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Chapter 54
Medical Management
Francine Blei
Chylorrhea (“Chyle leak”) occurs when chyle (see Table 54.1), a milky, triglyceride-rich
liquid, extravasates, most commonly into the peritoneal cavity (chylous ascites) or
pleural space (chyloperitoneum). Chyle leakage can also result in chyluria (chyle in
the urine), chyloptysis (chyle in the sputum), chylopericardium, or cutaneous chyle
leakage.
Chyle is a creamy white fluid formed in intestinal lacteals during digestion. It is
normally transported through the lymphatics, entering the venous circulation via the
thoracic duct. Chyle is rich in triglycerides, protein, and white blood cells, especially T lymphocytes. Accordingly, patients with chylorrhea have low serum levels
of protein, lymphocytes, and fat-soluble vitamins, and develop a metabolic acidosis
due to electrolyte imbalances. Immune dysfunction can arise through loss of immunoglobulins and lymphocytes1 (Table 54.2).
Obstruction or direct trauma to the lymphatics may lead to leakage of chyle into
the pleural or peritoneal cavity. Chyle leaks can occur in the prenatal, pediatric, and
adult populations, with age-related etiological correlation. In adults, chyle leaks are
more commonly related to malignancy. Symptoms of chyle leak can be acute or
insidious, ranging from nonspecific discomfort to respiratory distress and abdominal distension. During normal digestion, chylomicrons are absorbed by the small
intestines, then traverse the omental lymphatics to the cisterna chyli, anterior to the
lumbar vertebrae. The cisterna chyli connects to the lumbar, thoracic, and hepatic
lymphatics, ultimately forming the thoracic duct, which deposits lymph (all but that
from the right chest, arm, neck, and head) into the venous system. When normal
lymphatic vessels are obstructed (e.g., hypoplasia, extrinsic compression, fibrosis),
or damaged (e.g., trauma), chylous leaks (also called chylous effusions) can occur.
Chylothorax can result from trauma (after cardiac, pulmonary, esophageal, or
head and neck surgery; thoracic duct trauma; cardiopulmonary resuscitation; central
F. Blei
Vascular Birthmark Institute,
Roosevelt Hospital, New York, NY, USA
B.-B. Lee et al. (eds.), Lymphedema,
DOI 10.1007/978-0-85729-567-5_54, © Springer-Verlag London Limited 2011
449
450 F. Blei
Table 54.1 Properties of chyle
High protein (albumin)
High triglyceride level (>110 mg/dL)
White blood cells – especially T cells
Table 54.2 Consequences of chyle leak
Hypoproteinemia (e.g., loss of albumin)
Lymphopenia
Hypocalcemia
Hyponatremia
Metabolic acidosis
Deficiencies of fat-soluble vitamins (A, D, E, K)
Immunocompromise/susceptibility to infection (loss of immunoglobulins, T lymphocytes)
venous catheter placement; or diaphragmatic hernia repair), malignancy, congenital
defects, or infections (e.g., tuberculosis). Trauma-related chyle leaks can be due to
blunt damage to the thoracic duct or to incomplete ligation of the lymphatic vessels.
Chylothorax may also result from lymphatic malformations, lymphangiectasia,
lymphangioleiomyomatosis, lymphatic obstruction (e.g., due to congenital anomalies or hypoplasia, or as a consequence of malignancy).
2-5
Chylothorax has a high
morbidity, often presenting as a pleural effusion, and diagnosis can be made by
analysis of fluid from pleurocentesis. Chylothorax may be associated with chyloptysis or chyle-containing sputum.
Lymphoscintigraphy or lymphangiography may be useful to define anatomy.
6
7,8
Shackcloth recommends administration of ingested cream to patients prior to esophageal surgery, because this intervention helps to identify the thoracic duct and surrounding lymphatic channels, decreasing the chance of trauma and post-operative
chylothorax.
