Giant left atrial appendage aneurysm in a neonate.

Giant aneurysm of the left atrial appendage (LAA) is a rare condition typically presenting in adulthood. This case report describes the investigations and surgical management of a giant LAA aneurysm in a neonate, emphasizing the role of echocardiography and cardiovascular magnetic resonance imaging in preoperative evaluation as well as challenges in surgical repair of this defect.

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.

Surgical outcome following treatment of isolated subaortic obstruction.

Surgical and nonsurgical patients with isolated subaortic stenosis (SAS) were compared to determine the important factors contributing to the timing of surgical intervention. This study reviews 49 consecutive patients (27 surgical and 22 nonsurgical) aged 1.8 to 15.9 years with isolated SAS. The preoperative peak left ventricular outflow tract (LVOT) gradient in surgical patients was significantly higher than the gradient in nonsurgical patients (59.0 +/- 30.4 vs 22.77 +/- 13.9 mm Hg, P = .0001). The progression in LVOT gradient analyzed by echo Doppler was significantly higher in the surgical group compared with the nonsurgical group (10.48 +/- 9.7 vs 1.56 +/- 6.5 mm Hg/y, P = .007). Repeat surgical intervention was required in 22% of patients in the surgical group for recurrence of SAS, and 4% needed a third surgery. The progression in the severity of aortic regurgitation (AR) was not significantly different in the surgical and nonsurgical groups. There was a significant association between the development of AR and patients undergoing surgery (P = .045). AR may not be a reliable indication for early operative intervention in isolated SAS as there was no significant difference in its progression with surgical and nonsurgical patients. Asymptomatic patients with isolated SAS may warrant surgical intervention on the basis of progression of LVOT gradient, rather than the development or progression of AR.

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.

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.

Covert fears and anxiety in asthma and congenital heart disease.

AIM: To compare anxiety, fears and behavioural problems in children with asthma and children with congenital heart disease, and with the normative population. To also review the influence of maternal anxiety, time since diagnosis and severity of disease. DESIGN: Children administered Fear Survey Scale (FSSC-R) and Child Manifest Anxiety Scale (R-CMAS). Mothers given Child Behaviour Checklist (CBCL) and State Trait Anxiety Scale (STAI-S and STAI-T). Normative means and SDs compared with means and SDs for both medical groups. The mother's scores on the STAI-S and STAI-T scales were correlated with the child's scores on the FSSC-R and the R-CMAS. SETTING: Outpatient Asthma and Cardiology multidisciplinary Clinics at a tertiary care paediatric facility, Alberta Children's Hospital. SUBJECTS: Forty children with asthma (aged 6--17 years) were compared with 39 children with congenital heart disease. Intake questionnaires and interviews determined these children to be without obvious psycho-social problems. RESULTS: Children with asthma and children with congenital heart disease had more medical fears, and more physiological anxiety than normative samples. Increased maternal anxiety was correlated in both groups with increased child anxiety, medical fears and behavioural problems in the child. Similarly, increased severity of asthma or cardiac problems was associated with more physiological anxiety and more fears. Less time since diagnosis of the disease adversely affected social interactions in both groups of children. CONCLUSION: Physiological anxiety, medical fears and maternal anxiety are important issues requiring attention in asthma and cardiac disease, even in the absence of obvious psychosocial problems. There may be specific problems with a recent diagnosis of a chronic illness.

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.

Determination of boronophenylalanine in biological samples using precolumn o-phthalaldehyde derivatization and reversed-phase high-performance liquid chromatography.

A reversed-phase high-performance liquid chromatographic method for the detection of boronophenylalanine is described. Determination was obtained by precolumn reaction of o-phthalaldehyde with a mixture of standard amino acids containing boronophenylalanine and separating the corresponding o-phthalaldehyde derivatives, using a Kromasil C-18, 250 x 4.6 mm, 5-microm particle size column, a step gradient with two buffers, a flow rate of 1.2 ml/min, a column temperature of 23 degrees C, and fluorimetric detection (excitation and emission wavelengths of 330 and 430 nm, respectively). The use of such a method for assaying boronophenylalanine in biological samples was tested in neutralized perchloric acid blood and cerebral tissue extracts of rats treated with intracarotid administration of 300 mg/kg of body weight boronophenylalanine. Results of these experiments showed that the present HPLC method represents a valid alternative to currently available analytical techniques for assaying boronophenylalanine based on boron determination in terms of reproducibility, recovery, or sensitivity. Therefore, it is suggested that the present method may routinely be used in all preclinical and clinical studies in which quantification of circulating and tissue concentrations of boronophenylalanine is critical for the application of boron neutron capture therapy.

