J of Nuclear Medicine Technology, first published online February 2, 2018 as doi:10.2967/jnmt.117.203281
1 Lymphoscintigraphy of chylous anomalies: chylothorax, chyloperitoneum ,
2 chyluria and lymphangiomatosis. Fifteen year experience in a pediatric setting and
3 review of the literature
4 Dr Sophie Turpin1
5 Dr Raymond Lambert1
6 1. Nuclear Medicine, Medical Imaging
7 CHU Sainte-Justine
8 3175, Chemin de la Côte-Sainte-Catherine
9 Montréal H3T 1C5
10 Téléphone: 514-345-4931
11 Télécopieur: 514-345-2120
12 Correspondance: Dr Sophie Turpin [email protected]
13 No reprints
14 Word Count: main text 2317 , abstract 241 ,whole manuscript 3676
15 Running head: Lymphoscintigraphy for chylous effusions
16
1
17 ABSTRACT
18
19 Objective: In the pediatric setting, lymphoscintigraphy is mostly used for the
20 evaluation of lymphedema . Only a few cases of chylous anomalies and lymphatic
21 malformations imaged with lymphoscintigraphy, have been reported in the literature.
22 The aim of this study was to review the use of lymphoscintigraphy in those pathologies
23 Methods: All lymphoscintigraphies obtained between 2001 and 2017 in our hospital
24 for chylous anomalies, were retrospectively reviewed. Results were correlated to
25 clinical and radiological findings. Lymphoscintigraphy consisted of sequential imaging
26 after injection of 100-250 μci 99mTc-filtered sulfur colloid at the level of the feet
27 and/or hands .
28 Results: Twenty five studies were performed in 21 patients. Fourteen studies were
29 obtained for the evaluation of chylothorax. Eleven were performed for
30 chyloperitoneum , chyluria, chylopericardium, exsudative enteropathy or
31 lymphangiomatosis. Ten studies were positive for lymphatic leak , 1 was dubious. After
32 correlation with radiological findings and follow-up , there were seven true negative
33 and five false negative ( previous 67Ga interfering activity in 1, injection on the hands
34 only in 3 , low lipid diet in 1). One study became positive after injecting on the feet and
35 another one after switching to a high lipid diet.
2
36 Conclusion: Lymphoscintigraphy is an useful tool to image lymphatic anomalies in
37 children . To optimize results , it is suggested to inject the 4 extremities , to have the
38 patient under a high lipid diet, to withhold octreotide and use single photon emission
39 tomography with computed tomography (SPECT/CT).
40
41 Keywords: lymphoscintigraphy; children; chylothorax; chyloperitoneum;
42 lymphangiomatosis.
43
3
44 Introduction
45 Lymphatic anomalies are globally rare in newborns and children but in some cases can
46 lead to significant morbidity and mortality. Of interest are the chylous effusions that can
47 occur in the thorax, the abdomen, the pericardium or the urinary tract. In a tertiary
48 pediatric setting, chylothorax and chyloperitoneum are encountered more and more
49 frequently as patients have surgeries from a very young age for congenital heart disease.
50 Patients with vascular or lymphatic congenital malformations are also referred in a
51 tertiary pediatric center and may need imaging. The purpose of our study was to review
52 our experience with such patients in the last fifteen years.
53 Material and Methods
54 Between 2001 and 2017, 171 lymphoscintigraphic studies were performed in our
55 hospital. After excluding patients evaluated for lymphedema, Klippel-Trenaunay and
56 sentinel node mapping, twenty five studies in 21 patients were done for lymphatic
57 anomalies chylothorax, chylopericardium, chyloperitoneum, chyluria ,
58 lymphangiomatosis or lymphangiectasia as clinical presentation.
59 The study was approved by the Medical Affair Direction, allowing the conduction
60 of this retrospective study on file without the patient`s and parental consents.
