Impact of Intracystic Hemorrhage on Therapeutic Outcomes in Macro/Mixed Cystic Lymphatic Malformation: A Retrospective Cohort Study

Abstract

Objectives: This research aims to examine the impact of intracystic hemorrhage (ICH) on therapeutic outcomes in children with macro or mixed cystic lymphatic malformation (cLM). Methods: This retrospective study included macro/mixed cLM cases with or without ICH who underwent treatment between January 2019 and June 2024. All patients were diagnosed using preoperative imaging findings and intraoperative indocyanine green (ICG) lymphography. The baseline data of enrolled cases were retrospectively collected. The clinical characteristics were documented, including gender, age, histological typing, location, maximum diameter, and intracystic condition. Patients with or without ICH were divided into two groups. The dependent variables for predicting an excellent outcome were analyzed using multivariable logistic regression models after adjusting for potential factors using a univariable regression model. Postoperative variables, including duration of negative drainage, local infection, scar hyperplasia, and follow-up, were compared between the two groups. Results: A total of 83 cLM patients were included (ICH group: n = 36 and without ICH group: n = 47). A complete absence of afferent lymphatic vessels was demonstrated using intraoperative ICG lymphography, suggesting the isolated nature of ICH cases. ICH (p = 031; OR, 2.560; 95% CI, 1.089-6.020) was identified as the main predictor, and younger patients (p = 035; OR, 0.415; 95% CI, 0.183-0.940) had a lower potential for excellent outcomes. For the postoperative variables, the ICH group exhibited a shorter duration of negative drainage than the without ICH group (p < 0.001), while no significant differences were found regarding local infection (p = 0.693) and scar hypertrophy (p = 0.648). Conclusions: Although characterized by aggressive progression and compressive symptoms, ICH emerges as an independent favorable prognostic predictor in macro/mixed cLM management, potentially attributable to its isolated nature.

Keywords: children; cystic lymphatic malformation; indocyanine green; intracystic hemorrhage; therapeutic outcomes.

Figure 1

The selection of ICG injection points for cLM at the (a) face, (b) neck, (c) chest, (d) abdomen or back, (e) upper extremity, and (f) lower extremity. ICG, indocyanine green; cLM, cystic lymphatic malformation. Image drawn by Tao Han using BioRender.com.

Figure 2

Images of macro/mixed cLM without ICH. (a) A 4-year-old female patient with macro cLM of the right neck. (b) Preoperative MRI indicated macro cysts without ICH. (c) Intraoperative ICG lymphography finding with one inflow from posterior auricular region draining into the lesion. (d) A 4-month-old female patient with mixed cLM of the left chest. (e) Preoperative MRI indicated mixed cysts without ICH. (f) Intraoperative ICG lymphography finding with one inflow from areola region draining into the lesion. Arrow head, lymph vessel; circle, region of the lesion. cLM, cystic lymphatic malformation; ICH, intracystic hemorrhage; MRI, magnetic resonance imaging; ICG, indocyanine green.

Figure 2

Images of macro/mixed cLM without ICH. (a) A 4-year-old female patient with macro cLM of the right neck. (b) Preoperative MRI indicated macro cysts without ICH. (c) Intraoperative ICG lymphography finding with one inflow from posterior auricular region draining into the lesion. (d) A 4-month-old female patient with mixed cLM of the left chest. (e) Preoperative MRI indicated mixed cysts without ICH. (f) Intraoperative ICG lymphography finding with one inflow from areola region draining into the lesion. Arrow head, lymph vessel; circle, region of the lesion. cLM, cystic lymphatic malformation; ICH, intracystic hemorrhage; MRI, magnetic resonance imaging; ICG, indocyanine green.

Figure 3

Images of macro/mixed cLM with ICH. (a) A 2-year-old male patient with macro cLM of the right temporal region. (b) Preoperative MRI indicated macro cysts with ICH. (c) Intraoperative ICG lymphography finding without any inflow draining into the lesion. (d) A 2-month-old male patient with mixed cLM of the right chest. (e) Preoperative MRI indicated mixed cysts with ICH. (f) Intraoperative ICG lymphography finding without any inflow draining into the lesion. Arrow head, lymph vessel; circle, region of the lesion. cLM, cystic lymphatic malformation; ICH, intracystic hemorrhage; MRI, magnetic resonance imaging; ICG, indocyanine green.

Figure 4

Forest plot from multivariable binary logistic regression analysis in cLM cases with excellent outcomes. OR, odds ratio; CI, confidence interval.

Figure 5

A 2-year-old male patient suffered from macro cLM with ICH in left forearm. (a) Clinical appearance preoperatively. (b) Preoperative MRI indicated macro cysts with ICH. (c) ICG lymphography showing that one lymph vessel on the ulnar side of the left forearm bypassed the lesion and flowed into the cubital fossa lymph node. No communication between lymph vessel and the lesion cite was observed. (d) Gross observation of ICH intraoperatively. (e) No ICG was found inside the lesion. (f) Twelve months postoperatively, complete regression of cLM met the criterion of excellent curative effect. Arrow head, lymph vessel; circle, region of the lesion; triangle, lymph node. cLM, cystic lymphatic malformation; ICH, intracystic hemorrhage; MRI, magnetic resonance imaging; ICG, indocyanine green.

Figure 6

A 7-year-old female patient suffered from mixed cLM with ICH in left neck. (a) Clinical appearance preoperatively. (b) Preoperative MRI indicated mixed cysts with ICH. (c) ICG lymphography showed that one lymph vessel from posterior auricular region bypassed the lesion, and no communication between lymph vessel and the lesion cite was observed. (d) Gross observation of ICH intraoperatively. (e) No ICG was found inside the lesion. (f) Sixteen months postoperatively, complete regression of cLM met the criterion of excellent curative effect. Arrow head, lymph vessel; circle, region of the lesion; triangle, lymph node. cLM, cystic lymphatic malformation; ICH, intracystic hemorrhage; MRI, magnetic resonance imaging; ICG, indocyanine green.

Figure 7

Postoperative pathology. (a) cLM without ICH: structure of lymphatic vessel wall and lymphoid tissue. (b) cLM with ICH: similar lymphatic vessel wall structure and lymphoid tissue, accompanied by intracystic blood clots. cLM, cystic lymphatic malformation; ICH, intracystic hemorrhage.