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CC BY 4.0 · European J Pediatr Surg Rep. 2023; 11(01): e36-e39
DOI: 10.1055/a-2107-0409
Case Report

Combined Pre- and Postnatal Minimally Invasive Approach to a Complex Symptomatic Congenital Pulmonary Airway Malformation

Francesco Macchini
1   Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardia, Italy
,
Stefano Mazzoleni 
1   Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardia, Italy
,
Giacomo Cavallaro 
2   Department of Neonatal Intensive Care Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardia, Italy
,
Nicola Persico
3   Department of Obstetrics and Gynecology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardia, Italy
,
Irene Borzani
4   Department of Pediatric Radiology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardia, Italy
,
Ernesto Leva
1   Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Lombardia, Italy
› Author Affiliations Funding “This study was partially funded by the Italian Ministry of Health—Current research IRCCS.”
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Abstract

Congenital pulmonary airway malformation (CPAM) is a rare congenital lung lesion that usually remains asymptomatic during the fetal and neonatal period. However, it can occasionally cause prenatal cardiocirculatory failure and fetal hydrops, requiring a thoraco-amniotic shunt (TAS) placement. In other cases, it can also cause symptoms at birth (such as respiratory distress) and may require urgent surgical intervention. Thoracoscopic lobectomy for neonates is rarely reported. Here, we report a case of right macrocystic CPAM causing fetal hydrops at 27 weeks of gestation. The fetus was treated with a TAS placement that successfully resolved the hydrops. At 39 weeks of gestation, a male neonate was born (weight 2,850 g). The TAS spontaneously displaced during delivery, causing an open pneumothorax (PNX), initially treated with a drainage. His condition gradually worsened, requiring ventilatory support. Computed tomography (CT) scan showed different giant cysts in the context of the right lower lobe, left mediastinal shift, and compression of the rest of the lung. An urgent surgical management was required. A thoracoscopic right lower lobectomy was performed at 10 days of life (weight 2,840 g). The postoperative course was uneventful; the child remained totally asymptomatic and showed a good recovery. To the best of our knowledge, this is the first reported case of open iatrogenic PNX following TAS positioning and the second of neonatal thoracoscopic lobectomy in a newborn weighting less than 3 kg. The purpose of this report is to indicate that minimally invasive surgery is feasible, safe, and effective for the resection of CPAM, even in small newborns.

Keywords

congenital pulmonary airway malformation - thoraco-amniotic shunt - thoracoscopic lobectomy - neonate - case report


Publication History

Received: 13 April 2022

Accepted: 08 February 2023

Accepted Manuscript online:
07 June 2023

Article published online:
17 July 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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