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Hepatic hydrothorax (HH) is a significant complication of cirrhosis associated with increased mortality. Liver transplantation (LT) remains the best treatment modality. We aim to assess predictors of mortality and the survival benefit of LT in patients with HH. A prospectively maintained cohort of adult patients with cirrhosis, being evaluated for LT at our institution, was retrospectively reviewed from 2015 to 2020. The primary outcome was death or LT. Cox proportional hazard regression identified associations between covariates and death. We calculated the years saved due to LT by comparing patients who were on the waiting list with patients who received an LT. This was done by calculating the area under the Kaplan-Meier curve. Censoring occurred at the time of the last follow-up or death. Patients with refractory HH had the lowest median survival of only 0.26 years. Within the HH group, having a refractory HH group was significantly associated with an increased risk of mortality (HR 1.73; 95% CI 1.06-2.81; p -value 0.03). Refractory HH was also significantly associated with mortality when evaluated in the entire cohort and after adjusting for other covariates (HR 1.48, 95% CI 1.03-2.11; p -value 0.03). Patients with refractory HH had the highest 1-year survival benefit with LT (0.48 y), followed by patients with non-refractory HH (0.28 y), then patients with other complications of cirrhosis (0.19 y). In this large study evaluating the prognostic impact of HH on patients with cirrhosis, refractory HH was an independent predictor of mortality. LT provides an additional survival benefit to patients with HH compared with those without HH., (Copyright © 2023 American Association for the Study of Liver Diseases.)

Purpose:  Hepatic hydrothorax (HH) is defined as transudate in the pleural cavity in patients with decompensated liver cirrhosis (DC) without concomitant cardiopulmonary or pleural disease. It is associated with high short-term mortality. HH can evolve via translocation through diaphragmatic gaps. The aim of this study was to evaluate the feasibility and safety of injecting ultrasound contrast medium into the peritoneal cavity to detect HH., Materials and Methods:  This study included patients with concomitant ascites and pleural effusion who were admitted to our hospital between March 2009 and February 2019. A peritoneal catheter was inserted and ultrasound contrast medium was injected into the peritoneal cavity. In parallel, the peritoneal and pleural cavities were monitored for up to 10 minutes., Results:  Overall, 43 patients were included. The median age was 60 years and the majority of patients were male (n = 32, 74 %). Most patients presented with right-sided pleural effusion (n = 32, 74 %), 3 (7 %) patients with left-sided and 8 (19 %) patients had bilateral pleural effusion. In 12 (28 %) patients ascites puncture was not safe due to low volume ascites. Thus, the procedure could be performed in 31 (72 %) patients. No adverse events occurred. In 16 of 31 (52 %) patients we could visualize a trans-diaphragmic flow of microbubbles. The median time until transition was 120 seconds., Conclusion:  Our clinical real-world experience supports the safety and feasibility of intraperitoneal ultrasound contrast medium application to detect HH in patients with DC, as a non-radioactive real-time visualization of HH. Our study comprises the largest cohort and longest experience using this method to date., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Background: Spontaneous bacterial empyema (SBEM) is a rare complication of hepatic hydrothorax characterized by hydrothorax infection in the absence of pneumonia., Aims and Methods: We conducted this study to compare clinical outcomes in SBEM patients who underwent early thoracentesis (ET) (≤ 24 h from presentation) versus those who underwent delayed thoracentesis (DT). All patients diagnosed with SBEM at Mayo Clinic Rochester, Minnesota from January 1st 1999 to December 31st 2020 were reviewed. Demographics, pleural fluid studies, laboratory results and clinical outcomes were analyzed., Results: A total of 54 SBEM patients (27 ET and 27 DT) were identified with 38 (70.4%) of patients presenting with right-sided effusions. Both groups had similar baseline characteristics. The rate of ICU admission was significantly higher in the DT group (15 (55.6%) vs. 7 (25.9%) patients, P = 0.027). Patients with DT had similar rate of AKI (11 (40.7%) vs. 6 (22.2%) patients, P = 0.074). In-hospital mortality (11 (40.7%) vs. 2 (7.4%) patients, P = 0.004), 3-month mortality (16 (59.3%) vs. 2 (7.4%) patients, P < 0.001) and 1-year mortality rate (21 (77.8%) vs. 6 (22.2%) patients, P < 0.001) were higher in the DT group., Conclusion: Patients with SBEM who underwent thoracentesis after 24 h from presentation (DT) had higher rates of mortality and ICU admission compared to patients who received early thoracentesis. Thoracentesis should be performed early in patients with suspected SBEM since it may improve survival., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2022. Published by Elsevier Ltd.)

