G Model

YPRRV-1116; No. of Pages 2 Paediatric Respiratory Reviews xxx (2016) xxx–xxx

Contents lists available at ScienceDirect

Paediatric Respiratory Reviews

Editorial

‘‘Oesophageal Atresia- We need to be seeing ya!’’

In recent decades, much has changed in our expectations for the outcomes of children born with oesophageal atresia and tracheooesophageal fistula [OA/TOF]. Things moved slowly, following the initial description of OA by Thomas Gibson in 1697 and, nearly 250 years later when the first successful, single-step surgery for OA by Cameron Haight was performed at the University of Michigan Hospital in 1941. This success was a long time coming and followed the work of surgeons Harry Richter in 1913, and subsequently Thomas Lanman, Robert Shaw N. Logan Leven and William Ladd who had operated on infants with OA and TOF who all died in the mid to late 1930s [1]. In the 75 years since Haight’s first success, reported survival has increased to be routinely >90% [2,3]. Further improvements in survival are seen in specialised paediatric hospitals, especially for those with the most common abnormality of OA with a distal TOF [3]. With improvements in surgical techniques, ventilatory strategies in the neonatal intensive care unit and the care of preterm infants, much of the early mortality is related to more common co-morbidities of associated congenital anomalies, low birthweight and pre-operative ventilatory requirements [4]. When we fast forward to 2016 we see that much has changed. Operations can be performed via thoracoscopy or thoracotomy, the neonatal unit stay is measured in weeks not months and better coordinated follow-up services for OA/TOF children are becoming more commonplace. Survival with good quality of life is now an expectation. With systematic reviews of children with OA/TOF has come the realisation that there are significant on-going respiratory and gastro-intestinal problems that are under-reported and these can be challenging to manage into adulthood [2,5]. Accordingly, in this mini symposium, we have gathered a series of papers reflecting where the main challenges lie in managing children with OA/TOF from birth to adolescence to serve as a useful resource for clinicians. The first paper in the mini-symposium deals with the perioperative management of infants with OA/TOF. Written by Hunt and colleagues from Melbourne, the paper emphasises the improved neonatal outcomes with delayed surgery, the presence of co-morbidities in approximately 60% of infants, and the ways in which the risks of longer term adverse outcomes are being mitigated with modern surgical and ventilatory strategies [6]. The need for a rigorous assessment of co-morbidities, with particular consideration of the VACTERL [Vertebral anomalies, ano-rectal malformations, cardiac abnormalities, TOF/OA, renal and limb abnormalities] which occurs in approximately one third of cases of OA/TOF [7] and CHARGE [Coloboma, heart defects, ano-rectal malformations, retarded growth, genital hypoplasia and ear abnormalities] syndromes as well as trisomies is emphasised.

One key determinant of the outcome is the surgical approach taken. In the second paper by Teague and Karpelowsky from Australia, a detailed explanation of the surgical considerations in planning and executing the OA/TOF surgery are presented for a non-surgical audience [8]. The question of bronchoscopy at the time of primary repair is addressed and the advantages of this timely additional investigation are considered, particularly in the light of the high prevalence of tracheomalacia and its implications for weaning from ventilatory support and ongoing respiratory management in infancy and early childhood. The key operative goals of restoration of oesophageal continuity and ligation of the tracheo-oesophageal fistula are explained, highlighting the key operative steps involved. Special consideration is given to the subset of infants with ‘Long gap’’ OA and the options of the ‘Foker’’ [traction] procedure is contrasted with the older methods of either delayed anastomosis after several weeks to months or oesophageal replacement with a stomach/intestinal ‘‘conduit’’ interposition with their known higher morbidity and mortality rates [9]. Anticipated surgical sequelae are outlined and more common problems such as strictures and dysmotility and an approach to management of these issues is discussed. A novel surgical approach to the common problem of tracheobronchomalacia is addressed by Bairdain, Zurakowski, Baird and Jennings from the United States [10]. A major challenge has been the lack of a standardised scoring system for the severity of airway malacia and its correlation with clinical events such as ‘‘apparent life threatening events [ALTEs]’’ or ‘‘dying spells’’ with airway collapse and hypoxaemia. In an interesting paper, the new term ‘‘excessive dynamic airway collapse [EDAC]’’ with a grading system of severity is proposed. The authors present their initial favourable experience with their grading system and its correlations with symptoms and improvements after surgical intervention, mainly with tracheo-bronchopexy, in thirty heterogeneous patients over a three year period. Feeding difficulties are uniformly experienced throughout life by people with OA/TOF. These can range in severity but the problems with establishing feeds, moving on to solids and maintaining a satisfactory weight gain are ubiquitous. These issues are addressed by Mahoney and Rosen from Boston in a paper that draws upon their considerable experience in this field [11]. Physiologic evidence is presented which incorporates consideration of oropharyngeal dysphagia and oesophageal dysphagia related to dysmotility, oesophageal outlet obstruction and oesophageal inflammation. The frequent presentation of such children with respiratory problems is established but often under-appreciated [12]. Pleasingly, Mahoney and Rosen provide a comprehensive review of the issues involved; suggest relevant

