A LONG FORGOTTEN PENNY - A Tale With a Rare Happy Ending- Dr Christina Harry Royal Hospital for Sick Children, Glasgow UK

A LONG FORGOTTEN PENNY -A Tale With a Rare Happy Ending- Harry C1, Spenceley 1, Kusnirikova Z2, Davies K2, Patel Y3, On Wei J3, Ghent R4, Danton M5 1. Paediatric Intensive Care Unit, Royal Hospital for Sick Children, Glasgow, UK 2. Department of Anaesthesia, Aberdeen Royal Infirmary, UK 3. Department of Paediatric Surgery, Royal Aberdeen Children’s Hospital, UK 4. Department of Anaesthesia, Royal Hospital for Sick Children, Glasgow, UK 5. Department of Paediatric Cardiac Surgery, Royal Hospital for Sick Children, UK

A tale of two cities . . .

Upper GI Endoscopy

Case Presentation Further admissions with haematemsis 2 further presentations Further endoscopy Gastrostomy inserted Discussed with Glasgow Royal Hospital for Sick Children Arranged for CT angiogram & retrieval to Glasgow

3rd April 2014 Life threatening haemorrhage following intubation for CT angiogram Major haemorrhage protocol Sengstaken-Blakemore tube Adult cardiothoracics from adjacent hospital performed left lateral thoracotomy

On arrival to Aberdeen . . . Airway Breathing Circulation Intact & secured Breathing Pressures of 32/5 & FiO2 of 1.0 Circulation Adrenaline infusion of 0.5mcg/kg/min Heart rate 170; BP 90mmHg

Options?

Theatre Team Aberdeen Team Glasgow Team Theatre staff Paediatric anaesthetist Adult perfusionist General paediatric surgical consultant Glasgow Team Paediatric cardiothoracic surgical consultant Paediatric cardiac anaesthetist Paediatric perfusionist Paediatric theatre nurses

Complications Failed extubations Renal failure Malaena Upper GI scope showed no indentation No further bleeding No evidence of neurological impairment No evidence of mediastinitis

Nasogastric Tubes Associated with Aorto-oesophageal fistulas with vascular anomalies Author Vascular Anomaly Age of Admission Day of GI haemorrhage Survival Arcineigas et al. 1979 Double aortic arch 4 years 19th day post surgery Survived McKeating et al. USA, 1990 3 months 17th day of hospitalisation Died Yahagi et al. Japan, 1992 9 days 8 day post operative Heck et al. USA, 1993 6 weeks 3 weeks 14th day post surgery 7th week of hospitalisation Sigalet et al. Canada, 1994 59 days post surgery ? Othersen et al. USA, 1996 5 weeks 2 months 10 days post aortopexy 48th day of hospitalisation Angelini et al. Italy, 2002 Day 23 post surgery Chaikitpinyo et al. Thailand, 2004 14th day of hospitalisation D’Angelis et al. Argentina, 2006 38 days post surgery Van Woerkum et al. Netherlands, 2006 9 weeks 22 days post surgery

References Arciniegas E et al. ‘Surgical management of congenital vascular rings.’ J Thorac Cardiovasc Surg. 1979. 5:721-7 McKeating et al. ‘Fatal Aortoesophageal Fistula Due to Double Aortic Arch: An Unusual Complication of Prolonged Nasogastric Intubation.’ Journal of Paediatric Surgery. 1990. 25(12):1298-1300 Yahagi et al. ‘Double aortic arch presenting as massive haematemesis after removal of a nasogastric tube.’ Canadian Journal of Anaesthesia. 1992. 39:894 Heck et al. ‘Esophageal-Aortic Erosion Associated with Double Aortic-Arch and Tracheomalacia.’ Texas Heart Institute Journal. 1993; 20:126-9 Sigalet et al. ‘Aortoesophageal Fistula: Congenital and Acquired Causes.’ Journal of Pediatric Surgery. 1994; 29(9):1212-4 Othersen et al. ‘Aortoesophageal fistula and double aortic arch: two important points in management.’ Journal Paediatric Surgery. 1996; 31(4):594-5 Angelini et al. ‘Fatal aortoesophageal fistula in two cases of tight vascular ring.’ Cardiology in the Young. 2002; 12:172-6 Chaikitpinyo et al.’ Aortoesophageal fistula: a life threatening cause of upper GI haemorrhage in double aortic arch. A Case Report.’ J. Med Assoc of Thailand. 2004; 87(8):992-5 D’Angelis et al.’ Successful surgical treatment of a 4-month infant after exsanguination for aortoesophageal fistula.’ Journal of Pediatric Surgery. 2006; 41:848-9 Van Woerkum et al.’ Aortoesophageal fistula due to double aortic arch and prolonged nasogastric intubation: case report and review of the literature.’ Eur J Pediatrics. 2006; 165:660-1