149 results on '"Muthialu N"'
Search Results
2. EARLY SURGICAL INTERVENTION AMELIORATES RESPIRATORY FAILURE IN GIANT CONGENITAL PULMONARY AIRWAY MALFORMATION
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RAMASWAMY, M., primary, MANN, E., additional, MCINTYRE, D., additional, BEEMAN, A., additional, CALDER, A., additional, and MUTHIALU, N., additional
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- 2022
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3. P0398 / #971: CHEST PHYSIOTHERAPY FOR SLIDE TRACHEOPLASTY PATIENTS IN PAEDIATRIC INTENSIVE CARE
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Woyda, J., primary, Muthialu, N., additional, and Shkurka, E., additional
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- 2021
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4. (668) Stem-Cell Based Tissue-Engineered Tracheal Transplant in Pediatric Patients: A Single-Centre Experience
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Ramaswamy, M., Butler, C., McIntyre, D., McIntosh, N., Mann, E., Hewitt, R., De Coppi, P., Elliott, M., and Muthialu, N.
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- 2023
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5. Bronchopleural fistula in a newborn undergoing ECMO-transbronchial closure
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Saleemi, M S, McLaren, C, Sharma, B K, Muthialu, N, Roebuck, D, and Ng, C
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- 2013
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6. Explantation of Mechanical Circulatory Support Devices to Recovery is Possible and Should Be Considered in Selected Conditions in Children
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Ramaswamy, M., primary, Khushnood, A., additional, Kostolny, M., additional, Reinhardt, Z., additional, Chila, T., additional, De Rita, F., additional, Burch, M., additional, Hasan, A., additional, and Muthialu, N., additional
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- 2019
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7. Pediatric Lung Transplantation: Fit for Split?
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Martens, T., primary, Kanakis, M., additional, Spencer, H., additional, and Muthialu, N., additional
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- 2019
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8. The multi-disciplinary management of complex congenital and acquired tracheo-oesophageal fistulae
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Thakkar, H. S., primary, Hewitt, R., additional, Cross, K., additional, Hannon, E., additional, De Bie, F., additional, Blackburn, S., additional, Eaton, S., additional, McLaren, C. A., additional, Roebuck, D. J., additional, Elliott, M. J., additional, Curry, J. I., additional, Muthialu, N., additional, and De Coppi, P., additional
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- 2018
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9. Airway Clearance Following Lung Transplant: Current UK Practice
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Shkurka, E., primary, Spencer, H., additional, and Muthialu, N., additional
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- 2018
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10. Supplemental Use of Blood Cardioplegia Before Graft Implantation in Pediatric Heart Transplantation
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Ramaswamy, M., primary, Beeman, A., additional, Henwood, S., additional, Hawkyard, A., additional, Robertson, A., additional, Ridout, D., additional, Walker, A., additional, Patel, R., additional, Simmonds, J., additional, and Muthialu, N., additional
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- 2018
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11. F-062SLIDE TRACHEOPLASTY FOR TRACHEAL STENOSIS IN CHILDREN: EXPERIENCE FROM 20 YEARS IN A NATIONAL REFERRAL CENTRE
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Asadi, Nizar, primary, Butler, C, additional, Mcintyre, D, additional, Mcintosh, N, additional, Roebuck, D, additional, Mclaren, C, additional, Wallis, C, additional, Muthialu, N, additional, Hewitt, R, additional, and Elliott, M, additional
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- 2017
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12. V-038SLIDE TRACHEOPLASTY FOR CONGENITAL TRACHEAL STENOSIS IN CHILDREN: OPERATIVE TECHNIQUE
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Asadi, Nizar, primary, Butler, C, additional, Bieli, C, additional, Mcintyre, D, additional, Mcintosh, N, additional, Wallis, C, additional, Roebuck, D, additional, Mclaren, C, additional, Muthialu, N, additional, Hewitt, R, additional, and Elliott, M, additional
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- 2017
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13. G366(P) The presentation of an oesophageal button battery is a paediatric emergency
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Selby, L, primary, Curry, J, additional, Hewitt, R, additional, Muthialu, N, additional, and Wallis, C, additional
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- 2017
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14. Nissen's Fundoplication Is Safe and Prevents Decrease in Forced Expiratory Volume in One Second (FEV 1 ) in Paediatric Lung Transplant Patients
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Pabary, R., primary, Aurora, P., additional, Curry, J., additional, Hulsker, C., additional, Muthialu, N., additional, and Spencer, H., additional
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- 2017
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15. The Safety and Efficacy of Total Lymphoid Irradiation in Pediatric Patients with Progressive Chronic Lung Allograft Dysfunction
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Low, G., primary, Aurora, P., additional, Gaze, M., additional, Muthialu, N., additional, and Spencer, H., additional
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- 2017
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16. Treatment of postpneumonectomy syndrome with tissue expanders: The Great Ormond Street Hospital experience
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Lloyd, M.S., Wallis, C., Muthialu, N., Elliott, M., and Bulstrode, N.W.
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- 2014
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17. Successful orthotopic heart transplantation using a donor heart with ALCAPA
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Simmonds, J. D., primary, Mustafa, M., additional, Fajardo Jaramillo, D. P., additional, Bellsham-Revell, H. R., additional, Marek, J., additional, Burch, M., additional, Tsang, V. T., additional, and Muthialu, N., additional
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- 2016
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18. (1027) - Supplemental Use of Blood Cardioplegia Before Graft Implantation in Pediatric Heart Transplantation
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Ramaswamy, M., Beeman, A., Henwood, S., Hawkyard, A., Robertson, A., Ridout, D., Walker, A., Patel, R., Simmonds, J., and Muthialu, N.
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- 2018
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19. (746) - Airway Clearance Following Lung Transplant: Current UK Practice
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Shkurka, E., Spencer, H., and Muthialu, N.
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- 2018
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20. (728) - Nissen's Fundoplication Is Safe and Prevents Decrease in Forced Expiratory Volume in One Second (FEV1) in Paediatric Lung Transplant Patients
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Pabary, R., Aurora, P., Curry, J., Hulsker, C., Muthialu, N., and Spencer, H.
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- 2017
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21. (724) - The Safety and Efficacy of Total Lymphoid Irradiation in Pediatric Patients with Progressive Chronic Lung Allograft Dysfunction
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Low, G., Aurora, P., Gaze, M., Muthialu, N., and Spencer, H.
