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Diagnostic tools for congenital lymphatic disorders;
Modern time lymphography
Kimmo Lappalainen
Helsinki University Central Hospital
Thoracic duct embolization; Indications and technical
aspects
• No disclosures
Methods and History
1. Pedal lymphography
2. Intranodal lymphography
3. Intracuteneous MR lymphography
4. Intranodal dynamic MR lymphography
1. Conventional Pedal Lymphography
• Method of choise for imaging of lymphaticsystem for decades
– Especially staging of malignant diseases
• Lymphomas
• Genitourinary malignant tumors
– Leakage
Labeled Ethiodol in the USA
Lipiodol was first synthesized by Marcel Guerbet in the Paris School of Pharmacy in 1901
Used for myelography by two French physicians, Jacques Forestier and Jean Sicard in 1921
Lipiodol was often used as a contrast medium at hysterosalpingography
Pediatric dose 0.25 ml / kg
Lipiodol lymphography: Left image: After the injection of contrast material (lymphangiographic phase) the normal lymphatic vessels of the minor pelvis are visualized. Right image: 24 hours after the injection normal inguinal, pelvic and paralumbar lymph nodes are demonstrated (nodal phase).
2. Intranodal (dynamic) lymphography
• Direct puncture to the groin lymphatic nodes
– Spinal needles
– Very slow injection of Lipiodol
Intranodal ( dynamic ) lymphography
• Direct puncture to the groin lymphatic nodes
– Spinal needles
– Very slow injection of Lipiodol
– Works sometimes like diagnostic tool
– But now used mostly as a pretreatment mapping
# 1. Patient 8 years old boy
• Schimmelpenning sdr.
• Lower limb oedema and patholical fracture
• Chylusuria
• Leakage / where?
Dr. Itkin
Intranodal ( dynamic ) lymphography
• Direct puncture to the groin lymphatic nodes
– Spinal needles
– Very slow injection of Lipiodol
– Works sometimes like diagnostic tool
– But now used mostly as a pretreatment mapping
1. Cope C. Diagnosis and treatment of postoperative chyle leakage viapercutaneous transabdominal catheterization of the cisterna chyli: a preliminary study. J Vasc Interv Radiol 1998; 9:727–734.
2. Itkin M, Kucharczuk JC, Kwak A, Trerotola SO, Kaiser LR. Nonoperativethoracic duct embolization for traumatic thoracic duct leak: experience in 109 patients. J Thorac Cardiovasc Surg 2010; 139:584–589.
3. Nadolski GJ, Itkin M. Feasibility of ultrasound-guided intranodallymphangiogram for thoracic duct embolization. J Vasc Interv Radiol2012; 23:613–616.
4. Rajebi MR, Chaudry G, Padua HM, Alomari A et al. Intranodallymphangiography:Feasibility and preliminary experience in children. J Vasc IntervRadiol2011; 22:1300–1305.
# 1. Postoperative leakage
• Healthy very active woman, 50 years old
• Laparoscopic hernia repair
• Postoperative distended abdomen
Pär Gerwins, MD PhDProfessor of vascular biologyDepartment of radiology/interventional radiologyUppsala university hospital751 85 Uppsala, [email protected]
* = lymphnode
*
*
*
Normal lymphvessel
leakage
Needle in lymphnode
Nodal lymphangiography shows leakage above the inguinalligament
Pär Gerwins, MD PhDProfessor of vascular biologyDepartment of radiology/interventional radiologyUppsala university hospital751 85 Uppsala, [email protected]
Mo
rnin
gw
eigh
t
sclerotherapy
Leakage stopped after sclerotherapy
# 2. Postoperative leakage
• Male 66 years old
• Oesophageal tumor, radical surgery
• Postoperative chylus leakage
– Two reoperations with no responce
# 3. Postoperative leakage
• A boy born 2005
• 2006 thoracoscopy and resection of a kind of cyst or tumor: PAD; broncial cyst oreosophagial duplication
• Frequent pleural effusions and punctures
• 2018 review of the tissue blocks : LM
• -> lymfography the 17th May 2018
Glue
Plastic Bronchitis
• Percutaneous Lymphatic Embolization of Abnormal• Pulmonary Lymphatic Flow as Treatment of Plastic• Bronchitis in Patients With Congenital Heart Disease• Yoav Dori, MD, PhD; Marc S. Keller, MD; Jonathan J. Rome,
MD;• Matthew J. Gillespie, MD; Andrew C. Glatz, MD, MSCE;
Kathryn Dodds, MSN, CRNP;• David J. Goldberg, MD; Samuel Goldfarb, MD; Jack Rychik,
MD; Maxim Itkin, MD
(Circulation. 2016;133:1160-1170. DOI: 10.1161/CIRCULATIONAHA.115.019710.)
