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, Swedenpar.gerwins@akademiska.se

* = 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, Swedenpar.gerwins@akademiska.se

Mo

rnin

gw

eigh

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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.)

https://www.google.fi/url?sa=i&rct=j&q=&esrc=s&source=images&cd=&cad=rja&uact=8&ved=2ahUKEwj997zhho3iAhUR4KYKHSvgC_gQjRx6BAgBEAU&url=https://usa.healthcare.siemens.com/angio/nexaris-therapy-suites/nexaris-angio-mr-ct&psig=AOvVaw3XvML-HdkKrNFJ_11bTpmx&ust=1557443346731478

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 !