Myocardial fat overgrowth in Proteus syndrome.

Proteus syndrome (PS) is a rare, mosaic disorder with asymmetric and distorting overgrowth of the skeletal system, skin, and adipose tissues. Cardiac abnormalities are rare in this syndrome and only two prior cases have been reported. Many patients with PS followed at our institution underwent transthoracic echocardiograms for preoperative evaluation or as work-up for associated pulmonary disease. Some were noted to have prominent, focal echodense areas in the myocardium. We further investigated cardiac findings in a cohort of children and adult patients with PS. Patients with abnormal echocardiograms were referred for cardiac magnetic resonance imaging, Holter monitoring, and exercise treadmill testing. Twenty children and adults with PS, age 24 months to 50 years old, underwent transthoracic echocardiograms. Seven patients (35%) had focal bright echodense areas within the myocardium suggesting fatty infiltration. The majority of patients had significant involvement of the interventricular septum. The cardiac characteristics of all patients with fatty infiltration on transthoracic echocardiograms were compared to Proteus patients without these findings. There were no significant differences in chamber sizes, mass, systolic or diastolic function. No increased risk of conduction defects or arrhythmias was found. This study shows that abnormal fat overgrowth is a common finding in the myocardium in patients with Proteus syndrome; however, it is not associated with functional derangements or arrhythmias. Further evaluation of a larger number of Proteus patients is needed in order to determine the frequency and prognosis of cardiac involvement. Published 2014. This article is a U.S. Government work and is in the public domain in the USA.

Marsupialization: a conservative approach for treating dentigerous cyst in children in the mixed dentition.

Dentigerous cysts are usually encountered in the practice of pediatric dentistry. The treatment modalities range from marsupialization to enucleation of the lesion and are based on the involvement of the lesion with the adjacent structures. However, loss of a permanent tooth in the management of a dentigerous cyst can be devastating to a child who has already a congenitally missing tooth. The first case describes the technique of marsupialization in which we extracted the grossly carious deciduous 1st molar and created a window through the extracted socket to decompress the lesion. In this case the 2nd premolars were congenitally missing on both sides of the mandible for which we had not gone for enucleation of the dentigerous cyst along with the developing 1st premolar. The second case is a developmental type of a big dentigerous cyst where marsupialization was followed by enucleation of the cystic lining but without removal of the affected tooth. Both the teeth erupted in the oral cavity.

Natural prevalence of antibody titers to glucosyltransferase of Streptococcus mutans in serum in high and low caries active children.

In this investigation, serum immunoglobulin G (IgG) and immunoglobulin A (IgA) titers, as well as total immunoglobulin concentration (IgG + IgA + IgM), were found to be raised with the increase in the number of dental caries. Only the total serum antibody titer in high dental caries (HDC) group was found to be significantly raised as compared to no dental carries (NDC) group. Although the IgG and IgA titers were raised in blood with the increased number of caries, the results were not statistically significant. However, we could not find any correlation between serum antibodies and dental caries except that there was an increased trend of serum antibodies to GTF with the increased number of carious lesions.

Natural prevalence of antibody titres to GTF of S. mutans in saliva in high and low caries active children.

The present study was conducted to find out the role of natural immunity against dental caries. Whole stimulated saliva of various caries from active children was collected. Antigen glucosyltransferase (GTF) was prepared from Streptococcus mutans serotype C and the antibody titre was estimated using ELISA. Salivary IgG, s-IgA and total immunoglobulins (IgG + s-IgA+ IgM) concentration were assayed. The result showed an increased level of salivary IgG concentration with the increased number of dental caries, which was not statistically significant. But there were significant decrease of s-IgA titre as well as total immunoglobulin concentration in saliva with the increased number of dental caries. This study showed that because of lack of local immunity the children are more prone to dental caries.

A game of colours, changing emotions in children: a pilot study.

