CHYLOUS ASCITES Evaluation and Management

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Review ArticleChylous Ascites: Evaluation and Management

Said A. Al-Busafi, 1,2 Peter Ghali, 1 Marc Deschnes, 1 and Philip Wong 1

Hepatology Unit, Department of Gastroenterology, Royal Victoria Hospital, McGill University Health Center, Montreal, QC, CanadaDepartment of Medicine, College of Medicine and Health Science, Sultan Qaboos University, P.O. Box , Muscat, Oman

Correspondence should be addressed to Said A. Al-Busa ; busa [email protected]

Received September ; Accepted December ; Published February

Academic Editors: D. Morioka, O. opcu, and M. Watanabe

Copyright Said A. Al-Busa et al. Tis is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Chylous ascites re ers to the accumulation o lipid-rich lymph in the peritoneal cavity due to disruption o the lymphatic systemsecondary to traumatic injury or obstruction. Worldwide, abdominal malignancy, cirrhosis, and tuberculosis are the commonestcauses o CA in adults, the latter being most prevalent in developing countries, whereas congenital abnormalities o the lymphaticsystem and trauma are commonest in children. Te presence o a milky, creamy appearing ascitic uid with triglyceride contentabove mg/dL is diagnostic, and, in the majority o cases, unless there is a strong suspicion o malignancy, urther investigationsare not required in patients with cirrhosis. I an underlying cause is identi ed, targeted therapy is possible, but most caseswill be treated conservatively, with dietary support including high-protein and low- at diets supplemented with medium-chaintriglycerides, therapeutic paracentesis, total parenteral nutrition, and somatostatins. Rarely, resistant cases have been treated by transjugular intrahepatic portosystemic shunt, surgical exploration, or peritoneovenous shunt.

1. Introduction

Chylous ascites (CA) is an uncommon orm o ascites,de ned as the leakage o the lipid-rich lymph into theperitoneal cavity [ ]. Damage or obstruction to the lymphaticsystem or one o its tributaries produces ascites with a turbidor milky appearance rom the high triglyceride content [ ].Asellius, in , rst described the lymphatic systemin a dogafer observing vessels in the mesentery containing a whitemilky uid [ ] and, in , Morton reported the rst case o CA in a -year-old boy who died with tuberculosis [ ].

Te reported incidence o CA is approximately in, admissions at a large university-based hospital over-year period [ ]. However, it is believed that the incidence

has increased, probably because o prolonged survival o patients with cancer and more aggressive cardiothoracic andabdominal interventions as well as laparoscopic surgery andtransplantation [ ]. Tis trend is supported by the ndingo a per , incidence in the last years o the study [].Te reported incidence would also probably greatly increasei paracentesis and an appropriate analysis o the ascitic

uid were per ormed with all patients with ascites [ ]. Teprognosis basically varies based on the underlying cause. In

the same study, the -year mortality rate was %, whichincreased to % when a malignancy was the underlyingcause. Other study that included a greater proportion o congenital or traumatic cases has reported a lower mortality rate ( % in adults and % in children) [ ]. Te mortality becomes even lower in selected groups, such as those withpostoperative CA [ ].

Te aim o this review is to outline the causes o chylousascites, present a paradigm or investigations, and describethe various management options.

2. Anatomy of the Lymphatic System

Te lymphatic system includes lymph, lymphatic vessels,lymphatic tissues, and red bone marrow ( Figure ) [ ]. Itis a one-way drainage system which allows the return o excess interstitial uids and proteins to the vascular system[ ]. Lymph passes rom lymphatic capillaries into lymphatic vessels and then through lymph nodes into lymph trunks(Figure ). Te thoracic duct, the main duct or the returno lymph to blood, is about cm long and begins asdilationcalledthe cisternachyli anteriorto thesecond lumbar

Hindawi Publishing CorporationISRN Hepatology Volume 2014, Article ID 240473, 10 pageshttp://dx.doi.org/10.1155/2014/240473
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Right internal jugular vein

Right subclavian vein

Axillary lymph nodes

Supratrochlear lymph nodes

Cervical lymphatics

Cisterna chyli

Inguinal lymph nodes

Aorta

Iliac lymph nodes

Right lymphatic ductToracic (lef lymphatic) duct

Lef subclavian vein

Toracic (lef lymphatic) duct

F : Routes or drainageo lymph rom lymph trunksintothe thoracic andrightlymphaticducts. Tegreen arrowsindicate thedirectiono lymph ow.

vertebra. Te cisterna chyli receives lymph rom the right andlef lumbar trunks and rom the intestinal trunk. Chylouseffusions develop when these are injured or obstructed [ ].

3. Constituents of Chyle

One o the major unctions o the gut lymphatics is themaintenance o the interstitial uid volume and compositionand the transport o lipids. Lymph is composed o protein,

lymphocytes, immunoglobulins, and products o digestionincluding lipids in the orm o chylomicrons [ ]. More than% o thetotalbody lymph originates in the gut and liver [ ].

In the gut, long-chain triglycerides (LC ) are converted intomonoglycerides and ree atty acids (FFA) and absorbed aschylomicrons. Tis explains the high content o triglyceridesand the milky and cloudy appearance o lymph [ ]. Short-and-medium chain triglycerides (MC ), which make upapproximately one-third o dietary at, are absorbed directly by the portal venous system. Tis particular act orms thebasis or the use o MC as an oral diet in the conservativemanagement o CA.

Based on animal experiments, Blalock et al. concluded

that obstruction o the thoracic duct alone is not sufficient tocause CA [ ]. Patients with a limited reserve o anastomoticchannels are at greater risk o developing persistent asciteswhen obstruction or injury o the lymphatic channels occurs.

