Vol.:(0123456789) 1 3
Journal of Pediatric Endoscopic Surgery
https://doi.org/10.1007/s42804-019-00005-0
ORIGINAL RESEARCH
Is thoracoscopic lung‑sparing surgery in treatment of congenital
pulmonary airway malformation feasible?
M. Lima
1
· S. D’Antonio
1
· N. Di Salvo
1
· M. Maffi
1
· M. Libri
1
· T. Gargano
1
· G. Ruggeri
1
· V. D. Catania
1
Received: 14 December 2018 / Revised: 20 January 2019 / Accepted: 1 March 2019
© Springer Nature Singapore Pte Ltd 2019
Abstract
Purpose Lung-sparing strategies such as segmentectomy or atypical resection have been advocated for small congenital
pulmonary airway malformation (CPAM), even by thoracoscopy. The aims of our study were to evaluate surgical and clinical
outcome of patients undergoing lung-sparing surgery and to determine whether thoracoscopy is superior to thoracotomy.
Methods We conducted a retrospective review of patients who underwent lung-sparing resection for CPAM from 2004
to 2018. Demographic data, presenting symptoms, size and location of the CPAM, operative and post-operative data were
collected and analyzed according to surgical technique (thoracoscopy—TS, vs thoracotomy—TO). Data were compared
using Fisher’s exact test for qualitative values and Mann–Whitney test for quantitative values. P values less than 0.05 were
considered as statistically significant.
Results 167 lung-sparing surgery procedures were performed (segmentectomy n = 21 or atypical resection n = 146). 67
procedures were completed in TS. All histological examination revealed negative margins for residual CPAM. Patients in
the TS group were older and presented a higher weight compared to the TO group. TS was associated with shorter duration
of pleural drainage and shorter hospital stay. Rate of conversion was 35% (n = 29). Location of CPAM in the lower lobe and
CPAM size greater than 5 cm were predictor factors of conversion from TS to TO.
Conclusion Lung-sparing surgery for CPAM is a safe and feasible technique in pediatric patients. TS results in reduced post-
operative morbidity compared to TO and should be proposed as first surgical approach for selected patients.
Keywords Congenital pulmonary malformation · Congenital cystic adenomatoid malformation · Thoracoscopy · Atypical
resection · Segmentectomy
Introduction
Congenital pulmonary airway malformation (CPAM) is a
developmental malformation of the lower respiratory tract.
CPAMs include congenital adenomatoid cystic malforma-
tion (CCAM), bronchopulmonary sequestration, broncho-
genic and foregut cysts, bronchial atresia with subsequent
distal emphysematous progression/development, and con-
genital lobar emphysema [1, 2].
Although rare, it is the most common congenital lung
lesion. Data from large population registries suggest an
incidence of congenital lung cysts in the range of 1 per
8300–35,000 live births [3, 4]. Large-cyst subtypes account
for about 70% of CPAMs, or 2–8 per 100,000 live births.
The diagnosis of congenital pulmonary malformations has
increased significantly with the widespread use of prenatal
ultrasound screening and, more recently, improvement of
malformative characterization with the use of fetal magnetic
resonance imaging [5, 6]. Prenatal management is usually
conservative because very few of these lesions affect preg-
nancy or early postnatal course requiring prenatal or urgent
postnatal surgery [7–12]. The optimal postnatal manage-
ment of CPAM is still under debate. On one hand, there is
a wide acceptance that postnatal symptomatic lesions (i.e.,
symptoms related to infection, hyperinflation, pneumotho-
rax, left to right shunting, and pulmonary hypoplasia due
to large mass) require surgical resection [13–17]. On the
other hand, the management of asymptomatic lung lesions
is controversial because of concerns related to operative
morbidity and mortality along with uncertain outcome due
* V. D. Catania
vdcatania1985@gmail.com
1
Department of Pediatric Surgery, Sant’Orsola Hospital,
University of Bologna, Via Massarenti, 11, 40138 Bologna,
Italy