Systematic literature review of lymphatic imaging-guided procedural management of Noonan syndrome

J Vasc Surg Venous Lymphat Disord. 2022 Sep;10(5):1192-1196.e3. doi: 10.1016/j.jvsv.2022.03.017. Epub 2022 May 10.


Objectives: To assess through literature case analysis how advances in lymphatic imaging, interventional radiology, and lymphatic vascular microsurgery illuminate and improve the lymphatic-flow status in select patients with Noonan syndrome (NS) who have undergone surgical intervention as a part of their comprehensive and individualized treatment plan. Also, we sought to illustrate the spectrum of lymphatic complications that can occur in this patient population when lymphatic flow through abnormal vasculature is surgically disrupted.

Methods: A literature review was performed by searching "Noonan AND Lymphatic AND Imaging" in the PubMed database. Inclusion criteria for this study were (1) diagnosis and clinical description of at least one original patient with NS, (2) imaging figures depicting lymphatic structure and function or a description of lymphatic imaging findings when a figure is not present, and (3) documentation of either lymphatic surgical intervention or lymphatic complications resulting from other procedures. Patient cases were first grouped by documented surgical intervention type, then clinical outcomes and lymphatic imaging results were compared.

Results: A total of 18 patient cases from 10 eligible publications were included in our review. Lymphatic imaging findings across all patients included lymphatic vessel dysplasia along with flow disruption (n = 16), thoracic duct malformations (n = 12), dermal lymphatic reflux (n = 7), and dilated lymphatic vessels (n = 4). Lymphovenous anastomosis (n = 4) resulted in rapid improvement of patient symptoms and signs. New-onset lymphatic manifestations noted over 10 to 20 years for two of these patients were chylothorax (n = 1), erysipelas (n = 1), and gradual-onset nonchylous scrotal lymphorrhea (n = 1). Targeted endovascular lymphatic disruption via sclerosis, embolization, or ablation (n = 8) results were mixed depending on the degree of central lymphatic involvement and included resolution of symptoms (n = 1), postoperative abdominal hemorrhage (n = 1), stable condition or minor improvement (n = 5), and death (n = 2). Large lymphatic vessel ligation or accidental incision (n = 6) occurred during thoracotomy (n = 4), scrotoplasty (n = 1), or inguinal lymph node biopsy (n = 1). These resulted in postoperative onset of new-onset regional lymphatic reflux (n = 5), chylothorax (n = 4), death (n = 3), or persistent or unchanged symptoms (n = 1).

Conclusions: Imaging of the central lymphatics enabled characterization of lymphatic developmental features and guided operative management of lymphatic vascular defects in patients with NS. This review of the literature suggests that the surgical preservation or enhancement of central lymphatic return in patients with NS may improve interventional outcomes, whereas the disruption of central lymph flow has significant potential to cause severe postoperative complications and worsening of the patient's clinical condition.

Keywords: Congenital lymphatic dysplasia; Lymphatic imaging; Lymphovenous anastomosis; Noonan syndrome; Thoracic duct decompression.

Publication types

  • Review
  • Systematic Review
  • Research Support, Non-U.S. Gov't
  • Research Support, N.I.H., Extramural

MeSH terms

  • Humans
  • Lymphatic Vessels* / diagnostic imaging
  • Lymphatic Vessels* / surgery
  • Noonan Syndrome* / diagnostic imaging
  • Noonan Syndrome* / surgery
  • Surgery, Computer-Assisted*