61 After explanation of the procedure to the patient and parents, 3.7 MBq (100 uci) in
62 infants and toddlers using a 27G x 0.5 inch needle to 9.25 MBq (250 uci) in the older
63 children using a 25 G x 0.5 inch needle of 99mTc-filtered sulfur colloid were injected
64 using a tuberculine syringe, under sterile conditions, at the level of the feet and/or
4
65 hands. Injected volume was between 0.1 to 0.25 cc. In most cases, intradermal
66 injections were performed at the level of the first interdigital spaces but some very tiny
67 patients had subcutaneous injections. We were not able to inject some patients in the
68 four limbs due to their clinical condition and/or the presence of a saturometer device or
69 IV access. Lymphoscintigraphy consisting of anterior and posterior sequential dynamic
70 images of the regions of interest were performed for 60-120 minutes followed by
71 delayed imaging if needed , including 24 hours study (Figure 1). If present, the drain
72 casing was also imaged.
73 Lymphoscintigraphy results were correlated to clinical and paraclinical data and
74 to follow up. Studies were considered false negative if the abnormalities were proven
75 to be present at time of lymphoscintigraphy by other modalities or clinical findings.
76 Results
77 Out of our 21 patients, 6 were 4 month old and younger, 7 between the ages of
78 10 months and 5 years and the remaining were 8 years and older (Table 1). No patients
79 was receiving octreotide at the time of examination. However, drains were present in
80 some patients and some children were parentally fed.
81 Patients 1 to 7 developed chylothorax following cardiac surgery for congenital
82 heart disease - mostly Fontan and Damus-Kaye-Stansel procedures - or cardiac
83 transplantation in one patient. We had only 2 studies demonstrating pleural
84 accumulation, in patients 2 and 7. Another study, in patient 6 was equivocal for left
5
85 sided chylothorax. Two studies (patient 1 and 3) were true negative. Two studies (
86 patients 4 and 5) did not show any abnormal uptake and were considered false
87 negative. However the patients were injected only on the hands due to clinical
88 instability. Patients 8 to 11 presented themselves with congenital chylothorax. The
89 lymphoscintigraphy was negative in patients 8 to 10. However, patient 8 was injected
90 only on the hands. Patient 10 diagnosis was changed from congenital chylothorax to
91 pseudochylothorax following pneumonia during follow-up. In patient 11, a one-month-
92 old baby also known for gastroschisis, the lymphoscintigraphy was normal after
93 injection on the hands. However, it was positive after injection, a few days later, on the
94 feet (Figure 2).
95 Patients 12 to 14 were known for lymphangiomatosis and chylothorax. Patient
96 12 was a 15-year-old boy with Gorham's disease. He was evaluated for recurent
97 chylothorax and the study was positive after injection in both hands. (Figure 3). Patient
98 13 lymphoscintigraphy was non diagnostic due to 67Ga interference from a previous
99 study. Patient 14 was imaged three times between age 12 and 15 . She had a history of
100 congenital chylothorax and hydrops fetalis. At age 12, she was evaluated for
101 chylothorax and the study was a true negative. At age 15, she was imaged again for
102 lymphatic malformations and chylopericardium necessitating drainage. After a normal
103 lymphoscintigraphy , the patient was switched from a normal diet to a high lipid diet.
104 Control lymphoscintigraphy demonstrated soft tissue lymphangiomatosis but while
105 there was no frank accumulation in the pericardium, some activity was demonstrated in
6
106 the drainage bag (Figure 4). Patient 15 had a history of recurrent chylothorax and
107 chyloperitoneum since birth. The underlying pathology was also lymphangiomatosis.
