hydrocephalus, obstructive hydrocephalus with a ventriculo-cisternal block and post-meningocele or post-myelomeningocele hydrocephalus.