Assessment of the lymphatic system in patients with diffuse lymphangiomatosis by magnetic resonance imaging
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)
- et al.
Indirect magnetic resonance lymphangiography in patients with lymphedema preliminary results in humans
Eur J Radiol
(2006) - et al.
Reversible renal hypertension due to renal hygroma
Urology
(1989) - et al.
OK-432 therapy for lymphatic malformation in 32 patients (28 children)
Int J Pediatr Otorhinolaryngol
(2002) - et al.
Textbook of lymphology
(2007) - et al.
Cystic lymphangioma in children: report of 32 cases including lesions at rare sites
Surgery
(1971) - et al.
Disseminated lymphangiomatosis with skeletal involvement: detection with magnetic resonance lymphangiography
Lymphology
(2007) Genital lymphangioma with recurrent cellulitis in men
Int J Dermatol
(2006)- et al.
CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients
AJR
(2000) - et al.
Atypical abdominal paediatric lymphangiomatosis: diagnosis aided by diffusion-weighted MRI
Pediatr Radiol
(2006) - et al.
Lymphangiomas in children: MR imaging
Radiology
(1989)
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 GynecologyCitation 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 RadiologyCitation 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 ResearchCitation 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 ImagingLymph Node Metastases Detection Using Gd<inf>2</inf>O<inf>3</inf>@PCD as Novel Multifunctional Contrast Imaging Agent in Metabolic Magnetic Resonance Molecular Imaging
2022, Contrast Media and Molecular ImagingLymphangiomatosis: Rare cause of pleural effusion
2021, Pneumologie