Meigs syndrome is a rare disease defined by the coexistence of benign ovarian neoplasm, ascites and hydrothorax, which mainly affects women over the age of 30. This clinical condition refers only to cases in which the ovarian neoformation is a fibroid, a thecoma, a granulosa cell tumor or a Brenner tumor with disappearance of symptoms and effusions after removal of the neoplasm. Meigs syndrome is most frequently characterized by the presence of an ovarian fibroid, which in childhood is very rare and not commonly associated with the disease. In this article we report the case of an 11- year-old girl who came to our observation for a high fever for five days accompanied by cough and abdominal pain; imaging methods revealed bilateral hydrothorax, ascites, and a voluminous expansive right ovarian formation. On histological examination, the mass showed a cellular fibroid and the diagnosis of Meigs syndrome was made. Furthermore, we present a review of the literature aimed at detecting the state of knowledge on this disease in pediatric age, giving particular emphasis to the condition for which, in the presence of pleural effusion and ascites, an ovarian neoformation is not necessarily malignant. KEY WORDS: CT, Meigs syndrome, Pediatric, Pelvic mass, Ultrasounds.

Background: Hepatic hydrothorax (HH) is described as pleural effusion secondary to liver cirrhosis after ruling out other etiologies. We aim to assess the efficacy of an indwelling pleural catheter (IPC) placement in refractory HH in this systematic review and meta-analysis., Methods: A comprehensive search of literature was performed from inception to December 2020. The authors reviewed, selected, and abstracted the data from eligible studies into Covidence, a systematic review software. Cochrane criteria was used to rate each study for the risk of bias. The data abstracted were described using a random-effects model. Heterogeneity was evaluated using the I2 test., Results: Ten studies involving a total of 269 patients were included. The studies were analyzed for the proportion of pleurodesis achieved, the average time to pleurodesis, total complication rate, pleural infection rate, and mortality. A proportion of 47% of the total subjects included achieved spontaneous pleurodesis in an average duration of 104.3 days. The frequency of total complication rate was noted to be 30.36%. The incidence of pleural cavity infection was described to be 12.4% and death resulting from complications of IPC was 3.35%., Conclusion: The current management options for the refractory pleural effusion in HH include repeated thoracenteses, transjugular intrahepatic portosystemic shunt, surgical repair of defects in the diaphragm, and liver transplantation. However, the cost, eligibility, and availability can be some of the major concerns with these treatment modalities. With this meta-analysis, we conclude that IPCs can provide an alternative therapeutic option for spontaneous pleurodesis., Competing Interests: Disclosure: There is no conflict of interest or other disclosures., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Spontaneous bacterial empyema (SBE) is an infection of a preexisting hepatic hydrothorax (HH). We aim to describe the experience in managing SBE in a liver transplant (LT) referral center and assessing the incidence and mortality rates of SBE after conducting a systematic review., Methods: 992 patients with cirrhosis were retrospectively reviewed from 2015 to 2020. SBE was diagnosed by (i) positive microbiological culture and polymorphonuclear leukocyte count >250 cells/µL or (ii) negative microbiological culture, compatible clinical course, and polymorphonuclear count >500 cells/µL in pleural fluid. Furthermore, we conducted a comprehensive literature search of MEDLINE, EMBASE, and Google Scholar for studies evaluating SBE., Results: Twelve patients (10.4%) had spontaneous bacterial empyema out of 115 patients with HH. Five patients underwent LT, 6 had died, and 1 did not get transplanted and was alive throughout the duration of follow-up. Ten studies were included in the systematic review. Pooled incidence in patients with HH was 19.03%. Only 20.69% of the patients received a LT. Pooled mortality rate was 46.45%, with only 3.45% of the patients dying post-transplant., Conclusion: SBE is a severe complication of cirrhosis and HH. LT may provide a survival benefit. Thus, patients should be considered for early transplant.