9
Chylothorax can be identified (and treated) prenatally.10 Caserio, reported 29
cases of congenital chylothorax, 94% diagnosed in utero, 66.7% of which had fetal
hydrops, with an overall survival rate of 56% at 3 years of age.11 Ergaz reported 11
neonates with congenital chylothorax, nine of whom were diagnosed prenatally,
five of whom underwent intrauterine pleurocentesis.12 Most of the patients (8) had
chromosomal abnormalities.
Chylous ascites, due to disruption of the abdominal lymphatics, can manifest as
abdominal distention or dyspnea. In adults, this is most commonly due to malignant
obstruction. Other etiologies include lymphangiectasia, cirrhosis, trauma, and lymphangioleiomyomatosis.13 Trauma-induced chylous ascites may be due to secondary
portal hypertension, which can disrupt the integrity of the lymphatics. Retroperitoneal
lymph node dissection, abdominal aortic aneurysm, and renal surgeries are most
commonly associated with chylous ascites (Table 54.3).
14
Treatment of chylorrhea often begins with dietary modification: enteral nutrition with either a no fat or a low-fat diet and medium chain triglycerides (MCT)
54 Medical Management
Table 54.3 Etiologies of chyle leakage
Chylous pleural effusion Chylous ascites
Cardiac surgery Abdominal, spinal, renal surgery
Trauma to thoracic duct or lacteals Trauma to lacteals
Radiation Radiation
Malignancy Malignancy
Indwelling intravenous port placement Cirrhosis, chronic liver disease
Lymphangiectasia Lymphangiectasia
Lymphangioleiomyomatosis Lymphangioleiomyomatosis
Lymphatic malformation Lymphatic malformation
Congenital anomalies of thoracic duct or lymphatics
Infection (e.g., tuberculosis)
451
oil. MCTs are directly absorbed into the portal system, bypassing the lymphatics.
Total parenteral nutrition with bowel rest may also ameliorate chyle leaks. If
nutritional measures are inadequate, medical or surgical interventions may be
effective.
Medical Therapies for Chylorrhea
Octreotide (Sandostatin, Novartis Pharmaceuticals) is a long-acting somatostatin
analog that inhibits many hormones (including growth hormone, glucagon, and
insulin), diminishes secretions from the intestine and pancreas, and decreases intestinal motility. This drug is approved by the Food and Drug Administration for the
treatment of acromegaly and carcinoid syndrome; however, there are many published reports documenting efficacy in managing primary or secondary chylothorax
and chylous ascites,
5,14-25
including in newborns.26 Octreotide may indirectly decrease
chyle secretion via binding to lymphatic somatostatin receptors and reducing bile
acid secretion into the intestine. It has also been effective in traumatic thoracic duct
trauma-related chyle leak.
27
There are several reports of successful antenatal (in utero) treatment with intra-
pleural OK-432 (Picibanil) for fetal chylothorax.
28-30
Matsukum showed successful
results with postnatal treatment with OK-432 for unrelenting octreotide-resistant
congenital chylothorax in newborns.
31
Intravenous etilefrine hydrochloride was shown to decrease chyle leaks in eight
out of ten patients. The authors conclude: “by inducing contraction of the smooth
muscle fibres present in the wall of the main thoracic chyle ducts, etilefrine can be
considered as a useful adjunct in the management of post-operative chyle leak.”32
One report demonstrates that sirolimus (Rapamycin, Wyeth Pharmaceuticals) ameliorated after lung transplant chylothorax in a patient with lymphangioleiomyoma-
33
tosis.
There is one report of Factor XIII infusion for recalcitrant chylothorax after
lung transplantation in a patient with underlying lymphangioleiomyomatosis.
34
452 F. Blei
Percutaneous embolization may be effective
35-37
for recurrent chylothorax after
neck surgery, with percutaneous thoracic duct embolization as primary treatment.
What is evident is that there is no consensus for the management of chylorrhea,
nor have there been any randomized case control studies.
Non-surgical treatments for chylorrhea
Octreotide
OK432
Etiliefrine
Sirolimus
Factor XIII
Sclerotherapy
Radiation
22,38
References
1. Spain D, McCLure S. Chylothorax and chyous ascites. In: Gottschlich M, Fuhrman M,
Hammond K, eds. The Science and Practice of Nutrition Support: A Case-Based Core
Curriculum. Dubuque: Kendall/Hunt Publishing; 2000:479-489.