4. Pathophysiology

Te principal mechanisms or CA ormation are related todisruption o the lymphatic system, rom any cause. Treebasic mechanisms have been proposed using lymphangiog-raphy and inspection at laparotomy [ ]:

( ) exudation o lymph through the walls o retroperi-toneal megalymphatics into the peritoneal cavity,

which occurs with or without a visible stula (i.e.,congenital lymphangiectasia),

( ) leakage o lymph rom the dilated subserosal lym-phatics on the bowel wall into the peritoneal cavity which is due to malignant in ltration o the lymphnodes obstructing the ow o lymph rom the gut tothe cisterna chili,

( ) direct leakage o lymph through a lymphoperitonealstula associated with retroperitoneal megalymphat-

ics due to acquired lymphatic disruption as a result o trauma or surgery.

In addition, the increased caval and hepatic venouspressures caused by constrictive pericarditis, right-sidedheart ailure, and dilated cardiomyopathy may precipitateCA through large increase in production o hepatic lymph[ , ]. Finally, cirrhosis also causes an increased ormationo hepatic lymph [ ]. In act, decompression o the portal vein in patients with portal hypertension has been shown torelieve lymphatic hypertension [ ].

5. Etiology

CA may be divided into traumatic and atraumatic causes( able ), in which the underlying etiology determines theongoing evaluation and long-term management. Abdominalmalignancy and cirrhosis are the commonest causes in devel-oped countries and account or over two-thirds o all cases,whereas chronic in ections like tuberculosis and lariasisaccount or the majority o the cases in developing countries[ ].

. . Atraumatic CA ( able ). In a recent systematic review including studies rom developing and developed coun-tries (with a total o patients)who hadatraumatic chylous


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: Etiological classi cation o chylous ascites.

Atraumatic [ ] raumatic(I) Neoplastic Cardiac (I) IatrogenicSolid organ cancers Constrictive pericarditis (A) SurgicalLymphoma Congestive heart ailure Abdominal aneurysm repair

Sarcoma Gastrointestinal Retroperitoneal lymphadenectomy Carcinoid tumors Celiac sprue Placement o peritoneal dialysis catheterLymphangioleiomyomatosis Whipples disease In erior vena cava resectionChronic lymphatic leukemia Intestinal malrotation Pancreaticoduodenectomy (II) Diseases Small bowel volvulus Vagotomy (A) Congenital Menetrier disease Radical and laparoscopic nephrectomy Primary lymphatic hypoplasia In ammatory Nissen undoplicationKlippel- renaunay syndrome Pancreatitis Distal splenorenal shuntsYellow nail syndrome Fibrosing mesenteritis Laparoscopic adrenalectomy Primary lymphatic hyperplasia Retroperitoneal brosis Gynecological surgery Lymphangioma Sarcoidosis (B) Nonsurgical

Familial visceral myopathy Systemic lupus erythematosus Radiotherapy (B) Acquired Behcets disease (II) NoniatrogenicCirrhosis Peritoneal dialysis Blunt abdominal traumaInfectious Hyperthyroidism Battered child syndromeuberculosis Nephrotic syndrome Penetrating abdominal trauma

Filariasis Drugs Shear orces to the root o the mesentery Mycobacterium avium in AIDS Calcium channel blockers (III) IdiopathicAscariasis Sirolimus Rule out lymphoma

ascites, the most common causes in adults were malignancy

( %), cirrhosis ( %),mycobacterium in ection ( %), anda variety o uncommon causes ( %) [ ]. Inchildren, themostcommon causes were lymphatic anomalies ( %) ollowedby a variety o uncommon causes ( %). Other causes o CA include trauma, including surgical and radiotherapy, andother atraumatic, including congenital, in ammatory, andsystemic disorders.

. . . Neoplastic Causes. Te most common cause o CAin adults is malignancy. Among the group o malignancies,lymphoma accounts or at least one-third o the cases [ ].

umors through direct invasion or extrinsic compressionlead to disruption o normal lymphatic ow [ ]. In addi-

tion to lymphomas, other tumors that cause CA may ariserom the intra-abdominal solid organ malignancies such as

stomach, esophagus, pancreas, endometrial, and prostate,which account or % o allmalignant causes[ ]. Carcinoidtumours andKaposi sarcoma account or % and %, respec-tively, o the malignancy-related cases. Lymphangioleiomy-omatosis is a rare benign tumor o lymphatic channelsand lymph nodes, clinically mani ested by chylous effusionsincluding CA [ ].

. . . Congenital. Congenital lymphaticanormalities are pre-dominant cause in the pediatric population, wich accounts

or % o all causes. In contrast, lymphatic anomalies

account only or % o atraumatic CA in adults [ ]. Pri-

mary lymphatic hyperplasia has been recognized as causeo chylous ascites [ ]. It consists o two principal patterns:bilateral hyperplasia in which the lymphatics are notgrossly dilated and contain valves and lymphangiectasia in whichlymphatics are grossly dilated in the wall o small bowel andhave no valves. Te primary intestinal lymphangiectasia isresponsible or the majority o the cases in children [ ].

Primary lymphatic hypoplasia is another conditionseen most commonly in children and presents with lym-phedema, chylothorax, CA, or combination [ ]. Te Klippel-renaunay syndrome is an autosomal dominant inherited

disorder characterized by venous and lymphatic hypoplas-tic mal ormations which can mani est as lower limb lym-

phedema and it is ofen associated with chylous ascites[ ]. Te yellow-nail syndrome is a childhood disorder o unknown etiology. Te patients have hypoplastic or aplasticlymphatics leading to the characteristic eatures o lowerlimb lymphedema, pleural effusion, and/or CA and a yellow discoloration with dystrophy o the nails [ ].

. . . Cirrhosis. Although ascites is a common mani estationo hepatic cirrhosis, CA presents in . % o patients withcirrhosis [ , , ]. Recently, a systematic review showedthat cirrhosis was responsible or % o atraumatic chylousascites [ ]. Tis discrepancy was explained by the authors tobe related to the under diagnosis.