Case: We report the case of a 27-year-old man who presented after a 30-foot fall with a hemopneumothorax, multiple rib fractures, and left shoulder dislocation with greater tuberosity fracture. During attempted arthroscopic repair, the patient developed pulseless electrical activity arrest as the result of intraoperative tension hydrothorax. We hypothesize that this was caused by a traumatic soft-tissue communication to his shoulder joint from the apex of his lung., Conclusion: To our knowledge, this is a unique and unreported complication of shoulder arthroscopy and should increase awareness about possibly delaying surgery in the setting of significant soft-tissue trauma., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B711)., (Copyright © 2021 by The Journal of Bone and Joint Surgery, Incorporated.)

Cirrhotic cardiomyopathy (CCM) is a chronic cardiac dysfunction in patients with cirrhosis and is characterized by altered diastolic relaxation, blunted contractile response to stress, and electrophysiological abnormalities;however, causes of CCM are unknown. Moreover, reduced cardiac afterload due to cirrhosis-related vasodilatation often masks cardiac insufficiency, whereas rapid hemodynamic overload reveals the presence of cirrhotic cardiomyopathy. Herein, we present the case of previously unrecognized cirrhotic cardiomyopathy that became overt with the development of severe acute cardiac failure. The rapidly worsening hepatic hydrothorax increased cardiac preload and intrathoracic pressure, which impaired cardiac filling. Furthermore, cardiac contractile function might have been worsened by hypoxia due to passive atelectasis and concomitant anemia.

Objective: To assess causative factors, associated imaging findings, and CT course of round atelectasis (RA)., Materials and Methods: We retrospectively reviewed CT reports for "round" or "rounded atelectasis" over a 5-year time frame. Patients with at least 2 CT scans a minimum of 6 months apart were included. Electronic medical records and clinical and imaging follow-up was reviewed for all cases., Results: Study population included 50 individuals with mean age of 63 years, and 59 unique instances of RA. The most commonly associated etiologies were hepatic hydrothorax (26%, n = 13) and asbestos exposure (26%), followed by post-infectious pleural inflammation (22%), congestive heart failure (12%), and end stage renal disease (8%). RA was found in the right lower lobe in over half of cases (n = 30). Association with one or more pleural abnormality was identified in all cases, including thickening (88%), fluid (60%), or calcification (40%). Nearly one third (n = 19) demonstrated intra-lesional calcification. In those who underwent PET/CT (20%), lesions demonstrated an average SUV of 2.2 (range 0-7.8). CT course over mean follow up of 32 months (range 6-126 months), demonstrated RA to remain stable (n = 26) or decrease (n = 26) in size in the majority (88%) of cases., Conclusion: Round atelectasis may arise from diverse etiologies beyond asbestos, and will most often decrease or remain stable in size over serial exams. Accurate identification may obviate the need for added diagnostic interventions., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: The objective was to evaluate if existence of hydrothorax in initial chest radiograph predicts treatment outcome in patients with primary spontaneous pneumothorax who received needle thoracostomy., Methods: This is a retrospective cohort study carried out from January 2011 to August 2014 in 1 public hospital in Hong Kong. All consecutive adult patients aged 18years or above who attended the emergency department with the diagnosis of primary spontaneous pneumothorax with needle aspiration performed as primary treatment were included. Age, smoking status, size of pneumothorax, previous history of pneumothorax, aspirated gas volume and presence of hydropneumothorax in initial radiograph were included in the analysis. The outcome was success or failure of the needle aspiration. Logistic regression was used to identify the predicting factors of failure of needle aspiration., Result: There were a total of 127 patients included. Seventy-three patients (57.5%) were successfully treated with no recurrence upon discharge. Among 54 failure cases, 13 patients (10.2%) failed immediately after procedure as evident by chest radiograph and required second treatment. Forty-one patients (32.3%) failed upon subsequent chest radiographs. Multivariate logistic regression showed factors independently associated with the failure of needle aspiration, which included hydropneumothorax in the initial radiograph (odds ratio [OR]=4.47 [1.56i12.83], P=.005), previous history of pneumothorax (OR=3.92 [1.57-9.79], P=.003), and large size of pneumothorax defined as apex-to-cupola distance ≥5cm (OR=2.75 [1.21-6.26], P=.016)., Conclusions: Hydropneumothorax, previous history of pneumothorax, and large size were independent predictors of failure of needle aspiration in treatment of primary spontaneous pneumothorax., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Spontaneous bacterial empyema, a complication of hepatic hydrothorax in cirrhotic patients, is a rare but nevertheless important medical entity. The diagnosis, treatment and prognosis of spontaneous bacterial empyema differs from the "usual" empyema, and avoiding proper diagnosis or treatment delay may have far reaching consequences. We report on a patient who was diagnosed in our department with spontaneous bacterial empyema, review the main current literature on the subject and elaborate on the specific therapeutic considerations related to the case management.