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- 2017
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22. 110-I * PULMONARY ARTERY SLING REPAIR: SINGLE-CENTRE EXPERIENCE WITH ANALYSIS OF RISK FACTORS
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Muthialu, N., primary, Bezuska, L., additional, Nakao, M., additional, Elliott, M. J., additional, and Tsang, V., additional
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- 2014
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23. One-stage sequential bilateral thoracic expansion for asphyxiating thoracic dystrophy (Jeune syndrome)
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Muthialu, N., primary, Mussa, S., additional, Owens, C. M., additional, Bulstrode, N., additional, and Elliott, M. J., additional
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- 2014
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24. Direct implantation of scimitar vein to the left atrium via sternotomy: a reappraisal
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Jensen, H., primary, Muthialu, N., additional, Furci, B., additional, Yates, R., additional, Kostolny, M., additional, and Tsang, V., additional
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- 2013
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25. Functional outcomes after the Ross (pulmonary autograft) procedure assessed with magnetic resonance imaging and cardiopulmonary exercise testing
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Puranik, R., primary, Tsang, V. T., additional, Broadley, A., additional, Nordmeyer, J., additional, Lurz, P., additional, Muthialu, N., additional, Derrick, G., additional, Walker, F., additional, Cullen, S., additional, de Leval, M., additional, Bonhoeffer, P., additional, Taylor, A. M., additional, and Muthurangu, V., additional
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- 2009
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26. G366(P) The presentation of an oesophageal button battery is a paediatric emergency
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Selby, L, Curry, J, Hewitt, R, Muthialu, N, and Wallis, C
- Abstract
AimsDamage related to button battery ingestion is well recognised; tissues act as a conductor between negative and positive poles of the battery, producing sodium hydroxide and mucosal burns. Urgent removal is recommended but severe consequences of oesophageal lodgement are less well-recognised. We describe 8 cases of oesophageal button battery ingestion, highlighting importance of the oesophageal button battery as a paediatric emergency. Damage occurs within 1–2 hours of ingestion; delays before removal can be life-threatening.MethodsWe reviewed 8 cases of oesophageal button batteries over a 3 year period, noting time from ingestion to removal and the associated morbidities, from minor oesophageal injury to complex, tracheal and oesophageal surgery for perforation and erosion.ResultsAbstract G366 Table 1DemographiesIngestion to removalMorbidityFollow up5y,F3 hoursOesophageal mucosal injury.Outpatient clinic.17m, F3 hoursMediastinitis and tracheal- oesophageal fistula.Nasogastric feeds, surveillance endoscopies.23m, M4 hoursOesophageal mucosal injury.Oesophageal dilatations, outpatient review.13m, F5-7 hoursOesphageal mucosal injury.Oesophageal dilatations.2y,F7 daysTwo tracheal repairs, oesophagectomy.Tracheal stenting, oesophageal replacement surgery.2y, F7 daysAcquired tracheal-oesophageal fistula, oesophageal stricture, tracheal stenosis, required 2 tracheal repairs.Dilatations of trachea and oesophagus.2y, M5 monthsOesophageal resection and oesophageal atresia.Oesophageal dilatations.3.5y, FEmbedded batteryBattery removed from oesophageal pouch, oesophageal atresia.Oesophageal dilatations, possible pouch surgery.ConclusionsIngested button batteries lodged in the oesophagus can cause tissue injury in under 2 hours. Common sites for lodgement are the thoracic inlet, aortic arch, right main bronchus and gastro-oesophageal junction. Further damage occurs as a result of residual battery charge and time of contact between the battery and mucosal surfaces. Mortality is described due to aorto- oesophageal fistula. It is imperative batteries are removed immediately irrespective of anaesthetic starvation times and that oesophageal battery ingestion is highlighted as a paediatric emergency. There is no national guideline for the management of these children in the UK. Interventions must focus on increasing awareness of the dangers of button battery ingestion.
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- 2017
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27. India and the management of road crashes.
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Muthialu N
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- 2006
28. Stem-Cell Based Tissue-Engineered Tracheal Transplant in Pediatric Patients: A Single-Centre Experience.
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Ramaswamy, M., Butler, C., McIntyre, D., McIntosh, N., Mann, E., Hewitt, R., De Coppi, P., Elliott, M., and Muthialu, N.
- Subjects
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CHILD patients , *STEM cell transplantation , *PATIENTS' attitudes , *METAL erosion , *TRACHEAL stenosis , *EXERCISE tolerance - Abstract
To report our institution's experience of employing autologous stem-cell based decellularized tissue-engineered graft in reconstructing trachea in paediatric patients when conventional reconstructive surgical interventions have failed. Three paediatric patients underwent autologous stem-cell seeded decellularized tracheal graft in the last twelve years. Their pre-operative characteristics including primary diagnosis, previous airway interventions, and post-operative outcomes including survival and quality of life in regard to respiratory status were evaluated. Out of three transplanted pediatric patients, two have survived till date. The first patient, male, with multiple co-morbidities, was ten years when transplanted (now 23 years, 12.6 years follow-up) having undergone pericardial patch tracheoplasty as a neonate for congenital tracheal stenosis followed by cadaveric tracheal homograft at two years of age for erosion of an implanted metal tracheal stent. This patient now has a metal stent in the trachea to support the graft. The second patient, female, with co-morbidities of absent right lung and cleft palate, was sixteen years at transplantation having undergone two tracheoplasty procedures in infancy and at three years of age for congenital tracheal stenosis. This patient died three weeks post-transplantation due to acute airway obstruction likely from an intrathoracic bleed. This case highlighted consideration of using tracheal stent post-transplantation until the graft can attain strength. The third patient, female, with no co-morbidities, was transplanted at four years of age (now 9 years, 5.4 years follow-up) having undergone two tracheoplasty repairs at two and four years of age for a caustic tracheo-esophageal injury sustained after ingesting a button battery. Surviving two patients have regular airway surveillance including bronchoscopy, ciliary and biopsy assessments. Both have an acceptable quality of life, a good exercise tolerance, and does not require any respiratory support. Autologous stem-cell seeded, decellularized tissue-engineered graft usage can be considered a feasible option for tracheal reconstruction in paediatric patients when conventional surgical interventions have failed until a suitable alternative is found for which further research is required. [ABSTRACT FROM AUTHOR]
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- 2023
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29. The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey
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Casper M. Kersten, Sergei M. Hermelijn, Dhanya Mullassery, Nagarajan Muthialu, Nazan Cobanoglu, Silvia Gartner, Pietro Bagolan, Carmen Mesas Burgos, Alberto Sgrò, Stijn Heyman, Holger Till, Janne Suominen, Maarten Schurink, Liesbeth Desender, Paul Losty, Henri Steyaert, Suzanne Terheggen-Lagro, Martin Metzelder, Arnaud Bonnard, Rony Sfeir, Michael Singh, Iain Yardley, Noor R. V. M. Rikkers-Mutsaerts, Cornelis K. van der Ent, Niels Qvist, Des W. Cox, Robert Peters, Michiel A. G. E. Bannier, Lucas Wessel, Marijke Proesmans, Michael Stanton, Edward Hannon, Marco Zampoli, Francesco Morini, Harm A. W. M. Tiddens, René M. H. Wijnen, Johannes M. Schnater, Paediatric Pulmonology, Institut Català de la Salut, [Kersten CM, Hermelijn SM] Department of Pediatric Surgery, Erasmus MC Sophia Children’s Hospital, Rotterdam, The Netherlands. [Mullassery D, Muthialu N] Department of Pediatric Surgery, Great Ormond Street Hospital, London, UK. [Cobanoglu N] Department of Pediatric Pulmonology, Ankara University School of Medicine, Ankara, Turkey. [Gartner S] Unitat de Pneumologia Pediàtrica i Fibrosi Quística, Vall d’Hebron Hospital Universitari, Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus, Kindergeneeskunde, MUMC+: MA Medische Staf Kindergeneeskunde (9), RS: FHML non-thematic output, Pediatrics, Pediatric Surgery, and Radiology & Nuclear Medicine
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técnicas de investigación::métodos::diseño de la investigación [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS] ,congenital lung abnormalities ,congenital pulmonary airway malformation ,core outcome set ,outcome parameters ,consensus ,CYSTIC ADENOMATOID MALFORMATION ,INFANTS ,CHILDREN ,LOBECTOMY ,Pediatrics ,ARGUMENT ,POSTNATAL MANAGEMENT ,Investigative Techniques::Methods::Research Design [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT] ,Medicine and Health Sciences ,MANAGEMENT ,POSTNATAL ,LUNG MALFORMATIONS ,PREDICTORS ,Science & Technology ,LESIONS ,Presa de decisions ,NATURAL-HISTORY ,Reconstructive and regenerative medicine Radboud Institute for Health Sciences [Radboudumc 10] ,Pulmons - Malalties ,Ciencias de la información::análisis de sistemas::técnica Delfos [CIENCIA DE LA INFORMACIÓN] ,Respiratory Tract Diseases::Lung Diseases [DISEASES] ,Pediatrics, Perinatology and Child Health ,Information Science::Systems Analysis::Delphi Technique [INFORMATION SCIENCE] ,Malalties congènites ,enfermedades respiratorias::enfermedades pulmonares [ENFERMEDADES] ,Life Sciences & Biomedicine - Abstract
Anomalías pulmonares congénitas; Consenso; Parámetros de resultado Anomalies pulmonars congènites; Consens; Paràmetres de resultat Congenital lung abnormalities; Consensus; Outcome parameters Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.
