Our Ladys Hospital for Sick Children

Crumlin, Ireland

Our Ladys Hospital for Sick Children

Crumlin, Ireland

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McElhinney D.B.,Stanford University | Quartermain M.D.,Wake forest University | Kenny D.,Our Ladys Hospital for Sick Children | Alboliras E.,Banner Childrens Specialists | Amin Z.,Georgia Regents University
Circulation | Year: 2016

Background - Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors. Methods and Results - All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age ≥17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size-ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion. Conclusions - In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical. © 2016 American Heart Association, Inc.


Byrne S.,Our Ladys Hospital for Sick Children | Kearns J.,Our Ladys Hospital for Sick Children | Carolan R.,Our Ladys Hospital for Sick Children | Mc Menamin J.,Our Ladys Hospital for Sick Children | And 2 more authors.
Epilepsia | Year: 2011

GLUT-1 deficiency syndrome (GLUT-1 DS) is a disorder of cerebral glucose transport associated with early infantile epilepsy and microcephaly. We report two boys who presented with refractory absence epilepsy associated with hypoglycorrhachia, both of whom have genetically confirmed GLUT-1 DS. We propose that these children serve to expand the phenotype of GLUT-1 DS and suggest that this condition should be considered as a cause of refractory absence seizures in childhood. © Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.


Priest J.R.,Childrens Hospitals and Clinics of Minnesota | Williams G.M.,Childrens Hospitals and Clinics of Minnesota | Mize W.A.,Childrens Hospitals and Clinics of Minnesota | Dehner L.P.,University of Washington | McDermott M.B.,Our Ladys Hospital for Sick Children
International Journal of Pediatric Otorhinolaryngology | Year: 2010

Objectives: Nasal chondromesenchymal hamartoma (NCMH) is an uncommon chondro-stromal tumor of the nasal cavity and paranasal sinuses in infancy and childhood. Pleuropulmonary blastoma (PPB) is also a rare malignancy of lung and pleura in childhood and is the sentinel disease of an important familial tumor and dysplasia syndrome. This study identified NCMH in PPB patients. Methods: The International PPB Registry collects cases of PPB using central pathology review and evaluation of clinical records. The Registry also evaluates PPB literature. Examples of NCMH occurring with PPB were identified. Clinical records, digital radiography and pathologic specimens of PPB-associated NCMH cases were analyzed. Results: Among approximately 625 cases of PPB, four children developed NCMH. These cases are among 28 total reported NCMH cases. NCMH presented with sinonasal congestion and visible polypoid nasal masses and were diagnosed from ages 7 to 15 years, similar to older reported NCMH cases. NCMH involved the nasal cavity, paranasal sinuses and upper nasopharynx, was bilateral in three children and locally recurrent in one. In two children, NCMH had the characteristic pathologic spectrum of immature nodules of cartilage surrounded by spindle cell stroma, whereas the other two NCMH displayed mature chondroid nodules and a less varied fibrous stroma. NCMH was not identified in family members with PPB. Conclusions: NCMH developing in four children with PPB indicates that NCMH is part of the heredofamilial disease complex associated with PPB. Otorhinolaryngologists and pediatric oncologists should be aware that these two rare conditions occur together and that affected patients may have a familial predisposition to childhood malignant and dysplastic disease. © 2010 Elsevier Ireland Ltd.


Breatnach C.,Our Ladys Hospital for Sick Children | Conlon N.P.,Our Ladys Hospital for Sick Children | Stack M.,Our Ladys Hospital for Sick Children | Healy M.,Our Ladys Hospital for Sick Children | O'Hare B.P.,Our Ladys Hospital for Sick Children
Pediatric Critical Care Medicine | Year: 2010

Objective: To compare neurally adjusted ventilatory assist ventilation with pressure-support ventilation. Design: Prospective, crossover comparison study. Setting: Tertiary care pediatric and neonatal intensive care unit. Patients: Sixteen ventilated infants and children: mean age = 9.7 months (range = 2 days-4 yrs) and mean weight = 6.2 kg (range = 2.4-13.7kg). Interventions: A modified nasogastric tube was inserted and correct positioning was confirmed. Patients were ventilated in pressure-support mode with a pneumatic trigger for a 30-min period and then in neurally adjusted ventilatory assist mode for up to 4 hrs. Measurements and Main Results: Data collected for comparison included activating trigger (neural vs. pneumatic), peak and mean airway pressures, expired minute and tidal volumes, heart rate, respiratory rate, pulse oximetry, end-tidal CO2 and arterial blood gases. Synchrony was improved in neurally adjusted ventilatory assist mode with 65% (±21%) of breaths triggered neurally vs. 35% pneumatically (p < .001) and 85% (±8%) of breaths cycled-off neurally vs. 15% pneumatically (p = .0001). The peak airway pressure in neurally adjusted ventilatory assist mode was significantly lower than in pressure-support mode with a 28% decrease in pressure after 30 mins (p = .003) and 32% decrease after 3 hrs (p < .001). Mean airway pressure was reduced by 11% at 30 mins (p = .13) and 9% at 3 hrs (p = .31) in neurally adjusted ventilatory assist mode although this did not reach statistical significance. Patient hemodynamics and gas exchange remained stable for the study period. No adverse patient events or device effects were noted. Conclusions: In a neonatal and pediatric intensive care unit population, ventilation in neurally adjusted ventilatory assist mode was associated with improved patient-ventilator synchrony and lower peak airway pressure when compared with pressure-support ventilation with a pneumatic trigger. Ventilating patients in this new mode seem to be safe and well tolerated. © 2010 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.