Purpose of Review: Pleural effusion is a common clinical problem resulting from a wide range of diseases. Treatment options include targeting the primary cause or, in persistent cases, invasive removal of the excess fluid from the pleural cavity. In this review, we summarize the experimental data concerning pharmacological agents that influence pleural fluid resorption and examine their potential as a novel noninvasive treatment strategy., Recent Findings: Recently published evidence indicates that adrenergic agents and corticosteroids can increase pleural fluid clearance from the cavity. On the contrary, paracetamol and certain nonsteroid anti-inflammatory drugs can impede fluid outflow. These concepts are based on data extracted by in-vivo studies using provoked hydrothoraces in rabbits and mice, as well as by ex-vivo electrophysiological experiments using sheep and human pleural tissue., Summary: In conclusion, the available experimental data indicate that certain pharmacological agents may impact fluid resorption, thus affecting pleural fluid accumulation and the rate of pleural effusion resolution.

Hepatic hydrothorax is defined as a pleural effusion in patients with liver cirrhosis in the absence of cardiopulmonary disease. The estimated prevalence among patients with liver cirrhosis is approximately 5-6%. The pathophysiology involves the passage of ascitic fluid from the peritoneal cavity to the pleural space through diaphragmatic defects. The diagnosis is made from clinical presentation and confirmed by diagnostic thoracentesis with pleural fluid analysis. The initial medical management is sodium restriction and diuretics, but liver transplantation provides the only definitive therapy. For patients who are not transplant candidates and those who await organ availability, other therapeutic modalities that are to be considered include transjugular intrahepatic portosystemic shunt placement, videoassisted thoracoscopic surgery repair, pleurodesis, and vasoconstrictors (eg, octreotide and terlipressin). The primary therapeutic goals are to reduce ascitic fluid production and improve symptoms to bridge the time for liver transplantation.

Pulmonary concerns in liver transplant candidates have intraoperative and outcome implications. Evolving MELD exception policies address transplant priority for problems such as hepatopulmonary syndrome, portopulmonary hypertension, and hemorrhagic hereditary telangiectasia. Other pulmonary issues such as refractory hepatic hydrothorax, advanced chronic obstructive lung disease (including alpha-1 antitrypsin deficiency) and indeterminate pulmonary nodules may affect liver transplant consideration. Herein, we discuss current pulmonary-related contraindications, indications and MELD exception policies for liver transplantation, suggesting future considerations., (Copyright © 2013 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)

A 64-year-old woman with liver cirrhosis caused by hepatitis C was presented with aggravated dyspnea. She had refractory hepatic hydrothorax, requiring pleural puncture and drainage of approximately 3,000 ml per week. Four days after the last puncture, she consulted the emergency department and chest films revealed right tension pneumothorax. A drainage tube was inserted to her right pleural cavity, but middle and lower lobes were not expanded, and air leaks persisted. We thought that she was in high-risk of infections, like empyema, and needed surgical treatment to close the pulmonary fistula promptly. Considering her poor general condition, we performed local anesthetic thoracoscopic talc poudrage, and air leaks were controlled successfully. Perioperative period was uneventful.

Intra-abdominal fluid may migrate readily into the pleural space through naturally occurring holes in the diaphragm or intradiaphragmatic lymphatics. Although any type of fluid in the abdomen may migrate, additional pathologic mechanisms are involved in the development of chylous ascites/chylothorax, yellow nail syndrome, urinothorax, pancreaticopleural fistulas, or other connections. In the differential diagnosis of the large list of potential pleural fluid causes, intra-abdominal sources should be entertained by the practicing physician in the right clinical context., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Hepatic hydrothorax is a major pulmonary complication of liver disease occurring in up to 5-10% of patients with cirrhosis., Case Presentation: We report four observations of the development of pneumothorax ex-vacuo or trapped lung in the setting of hepatic hydrothorax. The diagnosis of trapped lung was made based on the presence of a hydropneumothorax after evacuation of a longstanding hepatic hydrothorax with failure of the lung to re-expand after chest tube placement in three of the four cases. Two patients underwent surgical decortication with one subsequent death from post-operative bleeding. The other two patients remarkably had spontaneous improvement of their "trapped lung" without surgical intervention., Conclusions: While pneumothorax ex-vacuo is a known phenomenon in malignant effusions, to our knowledge, it has never been described in association with hepatic hydrothoraces. The pathophysiology of this phenomenon remains unclear but could be related to chronic inflammation with development of a fibrous layer along the visceral pleura.