2. McGrath EE, Blades Z, Anderson PB. Chylothorax: aetiology, diagnosis and therapeutic
options. Respir Med. 2010;104(1):1-8.
3. Teichgraber UK, Nibbe L, Gebauer B, Wagner HJ. Inadvertent puncture of the thoracic duct during
attempted central venous catheter placement. Cardiovasc Intervent Radiol. 2003;26(6):569-571.
4. Fishman SJ, Burrows PE, Upton J, Hendren WH. Life-threatening anomalies of the thoracic
duct: anatomic delineation dictates management. J Pediatr Surg. 2001;36(8):1269-1272.
5. Soto-Martinez M, Massie J. Chylothorax: diagnosis and management in children. Paediatr
Respir Rev. 2009;10(4):199-207.
6. Lim KG, Rosenow EC 3rd, Staats B, Couture C, Morgenthaler TI. Chyloptysis in adults: pre-
sentation, recognition, and differential diagnosis. Chest. 2004;125(1):336-340.
7. Le Pimpec-Arthes F, Badia A, Febvre M, Legman P, Riquet M. Chylous reflux into localized
pulmonary lymphangiectasis. Ann Thorac Surg. 2002;74(2):575-578.
8. Sashida Y, Nishizeki O, Higaonna K, Arashiro K. Blunt thoracic duct injury in the neck diag-
nosed by lymphoscintigraphy. J Plast Reconstr Aesthet Surg. 2008;61(9):1114-1115.
9. Shackcloth MJ, Poullis M, Lu J, Page RD. Preventing of chylothorax after oesophagectomy by
routine pre-operative administration of oral cream. Eur J Cardiothorac Surg. 2001;20(5):
1035-1036.
10. Dendale J, Comet P, Amram D, Lesbros D. Prenatal diagnosis of chylothorax. Arch Pediatr.
1999;6(8):867-871.
11. Caserio S, Gallego C, Martin P, Moral M, Pallas C, Galindo A. Congenital chylothorax: from
foetal life to adolescence. Acta Paediatr. 2010;99(10):1571-1577.
12. Ergaz Z, Bar-Oz B, Yatsiv I, Arad I. Congenital chylothorax: clinical course and prognostic
significance. Pediatr Pulmonol. 2009;44(8):806-811.
13. Gaba RC, Owens CA, Bui JT, Carrillo TC, Knuttinen MG. Chylous ascites: a rare complica-
tion of thoracic duct embolization for chylothorax. Cardiovasc Intervent Radiol. 2010;34(suppl
2):245-249.
14. Ferrandiere M, Hazouard E, Guicheteau V, et al. Chylous ascites following radical nephrec-
tomy: efficiency of octreotide as treatment of a ruptured thoracic duct. Intensive Care Med.
2000;26(4):484-485.
54 Medical Management
15. Ulibarri JI, Sanz Y, Fuentes C, Mancha A, Aramendia M, Sanchez S. Reduction of lymphor-
rhagia from ruptured thoracic duct by somatostatin. Lancet. 1990;336(8709):258.
16. Bliss CM, Schroy IP. Primary intestinal lymphangiectasia. Curr Treat Options Gastroenterol.
2004;7(1):3-6.
17. Maayan-Metzger A, Sack J, Mazkereth R, Vardi A, Kuint J. Somatostatin treatment of con-
genital chylothorax may induce transient hypothyroidism in newborns. Acta Paediatr.
2005;94(6):785-789.
18. Chan SY, Lau W, Wong WH, Cheng LC, Chau AK, Cheung YF. Chylothorax in children after
congenital heart surgery. Ann Thorac Surg. 2006;82(5):1650-1656.
19. Siu SL, Lam DS. Spontaneous neonatal chylothorax treated with octreotide. J Paediatr Child
Health. 2006;42(1–2):65-67.
