Pseudo-Meigs syndrome caused by a rapidly enlarging hydropic leiomyoma with elevated CA125 levels mimicking ovarian malignancy: a case report and literature review.

Pseudo-Meigs syndrome is a rare syndrome characterized by hydrothorax and ascites associated with pelvic masses, and patients occasionally present with elevated serum cancer antigen-125 (CA125) levels. Hydropic leiomyoma (HLM) is an uncommon subtype of uterine leiomyoma characterized by hydropic degeneration and secondary cystic changes. Rapidly enlarging HLMs accompanied by hydrothorax, ascites, and elevated CA125 levels may be misdiagnosed as malignant tumors. Here, we report a case of HLM in a 45-year-old Chinese woman who presented with ascites and hydrothorax. Preoperative abdominopelvic CT revealed a giant solid mass in the fundus uteri measuring 20 × 15 × 12 cm. Her serum CA125 level was elevated to 247.7 U/ml, while her hydrothorax CA125 level was 304.60 U/ml. The patient was initially diagnosed with uterine malignancy and underwent total abdominal hysterectomy and adhesiolysis. Pathological examination confirmed the presence of a uterine hydropic leiomyoma with cystic changes. After tumor removal, the ascites and hydrothorax subsided quickly, with no evidence of recurrence. The patient's serum CA125 level decreased to 116.90 U/mL on Day 7 and 5.6 U/mL on Day 40 postsurgery. Follow-up data were obtained at 6 months, 1 year, and 2 years after surgery, and no recurrence of ascites or hydrothorax was observed. This case highlights the importance of accurate diagnosis and appropriate management of HLM to achieve successful outcomes.

The CIRrhotic Ascites Severity (CIRAS) model predicts hepatic hydrothorax at all stages of ascites.

BACKGROUND: Hepatic hydrothorax (HH) is a rare but severe manifestation of cirrhotic ascites. Whether HH development relates to ascites severity is uncertain and simple clinical models to predict HH from all stages of ascites are missing. The recently published CIRrhotic Ascites Severity (CIRAS) model using only ascites-related variables may serve this purpose. AIM: We investigated if the CIRAS model within one year predicts the development of HH requiring thoracentesis in patients with cirrhosis and ascites. METHODS: We used data from 1090 patients with cirrhosis and all severities of ascites enrolled in three randomized clinical trials with available CIRAS model scores and no history of HH. Fine and Gray regression was applied to estimate the CIRAS model's ability to predict HH. RESULTS: Thirty-five patients developed HH requiring thoracentesis. The CIRAS model stratified patients at different risks for HH and increasing CIRAS score was associated with a higher risk for HH (sHR 1.49 [95% CI: 1.19-1.86]). The CIRAS model's discriminatory ability achieved an AUC of 0.67 (95% CI: 0.56-0.77); higher than of the cirrhosis severity scores Child-Pugh and MELD variants. CONCLUSION: The CIRAS model predicts the development of HH in cirrhosis patients with any grade of ascites, suggesting a potential for improved pre-emptive HH management. This complements the general movement towards personalised treatments and care.

Management of refractory hepatic hydrothorax: a report of two cases and review of the literature.

Hepatic hydrothorax is a transudative pleural effusion in patients with cirrhosis. A 56-year-old cirrhotic patient presented with dyspnea and desaturation; his chest images showed a right pleural effusion. Another 66-year-old woman with cirrhosis, developed during her hospitalization acute respiratory failure, and her chest X- ray showed left pleural effusion. Initially, both patients were prescribed a dietary sodium restriction and diuretics. Nevertheless, they didn't have a good response so a chest tube was placed, and an octreotide infusion partially reduced the volume of the pleural drainage allowing a pleurodesis. We report two cases of refractory hepatic hydrothorax with multiple treatments including octreotide and pleurodesis.

Pulmonary Complications of Portal Hypertension.

