Paediatric Surgery (Ukraine).2022.2(75):31-42; doi 10.15574/PS.2022.75.31
Krivchenya D. Yu.^1,2, Rudenko Ye. O.^1,2, Prytula V. P.^1,2, Hussaini S. F.^1,2, Shulzhyk I. I.^2
1Bogomolets National Medical University, Kyiv, Ukraine
²National Children Specialized Hospital «OHMATDYT», Kyiv, Ukraine

For citation: Krivchenya DYu, Rudenko YeO, Prytula VP, Hussaini SF, Shulzhyk II. (2022). Surgical treatment of pulmonary sequestration in children. Paediatric Surgery (Ukraine). 2(75): 31-42; doi 10.15574/PS.2022.75.31.
Article received: Jan 04, 2022. Accepted for publication: Apr 20, 2022.

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.
REFERENCES

1. Adzick NS, Harrison MR, Crombleholme TM et al. (1998). Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 179: 884-889. https://doi.org/10.1016/S0002-9378(98)70183-8

2. Albanese CT, Sydorak RM, Tsao K, Hanmin L. (2003). Thoracoscopic lobectomy for prenatally diagnosed lung lesions. J Pediatr Surg. 38 (4): 553-555. https://doi.org/10.1053/jpsu.2003.50120; PMid:12677564

3. Bratu I, Flageole H, Chen MF, Di Lorenzo M, Yazbeck S, Laberge JM. (2001). The multiple facets of pulmonary sequestration. J Pediatr Surg. 36 (5): 784-790. https://doi.org/10.1053/jpsu.2001.22961; PMid:11329590

4. Brown SC, Dch F, Laat MD, Proesmans M, De Boeck K, Van Raemdonck D, Louw J, Heying R, Cools B, Eyskens B, Gewillig M. (2012). Treatment strategies for pulmonary sequestration in childhood: resection, embolization, observation? Acta cardiologica. 67 (6): 629-634. https://doi.org/10.1080/AC.67.6.2184664; PMid:23393932

5. Chowdhury M, Samuel M, Ramsay A, Constantinoua J, McHugha K, Pierroa A. (2004). Spontaneous postnatal involution of intraabdominal pulmonary sequestration. J Pediatr Surg. 39 (8): 1273-1275. https://doi.org/10.1016/j.jpedsurg.2004.04.022; PMid:15300545

6. Clements BS, Warner JO. (1987). Pulmonary sequestration and related congenital bronchopulmonary-vascular malformations: nomenclature and classification based on anatomical and embryological considerations. Thorax. 42 (6): 401-408. https://doi.org/10.1136/thx.42.6.401; PMid:3660297 PMCid:PMC460770

7. Conran RM, Stocker JT. (1999). Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatric and Developmental Pathology. 2 (5): 454-463. https://doi.org/10.1007/s100249900149; PMid:10441623

8. Crushell E, Said A, Al-Hassann A, Walsh K. (2002). Occlusion of an aberrant artery to a pulmonary sequestration using a duct occlude. J Interv Cardiol. 15: 415-416. https://doi.org/10.1111/j.1540-8183.2002.tb01078.x; PMid:12440188

9. Curros F, Chigot V, Emond S, Sayegh N, Revillon Y, Scheinmann P, Lebourgeois M, Brunelle F. (2000). Role of embolisation in the treatment of bronchopulmonary sequestration. Pediatric radiology. 30 (11): 769-773. https://doi.org/10.1007/s002470000332; PMid:11100493

10. Durell J, Thakkar H, Gould S, Fowlerb D, Lakhoo K. (2016). Pathology of asymptomatic, prenatally diagnosed cystic lung malformations. J Pediatr Surg. 51 (2): 231-235. https://doi.org/10.1016/j.jpedsurg.2015.10.061; PMid:26653945

11. Felker RE, Tonkin ILD. (1990). Imaging in Pulmonary Sequestration. Am J Roentgenol. 154 (2): 241-249. https://doi.org/10.2214/ajr.154.2.2105007; PMid:2105007

12. Genç O, Gürkök S, Dakak M, Gözübüyük A, Özkan M, Çaylaket H. (2006). Pulmonary sequestration and surgical treatment. Asian Cardiovascular and Thoracic Annals. 14 (1): 3-6. https://doi.org/10.1177/021849230601400102; PMid:16432109

13. Glüer S, Scharf A, Ure B. (2002). Thoracoscopic resection of extralobar sequestration in a neonate. J Pediatr Surg. 37 (11): 1629-1631. https://doi.org/10.1053/jpsu.2002.36199; PMid:12407554

