Chyle leak after major pancreatic surgery. Is this an underestimated complication? A narrative review of the literature.

PURPOSE: Postoperative chyle leak, termed 'chylous ascites', is a rare complication with a reported frequency of only one in 20464 abdominal operations. The purpose of this study was to summarize the available scientific data reviewing the most relevant studies for this type of postoperative complication after pancreatic surgery, highlighting at the same time the necessity for pancreatic surgeons to retain a high level of clinical suspicion for the early diagnosis and its therapeutic management. METHODS: A thorough literature search in Pubmed and Google Scholar, under the terms' chylous ascites OR chyle leak AND pancreas OR pancreatic', since the year of inception until 19th of February 2021 was conducted by the authors and the associated results are presented in this narrative review. RESULTS: Chyle leak is a rare complication following pancreatic surgery. Patients may suffer from exudative enteropathy and malnutrition leading to repeated infections and impaired wound healing or even death secondary to sepsis. Several studies have highlighted the issue of increased hospital stay, while others failed to reach statistical significance as far as hospital stay or survival are concerned. Researchers found that patients with diffuse chyle leak tended to have a worse 3-year survival rate (18.8%), which can be attributed to postoperative complications and early demise due to immunosuppression associated with the leak, or delayed adjuvant chemotherapy Conclusion: Further clinical research is needed to enhance prevention, diagnosis, treatment and long-term prognosis of this relevant surgical problem that shows trends of increase due to the great number of major operations which are performed nowadays.

Cytomorphology of Mycobacterium avium intracellulare-associated ascites.

Ascites due to Mycobacterium avium intracellulare (MAI) infection is extremely rare and associated with a poor outcome. The cytomorphology of this condition has not been previously reported. We present a unique case of a 45-year-old woman with iatrogenic immunodeficiency who developed MAI-associated chylous ascites. The ascitic fluid cytology showed numerous lymphocytes and foamy histiocytes with abundant intracytoplasmic MAI organisms. The diagnosis was confirmed by tissue biopsy showing MAI mesenteritis. It is important to consider MAI-associated ascites in the differential diagnosis whenever ascitic fluid shows a predominant population of lymphocytes and macrophages, especially in immunocompromised patients.

Successful Conversion Surgery Following Chylous Ascites After Nivolumab for Advanced Gastric Cancer.

CASE REPORT: A 75-year-old man who underwent nivolumab as the third-line chemotherapy for advanced gastric cancer had chylous ascites (CA) after the primary tumor shrank remarkably. Total parenteral nutrition and subcutaneous octreotide were initiated, and CA disappeared. Nivolumab was continued for a total of 23 courses. Computed tomography showed lymph node swelling; however, staging laparoscopy showed that peritoneal metastasis had disappeared. Therefore, conversion surgery and distal gastrectomy with D1+ dissection were performed. RESULTS: The pathological diagnosis was type IV, poorly differentiated adenocarcinoma (por2) with signet ring cells, ypT2 (muscularis propria), without lymphatic or venous invasion, and no involvement of the proximal and distal margins. After the operation, no recurrence was observed over 7 months with no adjuvant chemotherapy. CONCLUSION: Nivolumab has the potential to lead to R0 resection for patients with peritoneal carcinomatosis gastric cancer. To our knowledge, this is the first report of successful conversion surgery after nivolumab-related chylous ascites.

Characterization of ANGPTL4 function in macrophages and adipocytes using Angptl4-knockout and Angptl4-hypomorphic mice.