Portopulmonary hypertension (POPH), hepatopulmonary syndrome, and hepatic hydrothorax constitute significant complications of portal hypertension, with important implications for management and liver transplantation (LT) candidacy. POPH is characterized by obstruction and remodeling of the pulmonary resistance arterial bed. Hepatopulmonary syndrome is the most common pulmonary vascular disorder, characterized by intrapulmonary vascular dilatations causing impaired gas exchange. LT may improve prognosis in select patients with POPH. LT is the only effective treatment of hepatopulmonary syndrome. Hepatic hydrothorax is defined as transudative pleural fluid accumulation that is not explained by primary cardiopulmonary or pleural disease. LT is the definitive cure for hepatic hydrothorax.

The impact of MELD exception points for hydrothorax on posttransplant mortality: a propensity score-matched analysis.

BACKGROUND: Hepatic hydrothorax is a challenging complication of end-stage liver disease, and.patients with this complication can receive model for end-stage liver disease (MELD) exception points if they meet specific criteria as defined by United Network for Organ Sharing (UNOS). This research aimed to analyze the effect of receiving MELD exception points for hepatic hydrothorax on posttransplant mortality, using a national transplant database. METHODS: Patients >18 years in the UNOS database awaiting liver transplant between 2012 and 2023 were identified based on their petition for MELD exception points. Using a 1: 1 propensity score-matched analysis, 302 patients who received MELD exception points for hepatic hydrothorax were compared with 302 patients who did not receive MELD exception points.Demographic, clinical and laboratory values were compared. The primary outcome was posttransplant mortality. Multivariate logistic regression controlled for potential confounders. RESULTS: No significant difference was observed in mean age (58.20 vs 57.62 years), mean initial MELD score (16.93 vs 16.54), or mean Child-Pugh score (9.77 vs 9.74) in patients with hepatic hydrothorax receiving MELD exception points versus their matched cohort who did not recieve exception points. The proportion of males was slightly higher among patients who received MELD exception points (57.6% males vs 53.6% males). A majority of patients in both groups had Child-Pugh grade C (>56%). Patients receiving MELD exception points for hepatic hydrothorax had a statistically significant 44% decrease in the odds of posttransplant death compared to those who did not (OR 0.56; 95% CI 0.37-0.88; P  = 0.01). Among the combined cohort, each year increase in age resulted in a 3.9% increase in mortality (OR 1.04; 95% CI 1.01-1.07; P  = 0.005), and every one-unit increase in serum creatinine resulted in a 40% increase in mortality (OR 1.40; 95% CI 1.03-1.92; P  = 0.03). CONCLUSION: Receiving MELD exception points for hepatic hydrothorax is associated with a significant reduction in the odds of posttransplant mortality. These findings underscore the importance of MELD exception points for hepatic hydrothorax among patients with decompensated cirrhosis, potentially improving patient prioritization for liver transplantation and influencing clinical decision-making.

National registry of thoracoamniotic shunting using a double-basket catheter: A post-marketing surveillance registry of 295 patients with fetal hydrothorax.

OBJECTIVE: We report on the 1-year outcomes of fetuses who underwent thoracoamniotic shunting (TAS) with a double-basket catheter for fetal hydrothorax (FHT) using a national post-marketing surveillance registry. METHODS: This surveillance study was conducted for cases enrolled between 2011 and 2019. Preoperative, operative, and postoperative findings and outcomes had to be reported through the national surveillance website. RESULTS: Forty-three institutes enrolled 295 patients. In 60 patients, associated abnormalities were diagnosed after birth. Among the 235 patients with isolated hydrothorax, the survival rate at 1 year of age was 70.5%. Among these patients, 89% did not require oxygen or other respiratory support. The 1-year survival rate in complicated cases was 27%. Trisomy 21 was the most common associated problem (40%). Catheter associated problems, including dislocation (25%), obstruction (24%), and preterm rupture of the membranes (6%) were observed, but there were no severe maternal complications. TAS before 29.5 weeks of gestation (odds ratio [OR] 0.16) and skin edema and ascites (OR 0.06) were risk factors for death at one year, whereas appropriate shunt location for >28 days (OR 4.2) was a protective factor. CONCLUSION: We report a favorable survival rate in fetuses with isolated FHT treated with this double-basket catheter.

Diagnosis and Management of Hepatic Hydrothorax.