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- 2022
30. Development of a core outcome set for congenital pulmonary airway malformations: study protocol of an international Delphi survey
- Author
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René M H Wijnen, Maarten Schurink, Nagarajan Muthialu, Pietro Bagolan, Nazan Cobanoglu, Dhanya Mullassery, Holger Till, M. Singh, M. Boon, Sergei Hermelijn, Casper Kersten, Silvia Gartner, Carmen Mesas Burgos, Alberto Sgro, Stijn Heyman, Janne Suominen, Liesbeth Desender, Paul Losty, Kjetil Ertresvag, Harm A W M Tiddens, Marco Schnater, S.M. Hermelijn, C.M. Kersten, J.M. Schnater, R.M.H. Wijnen, H.A.W.M. Tiddens, S.C.M. Cochius, J. Suominen, M. Pakarinen, L. Martelius, S. Heyman, D. Vervloessem, H. Steyaert, A. Sgrò, P. Gamba, M. Schurink, S. van der Heide, J. Roukema, N. Rikkers-Mutsaerts, S. Terheggen-Lagro, S. de Beer, E. Haarman, H. Till, G. Singer, M. Metzelder, P Sezen, L. Desender, H. Schaballie, N. Cobanoglu, G. Gollu, M. Stanton, A. Bonnard, R. Sfeir, N. Muthialu, D. Mullassery, C. Wallis, D. Cox, P. Bagolan, F. Morini, C. Mesas Burgos, P. Conner, E. Caffrey Osvald, C. Bitkover, H. Decaluwé, M. Proesmans, J. Deprest, S. Gartner, A. Lain, P.D. Losty, I. Sinha, I. Yardley, L. Wessel, K. Zahn, T. Schaible, N. Qvist, M. Zampoli, G. Aksnes, C. K. van der Ent, K.M. Winter-de Groot, R. Peters, E. Hannon, Q. Jöbsis, M. Bannier, Institut Català de la Salut, [Hermelijn S, Kersten C] Pediatric Surgery, Erasmus MC Sophia Children's Hospital, Rotterdam, The Netherlands. [Mullassery D, Muthialu N] Pediatric Surgery, Great Ormond Street Hospital for Children, London, UK. [Cobanoglu N] Pediatric Pulmonology, Ankara University Faculty of Medicine, Ankara, Turkey. [Gartner S] Servei de Pneumologia Pediàtrica, Vall d'Hebron Hospital Universitari, Barcelona, Spain, Vall d'Hebron Barcelona Hospital Campus, Pediatric Surgery, Pediatrics, Amsterdam Reproduction & Development (AR&D), Pediatric surgery, HUS Children and Adolescents, Lastenkirurgian yksikkö, Children's Hospital, University of Helsinki, MUMC+: MA Medische Staf Kindergeneeskunde (9), Kindergeneeskunde, and RS: FHML non-thematic output
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paediatric intensive & ,Delphi Technique ,Outcome Assessment ,Delphi method ,Outcome (game theory) ,law.invention ,ARGUMENT ,0302 clinical medicine ,Randomized controlled trial ,Informed consent ,law ,Surveys and Questionnaires ,Outcome Assessment, Health Care ,030212 general & internal medicine ,Child ,Pulmonologists ,Congenital pulmonary airway malformation ,General Medicine ,Research Personnel ,3. Good health ,Ciencias de la información::análisis de sistemas::técnica Delfos [CIENCIA DE LA INFORMACIÓN] ,Research Design ,neonatology ,paediatric intensive & critical care ,paediatric surgery ,paediatric thoracic medicine ,paediatric thoracic surgery ,Consensus ,Humans ,Infant, Newborn ,Respiratory Tract Diseases::Lung Diseases [DISEASES] ,030220 oncology & carcinogenesis ,Medicine ,Information Science::Systems Analysis::Delphi Technique [INFORMATION SCIENCE] ,Protocols clínics ,Malalties congènites ,técnicas de investigación::métodos::diseño de la investigación [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS] ,medicine.medical_specialty ,03 medical and health sciences ,All institutes and research themes of the Radboud University Medical Center ,Intensive care ,Investigative Techniques::Methods::Research Design [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT] ,MANAGEMENT ,medicine ,Intensive care medicine ,business.industry ,Infant ,Newborn ,medicine.disease ,Reconstructive and regenerative medicine Radboud Institute for Health Sciences [Radboudumc 10] ,Health Care ,Pulmons - Malalties ,critical care ,Critical appraisal ,3121 General medicine, internal medicine and other clinical medicine ,PEDIATRIC SURGEONS ,Surgery ,enfermedades respiratorias::enfermedades pulmonares [ENFERMEDADES] ,business - Abstract
Neonatologia; Cirurgia pediàtrica; Medicina toràcica pediàtrica Neonatología; Cirugía pediátrica; Medicina torácica pediátrica Neonatology; Paediatric surgery; Paediatric thoracic medicine Introduction A worldwide lack of consensus exists on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) even though the incidence is increasing. Either a surgical resection is performed or a wait-and-see policy is employed, depending on the treating physician. Management is largely based on expert opinion and scientific evidence is scarce. Wide variations in outcome measures are seen between studies making comparison difficult thus highlighting the lack of universal consensus in outcome measures as well. We aim to define a core outcome set which will include the most important core outcome parameters for paediatric patients with an asymptomatic CPAM. Methods and analysis This study will include a critical appraisal of the current literature followed by a three-stage Delphi process with two stakeholder groups. One surgical group including paediatric as well as thoracic surgeons, and a non-surgeon group including paediatric pulmonologists, intensive care and neonatal specialists. All participants will score outcome parameters according to their level of importance and the most important parameters will be determined by consensus. Ethics and dissemination Electronic informed consent will be obtained from all participants. Ethical approval is not required. After the core outcome set has been defined, we intend to design an international randomised controlled trial: the COllaborative Neonatal NEtwork for the first CPAM Trial, which will be aimed at determining the optimal management of patients with asymptomatic CPAM. The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
- Published
- 2021
31. Determinants of exercise capacity after arterial switch operation for transposition of the great arteries
- Author
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Gill Riley, Carlo Pace Napoleone, Graham Derrick, Alessandro Giardini, Sachin Khambadkone, Nicole Rizzo, Fernando M. Picchio, Nagarajan Muthialu, Giardini A, Khambadkone S, Rizzo N, Riley G, Napoleone CP, Muthialu N, Picchio FM, and Derrick G.