290 surgical procedures for ulnar nerve entrapment at the elbow: physiopathology, clinical experience and results.

Ulnar nerve entrapment at the elbow is an important and relatively frequent pathological condition that may be related to different causes depending on individual or external factors. The cause of the nerve lesion is also idiopathic in about one-quarter to one-third of cases. This variable aetiopathogenetic presentation has often suggested different diagnostic and clinical approaches and, moreover, various surgical procedures. We present our 8-years surgical experience with 290 cases of ulnar nerve entrapment at the elbow analysing the salient clinical features and the results of the surgical treatment in the light of the relevant literature available on this topic.

Maldevelopmental pathology of the vertebral artery in infancy and childhood.

The vertebral artery (VA), whose embryogenesis differs from that of any other vessel, is characterized by a great variety of malformations and anomalies. Some of the malformations are truly pathological (that is symptomatic); the anomalies are either found by chance postmorten or by angiography. All of these should be kept in mind by the surgeon approaching the deep cervical and craniospinal regions as well as by the interventional radiologist. In the context of a literature review we discuss the width and length anomalies of the VA, tortuosity and kinking, course anomalies, duplication and fenestration, persistence of primitive arteries, and anomalies of collateral branches. Other pathologies of the cervical VA associated exclusively with genetic diseases, such as spontaneous aneurysms and arteriovenous fistulae in neurofibromatosis type 1 are also considered.

Cavernous hemangioma of the parietal bone. Case report and review of the literature.

Intraosseous cavernous hemangiomas are a rare finding in the calvarium. It is a benign tumor arising from the intrinsic vasculature of the bone. We report one case observed in a 20 year-old male. The diagnostic peculiarities and therapeutic implications of this lesion are discussed and the available literature on this subject is reviewed. These tumors do not recur once a radical surgical removal is performed.

The surgical treatment of pituitary adenomas in the eighth decade.

BACKGROUND: The surgical treatment of pituitary adenomas in elderly patients (i.e., over 70 years of age) is a special problem because of the increased rate of perioperative complications and the reduced tolerance of postoperative fluid and electrolyte imbalance. Therefore, the unquestionable progress in the pharmacological and radiotherapy may not allow these patients the option of radical surgical treatment. We report our experience with the transsphenoidal procedure for pituitary adenomas in aged patients in an attempt to contribute to a better definition of the actual role of surgery. METHODS: Transsphenoidal surgery was performed in 11 patients over 70 years of age affected by various histological types of pituitary micro- and macroadenomas, ranging from Hardy Grade I through IIIc. Special care was dedicated to the postoperative treatment, in particular to water and electrolyte balances, and to the immediate treatment of any pathological variation of these parameters. RESULTS: We had no mortality and no postoperative adjunctive morbidity. All the patients recovered well from the operation with an average hospital stay of 20 days. The tumor removal was complete in six cases and partial in the remaining five. With an average follow-up of 2 years, we did observe only one case of symptomatic recurrence of the disease. CONCLUSIONS: Transsphenoidal surgery in the elderly is feasible and quite safe in the hands of an experienced team, if special care is devoted to the preoperative selection of patients and to the postoperative treatment of fluid and electrolyte imbalance.

Multiple skull base meningioma: case report.

BACKGROUND: The incidence of multiple skull base meningiomas varies from 1 to 3% in different series. Skull base meningiomas are rare. The pathogenetic role of low-dose radiation seems to be fairly well established in the oncogenesis of meningiomas. Calvarial location and multiplicity seem to be among the distinctive features of radiation-induced meningiomas. Skull base location is a very rare occurrence, mainly because the path of irradiation does not significantly involve this region. CASE REPORT: We describe a rare case of simultaneous occurrence of two skull base meningiomas in a 66-year-old female. This patient underwent low-dose irradiation for tinea capitis when she was 8 years old. The patient complained of nuchal pain, paresthesias in both hands, and progressive weakness on her right side. She was admitted to the hospital in September 1994. An MRI showed two masses, one located at the level of the tuberculum sellae and the other at the foramen magnum. These seemed very likely to be multiple meningiomas. The latter lesion, which was more symptomatic and dangerous, was operated on first. Six months later, elective treatment of the suprasellar meningioma was performed with success. CONCLUSION: The actual role of previous head irradiation in the oncogenesis of the present meningiomas remains somewhat unclear. Proper management and judicious use of skull base surgery techniques were key factors in the successful treatment of the patient.