Angiopoietin-like protein (ANGPTL)4 regulates plasma lipids, making it an attractive target for correcting dyslipidemia. However, ANGPTL4 inactivation in mice fed a high fat diet causes chylous ascites, an acute-phase response, and mesenteric lymphadenopathy. Here, we studied the role of ANGPTL4 in lipid uptake in macrophages and in the above-mentioned pathologies using Angptl4-hypomorphic and Angptl4-/- mice. Angptl4 expression in peritoneal and bone marrow-derived macrophages was highly induced by lipids. Recombinant ANGPTL4 decreased lipid uptake in macrophages, whereas deficiency of ANGPTL4 increased lipid uptake, upregulated lipid-induced genes, and increased respiration. ANGPTL4 deficiency did not alter LPL protein levels in macrophages. Angptl4-hypomorphic mice with partial expression of a truncated N-terminal ANGPTL4 exhibited reduced fasting plasma triglyceride, cholesterol, and NEFAs, strongly resembling Angptl4-/- mice. However, during high fat feeding, Angptl4-hypomorphic mice showed markedly delayed and attenuated elevation in plasma serum amyloid A and much milder chylous ascites than Angptl4-/- mice, despite similar abundance of lipid-laden giant cells in mesenteric lymph nodes. In conclusion, ANGPTL4 deficiency increases lipid uptake and respiration in macrophages without affecting LPL protein levels. Compared with the absence of ANGPTL4, low levels of N-terminal ANGPTL4 mitigate the development of chylous ascites and an acute-phase response in mice.

3-Methylcholanthrene Induces Chylous Ascites in TCDD-Inducible Poly-ADP-Ribose Polymerase (Tiparp) Knockout Mice.

TCDD-inducible poly-ADP-ribose polymerase (TIPARP) is an aryl hydrocarbon receptor (AHR) target gene that functions as part of a negative feedback loop to repress AHR activity. Tiparp-/- mice exhibit increased sensitivity to the toxicological effects of 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD), including lethal wasting syndrome. However, it is not known whether Tiparp-/- mice also exhibit increased sensitivity to other AHR ligands. In this study, we treated male Tiparp-/- or wild type (WT) mice with a single injection of 100 mg/kg 3-methylcholanthrene (3MC). Consistent with TIPARP's role as a repressor of AHR signaling, 3MC-treated Tiparp-/- mice exhibited increased hepatic Cyp1a1 and Cyp1b1 levels compared with WT mice. No 3MC-treated Tiparp-/- mice survived beyond day 16 and the mice exhibited chylous ascites characterized by an accumulation of fluid in the peritoneal cavity. All WT mice survived the 30-day treatment and showed no signs of fluid accumulation. Treated Tiparp-/- mice also exhibited a transient and mild hepatotoxicity with inflammation. 3MC-treated WT, but not Tiparp-/- mice, developed mild hepatic steatosis. Lipid deposits accumulated on the surface of the liver and other abdominal organs in the 3MC-Tiparp-/- mice. Our study reveals that Tiparp-/- mice have increased sensitivity to 3MC-induced liver toxicity, but unlike with TCDD, lethality is due to chylous ascites rather than wasting syndrome.

Chylous ascites following laparoscopic live donor nephrectomy: A new improved treatment paradigm.

Chylous ascites is a difficult, albeit uncommon complication of laparoscopic live donor nephrectomy (LLDN). Lymphatic leak is believed to be a result of injury to the cisterna chyli, regional lymph nodes, or other peri-aortic lymphatics intraoperatively. Recommended management with dietary modifications can result in malnutrition and immunodeficiency. We present four patients who developed chylous ascites following LLDN. Approach to these patients evolved over time. Our initial two patients were successfully treated with a combination of surgical intervention followed by drain placement, after the failure of conservative management. The latter two cases were successfully treated with prompt intra-abdominal drain placement, without dietary modifications. Our cohort challenges the standard of care for treatment of chylous ascites after LLDN. We believe that prompt diagnosis and placement of an intra-abdominal drain can be used safely in select patients that develop this complication. We hypothesize that continuously draining the lymphatic leak, thus avoiding the re-accumulation of ascites, allows bowel and mesentery to make contact and adhere to the retroperitoneal tissue. We believe that prompt, initial, percutaneous drain placement is a viable alternative to both conservative and reoperative management in the treatment of chylous ascites after LLDN and should be considered as a reasonable first-line therapy.