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Cirrhotic patients may present with CA as an initialpresentation or might present at a later stage o the diseasedue to complications such as shunt surgery, sclerotherapy-related thoracic duct injury, or hepatocellular carcinoma[ ]. However, unless clinically indicated, an aggressiveapproach to exclude malignancy is not warranted. Other

cause o CA in cirrhotic patients that should be consideredis portal vein thrombosis [ ].

. . . Infectious. Lymphatic lariasis and peritoneal tuber-culosis are the most common in ectious causes o CA andaccount or the majority o the cases in the developing coun-tries [ ]. Low socioeconomic status, malnutrition, cirrhosis,HIV in ection,diabetes mellitus, underlyingmalignancy, andambulatory peritonealdialysis are risk actors or tuberculousCA [ ]. Lymphatic lariasis causes severe in ammatory reaction in lymphatic vessels leading to lymphedema and CA[ ]. In ection with Mycobacterium avium-intracellulare hasbeen reported to cause CA in AIDS patients [ ]. A recent

systematic review showed that in ections with mycobacterialspecies, as MAI in ection and tuberculosis, contribute to %o all cases o atraumatic CA [ ].

. . . In ammatory. A variety o in ammatory causes havebeen reported to be associated with CA. Both acute pancre-atitis and chronic pancreatitis have been associated with CA[ ]. wo mechanisms have been proposed to play a role inthe development o CA by pancreatitis, which are compres-sion o lymphatic channels or direct damage by pancreaticenzymes [ ]. Fibrosing mesenteritis is rare benign processthat involves in ammation, at necrosis, and brosis o themesentery [ ]hasbeenalsoreportedtocauseCA[ ].Otherrare in ammatory causes include idiopathic retroperitoneal

brosis, [ ] sarcoidosis, [ ] systemic lupus erythematous,[ ] peritoneal dialysis, [ ] and hyperthyroidism [ ].

. . . Other Causes. Constrictive pericarditis has been alsoreported to cause CA [ , ]. It causes impaired lymphdrainage with thoracic duct dilatation and hypertensionleading to an increase in hepatic venous pressure, thereby increasing lymph production [ ]. Congestive heart ailurecan also cause CA by increasing ormation o and impairedlymphatic drainage [ , , ]. CA may develop as a resulto heart ailure secondary to thyrotoxic cardiomyopathy andresolve promptly i treated appropriately [ ]. Te nephroticsyndrome has been reported or unknown mechanism tocause chylous effusions including CA [ , ]. A study including patients with nephritic syndrome and ascites,showed that % o those who underwent paracentesis( / ) had CA. However, the diagnosis was based ondetection o opalescent effusion rather than by checking thetriglyceride level o peritoneal uid [ ].

Celiac disease and Whipples disease can cause CA due tomesenteric node hyperplasia [ ]. Calcium channel blockershave also been implicated as a cause o CA in patients under-going peritoneal dialysis [ ]. In addition, sirolimus, inrenal transplant setting, has also been reported to cause CA[ ].

. . raumatic CA ( able )

. . . Postoperative. Surgical interventions are well-knowncauses o CA secondary to direct lymphatic vessels injury.CA can occur as early as week afer abdominal surgery because o disruption o the lymphatic vessels or as late asseveral weeks to months because o adhesions or extrinsiccompression o lymphatic vessels [ ]. A retrospective study over a -year period o a cohort including , oncologicalpatients undergoing abdominal surgical procedures revealed

. % incidence o postoperative chylous ascites. . % o patients who underwent retroperitoneal, esophageal, gastric,or cytoreductive surgeries developed CA [ ]. Other sur-gical procedures that can result in CA include aortic andabdominalaneurysm repair[ ], retroperitoneal lymphnodedissection [ ], in erior vena cava resection [ ], catheterimplantation or peritoneal dialysis [ ], distal splenorenalshunts [ ], small bowel transplantation [ ], liver transplan-tation [ ], choledochal cyst excision [ ], pancreaticoduo-denectomy [ ], anterior spinal surgery [ ], laparoscopicsurgeries including Nissen undoplication [ ], Roux-en-Y gastric bypass [ ], adrenalectomy [ ], cholecystectomy [ ], and donor nephrectomy [ ].

. . . Radiotherapy. Abdomen and pelvic radiation is a com-mon cause o CA [ ]. In a review done at Mayo clinic involv-ing patients who received whole abdomen irradiation

or gynecologic malignancies, a % incidence o CA wasreported [ ]. Irradiation to abdomen can cause brosis o lymphatic vessels within small bowel and mesentery leadingto obstruction and extravasation o lymph [ ] which istypically observed afer a mean o months afer radiationtherapy [ ].

. . . Noniatrogenic Causes. In contrast to direct injury o lymphatic vessels during surgery, blunt abdominal traumacauses CA through hyperextension and hyper exion leadingto rupture o lymphatic vessels and lymph leakage [ ].Penetrating abdominal trauma has also been reported tocause CA [ ]. Te battered child syndrome, which can leadto blunt abdominal trauma, accounts or approximately %o cases o CA in children [ ]. Tere ore, it is very importantto exclude this diagnosis in any child presenting with CA.

6. Complications of Chylous Ascites

Loss o chyle into peritoneal cavity can lead to seriousconsequences because o the loss o essential proteins, lipids,immunoglobulins, vitamins, electrolytes, and water. Whilerepeated therapeutic paracentesis provides relie rom symp-toms, the nutritional de ciency will continue to persistor deteriorate unless de nitive therapeutic measures areinstituted to stop leakage o chyle into the peritoneal space.In act, in postoperative settings, this may cause increasedmortality [ ]. Tere ore, it is very important to provideadequate nutritional support replenishing uid loss, vitaminde ciencies, and electrolyte loss while speci c therapeuticmeasures are planned.


