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BACKGROUND: Pfeiffer syndrome is characterized by craniosynostosis, mid-face hypoplasia, broad thumbs, and often multilevel airway obstruction. Airway management is often required, including the use of positive airway ventilation, nasopharyngeal airway (NPA), or tracheostomy. OBJECTIVE: The objective of this study was to assess the impact an airway adjunct can have on feeding difficulties in children with Pfeiffer syndrome. METHODS: Retrospective review of patients diagnosed with Pfeiffer syndrome from January 1998 to January 2020 at one of England's 4 supraregional Craniofacial Units, Alder Hey Children's Hospital. Speech & Language Therapy case notes and medical notes were used to gather data, as well as the Oral Feeding Score component of the UK Craniofacial Outcome Score. RESULTS: Eleven patients were included. Six patients had no airway adjunct (55%): 3 had tracheostomy (27%) and 2 patients had NPA (18%). All patients with airway adjuncts were percutaneous endoscopic gastrostomy/percutaneous endoscopic jejunostomy fed. Those who did not require an airway adjunct had an Oral Feeding Score of 4.60 (SD: 0.49). The children who went on to have an airway adjunct had a mean preintervention Oral Feeding Score of 2.4 (SD: 0.8). The mean feeding score (postairway adjunct) in the NPA group was 2.0, compared with the tracheostomy group scoring 3.0. CONCLUSIONS: Children with Pfeiffer syndrome who require airway intervention have more significant feeding problems requiring feeding intervention. Although there were small numbers included in this study, there is a suggestion that airway adjuncts can contribute to feeding difficulties, particularly NPAs.
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Acrocefalossindactilia , Obstrução das Vias Respiratórias , Humanos , Criança , Lactente , Acrocefalossindactilia/cirurgia , Manuseio das Vias Aéreas , Obstrução das Vias Respiratórias/cirurgia , Nasofaringe , Traqueostomia , Estudos RetrospectivosRESUMO
BACKGROUND: Apert syndrome is a genetic disorder characterized by craniofacial abnormalities and premature closure of the coronal sutures. The restriction of cranial development may have a subsequent effect on paranasal anatomy development. AIM: The aim of the study was to gain an understanding of paranasal sinus anatomical variations seen in children with Apert syndrome. MATERIALS AND METHODS: This was a retrospective review of computed tomography and magnetic resonance images of children with Apert syndrome from 2000 to 2020. Images were reviewed to identify anatomical variations in paranasal sinus anatomy. RESULTS: Twenty-one patients were included in the study. The most commonly seen variation was septal deviation in 86% of cases, with 60% of patients having a septal defect. The presence of protrusion or dehiscence of the infraorbital nerve, carotid canal and Vidian nerve, and presence of a concha bullosa were not observed in any patients. Keros type I was the most commonly observed olfactory fossa depth in 79% of patients, and type I Kuhn cells were observed in 83% of patients. CONCLUSIONS: To our knowledge, this is the first study which describes the prevalence of variations in paranasal sinus anatomy found in children with Apert syndrome. Septal deviation, type I Kuhn cells and Keros type I olfactory fossa depth were observed in a higher prevalence in our cohort than in the general population. As such, assessment for the presence of chronic rhinosinusitis and nasal obstruction should be evaluated as part of the multidisciplinary assessment.
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Acrocefalossindactilia , Deformidades Adquiridas Nasais , Seios Paranasais , Sinusite , Acrocefalossindactilia/diagnóstico por imagem , Criança , Humanos , Septo Nasal , Seios Paranasais/anatomia & histologia , Seios Paranasais/diagnóstico por imagem , Estudos RetrospectivosRESUMO
INTRODUCTION: Apert syndrome is a multisystem genetic disorder typically characterized by craniosynostosis and syndactyly. Studies also report an increased incidence of hearing loss in children with Apert syndrome in comparison to the general population. The aim of this study was to gain an understanding of the inner ear radiological anatomical variations seen in children with Apert syndrome and correlate these with audiological outcomes. MATERIALS AND METHODS: This was a retrospective review of computed tomography imaging of patients with Apert syndrome. Radiological images were examined for anatomical variations in inner ear structures. These were correlated with audiological testing. RESULTS: Nineteen patients were included in the study. The most commonly observed anomaly was an absent bony window of the lateral semi-circular canal (SCC) in 11 patients (58%), followed by an enlarged lateral SCC in 12 patients (63%). This combination of anomalies was seen collectively in 42% of patients and together these give the appearance of a 'rectangular vestibular cavity'. Audiological results were available in 11 patients and 9 of these patients had a conductive hearing loss. CONCLUSION: To the authors' knowledge, this is the first study that reports radiological findings alongside audiological testing in Apert syndrome and describes the appearance of a 'rectangular vestibular cavity'.