Purpose of Review: Spontaneous bacterial empyema (SBEM) is defined as spontaneous infection of a preexisting hepatic hydrothorax. SBEM is diagnosed after exclusion of pneumonia, and, in patients with liver cirrhosis, it is still associated with significant morbidity and mortality. To date, studies focusing on SBEM are rare. This review will highlight the recent data focusing on the clinical characteristics, bacteriology, management, and outcome predictors of cirrhotic patients with SBEM., Recent Findings: The Enterobacteriaceae (Escherichia coli and Klebsiella pneumoniae) are the major causative pathogens isolated from SBEM patients. Therefore, the cornerstone of therapy is antibiotic therapy with immediate empirical use of third-generation cephalosporins as first-line treatment. Chest-tube placement is not necessary. Regression analysis identified three independent factors related to poor outcome: high score of the model for end-stage liver disease-sodium (MELD-Na), initial ICU admission, and initial antibiotic treatment failure. High MELD-Na score may be a useful predictor of SBEM mortality in cirrhotic patients., Summary: Although SBEM is a rare complication of cirrhosis, the high mortality rate should increase physicians' index of suspicion in cirrhotic patients with hydrothorax and prompt immediate diagnostic thoracentesis. The MELD-Na score rather than Child-Pugh score may be a strong predictor of in-hospital mortality of SBEM patients.

Hepatic hydrothorax is defined as a transudative pleural effusion, usually greater than 500 mL, in patients with portal hypertension without any other underlying primary cardiopulmonary cause. It develops most likely because of diaphragmatic defects that allow for passage of fluid from the peritoneal space to the pleural space. Because of the mechanical constraints of the thoracic cavity, this complication of portal hypertension can be challenging to treat because patients will become symptomatic when as little as 500 mL of fluid is present in the pleural space. Treatments include salt restriction, diuretics, thoracentesis, transjugular intrahepatic portosystemic shunt, video-assisted thoracoscopy, and pleurodesis. It is important to note that a chest tube is not a potential treatment option; a hepatic hydrothorax should not be treated with a chest tube unless there is frank pus in the pleural fluid or a pneumothorax is present. The ultimate treatment is a liver transplant; the development of a hepatic hydrothorax thus warrants a referral to a liver transplant center., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Parenteral selenium (Se) and vitamin E (Vit E) were administered to all newborn kids at a Boer goat farm where there was previous high neonatal mortality assumed to be due to nutritional myopathy. All treated kids were affected by severe respiratory distress and died within 8 hours of Se/Vit E administration. Gross lesions included severe pulmonary edema, hydrothorax, and hydropericardium. The primary histopathologic finding was severe, acute, and monophasic myocardial contraction band necrosis. The diagnosis was accidental acute selenosis based on trace mineral analysis of the liver. This case highlights an important differential diagnosis in cases of acute myocardial contraction band necrosis and sudden death in goats and emphasizes the need for caution when administering parenteral Se/Vit E preparations.

Hepatic hydrothorax usually presents in association with ascites, but there are rare cases when it does not. This case helps to support the differential of hepatic hydrothorax in patients who have a history of liver cirrhosis, portal hypertension, and recurrent pleural effusions without ascites. We hope to support the conclusion that a patient with recurrent pleural effusions, without ascites, does not exclude gastrointestinal involvement in its etiology.