20. Barili F, Polvani G, Topkara VK, et al. Administration of octreotide for management of post-
operative high-flow chylothorax. Ann Vasc Surg. 2007;21(1):90-92.
21. Mincher L, Evans J, Jenner MW, Varney VA. The successful treatment of chylous effusions in
malignant disease with octreotide. Clin Oncol (R Coll Radiol). 2005;17(2):118-121.
22. Panthongviriyakul C, Bines JE. Post-operative chylothorax in children: an evidence-based
management algorithm. J Paediatr Child Health. 2008;44(12):716-721.
23. Pratap U, Slavik Z, Ofoe VD, Onuzo O, Franklin RC. Octreotide to treat postoperative chy-
lothorax after cardiac operations in children. Ann Thorac Surg. 2001;72(5):1740-1742.
24. Valentine CN, Barresi R, Prinz RA. Somatostatin analog treatment of a cervical thoracic duct
fistula. Head Neck. 2002;24(8):810-813.
25. Markham KM, Glover JL, Welsh RJ, Lucas RJ, Bendick PJ. Octreotide in the treatment of
thoracic duct injuries. Am Surg. 2000;66(12):1165-1167.
26. Bulbul A, Unsur EK. Octreotide as a treatment of congenital chylothorax. Pediatr Pulmonol.
2010;45(6):628. author reply 9–30.
27. Rosing DK, Smith BR, Konyalian V, Putnam B. Penetrating traumatic thoracic duct injury
treated successfully with octreotide therapy. J Trauma. 2009;67(1):E20-E21.
28. Takahashi M, Kurokawa Y, Toyama H, Hasegawa R, Hashimoto Y. The successful manage-
ment of thoracoscopic thoracic duct ligation in a compromised infant with targeted lobar deflation. Anesth Analg. 2001;93(1):96-97.
29. Chen M, Chen CP, Shih JC, et al. Antenatal treatment of chylothorax and cystic hygroma with
OK-432 in nonimmune hydrops fetalis. Fetal Diagn Ther. 2005;20(4):309-315.
30. Nygaard U, Sundberg K, Nielsen HS, Hertel S, Jorgensen C. New treatment of early fetal
chylothorax. Obstet Gynecol. 2007;109(5):1088-1092.
31. Matsukuma E, Aoki Y, Sakai M, et al. Treatment with OK-432 for persistent congenital chy-
lothorax in newborn infants resistant to octreotide. J Pediatr Surg. 2009;44(3):e37-e39.
32. Guillem P, Papachristos I, Peillon C, Triboulet JP. Etilefrine use in the management of post-
operative chyle leaks in thoracic surgery. Interact Cardiovasc Thorac Surg. 2004;3(1):156-160.
33. Ohara T, Oto T, Miyoshi K, et al. Sirolimus ameliorated post lung transplant chylothorax in
lymphangioleiomyomatosis. Ann Thorac Surg. 2008;86(6):e7-e8.
34. Shigemura N, Kawamura T, Minami M, et al. Successful factor XIII administration for persis-
tent chylothorax after lung transplantation for lymphangioleiomyomatosis. Ann Thorac Surg.
2009;88(3):1003-1006.
35. Repko BM, Scorza LB, Mahraj RP. Recurrent chylothorax after neck surgery: percutaneous thoracic
duct embolization as primary treatment. Otolaryngol Head Neck Surg. 2009;141(3):426-427.
36. Patel N, Lewandowski RJ, Bove M, Nemcek AA Jr, Salem R. Thoracic duct embolization: a
new treatment for massive leak after neck dissection. Laryngoscope. 2008;118(4):680-683.
37. Itkin M, Kucharczuk JC, Kwak A, Trerotola SO, Kaiser LR. Nonoperative thoracic duct embo-
lization for traumatic thoracic duct leak: experience in 109 patients. J Thorac Cardiovasc Surg.
2010;139(3):584-589. discussion 9–90.
38. Smoke A, Delegge MH. Chyle leaks: Consensus on management? Nutr Clin Pract. 2008;
23(5):529-532.