14. Huang D, Habuding A, Yuan M, Yang G, Cheng K, Luo D, Xu C. (2021). The clinical management of extralobar pulmonary sequestration in children. Pediatric Pulmonology. 56 (7): 2322-2327. https://doi.org/10.1002/ppul.25433; PMid:33930250

15. Imakiire T, Obuchi T, Hamanaka W. (2011). Surgical treatment for pulmonary sequestration. Nippon Kokyuki Geka Gakkai Zasshi. 25 (6): 595-599. https://doi.org/10.2995/jacsurg.25.595

16. Ito F, Asaoka M, Nagai N, Hayakawa F. (2003). Upper thoracic extralobar pulmonary sequestration with anomalous blood supply from the subclavian artery. J Pediatr Surg. 38 (4): 626-628. https://doi.org/10.1053/jpsu.2003.50138; PMid:12677582

17. Kestenholz PB, Schneiter D, Hillinger S, Lardinois D, Wederet W (2006). Thoracoscopic treatment of pulmonary sequestration. Eur J Cardio Thorac Surg. 29 (5): 815-818. https://doi.org/10.1016/j.ejcts.2006.02.018; PMid:16600607

18. Krivchenya DU, Rudenko EO, Prytula VP, Benzar IM, Shulzhik II, Goncharenko AV. (2016). Method of diaphragmoplasty using bound pericardial patch in children. Patent 113225 Ukraine, IPC А 61 В 17/00. Bogomolets National Medical University. No 201605548. Bulletin 2.

19. Krivchenya, DY, Rudenko YO. (2018). Pulmonary sequestration in children. Postnatal course, diagnosis and surgical treatment. Surgery of Ukraine. 1 (65): 80-88. https://doi.org/10.30978/SU2018180

20. Lababidi Z, Dyke Ii PC. (2003). Angiographic demonstration of spontaneous occlusion of systemic arterial supply in pulmonary sequestration. Pediatr. Cardiol. 24 (4): 406-408. https://doi.org/10.1007/s00246-002-0345-7; PMid:12360384

21. Laberge JM, Puligandla P, Flageole H. (2005). Asymptomatic congenital lung malformations. Seminars in pediatric surgery. 14 (1): 16-33.; https://doi.org/10.1053/j.sempedsurg.2004.10.022; PMid:15770585

22. Laberge JM, Puligandla P. (2008). Congenital malformations of the lungs and airways. In Pediatric Respiratory Medicine. Taussig LM, Landau LI, eds. Philadelphia: Mosby: 907-942. https://doi.org/10.1016/B978-032304048-8.50068-2

23. Lo HP, Oldham KT. (2003). Congenital malformations of the lung. In Newborn Surgery. London: Arnold: 295-307. https://doi.org/10.1201/b13479-34

24. Makarov AV, Sokur PP, Kutsenok LG. (2007). Pulmonary sequestration blood supply. Surgery of children's age. 4 (1,14): 13-19.

25. Sade RM, Clouse M, Ellis FH. (1974). The spectrum of pulmonary sequestration. Ann Thorac Surg. 18 (6): 644-658. https://doi.org/10.1016/S0003-4975(10)64417-7

26. Tashtoush B, Memarpour R, Gonzalez J, Gleason JB, Hadeet A. (2015). Pulmonary sequestration: a 29 patient case series and review. Journal of clinical and diagnostic research: JCDR. 9 (12): AC05-AC08. https://doi.org/10.7860/JCDR/2015/16004.7006; PMid:26816878 PMCid:PMC4717683

27. Tokel K, Boyvat F, Varan B. (2000). Coil embolization of pulmonary sequestration in two infants: a safe alternative to surgery. AJR Am J Roentgenol. 175: 993-995. https://doi.org/10.2214/ajr.175.4.1750993; PMid:11000150

28. Trabalza Marinucci B, Maurizi G, Vanni C, Cardillo, G, Poggi C, Pardi V, Inserra A, Rendina EA. (2020). Surgical treatment of pulmonary sequestration in adults and children: long-term results. Interactive cardiovascular and thoracic surgery. 31 (1): 71-77. https://doi.org/10.1093/icvts/ivaa054; PMid:32300793

29. Yong W, Fan L. (2011). Pulmonary sequestration: a retrospective analysis of 2625 cases in China. Eur. J. Cardiothorac. Surg. 40 (1): e39-e42. https://doi.org/10.1016/j.ejcts.2011.01.080; PMid:21459605

30. Yucel O, Gurkok S, Gozubuyuk A, Caylak H, Sapmaz E, Kavakli K, Dakak M, Gencet O. (2008). Diagnosis and surgical treatment of pulmonary sequestration. The Thoracic and cardiovascular surgeon. 56 (3): 154-157. https://doi.org/10.1055/s-2007-965572; PMid:18365974