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Acrocefalossindactilia , Craniossinostoses , Orelha Interna , Perda Auditiva Neurossensorial , Perda Auditiva , Acrocefalossindactilia/complicações , Acrocefalossindactilia/diagnóstico por imagem , Criança , Craniossinostoses/complicações , Orelha Interna/anormalidades , Orelha Interna/diagnóstico por imagem , Perda Auditiva/complicações , Perda Auditiva Condutiva/etiologia , Perda Auditiva Neurossensorial/etiologia , Humanos , Estudos RetrospectivosRESUMO
OBJECTIVE: To compare visual estimation versus ImageJ calculation of tympanic membrane perforation size in the paediatric population between clinicians of different experience. METHODS: Five images of tympanic membrane perforations in children, captured using an otoendoscope, were selected. The gold standard was the ImageJ results by one consultant otologist. Consultants, registrars and Senior House Officers or equivalent were asked to visually estimate and calculate the perforation size using ImageJ software. RESULTS: The mean difference in variation from gold standard between visual estimation and ImageJ calculation was 12.16 per cent, 95 per cent CI (10.55, 13.78) p < 0.05, with ImageJ providing a more accurate estimation of perforation. Registrars were significantly more accurate at visual estimation than senior house officers. There was no statistically significant difference in ImageJ results between the different grades. CONCLUSION: Using ImageJ software is more accurate at estimating tympanic membrane perforation size than visual assessment for all ENT clinicians regardless of experience.
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Perfuração da Membrana Timpânica , Criança , Humanos , Software , Consultores , Membrana Timpânica/diagnóstico por imagemRESUMO
Heterogeneities existing in the patient during treatment are neglected, as the treated subject is considered homogeneous in most of the commercially-available treatment planning systems (TPSs) used for high dose rate (HDR) brachytherapy. The choice of a suitable dosimeter for experimental dosimetry near the HDR source is crucial, mainly due to existence of steep dose gradients. The present work aimed to assess the effect of rectal air heterogeneity and applicator attenuation in the HDR Ir-192 brachytherapy treatment of carcinoma uterine cervix by utilizing GAFCHROMIC EBT2 film dosimetry. The dose to rectal walls under the condition of rectal air heterogeneity was measured experimentally using EBT2 film in a rectal phantom, and the measurements were validated by the Monte Carlo (MC) simulations. The applicator attenuation was measured by EBT2 film for a commonly used stainless steel uterine tube in a homogeneous water equivalent phantom. The measured doses were compared with the TPS calculated values. In case of the air cavity, the measured dose at the closest rectal surface was 12.8% less than the TPS calculated value due to lack of back scattering, whereas at the farthest rectal surface, it was higher by 24.5% due to no attenuation. The magnitude of attenuation due to the metal applicator was measured as high as 2% when compared with the TPS calculation. The dose reduction at the nearest rectal surface due to the effect of rectal air has indicated a clinically favorable dose distribution within the rectum, whereas the shielding effect posed by the metallic applicator was found to be less significant. Mutual agreement of the measured doses with the MC calculated dose values confirmed the suitability of EBT2 film for clinical dosimetry in HDR brachytherapy.