453
Chapter 55
Surgical Management
Richard G. Azizkhan and Jesse A. Taylor
Introduction
This chapter focuses on surgical interventions for treating chyle reflux. As with
many areas of vascular medicine, multidisciplinary management is the norm, with
surgeons, medical specialists, and interventional radiologists all playing important
roles. An understanding of chyle transportation from the bowel lacteals to the
thoracic duct and venous system is important in planning treatment. When flow is
disrupted, the anatomic location of a blockage or rupture in this ductal system may
translate into chylous ascites, chylothorax, chylous cyst, chylopericardium, chyloptysis, chyluria, and skin lesions with chylorrhea. The goal of therapy is to restore
balance to the lymphatic system through decreased lymph production, facilitation of
physiological lymphatic pathways, or removal of regions of lymphatic dysplasia.
Drainage Procedures
Removal of pooled chylous fluid is another adjunct to surgical treatment. For example, compression garments and mechanical massage have shown good results in
limiting and/or controlling lymphedema, cellulitis, and lymphangitis in extremities.1 When disrupted flow results in a chylothorax or chylous ascites, thoracentesis
and paracentesis may be used to relieve symptoms of respiratory failure and improve
hemodynamics. Wheeler et al.2 have described thoracentesis as a means of treating
life-threatening neonatal tension chylothorax. To obtain continuous drainage, tube
thoracostomy has been shown to be effective. In a study of 29 patients, Marts et al.3
R.G. Azizkhan (*)
Department of Surgery and Pediatrics,
Cincinnati Children’s Hospital Medical Center,
Cincinnati, OH, USA
B.-B. Lee et al. (eds.), Lymphedema,
DOI 10.1007/978-0-85729-567-5_55, © Springer-Verlag London Limited 2011
455
456 R.G. Azizkhan and J.A. Taylor
were able to combine medium chain triglyceride (MCT) therapy with chest tube
placement for a cure rate of 79% of adult patients presenting with a traumatic or
iatrogenic chylothorax. Though there is no consensus as to when drainage procedures should be abandoned in favor of surgery, most authors recommend surgery for
persistent disease after two attempts at drainage.
1-3
The literature does not support drainage procedures as primary treatment for
cutaneous chyle reflux, especially in the setting of lymphangiomyomatosis.
4,5
Converting an internally draining system into an externally draining system may
worsen chyle losses and hasten malnutrition.
Image-Guided Approaches
Dilated lymphatic channels and resultant chylorrhea are often the most symptomatic manifestation of chyle reflux. The cutaneous manifestation presents as milialike lesions often in the external genitalia, thigh, or trunk.
megalymphatics, dilated channels, and “lakes,” luminal obliteration has become
increasingly utilized. Toxic agents may be directly injected into the lumen of such
vessels, causing obliterative lymphangitis. Commonly employed agents include tetracycline, doxycycline, alcohol, OK-432, and bleomycin.
8,9
may be obliterated by placing intraluminal coils. Two teams have advocated percutaneous lymphangiography-guided cannulation and embolization as primary treatment for chylous leak of the thoracic duct after head and neck surgery.
lesions with lower degrees of lymphatic dilation have been successfully managed
with the use of radiation therapy and lasers such as the carbon dioxide, yttrium aluminum garnet, and potassium titanyl phosphate laser. Carati et al.12 summarized
their experience in a double-blinded randomized control trial with a low-level 904nm laser in which one third of postmastectomy lymphedema patients demonstrated
decreased limb volume and limb hardness.
6,7
To address
Alternatively, vessels
10,11
Superficial
Open Surgical Approaches
Treatment of Cutaneous Chylorrhea and Chylorrhagia
Removing a region of lymphatic dysplasia is often the most expedient route to
symptomatic relief. This principle is true in almost all regions of the body, including
the skin, bowel, thorax, and abdomen.13 Ruptured lymphatics are identified after the
patient ingests a fatty meal and the leaking lymphatic ducts are ligated, oversewn,
clipped, or excised with diathermy. Cutaneous lesions in the groin area have a high
rate of recurrence, and multimodal therapy with excision and laser or sclerotherapy
works well (Fig. 55.1).
