Life-threatening complications in patients with thoracic and abdominal lymphatic malformations

Authors

DOI:

https://doi.org/10.30978/GS-2023-1-36

Keywords:

lymphatic malformations, minimally invasive surgery, conversion, lymphorrhea, coagulopathy

Abstract

The term «lymphatic malformations» (LMs) refers to a wide spectrum of disorders with clinical manifestations that can vary from asymptomatic to life‑threatening.

Objective — to analyze the factors and pathological conditions that necessitate the use of emergency surgical procedures in patients with thoracic and abdominal LMs.

Materials and methods. The retrospective study of medical charts of patients with LMs was performed for a period from 2012 to 2021. Among 240 patients with LMs, 55 (22.9%) were diagnosed with lesions of the abdominal or thoracic cavity. 5 (9.1%) required an emergency surgical procedure.

Results. Among 38 patients with abdominal LMs, only one (2.6%) required emergency surgery. This patient underwent laparotomy and subtotal bowel resection for total mesenteric thrombosis. The postoperative period was complicated by short bowel syndrome. Mediastinal LMs were diagnosed in 17 patients, 14 (73.7%) of whom had neck LM extension. In 4 cases, mediastinal LMs were complicated by intrathoracic tension syndrome. It was caused by a lymphatic leak into the pleural cavity in 1 case and by sudden enlargement of LMs, resulting from intracystic hemorrhage, in 3 other cases. A pleural drain with subsequent sclerotherapy was used in a patient with chylothorax. Patients with intracystic hemorrhage underwent thoracotomy and partial LM resection. They also received an injection of a sclerosing agent into the residual cysts. In uncomplicated cases, minimally invasive methods were preferred, with laparoscopic resections of abdominal LMs in 22 (78.6%) patients and sclerotherapy under ultrasound guidance in 7 (36.8%) patients with mediastinal LMs.

Conclusions. Intrathoracic tension syndrome and thrombotic complications are potentially dangerous and life‑threatening conditions that pose a risk to patients with visceral LMs and require emergency interventions. Minimally invasive technologies were preferred in uncomplicated cases of thoracic and abdominal LMs, whereas open surgeries were the method of choice in complicated cases.

References

Benzar IM, Levytskyi AF, Diehtiarova DS, Godik OS, Dubrovin OG. Treatment of lymphatic malformations in children: 10 years of experience. Paediatric surgery. Ukraine. 2022;2(75):5-14. http://doi.org/10.15574/PS.2022.75.5. Ukrainian.

Benzar I., Levytskyi A., Diehtiarova D. et al. Vascular anomalies in newborns: clinical presentation, complications, and peculiarities of therapy. Wiad Lek. 2020;73(9 cz. 2):1934-9. http://doi.org/10.36740/WLek202009207.

Cooke-Barber J, Dasgupta R. Management of visceral vascular anomalies. Seminars in Pediatr Sur. 2020;29(5):150977. http://doi.org/10.1016/j.sempedsurg.2020.150977.

Dompmartin A, Acher A, Thibon P, et al. Association of localized intravascular coagulopathy with venous malformations. Arch. Dermatol. 2008;144:873-7. http://doi.org/10.1001/archderm.144.7.873.

Francavilla ML, White CL, Oliveri B, Lee EY, Restrepo R. Intraabdominal lymphatic malformations: pearls and pitfalls of diagnosis and differential diagnoses in pediatric patients. AJR Am J Roentgenol. 2017;208(3):637-49. http://doi.org/10.2214/AJR.16.17008.

Gasparella P, Singer G, Castellani C, Sorantin E, Haxhija EQ, Till H. Giant lymphatic malformation causing abdominal compartment syndrome in a neonate: a rare surgical emergency. J Surg Case Rep. 2020;26;2020(8):rjaa252. http://doi.org/10.1093/jscr/rjaa252.

Ghaffarpour N, Burgos CM, Wester T. Surgical excision is the treatment of choice for cervical lymphatic malformations with mediastinal expansion. J Pediatr Surg. 2018;53(9):1820-4. http://doi.org/10.1016/j.jpedsurg.2017.10.048.

Gurevich A, Hur S, Singhal S, DiBardino D, Haas AR, Hansen-Flaschen JH, Nadolski G, Itkin M. Nontraumatic chylothorax and chylopericardium: diagnosis and treatment using an algorithmic approach based on novel lymphatic imaging. Ann Am Thorac Soc. 2022; 19(5):756-62. http://doi.org/10.1513/AnnalsATS.202103-262OC.

Kulungowski AM, Patel M. Lymphatic malformations. Semin Pediatr Surg. 2020 Oct;29(5):150971. http://doi.org/10.1016/j.sempedsurg.2020.150971.

Liu Q, Fu J, Yu Q, Gong W, Li P, Guo X. Laparoscopic surgery of intra-abdominal lymphatic malformation in children. Exp Ther Med. 2022;19;24(3):581. http://doi.org/10.3892/etm.2022.11519.

Mack JM, Verkamp B, Richter GT, Nicholas R, Stewart K, Crary SE. Effect of sirolimus on coagulopathy of slow-flow vascular malformations. Pediatr Blood Cancer. 2019;66(10):e27896. http://doi.org/10.1002/pbc.27896.

Ricci KW, Brandão LR. Coagulation issues in vascular anomalies. Semin Pediatr Surg. 2020 Oct;29(5):150966. http://doi.org/10.1016.

Scuglia M, Conforti A, Valfrè L, Totonelli G, Iacusso C, Iacobelli BD, Meucci D, Viggiano M, Fusaro F, Diociaiuti A, Morini F, El Hachem M, Bagolan P. Operative Management of Neonatal Lymphatic Malformations: Lesson Learned From 57 Consecutive Cases. Front Pediatr. 2021;18;9:709223. http://doi.org/10.3389/fped.2021.709223.

Sun JD, Shum T, Behzadi F, Hammer MM. Imaging findings of thoracic lymphatic abnormalities. Radiographics. 2022;42(5):1265-82. http://doi.org/10.1148/rg.220040.

Tutor JD. Chylothorax in infants and children. Pediatrics. 2014;133(4):722-33. http://doi.org/10.1542/peds.2013-2072.

Ueno S, Fujino A, Morikawa Y, et al. Treatment of mediastinal lymphatic malformation in children: an analysis of a nationwide survey in Japan. Surg Today. 2018;48(7):716-25. http://doi.org/10.1007/s00595-018-1640-0.

Zamora AK, Barry WE, Nowicki D, et al. A multidisciplinary approach to management of abdominal lymphatic malformations. J Pediatr Surg. 2021;56(8):1425-9. http://doi.org/10.1016/j.jpedsurg.2020.10.007.

Zhuo KY, Russell S, Wargon O, Adams S. Localised intravascular coagulation complicating venous malformations in children: Associations and therapeutic options. J Paediatr Child Health. 2017;53(8):737-41. http://doi.org/10.1111/jpc.13461.

Downloads

Published

2023-04-13

How to Cite

1.
Prytula V, Rudenko Y, Gorbatiuk O, Nakonechnyi A, Susak Y. Life-threatening complications in patients with thoracic and abdominal lymphatic malformations. ЗХ [Internet]. 2023Apr.13 [cited 2024Dec.22];(1):36-40. Available from: http://generalsurgery.com.ua/article/view/276895

Issue

Section

Original Research