Congenital Lung Malformations: Unresolved Issues and Unanswered Questions

Abstract

Advances in prenatal and postnatal diagnosis, perioperative management, and postoperative care have dramatically increased the number of scientific reports on congenital thoracic malformations (CTM). Nearly all CTM are detected prior to birth, generally by antenatal ultrasound. After delivery, most infants do well and remain asymptomatic for a long time. However, complications may occur beyond infancy, including in adolescence and adulthood. Prenatal diagnosis is sometimes missed and detection may occur later, either by chance or because of unexplained recurrent or persistent respiratory symptoms or signs, with difficult implications for family counseling and substantial delay in surgical planning. Although landmark studies have been published, postnatal management of asymptomatic children is still controversial and needs a resolution. Our aim is to provide a focused overview on a number of unresolved issues arising from the lack of an evidence-based consensus on the management of patients with CTM. We summarized findings from current literature, with a particular emphasis on the vigorous controversies on the type and timing of diagnostic procedures, treatments and the still obscure relationship between CTM and malignancies, a matter of great concern for both families and physicians. We also present an algorithm for the assessment and follow-up of CTM detected either in the antenatal or postnatal period. A standardized approach across Europe, based on a multidisciplinary team, is urgently needed for achieving an evidence-based management protocol for CTM.

Surgical outcomes for pediatric congenital lung malformation: 13 years’ experience

“…Surgery in the form of lobectomy or lesser resection is generally safe and the patient can be extubated at the end of the maneuver, with a reasonable postoperative c o u r s e , o t h e r t h a n s o m e o c c a s i o n a l t r e a t a b l e complications. [26]…”

“…There remains a lack of an evidence-based consensus on the management of CLM patients. Annunziata et al [26] proposed an algorithm for the assessment and follow-up of congenital thoracic malformations (CTM) detected either in the antenatal or postnatal period (Fig. 2).…”

Congenital Pulmonary Adenomatoid Malformation: Unusual Revelation

“…• Persistent pulmonary interstitial emphysema: may appear as a focal cyst or regional small bubbly lesions [10] [12]. The therapeutic management of CPAM can be pharmacologic (treatment of complications like our case), surgical or simple follow-up, depending on the severity of the disease and its evolution.…”

“…Surgical resection is required if CPAM is symptomatic without improvement. The asymptomatic form has controversial managements according to different teams, most advocate elective resection particularly with the notion of pulmonary compensation in children that avoid risks of infection and malignant evolution [12] [13] [14].…”

Evaluation of Pulmonary Function in Children Undergoing Lung Resection Due to Congenital Lung Malformations

“…Congenital lung malformations are a large spectrum of disorders such as congenital pulmonary airway malformation (CPAM), congenital lobar emphysema (CLE), bronchogenic cyst (BC), bronchopulmonary sequestration (BPS) 1 . Surgical resection is the definitive treatment for these lesions with a controversy of elective surgery or conservative treatment in the management of asymptomatic patients and standard surgical treatment method is lobectomy [2][3][4] . After resection of abnormal congenital lesion, lung volume and function may be expected to improve.…”