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. 2016 May;51(5):525-30.
doi: 10.1002/ppul.23255. Epub 2015 Jul 29.

Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations

Nigel J Hall  1   2 , Priscilla P L Chiu  1 , Jacob C Langer  1
Affiliations

Morbidity after elective resection of prenatally diagnosed asymptomatic congenital pulmonary airway malformations

Nigel J Hall et al. Pediatr Pulmonol. 2016 May.

Abstract

Background/aim: The optimal management of prenatally diagnosed asymptomatic congenital pulmonary airway malformations (CPAM) is controversial. Since there is a paucity of data relating to surgical risks in this specific population, we reviewed our experience to further inform this controversy.

Methods: Ethically approved 10 year (2004-2013) retrospective review. Patients were included only if the CPAM was diagnosed prenatally and remained asymptomatic. Indication for surgery was physician recommendation and/or parental choice.

Main results: Sixty patients were identified. Median age at surgery was 6.5 months (range 65 days to 9.6 years). Resections were performed thoracoscopically (n = 51, one conversion) or by thoracotomy (n = 9). Surgical time was 2.5 hr (43 min to 4.75 hr). A chest drain was used in 58/60 and remained in situ 53 hr (23-108). There were no intra-operative complications or blood transfusions. All patients were extubated at the end of the procedure with no re-intubations. Post-operative hospitalization was 73.4 hr (23.8 hr to 4.2 days). Overall, complications occurred in 14/60 (23%). Eleven were minor but three were major: tension pneumothorax associated with new presentation of a small previously undiagnosed diaphragmatic hernia 5 days following resection; aggressive fibromatosis of the chest wall in the region close to resection 2 years later; and near-fatal hypovolemic cardiac arrest due to massive haemorrhage from a feeding vessel on postoperative day 7. There were no deaths and no cases of pleuropulmonary blastoma.

Conclusion: Resection of prenatally diagnosed asymptomatic CPAM is associated with a significant risk of complications, which may be life threatening. These data contribute to a balanced discussion of risks and benefits for these children.

Keywords: bronchopulmonary sequestration; congenital cystic adenomatoid malformation; congenital malformations; lobectomy; pleuropulmonary blastoma; prenatal diagnosis.

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