108 She was imaged twice and her lymphatic studies were true negative.
109 Patient 16 to 20 were evaluated for chyloperitoneum and/or for the possibility
110 of intestinal lymphangiectasia. Patient 16 was a ten-month-old patient with complex
111 cardiopathy, status post Damus-Kaye-Stansel and Right Ventricle - Pulmonary Artery
112 conduit cardiac surgery and gastrostomy. He developed chyloperitoneum which was
113 demonstrated on the lymphoscintigraphy after injection in the four extremities ( Figure
114 5). Patient 17 was also a ten-month-old patient with recurrent chyloperitoneum
115 following sclerotherapy. After injection in both feet, extravasation of the tracer was
116 demonstrated in the region of the cisterna chyli. Abnormal dysplasic lymphatics with
117 intra-abdominal leak were confirmed by lymphography (Figure 6).
118 Finally, patient 21 was evaluated for chyluria, without history of previous
119 cancer, surgery or filariasis. No leak was demonstrated on lymphoscintigraphy .
120 Investigation over a period of three years did not establish the origin of the chyluria.
121 Discussion
122 The thoracic duct is the main collecting system of the body's lymph and chyle.
123 Lymph originating from the abdomen and lower extremities drains through lumbar
124 lymph nodes to the cisterna chyli located in front of L2. The thoracic duct then travels
125 in the retrocrural space on the right side of the aorta , then crosses the midline at the
7
126 level of T5 , through the aortic arch to drain in the left subclavian vein (1,2,3,4) . The
127 right side of the upper body and the head drains into the right lymphatic duct which
128 empties in the right subclavian vein (Figure 7). Anatomical variations are frequent (3).
129 Chyle is responsible for ingested fats' transport, most of it originating from the
130 liver and gastro intestinal tract. Chyle flow increases significantly after a meal,
131 depending on the diet , potentially influencing lymphoscintigraphy (2,4,5). Transport
132 along the thoracic duct is mediated by pressure gradient between the thorax and the
133 abdomen, tissue hydrostatic pressure and Bernoulli`s effect at the entrance of the
134 subclavian vein (3,5) . More than 2 liters of lymph empty in the circulation each day.
135 Chyle is responsible for the transport of lipids from the GI tract into the circulation but
136 also for the transfer of fluids , proteins and lymphocytes between the interstitial and the
137 intravascular compartments (6) .
138 Chylothorax is probably the most frequent type of abnormal chylous
139 accumulation. It can be caused by non iatrogenic trauma, surgery, invasive diseases and
140 finally it can be congenital (3,5,7) . Congenital chylothorax is found in the neonatal
141 period and can be associated malformations such as Noonan`s disease or Down's
142 syndrome, hydrops fetalis, congenital lymphangiectasia or lymphangiomatosis. It is
143 suggested that the presence of a congenital weakness associated with the trauma of
144 birth may cause congenital chylothorax in some patients (3).
145 Due to the localization of the thoracic duct, post traumatic chylothorax is more
146 frequent on the right side in adults (8) . However, in children , it can be found on the
8
147 left side, following cardiac surgery involving the aortic region ( esophageal atresia
148 repair, aortic coarctation repair, Blalock-Taussig .... ) (9). It is most frequent following
149 heart transplantation and Fontan procedures and chyloperitoneum may also occurs (10).
150 Post surgical chylothorax is rare in adults, as a complication of 0.25 to 0.5% of
151 intrathoracic procedures but more frequent in children, between 2.5 and 4.7% (5,10).
152 Finally, mostly in adults, chylothorax may complicate lymphoma, esophageal cancer ,
153 tuberculosis, filiariasis, lymphangioleiomyomatosis and Gorham`s disease as in some
154 of our patient.
155 For the evaluation of chylous effusion, the lymphoscintigraphy procedure is
156 similar to the one used for lymphedema. However, the 4 extremities must be injected ,
157 which can be difficult in very young patients or in intensive care patients. Due to the
158 lack of availability of 99mTc antimony, 99mTc Dextran and 99mTc nano-albumin, the
159 radiotracer of choice is Tc99-m filtered sulfur colloid. Intradermal injections in the
160 interdigital spaces are usually used but can be replaced by subcutaneous ones. Injected
161 activities range from 3.7 to 9.25 MBq in small volumes around 0.1 ml. More invasive
162 subfascial injections, allowing the evaluation of the deep lymphatic system are not used
163 in our institution(1). Treatment options include parenteral low fat diet and octreotide to
164 reduce the intestinal chyle production, drainage and thoracic duct ligation (4,10).