Chylous ascites, anti-interferon-gamma autoantibody, and angioimmunoblastic T-cell lymphoma: a rare but intriguing connection over Mycobacterium avium.

We report a case of non-AIDS (acquired immunodeficiency syndrome), non-CAPD (Continuous Ambulatory Peritoneal Dialysis), non-cirrhotic, Mycobacterium avium peritonitis, which is a rare form of mycobacterial infection. A 66-year-old Japanese man who had been treated previously for angioimmunoblastic T-cell lymphoma (AITL), had developed disseminated M. avium infection. Antimycobacterial regimen improved his symptoms; however, following an interruption in treatment, he developed chylous ascites. The patient died of uncontrolled peritonitis despite intensive treatment. Anti-interferon-γ autoantibody was positive, and AITL was presumed to be involved in autoantibody production. A rare coexistence of chylous ascites, autoantibody, and AITL taught us an intriguing lesson on the pathogenesis of M. avium infection. Particularly, we conclude that treatment strategies for M. avium infection should aim to restore immunity.

A female infant with phacomatosis pigmentovascularis and congenital chylous ascites: A case report.

RATIONALE: Phacomatosis pigmentovascularis (PPV) is a rare syndrome characterized by capillary malformation and pigmentary nevus. Congenital chylous ascites (CCA) is also a rare disease that results from maldevelopment of the lymphatic system. We report a case of a 5-month-old girl, who had both PPV and CCA. PATIENT CONCERNS: A 5-month-old girl is reported, who presented extensive nevus flammeus and an aberrant Mongolian spot with congenital chylous ascites. DIAGNOSES: The expression of extensive nevus flammeus and an aberrant Mongolian spot with congenital chylous ascites, that was diagnosed as type IIb phacomatosis pigmentovascularis. INTERVENTIONS: Conservative treatment included administration of somatostatin, MCT-based diet or TPN with drainage of ascitic fluid. Surgery was taken into account after failed conservative treatments. Before surgery, it is necessary to locate the abnormal lymphatic vessels. OUTCOMES: Conservative treatment and surgery sometimes functioned limitedly on CCA. LESSONS: According to the classification system of ISSVA (the International Society for the Study of Vascular Anomalies), this case meet the classification of CLM included in combined vascular malformations. It is likely to there is a connection between these two congenital diseases.

Infant fatality case with excessive chylous ascites.

An 11-month-old boy with marked abdominal distension was found dead in the prone position at home. Since there were many bruises in the non-protruding regions of the head, face, and abdomen, a medicolegal autopsy was performed the following day. The boy was smaller than average (height: 68.5 cm; weight: 7.8 kg); his extremities were thin; and his abdomen was remarkably bulging. Chylous ascites (1600 mL) was observed in the peritoneal cavity and chylous pleural effusion (left: 5 mL; right: 10 mL) in the thoracic cavity. A fibrous induration, approximately 2.0 × 1.5 cm in size, was observed in the root of the small bowel mesentery. Congenital chylothorax and chylous ascites were suspected. However, the remarkably withered thymus and an old injury in the superior labial frenulum suggested that the chylous ascites may have been further deteriorated by injuries sustained during physical abuse. Examination suggested that the death was sudden. Thus, we inferred that the cause of death was circulatory and respiratory failure due to excessive chylous ascites. Among the reported cases of chylous ascites in pediatric patients, some patients experiencing abuse were identified on the basis of their chief complaints of vomiting or abdominal distension. Medical and child welfare staff should be made aware of this information.

Primary Volvulus of the Small Intestine Exhibiting Chylous Ascites: A Case Report.