14
Percutaneous drainage tubes are often employed in the
55 Surgical Management
a
b
c
457
Fig. 55.1 An 18-year-old male patient with a complex capillary venolymphatic malformation
involving the groin, pelvis and left leg. Patient was draining more than 300 mL/day of lymphatic
fluid from the lymphatic vesicles in the pubic region and experienced numerous episodes of lifethreatening sepsis. (a) Photograph just prior to hemiscrotal resection. (b) Intraoperative photograph demonstrating a massive lymphatic varix to the resected hemiscrotum. (c) 6 months following
a second operative resection of residual suprapubic involvement and following CO
of residual vesicles
laser treatment
2
postoperative phase to decrease the incidence of seroma formation. As previously
stated, such drains transform an internally draining system into an externally
draining system, and the amount of drainage must be monitored closely to avoid
complications associated with high volume chyle loss.
A second surgical option is to reconstruct damaged lymphatics in an attempt to
restore “physiological” lymphatic flow. The goal of physiological surgical intervention
458 R.G. Azizkhan and J.A. Taylor
is to bypass disrupted or malformed lymphatics and to restore physiological balance
to the lymphatic system. Prior to undergoing physiological intervention, Kim et al.13
recommend that patients first undergo a trial of complex physical therapy (CPT)
that includes manual lymph drainage, compression bandages, stockings or serial
compression devices. Used primarily for its high rate of symptom amelioration,
CPT also delays progression of chyle reflux to chronic interstitial edema, which is
not amenable to reconstruction.15 However, Campisi16 believes that compression
does not affect the microcirculation and attributes high nonresponse rates of 30–40%
to this failure. Consequently, microsurgical techniques have been developed to
create super-microsurgical anastomoses between lymphatics and venules.
The most common surgical approaches are through lymphaticovenous anastomoses and lymphatico-veno-lymphatic anastomoses. Both can be performed endto-end or end-to-side, and both have been reported at vessel diameters of as little as
0.3 mm.17 In a report of 39 cases, Narushima18 found a positive correlation between
the number of anastomoses to symptomatic improvement and decreased limb diameter. In another study, Kobayashi15 described a technique in which he telescoped, or
invaginated, multiple small lymphatic channels into a single venule. Like Narushima,
he documented both symptomatic and functional improvement in his cohort. The
downside to microsurgical techniques is their technical difficulty and variable longterm patency rates.
18
Treatment of Chylothorax
With the development of a chylothorax, chest tube drainage is often implemented
first to alleviate respiratory dysfunction. MCT diet, thoracentesis, and percutaneous
obliteration follow as definitive therapy and/or helpful adjuncts. Videoscopicassisted thoracoscopy (VATS) is a safe and effective technique to evaluate the entire
thorax and allow for intervention based on the amount and location of output of the
chylous leak.19 When output is less than 500 mL/day, pleural abrasion and pleurodesis with talc or other agents has been shown to be effective.20 Fibrin glue can be
used during the VATS procedure to help manage chylous leakage when no focal
drainage point can be determined.21 With diffuse drainage, partial pleurectomy has
also been demonstrated to resolve symptoms.
For high output chylous drainage or drainage exceeding 1 L/day, ligation of the
thoracic duct can be achieved with VATS or thoracotomy. The technique may involve
the injection of methylene blue dye or consumption of a high fat content material,
such as a butter/cream mixture, and observation for focal output. If diffuse drainage
is seen, en masse tissue ligation can be undertaken as the thoracic duct generally follows posterior to the esophagus, and anteromedial to the azygos vein in the lower
chest.23 Studies have shown a 90% resolution of the leak and earlier chest tube
removal when the thoracic duct is ligated in both pediatric and adult populations.
Alternatively, cannulation of the cysterna chyli with coil embolization of the thoracic
duct may be performed. Partial or full response has been shown to be around 95%
when either the cysterna chyli or thoracic duct can be identified and accessed.
22
24,25
19