Surgical Treatment of Congenital Bronchiectasis in Childhood
Authors: Sultanov Kh.X. , Aliyev M.M. , Aytmuratov I.R. 
Published: April 2024

Background and purpose: Congenital bronchiectasis (CB) is still an important problem for certain serious pulmonary infections. Surgical treatment of childhood CB has not been discussed extensively because of decline in prevalence and increased experience dealng with this disease. Although the incidence has declined over the past years in societies with high socioeconomic status, CB is still an important health problem in our country. This disease can be diffuse or focal. Diffuse CB is thought to occur owing to underlying systemic illness that causes abnormalities in airway clearance. For patients with bilateral disease, lung transplantation would be the only probable surgical approach. For patients with focal disease, conversely, the removal of the diseased lung had played the main role in the treatment of this disease. Recently, it has been shown that patients with an earlier and more localized disease can be successfully controlled by conservative approaches. It remains controversial as to which children would benefit from surgery and surgical points that may affect the outcome. Therefore, a retrospective study was conducted to evaluate the results of surgical treatment of CB in children. Patients and Methods: We retrospectively reviewed the medical records of 79 consecutive children who underwent surgery for CB in our clinic between 2006 and 2021. Patient demographics, clinical features, radiologic examinations, details of surgery including type of resection, operative morbidity, mortality and outcomes were analyxed. Results: Seventy-nine patients underwent 83 pulmonary resections during the study period. The mean ages at diagnosis of CB and at the time of surgery were 8.10 ± 4.10 years (range, 1 to 17 years) and 9.70 ± 4.20 years (range, 2 to 18 years), respectively. There were 44 males (55.7%) and 35 females (44.3%). The causes of bronchiectasis were congenital hypoplasy of bronchi (n = 31), secondary bronchiectasis was additional nonspecific pulmonary infection (n = 42) and foreign body aspiration (n = 6). Chest X-rays, bronchography and bronchoscopy (n = 79), chest HRCT (n = 61), angiopulmonography (n = 23) were used, and pulmonary function tests (n = 48) were performed. The types of resections were segmenectomy (26.5%), lobectomy (35.4%), and lobectomy with segmentectomy (17.8%), bilobectomy (8.9%) and pneumonectomy (11.4%). Four patients with bilateral bronchiectasis subsequently required second operation for the other lung. Two of those had undergone right lower lobectomy, by one child underwent right upper lobe, and another left lower lobectomies. One patient with incomplete resection subsequently required second operation for ongoing bronchiectasis underwent complementary right pneumonectomy. Morphological study (n=39) Postoperative complications were encountered in 6 patients (7.6%). In terms of long-term outcomes of treatment 78 (98.7%) patients were followed-up at least 6 months up to 14 years. The postoperative status of the patients as follows: “well” in - 49 patients (60.7%), “improved” in 23 patients (29.5%), “ orsened” in 6 patients (7.7%). And unfortunately two patients “died” (2.6%). Conclusions: The decision for bronchiectasis surgery should be made in cooperation with the chest diseases unit. Anatomic localization of the disease should be mapped clearly by radiologic and angiographic investigations. A radiologic and morphologic evidence of reversal of airway abnormality has been shown in cases of post-infectious bronchiectasis and congenital defects of bronchial wall. The morbidity and mortality rates of bronchiectasis surgery are within acceptable ranges. Most of the children benefit from surgery, especially when complete excision is accomplished. Segmentectomy and lobectomy are well tolerated in children without increase in morbidity and mortality. Therefore, resection of damaged part of the lung tissue may be preferred instead of removing high volume lung tissue.