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Male ,medicine.medical_specialty ,Time Factors ,Heart disease ,Adolescent ,Transposition of Great Vessels ,Vital Capacity ,Physical exercise ,Risk Assessment ,Ventricular Outflow Obstruction ,Cohort Studies ,Young Adult ,Oxygen Consumption ,Postoperative Complications ,Heart Rate ,Predictive Value of Tests ,Internal medicine ,Heart rate ,Medicine ,Ventricular outflow tract ,Humans ,Cardiac Surgical Procedures ,Child ,Retrospective Studies ,Analysis of Variance ,Exercise Tolerance ,business.industry ,Incidence ,Age Factors ,Transposition of the great vessels ,medicine.disease ,medicine.anatomical_structure ,Treatment Outcome ,Great arteries ,Echocardiography ,Pulmonary valve ,Child, Preschool ,Circulatory system ,Multivariate Analysis ,Cardiology ,Exercise Test ,Female ,Cardiology and Cardiovascular Medicine ,business ,Blood Flow Velocity ,Follow-Up Studies - Abstract
Patients who undergo the arterial switch operation for transposition of the great arteries (TGA) are at risk of reduced exercise capacity, with most reports focusing on chronotropic incompetence as the cause. Residual right ventricular outflow tract (RVOT) obstruction is relatively common after the arterial switch operation, but its effect on exercise capacity is unknown. We studied 60 patients (44 males, age 13.3 +/- 3.4 years) who had undergone a neonatal arterial switch operation using the cardiopulmonary exercise test and transthoracic echocardiography. The peak exercise oxygen uptake (VO(2)), and heart rate were recorded and are expressed as the percentage of predicted values. The greatest velocity detected by echocardiography across the pulmonary valve, pulmonary trunk, or pulmonary branches was used in the analysis as an index of RVOT obstruction. The peak VO(2)% was 84 +/- 15%, and the peak heart rate percentage was 97 +/- 8%. Of the 60 patients, 29 had an abnormal peak VO(2)% (or =84%) and 3 (5%) had an abnormal peak heart rate percentage (or =85%). The maximal RVOT velocity was 2.3 +/- 0.6 m/s, and it correlated with the peak VO(2)% (r = -0.392, p = 0.004). On multivariate analysis, the presence of residual RVOT obstruction (p = 0.0007) was the only variable associated with a reduced peak VO(2)%. Patients with a RVOT maximal velocityor =2.5 m/s had a lower peak VO(2)% than those with lower velocities (p0.0001). No relation was found between age at testing and the peak VO(2)%. In conclusion, a reduced exercise capacity is relatively common in children and young adults who have undergone an arterial switch operation, but it does not decrease with age. The presence of residual RVOT obstruction seems to have an effect on exercise capacity.
- Published
- 2009
32. Novel approach for tracheal resection in Morquio a syndrome with end-stage critical airway obstruction: a UK case series.
- Author
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Kenth J, Maughan E, Butler CR, Gabrie J, Rouhani M, Silver B, Ogunbiyi OK, Wilkinson S, Nandi R, Walker R, Muthialu N, Jones S, Hewitt R, and Bruce IA
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- Humans, Female, Male, Adolescent, Child, Young Adult, United Kingdom, Adult, Mucopolysaccharidosis IV surgery, Airway Obstruction surgery, Trachea surgery
- Abstract
Background: Mucopolysaccharidosis (MPS) type IVA is a rare lysosomal storage disorder caused by aberrations of the N-acetyl-galactosamine-6-sulfatase (GALNS) enzyme. MPS IVA is associated with a wide gamut of respiratory and airway disorders that manifest in a continuum of severity. In individuals exhibiting severe phenotypic expression, terminal stages of the disease frequently culminate in life-threatening, critical airway obstruction. These manifestations of end-stage disease are engendered by an insidious progression of multi-level airway pathologies, comprising of tracheomalacia, stenosis, tortuosity and 'buckling'. Historically, the management of end-stage airway disease has predominantly leaned towards palliative modalities. However, contemporary literature has posited that the potential benefits of tracheal resection with aortopexy, performed under cardiopulmonary bypass (CPB), may offer a promising therapeutic option. In this context, we report on outcomes from patients undergoing a novel approach to tracheal resection that is combined with manubrial resection, leading to improved airway calibre, obviating the requisition for CPB., Results: In this study, seven patients with severe MPS IVA exhibited clinical symptoms and radiological evidence indicative of advanced airway obstruction. All patients had a tracheal resection with a partial upper manubriectomy via transcervical approach, which did not require CPB. The surgical cohort consisted of 5 females and 2 males, the median age was 16 years (range 11-19) and the median height was 105.6cm (range 96.4-113.4). Postoperatively, significant improvements were seen in forced expiratory volume in 1 second (FEV1), with a mean increase of 0.68 litres (95% CI: 0.45-0.91; SD: 0.20). Notably, other spirometry variables also showed meaningful improvements, providing evidence of positive treatment effects. Furthermore, there were no major long-term complications, and the procedure resulted in a significant enhancement in patient-reported domains using PedsQL (version 4.0)., Conclusions: This study represents the largest case series to date, on tracheal resection in patients with severe MPS IVA. Our findings demonstrate the effectiveness of the transcervical approach with partial manubriectomy for improving respiratory function and quality of life for individuals with advanced airway obstruction. Tracheal resection presents a promising treatment modality for severe cases of MPS IVA. Successful outcomes rely on meticulous multidisciplinary assessment, judicious decision-making, and appropriate timing of tracheal surgery. Further research and long-term follow-up studies are warranted to validate the long-term efficacy and safety of this approach., (© 2024. The Author(s).)
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- 2024
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33. Neosartorya udagawae pulmonary infection requiring a surgical treatment in a paediatric haematopoietic progenitor cell recipient.
