Pulmonary sequestration in children. postnatal course, diagnosis and surgical treatment

Abstract

The aim — to optimize the diagnostic and treatment of pulmonary sequestration in children on the basis of clinical and diagnostic data and treatment results study.Materials and methods. The study included 14 patients aged 8 days to 17 years, 3 of which were newborns, 4 — from 1 to 12 months, 3 — from 1 to 3 years, 2 — 7­8 years, and 2 — 15 — 17 years old. There were 8 (57.1 %) boys and 6 (42.9 %) girls. Postnatal diagnosis was based on contrast computed tomography or angiography. In all cases the surgical treatment was performed. In 5 cases, the diagnosis of pulmonary sequestration was established intraoperatively during the surgery for mediastinal tumor, congenital diaphragmatic hernia or polycystic lung.Results and discussion. Ten (71.4 %) patients had intralobar sequestration and another 4 (28.6 %), extralobar one (p = 0.099), left­side localization was in 9 (64.3 %) cases, right­side in 5 (35.7 %). Associated malformations were found in 7 (50.0 %) patients and included polycystic lung (n = 3), lung hypoplasia (n = 2), diaphragmatic hernia (n = 2), pericardial defect (n = 1), thoracic dystopia of kidney (n = 1), Waardenburg syndrome (n = 1). Twelve (85.7 %) patients had a symptomatic disease presented with respiratory or hemodynamic disorders, signs of inflammation and hemophtysis. Lobectomy (n = 4), segmental lung resection (n = 4) or division of aberrant vessels (n = 2) were used in cases of intralobar disease depending on the extent of lesion while sequesterectomy (n = 4) was performed in extralobar sequestration. Simultaneous diaphragmoplasty, including that with use of bounded autopericardial flap (n = 1) was performed in 2 cases of associated diaphragmatic hernia. There were no postoperative complications and mortality. Late follow­up examination within the period from 4 to 22 years after the operation showed positive results in all 100 % of cases.Conclusions. Pulmonary sequestration, since the diagnosis is established, is subject to compulsory surgical correction, the effectiveness of which reaches 100 %. Correction of intralobar sequestration consists of lobectomy or segmental resection of the lungs, in the case of extralobar disease sequesterectomy is indicated. Associated congenital diaphragmatic hernia can be effectively corrected by diaphragmoplasty, including that with use of autopericardium.