Minimal Lymphatic Leakage in an Infant WithChylothorax Detected by Lymphoscintigraphy SPECT/CT

abstractA 7-month-old girl with history of persistent left chylous pleural effu-sion was referred for lymphoscintigraphy. A previous chest computedtomography (CT) scan demonstrated a small to moderate-sized leftpleural effusion but could not identify the lymphatic leakage site.Lymphoscintigraphy using filtered 99mTc sulfur colloid showed mini-mal focal activity in the lower chest. A correlative single-photon emis-sion computed tomography (SPECT)/CT localized this activity to distalparaesophageal region, being highly suggestive of the site of lym-phatic leakage. Subsequent lymphangiography confirmed these find-ings, revealing an abnormal lymphatic branch at the level of T10 andT11 vertebrae with retrocrural extravasation toward the left hemi-thorax. Thoracic duct embolization was accomplished at and proximalto the site of chyle leak using a platinum coil and n-Butyl cyanoacry-late glue. The patient was followed up for .24 months and demon-strated no recurrence of pleural effusion. No ascites or othercomplications related to the procedure were noted. The case demon-strates that 99mTc sulfur colloid lymphoscintigraphy SPECT/CT can bea useful modality for detecting the chyle leakage site in children withchylothorax even when the amount of leakage is minimal. Pediatrics2014;134:e606–e610

AUTHORS: Jigang Yang, MD, PhD,a,b Ion Codreanu, MD,PhD,b,c and Hongming Zhuang, MD, PhDb

aDepartment of Nuclear Medicine, Beijing Friendship Hospital ofCapital Medical University, Beijing, China; bDepartment ofRadiology, The Children’s Hospital of Philadelphia, Philadelphia,Pennsylvania; and cDepartment of Radiology, MedparkInternational Hospital, State University of Medicine andPharmacy Nicolae Testemitanu, Chisinau, Moldova

KEY WORDSlymphoscintigraphy, chylothorax, SPECT/CT

ABBREVIATIONSCT—computed tomographySPECT—single-photon emission computed tomographyTD—thoracic ductTDE—thoracic duct embolization

Dr Yang reviewed the literature and drafted the initialmanuscript; Dr Codreanu reviewed the literature, drafted theinitial manuscript, and revised the manuscript; and Dr Zhuangreviewed the literature, reviewed and revised the manuscript,and was involved in the direct care of the patient described; andall authors approved the final manuscript as submitted.

www.pediatrics.org/cgi/doi/10.1542/peds.2013-2689

doi:10.1542/peds.2013-2689

Accepted for publication Jan 21, 2014

Address correspondence to Ion Codreanu, MD, PhD, Departmentof Radiology, The Children’s Hospital of Philadelphia, 34th andCivic Center Boulevard, Philadelphia, PA 19104. E-mail:codrion@yahoo.com

PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).

Copyright © 2014 by the American Academy of Pediatrics

FINANCIAL DISCLOSURE: The authors have indicated they haveno financial relationships relevant to this article to disclose.

FUNDING: No external funding.

POTENTIAL CONFLICT OF INTEREST: The authors have indicatedthey have no potential conflicts of interest to disclose.

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Lymphoscintigraphy is an imaging tech-nique used to identify the pathways oflymphatic flow after injection of a ra-diopharmaceutical that is absorbedby the lymphatics. The technique isnontraumatic with no known adverseeffects and is often used for evaluatingpatients with suspected lymphedemaor lymphatic leakage. The method hasbeen refined in recent decades, andadvances in SPECT/CT imaging havesignificantly increased its diagnosticpotential.

Chylothorax is a type of pleural effusionresulting from accumulation of lym-phatic fluid (chyle) in the pleural cavity.Most often, it is caused by disruption orobstruction of the thoracic duct (TD) or1 of the main lymphatic vessels thatdrain to it. The diagnosis is commonlyconfirmed by the presence of chylomi-crons and triglycerides in the aspiratedpleural fluid.1–4 In children, pleural ef-fusion is usually defined as chyle whenit contains .1.1 mmol/L triglycerides(with oral fat intake) and has a totalcell count $1000 cells/mL with a lym-phocyte fraction.80%.1 The conditionshould be occasionally differentiatedfrom pseudochylothorax (cholesterolpleurisy), which represents long-standingfluid in a fibrotic pleura with a highcontent of cholesterol but no trigly-cerides or chylomicrons.3 Chylothoraxis commonly associated with signifi-cant morbidity and mortality. If theleaking vessel cannot be identified, thismay limit the treatment options to suchprocedures as drainage of the fluidout of the pleural space and omittingfat from the diet, pneumoperitonealshunting, or surgical or chemicalpleurodesis. Loss of chyle through ex-cessive pleural drainage may result inelectrolyte, nutritional, and immuno-logic complications. In some patients,the effusion may also persist despiteoptimal medical therapy.5 Therefore,localizing the leaking vessel becomesof paramount importance, especially in

pediatric patients. Herein we reporta case of minimal lymphatic leakagein a pediatric patient with persistentchylothorax detected by filtered 99mTcsulfur colloid lymphoscintigraphySPECT/CT.