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In addition, continued loss o lymphocyte-rich lymphinto the peritoneal space and enormous loss o protein ingastrointestinal tract lead to hypogammaglobulinemia andthere ore increased susceptibility to in ection [ ]. Prolongedthoracic duct drainage has been used previously to induceimmunosuppression in several diseases including rheuma-

toid arthritis and myasthenia gravis [ ].Te bioavailability o certain drugs could be drastically impaired in the presence o signi cant chyle leak. Tere arereports o this phenomenon in patients with chylothorax-causing subtherapeutic digoxin [ ], amiodarone [ ], andcyclosporine [ ] levels in the serum. Sequestration o drugsin chyleshould be recognizedearly, to preventsubtherapeuticplasma levels in patients undergoing drainage o CA.

7. Evaluation and Diagnosis

Te diagnostic approach o CA consists o rst suspectingthe diagnosis, then con rming the presence o chyle in the

peritoneal cavity, and nally determining the underlyingabnormality. A care ul history, physical examination, anddiagnostic paracentesis are the key in the initial evaluationo any patient presenting with ascites.

. . Clinical Findings. Progressive and painless abdominaldistention ( %) and nonspeci c pain ( %) are the mostcommon presenting symptoms in CA, occurring over acourse o weeks to months depending on the underlyingcause [ ]. Patients who have undergone abdominal orthoracic surgery may present with an acute onset o CA [ ].Patients may also present weight gain and dyspnea resulting

rom increased abdominal girth [ ]. Other eatures include

weight loss, anorexia, malaise, steatorrhea, malnutrition,enlarged lymph nodes, evers, and night sweats [ , , ].However, most ofen the diagnosis o CA is not suspectedbe ore per orming a diagnostic paracentesis [ ].

Physical signs that may be present on examinationinclude ascites, pleural effusions, lower extremity edema,lymphadenopathy, cachexia, temporal wasting, and hernias[ ]. Other ndings depend on the underlying cause.

. . Laboratory Findings. Abdominal paracentesis is themost important diagnostic tool in evaluating and managingpatients with ascites. Incontrast to theyellowandtransparentappearance o ascites due to cirrhosis and portal hyperten-sion, chyle typically has a cloudy and turbid appearance( able ). Tis should be distinguished rom pseudochylousascites, in which the turbid appearance is due to cellulardegeneration rom in ection or malignancy without actually containing high levelso triglycerides [ ]. Dependingon theclinical suspicion, ascitic uid should be sent or cell count,culture,Gramstain, totalprotein,albumin, triglyceridelevels,glucose, lactate dehydrogenase, amylase, and cytology [ ].Te serum to ascites albumin gradient (SAAG) should becalculated to determine i the ascites is related to portalhypertension or other causes [ ]. Te triglyceride levels inascitic uid are very important in de ning CA. riglyceride values are typically above mg/dL, although some authors

: Characteristics o ascitic uids in chylous ascites (adaptedrom C ardenas and Chopra) [ ].

Color Milky and cloudy riglyceride level Above mg/dL

Cell count Above (lymphocytic predominance)otal protein Between . and . g /dL

SAAG Below . g/dL

Cholesterol Low (ascites/serum ratio < )Lactate dehydrogenase Between and IU/LCulture Positive in selected cases o tuberculosisCytology Positive in malignancy Amylase Elevated in cases o pancreatitisGlucose Below mg/dL

IU: international units; SAAG: serum-ascites albumin gradient.

Is elevated above . g/dL in CA secondary to cirrhosis.

use a cutoff value o mg/dL [, ]. A tuberculosis smearand culture and adenosine deaminase activity (ADA) shouldbe per ormed in selected caseswhentuberculosis is suspected[ ]. ADA has high sensitivity and speci city in the diagnosiso tuberculous peritonitis [ ]. In contrast, its utility in pop-ulations with high prevalence o cirrhosis such as the UnitedStates is limited [ ]. Te diagnosis o tuberculous peritonitisusually requires a peritoneal biopsy via laparoscopy [ ].

Standard blood tests, including a complete blood count,electrolytes, liver tests, total protein, albumin, lactate dehy-drogenase, triglycerides, cholesterol, amylase, and lipaseshould be per ormed. Additional testing should be basedupon the clinical setting [ ].

. . Imaging Studies

. . . Computed omography (C ). Chyle has a water density appearance on C which can be readily distinguished romacute hemorrhage in the setting o trauma [ ]. C o theabdomen is use ul in identi ying pathologic intra-abdominallymph nodes and masses. In the setting o postoperative ortraumaticcauses o CA,it also helps in determining theextentand localization o uid, particularly, i there is a suspicion o thoracic duct injury [ , ]. Other nding onC might suggestthat CA is ormation o uid- uid level [ ]. Another C

technique reported is the direct opaci cation o the thoracicduct with oral at emulsions [ ].

. . . Lymphoscintigraphy. Lymphoscintigraphy allows unc-tional assessment o lymphatic transport and so can be usedto detect abnormal lymphatic drainage in CA. It is use ul

or detecting patients or surgery and assessing the effect o treatment [ ]. It can be used when lymphangiography iscontraindicated [ ]. Its advantages include no adverse effects,no contraindications, and the ability to per orm repetitivestudies. Te technical challenges o this technique and itsrare implementation may make it an un avorable diagnosticmodality [ ].


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. . . Lymphangiography. Lymphangiography use has beendeclining with the availability o noninvasive imaging, butit remains the gold standard in de ning cases o lymphaticobstruction. It has been success ully used to detect abnormalretroperitoneal nodes, leakage rom dilated lymphatics, s-tulization, and patency o the thoracic duct [ , , ].

In addition, lymphangiography is also used in treatingpatients with chyle leakages who are resistant to conservativeapproach [ ]. However, it is associated with complica-tions including contrast hypersensitivity, tissue necrosis, atembolism, and even transient lymphedema and CA [ , ].