http://dx.doi.org/10.1016/j.prrv.2016.01.003 1526-0542/ß 2016 Published by Elsevier Ltd.

Please cite this article in press as: Fitzgerald DA, Karpelowsky J. ‘‘Oesophageal Atresia- We need to be seeing ya!’’. Paediatr. Respir. Rev. (2016), http://dx.doi.org/10.1016/j.prrv.2016.01.003

G Model

YPRRV-1116; No. of Pages 2 2

Editorial / Paediatric Respiratory Reviews xxx (2016) xxx–xxx

investigations and considered management strategies are outlined. Motility, digestive and nutritional problems are further reviewed by Gottrand, Michaud, Sfeir and Gottrand from the French National Reference centre for congenital malformations of the esophagus in Lille [13]. Further physiologic data regarding oesophageal dysmotility and its implications are presented with useful summaries of the frequency of the problems that have been reported in the literature. They draw upon their considerable experience and present a very practical summary of current management approaches centred upon a multi-disciplinary approach to the problems faced by children with OA/TOF. In the final paper in the mini-symposium, IJsselstijn and colleagues from Rotterdam present their vast experience in the multi-disciplinary follow-up of children with OA/TOF [14]. The challenges of optimising growth and development are well appreciated [15,16]. The needs are best met through a multidisciplinary approach to management which is well developed in Rotterdam. The authors highlight the need for greater evidence for assessing interventions for OA/TOF children, with a particular emphasis on developmental outcomes into the school years and long-term nutritional outcomes. Given that there are no standardised guidelines for the followup of children with OA/TOF, it is time to generate consensus in this field. This is particularly relevant for respiratory outcomes where the data is remarkably limited. Such information could include perinatal demographics, anaesthetic and operative data, neonatal ventilation requirements, growth parameters in infancy and early childhood, details of feeding problems, strictures, gastro-oesophageal reflux management, presence of tracheomalacia, frequency of chest infections, co-existing abnormalities [eg VACTERL and CHARGE syndromes], chest wall deformities, lung function, exercise capacity, neuro-cognitive assessments and educational outcomes. Obviously, based on local longitudinal care in multidisciplinary teams, international collaboration with well-maintained databases could provide reliable information for clinicians, ideally in the form of evidence based guidelines, to better inform families about the long term issues facing children with OA/TOF and how these problems can be optimally managed. References [1] Bae J, Widmann WD, Hardy MA. Cameron Haight: Pioneer in the Treatment of Esophageal Atresia. Current Surgery 2005;62:327–9. [2] Rintala R, Sistonen S, Pakarinen M. Outcome of esophageal atresia beyond childhood. Semin Pediatr Surg 2009;18:50–6. [3] Wang B, Tashiro J, Allan BJ, Sola JE, Parikh PP, Hogan AR, Neville HL, Perez EA. A nationwide analysis of clinical outcomes among newborns with esophageal

[4] [5]

[6]

[7]

[8] [9]

[10]

[11] [12] [13]

[14]

[15]