165 Withholding medication or reintroducing a high fat diet may be needed to decrease the
166 possibility of false negative results as in patient 14.
9
167 Only a few cases of lymphoscintigraphies performed in very young patients are
168 found in the literature: a 2 months old boy with congenital chylothorax (11) and a
169 neonate with chylothorax following cardiac surgery (12). We had 9 patients less than
170 one year old in our population and some of those patients were more of a challenge to
171 inject.
172 Chylothorax is differentiated from pseudo chylothorax by its high triglyceride
173 contents (4), the latter being a complication of long standing exusdate rich in
174 cholesterol due to fatty degeneration of cells (7,8). Pseudochylothorax following lung
175 infection was eventually demonstrated in patient 10.
176 Among rare causes of chylothorax, we can include lymphangioleiomyomatosis
177 and haemangiomatosis (4). Lymphangioleiomyomatosis is characterized by smooth
178 muscle proliferation in the lungs, lymph nodes and thoracic duct. It is found almost
179 exclusively in women and leads to respiratory failure. A majority of patients will
180 present themselves with chylothorax (4,7). Haemangiomatosis or Gorham`s disease is
181 characterized by vascular and lymphatic proliferation in the bones or in the soft tissues.
182 Intrathoracic lesions, such as in one of our patient , are found in less than 1% of cases
183 (4,13). Complications include chylothorax, disseminated intravascular coagulation,
184 infection and malnutrition.
185 Some of our patients had chyloperitoneum, most of the time associated with
186 other pathologies. Chyloperitoneum can be exsudative, with chyle retrodiffusion,
187 following central obstruction by malignancy, infection or fistulous with the presence
10
188 of enlarged retroperitoneal lymphatics (14) . Chyloperitoneum may also occur
189 following trauma or surgery as in patient 16 or be congenital in origin, associated with
190 intestinal lymphangiectasia as in patient 15 or with lymphangiomatosis as in patient 17
191 (15) .
192 Primary intestinal lymphangiectasia is characterized by intestinal
193 megalymphatics and protein-losing enteropathy and lymphoscintigraphy can
194 demonstrated abnormal accumulation of the tracer not only in the digestive tract but in
195 various effusion as shown in 41 patients by Wen et al (16).
196 Chyluria (14) is secondary to a fistula between the para aortic lymphs nodes
197 and the kidney, with backflow toward the renal lymph nodes and extravasation in the
198 collecting system. It is associated with filariasis, cancer, abdominal surgery , including
199 live-donor nephrectomy, renal transplantation and oncological procedures (17,18,19).
200 or lymphangiitis (20). Forty-one patients with chyluria were imaged using 99mTc-
201 Dextran and only the early appearance of kidney or pelvis activity was indicative of
202 lymphourinary fistula (20). We did not find any early urinary leak in our last patient
203 imaged, even after using SPECT/CT to improve visualization of the thoracic duct (21).
204 Chylopericardium is very rare, either secondary to congenital malformations or as
205 consequence of trauma, surgery, Gorham's disease or tumors (22). Chylopericardium
206 could not be clearly demonstrated in patient 15.
11
207 One of the largest study using lymphoscintigraphy in chylothorax, chyloperitoneum and
208 chyluria was published in 1998 (23). As the study originated from Asia, 11 out the 18
209 patients presented themselves with chyluria secondary to filiaris. The authors used
210 99mTc-Dextran or 99mTc-Antimony. There were no children in their cohort. Five
211 studies were normal and the remaining 6 demonstrated obstruction (5) or lymphorenal
212 fistula (1). The remaining 7 patients presented a combination of chylothorax / chyluria/
213 chyloperitoneum of various but non malignant etiologies. Enhanced lymph flow was
214 found in one, lymph reflux in another patient and obstruction in the remaining 5.