. . Laparoscopy. Ascites o unknown etiology is a commonindication or laparoscopy in patients with ascites especially when tuberculosis or malignancy is suspected [ ]. It wasreported to be perhaps the best and most de nitive methodto diagnose intestinal lymphangiectasia [ ].

. . Laparotomy. Early reoperation has been recommended

or postoperative CA to address the underlying cause aswell as or providing treatment [ ]. It is advocated todo combined pre- and intraoperative lymphangiography to

acilitate success ul treatment o postoperative CA [ ].

8. Management of CA

Few studies have addressed the best treatment regimens orCA [ ]. Nutritional regimens and pharmacological and sur-gical therapies exist but there is stilla lacko a clear consensuson the optimal management o CA [ ]. reatment o theunderlying cause is an important initial step in managingthese patients. In most cases, particularly, in patients with

in ectious, in ammatory, or hemodynamic cause, this willresult in resolution o symptoms and o the ascites [ ].

. . Medical reatment. Medical management o CA is basedon the theory that decreasing chyle ow will allow orspontaneous closure o the chyle leak [ ]. However, there isno precise, unctionalmethod ormonitoring the response totherapy [ ].

. . . Dietary Terapy. Based on the limited studies and noclear consensus, it is a reasonable approach or patients inwhom the cause was not ound or or those who did notrespond to treatment o the underlying cause to recommendthe nutritional therapy. Although it is common in practiceto recommend bowel rest and dietary modi cation, enteral

eedings, or the use o total parenteral nutrition ( PN),de nitive evidence supporting one nutrition therapy overanother does not exist [ ]. Goal o nutrition therapy is todecrease production o chyle, replace uid and electrolytes,and maintain or improve nutrition status [ ].

A reasonable approach is to recommend a high-proteinand low- at diet with MC . Dietary restriction o LCprevents their conversion into monoglycerides and FFA,which are transported as chylomicrons to the intestinallymph ducts. In contrast, MC are absorbed directly intointestinal cells and transported as FFA and glycerol directly

to the liver via the portal vein. Tus, a low- at diet with MCsupplementation reduces the production and ow o chyle[ ]. Patientswith advanced cirrhosis, MC oil shouldnotbeused as narcosis and coma may occur. Such patients shouldbe managed with a low-sodium diet and diuretics such asspironolactone [ ].

Patients who do not respond to the above measuresshouldhavebowel rest to reduce lymph owandbe started onPN[ ]. PNis theoretically superior to any enteral eedings

because the bowel is bypassed. Te presence o intraluminalwater alone has been shown to increase thoracic duct lymph

ow [ ]. PN along with somatostatin or octreotide canrelieve the symptoms and rapidly close the stula in patientswith CA [ ]. Tis approach appears to be an effectivetherapy or the treatment o CA caused by various disorders[ ].

. . . Pharmacology. Tere are other medical measureswhich have been described in literature as either case reportsor small observational studies. Orlistat, a reversible inhibitoro gastric and pancreatic lipases, was reported to minimizeascites and triglyceride levels in ascitic uid in a patientwith CA due to cirrhosis [ ]. Case reports have suggestedthat both somatostatin and octreotide either alone or incombination with PN are effective in the management o CA due to different causes [ , , ]. Te mechanismmay involve inhibition o lymph uid excretion throughspeci c receptors ound in the normal intestinal wall o lym-phatic vessels [ ]. In case reports, a promising treatment,etile rine, a sympathomimetic drug, was shown to causeresolution o postesophagectomy chylous effusions [ ].

. . Abdominal Paracentesis. In patients with symptomaticascites, a therapeutic paracentesis should be per ormed torelieve symptoms and could be repeated as needed [ ].Unless the patient has cirrhosis, the replacement o albu-min to prevent postparacentesis circulatory dys unction isnot recommended. Repeated large-volume paracentesis is areasonable option or patients whohaveend-stagedisease notamenable to medical or surgical treatment.

. . ransjugular Intrahepatic Portosystemic Shunt ( IPS).Te use o IPS to success ully treat CA has been reportedin patients with cirrhosis and CA resistant to conservative

therapy and who have reasonable liver unction [ ].However, the placement o IPS in a patient with cirrhosisis associated with signi cant problems so patients must beselected care ully.

. . Peritoneovenous Shunting. In the past, peritoneovenousshunts (LeVeen or Denver shunts) were considered options

orpatients who were re ractory to medical therapy and poorcandidates or surgery. However, these shunts wereassociatedwith a high rate o serious complications, such as sepsis,disseminated intravascular coagulation, hypokalemia, smallbowel obstruction, and risk or air embolism, and are thusseldom used [ ]. In addition, the high viscosity o the chyle


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results in a high rate o shunt occlusion in majority o thecases [ , , ].

. . Angiography. In addition, to make diagnosis, lymphan-giography with or without embolization is another promising

technique which has been described or the literature in thetreatment o postoperative CA when conservative therapy ails [ , , ].

. . Surgical reatment. I the above conservative manage-ment is not success ul in treating CA, surgical interventionmay be bene cial especially in patients with postoperative,neoplastic, and congenital causes [ ]. Preoperative lymphan-giography or lymphoscintigraphy is help ul in identi yingthe anatomical location o the leakage or the presence o a stula [ ]. Laparotomy is also essential in the diagnosisand management o acute chylous peritonitis. In a review

where all patients were initially treated conservatively withdietary therapy, surgery ( stula closure, bowel resection, orinsertion o a peritoneovenous shunt) was per ormed inpatients who ailed conservative therapy ( %). Closure o aretroperitoneal stula, when present, was the most success-

ul operation [ ]. However, in postoperative CA, surgicalreinterventions are associated with signi cant incidence o morbidity and mortality [ ]. In addition, surgery may occasionally ail to identi y the leak. Some promising new techniques (e.g., use o octreotide,etile rine, or angiography),whichalone or in combination with well-established con-servative measuresmay have the potential to avoid surgicalreinterventions [ ].