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Braquiterapia/métodos , Dosimetria Fotográfica , Planejamento da Radioterapia Assistida por Computador/métodos , Reto/fisiopatologia , Neoplasias Uterinas/fisiopatologia , Neoplasias Uterinas/radioterapia , Absorção , Braquiterapia/instrumentação , Feminino , Humanos , Dosagem Radioterapêutica , Reto/efeitos da radiaçãoRESUMO
OBJECTIVE: Topical ciprofloxacin and dexamethasone have both been shown to disrupt healing of tympanic membrane perforations in animal models. There have been no clinical studies evaluating the effect of ciprofloxacin-dexamethasone (CD) ear drops on success of tympanoplasty. We compare perforation closure rates in pediatric endoscopic tympanoplasty with and without use of postoperative CD. STUDY DESIGN: Retrospective comparative cohort study. SETTING: Tertiary referral centre. PATIENTS: One hundred sixty-two totally endoscopic tympanoplasties with porcine-derived collagen graft in children, mean age 12.0âyears (range 2.3-17.9âyrs). INTERVENTION: Prescription of CD versus no ear drops in the immediate postoperative period. MAIN OUTCOME MEASURE: Perforation closure rate 2âmonths after totally endoscopic tympanoplasty. RESULTS: Postoperative CD was given to 65 (40%) ears and no drops given to the remainder. Overall, successful closure of tympanic membrane perforation was achieved in 140 (86%) of ears. The closure rate was not significantly different in those ears given CD postoperatively than those not given CD (54/65 [83%] vs 86/97 [89%], Fisher's pâ=â0.35). Multiple logistical regression revealed no confounding effect of other variables on outcome including age, revision surgery, graft position, or type of postoperative packing material. CONCLUSIONS: Our results reveal no harm or benefit with prescription of drops containing ciprofloxacin and dexamethasone on success of perforation closure after tympanoplasty. Allocation to treatment in this retrospective study was nonrandomized and was predominantly based on a change in practice. No other variables are known to have influenced this finding but a randomized prospective study could be justified for more reliable evidence.
Assuntos
Ciprofloxacina , Dexametasona , Perfuração da Membrana Timpânica , Timpanoplastia , Administração Tópica , Adolescente , Criança , Pré-Escolar , Ciprofloxacina/administração & dosagem , Ciprofloxacina/uso terapêutico , Dexametasona/administração & dosagem , Dexametasona/uso terapêutico , Humanos , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento , Perfuração da Membrana Timpânica/tratamento farmacológico , Perfuração da Membrana Timpânica/cirurgia , Timpanoplastia/métodosRESUMO
AIM: The aim of this work was to assess the suitability of the use of a Gafchromic EBT2 film for the measurement of anisotropy function for microSelectron HDR (192)Ir (classic) source with a comparative dosimetry method using a Gafchromic EBT2 film and thermoluminescence dosimeters (TLDs). BACKGROUND: Sealed linear radiation sources are commonly used for high dose rate (HDR) brachytherapy treatments. Due to self-absorption and oblique filtration of radiation in the source capsule material, an inherent anisotropy is present in the dose distribution around the source which can be described by a measurable two-dimensional anisotropy function, F(r, θ). MATERIALS AND METHODS: Measurements were carried out in a specially designed and locally fabricated PMMA phantom with provisions to accommodate miniature LiF TLD rods and EBT2 film dosimeters at identical radial distances with respect to the (192)Ir source. RESULTS: The data of anisotropy function generated by the use of the Gafchromic EBT2 film method are in agreement with their TLD measured values within 4%. The produced data are also consistent with their experimental and Monte Carlo calculated results for this source available in the literature. CONCLUSION: Gafchromic EBT2 film was found to be a feasible dosimeter in determining anisotropy in the dose distribution of (192)Ir source. It offers high resolution and is a viable alternative to TLD dosimetry at discrete points. The method described in this paper is useful for comparing the performances of detectors and can be applied for other brachytherapy sources as well.
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Cochlear implantation is a safe and reliable treatment for children with severe to profound hearing loss. The primary benefit of these medical devices in children is the acquisition of hearing, which promotes development of spoken language. The present paper reviews published literature demonstrating predictive effects of a number of factors on acquisition of hearing development and speech recognition. Of the many variables that contribute to an individual child's development after implantation, age at implantation, the presence of medical comorbidities, social determinants of health, and the provision of bilateral versus unilateral hearing are those that can vary widely and have consistently shown clear impacts. Specifically, age of implantation is crucial to reduce effects of deafness on the developing auditory system and capture the remarkable plasticity of early development. Language development after cochlear implantation requires therapy emphasizing hearing and oral communication, education, and other support which can be influenced by known social determinants of health; specifically, outcomes in children decline with reductions in socioeconomic status and levels of parental education. Medical co-morbidities also slow rates of progress after cochlear implantation. On the other hand, benefits of implantation increase in children who are provided with access to hearing from both ears. In sum, cochlear implants promote development of hearing in children and the best outcomes are achieved by providing early access to sound in both ears. These benefits can be limited by known social determinants of health which restrict access to needed support and medical comorbidities which add further complexity in care and outcome.
