Surgical treatment of pulmonary sequestration in children

Abstract

Pulmonary sequestration is a complex malformation of the lungs, which is based on a violation of the development of their bronchopulmonary and vascular components. The multifactorial nature of morphological and functional disorders is clinically manifested by respiratory distress syndrome and heart failure with a threat of a critical outcome for a newborn child, despite the sufficient development of the respiratory system as a whole. Purpose - optimization of diagnosis and treatment of lung sequestration in children based on the study of clinical and diagnostic data, as well as the results of surgical treatment. Materials and methods. The study included 18 patients aged from 8 days to 18 years, including newborns - 6, children from 1 to 12 months - 5, from 1 to 3 years - 3, from 7 to 9 years - 2 and from 15 to 18 years - 2. Median age was 4.5 months. Research methods included evaluation of clinical symptoms, chest X-ray, contrast-enhanced CT, and angiography. Prenatal diagnosis was carried out using ultrasonography and fetal MRI. In all cases, open surgical treatment was used. Results. Intrapulmonary sequestration was diagnosed in 13 (72.2%) patients, extrapulmonary - in 5 (27.8%), p=0.082. Left-sided localization in 11 (61.1%) patients, right-sided localization in 7 (38.9%) patients, р=0.3. Associated malformations had 9 (50%) patients: polycystic (n=4) or hypoplasia (n=2) of the lung, diaphragmatic hernia (n=3), pericardial defect (n=1), thoracic dystopia of the kidney (n=2), Waardenburg syndrome (n=1), ventricular septal defect (n=1), innominate artery tracheal compression (n=1). Symptomatic course was noted in 15 (83.3%) patients, asymptomatic - in 3 (16.7%) cases (p=0.021). Main symptoms were as follows: pulmonary bleeding, hemophthisis, respiratory and hemodynamic disorders, signs of inflammation. Lobectomy (n=8), atypical segmental lung resection (n=4) and transection of aberrant vessels (n=1) were performed for intrapulmonary sequestration, and sequestrectomy (n=4) for extrapulmonary sequestration. In cases of associated diaphragmatic hernia (n=3), simultaneous diaphragmatic plasty was performed, including using a non-free pericardial flap (n=1) or a PTFE patch (n=1), and in case of innominate artery compression of the trachea, aortopexy (n=1). In 17 (94.4%) cases, a positive result of surgical treatment was noted. Postoperative complications (intrathoracic bleeding) and lethality were observed in 1 (5.6%) case. Patients were examined in the long-term period from 2 months to 30 years after surgery. Conclusions. Surgical correction of pulmonary sequestration is appropriate as the diagnosis is made, mainly in the neonatal period. Given the complexity of the pathology, especially in the presence of associated malformations, and the expediency of early correction in the neonatal period, thoracotomy access should be preferred as safer for sequential division of arterial and then venous vessels, and for atypical segmental lung resection or lobectomy, depending on peculiarities of sequester. The presence of associated defects requires simultaneous correction. In the case of associated diaphragmatic hernia, autopericardial defect plastics or a synthetic patch are appropriate as an alternative to simple suturing. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Keywords: congenital malformations of the lungs, lung sequestration, surgical treatment, children.