PATIENT PRESENTATION

A 7-month-old girl with history of per-sistent left chylous pleural effusionand clinical concern for lymphatic leakwas referred to the nuclear medicinedepartment for a lymphoscintigraphystudy. Aprevious chest CTscan revealeda small to moderate-size left pleuraleffusion but was unable to localize thesite of lymphatic leakage.

A total of 438 mCi filtered 99mTc sulfurcolloid was injected subcutaneouslyin divided aliquots into the interdigitalweb spaces between the great andsecond toes of each foot. The injectionsite was covered with a cotton ball andbandage to prevent leakage of tracerthrough the needle puncture site. Bothfeet were gentlymassaged for 2minutesat the injection sites to promote uptakeof the tracer into lymphatic channelsand lymphatic flow. Multiple staticimages of the body taken in multipleprojections #90 minutes after injec-

tion using a Forte Nuclear Medicine(Royal Philips, Amsterdam, Netherlands)g camera proved unremarkable. Sub-sequent delayed images were obtained∼7.5 hours after injection, supple-mented by SPECT/CT images of theupper abdomen and thorax. Both sub-clavian veins and cardiac silhouettecould be clearly identified on theseplanar delayed images, confirming flowof the radiotracer from the TD into thevasculature and cardiac blood pool. Asuspicious focus of minimal tracer ac-tivity in the left lower chest was alsonoted on delayed planar images (Fig 1),localizing to distal paraesophageal re-gion on SPECT/CT images (Fig 2). TheSPECT/CT findings were particularly sug-gestive of the site of lymphatic leakage.

After consultation with interventionalradiology, a team decision was madeto perform an ultrasound-guided intra-nodal lymphangiography followed bythoracic duct embolization (TDE). Theprocedure was performed under gen-eral anesthesia. The lymphnodes in eachgroin were punctured using 25-gaugeneedles under ultrasound guidance.When test injections of contrast showedsatisfactory position and opacificationof efferent cephalad lymphatic vessels,

FIGURE 1Planar lymphoscintigraphy revealing minimal focal activity in the lower chest, slightly more prominenton posterior view (arrow). RT, right; LT, left.

CASE REPORT

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bilateral injections of approximately2mLethiodized poppyseed oil (Ethiodol;Savage Laboratories, Melville, NY) weremade under fluoroscopic guidance,followed by 2 mL saline. As the lumbartrunks began to opacify the cisternachyli, a 22-gauge Chiba needle (CookMedical, Bloomington, IN) was usedunder fluoroscopic guidance to punc-ture the lower cisterna percutaneouslyfrom an anterior transabdominal ap-proach. A stiff guidewire (V18 Control;Boston Scientific, Natick, MA) was ad-

vanced into the cisterna chyli and ma-nipulated cephalad into the TD. Over thewire, a 2.3F rapid transit microcatheterwas placed farther into the TD. Contrastinjection of the TD demonstrated a left-ward abnormal lymphatic branch atthe level of T10 to T11, with retrocruralextravasation of contrast toward theleft basal hemithorax (Fig 3A). Severalunsuccessful attempts were made toselectively enter this channel using avariety of guidewires and a rapid tran-sit microcatheter. Accordingly, a deci-

sion was made to embolize the TD atand below this point to stop the leak.One 0.5-cm straight platinum coil wasplaced (Fig 3B), followed by injection of0.5 mL n-Butyl cyanoacrylate (Trufill;Cordis Corporation, Warren, NJ) diluted1:1 in Ethiodol. The microcatheter wasimmediately removed. The lower TD, theleaking branch, and the cisterna chylidown to the entrances of the right andleft lumbar trunks were occluded. Thepatient was followed up for 24 monthsand demonstrated no recurrence ofpleural effusion. No ascites or othercomplications related to the procedurewere noted.

DISCUSSION

Chylothorax has been reported as themost common form of pleural effusionin the first few days of life, and under-standing itspathophysiologymayassistin clinical decision-making.4,6 Potentialcauses of chylothorax in children canvary and have been previously groupedinto such categories as congenital,traumatic, related to high central ve-nous pressure, and malignancies aswell as miscellaneous causes such asbenign tumors, granulomatous disease,and transdiaphragmatic movement ofchylous ascites.4,7–13 Tumors have beenreported to account for .50% of allcases of chylothorax, and traumas arethe second major cause.7 Therefore,patients presenting with nontraumaticor idiopathic chylothorax should un-dergo an appropriate workup to ex-clude a neoplastic etiology, especiallylymphomas. Traumatic events result-ing in chylothorax are often obvious,even though minor traumas affectingintrathoracic pressure such as severecoughing or vomiting, sudden stretch-ing, and hyperextension injuries havealso been reported as precipitating fac-tors.7 Because no other obvious causesfor chylothorax were revealed in ourpatient apart from an abnormal lym-phatic branch at the level of T10 to T11

FIGURE 2Associated SPECT/CT images (axial, coronal, and sagittal views) localizing the abnormal focal activity tothe distal paraesophageal region (arrows).

