Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition

Abstract

In adults, congenital pulmonary malformations are candidates for surgery due to symptoms. A pre-natal diagnosis is simple and effective, and allows an early thoracoscopic surgical treatment. A retrospective study was performed to assess management in two different populations of adults and children to define the best strategy.SUBJECTS AND METHODS:Pulmonary malformations followed at the University Hospital from 2000 to 2012 were reviewed. Clinical history, malformation site, duration of hospitalisation, complications and pathology examinations were collected.RESULTS:A total of 52 cases (33 children, 19 adults) were identified. In children, 28 asymptomatic cases were diagnosed pre-natally and 5 during the neonatal period due to infections. Surgery was performed on the children between the ages of 2 and 6 months. Nineteen adults underwent surgery, 16 because of symptoms and 3 adults for anomalies mimicking tumours. The mean age within the adult group was 42.5 years. In children, there was one thoracotomy and 32 thoracoscopies, with 7 conversions for difficult exposure, dissection of vascular pedicles, bleeding or bronchial injury. In the adults, there were 15 thoracotomies and 4 thoracoscopies, with one conversion. Post-operative complications in the adults were twice as frequent than in children. The mean time of the children's hospitalisation was 7.75 days versus 7.16 days for the adults. Pathological examinations showed in the children: 7 sequestrations, 18 congenital cystic pulmonary malformations (CPAM), 8 CPAM associated sequestrations; in adults: 16 sequestrations, 3 intra-pulmonary cysts.CONCLUSION:Early thoracoscopic surgery allows pulmonary parenchyma conservation with pulmonary development, reduces respiratory and infectious complications, eliminates a false positive cancer diagnosis later in life and decreases risks of thoracic parietal deformation.

Outcome of Bronchopulmonary Sequestration with Massive Pleural Effusion after Intrafetal Vascular Laser Ablation

“…However, there are several arguments for early postnatal sequestrectomy, like improvement of compensatory lung growth, reduction of respiratory or infectious complications, and the potential risk for malignant transformation. In contrast, other studies do not recommend early surgery [23][24][25][26][27]. This heterogeneous approach might explain the different rate of sequestrectomy in different studies.…”

A Rare Case of Congenital Pulmonary Airway Malformation Associated with Intralobar Pulmonary Sequestration in an Adult - Case Report

“…Usually, in these cases the performance of a lobectomy is recommended. (5) The particularity of the presented case is given by the fact that this patient of 39 years old had no previous respiratory symptoms and no cases of type I CPAM associated with PS were cited before.…”

Lung Sequestration in an Octogenarian Male

“…Differential diagnoses of such lesions in the elderly include benign and malignant tumors, infections, and malformations such as pulmonary sequestration. 3 Primary lung cancer and metastases of various cancers and a variety of benign lung lesions such as neurogenic tumors need to be considered; PET/CT scan frequently helps in establishing diagnosis. 15 It should be noted that this type of malformation is diagnosed extremely rarely in individuals older than 60 years.…”

“…The intralobar type accounts for approximately 75% of cases; extralobar pulmonary sequestration accounts for 25% of cases, is located extrapulmonarily and forms its own visceral pleura. 3,4 The majority of these lesions are diagnosed in children; intrauterine diagnosis using prenatal ultrasound and magnet resonance imaging are available. 5 Intrauterine interventional procedures are an exciting new therapeutic option.…”