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Implante Coclear , Implantes Cocleares , Perda Auditiva/fisiopatologia , Desenvolvimento da Linguagem , Fatores Etários , Criança , Pré-Escolar , Feminino , Perda Auditiva/psicologia , Perda Auditiva/cirurgia , Testes Auditivos , Humanos , Lactente , Masculino , Fala , Percepção da FalaRESUMO
OBJECTIVES: Minimizing soft tissue reduction during the placement of percutaneous implants has been encouraged to improve cosmesis and perhaps decrease soft tissue complications. However, the fidelity of sound transmission and retention of transcutaneous magnet devices relates to the intervening soft tissue thickness. The objective of this study was to examine age-related variability in scalp thickness in patients receiving either percutaneously implanted or transcutaneous magnet-retained auditory prostheses. METHODS: Children undergoing percutaneous implant retained prostheses or cochlear implantation underwent two scalp thickness measurements: 1) a superior measurement at the conventional marked site for cochlear implant receiver stimulator and 2) an inferior measurement 1.5 cm below the superior measurement representing the conventional marked site for cochlear implant fantail. Linear regression was used for statistical analysis. RESULTS: Eighty-five children (median age 6 years; range 9 months-17 years) were examined. Scalp thickness at the two sites ranged from 1 to 10 mm (superior mean = 4.1 mm; inferior mean = 4.3 mm). A strong correlation was observed between age and scalp thickness at the superior (R = 0.62, p < 0.01) measurement, while a moderate correlation was observed at the inferior (R = 0.45, p < 0.01) sites. Children younger than 7 years displayed little variability in scalp thickness, and no correlation between age and scalp thickness (R = 0.002, p = 0.74). CONCLUSION: In older children, scalp thickness varies significantly with age. However, children younger than 7 years consistently have scalp thicknesses of 3-4 mm. Lack of variability in scalp thickness in children younger than 7 years argues against the use of an age-adjusted approach to soft tissue management in this age group.
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Implante Coclear , Implantes Cocleares , Seleção de Pacientes , Couro Cabeludo/patologia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Feminino , Humanos , Lactente , MasculinoRESUMO
Dengue hemorrhagic fever (DHF) is a potentially lethal complication of mosquito borne viral disease, Dengue Fever. Thrombocytopenia is a constant finding in DHF/Dengue Shock Syndrome (DSS). We report two cases that fulfilled the WHO criteria of DSS: high fever, positive tourniquet test, severe thrombocytopenia (<10,000/mm(3)), hemo-concentration (Hematocrit increase >20%), hypotension and bleeding refractory to routine therapeutic measures, who showed dramatic improvement after receiving Intravenous Anti-D globulin (IV anti-D).
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Hemorragia Gastrointestinal/tratamento farmacológico , Hemorragia/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Pneumopatias/tratamento farmacológico , Imunoglobulina rho(D)/uso terapêutico , Dengue Grave/complicações , Trombocitopenia/complicações , Adolescente , Pré-Escolar , Feminino , Hemorragia Gastrointestinal/etiologia , Hemorragia/etiologia , Humanos , Pneumopatias/etiologia , MasculinoRESUMO
Cardiac interventions often result in high radiation dose to patient's skin, so a reliable indicator in terms of a commonly used dose descriptor is required to monitor skin exposures. In the present study, Gafchromic XR-RV3 film was used to measure the peak skin dose (PSD) during 40 coronary angiography (CA) and 50 percutaneous transluminal coronary angioplasty (PTCA) procedures. Corresponding values of kerma-area product (PKA), fluoroscopy time (FT) and reference air-kerma (Ka,r) were recorded and correlated with PSD. Doses to patient's eyes and thyroid were also measured by using thermoluminescent dosimeters (TLDs) during PTCA procedures. The average dose to thyroid was about six times higher than the average dose to eyes. The mean values of PSD, PKA and FT were 1140 mGy, 97 Gy cm2 and 15.7 min for PTCA and 290 mGy, 21.1 Gy cm2 and 2.4 min for CA procedures, respectively. One in seven patients of PTCA procedure received PSD >2 Gy. With respect to FT, PKA may be used as a better predictor of skin exposures because the correlation of PSD with PKA was found better than with FT for both CA and PTCA procedures.
