Intestinal and mesenterial cystic lymphatic malformations in children: clinical course features and treatment tactics
DOI:
https://doi.org/10.15574/PS.2023.78.79Keywords:
abdominal cysts, lymphatic malformations, intestine, laparoscopy, laparotomy, childrenAbstract
Lymphatic malformations (LMs) - are congenital vascular malformations caused by abnormal embryologic development of the lymphatic system with variable clinical manifestation. Abdominal LMs account approximately 5% of all LMs. The main treatment option for abdominal LMs is surgical.
Purpose - to analyze our experience in intestinal and omental LMs diagnosis and treatment, to reveal factors that could influence the complications occurrence risks, that could improve abdominal LMs treatment results in children.
Materials and methods. Among 240 pediatric patients, who underwent cystic LMs treatment in a period from December 2012 to July 2022, 43 (19.1%) were diagnosed abdominal and retroperitoneal LMs. 18 (41.8%) patients with intestinal and mesentery affection were included into the study group. Median follow up was 20.7 (95% CI: 12-27) months.
Results. Male patients predominated in the study group. Median age at admission was 2.5 (95% CI: 0.75-5) years. 12 (66.6%) had an emergency admission. In 7 (38.8%) children LMs were complicated by intestinal obstruction (n=5; 71.4%), intracystic bleeding and LM cyst infection were observed in n=1, 14.3%) cases both. Surgical treatment was applied in 16 (88.8%) patients, among which 7 (43.7%) underwent laparoscopy. In 4 (25%) cases conversion to laparotomy took place, 5 (31.3%) underwent laparotomic surgeries. No recurrences after surgical treatment were observed. Systemic therapy with mTOR-inhibitors was prescribed in a child with symptomatic total mesentery affection, observational tactics was chosen for asymptomatic child.
Conclusions. Intestinal and mesenterial LMs can be asymptomatic or manifest complications, which were observed in 7 (38.8%) children of our group. Macrocystic intestinal LMs are of higher risks of complications development (р=0.0236). Surgical treatment is effective for local intestinal and mesentery affection, excellent treatment result was achieved in 94.1% of patients. In cases with total mesentery affection mTOR-inhibitors systemic therapy is the treatment of choice.
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.
References
Benzar IM, Levytskyi AF, Diehtiarova DS, Godik OS, Dubrovin OG. (2022). Treatment of lymphatic malformations in children: 10 years of experience. Paediatric Surgery (Ukraine). 2 (75): 5-14. https://doi.org/10.15574/PS.2022.75.5
Dasgupta R, Fishman SJ. (2014). ISSVA classification. Seminars in Pediatric Surgery. 23 (4): 158-161. https://doi.org/10.1053/j.sempedsurg.2014.06.016; PMid:25241091
Dubois J, Thomas-Chaussé F, Soulez G. (2019). Common (Cystic) Lymphatic Malformations: Current Knowledge and Management. Techniques in Vascular and Interventional Radiology. 22 (4): 100631. https://doi.org/10.1016/j.tvir.2019.100631; PMid:31864533
Elbaaly H, Piché N, Rypens F, Kleiber N, Lapierre C, Dubois J. (2021). Intra-abdominal lymphatic malformation management in light of the updated International Society for the Study of Vascular Anomalies classification. Pediatric Radiology. 51 (5): 760-772. https://doi.org/10.1007/s00247-020-04930-8; PMid:33559726
Francavilla ML, White CL, Oliveri B, Lee EY, Restrepo R. (2017). Intraabdominal Lymphatic Malformations: Pearls and Pitfalls of Diagnosis and Differential Diagnoses in Pediatric Patients. American Journal of Roentgenology. 208 (3): 637-649. https://doi.org/10.2214/AJR.16.17008; PMid:28004970