In addition, to prevent postoperative CA, it was oundthat the milk test is a sa e and effective method ollowingpancreatectomy [ ]. In children, brin glue application orcontrol o lymph leakage is also effective in prevention andmanagement o postoperative CA [ ] as well as manage-ment o congenital CA [ ].

9. Conclusion

In summary, CA is a relatively uncommon disorder. Malig-nancy andcirrhosisare the leading causes o this condition inadults. In contrast, congenital abnormalities o the lymphaticsystem and trauma are common causes in children. Paracen-tesis with con rmation o elevated triglyceride is consideredto be the gold standard or diagnosis o CA. In a cirrhoticpatient, unless there is a strong suspicion o malignancy,there is no need or unnecessary and invasive diagnostictests to rule out a malignant cause. reatment o the under-lying cause is an important initial step in managing thesepatients. Conservative approach includes the use o a low-

at diet, MC intake, paracentesis, PN, and somatostatins.Other treatment options or resistant cases include IPS,surgical exploration, and peritoneovenous shunt. However,some promising new techniques such as use o etile rineor percutaneous embolisation o cisterna chyli are waiting

urther evaluation.

Conflict of Interests

Te authors declare that there is no con ict o interestsregarding the publication o this paper.

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, .[ ] B. . Maywood, L. Goldstein, and R. W. Busuttil, Chylous

ascites afer a Warren shunt, American Journal of Surgery , vol., no. , pp. , .

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[ ] W. G. Rector Jr., Spontaneous chylous ascites o cirrhosis, Journal of Clinical Gastroenterology , vol. , no. , pp. ,

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[ ] . Almakdisi, S. Massoud, and G. Makdisi, Lymphomas andchylous ascites: review o the literature, Oncologist , vol. , no.

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[ ] V. J. Ferrans, Z. X. Yu, W. K. Nelson et al., Lymphangi-oleiomyomatosis (LAM):a reviewo clinical andmorphological

eatures, Journalof Nippon MedicalSchool ,vol. ,no. ,pp. , .

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of Medical Genetics, vol. , no. , pp. , .[ ] P. M. Duhra, E. M. M. Quigley, and M. N. Marsh, Chylous

ascites, intestinal lymphangiectasia and the yellow-nail syn-drome, Gut , vol. , no. , pp. , .

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[ ] L. Vargas- ank, R. Estay, L. Ovalle, J. R. Soto, and M. E.Villanueva, Esophageal sclerotherapy and chylous ascites,Gastrointestinal Endoscopy , vol. , no. , p. , .

[ ] F. M. Gomez Soto, F. Marcos Sanchez, A. I. Franco Moreno,A. Viana Alonso, A. I. Munoz Ruiz, and A. D. Perez-Navarro,Chylous ascites chylosus as a mani estation o hepatocarci-noma, Gastroenterologia y Hepatologia, vol. , no. , pp.

, .[ ] R. W. L. Leong, A. K. House, and G. P. Jeffrey, Chylous ascites

caused by portal vein thrombosis treated with octreotide, Journal of Gastroenterology and Hepatology , vol. , no. , pp.

, .[ ] J. B. Mehta, A. Dutt, L. Harvill, and K. M. Mathews, Epidemi-

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Valle, uberculousperitonitis:a study comparing cirrhoticandnoncirrhotic patients, Journal of Clinical Gastroenterology , vol.

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Presenting mani estation o pancreatic carcinoma, New YorkState Journal of Medicine, vol. , no. , pp. , .

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[ ] C. K. Cheung and A. Khwaja, Chylous ascites: an unsualcomplication o peritoneal dialysis. A case report and literaturereview, Peritoneal Dialysis International , vol. , no. , pp.

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Yoshino, Graves disease with intractable diarrhea, chylousascites, and chylothorax: a case report, Tyroid , vol. , no. ,pp. , .

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.[ ] H. A. Cakmak, G. Yenidunya, B. Karadag, and Z. Ongen,

Development o chylothorax and chylous ascites in a patientwith congestive heart ailure, urk Kardiyoloji Derne gi Arsivi, vol. , no. , pp. , .

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silpa, and S. Eiam-Ong, A rare case o chylous ascites andchyluria in an adult nephrotic syndrome with ocal segmental

glomerulosclerosis, Journal of the Medical Association of Tai-land , vol. , supplement , pp. S S , .[ ] J. Lindenbaum and S. S. Scheidt, Chylous ascites and the

nephrotic syndrome.Report o a case,associated withrenal veinthrombosis, Te American Journal of Medicine , vol. , no. ,pp. , .

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, no. , p. , .[ ] K. Yoshimoto, S. Saima, Y. Nakamura et al., Dihydropyri-

dine type calcium channel blocker-induced turbid dialysate in

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Wu, Lercanidipine-induced chyloperitoneum in patients onperitonealdialysis, Peritoneal Dialysis International , vol. , no.

, pp. , .[ ] G. Castro, C. Freitas, I. Beirao, G. Rocha, A. C. Henriques, and

A. Cabrita, Chylousascitesin a renal transplant recipient undersirolimus (rapamycin) treatment, ransplantation Proceedings , vol. , no. , pp. , .

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[ ] E. Oltho , J. D. Blankensteijin, and G. J. M. Akkersdijk, Chy-loperitoneum ollowing abdominal aortic surgery, Vascular , vol. , no. , pp. , .

[ ] J. Baniel, R. S. Foster, R. G. Rowland, R. Bihrle, and J. P.Donohue, Managemento chylous ascites afer retroperitoneallymph node dissection or testicular cancer, Journal of Urology , vol. , no. , part , pp. , .

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[ ] R. A. Weseman, Review o incidence and management o chylous ascites afer small bowel transplantation, Nutrition inClinical Practice, vol. , no. , pp. , .