Purpose: To evaluate the effectiveness of the PleurX catheter in the management of recurrent non-malignant pleural effusions., Methods: All subjects who underwent a PleurX catheter placement between 2003 and 2009 were evaluated. General demographic data, time to pleurodesis, complications, and a satisfaction questionnaire were collected. The subjects were divided into two groups. Group I included patients with non-malignant effusions and group II included patients with malignant effusions., Results: A total of 64 subjects were included in the final data analysis. A total of 23 subjects were included in group I and 41 subjects were included in group II. The diagnoses in group I included congestive heart failure (CHF; 13), hepatic hydrothorax (8), traumatic bloody (1), and idiopathic exudative (1). The diagnoses in group II included lung cancer (20), breast cancer (11), colon cancer (5), prostate cancer (2), B-cell lymphoma (2), and mesothelioma (1). The time to pleurodesis was 36 ± 12 days for group II compared to 110.8 ± 41 days for group I (p < 0.0001). The mean satisfaction score was similar in both groups (3.8 ± 0.4). Time to pleurodesis was significantly shorter in hepatic hydrothorax compared to CHF (73.6 ± 9 days vs. 113 ± 36 days, p = 0.006). There was one case of exit site infection in a patient with hepatic hydrothorax. Among subjects who were alive at 3 months after the catheter removal, none had recurrence of their pleural effusion., Conclusion: The Denver catheter was effective in achieving pleurodesis in non-malignant pleural effusions. The complication rate was low and patient satisfaction was high.

Congenital volvulus is a life-threatening condition, both for the fetus and for the newborn. A volvulus is a twist of small bowel loops or a proximal part of the colon around the mesenteric artery or its branches. The potential consequences of volvulus are ileus and necrosis of the intestinal wall. Prenatal diagnosis of midgut volvulus is difficult. It should be suspected antenatally when polyhydramnios, intestinal dilatation, ascites and/or signs of fetal anemia are present on ultrasound assessment. We report a case of a congenital midgut volvulus associated with fetal anemia. The fetal ultrasound performed at 32 weeks' gestation showed a polyhydramnios, hydrothorax, thick ascites accumulation around the liver and the suspicion of a dilated bowel loop. Additionally, Doppler examination showed an increased value of peak systolic velocity in the middle cerebral artery. Cordocentesis confirmed significant fetal anemia. At 34 weeks, because of the suspicion of idiopathic meconium ileus and secondary anemia, a Cesarean section was performed after the administration of steroids. During the laparatomy, performed postnatally, a midgut volvulus was diagnosed. The affected portion of the ileum was resected and end-to-end anastomosis performed. An antenatal diagnosis of midgut volvulus should be considered when signs of fetal anemia, including an increased value of peak systolic velocity in the middle cerebral artery, are present with polyhydramnios, fetal ascites, dilated bowel loops on antenatal ultrasound. An assessment of the fetal hemodynamic status should be a part of the ultrasound assessment for patients with nonspecific fetal bowel pathologies, including congenital volvulus., (Copyright (c) 2010 S. Karger AG, Basel.)

Prenatal diagnosis of generalized arterial calcification of infancy (GACI) (OMIM #208000) is difficult and rare. There are various known gene mutations in ENPP1 (ectonucleotide pyrophosphatase/phosphodiesterase 1) locus 6q22-q23. We present a case of suspected intrauterine diagnosis at 29 weeks of gestation in a consanguineous couple. The sonographic findings were fetal hydrops (hydrothorax, skin edema, ascites, pericardial effusion and polyhydramnion), echogenic great arteries and pathological Doppler findings. An intrauterine therapy with bisphosphonates was considered, but delayed due to rapid deterioration in fetal Doppler flows with suspected fetal asphyxia. The couple was informed about the most unfavorable prognosis in fetal hydrops, however, they opted for elective delivery. A cesarean section was performed. Early neonatal death occurred due to primary intracranial hemorrhage. Postmortem and genetic testing confirmed a novel mutation in the ENPP1 gene., (Copyright (c) 2009 S. Karger AG, Basel.)

Background: Primary fetal pleural effusion can be associated with high perinatal morbidity and mortality, especially when it is associated with the presence of fetal hydrops. Pleuroamniotic shunting results in effective drainage and lung expansion which prolong the pregnancy and improve neonatal survival. Intrathoracic displacement of the shunt is a rare but a known complication of shunt insertion and can cause some infant morbidity. We present a case of successful antenatal treatment of primary fetal pleural effusion complicated with intrathoracic displacement of the shunt without any fetal and long-term infant morbidity., Case Report: Our case presented with severe bilateral fetal pleural effusion, causing fetal hydrops treated successfully by pleuroamniotic shunting at 25 weeks of gestation which was complicated by dislodgement of the shunt into the fetal chest documented by antenatal ultrasound at 30 weeks. There was no recurrence of fetal pleural effusion or other antenatal complication. After delivery there were no postnatal complications, so removal of the shunt was not considered, and clinical follow-up of the infant to the age of 12 months was uneventful., Conclusion: The complication of intrathoracic displacement of a pleuroamniotic shunt can be recognized antenatally by ultrasound. Since there are no postnatal pulmonary complications related to the presence of the shunt inside the infant chest, conservative management in these cases appears to be a safe approach., ((c) 2009 S. Karger AG, Basel.)