Gafar A, Batikhe M. (2018). Surgical management for mesenteric cysts in pediatric patients: a single center experience. International Surgery Journal. 5 (4): 1217-1221. https://doi.org/10.18203/2349-2902.isj20181027
Gastineau S, Paolella G, Talbotec C, Verkarre V, Sauvat F, Goulet O et al. (2018). Successful Use of Sirolimus in Children with Large Mesenteric Lymphatic Lesions. Journal of Pediatric Gastroenterology and Nutrition. 66 (1): e24-e25. https://doi.org/10.1097/MPG.0000000000001703; PMid:28777125
Jayasundara J, Perera E, Chandu de Silva M, Pathirana A. (2017). Lymphangioma of the jejunal mesentery and jejunal polyps presenting as an acute abdomen in a teenager. The Annals of The Royal College of Surgeons of England. 99 (3): e108-e109. https://doi.org/10.1308/rcsann.2017.0012; PMid:28252346 PMCid:PMC5450291
Jiao-ling L, Hai-Ying W, Wei Z, Jin-Rong L, Kun-Shan C, Qian F. (2018). Treatment and prognosis of fetal lymphangioma. European Journal of Obstetrics & Gynecology and Reproductive Biology. 231: 274-279. https://doi.org/10.1016/j.ejogrb.2018.10.031; PMid:30482553
Kim S-H, Kim H-Y, Lee C, Min HS, Jung S-E. (2016). Clinical features of mesenteric lymphatic malformation in children. Journal of Pediatric Surgery. 51 (4): 582-587. https://doi.org/10.1016/j.jpedsurg.2015.11.021; PMid:27106580
Lee S, Park J. (2018). Abdominal Lymphatic Malformation in Children. Advances in Pediatric Surgery. 24 (2): 60. https://doi.org/10.13029/aps.2018.24.2.60
Liu Q, Fu J, Yu Q, Gong W, Li P, Guo X. (2022). Laparoscopic surgery of intra-abdominal lymphatic malformation in children. Experimental and Therapeutic Medicine. 24 (3): 581. https://doi.org/10.3892/etm.2022.11519; PMid:35949324 PMCid:PMC9353511
Mäkinen T, Boon LM, Vikkula M, Alitalo K. (2021). Lymphatic Malformations: Genetics, Mechanisms and Therapeutic Strategies. Circulation research. 129 (1): 136-154. https://doi.org/10.1161/CIRCRESAHA.121.318142; PMid:34166072
Makni A, Chebbi F, Fetirich F, Ksantini R, Bedioui H, Jouini M et al. (2012). Surgical Management of Intra-Abdominal Cystic Lymphangioma. Report of 20 Cases. World Journal of Surgery. 36 (5): 1037-1043. https://doi.org/10.1007/s00268-012-1515-2; PMid:22358782
Méndez-Gallart R, Bautista A, Estévez E, Rodríguez-Barca P. (2011). Abdominal Cystic Lymphangiomas in Pediatrics: Surgical Approach and Outcomes. Acta Chirurgica Belgica. 111 (6): 374-377. https://doi.org/10.1080/00015458.2011.11680776; PMid:22299324
Rollins M, Feola G, Arnold R, Barnhart D, Scaife E, Russell K. (2013). Sclerotherapy for Intra-abdominal Lymphatic Malformations in Children. European Journal of Pediatric Surgery. 24 (4): 317-321. https://doi.org/10.1055/s-0033-1349058; PMid:23846982
Romeo V, Maurea S, Mainenti PP, Camera L, Aprea G, Cozzolino I, Salvatore M. (2015). Correlative imaging of cystic lymphangiomas: ultrasound, CT and MRI comparison. Acta radiologica open. 4 (5): 2047981614564911. https://doi.org/10.1177/2047981614564911; PMid:26019889 PMCid:PMC4437906
Tasnádi G. (1993). Epidemiology and etiology of congenital vascular malformations. Semin Vasc Surg. 6: 200-203. PMID: 8305974.
Wiegand S, Wichmann G, Dietz A. (2018). Treatment of Lymphatic Malformations with the mTOR Inhibitor Sirolimus: A Systematic Review. Lymphatic research and biology. 16 (4): 330-339. https://doi.org/10.1089/lrb.2017.0062; PMid:29924669
World Medical Association. (2013). Declaration of Helsinki: ethical principles for medical research involving human subjects. JAMA. 310 (20): 2191-2194. https://doi.org/10.1001/jama.2013.281053; PMid:24141714
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