[ ] S. As ar, R. Lowndes, and W. J. Wall, Chylous ascites afer livertransplantation, ransplantation , vol. , no. , pp. ,

.[ ] P. H.Y. Chung,K. K.Y. Wong, and P. K.H. am,Chylousascites

ollowing choledochal cyst excision and Ladds procedure, Journal of Paediatrics and Child Health, vol. , no. , pp.

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Busch, . M. van Gulik, and D. J. Gouma, Chylous ascites aferpancreaticoduodenectomy: introduction o a grading system, Journal of the American College of Surgeons, vol. , no. , pp.

, .[ ] I.-C. Su and C.-M. Chen, Spontaneous healing o retroperi-

toneal chylous leakage ollowing anterior lumbar spinalsurgery: a case report and literature review, European Spine Journal , vol. , supplement , pp. S S , .

[ ] . S. Bacelar, A. C. de Albuquerque, P. C. de Arruda, A. A.Ferraz, and E. M. Ferraz, Postoperative chylous ascites: a rarecomplication o laparoscopic Nissen undoplication, JSLS, vol., no. , pp. , .

[ ] J. E. Hidalgo, A. Ramirez, S. Patel et al., Chyloperitoneumafer laparoscopic Roux-en-Y gastric bypass (LRYGB), Obesity Surgery , vol. , no. , pp. , .

[ ] P. de Sousa, L. Viart, J. Petit, and F. Saint, Chylous ascitesafer laparoscopic adrenalectomy trans-peritoneal: anatomicaldistribution o lymph nodes and management, Progres enUrologie, vol. , no. , pp. , .

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[ ] S. S. Lentz, M. F. Schray, and . O. Wilson, Chylous ascitesafer whole-abdomen irradiation or gynecologic malignancy,International Journal of Radiation Oncology, Biology, Physics, vol. , no. , pp. , .

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.[ ] Y.-K. Keung, R. P. Whitehead, and E. Cobos, Chemotherapy

treatment o chyloperitoneum and peritoneal carcinomatosis

due to cervical cancerreviewo literature, Gynecologic Oncol-ogy , vol. , no. , pp. , .

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American Surgeon, vol. , no. , pp. , .[ ] J. M. Plummer, M. E. McFarlane, and A. H. McDonald,

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ascites, Journal of Medicine, vol. , no. - , pp. , .[ ] M. R. Camiel, D. L. Benninghoff, and L. L. Alexander, Chylous

effusions, extravasation o lymphographic contrast material,hypoplasia o lymph nodes and lymphocytopenia, Chest , vol.

, no. , pp. , .[ ] M. D. aylor, S. S. Kim, and L. J. Vaias, Terapeutic digoxin

level in chylous drainage with no detectable plasma digoxinlevel, Chest , vol. , no. , pp. , .

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[ ] B. A. Runyon, E. A. Akriviadis, and A. J. Keyser, Te opacity o portal hypertension-related ascites correlates with the uidstriglyceride concentration, AmericanJournal of ClinicalPathol-ogy , vol. , no. , pp. , .

[ ] B.A. Runyon,Managemento adultpatients with ascites due tocirrhosis: an update, Hepatology , vol. , no. , pp. ,

.[ ] A. Riquelme, M. Calvo, F. Salech et al., Value o adeno-

sine deaminase (ADA) in ascitic uid or the diagnosis o tuberculous peritonitis: a meta-analysis, Journal of Clinical Gastroenterology , vol. , no. , pp. , .

[ ] D. J. Hillebrand, B. A. Runyon, W. G. Yasmineh, and G. P.Rynders, Ascitic uid adenosine deaminase insensitivity indetecting tuberculous peritonitis in the United States, Hepatol-ogy , vol. , no. , pp. , .

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[ ] B. A. Runyon, Current concepts: care o patients with ascites,Te New England Journal of Medicine , vol. , no. , pp.

, .[ ] J. F. Hibbeln, M. D. Wehmueller, and A. C. Wilbur, Chylous

ascites: C and ultrasound appearance, Abdominal Imaging , vol. , no. , pp. , .

[ ] D. L. Day and W. J. Warwick, Toracic duct opaci cation orC scanning, American Journal of Roentgenology , vol. , no.

, pp. , .[ ] M. H. Pui and .-C. Yueh, Lymphoscintigraphy in chy-

luria, chyloperitoneum and chylothorax, Journal of Nuclear Medicine, vol. , no. , pp. , .

[ ] J. . Andrews and L. J. Binder, Lymphoscintigraphy pre- andpost-surgical lymphatic leak repair, Australasian Radiology , vol. , no. , pp. , .


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ISRN Hepatology

[ ] P. B. Sachs, M. G. Zelch, . W. Rice, M. A. Geisinger, B. Risius,and G. K. Lammert, Diagnosis and localization o lacerationo the thoracic duct: use ulness o lymphangiography and C , American Journal of Roentgenology , vol. , no. , pp. ,

.[ ] C. Cope, Diagnosis and treatment o postoperative chyle

leakage via percutaneous transabdominal catheterization o thecisterna chyli: a preliminary study, Journal of Vascular and Interventional Radiology , vol. , no. , pp. , .

[ ] S. Kohnoe, I. akahashi, H. Kawanaka, M. Mori, K. Okadome,and K. Sugimachi, Combination o preoperative lymphan-giography using lipiodol and intraoperative lymphangiography using Evans Blue acilitates the accurate identi cation o post-operative chylous stulas, Surgery oday , vol. , no. , pp.

, .[ ] . Matsumoto, . Yamagami, . Kato et al., Te effectiveness

o lymphangiography as a treatment method or various chyleleakages, British Journal of Radiology , vol. , no. , pp.

, .[ ] M. J. Nube, P. H. J. Slee, G. H. Ooms, and K. J. Heering,

Lymphoedema and chylo ascites; an unusual complication o lymphography, Netherlands Journal of Medicine, vol. , no. ,pp. , .