BACKGROUND: Primary volvulus of the small intestine associated with chylous ascites is very rare, with only four reported cases. In this paper, we report a new case of primary volvulus associated with chylous ascites. CASE PRESENTATION: The patient was a 70-year-old man. After experiencing bloating and abdominal pain for several hours, he called an ambulance and underwent an emergency examination at our hospital. Abdominal distension, pressure pain, and rebound tenderness were observed throughout his entire abdomen. The patient had a history of hypertension for which he was receiving oral treatment. Abdominal contrast-enhanced computed tomography (CT) revealed an edematous change in the intestinal membrane and volvulus of the small intestine. As findings suggestive of ischemia were observed in part of the intestines, emergency surgery was performed on the day of admission. Open surgery revealed approximately 500 mL of chylous ascites in the abdominal cavity. The small intestine had twisted 180° in a counter-clockwise direction at the root of the superior mesenteric artery, and the mesentery appeared milky white with edematous changes extending 75 to 240 cm from the ligament of Treitz. There was no evidence of intestinal necrosis; therefore intestinal resection was not performed. The volvulus of the small intestine was corrected. Moreover, because there was no other underlying disease observed, surgery was completed. The ascites collected during surgery revealed high levels of triglycerides at 332 mg/dL, and chylous ascites was diagnosed. An abdominal CT performed on the third day after surgery showed an improvement in intestinal edema, and primary volvulus of the small intestine associated with chylous ascites was diagnosed. Postoperative progress was good, and the patient was discharged on hospital day 10.

Feeding Angptl4-/- mice trans fat promotes foam cell formation in mesenteric lymph nodes without leading to ascites.

Angiopoietin-like 4 (ANGPTL4) regulates plasma triglyceride levels by inhibiting LPL. Inactivation of ANGPTL4 decreases plasma triglycerides and reduces the risk of coronary artery disease. Unfortunately, targeting ANGPTL4 for the therapeutic management of dyslipidemia and atherosclerosis is hampered by the observation that mice and monkeys in which ANGPTL4 is inactivated exhibit lipid accumulation in the mesenteric lymph nodes (MLNs). In mice these pathological events exclusively unfold upon feeding a high saturated FA diet and are followed by an ultimately lethal pro-inflammatory response and chylous ascites. Here, we show that Angptl4-/- mice fed a diet rich in trans FAs develop numerous lipid-filled giant cells in their MLNs, yet do not have elevated serum amyloid and haptoglobin, do not exhibit ascites, and survive, unlike Angptl4-/- mice fed a saturated FA-rich diet. In RAW264.7 macrophages, the saturated FA, palmitate, markedly increased markers of inflammation and the unfolded protein response, whereas the trans-unsaturated elaidate and the cis-unsaturated oleate had the opposite effect. In conclusion, trans and saturated FAs have very distinct biological effects in macrophages. Furthermore, lipid accumulation in MLNs is uncoupled from activation of an acute-phase response and chylous ascites, suggesting that ANGPTL4 should not be fully dismissed as target for dyslipidemia.

Multifocal Kaposiform Hemangioendothelioma Causing Massive Fetal Chylous Ascites.

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that usually occurs in soft tissues of the extremity and rarely in the retroperitoneum. We report a unique case of isolated massive fetal ascites attributed to KHE, involving the retroperitoneum and multiple visceral organs, along with the Kasabach-Merritt phenomenon. We suspect that retroperitoneal KHE might have caused massive fetal ascites because of its high potential to invade the lymphatic vessels aggressively in the retroperitoneal space, which possibly permits intestinal lymph leakage into the peritoneal cavities.

[CONGENITAL CHYLOUS ASCITES: A CASE REPORT].

Congenital chylous ascites is a rare polietiologic entity, requiring close study of the infant's organism by visualization methods in order to diagnose the bening or malignant underlying pathology. In the article is given a report on case of congenital chylous ascites in infant, caused by lymphangioma in the peritoneal cavity. Atypical clinic and the lack of diagnostic standards led to the later detection of the ascite's origin.