215 Lymphoscintigraphy was conclusive in 72% of their patients.
216 In an European study of 16 adult patients (14), diagnosis was obtained by
217 lymphoscintigraphy in 4, CT scan in 6 and combination of both modalities in 6. Five
218 patients had chyluria, 8 chylothorax and 4 chyloperitoneum. Only 2 of 5 cases of
219 chyluria were secondary to filiaris. Nine patients had chyle leak following surgery for
220 neoplasia and 2 secondary to tuberculosis.A traumatic origin was therefore the most
221 frequent and not filiaris.
222 The only large pediatric study using lymphoscintigraphy included 5 neonates and 10
223 patients between 1.5 yo and 8 yo (24). The population was different from ours as most
224 of the children affected had lymphatic dysplasia, including congenital aplasia and
225 hypoplasia, with lymphedema. Chylous effusion were present but not the main clinical
226 pattern in most.
12
227 Single-photon emission computed tomography with computed tomography
228 (SPECT-CT) has shown to improve localization is suggested and We have started to
229 use SPECT-CT in some of our more recent patients (21,25,26,) with proper
230 immobilization precluding the use of sedation. More recent equipment may improve
231 even detection of small leakage (27). An alternative would to fuse the SPECT images
232 with contemporary diagnostic CT or magnetic resonance imaging.
233 Conclusion:
234 Lymphoscintigraphy can be performed in children in a variety of settings such as
235 chylothorax, chylopericardium, chyloperitoneum, chyluria, lymphangiectasia and
236 lymphangiomatosis. While technically challenging in babies due to their small size, the
237 test is minimally invasive, with low radiation exposure and no side effects, in infants
238 and children that otherwise undergo major morbidity . We recommend to inject the four
239 limbs, to have the patient under a fatty diet, without octreotide and use SPECT-CT to
240 improve diagnostic accuracy.
241 Financial Disclosure
242 There are no financial interests, direct or indirect, that exist or may be perceived to
243 exist for individual contributors in connection with the content of this paper.
244 There are no sources of outside support for this project
245
246 Conflicts of interest:
13
247
248 Sophie Turpin: No conflict of interest to declare
249 Raymond Lambert : No conflict of interest to declare
250
14
251 References:
252 1. Szuba A, Shin WS, Strauss HW, Rockson S. The third circulation:
253 radionuclide lymphoscintigraphy in the evaluation of lymphedema. J Nucl
254 Med. 2003;44:43-57
255 2. Kato T, Takase K, Ichikawa H, Satomi S, Takahashi S. Thoracic duct
256 visualization: combined use of multidetector-row computed tomography and
257 magnetic resonance imaging. J Comput Assist Tomogr. 2011;35:260-265
258 3. Bessone LN, Ferguson TB, Burford TH. Chylothorax. Ann Thor Surg.
259 1971;12:527-550
260 4. McGrath EE, Blades Z, Anderson PB. Chylothorax: aetiology, diagnosis and
261 therapeutic options. Resp Med. 2010;104:1-8
262 5. Valentine VG, Raffin TA. The management of chylothorax. Chest. 1992;
263 102:586-591.
264 6. Zuluaga MT. Chylothorax after surgery for congenital heart. Curr Opin
265 Pediatr. 2012;24:291-294
266 7. Hillerdal G. Chylothorax and pseudochylothorax. Eur Resp J. 1997;10:1157-
267 1162
268 8. Macnab DS, Scarlett EP. Traumatic chylothorax due to intrathoracic rupture of
269 the thoracic duct. Canadian Med Ass J. 1932;27:29-36.
270 9. Fairfax AJ, McNabb WR, Spiro SG. Chylothorax: a review of 18 cases.