Hepatic hydrothorax is a pleural effusion (typically ≥500 mL) that develops in patients with cirrhosis and/or portal hypertension in the absence of other causes. In most cases, hepatic hydrothorax is seen in patients with ascites. However, ascites is not always found at diagnosis and is not clinically detected in 20% of patients with hepatic hydrothorax. Some patients have no symptoms and incidental findings on radiologic examination lead to the diagnosis of the condition. In the majority of cases, the patients present with symptoms such as dyspnea at rest, cough, nausea, and pleuritic chest pain. The diagnosis of hepatic hydrothorax is based on clinical manifestations, radiological features, and thoracocentesis to exclude other etiologies such as infection (parapneumonic effusion, tuberculosis), malignancy (lymphoma, adenocarcinoma) and chylothorax. The management strategy involves a stepwise approach of one or more of the following: Reducing ascitic fluid production, preventing fluid transfer to the pleural space, fluid drainage from the pleural cavity, pleurodesis (obliteration of the pleural cavity), and liver transplantation. The complications of hepatic hydrothorax are associated with significant morbidity and mortality. The complication that causes the highest morbidity and mortality is spontaneous bacterial empyema (also called spontaneous bacterial pleuritis).

Left hepatic hydrothorax without ascites.

Hepatic hydrothorax (HH) is a complication in patients with cirrhosis and portal hypertension. It predominantly presents in the right pleural cavity and usually associates with ascites. Few cases of HH occurring without detectable ascites have been reported. This case report comprehensively presents a case of a refractory left unilateral HH without ascites. The patient benefited from palliative care and the HH was managed using a semipermanent indwelling pleural catheter until she died 3 months after diagnosis.

Complications of Peritoneal Dialysis Part I: Mechanical Complications.

Peritoneal dialysis (PD) is a form of KRT that offers flexibility and autonomy to patients with ESKD. It is associated with lower costs compared with hemodialysis in many countries. However, it can be associated with unexpected interruptions to or discontinuation of therapy. Timely diagnosis and resolution are required to minimize preventable modality change to hemodialysis. This review covers mechanical complications, including leaks, PD hydrothorax, hernias, dialysate flow problems, PD-related pain, and changes in respiratory mechanics. Most mechanical complications occur early, either as a result of PD catheter insertion or the introduction of dialysate and consequent increased intra-abdominal pressure. Late mechanical complications can also occur and may require different treatment.

Pleural effusion portends a poor prognosis in patients on continuous ambulatory peritoneal dialysis.

AIMS: Pleural effusion is not an infrequent complication in patients undergoing continuous ambulatory peritoneal dialysis. However, there is not adequate data to evaluate pleural effusion and prognosis in clinical practice. In this study, we validated this potential association by a multicenter cohort. METHODS: We screened 1,162 patients who met the inclusion criteria with PD. According to the existence of pleural effusion on stable dialysis (4-8 weeks after dialysis initiation), the participants were divided into pleural effusion and non-pleural effusion groups. The hazard ratios (HRs) of all-cause and cause-specific death were estimated with adjustment for demographic characteristics and multiple potential clinical confounders. Subgroup analysis and propensity score matching (PSM) were used to further verify the robustness of the correlation between hydrothorax and prognosis. RESULTS: Pleural effusion was found in 8.9% (104/1162) of PD individuals. After adjusting for the confounding factors, patients with pleural effusion had significantly increased HRs for all-cause death was 3.06 (2.36-3.96) and cardiovascular death was 3.78 (2.67-5.35) compared to those without pleural effusion. However, it was not associated with infectious and other causes of death. After PSM, the HR of all-cause mortality was 3.56 (2.28-5.56). The association trends were consistent in the subgroup sensitivity analysis. CONCLUSION: Pleural effusion is not rare in PD, and is significantly associated with overall and cardiovascular mortality, which is independent of underlying diseases and clinically relevant indicators.

A case of refractory hepatic hydrothorax due to pleuroperitoneal communication successfully controlled by diaphragmatic plication and subsequent peritoneovenous shunting.

