Kiarash Taghavi1, Sharman P Tan Tanny2, Alisa Hawley3, Jo-Anne Brooks3, John M Hutson4, Warwick J Teague2, Sebastian K King5, Michael Nightingale2. 1. Department of Paediatric Surgery, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia; Department of Paediatric Urology, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia. Electronic address: kiarash.taghavi@rch.org.au. 2. Department of Paediatric Surgery, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia; F. Douglas Stephens Surgical Research Group, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, VIC 3052, Australia; Department of Paediatrics, The University of Melbourne, Parkville, VIC 3010, Australia. 3. Department of Paediatric Surgery, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia; F. Douglas Stephens Surgical Research Group, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, VIC 3052, Australia; Department of Neonatal Medicine, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia. 4. Department of Paediatric Surgery, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia; Department of Paediatric Urology, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia; F. Douglas Stephens Surgical Research Group, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, VIC 3052, Australia; Department of Paediatrics, The University of Melbourne, Parkville, VIC 3010, Australia. 5. Department of Paediatric Surgery, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia; F. Douglas Stephens Surgical Research Group, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, VIC 3052, Australia; Department of Paediatrics, The University of Melbourne, Parkville, VIC 3010, Australia; Department of Gastroenterology and Clinical Nutrition, The Royal Children's Hospital, 50 Flemington Road, Parkville, VIC 3052, Australia.
Abstract
BACKGROUND: The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years. METHODS: The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948-2017). Data included presentation, diagnostic workup, surgical management, and outcomes. RESULTS: Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%. CONCLUSIONS: We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable. LEVEL OF EVIDENCE: IV. Crown
BACKGROUND: The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years. METHODS: The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948-2017). Data included presentation, diagnostic workup, surgical management, and outcomes. RESULTS: Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%. CONCLUSIONS: We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable. LEVEL OF EVIDENCE: IV. Crown