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