In general, control of hepatic hydrothorax is difficult, and patients have a poor prognosis. A case in which hepatic hydrothorax was well controlled for a long time after diaphragm plication and subsequent Denver shunt placement is reported. A 70-year-old man with decompensated liver cirrhosis presented with progressive exertional dyspnea. 5 years before admission, hepatic ascites associated with portal hypertension appeared, and a left pleural effusion subsequently developed. The pleural effusion was not controlled by salt restriction and diuretics. Based on the clinical findings, the existence of pleuroperitoneal communication was strongly suspected, and surgical diaphragmatic plication was performed. After the treatment, the pleural effusion did not accumulate, but ascites increased significantly, and conservative therapy was ineffective. For the treatment of massive ascites, a peritoneovenous shunt (Denver shunt®) was placed. Although more than 2 years have passed, the thoracoabdominal effusions have not accumulated, and the patient has been asymptomatic. The present case suggests that multidisciplinary treatment may improve the prognosis of patients with refractory thoracoabdominal effusions.

Refractory hepatic hydrothorax is associated with increased mortality with death occurring at lower MELD-Na compared to cirrhosis and refractory ascites.

BACKGROUND AND AIMS: Although refractory hepatic hydrothorax (RH) is a serious complication of cirrhosis, waitlisted patients do not receive standardized Model for End-stage Liver Disease (MELD) exemption because of inadequate evidence suggesting mortality above biochemical MELD. This study aimed to examine liver-related death (LRD) associated with RH compared to refractory ascites (RA). APPROACH AND RESULTS: This was a retrospective cohort study of Veterans with cirrhosis. Eligibility criteria included participants with RH or RA, followed from their first therapeutic thoracentesis/second paracentesis until death or transplantation. The primary outcome was LRD with non-LRD or transplantation as competing risk. Of 2552 patients with cirrhosis who underwent therapeutic thoracentesis/paracentesis, 177 met criteria for RH and 422 for RA. RH was associated with a significantly higher risk of LRD (adjusted HR [aHR] 4.63, 95% CI 3.31-6.48) than RA overall and within all MELD-sodium (MELD-Na) strata (<10 aHR 4.08, 95% CI 2.30-7.24, 10-14.9 aHR 5.68, 95% CI 2.63-12.28, 15-24.9 aHR 4.14, 95% CI 2.34-7.34, ≥25 aHR 7.75, 95% CI 2.99-20.12). LRD was higher among participants requiring 1 (aHR 3.54, 95% CI 2.29-5.48), 2-3 (aHR 4.39, 95% CI 2.91-6.63), and ≥4 (aHR 7.89, 95% CI 4.82-12.93) thoracenteses relative to RA. Although participants with RH and RA had similar baseline MELD-Na, LRD occurred in RH versus RA at a lower MELD-Na (16.5 vs. 21.82, p =0.002) but higher MELD 3.0 (27.85 vs. 22.48, p <0.0001). CONCLUSIONS: RH was associated with higher risk of LRD than RA at equivalent MELD-Na. By contrast, MELD 3.0 may better predict risk of LRD in RH.

A Case Report of Tension Hydrothorax Incited by Bowel Perforation.

We report the case of a 34-year-old man who developed cardiac arrest due to tension hydrothorax from colonic perforation. Tension hydrothorax, an entity characterized by pleural effusion leading to mediastinal compression, has not been reported in association with intraabdominal inflammation. Our patient developed respiratory insufficiency after repair of colonic perforation, followed by respiratory failure and cardiac arrest. Transthoracic echocardiography provided rapid diagnosis during decompensation and prompted a lifesaving thoracostomy. Clinicians should consider tension hydrothorax as a rare cause of hemodynamic collapse, even in the absence of liver failure, and use bedside tools like transthoracic echocardiography to facilitate diagnosis and intervention.

Hydrothorax and air fluid levels in the right chest - a diagnostic dilemma.

SUMMARY: A diaphragmatic defect that permits abdominal contents to herniate into the right side of the chest is rare. In adults with right-sided diaphragmatic hernias, few occur without a history of trauma, and even fewer are symptomatic. This case report illustrates such a case and the rare entity of an anterolaterally located hernia. Although uncommon and easily missed, consequences of diaphragmatic hernias can be disastrous. The importance of a combination of high clinical suspicion and the use of computed tomography (CT) to aid diagnosis, and the dangers of a surgical condition being incorrectly assessed and admitted to a non-surgical specialty are highlighted in this case.

Pleuroperitoneal communication in a patient on automated peritoneal dialysis: A rare but important complication.