AIM: To analyse the effect of colours on children's emotions and variations in anxiety levels using six different coloured attires, white, pink, yellow, green, purple and black. METHODS: Seventy-two children aged between 5-9 years old who had come for restorative treatment in the Department of Paediatric and Preventive Dentistry, I.T.S. Dental College, Muradnagar, Ghaziabad, India were chosen and divided according to Corah's dental anxiety scale into 4 levels of anxiety. Eighteen children from each level were then assigned six different colours (3 children for each colour). A two factor study was conducted to determine the influence of colours, anxiety levels or facial expressions on oxygen saturation levels and pulse rate of the children. Data was analysed using SPSS 16.0 for ANOVA. RESULTS: There was significant difference between colours influencing oxygen saturation levels (p = 0.001) and pulse rate (p = 0.038). A post-hoc test indicated that black colour was significantly different from most of the other colours. No interaction effect was found to exist between colours and facial expression. CONCLUSIONS: Data suggested that yellow, pink and purple colours may have reduced anxiety among children whereas black colour could have enhanced it.

EMI shielding of ABS composites filled with different temperature-treated equal-quantity charcoals.

Acrylonitrile-butadiene-styrene (ABS) composites were prepared by dry mixing equal-quantity (20 wt%) charcoals treated at different temperatures followed by hot compression. Processing parameters were kept the same. Seven samples of the same charcoal were modified for carbonization at different temperatures varying from 500 °C to 1100 °C in steps of 100 °C. Temperature treatment of charcoal crafts an increase in the conductivity of ABS composites, primarily accountable for the enhancement of shielding. The electromagnetic shielding effectiveness in the X-band (8.2-12.4 GHz) has been discovered to significantly increase for composites with ascending temperature-treated charcoals. An abrupt increase in the conductivity of ABS composites containing equal quantities of charcoal subjected to enhanced temperature treatments truly explains the effective absorption behaviour. The composite containing 1100 °C temperature-treated charcoal shows absorption-dominated SE of ∼36.8 dB at 11.6 GHz. Dielectric behaviour proclaims a decrease in dielectric loss (ε'') with an increase in the frequency. Besides this, the increased carbonization temperatures also show increased porosity and high dielectric losses. The scanning electron microscopy studies validate the increase in the porosity of charcoals due to the increase in the treatment temperature. The results are promising for the development of custom-made shielding composites possessing equal quantities of charcoal treated at enhanced temperatures.

Intercomparison of salivary nitric oxide as a biomarker of dental caries risk between caries-active and caries-free children.

AIM: To determine the efficacy of salivary nitric oxide as a biomarker for dental caries risk in children and to compare the salivary nitric oxide levels in caries-active and caries-free children. METHODS: One hundred children aged 6-12 years were selected and were divided into two groups according to DMFT index: as caries-free consisting of 50 individuals (DMFT/dmft = 0) and caries-active group (DMFT/dmft ≥3) consisting of 50 individuals. The saliva collected was estimated for nitric oxide level by Griess reaction method. Paired t test was used to correlate the difference of mean salivary nitric oxide levels between caries-active and caries-free groups. RESULTS: The mean level of nitric oxide in saliva of the control group was 581.3 ± 134.6 µg/mL and that of caries-active group was 335.4 ± 111.1 µg/mL, respectively. The mean salivary level of nitric oxide was higher in the caries-free group as compared with the caries-active group which was statistically significant (p = 0.000). CONCLUSIONS: Salivary nitric oxide was higher in the caries-free group as compared to the caries-active group which suggests that increased production of nitric oxide in the oral cavity may contribute to lower caries incidence in children.

Reoperation for brain metastases.

We report the results of reoperation for brain metastases in 21 patients with recurrent tumors following initial successful resection. The tumor recurrences were local (original site) in 14 patients, and occurred at other sites in the brain in the remaining seven. Time to CNS recurrence ranged from 3 to 30 months. At time of repeat craniotomy, disease was limited to the CNS in 12 (57%) of the patients. Median survival following second craniotomy was 9 months, and the actuarial 2-year survival was 25%. Neurological improvement was seen in two thirds of the patients; the median duration of neurological improvement was 6 months. There was no mortality, and only one patient developed increased deficit following surgery. We conclude that repeat resection of brain metastases is an important therapeutic option in selected patients, and should be considered in symptomatic patients with accessible mass lesions before the use of other experimental treatment.