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Tatarinova OS, Furness CL, Borman AM, Barber J, Muthialu N, and Ferreras-Antolin L
- Abstract
Neosartorya udagawae is a known cause of fungal infection in humans and animals. It is found to be more refractory to antifungal treatment in comparison to other Aspergillus species. With this report we present a case of proven invasive infection with Neosartorya udagawae in a child with chronic myeloid leukaemia after haematopoietic stem cell transplant. The patient received several lines of antifungal therapy including dual therapy appropriate to the antifungal susceptibility profile with progression of the invasive fungal disease requiring left lung upper lobe lobectomy. The case emphasizes the importance of early biopsy with antifungal susceptibility testing for targeted therapy and demonstrates the potential requirement for surgical management in addition to appropriate antifungal treatment., (© 2024 The Authors. Published by Elsevier B.V. on behalf of International Society for Human and Animal Mycology.)
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- 2024
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34. Pedicled pericardial patch in tracheal reconstruction in children - Novel technique to provide vascularized tissue for salvaging difficult airways.
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Beeman A, Ramaswamy M, Butler C, McIntyre D, Mann E, Hewitt R, Chippington S, and Muthialu N
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- Child, Humans, Infant, Trachea surgery, Trachea injuries, Tracheostomy, Reoperation, Bronchoscopy, Plastic Surgery Procedures
- Abstract
Background: Management of large central airway defects are often complex. Children who present with these defects have multiple co-morbidities or have had previous surgeries. Surgical options include various tissue cover for these defects without longer term benefits. Vascularized autologous pericardial patch offers a better solution to these defects by providing vascularity and potential for remodelling in future., Methods: 41 children (M:F of 24:17) were operated for large trachea-bronchial defects between January 2015 and August 2022. The median age of was 12 months with median weight of 8.9 kg (IQR 3.3 kg-17.7 kg) Causes leading to the central tracheal defect include failed repair of previous trachea-oesophageal fistula (TOF) (n = 21) and acquired fistula due to button battery injury (n = 11). Surgical repair consisted of autologous pedicled pericardial patch repair for the airway defect under cardiopulmonary bypass., Results: There were two operative deaths related to extensive sepsis and necrosis of reconstructed trachea. Four children had further reoperation with additional patch. Bronchoscopy was used as surveillance in all these children, with use of airway stents (biodegradable stent) in 9 children. The median ventilation time was 8 days, with tracheostomy being needed in 5 for long term support., Conclusions: Autologous pericardial patch is a versatile technique and can be used to salvage large tracheal defects when other method have failed or not feasible. Tracheomalacia at the site of repair could be managed with biodegradable stents. Vascularity and ciliary function of the patch still needs to be evaluated., Competing Interests: Declaration of competing interest We, all the authors, state that there is no conflict of interest in the work involved – and state this in title page of the manuscript as well., (Copyright © 2024. Published by Elsevier B.V.)
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- 2024
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35. Intracardiac migration of distal catheter-a rare complication of VP shunt insertion: case report and literature review.
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Hobbs E, Thompson DNP, Muthialu N, and Silva AHD
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- Humans, Child, Catheters adverse effects, Catheterization, Ventriculoperitoneal Shunt adverse effects, Ventriculoperitoneal Shunt methods, Foreign-Body Migration diagnostic imaging, Foreign-Body Migration etiology, Foreign-Body Migration surgery
- Abstract
Intracardiac migration is a rare complication of ventriculoperitoneal shunt insertion. Only 15 cases have been reported, 7 of which were paediatric cases, treated with techniques including interventional radiography, open thoracotomies and direct extraction through the initial shunt incision. The authors report the youngest case of intracardiac shunt migration complicated by significant coiling and knotting within the cardiac chambers and pulmonary vasculature. Migration likely began when the SVC was pierced during initial shunt placement and progressed due to negative intrathoracic pressure. Extrusion was achieved combining thoracoscopic endoscopy, interventional fluoroscopy screening and a posterolateral neck incision with uncoiling of the shunt via a Seldinger guide wire. This offered a minimally invasive solution with rapid post-operative recovery., (© 2023. The Author(s).)
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- 2024
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36. Open and Thoracoscopic Aortopexy for Airway Malacia in Children: 15 Year Single Centre Experience.
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Sutton L, Maughan E, Pianosi K, Jama G, Rouhani MJ, Hewitt R, Muthialu N, Butler C, and De Coppi P
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- Humans, Child, Infant, Retrospective Studies, Aorta surgery, Sternotomy adverse effects, Sternotomy methods, Tracheobronchomalacia surgery, Tracheomalacia surgery
- Abstract
Objectives: The objective was to report and analyse the characteristics and results of open aortopexy and thoracoscopic aortopexy for the treatment of airway malacia in a paediatric population., Methods: We report a retrospective consecutive case series of paediatric patients undergoing aortopexy for the treatment of airway malacia at a quaternary referral centre between December 2006 and January 2021. Outcome measures included days to extubation, continued need for non-invasive ventilation, further intervention in the form of tracheostomy and death., Results: 169 patients underwent aortopexy: 147 had open procedures (135 via median/limited median sternotomy and 12 thoracotomy) and 22 thoracoscopic. Mean follow up was 8.46 yrs (range 1-20 yrs). Most common site of airway malacia was the trachea (n = 106, 62.7 %), and 48 (28.4 %) had additional involvement at the bronchi with tracheobronchomalacia (TBM). 15 (8.9 %) had bronchomalacia (BM) only. Incidence of bronchial disease was lower in the thoracoscopic than open group (13.6 % vs 40.82 %; p < 0.0001). Mean time to extubation was 1.45 days, 2.59 days, 5.23 days in tracheomalacia, TBM and BM groups, respectively (p = 0.0047). Mean time to extubation was 1.35 days, 2 days, 3.67 days, and 5 days in patients with external vascular compression, TOF/OA, primary airway malacia, and laryngeal reconstruction, respectively (p = 0.0002). There were 21 deaths across the cohort, and all were in the open group. 71.4 % (n = 15) had bronchial involvement of their airway malacia., Conclusions: Open and thoracoscopic aortopexy are effective treatments for airway malacia in children. We have identified that involvement of the bronchi is a risk factor for adverse outcomes, and the optimum treatment for this patient cohort is still debatable., Level of Evidence: IV., Type of Study: Retrospective Study., (Copyright © 2023. Published by Elsevier Inc.)
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- 2024
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37. From 6-wk Lungs to 6 y: Increasing the Donor Pool for Pediatric Lung Transplantation.
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Purmessur R, Spencer H, Onwuka NB, Muthialu N, and Berman M
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- Female, Infant, Newborn, Humans, Child, Lung surgery, Tissue Donors, Tissue and Organ Procurement, Lung Transplantation, Persistent Fetal Circulation Syndrome
- Abstract
Background: Lung transplantation in the pediatric population is a challenge. With the donor pool being so small and lungs from young donors rare and precious, every organ available needs to be utilized to its best potential., Case: Here, we describe the case of a 6-wk-old donor of double lungs to a 5-mo-old baby girl diagnosed with alveolar capillary dysplasia with misalignment of the pulmonary veins. The recipient is doing very well, 6 y after the transplant, now following normal growth., Discussion: The challenges facing pediatric cardiothoracic transplantation in terms of organ supply and demand are enormous., Conclusions: In this article, we discuss some of the issues around the shortage of organs and alternatives to increase the organ donor pool., Competing Interests: The authors declare no funding or conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)
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- 2024
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38. Lung transplantation in an 18-month-old with donor specific antibodies - The use of intraoperative, targeted plasma exchange.