Despite its rare frequency, a pleuroperitoneal communication is a well-documented complication for patients on peritoneal dialysis. It occurs in ~2% of continuous ambulatory peritoneal dialysis, with uncertain incidence for those on automated peritoneal dialysis. We report a case of a 30-year-old female patient with end-stage kidney disease with sudden dyspnea 2 days after starting automated peritoneal dialysis. Her chest x-ray revealed a significant pleural effusion on the right side. A thoracocentesis was performed, with a pleural glucose/plasma glucose of 1.08. Additionally, a computed tomography scan revealed a pleuroperitoneal communication upon dialysate infusion added with media contrast. A pleural-to-serum glucose gradient of greater than 50 mg/dL may indicate the diagnosis of a pleuroperitoneal communication in patients on peritoneal dialysis. Current literature also indicates that a pleural-to-serum glucose ratio above 1.0 may provide a more sensitive analysis. This case highlights the diagnosis process for this complication, with both laboratory and image findings corroborating the clinical hypotheses of a pleuroperitoneal communication in a patient on automated peritoneal dialysis.

International survey among hepatologists and pulmonologists on the hepatic hydrothorax: plea for recommendations.

BACKGROUND: The Hepatic hydrothorax is a pleural effusion related to portal hypertension; its diagnosis and therapeutic management may be difficult. The aims of this article are which follows: To gather the practices of hepatogastroenterologists or pulmonologists practitioners regarding the diagnosis and management of the hepatic hydrothorax. METHODS: Practitioners from 13 French- speaking countries were invited to answer an online questionnaire on the hepatic hydrothorax diagnosis and its management. RESULTS: Five hundred twenty-eight practitioners (80% from France) responded to this survey. 75% were hepatogastroenterologists, 20% pulmonologists and the remaining 5% belonged to other specialities. The Hepatic hydrothorax can be located on the left lung for 64% of the responders (66% hepatogastroenterologists vs 57% pulmonologists; p = 0.25); The Hepatic hydrothorax can exist in the absence of clinical ascites for 91% of the responders (93% hepatogastroenterologists vs 88% pulmonologists; p = 0.27). An Ultrasound pleural scanning was systematically performed before a puncture for 43% of the responders (36% hepatogastroenterologists vs 70% pulmonologists; p < 0.001). A chest X-ray was performed before a puncture for 73% of the respondeurs (79% hepatogastroenterologists vs 54% pulmonologists; p < 0.001). In case of a spontaneous bacterial empyema, an albumin infusion was used by 73% hepatogastroenterologists and 20% pulmonologists (p < 0.001). A drain was used by 37% of the responders (37% hepatogastroenterologists vs 31% pulmonologists; p = 0.26).An Indwelling pleural catheter was used by 50% pulmonologists and 22% hepatogastroenterologists (p < 0.01). TIPS was recommended by 78% of the responders (85% hepatogastroenterologists vs 52% pulmonologists; p < 0.001) and a liver transplantation, by 76% of the responders (86% hepatogastroenterologists vs 44% pulmonologists; p < 0.001). CONCLUSIONS: The results of this large study provide important data on practices of French speaking hepatogastroenterologists and pulmonologists; it appears that recommendations are warranted.

Clinical features of fetal hydrothorax associated with mucopolysaccharidosis-VII.

Mucopolysaccharidosis (MPS)-VII, called Sly disease, is a lysosomal storage disorder that can cause fetal hydrops, including fetal hydrothorax (FHT). We describe two fetal cases that received thoracoamniotic shunting for FHT, which was later found to be associated with MPS-VII by exome sequencing. Bilateral FHT accompanied by skin edema and ascites was found before 20 weeks of gestation in both cases. One fetus died in utero at 35 weeks of gestation, and the other survived with preterm delivery at 30 weeks of gestation. Both cases inherited compound pathogenic variants of GUSB from parents. Comparison with previously reported primary FHT cases revealed distinct clinical features in MPS-VII-associated FHT: early gestational age at diagnosis (<26 weeks), bilateral effusion, skin edema with ascites, and poor survival. A genetic analysis would be considered for FHT cases, with consideration of shunting when they show early-onset bilateral effusions with skin edema and ascites.