[ ] C. J. Ablan, F. N. Littooy, and R. J. Freeark, Postoperativechylous ascites: diagnosis and treatment. A series report andliterature review, Archives of Surgery , vol. , no. , pp.

, .[ ] J. B. Kinmonth, Disorders o the circulation o chyle, Journal

of Cardiovascular Surgery , vol. , no. , pp. , .[ ] A. Smoke and M. H. Delegge, Chyle leaks: consensus on

management? Nutrition in Clinical Practice, vol. , no. , pp. , .

[ ] C. R. Parrish and S. McCray, When chyle leaks: nutritionmanagement options, Practical Gastroenterology , vol. , no. ,pp. , .

[ ] S. K. Ohri, . Patel, L. A. Desa, and J.Spencer, Temanagemento postoperative chylous ascites. A case report and literaturereview, Journal of Clinical Gastroenterology , vol. , no. , pp.

, .[ ] J.Uriz, A.Cardenas, and V. Arroyo, Pathophysiology, diagnosis

and treatment o ascites in cirrhosis, Baillieres Best Practice &Research. Clinical Gastroenterology , vol. , no. , pp. ,

.[ ] S. Akbulut, D. Yilmaz, S. Bakir, E. Cucuk, and M. as, Acute

appendicitis together with chylous ascites: is it a coincidence?Case Reports in Medicine, vol. , Article ID , pages,

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[ ] B. S. Karagol, A. Zenciroglu, S. Gokce, A. A. Kundak, and M.S. Ipek, Terapeutic management o neonatal chylous ascites:report o a case and review o the literature, Acta Paediatrica , vol. , no. , pp. , .

[ ] J. Chen, R. K. Lin, and . Hassanein, Use o orlistat (xenical) totreat chylous ascites, Journal of Clinical Gastroenterology , vol.

, no. , pp. , .[ ] A. E. Yildirim, R. Altun, S. Can et al., Idiopathic chylous

ascites treated with total parenteral nutrition and octreotide. Acase report and review o the literature, European Journal of Gastroenterology and Hepatology , vol. , no. , pp. ,

.[ ] G. Joanny, A. Celia, G. Zeccolini, D. Del Biondo, and G. Breda,

Chylous ascites ollowing laparoscopic adrenalectomy: case

report and literature review, Archivio Italiano di Urologia e Andrologia, vol. , no. , pp. , .

[ ] D. X. Zhou, H. B. Zhou, Q. Wang, S. S. Zou, H. Wang, and H. P.Hu, Te effectiveness o the treatment o octreotide on chylousascites afer liver cirrhosis, Digestive Diseases and Sciences, vol.

, no. , pp. , .

[ ] G. Baiocchi, C. C. Faloppa, R. L. C. Araujo et al., Chylousascites in gynecologic malignancies: cases report and literaturereview, Archives of Gynecologyand Obstetrics, vol. ,no. , pp.

, .[ ] A. Widjaja, K. F. Gratz, J. Ockenga, S. Wagner, and M. P.

Manns, Octreotide or therapy o chylous ascites in yellow nailsyndrome, Gastroenterology , vol. , no. , pp. ,

[ ] P. Guillem, V. Billeret, M. L. Houcke, and J. P. riboulet,Success ul management o post-esophagectomy chylotho-rax/chyloperitoneum by etile rine, Diseases of the Esophagus, vol. , no. , pp. , .

[ ] G. J. de Vries, B. M. Ryan, M. De Bievre, A. Driessen, R.W. Stockbrugger, and G. H. Koek, Cirrhosis related chylousascites success ully treated with IPS, European Journal of

Gastroenterology and Hepatology , vol. , no. , pp. ,.[ ] . B. Kinney, S. L. Ferrara, F. J. Miller, A. C. Roberts, and

. Hassanein, ransjugular intrahepatic portosystemic shuntcreation as treatment or re ractory chylous ascites and chy-lothorax in a patient with cirrhosis, Journal of Vascular and Interventional Radiology , vol. , no. , part , pp. , .

[ ] B. G. Rosser, J. J. Poterucha, M. A. McKusick, and P. S. Kamath,Toracic duct-cutaneous stula in a patient with cirrhosis o the liver: success ul treatment with a transjugular intrahepaticportosystemicshunt, MayoClinic Proceedings, vol. , no. ,pp.

, .[ ] D. Rubinstein, I. McInnes, and F. Dudley, Morbidity and

mortality afer peritoneovenous shunt surgery or re ractory ascites, Gut , vol. , no. , pp. , .

[ ] D. Voros and S. Hadziyannis, Success ul management o postoperative chylous ascites with a peritoneojugular shunt, Journal of Hepatology , vol. , no. , p. , .

[ ] D. Mittleider, . A. Dykes, K. P. Cicuto, S. M. Amberson, and C.R. Leusner, Retrograde cannulation o the thoracic duct andembolization o the cisterna chyli in the treatment o chylousascites, Journal of Vascular and Interventional Radiology , vol.

, no. , part , pp. , .[ ] F. Benedix, H. Lippert, and F. Meyer, Post-surgical lympho-

cutaneous stula, chylous ascites and chylothoraxin requentbut serious complications: etiology, diagnosis and therapeuticoptions review, Zentralblatt f ur Chirurgie, vol. , no. , pp.

, .[ ] H. Aoki, N. akakura, S. Shiozaki, and H. Matsukawa, Milk-

based test as a preventive method or chylous ascites ollowingpancreatic resection, Digestive Surgery , vol. , no. , pp.

, .[ ] S. Zeidan, A. Delarue, A. Rome,and B. Roquelaure, Fibrin glue

application in the management o re ractory chylous ascites inchildren, Journal of Pediatric Gastroenterology and Nutrition, vol. , no. , pp. , .

[ ] B. Antao, D. Croaker, and R. Squire, Success ul managemento congenital chyloperitoneum with brin glue, Journal of Pediatric Surgery , vol. , no. , pp. E E , .


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