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Eur J Pediatr Surg 2024; 34(01): 063-068
DOI: 10.1055/a-2130-2564
Original Article

Respiratory and Musculoskeletal Long-Term Outcomes after Surgical Resection of Congenital Cystic Adenomatoid Malformation of the Lung in Newborns, Infants, and Toddlers

Matteo Busti
1   Newborn Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
2   Department of Systems Medicine, University of “Tor Vergata,” Rome, Italy
,
Angelo Zarfati 
1   Newborn Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
2   Department of Systems Medicine, University of “Tor Vergata,” Rome, Italy
,
Laura Valfre
1   Newborn Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
,
Andrea Conforti
1   Newborn Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
,
Pietro Bagolan
1   Newborn Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
2   Department of Systems Medicine, University of “Tor Vergata,” Rome, Italy
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Abstract

Introduction The long-term outcomes of children who underwent surgery for congenital cystic adenomatoid malformation of the lung (CCAML) are not well documented, particularly regarding orthopaedic and respiratory follow-up (FU). The aim of this study was to assess the long-term pulmonary and orthopaedic outcomes of surgically treated CCAML in newborns, infants, and toddlers.

Materials and Methods Retrospective examination of prospectively recorded data of consecutive patients with CCAML who underwent surgery at our tertiary referral institution from January 2000 to December 2015 (newborns, infants, and toddlers). Clinical, radiological, and surgical data, as well as FU data were revised. A multidisciplinary team followed the patients after discharge at scheduled time points.

Results Seventy-seven patients were included. After surgery, patients were followed for a median of 8 years (range: 1–19 years) until they reached a median age of 8 years (range: 2–19 years). Thirty patients (39%) developed wheezing and 21 (27%) had lower respiratory tract infections (LRTIs) within 4 years of age. However, more than 50% of patients with respiratory symptoms underwent complete remission in the following 4 years. Thirty-one patients (40%) developed at least one minimal musculoskeletal deformity. Eighteen (23%) had scoliosis, 17 (22%) thoracic asymmetry, 10 (12%) pectus excavatum, and 5 (6%) winged scapula.

Conclusions Patients operated for CCAML had good overall outcomes despite pulmonary symptoms and musculoskeletal sequelae. Even though these issues are frequently paucisymptomatic, trying to use less-invasive procedures (such as minimally axillary open “muscle-sparing” thoracotomy or thoracoscopy) may reduce this burden. A structured multidisciplinary FU is required.

Keywords

congenital cystic adenomatoid malformation - lung malformations - follow-up - respiratory - orthopaedic - musculoskeletal


Publikationsverlauf

Eingereicht: 07. Mai 2023

Angenommen: 14. Juli 2023

Accepted Manuscript online:
17. Juli 2023

Artikel online veröffentlicht:
08. September 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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