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.

[Neurosurgery in Rome: 1880-1970]. / La neurochirurgia a Roma: 1880-1970.

Among many factors contributing to the birth of Neurosurgery, the "awareness of feasibility" of neurosurgical procedures is especially significant. June 1st, 1885 is the arbitrary birthdate of Neurosurgery in Rome: Francesco Durante, Professor of Surgery at the Royal University, successfully resects an olfactory groove meningioma, arising admiration worldwide. Roberto Alessandri succeeds Durante in 1919. In roman hospitals outstanding surgeons perform from time to time neurosurgical procedures, sometimes with satisfactory results: Paolo Postempski, Cesare Antonucci, Guido Egidi and Raffaele Bastianelli, first director of the "Regina Elena" Cancer Institute (IRE) (April 1933) and friend of Harvey Cushing. Angelo Chiasserini sr. is however the first roman surgeon constantly committed to Neurosurgery and founder of the first neurosurgical unit in Italy, in 1937; also, from 1941 to 1944 he directs a neurosurgical service at the "Celio" military hospital of Rome, to which patients from the various battle theatres are referred. While Mario Margottini and Piero Frugoni follow Bastianelli's steps at the IRE, the roman neurosurgeons Felice Visalli and Libero Ugelli serve in the Army, during the greek-albanian conflict and in Russia, respectively. In May 1955 Beniamino Guidetti, an Olivecrona pupil, is called at "La Sapienza" University of Rome and elevates the neurosurgical clinic at the highest standards. The other main neurosurgical pole is founded in 1958 at the San Camillo Hospital: within both institutions modern neurosurgical teams are formed along with neuroradiological and neuroanesthesiological staffs.

[Surgery of lumbar disk hernia: historical perspective]. / Chirurgia dell'ernia discale lombare: evoluzione storica.

Although the clinical picture of discogenic sciatica is well known already in the ancient world, it is not until 1933 that WJ Mixter and JS Barr provide the correct pathogenetic interpretation and suggest surgery as the treatment of choice. The work of the American Authors was however based on the knowledge acquired during the previous centuries starting with Domenico Cotugno, who first suggested the neurogenic nature of sciatica (1764) and later with the neurologists of the french school Valleix, Lasègue, Dejerine, Sicard who elucidated the semeiology and debated in detail the etiopathogenesis of the condition. The german pathologists Schmorl and Andrae (1927-29) are to be credited for their contribution to the pathology of intervertebral disc, recognizing the frequency and degenerative (not neoplastic) nature of nucleus pulposus herniation. Surgery of disc herniation starts with Oppenheim and Krause (1909). Mixter and Barr used laminectomy and a transdural route although a more limited approach to the spinal canal had already been proposed by the italian Bonomo (1902), unknown to many. Love, of the Mayo Clinic (1937-39) introduced the extradural/interlaminar approach while Caspar and Yasargil (1977) applied the concepts of microsurgery to the procedure. The latest advances are represented by percutaneous and endoscopic techniques.

[Current trends and prospects of fetal neurosurgery]. / Attualità e prospettive della neurochirurgia fetale.

Fetal surgery failed up to now to correct early in gestation nervous system pathologies before an irreversible damage occurs. The major hindrance is the induction of preterm labour considered for fetal surgery what rejection is for organ transplantation. Pharmacological tocolytic control seems an essential step before a routine surgery is established. In the meanwhile miniinvasive endoscopic surgery holds out the best promises, minimizing fetal and maternal stress. There are many convincing experimental evidences that endoscopic surgery may prevent secondary neurological damage of spinal cord in the myelomeningocele. Vascularized latissimus dorsi muscle flap or skin graft were effectively used, with endoscopic techniques, to create a protective patch to preserve the nervous tissue from mechanical and/or chemical damage. Endoscopic implant of neurons-rich grafts in damaged cerebral sites, with beneficial behavioural effects and increased learning capacity in the recipient animal with respect to controls, disclose further perspectives to fetoscopic surgery.