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Issitt R, Shetty P, Crook R, Cross N, Henwood S, Broadhead M, Spencer H, Aurora P, Gupta A, Kallon D, Fenton M, and Muthialu N
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- Humans, Infant, HLA Antigens, Tissue Donors, Transplantation, Homologous, Plasma Exchange, Lung Transplantation
- Abstract
Background: Sensitised patients undergoing Human Leukocyte Antigen-incompatible transplantation are at increased risk of hyperacute rejection and may be predisposed to antibody-mediated rejection, chronic lung allograft dysfunction and higher mortality., Case: We present a case of primary lung transplantation in the setting of late identification of donor specific antibodies treated with intraoperative target plasma exchange. The patient was treated with fresh human plasma to a final volume of 1.5 times the patient's systemic circulation. From a pre-transplant mean fluorescence intensity of 5002, donor-specific antibodies were undetectable following plasma exchange on single antigen bead assay., Conclusions: This method represents a potential desensitisation technique for use in the intraoperative period., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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- 2023
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39. Tracheal Resection for Critical Airway Obstruction in Morquio A Syndrome.
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Frauenfelder C, Maughan E, Kenth J, Nandi R, Jones S, Walker R, Walsh B, Muthialu N, Bruce I, Hewitt R, and Butler C
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Introduction: The primary cause of death in Morquio A syndrome (mucopolysaccharidosis (MPS) IVA) is airway obstruction, brought about by an inexorable and pathognomonic multilevel airway tortuosity, buckling, and obstruction. The relative pathophysiological contributions of an inherent cartilage processing defect versus a mismatch in longitudinal growth between the trachea and the thoracic cage are currently a subject of debate. Enzyme replacement therapy (ERT) and multidisciplinary management continue to improve life expectancy for Morquio A patients by slowing many of the multisystem pathological consequences of the disease but are not as effective at reversing established pathology. An urgent need has developed to consider alternatives to palliation of progressive tracheal obstruction to preserve and maintain these patients' hard-won good quality of life, as well as to facilitate spinal and other required surgery. Case Report . Following multidisciplinary discussion, transcervical tracheal resection with limited manubriectomy was successfully performed, without the need for cardiopulmonary bypass, in an adolescent male on ERT with the severe airway manifestations of Morquio A syndrome. His trachea was found to be under significant compressive forces at surgery. On histology, chondrocyte lacunae appeared enlarged, but intracellular lysosomal staining and extracellular glycosaminoglycan staining was comparable to control trachea. At 12 months, this has resulted in a significant improvement in respiratory and functional status, with corresponding enhancement to his quality of life., Conclusion: This addressing of tracheal/thoracic cage dimension mismatch represents a novel surgical treatment approach to an existing clinical paradigm and may be useful for other carefully selected individuals with MPS IVA. Further work is needed to better understand the role and optimal timing of tracheal resection within this patient cohort so as to individually balance considerable surgical and anaesthetic risks against the potential symptomatic and life expectancy benefits., Competing Interests: The authors declare that have no conflicts of interest., (Copyright © 2023 Claire Frauenfelder et al.)
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- 2023
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40. COllaborative Neonatal Network for the first European CPAM Trial (CONNECT): a study protocol for a randomised controlled trial.
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Kersten CM, Hermelijn SM, Dossche LWJ, Muthialu N, Losty PD, Schurink M, Rietman AB, Poley MJ, van Rosmalen J, Zanen-van den Adel TPL, Ciet P, von der Thüsen J, Brosens E, Ijsselstijn H, Tiddens HAWM, Wijnen RMH, and Schnater JM
- Subjects
- Child, Infant, Newborn, Humans, Child, Preschool, Lung, Diagnostic Imaging, Netherlands, Randomized Controlled Trials as Topic, Quality of Life, Respiratory Insufficiency etiology
- Abstract
Introduction: Consensus is lacking on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM). For future studies, the CONNECT consortium (the COllaborative Neonatal Network for the first European CPAM Trial)-an international collaboration of specialised caregivers-has established consensus on a core outcome set of outcome parameters concerning respiratory insufficiency, surgical complications, mass effect and multifocal disease. These outcome parameters have been incorporated in the CONNECT trial, a randomised controlled trial which, in order to develop evidence-based practice, aims to compare conservative and surgical management of patients with an asymptomatic CPAM., Methods and Analysis: Children are eligible for inclusion after the CPAM diagnosis has been confirmed on postnatal chest CT scan and they remain asymptomatic. On inclusion, children are randomised to receive either conservative or surgical management. Subsequently, children in both groups are enrolled into a standardised, 5-year follow-up programme with three visits, including a repeat chest CT scan at 2.5 years and a standardised exercise tolerance test at 5 years.The primary outcome is exercise tolerance at age 5 years, measured according to the Bruce treadmill protocol. Secondary outcome measures are molecular genetic diagnostics, validated questionnaires-on parental anxiety, quality of life and healthcare consumption-, repeated imaging and pulmonary morbidity during follow-up, as well as surgical complications and histopathology. This trial aims to end the continuous debate surrounding the optimal management of asymptomatic CPAM., Ethics and Dissemination: This study is being conducted in accordance with the Declaration of Helsinki. The Medical Ethics Review Board of Erasmus University Medical Centre Rotterdam, The Netherlands, has approved this protocol (MEC-2022-0441). Results will be disseminated through peer-reviewed scientific journals and conference presentations., Trial Registration Number: NCT05701514., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2023
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41. Impact of Tracheal Arborization and Lung Hypoplasia in Repair of Pulmonary Artery Sling in Combination With Long-Segment Tracheal Stenosis.
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Rudrappa SC, Beeman A, Ramaswamy M, Khambadkone S, Derrick G, Kostolny M, Calder A, and Muthialu N
- Subjects
- Child, Humans, Infant, Pulmonary Artery surgery, Pulmonary Artery abnormalities, Retrospective Studies, Treatment Outcome, Trachea surgery, Lung, Tracheal Stenosis surgery, Tracheal Stenosis congenital, Heart Defects, Congenital surgery, Bronchial Diseases, Vascular Malformations
- Abstract
Background: Reimplantation of the left pulmonary artery (LPA) and slide tracheoplasty has been our standard approach of care for patients with pulmonary artery sling (PAS) and tracheal stenosis. We present our experience, with emphasis on tracheal arborization and hypoplastic lungs; and their impact on long-term outcome of children with PAS and tracheal stenosis. Methods: It is a retrospective comparative study. Data were classified and analyzed based on the type of tracheobronchial arborization and normal versus hypoplastic lungs. Results: Seventy-five children operated between January 1994 and December 2019 (67 with normal lungs and 8 with lung hypoplasia/agenesis) were included. Patients with hypoplastic lungs had higher rates of preoperative ventilation (87.5%), postoperative ventilation (10 vs 8 days, P = .621), and mortality (50% vs 9%, P = .009) compared with those with normal lungs. Nineteen patients had tracheal bronchus (TB) variety and 30 patients had congenital long-segment tracheal stenosis (CLSTS) variety of tracheobronchial arborization. Endoscopic intervention was needed in 47.4% of patients with TB type and 60% with CLSTS type. CLSTS patients had higher rates of preoperative ventilation (60% vs 47.4%, P = .386), longer periods of postoperative ventilation (13 vs 6.5 days, P = .006), and ICU stay (15 vs 11 days, P = .714) compared with TB type. Conclusion: Surgical repair of PAS with tracheal stenosis has good long-term outcomes. All variations of tracheal anatomy can be managed with slide tracheoplasty. Persistence of airway problems requires intervention during follow-up as tracheal stenosis continues to be the Achilles heel.