Surgical treatment of spinal cord compression from epidural metastasis.

PURPOSE: A retrospective study of the results of neoplastic cord compression was undertaken to determine the effectiveness of surgical treatment and to assess quality of life in patients undergoing extensive procedures with potential morbidity. PATIENTS AND METHODS: Over a 5-year period (1989 to 1993), a total of 110 patients underwent surgery. Fifty-five patients (50%) had undergone prior treatment, including 47 (43%) who had failed to respond to prior irradiation (RT). Before surgery, 48 patients (44%) were nonambulatory, with severe paresis being present in 20. Surgery included staged anterior-posterior resections in 53 patients (48%), anterior resections in 33 (30%), and posterior resection in six (5%), all of whom required spinal instrumentation for reconstruction; only 18 patients underwent resection without instrumentation. RESULTS: Postoperatively, 90 patients (82%) were improved, both in terms of pain relief and ambulatory status. Fifty-three patients (48%) experienced postoperative complications, related statistically to the following three factors: age over 65 years, prior treatment, and presence of paraparesis. The overall median survival duration was 16 months, with 46% alive at 2 years. Apart from primary tumor, the presence of preoperative paraparesis had the most significant impact on survival. CONCLUSION: Our data suggest that the effective surgical treatment of neoplastic compression requires anterior-posterior resection in most patients to achieve the goal of total tumor resection, with the majority requiring instrumentation. Long-term survival is feasible in a subset of patients with this aggressive surgical approach.

Detection of Trypanosoma cruzi DNA in blood by PCR is associated with Chagas cardiomyopathy and disease severity.

BACKGROUND: The significance of detection of Trypanosoma cruzi DNA in blood of antibody-positive patients for risk of development of Chagas heart disease is not well established. The objective of this study was to compare detection of T. cruzi DNA with known clinical and laboratory markers of Chagas cardiomyopathy (CC) severity. METHODS: This is a case-control study nested within a retrospective cohort developed in Brazil to understand the natural history of Chagas disease. The study enrolled 499 T. cruzi seropositive blood donors (SP-BD) and 488 frequency matched seronegative control donors (SN-BD) who had donated between 1996 and 2002, and 101 patients with clinically diagnosed CC. In 2008-2010 all enrolled subjects underwent a health questionnaire, medical examination, electrocardiograms and echocardiograms and polymerase chain reaction (PCR) analyses. A blinded panel of three cardiologists adjudicated the outcome of CC. Trypanosoma cruzi kinetoplast minicircle sequences were amplified by real-time PCR using an assay with a sensitivity of one parasite per 20 mL of blood. All testing was performed on coded samples. RESULTS: Rates of PCR detection of T. cruzi DNA were significantly (P = 0.003) higher in CC patients and SP-BD diagnosed with CC (79/105 [75.2 %]) compared with SP-BD without CC (143/279 [51.3%]). The presence of parasitaemia was significantly associated with known markers of disease progression such as QRS and QT interval duration, lower left ventricular ejection fraction, higher left ventricular index mass, and elevated troponin and NTpro-BNP levels. CONCLUSION: Trypanosoma cruzi PCR positivity is associated with presence and severity of cardiomyopathy, suggesting a direct role of parasite persistence in disease pathogenesis.

Meningeal nodules with features of extranodal sinus histiocytosis with massive lymphadenopathy.

We present an unusual case of multiple intracranial meningeal nodules in a 30-year-old man. Histologically, the nodules consisted predominantly of plasma cells, histiocytes, and lymphoid cells with Russell bodies and emperipolesis. Emperipolesis may be a clue to understanding the pathogenesis of the process that has features consistent with so-called sinus histiocytosis with massive lymphadenopathy in the extranodal location.

Quantification of aortic regurgitation by real-time 3-dimensional echocardiography in a chronic animal model: computation of aortic regurgitant volume as the difference between left and right ventricular stroke volumes.