Lipiodol itself can act as embolicmaterial
3. Intracutaneous MR Lymphography
• Our need
– Diagnosis of chronic lymphaoedema
– Diagnosis of lymphatic collections
• We started 2009
MR-lymphangiography at 3.0T—a feasibility
• Mike Notohamiprodjo, Ruediger G. H. BaumeisterTobias F. Jakobs, Karin A. Herrmann et al.
Published online: 6 June 2009# European Society of Radiology 2009
Technique
• Intracutaneous Gd injectio0.5 – 1 ml
• 3Dfl, voxel size 0.8 x 0.8 x 1.2 mm
• Typically added with fatsupression images, STIR, Dixon
Need for ic MR
• Chronic limb oedema
– Congenital
– Postoperative or trauma
– Post radiation therapy
• We perform MR imaging only if operative is an option ( = lymphovenotic fistula )
# 1. Patient
• Lady born 1958 . Sporty
• Year 1991 radical radical pelviclympadenectomy etc , ovarian cancer
• Right lower limb oedema and vulvar lymphaticleakage
• Left lower limb treated wit liposuction
# 2. Patient
• A girl 13 years old
• The right lower limb had always been thicker
• Reason for it?
Plastic Bronchitis
• Percutaneous Lymphatic Embolization of Abnormal• Pulmonary Lymphatic Flow as Treatment of Plastic• Bronchitis in Patients With Congenital Heart Disease• Yoav Dori, MD, PhD; Marc S. Keller, MD; Jonathan J. Rome,
MD;• Matthew J. Gillespie, MD; Andrew C. Glatz, MD, MSCE;
Kathryn Dodds, MSN, CRNP;• David J. Goldberg, MD; Samuel Goldfarb, MD; Jack Rychik,
MD; Maxim Itkin, MD
(Circulation. 2016;133:1160-1170. DOI: 10.1161/CIRCULATIONAHA.115.019710.)
4. Intranodal dynamic MR lymphography
Plastic Bronchitis
• Percutaneous Lymphatic Embolization of Abnormal• Pulmonary Lymphatic Flow as Treatment of Plastic• Bronchitis in Patients With Congenital Heart Disease• Yoav Dori, MD, PhD; Marc S. Keller, MD; Jonathan J. Rome,
MD;• Matthew J. Gillespie, MD; Andrew C. Glatz, MD, MSCE;
Kathryn Dodds, MSN, CRNP;• David J. Goldberg, MD; Samuel Goldfarb, MD; Jack Rychik,
MD; Maxim Itkin, MD
(Circulation. 2016;133:1160-1170. DOI: 10.1161/CIRCULATIONAHA.115.019710.)
A boy 14 years old
4 / 2018
A girl 3years 6 months old
• UVH (TA+VSD, LSVC). BDG, PA-banding ja LSVC-ligaatio 1/16, LSVC:n connection to thepulmonary artery 2/16. 26.3.2019 electiveTCPC 5 mm atriumfenestration. Postoperativea lot of lymphatic leakage especially to the leftpleura.
• CVP 18 mmHg
• It there a thoracic duct damage?
4 /2019
• Reoperation: 10.4.
• ( partielliin TCPC:n purkuun: LSVC siirrettiin vasempaan eteiseen ja eteistunneliin tehtiin toinen, aiempaa vähän isompi (6 mm) fenestraatio.)
A Girl born 13 /12 2017
• In the age 4 months severe cholestasis and ja fusiformic choledocus cyst
• Resection and Roux- Y – hepaticojenunostomy
• Chylys leakage in the abdominal cavity
Neonatal chylothorax
Embolization with lipiodol via nodal injection in groins
Lipiodol itself acts as embolic material and stops leak
Post embolization
Post embolization
A Male Patient Born 07/01 2002
• 16 years old, Noonan sdr.
• Many heart operations good ventricularfunction
• Problems:
– Very peculiar lymphatic vessels and channels
– Bilateral chylothorax
– Right lower limb oedema
6.6.2018
15.6.2018
6.6.2018
Some other interesting cases
• Patient with protein loosing eteropathy
• Sometimes connected to liver lympha leakageto the gut – but usually with congestion in liver.
Genital lyphatic leakage
• Previous pelvic lympadenectomy
• Chronic lyphatic leakage
• Wearing diapers
Lipiodol 50 % + STS 50 %
5 / 2012
Second session
11 / 2012
2012 2014
Central conducting lymphatic anomalyPär Gerwins
• 15 years old boy
• Leakage of chylus from scrotum
• Wears dipers
• Depressed
MR shows scrotaledema and pathologicallymp nodes/vessels in left groin
Nodal lymphangiographyshows ectatic lymphaticvessels along iliacvessels left side (*) and reflux to scrotum (→).
*
Direct puncture of refluxing lymphatic vessel to scrotum (→) and sclerotherapy (* marks sclerotherapy solution)
*
Before Aftersclerotherapy
Result: Normal scrotum and a happy teenager without signs of depression anymore
Thank you !