[16]

atresia and tracheoesophageal fistulas in the United States. J Surg Research 2014;190:604–12. Burge D, Shah K, Spark P, et al. Contemporary management and outcomes for infants born with oesophageal atresia. Br J Surg 2013;100:515–21. Gischler S, Mazer P, Duivenvoorden H, et al. Interdisciplinary structural followup of surgical newborns: a prospective evaluation. J Pediatr Surg 2009;44: 1382–9. Hunt RW, Perkins EJ, King S. Peri-operative management of neonates with oesophageal atresia and tracheo-oesophageal fistula. Paediatr Respir Rev 2016. http://dx.doi.org/10.1016/j.prrv.2016.01.002 Lautz TB, Mandelia A, Radhakrishnan J. VACTERL associations in children undergoing surgery for esophageal atresia and anorectal malformations: Implications for pediatric surgeons. Journal of Pediatric Surgery 2015;50: 1245–50. Teague WJ, Karpelowsky J. Surgical management of oesophageal atresia. Pediatr Respir Rev 2016. DOI: XXXXXXXXXXXXXXX. Deurloo J, Ekkelkamp S, Schoorl M, Heji H, Aronson D. Esophageal atresia: historical evolution of management and results in 371 patients. Ann Thorac Surg 2002;73:267–72. Bairdain S, Zurakowski D, Baird CW, Jennings RW. Surgical treatment of tracheo-bronchomalacia: a novel approach. Paediatr Respir Rev 2016. DOI XXXXXXXXXXXXXXXX.. Mahoney L, Rosen R. Feeding difficulties in children with esophageal atresia. Paediatr Respir Rev 2016. DOI XXXXXXXXXXXXXXXXXXXXXXXXXX. Beucher J, Wagnon J, Daniel V, et al. Long-term Evaluation of Respiratory Status after Eosophageal Atresia Repair. Pediatr Pulmonol 2013;48:188–94. Gottrand M, Michaud L, Sfeir R, Gottrand F. Motility, digestive and nutritional problems in esophageal atresia. Pediatr Respir Rev 2016. http://dx.doi.org/ 10.1016/j.prrv.2015.11.005 IJsselstijn H, Gischler SJ, Toussaint L, Spoel M, van der Cammen–van Zijp, Tibboel D. Growth and development after oesophageal atresia surgery: need for long-term multi-disciplinary follow-up. Paediatr Respir Rev 2016. http:// dx.doi.org/10.1016/j.prrv.2015.07.003 Pedersen RN, Markøw S, Kruse-Andersen S, Qvist N, Hansen TP, Gerke O, Nielsen RG, Rasmussen L, Husby S. Esophageal Atresia: Gastroesophageal functional follow-up in 5-15year old children. Journal of pediatric surgery 2013 Dec 31;48(12):2487–95. Olbers J, Gatzinsky V, Jo¨nsson L, Friberg LG, Abrahamsson K, Sille´n U, Gustafsson P. Physiological Studies at 7 Years of Age in Children Born with Esophageal Atresia. Eur J Pediatr Surg 2015;25:397–404.

Dominic A. Fitzgerald1,2,* Jonathan Karpelowsky2,3 1 Department of Respiratory Medicine, The Children’s Hospital at Westmead, Sydney, New South Wales, Australia 2 Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, New South Wales, Australia 3 Department of Surgery, The Children’s Hospital at Westmead, Sydney, New South Wales, Australia *Corresponding author. Department of Respiratory Medicine, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, New South Wales, Australia, 2145 Tel.: +61 2 9845 3397; fax: +61 2 9845 3396 E-mail address: Dominic.fi[email protected] (D.A. Fitzgerald).

Please cite this article in press as: Fitzgerald DA, Karpelowsky J. ‘‘Oesophageal Atresia- We need to be seeing ya!’’. Paediatr. Respir. Rev. (2016), http://dx.doi.org/10.1016/j.prrv.2016.01.003

"Oesophageal Atresia- We need to be seeing ya!".

"Oesophageal Atresia- We need to be seeing ya!". - PDF Download Free
566B Sizes 0 Downloads 8 Views