BACKGROUND: The accuracy of conventional 2-dimensional echocardiographic and Doppler techniques for the quantification of valvular regurgitation remains controversial. In this study, we examined the ability of real-time 3-dimensional (RT3D) echocardiography to quantify aortic regurgitation by computing aortic regurgitant volume as the difference between 3D echocardiographic-determined left and right ventricular stroke volumes in a chronic animal model. METHODS: Three to 6 months before the study, 6 sheep underwent surgical incision of one aortic valve cusp to create aortic regurgitation. During the subsequent open chest study session, a total of 25 different steady-state hemodynamic conditions were examined. Electromagnetic (EM) flow probes were placed around the main pulmonary artery and ascending aorta and balanced against each other to provide reference right and left ventricular stroke volume (RVSV and LVSV) data. RT3D imaging was performed by epicardial placement of a matrix array transducer on the volumetric ultrasound system, originally developed at the Duke University Center for Emerging Cardiovascular Technology. During each hemodynamic steady state, the left and right ventricles were scanned in rapid succession and digitized image loops stored for subsequent measurement of end-diastolic and end-systolic volumes. Left and right ventricular stroke volumes and aortic regurgitant volumes were then calculated and compared with reference EM-derived values. RESULTS: There was good correlation between RT3D left and right ventricular stroke volumes and reference data (r = 0.83, y = 0.94x + 2.6, SEE = 9.86 mL and r = 0.63, y = 0.8x - 1.0, SEE = 5.37 mL, respectively). The resulting correlation between 3D- and EM-derived aortic regurgitant volumes was at an intermediate level between that for LVSV and that for RVSV (r = 0.80, y = 0.88x + 7.9, SEE = 10.48 mL). RT3D tended to underestimate RVSV (mean difference -4.7 +/- 5.4 mL per beat, compared with -0.03 +/- 9.7 mL per beat for the left ventricle). There was therefore a small overestimation of aortic regurgitant volume (4.7 +/- 10.4 mL per beat). CONCLUSION: Quantification of aortic regurgitation through the computation of ventricular stroke volumes by RT3D is feasible and shows good correlation with reference flow data. This method should also be applicable to the quantification of other valvular lesions or single site intracardiac shunts where a difference between right and left ventricular cavity stroke volumes is produced.

Dural arteriovenous malformation of the major venous sinuses: an acquired lesion.

Arteriovenous malformations of the dura are thought to be congenital. However, arteriographic investigations of four patients who, after a head injury, developed dural arteriovenous fistulae with features of congenital malformations suggest that these abnormal communications may also be acquired. Thrombosis or thrombophlebitis in the dural sinus or vein may be the primary event in their formation. The pathogenesis is probably "growth" of the dural arteries normally present in the walls of the sinuses during the organization of an intraluminal thrombus. This may result in a direct communication between artery and vein or sinus, establishing an abnormal shunt. Ultimate fibrosis of the sinus wall and intraluminal thrombus may be the factors responsible for the spontaneous disappearance of such malformations.

Ependymal cyst of the quadrigeminal plate: case report.

A patient with an ependymal cyst of the quadrigeminal plate is reported. The patient presented with headache, diplopia, and evidence of increased intracranial pressure. A nonenhancing cyst with hydrocephalus was demonstrated by computed tomographic scan. The cyst was subtotally resected using a transtentorial approach. The causes, clinical presentation, and treatment of quadrigeminal plate ependymal cysts are discussed, together with a review of the literature.

Malignant meningioma within the third ventricle: a case report.

A case of a malignant meningioma within the 3rd ventricle is presented. Although malignant transformation of meningiomas has been described, occurrence within the ventricular system is extremely rare.

Ependymomas of the third ventricle.