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- 2023
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42. Anterior and posterior tracheopexy for severe tracheomalacia.
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Martens T, Schaballie H, Willekens J, Schelstraete P, Willems J, Muthialu N, and Desender L
- Abstract
Objectives: Congenital tracheomalacia can be the cause of respiratory failure in young children. Although the indication for surgical treatment has already been discussed vigorously, no clear guidelines about the modality are available., Methods: Through a sternotomy approach, a combination of posterior pexy and anterior tracheopexy using a tailored ringed polytetrafluoroethylene prosthesis is performed. Patient demographic characteristics, as well as operative details and postoperative outcomes, are included in the analysis., Results: Between 2018 and 2022, 9 children underwent the operation under review. All patients showed severe clinical symptoms of tracheomalacia, which was confirmed on bronchoscopy. The median age was 9 months. There was no operative mortality. Eight patients could be weaned from the ventilator. One patient died because of interstitial lung disease with bronchomalacia and concomitant severe cardiac disease. The longest follow-up now is 4 years, and shows overall excellent clinical results, without any reintervention., Conclusions: Surgical treatment of tracheomalacia through a combination of posterior and anterior pexy is feasible, with acceptable short- and midterm results., (© 2022 The Author(s).)
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- 2022
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43. Pediatric heart transplantation following donation after circulatory death, distant procurement, and ex-situ perfusion.
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Laurence C, Nachum E, Henwood S, Berman M, Large SR, Messer S, Kaul P, Baxter J, Quigley R, Osman M, Muthialu N, Davies B, Bohuta L, Kostolny M, Burch M, Fenton M, Andrews R, Thiruchelvam T, Hoskote A, and Simmonds J
- Subjects
- Adolescent, Adult, Child, Death, Graft Survival, Humans, Male, Perfusion methods, Retrospective Studies, Tissue Donors, Young Adult, Heart Transplantation, Tissue and Organ Procurement
- Abstract
Background: Limited availability of suitable donor hearts remains a challenge to pediatric heart transplantation, contributing to waitlist mortality. Controlled donation after circulatory death (DCD) has demonstrated success in adults. Early series of pediatric DCD heart transplantation using cold storage alone reported significant early mortality. We report a collaboration between 2 centers in the United Kingdom, combining expertise in adult DCD organ retrieval and pediatric transplantation., Methods: This retrospective series comprises 6 children (4 male, all >20 kg) undergoing DCD heart transplantation at Great Ormond Street Hospital between 1 February and 30 September 2020, following retrieval with direct procurement and perfusion using portable normothermic machine perfusion by the Royal Papworth Hospital service. Baseline characteristics and 1-year follow-up were compared to 9 children who underwent donation after brain death (DBD) transplants contemporaneously., Results: Mean DCD donor age was 24.67 years and mean DCD recipient age was 13.83 years. Mean functional warm ischemic time was 28.5 minutes and ex-situ heart perfusion time was 280 minutes. Median ICU and hospital stay were 9 and 17 days, respectively. All children survived to 1-year post-transplant. Survival and ICU and hospital stay were similar between the DCD and DBD cohorts. Performing DCD transplants resulted in a 66.7% increase in transplants for children >20 kg at GOSH during the study., Conclusions: This series demonstrates that DCD heart transplant can be performed safely with excellent short-term survival in children. Although the cohort is small, there was no significant difference in major outcomes compared to a DBD cohort., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)
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- 2022
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44. The Management of Asymptomatic Congenital Pulmonary Airway Malformation: Results of a European Delphi Survey.
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Kersten CM, Hermelijn SM, Mullassery D, Muthialu N, Cobanoglu N, Gartner S, Bagolan P, Mesas Burgos C, Sgrò A, Heyman S, Till H, Suominen J, Schurink M, Desender L, Losty P, Steyaert H, Terheggen-Lagro S, Metzelder M, Bonnard A, Sfeir R, Singh M, Yardley I, Rikkers-Mutsaerts NRVM, van der Ent CK, Qvist N, Cox DW, Peters R, Bannier MAGE, Wessel L, Proesmans M, Stanton M, Hannon E, Zampoli M, Morini F, Tiddens HAWM, Wijnen RMH, and Schnater JM
- Abstract
Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points) . The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.
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- 2022
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45. A cardiopulmonary bypass strategy to support a patient with vein of Galen malformation.
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Robertson A, Muthialu N, and Broadhead M
- Subjects
- Cardiopulmonary Bypass, Humans, Lung, Pulmonary Artery, Hypothermia, Induced, Vein of Galen Malformations complications, Vein of Galen Malformations surgery
- Abstract
We present a dissection of the patent ductus arteriosus and pulmonary artery for surgical repair utilising cardiopulmonary bypass in the setting of vein of Galen malformation. Several strategies were employed to attenuate the cerebral shunt including pH-stat, high cardiac index, restrictive venous drainage, continuous ventilation and deep hypothermic circulatory arrest. The patient recovered from surgery with no apparent neurological sequelae.
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- 2022
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46. Reappraisal of lung manifestations in the setting of Fontan circulation.
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Kanakis M, Martens T, Kostolny M, Petsios K, Giannopoulos N, and Muthialu N
- Subjects
- Heart Ventricles surgery, Humans, Lung surgery, Treatment Outcome, Fontan Procedure adverse effects, Heart Defects, Congenital surgery
- Abstract
Fontan circulation is a well-established palliation in patients with functional single ventricles. Absence of a sub-pulmonary pumping chamber creates a unique physiology in which blood flow is mainly guided by negative intrathoracic and elevated central venous pressures. Various pulmonary anatomic or pathophysiologic changes can jeopardize optimal Fontan circulation. Long-term survival of patients who have undergone the contemporary total cavopulmonary connection is satisfactory. Thorough literature review in conjunction with accumulated clinical experience can lead clinicians to extract conclusions regarding Fontan and lung interactions indicating the purpose of this review.
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- 2022
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47. Commentary: Slide tracheoplasty for congenital tracheal stenosis: Sliding by the missing pieces.
