Assessment of the lymphatic system in patients with diffuse lymphangiomatosis by magnetic resonance imaging

https://doi.org/10.1016/j.ejrad.2009.10.021Get rights and content

Abstract

Objective

To assess the lymphatic system in patients with diffuse lymphangiomatosis by magnetic resonance imaging.

Materials and methods

15 patients with diffuse lymphangiomatosis were examined by magnetic resonance imaging. Three locations were examined: first, the lower leg and foot region; second, the upper leg and the knee region; and third, the pelvic with retroperitoneal and abdominal region. For magnetic resonance lymphangiography a T1-weighted 3D spoiled gradient-echo and a T2-weighted 3D-TSE sequence was used.

Results

The size of the genital lymphangiomas, which were revealed in all patients, varied between 5 and 83 mm. In 47% of the patients lymphangiomas were detected at the level of the lower legs, and in 87% of the patients at the level of the upper leg and retroperitoneum. Furthermore, lymphangiomas were seen in the inguinal and pelvic region in 100% and intraabdominally in 40% of the patients. The lymphangiomas extended into the abdominal wall in 93% of the examined patients. A chylous pleural effusion was revealed in 20% and a chylous ascites in 13% of patients. 93% of patients suffered due to the diffuse lymphangiomatous pathologies from a lymphedema of the lower extremities, while a generalized lymphedema of the trunk was found in 87% of the patients.

Conclusion

Magnetic resonance imaging is a safe and accurate minimal-invasive imaging modality for the evaluation of the lymphatic system in patients with diffuse lymphangiomatosis. Since the localization and extension of the lymphangiomas are important prognostic factors, it is crucial to perform a safe radiologic evaluation with a high resolution for the patient's therapeutic planning.

Introduction

Diffuse lymphangiomatosis is a rare disorder characterized by a multifocal proliferation of complex, irregular lymphatic channels, involving soft tissue, viscera, retroperitoneum, eyes and skeletal system [1], [2], [3]. The cause of the disease is not yet fully understood, but the association of primary lymphatic dysplasia with a lymphatic proliferative process has been proposed. Diffuse lymphangiomatosis occurs mainly in children and adolescents under 20 years of age and its process carries a bad prognosis, especially if visceral involvement is present, which can lead to chylothorax, chylopericardium and hepatosplenomegaly [1], [2], [3]. A further consequence of intestinal lymphangiomatosis is the appearance of chylous ascites and protein loosing enteropathy. An aggravating and for the patient very compromising complication is the development of lymphedema in the affected regions [1]. Additionally, lymphphorrhea due to the formation of lympho-cutaneous fistulas and lymphcysts can occur [1]. Another feared and possibly life-threatening complication is infection [1], [4].

Up to now, magnetic resonance imaging (MRI), computed tomography (CT), ultrasonography (US) and conventional radiography have been used to assess the extent of pathologic changes in patients suffering from diffuse lymphangiomatosis, whereby several studies have demonstrated the advantages of MRI compared to the other radiologic imaging modalities [5], [6], [7], [8], [9].

The intention of the presented study was to exactly evaluate the complete extent of the pathologic lymphatic changes in a larger patient group suffering from diffuse lymphangiomatosis by means of magnetic resonance imaging using the magnetic resonance lymphangiography (MRL) technique [10] with a heavily T2-weighted 3D-TSE sequence and a T1-weighted 3D-VIBE sequence after intracutaneous contrast media application. To our knowledge the presented patient group with diffuse lymphangiomatosis is the largest in the medical literature examined so far by MRI.

Section snippets

Materials and methods

Between August 2005 and July 2007 15 patients (age 21–57 years; mean age 35 years; 4 females, 11 males) with diffuse lymphangiomatosis were referred for MRL. This study had been approved by our institutional review board, and informed consent had been obtained from all patients prior to MR imaging. The procedures were in accordance with the ethical standards of the World Medical Association (Declaration of Helsinki).

A total amount of 18 mL contrast material (Gadoteridol, Prohance®, Bracco-Byk

Results

All 15 patients tolerated the examinations well without complications. The size of the genital lymphangiomas varied in the examined patients between 5 and 83 mm. In 7 of the 15 patients (47%) lymphangiomas were detected at the level of the lower legs (Fig. 1, Fig. 2, Fig. 3, Fig. 4) and in 13 of the 15 patients (87%) in the region of the upper legs and retroperitoneum (Fig. 4, Fig. 5, Fig. 6, Fig. 7). In all patients (100%) the lymphangiomatous changes involved the inguinal and pelvic area (Fig.

Discussion

Generalized lymphangiomatosis was first described by Rodenber in 1828 [5]. Landing and Farber then classified lymphangiomas histologically into three categories [12]: (1) capillary or simple lymphangiomas, which are composed of capillary, thin-walled lymphatic channels, (2) cavernous lymphangiomas, consisting of dilated lymphatic channels, often with fibrous adventitial coats, and (3) cystic lymphangiomas, known as cystic hygroma, which are the most frequent manifestation and occurs most

Conclusions

In conclusion, magnetic resonance imaging using the MRL technique is a safe and accurate minimal-invasive imaging modality for the evaluation of the lymphatic circulation in patients with diffuse lymphangiomatosis. Since the localization and extension of the lymphangiomas are important prognostic factors, it is crucial to perform a safe radiologic evaluation with a high resolution for the patient's therapeutic planning.

References (20)

There are more references available in the full text version of this article.

Cited by (18)

  • Detection of mosaic 15q11.1-q11.2 deletion encompassing NBEAP1 and POTEB in a fetus with diffuse lymphangiomatosis

    2017, Taiwanese Journal of Obstetrics and Gynecology
    Citation Excerpt :

    Diffuse lymphangiomatosis is a rare congenital malformation of the lymphatics caused by primary lymphatic dysplasia and a lymphatic proliferative process that may involve soft tissues, visceral organs, bones, retroperitoneum, and eyes [1–3].

  • Peripheral Magnetic Resonance Lymphangiography: Techniques and Applications

    2016, Techniques in Vascular and Interventional Radiology
    Citation Excerpt :

    We believe these features are beneficial for imaging the small- and low-volume superficial lymphatic channels. Other extracellular MR contrast agents have also been safely used for MRL—gadopentate dimeglumine (Gd-DTPA, Magnevist),16,18 gadoterate meglumine (Gd-DOTA, Dotarem),25 gadoteridol (Gd-HPDO3A, Prohance),17,26,27 and gadodiamide (Gd-DTPA-BMA, Omniscan).22,28 As patients often feel mild-to-moderate pain during the intracutaneous injection, we use small gauge needles and mix a local anesthetic with the MR contrast agent before the intracutaneous injection.29

  • Imaging the lymphatic system

    2014, Microvascular Research
    Citation Excerpt :

    The technique is relatively non-invasive and can be used to identify anatomic and physiological abnormalities associated with lymphatic dysfunction in order to determine further treatment strategies (Lohrmann et al., 2009). MRL has shown promise in imaging the lymphatic system in multiple pathologies including breast cancer (Lu et al., 2013), lymphedema (Lohrmann et al., 2007; Rane et al., 2013) and diffuse lymphangiomatosis (Lohrmann et al., 2011). Imaging agents with better sensitivity and specificity are under development (McDermott et al., 2013) and will improve the ability of MRL to identify pathologies in the lymphatic system.

  • MRI of Lymphedema

    2023, Journal of Magnetic Resonance Imaging
View all citing articles on Scopus
View full text