271 Thorax.1986;41:880-885.
15
272 10. Chan EH, Russell JL, Williams WG, Van Arsdell GS, Coles JG, McCrindle
273 BW . Post operative chylothorax after surgery in children. Ann Thorac Surg.
274 2005;80:1864-1871.
275 11. Kuang-Tao Y. Detection of chylothorax and cervical cystic hygroma in
276 hydrops fetalis using lymphoscintigraphy. Clin Nucl Med. 2006;31:205-206.
277 12. Moadel-Sernick R, Crooke GA, Freeman L. Lymphoscintigraphy
278 demonstrating thoracic duct injury in an infant with hypoplastic left heart
279 syndrome. Clin Nucl Med. 2000;25:335-336.
280 13. Fukahori S, Tsuru T, Asagiri K, et al. Thoracic lymphangiomatosis with
281 massive chylothorax after a tumor biopsy and with disseminated intravenous
282 coagulation - lymphoscintigraphy, an alternative minimally invasive imaging
283 technique: report of a case. Surg Today. 2011;41:978-982
284 14. Deso S, Ludwig B, Kabutey NK, Kim D, Guermazi A.
285 Lymphangioscintigraphy in the diagnosis and localization of various chyle
286 leaks. Cardiovasc Intervent Radiol. 2012;35:117-126.
287 15. Noel AA, Gloviczki P, Bender CE, Whitley D, Stanson AW, Deschamps C.
288 Treatment of symptomatic primary chylous disorders. J Vascul Surg. 2001;
289 38:785-791.
290 16. Wen Z, Tong G, Liu Y, Meeks JK, Ma D, Yang J. The lymphoscintigraphic
291 manifestation of 99mTc-Dextran lymphatic imaging in primary intestinal
292 lymphangiectasia. Nucl Med Comm. 2014;35:493-500
16
293 17. Guglielmo N, Melandro F, Nudo F, et al. Chylous leakage after a laparoscopic
294 live-donor nephrectomy: case report and literature review. Experiment Clin
295 Transplant. 2016;3:338-340
296 18. Oh JK, Yoon HE, Chung YA. Lymphoscintigraphic demonstration of chyle
297 leak after kidney transplantation and gamma camera detection of radioactivity
298 in chylous aspirate. Clin Nucl Med. 2014;39:760-761
299 19. Lv S, Wang Q, Zhao W, et al. A review of the postoperative lymphatic
300 leakage. Oncotarget. 2017;8:69062-69075
301 20. Yuan Z, Luo Q, Chen L, Luo Q, Zhu R. The role of lymphoscintigraphy in
302 chyluria. Hell J Nucl Med. 2010;13:238-240.
303 21. Prevot N, Tiffet O, Avet J Jr, Quak E, Decousus M, Dubois F.
304 Lymphoscintigraphy and SPECT/CT using 99mTc filtered sulfur colloid in
305 chylothorax. Eur J Nucl Med Mol Imag. 2011;38:1746.
306 22. Mandarry MT, Ru XH, Wei ZQ. Primary idiopathic chylopericardium; a rare
307 case with a synopsis of the literature. Sing Med J. 2012;53:e156.
308 23. Pui MH, Yueh TC . Lymphoscintigraphy in chyluria, chyloperitoneum and
309 chylothorax J Nucl Med. 1998;39:1292-1296
310 24. Bellini C, Boccardo F, Campisi C, Villa G, Taddei G, Traggiai C, Bonioli E.
311 Lymphatic dysplasias in newborns and children: the role of
312 lymphoscintigraphy. J Pediatr. 2008;152:587-589
17
313 25. Weiss M, Schwarz F, Wallmichrath J, et al. Chylothorax and chylous ascites.
314 Clinical utility of planar scintigraphy and tomographic imaging with
315 SPECT/CT. Nuklearmedizin. 2015;54:197-240
316 26. Das J, Thambudori R, Soumendranath R. Lymphoscintigraphy combined with
317 single-photon emission computed tomography - computed tomography
318 (SPECT-CT): a very effective imaging approach for identification of the site
319 of leak in postoperative chylothorax. Indian J Nucl Med. 2015;30:177-179
320 27. Yang J, Codreanu I, Zhuang H. Minimal lymphatic leakage in an infant with
321 chylothorax detected by lymphoscintigraphy SPECT/CT. Pediatrics.
