Eur J Pediatr Surg 2017; 27(04): 324-329
DOI: 10.1055/s-0036-1593379
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Antenatally Detected Congenital Pulmonary Airway Malformations: The Oxford Experience

Hemanshoo Sudhir Thakkar
1   Department of Paediatric Surgery, John Radcliffe Hospital, Children's Hospital Headley Way, Oxford, Oxford, Oxfordshire, United Kingdom
,
Jonathan Durell
1   Department of Paediatric Surgery, John Radcliffe Hospital, Children's Hospital Headley Way, Oxford, Oxford, Oxfordshire, United Kingdom
,
Subhasis Chakraborty
2   Department of Paediatric Radiology, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
,
Bianca-Lea Tingle
3   Department of Anaesthetics, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
,
Arnwald Choi
3   Department of Anaesthetics, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
,
Darren J. Fowler
4   Department of Paediatric Pathology, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
,
Steve J. Gould
4   Department of Paediatric Pathology, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
,
Lawrence Impey
5   Department of Obstetrics and Fetal Medicine, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
,
Kokila Lakhoo
1   Department of Paediatric Surgery, John Radcliffe Hospital, Children's Hospital Headley Way, Oxford, Oxford, Oxfordshire, United Kingdom
› Author Affiliations
Further Information

Publication History

18 May 2016

15 August 2016

Publication Date:
10 October 2016 (online)

Abstract

Congenital airway pulmonary malformations are increasingly being diagnosed, but their management continues to remain controversial. Our approach has been to offer surgery to mitigate the risk of infection and possible malignancy. All patients routinely undergo a CT scan of the chest postnatally and once the diagnosis is confirmed, minimal access surgery is offered. Our anesthetists provide single-lung ventilation to enhance the operative view. We conducted a retrospective review over a 10-year period, during which 91 patients were prenatally suspected to have a cystic lung lesion. There were 88 live births of which 29 (33%) cases were initially managed conservatively based on CT findings. Five of these patients, however, became symptomatic needing surgery. A total of 64 (73%) patients underwent surgery with the most common lesions being congenital pulmonary airway malformations (CPAMs) (24), hybrid lesions (19), and pulmonary sequestrations (12). The median age at surgery was 5 months (1 day to 17 months). Using a minimal access approach, 41 (64%) cases were completed with 17 performed open from the onset. Open surgery was indicated in neonates who became symptomatic within the first few weeks of life as well as patients in respiratory distress that would not tolerate either single-lung ventilation or gas insufflation. There were six further conversions to open from minimal access surgery due to poor visualization or technical difficulties. One patient needed a perioperative blood transfusion and one patient had a more prolonged stay due to persistent air leak managed conservatively. Among asymptomatic patients, evidence of microscopic disease was seen, which included infection as well as two cases of tumors, one pleuropulmonary blastoma seen as part of a CPAM, and one rhabdomyomatous dysplasia seen in the CPAM component of a hybrid lesion. In our experience, excising asymptomatic lesions is safe with minimal complications. Single-lung ventilation in combination with thoracoscopy provides excellent vision. There is a risk of infection and a definite, albeit low, risk of malignancy, which may outweigh the benefits of conservative management.