Purpose of Review: This review highlights the pathophysiological mechanisms, incidence, clinical features, as well as the diagnosis and treatment of pleural effusions in the neonate., Recent Findings: Pleural effusions are rare except in hydropic neonates. Elevated pleural fluid/serum immunoglobulin G ratio may be a diagnostic marker for congenital chylothorax in utero. Chylothorax may be congenital or acquired. Hydrothoraces may appear at any time during the neonatal period and are related to infectious and noninfectious aetiologies. Haemothorax is defined as the presence of blood in the pleural space. Parenteral nutrition leakage may occur in a newborn with a venous central catheter leading to an effusion that looks like a chylothorax. The value of elevated pleural fluid N-terminal pro-brain natriuretic peptide levels as a marker of congestive heart failure is not yet established in neonates. More recently, in cases of chylothorax that did not resolve with drainage and bowel rest, the use of somatostatin or its analogue octreotide has been described with success. If conservative management fails after 5 weeks, surgical intervention is indicated., Summary: Clinicians must be aware of the wide range of disorders causing pleural effusions, the different types and clinical presentations, differential diagnosis, and how to treat each specific case.

Background: Meig's and Pseudo-Meig's syndromes have been reported in association with several malignancies but the concomitant existence of multiple synchronous benign and malignant tumors in association with Pseudo-Meigs' syndrome has not been reported in the published literature., Case: A 55-year old African American woman presented with mild asthma exercebation, right ovarian mass, hydrothorax and elevated CA-125 levels. Histological examination confirmed a right ovarian carcinoid tumor embedded within a mature cystic teratoma while the left ovary, fallopian tube and the uterus contained a poorly differentiated adenocarcinoma of the endometrium., Conclusion: This is the first case report of Pseudo-Meig's syndrome in association with ovarian carcinoid tumor and multiple synchronous benign and malignant pelvic tumors.

Background: Until now, the pathophysiology of hepatic hydrothorax has been moot. We discuss (on the basis of gross videothoracoscopy findings in 11 cases and the literature) the pathogenesis and clinical presentation of this complex condition., Methods: We prospectively studied 11 patients (age, 31-73 years; 6 men and 5 women) with refractory hepatic hydrothorax (Child-Pugh class B-C) who underwent thoracoscopic repair of diaphragmatic defects. The diaphragmatic defects were examined intraoperatively., Results: The diaphragmatic defects stemming from hepatic hydrothorax were classified into 4 morphologic types: type I, no obvious defect (1 patient); type II, blebs lying on the diaphragm (4 patients); type III, broken defects (fenestrations) in the diaphragm (8 patients); and type IV, multiple gaps in the diaphragm (1 patient). The type of diaphragmatic defect did not correlate with the volume occupied by the pleural effusion in the preoperative chest radiograms., Conclusions: The finding of this study allowed hepatic hydrothorax pathophysiology to be directly visualized, and further studies concerning the treatment of hepatic hydrothorax might be based on these mechanisms.

The frequency of hepatic hydrothorax in cirrhotic patients is reported to be approximately 5%. The pleural effusion is predominantly right-sided (85% of cases) but may be bilateral. Although most often accompanied by significant ascites, it can occur in its absence. We report a case of a right-sided acute hepatic hydrothorax as a result of residual motor blockade during anesthesia recovery and without previous evidence of clinical ascites. This complication should be considered by the anesthesiologist in every cirrhotic patient, with or without clinical evidence of ascites.