322 2014;134:e606-e610
323
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324 FIGURES
325
326 Figure 1: Normal lymphoscintigraphy after injection in the upper and lower extremities
327
19
328
329 Figure 2: Congenital Chylothorax. No abnormal accumulation after injection in both
330 hands: (A) anterior view (top) and posterior view (bottom) of the thorax with activity in
331 the axillary lymph nodes. Thoracic accumulation of the tracer (arrows), including in the
332 thoracic canal region after injection in both feet: (B) anterior view (top) and posterior
333 view (bottom) with visualization of the ilio-inguinal lymph nodes.
20
334
335 Figure 3: Gorham's disease. Abnormal accumulation of tracer in the mediastinum
336 (arrow) after injection on both upper and lower extremities (A). Magnetic Resonance
337 Imaging: diffuse infiltration of the mediastinum (arrow) secondary to
338 lymphangiomatosis (B) and presence of loculated chylothorax (arrow)(C).
339
21
340
341 Figure 4: Lymphangiomatosis in patient with chylopericardium necessitating drainage.
342 Normal study with standard diet: injection in the upper (A) and lower (C) extremities.
343 No activity in the collecting bag (E). Left shoulder and right inguinal region
344 lymphangiectasia (thin arrows) on lymphoscintigraphy performed after switching the
345 patient to high fat content diet: injection in the upper (B) and lower (D) extremities.
346 Activity (thick arrow) in the collecting bag (F).
22
347
348 Figure 5: Chyloperitoneum. After injection in the 4 extremities, abnormal
349 accumulation of tracer in the abdomen at 1 hour: anterior view (A), left lateral view (C)
350 and at 5 hours: anterior view (B) and left lateral view (D).
351
352
23
353
354 Figure 6: After injection in the lower extremities, lymphoscintigraphy image at 1 hour
355 demonstrating leak (arrow) (A). Diffuse intra-abdominal accumulation at 5 hour post
356 injection (B). Conventional lymphography showing dysplasic lymphatics (arrow) and
357 intra-abdominal leak (C)
358
24
359
360 Figure 7: Anatomy of the lymphatic system. Adapted from Wikimedia Commons
361
25
362 TABLE 1: Patients Characteristics: TN = True Negative; TP = True Positive; FN =
363 False Negative; TP = True Positive; F = female; M = male.
Patient Age Sex Presentation 1 11 months F Chylothorax post cardiac surgery TN 2 4 years M Chylothorax post cardiac surgery TP 3 4 months M Chylothorax post cardiac surgery TN 4 5 years F Chylothorax post cardiac surgery FN 5 1 months F Chylothorax post cardiac surgery FN 6 18 years M Chylothorax post cardiac surgery TP or FN 7 2 months M Chylothorax post cardiac surgery TP 8 1 month M Idiopathic Congenital Chylothorax FN 9 1 month F Idiopathic Congenital Chylothorax TN 10 4 years F Pseudo Chylothorax TN 11 1 month M Congenital Chylothorax /Gastrochisis TP 12 15 years M Chylothorax /Lymphangiomatosis TP 13 13 years F Chylothorax /Lymphangiomatosis FN 14 14 years F Chylothorax /Lymphangiomatosis See text 15 3 years F Chyloperitoneum/Lymphangiectasia TP 16 10 months M Chyloperitoneum post cardiac surgery TP 17 10 months M Chyloperitoneum/Lymphangiomatosis TP 18 9 years F Abdominal Lymphangiomatosis TP 19 16 years F Exsudative enteropathy TN 20 16 years M Exudative enteropathy TN 21 8 years M Chyluria NA
364
26