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vertebrae, such a minor injury accom-panied by variations of intrathoracicpressure may have precipitated thevessel rupture in this case.

Other potential causes of chylothorax inchildren include congenital lymphaticdysplasias, which may be encounteredas separate entities or be part of as-sociated conditions such as hydropsfetalis or Down, Noonan, or Turner syn-drome.4,11 Of note is that peripherallymphedema may not be always clini-cally evident at birth or during theearly years of life, despite the presenceof severe visceral lymphatic impair-ment.14 When such patients presentwith chylothorax, chylous pericardialeffusion, or chylous ascites, lympho-scintigraphy may show various degreesof lymphatic impairment, providingvaluable diagnostic clues.14 Venousthrombosis of the superior vena cavaor adjacent portions of subclavianveins may also precipitate TD rupturecaused by significantly elevated centralvenous pressure and should be in-cluded in the differential diagnosis inpatients with central venous catheters,recent thoracic surgery, or hypercoag-ulable states.4,8,12,13 When investigatingpediatric patients, one should also re-member that chylothorax in a child maybe a manifestation of child abuse.15–17

A radionuclide bone scan to detectpotential skeletal injuries may proveessential for reaching the diagnosis insuch patients and should be consid-ered among the requested investiga-tions when child abuse is suspected.

The initial diagnosis of chylothorax isusually made by pleural fluid analysis,regardless of its etiology. Subsequentinvestigations are directed at identify-ing the site of chyle leakage and theunderlying cause, which may becomeessential for guiding the therapy inpatients with persistent or recurrentdisease. Lymphangiography and lym-phoscintigraphy are 2 specificmethodsfor imaging lymphatic system that mayaid in finding the chyle leakage site.Lymphangiography enables radiographicvisualization of lymph vessels and nodesafter injection of a contrast medium.Although its utility in the diagnosis oflymph node pathology has decreasedwith recent advances in CTand MRI, themodality still has an important role inthe diagnosis of various lymphatic pa-thology, including identifying the siteof chyle leak or obstruction. Its use inchildren is also limited by the ability tocannulate the tiny lymphatic vesselsin these patients. Lymphoscintigraphy,on the other hand, is a faster and lesstraumatic procedure requiring just

an intradermal or subcutaneous injec-tion. Its main disadvantage, the lack ofanatomic correlation, has been over-come by recent advances in SPECT/CTimaging. Recent studies have alreadyreported the usefulness of lympho-scintigraphy SPECT/CT for identifyingthe site of lymphatic leakage in adultpatients with TD injuries.18,19 Ourcase suggests that lymphoscintigraphySPECT/CT is also suitable in pediatricpatients presenting with chylothoraxeven when the amount of chyle leakageis minimal. In this neonate, the tech-nique detected the site of lymphaticleakage, guiding the intranodal lymph-angiography followed by TDE. It is be-lieved that after the TD is occluded, newlymphovenous communications devel-op and “dormant” anastomoses re-open.20 Thus, TDEs performed at higherlevels allow a greater number of lym-phovenous communications and bettercollateral circulation. In our patient,the site of the lymphatic leak was low,and every attempt was made to selec-tively embolize only the leaking branch.However, repeated attempts to enterthis tiny vessel proved unsuccessful,and a decision was made to embolizethe TD at and below this point to stopthe leak. Despite a higher risk of sub-sequent complications, low-level embo-lizations including cisterna chyli havebeen described in the literature.21

Furthermore, successful TDEs at thislevel have been previously performedin our center in neonates.22 In this ne-onate, however, a lymphoscintigraphySPECT/CT was requested for the firsttime. We assume this might have beenrelated to the tiny caliber of the leakingvessel, making it undetectable by othermodalities and its subsequent cannu-lation impossible. The case also dem-onstrates that 99mTc sulfur colloidlymphoscintigraphy SPECT/CT can be auseful modality for detecting the chyleleakage site in children with chylo-thorax even when the amount of leak-age is minimal.

FIGURE 3A, Follow-up lymphangiography showing a leftward abnormal lymphatic branch at the level of T10 to T11vertebraewith retrocrural extravasationof contrast in that region (arrow). B, Embolization of the TDwasundertaken using a platinum coil (arrow) with subsequent administration of 0.5 mL of radiopaquecyanoacrylate glue.

CASE REPORT

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MR lymphography with intracutaneousand subcutaneous administration ofvarious lymphotropic paramagnetic con-trast agents is another emerging mo-

dality thatmayopennewperspectives forimaging lymphatic system, including inpatients with chylothorax.23,24 However,most of these lymphotropic contrast

agents are still in the preclinical phaseor under development, and validation inlarger studies is needed before theirroutine use in clinical practice.24

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Lymphoscintigraphy SPECT/CTMinimal Lymphatic Leakage in an Infant With Chylothorax Detected by

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