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Jegatheeswaran A and Muthialu N
- Subjects
- Constriction, Pathologic surgery, Humans, Infant, Retrospective Studies, Tracheal Stenosis congenital, Treatment Outcome, Plastic Surgery Procedures, Trachea abnormalities, Trachea surgery
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- 2022
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48. Risk Stratification of Slide Tracheoplasty for Pediatric Airway Stenosis.
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Beeman A, Ramaswamy M, Chippington S, Wallis C, Hayden M, Hewitt R, and Muthialu N
- Subjects
- Child, Constriction, Pathologic surgery, Humans, Infant, Retrospective Studies, Risk Assessment, Trachea abnormalities, Trachea surgery, Treatment Outcome, Plastic Surgery Procedures methods, Tracheal Stenosis congenital, Tracheal Stenosis surgery
- Abstract
Background: Slide tracheoplasty (STP) is the procedure of choice for treatment of long segmental congenital tracheal stenosis (LSCTS). Few studies predict factors leading to reintervention or mortality after STP. We analyzed a pediatric population to identify such factors and compared the outcome between 2 eras (1995-2012 and 2013-2017)., Methods: We analyzed 150 consecutive children who underwent STP from February 1995 to December 2017 in our hospital., Results: Median age and weight were 6.9 months and 6.1 kg. Average tracheal diameter of LSCTS was 2.3 mm. Tracheal stenosis extended into bronchus in 36 patients and distal malacia in 38. Median follow-up was 67 months; mortality was 12.7%. Balloon dilatation was required in 81 patients (54%), stents in 29 (19%), and reoperation in 4 (3%). The presence of malacia, preoperative extracorporeal membrane oxygenation, congenital anomalies, and single lung anatomy increased the risk for reintervention. Cox regression analysis revealed preoperative ventilation to be an independent factor predicting reintervention and single lung tracheal anatomy for mortality. In the current era (after 2013), survival improved from 88% to 97% and stent requirement was reduced from 25% to 11%., Conclusions: Slide tracheoplasty can be applied to various airway configurations seen in LSCTS. The requirement for reintervention such as balloon dilatation and stenting is high in the group requiring preoperative ventilation. Mortality is highest in the single lung anatomy group. Centralization of care allowed us to develop the multidisciplinary team expertise to manage this and other rare airway conditions with acceptable outcomes., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)
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- 2022
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49. Intrathoracic prosthesis in children in preventing post pneumonectomy syndrome: Its role in congenital single lung and post pneumonectomy situations.
- Author
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Quong WL, Bulstrode N, Beeman A, Ramaswamy M, Sivakumar B, Wallis C, Elliott MJ, and Muthialu N
- Subjects
- Child, Humans, Infant, Lung surgery, Retrospective Studies, Tissue Expansion Devices, Pneumonectomy adverse effects, Pneumonectomy methods, Quality of Life
- Abstract
Background: Postpneumopnectomy syndrome (PPS) is an extreme rotation and malposition of mediastinum causing dynamic and symptomatic central airway compression, arisingafter pneumonectomy or more uncommonly, in congenital single lung physiology. Affected patients present with severe respiratory compromise. Intrathoracic prosthesis placement is an evolving technique in children that mitigate the effects of thoracic dead space., Research Question: Assessment of clinical recovery and functional benefit in children undergoing placement of intrathoracic prosthesis following pneumonectomy or in congenital single lung situations., Study Design and Methods: Retrospective chart review of patients at Great Ormond Street Hospital from 2010 to 2020 was performed of all patients who underwent intrathoracic tissue expander placement. We summarize the outcomes of twenty four children, including those with both congenital and postpneumonectomy PPS etiology., Results: 24 Children who underwent placement of intrathoracic prosthesis for PPS in the study period with median age of 3.5 months and weight of 5 kg. Single lung etiology was congenital in 15 children (6 agenesis, 9 hypoplasia), and postpneumonectomy in 9 children. In seven patients, there was associated long segment tracheal stenosis. Pre operative ECMO was required in 2 patients, and pre operative ventilation was required in 12 patients all of whom had congenital single lung. Intrathoracic prosthesis placement was concurrent with intracardiac repair in 5 patients. There were no operative deaths, but one early postoperative death related to septicaemia. Median follow up was 75 months with 10 patients on continued respiratory support and 3 on nocturnal support with good quality of life. Two children needed reoperations for replacement of prosthesis., Conclusion: The use of tissue expanders is within the armamentarium of most plastic surgeons' practice. We also therefore advocate for a collaborative team approach involving Plastic and Cardiothoracic Surgery for surgical treatment of these patients. This multidisciplinary strategy has improved management of this rare and debilitating condition of PPS, thereby offering significant improvements in general progress of these sick children having single lung physiology. Evidence is still lacking on functional outcomes in these children and further work is necessary to prove that this is indeed achievable., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2021. Published by Elsevier Inc.)
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- 2022
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50. Lung Hypoplasia Associated With Ring-Sling Complex Is Usually Right-Sided.
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Ramaswamy M, Rudrappa S, Beeman A, Heatwole A, McIntosh N, McIntyre D, Hewitt R, and Muthialu N
- Subjects
- Constriction, Pathologic, Female, Humans, Infant, Lung abnormalities, Pulmonary Artery surgery, Retrospective Studies, Trachea abnormalities, Treatment Outcome, Abnormalities, Multiple surgery, Heart Defects, Congenital surgery, Lung Diseases complications, Respiratory System Abnormalities, Tracheal Stenosis congenital, Tracheal Stenosis surgery, Vascular Malformations complications
- Abstract
Background: Pulmonary artery sling (PAS) is usually associated with long-segment congenital tracheal stenosis (LSCTS). This combination of abnormalities can also be associated with lung hypoplasia abnormalities (hypoplasia, aplasia, or agenesis). This study analyzed the association of lung hypoplasia abnormalities with combined PAS and LSCTS and its influence on its surgical outcomes., Methods: All patients (0 to 18 years) who underwent surgical procedures for both PAS and LSCTS from 1995 to 2019 were analyzed retrospectively for mortality, ventilation days, and intensive care unit days by dividing them into those with normal lungs (group 1) and hypoplastic lungs (group 2)., Results: Included were 75 patients (30 girls [40%]), who were a median age of 5.7 months (interquartile range [IQR], 2.9-13.3 months), median weight of 5.5 kg (IQR, 4.1-7.9 kg), and had a median follow-up of 99.8 months (IQR, 54.5-152.0 months); of these, 8 patients (10.7%) had hypoplastic right lung, comprising hypoplasia in 7 (87.5%), aplasia in 1 (12.5%), and agenesis in 0 (0%). There was a significant difference in mortality (group 1, 9.0%; group 2, 50%; P = .007) but no significant difference in median ventilation days (group 1, 9.0; group 2, 9.0; P = .89) or in median intensive care unit days (group 1, 14.0; group 2, 11.5; P = .44)., Conclusions: Lung hypoplasia associated with PAS and LSCTS is usually right-sided. As a result of severe airway obstruction and single-lung physiology, there is a high requirement of preoperative cardiorespiratory support and a significant association with adverse surgical outcomes., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)
- Published
- 2022
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