Third ventricular ependymomas are rare tumors that have not been specifically examined. Four cases of these tumors are reported. The presenting symptoms included headache, ataxia, vertigo, and Parinaud's syndrome. All the patients underwent computed tomographic scanning and cerebral angiography, followed by craniotomy and microsurgical resection of the tumor. In addition, all patients had or developed symptomatic obstructive hydrocephalus requiring shunting procedures. Three of the patients are alive with a follow-up of 4 to 12 years. It is remarkable that these tumors are so rare, given that the ependymal surface area of the third ventricle is greater than that of the fourth. The management of these tumors should include aggressive surgical resection, radiation therapy, and cerebrospinal fluid diversion.

Congenital fourth ventricular midline outlet obstruction. Report of two cases.

Obstruction of the foramen of Magendie unrelated to illness or trauma is rare in adults. Two cases of congenital membranous occlusion of the foramen of Magendie in adults are presented. Analysis of the reported cases of fourth ventricular outlet obstruction disclosed only three similar cases.

Radiation-induced meningiomas: experience at the Mount Sinai Hospital and review of the literature.

From the records of The Mount Sinai Hospital, seven cases which met established criteria for radiation-induced meningiomas were identified. This represents the largest series of radiogenic meningiomas documented in North America and includes both intracranial and intraspinal tumors. The records and pathological specimens were reviewed and these data analyzed with other cases retrieved from the world literature. This study reveals that radiation-induced meningiomas can be categorized into three groups based on the amount of radiation administered: 1) low dose; 2) moderate dose and miscellaneous; and 3) high dose. The overwhelming majority of cases had received low-dose irradiation (800 rad) to the scalp for tinea capitis and the second largest group resulted from high-dose irradiation for primary brain tumors (greater than 2000 rad). The unique features distinguishing radiation-induced meningiomas from other meningiomas are reviewed. Although histologically atypical tumors were common in this series, overt malignancy was not encountered. The preoperative management of these lesions should include angiography to evaluate for large-vessel occlusive vasculopathy, a known association of meningiomas induced by high-dose irradiation. Given the propensity these tumors possess for recurrence, a wide bony and dural margin is recommended at surgical resection.

Indications and results of combined anterior-posterior approaches for spine tumor surgery.

Spinal instrumentation currently allows gross-total resection and reconstruction in cases of malignancies at all levels of the spine. The authors analyzed the results in 110 patients who underwent surgery for primary and metastatic spinal tumors over a 5-year period (1989-1993) at a single institution. Major primary sites of tumor included breast (14 cases), chordoma (14 cases), lung (12 cases), kidney (11 cases), sarcoma (13 cases), plasmacytoma (10 cases), and others (36 cases). Prior to surgery, 55 patients (50%) had received prior treatment. Forty-eight patients (44%) were nonambulatory, and severe paraparesis was present in 20 patients. Fifty-three patients (48%) underwent combined anterior-posterior resection and instrumentation. 33 (30%) underwent anterior resection with instrumentation, 18 (16%) underwent anterior or posterior resection alone, and the remaining six patients (5%) underwent posterior resection and instrumentation. Major indications for anterior-posterior resection included three-column involvement, high-grade instability, involvement of contiguous vertebral bodies, and solitary metastases. Postoperatively, 90 patients improved neurologically. The overall median survival was 16 months, with 46% of patients surviving 2 years. Fifty-three patients (48%) suffered postoperative complications. Despite the high incidence of complications, the majority of patients reported improvement in their quality of life at follow-up review. Our findings suggest that half of all patients with spinal malignancies require combined anterior-posterior surgery for adequate tumor removal and stabilization.

Posttraumatic pseudomeningomyelocele (enlarging fracture?) in a vertebral body. Case report.

An unusual case is reported in which a posttraumatic peudomeningomyelocele developed over many years inside the body of a fractured lumbar vertebra, eroding the pedicle and causing progressive neurological deficit. The wall of the sac was mostly formed by the scalloped bar bone, and partly by a membrane resembling the dura. The terminal part of the conus medullaris and some nerve roots of the cauda equina formed the contents and parts of the wall of the outpouching of the subarachnoid space into the vertebral body. A comparison is drawn between this lesion and formation of an "enlarging fracture" of the skull. The surgical technique used for obliteration of this pseudomeningomyelocele is described.