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Angiografia Coronária , Fluoroscopia , Doses de Radiação , Radiometria , Angioplastia Coronária com Balão , Cateterismo Cardíaco , Humanos , Radiografia Intervencionista , PeleRESUMO
It is well established that diagnostic X-ray practices must be optimised to keep patient radiation dose as low as compatible with providing the diagnostic information required. For effective optimisation of diagnostic exposures, the International Commission on Radiological Protection (ICRP) introduced the concept of diagnostic reference levels (DRLs) in 1996. The present study aimed to carry out an extensive dose survey of diagnostic radiography installations in the Uttarakhand region of India to establish local DRL Values for the different diagnostic practices. During the survey, air kerma values were measured for 297 diagnostic X-ray machines installed at 270 medical centres in the region and the entrance surface air kerma (K a,e) was estimated for ten commonly performed radiographic projections. These included chest posterior-anterior (PA), cervical spine anterior-posterior (AP), skull PA, abdomen AP, KUB (kidney, ureter and bladder), lumbar spine AP, lumbar spine lateral (LAT), pelvis AP, thoracic spine AP, and thoracic spine LAT. Wide variations were observed in the estimated values of K a,e for individual projections. The third quartile of the distribution of the median values of the estimated K a,e for a given projection was calculated to establish local DRL Values. The majority of the acquired dose data were found to be comparable to or less than the proposed national and international DRLs. The local DRL Values reported in this study may be used to improve radiological practice by reducing patient doses during radiography examinations. The obtained data may also contribute to a national patient dose database for establishing future national DRLs.
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Ar , Radiografia , Adulto , Humanos , Índia , Doses de Radiação , Valores de ReferênciaRESUMO
AIM: Dose verification to cochlea using metal oxide semiconductor field effect transistor (MOSFET) dosimeter using a specially designed multi slice head and neck phantom during the treatment of acoustic schwannoma by Gamma Knife radiosurgery unit. MATERIALS AND METHODS: A multi slice polystyrene head phantom was designed and fabricated for measurement of dose to cochlea during the treatment of the acoustic schwannoma. The phantom has provision to position the MOSFET dosimeters at the desired location precisely. MOSFET dosimeters of 0.2 mm x 0.2 mm x 0.5 µm were used to measure the dose to the cochlea. CT scans of the phantom with MOSFETs in situ were taken along with Leksell frame. The treatment plans of five patients treated earlier for acoustic schwannoma were transferred to the phantom. Dose and coordinates of maximum dose point inside the cochlea were derived. The phantom along with the MOSFET dosimeters was irradiated to deliver the planned treatment and dose received by cochlea were measured. RESULTS: The treatment planning system (TPS) estimated and measured dose to the cochlea were in the range of 7.4 - 8.4 Gy and 7.1 - 8 Gy, respectively. The maximum variation between TPS calculated and measured dose to cochlea was 5%. CONCLUSION: The measured dose values were found in good agreement with the dose values calculated using the TPS. The MOSFET dosimeter can be a suitable choice for routine dose verification in the Gamma Knife radiosurgery.
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Cóclea/efeitos da radiação , Neuroma Acústico/cirurgia , Doses de Radiação , Monitoramento de Radiação , Radiocirurgia , Humanos , Imagens de Fantasmas , Monitoramento de Radiação/métodos , Radiocirurgia/instrumentaçãoRESUMO
High-dose rate (HDR) 192Ir brachytherapy sources are commonly used for management of malignancies by brachytherapy applications. Measurement of source strength at the hospital is an important dosimetry requirement. The use of 0.6-cm(3) cylindrical ionization chamber is one of the methods of measuring the source strength at the hospitals because this chamber is readily available for beam calibration and dosimetry. While using the cylindrical chamber for this purpose, it is also required to determine the positioning error of the ionization chamber, with respect to the source, commonly called a distance error (c). The contribution of scatter radiation (M(s)) from floor, walls, ceiling, and other materials available in the treatment room also need to be determined accurately so that appropriate correction can be applied while calculating the source strength from the meter reading. Iterative methods of Newton-Raphson and least-squares were used in this work to determine scatter contribution in the experimentally observed meter reading (pC/s) of a cylindrical ionization chamber. Monte Carlo simulation was also used to cross verify the results of the least-squares method. The experimentally observed, least-squares calculated and Monte Carlo estimated values of meter readings from HDR 192Ir brachytherapy source were in good agreement. Considering procedural simplicity, the method of least-squares is recommended for use at the hospitals to estimate values of f (constant of proportionality), c, and M(s) required to determine the strength of HDR 192Ir brachytherapy sources.