McNicholas F.,Our Ladys Hospital for Sick Children | Prior C.,Child and Adolescent Psychiatry | Bates G.,Huntercombe Hospital
Clinical Child Psychology and Psychiatry | Year: 2013

A case is presented of an 11-year-old girl with pervasive refusal syndrome (PRS) who ultimately recovered acutely and completely after an 18-month paediatric hospitalisation. There was an apparent absence of previously proposed important aetiological factors in PRS, such as family pathology and markedly traumatic or abusive experiences, and her recovery was sudden and complete. The authors consider the differential diagnoses of PRS paying particular attention to the possibility of a conversion disorder or catatonia, given the absence of PRS in the North American literature. Consideration of catatonia is important as it has a diagnostic test and responds rapidly to appropriate treatment, in contrast to conventional treatment for PRS and conversion disorder. © The Author(s) 2012.


Dhar R.,Our Ladys Hospital for Sick Children | Reardon W.,Our Ladys Hospital for Sick Children | McMahon C.J.,Our Ladys Hospital for Sick Children
Cardiology in the Young | Year: 2015

We report a baby girl with an antenatal diagnosis of biventricular non-compaction and complete heart block detected at 22 weeks' gestation. Postnatal echocardiography confirmed severe biventricular non-compaction hypertrophic cardiomyopathy, multiple muscular ventricular septal defects, and mild-moderate pulmonary valve stenosis. Skeletal muscle biopsy confirmed complex 1 mitochondrial respiratory chain deficiency. An epicardial VVI pacemaker was implanted on day 3 of life and revised at 7 years of age. She remains stable at 8 years of age following pacing and medical treatment with carvedilol, aspirin, co-enzyme Q10, and carnitine. This represents the first report of biventricular non-compaction hypertrophic phenotype in association with congenital complete heart block and complex 1 mitochondrial respiratory chain deficiency in a child. © Cambridge University Press 2014.


Campbell M.,Trinity College Dublin | Doyle S.,Our Ladys Hospital for Sick Children | Humphries P.,Trinity College Dublin
Expert Review of Clinical Immunology | Year: 2014

Recent evidence suggests that the pro-inflammatory cytokine IL-18 may have utility as an anti-angiogenic agent in the eye. Numerous laboratories, including our own have demonstrated the ability of murine IL-18 to prevent neovascularization in the retina, choroid and cornea in pathological scenarios. Here, we summarize the potential use of IL-18 as an immunotherapy for wet age-related macular degeneration treatment, describing past and recent findings pertaining to its biological function in the eye. © 2014 Informa UK, Ltd.


Kenny D.,Rush University Medical Center | Hijazi Z.M.,Rush University Medical Center | Walsh K.P.,Our Ladys Hospital for Sick Children
Catheterization and Cardiovascular Interventions | Year: 2011

We describe a case of percutaneous tricuspid valve implantation in a 20-year-old man with previous tricuspid valve replacement in the setting of pulmonary atresia with intact ventricular septum. He developed symptomatic endocarditis-induced tricuspid regurgitation of the tricuspid bioprosthesis. Tricuspid valvar competence was restored with implantation of a 26-mm Edwards SAPIEN valve. Copyright © 2011 Wiley-Liss, Inc.


Oosthuizen J.C.,Our Ladys Hospital for Sick Children | Burns P.,Our Ladys Hospital for Sick Children | Russell J.D.,Our Ladys Hospital for Sick Children
International Journal of Pediatric Otorhinolaryngology | Year: 2010

Objective: The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors' experience in managing these lesions as well as current literature on the subject. Study design and methods: A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998-2008) was performed. Data collected: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved. Results: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used. Conclusion: Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations. © 2010 Elsevier Ireland Ltd. All rights reserved.


Liddy S.,St Vincents University Hospital | Oslizlok P.,Our Ladys Hospital for Sick Children | Walsh K.P.,Our Ladys Hospital for Sick Children
Catheterization and Cardiovascular Interventions | Year: 2013

Objectives To compare the success and complication rates for the different devices and patent ductus arteriosus (PDA) types. Background There are numerous devices available for transcatheter closure of PDA but data comparing the success and complication rates of the newer devices are lacking. Methods A retrospective review was undertaken of all cases of transcatheter PDA closure at a tertiary referral center. Results Transcatheter PDA closure was achieved in 176 of 177 cases. Mean age, weight, and minimum ductal diameter were 3.3 years, 15.0 kg, and 2.5 mm, respectively. Devices used were Amplatzer Duct Occluder I (ADO I, 50.3%), Amplatzer Duct Occluder II (ADO II, 28.2%), Amplatzer Duct Occluder II Additional Sizes (ADO II AS, 5.1%), flipper coils (14.1%), and "other" devices (2.3%). Regarding Amplatzer devices, arterial delivery had a significantly shorter fluoroscopy time as compared to venous delivery (3.7 vs. 5.0 min, P = 0.0068), with no significant difference in outcomes. Immediate residual shunt rates were 7.9% (7/89) for ADO I, 4.1% (2/49) for ADO II, 11.1% (1/9) for ADO II AS, and 0% (0/25) for flipper coils. Aortic protrusion rates were 9% (8/89) for ADO I, 14.3% (7/49) for ADO II, and O% for both ADO II AS (0/9) and flipper coils (0/25). Conclusions All devices achieved excellent occlusion rates with low complication rates, regardless of PDA type. Device delivery via arterial access was associated with a shorter fluoroscopy time as compared to venous delivery, with no significant difference in outcomes. ADO II AS may be preferable to ADO II in the younger infant. © 2013 Wiley Periodicals, Inc.

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