Background: Spontaneous bacterial empyema (SBEM) is a rare complication of portal hypertension. The characteristics and risk factors for SBEM are not well known. This study was performed to investigate the risk factors for SBEM in cirrhotic patients with hydrothorax., Methods: From July 1996 to December 1998, 862 cirrhotic patients were studied. All patients underwent chest radiography, abdominal sonography or computed tomography after admission to detect the existence of pleural effusion. Pleural fluid was obtained after thoracentesis and sent for analysis. The clinical and laboratory data from patients with sterile hydrothorax and from SBEM at the time of first episode were compared., Results: Seventeen patients had 26 episodes of SBEM during the study period, 56% (14 of 26) of these SBEM episodes were associated with spontaneous bacterial peritonitis (SBP) and 31% (8 of 26) were associated with bacteremia. The incidence of SBEM was 2% (17 of 862) in cirrhotic patients and 13% (17 of 132) in cirrhotics with hydrothorax. Patients with SBEM had a higher Child-Pugh score, lower serum albumin, prolonged prothrombin time, lower pleural fluid protein, and higher rate of associated SBP than patients with sterile hydrothorax. Multivariate analysis revealed that pleural fluid protein level (p = 0.0035) and presence of SBP (p = 0.0062) were predictive factors of SBEM. The hospitalization mortality rate of SBEM was 38%., Conclusions: Patients with advanced liver disease, low pleural fluid protein level, or SBP are predisposed to SBEM. A diagnostic thoracentesis should be performed in cirrhotic patients with pleural effusion when infection is suspected or clinical deterioration occurs.

Objective: Fetal hydrothorax may lead to hydrops and is associated with mortality as high as 50%. The objective of this study was to define the pathophysiology of fetal hydrothorax and its relation to hydrops., Methods: Measurements from echocardiograms of 33 fetuses diagnosed with hydrothorax were made, and included diameters of the thorax, heart, inferior vena cava, right ventricle (RV), left ventricle (LV) and aortic and pulmonary valves. Doppler-derived velocities were measured in the aorta and pulmonary artery just above the aortic and pulmonary valves. The ratio of the area of the effusion to the area of the thorax (effusion ratio) was calculated. Variables were converted into Z-scores from regression equations based on normal data. Features of fetuses with and without hydrops were compared., Results: Higher effusion ratios were noted in hydropic versus non-hydropic fetuses. Compared to a normal population, study subjects had smaller dimensions of LV, RV and aortic and pulmonary valves. They also had higher pulmonary artery peak velocities. The comparison between non-hydropic and hydropic fetuses revealed lower values for LV and pulmonary valve dimensions, and peak aortic velocity in hydropic fetuses. The severity of LV compression correlated significantly with effusion ratio., Conclusion: Fetal hydrothorax is accompanied by compression of the cardiac structures, resulting in altered cardiac hemodynamics. Echocardiographic assessment, including the measurement of effusion ratio, may be a useful tool in guiding fetal therapy., (Copyright 2003 ISUOG. Published by John Wiley & Sons, Ltd.)

We treated a case of primary fetal hydrothorax with hydrops. A pleuroamniotic shunt catheter inserted at 30 weeks accomplished resolution of hydrops and was maintained until cesarean delivery at 34 weeks with no need for further prenatal intervention. At age 9 months, the infant showed no effusion or pulmonary compromise.

Unlabelled: Pulmonary hypoplasia was diagnosed sonographically in 32 fetuses from 20 to 33 weeks of gestation. In addition to standard biometry, transverse thoracic diameter (TTD), sagittal thoracic diameter (TSD), thoracic circumference (TC) and lung diameter (LD) were measured in all cases and compared with known nomograms. The fetuses were divided into five groups according to the main sonographic findings: group 1-skeletal dysplasia; group 2-renal agenesis; group 3-diaphragmatic hernia; group 4-hydrothorax; and group 5-others. Severe pulmonary hypoplasia (PH) was diagnosed prenatally in all cases on the basis of LD measurements. In 17 (53.1 per cent) out of 32 cases TTD was below the 5th percentile while lower TSD measurements were recorded in 15 (46.8 per cent) fetuses. A thorax circumference below the 5th percentile for the respective gestational age was found in 15 cases (46.8 per cent) and a decreased LD/TC ratio in 25 cases (78.1 per cent). In 13 out of 32 fetuses pulmonary hypoplasia was diagnosed before, and in 19 cases after 24 weeks of gestation. Pulmonary hypoplasia was confirmed by autopsy in all cases., Conclusion: pulmonary hypoplasia can be sonographically detected before 24 weeks of gestation. In cases of skeletal dysplasia and renal agenesis pulmonary hypoplasia can be diagnosed by chest and lung measurements, whereas in diaphragmatic hernia and hydrothorax diagnosis of pulmonary hypoplasia is possible only by lung measurement., (Copyright 1999 John Wiley & Sons, Ltd.)