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PubMed1998

Pseudo-Meigs' syndrome caused by paraovarian fibroma.

Meigs' syndrome includes an ovarian tumor, usually fibroma, associated with hydrothorax and ascites. It is accepted that uterine tumors, like fibromas, can also be associated with ascites and hydrothorax, but this is extremely rare. The mechanism of formation of peritoneal and pleural effusion is not well documented. The most likely pathogenesis ascribes the fluid formation to the filtration of interstitial fluid in the peritoneal through the tumor capsule, and the diffusion to the pleural space through the diaphragm lymphatic vessels at the foramen of Bochdalek. Paraovarian fibromas are also extremely rare neoplasms, probably of paramesonephric origin. It has been hypothesised that they can develop by proliferation of connective tissue cells around the Wolfian remnants. In this article, probably for the first time, a case of paraovarian fibroma with ascites and hydrothorax is presented.

Ascites↗
PubMed2000

Noonan syndrome: a cryptic condition in early gestation.

Noonan syndrome is one of the most common of genetic syndromes and manifests at birth, yet it is usually diagnosed during childhood. Although prenatal diagnosis of Noonan syndrome is usually not possible, in a few cases the ultrasonographic findings suggested the diagnosis in utero. Reported sonographic clues include septated cystic hygroma, hydrothorax, polyhydramnios, and cardiac defects, such as pulmonic stenosis and hypertrophic cardiomyopathy. During a 6-year period, 46,224 live-born infants were delivered at the Chaim Sheba Medical Center. Seven newborn infants and four fetuses were found to have Noonan syndrome. One fetus showed transient nuchal translucency of 4 mm and bilateral neck cysts at the 13th gestational week. Both findings resolved spontaneously by the 18th gestational week, but during the third trimester this fetus developed hydrothorax, skin edema, and polyhydramnios. In the three other fetuses, first- and second-trimester ultrasonographic findings were normal, and the diagnosis of Noonan syndrome was suggested only during the third trimester. All three fetuses had polyhydramnios and skin edema. A cardiac malformation, hydrothorax, and a large head were present in one fetus. Sonographic facial findings were investigated. In all four fetuses posteriorly angulated, apparently low-set ears and depressed nasal bridge were identified. Wide nasal base was seen in two fetuses. In two fetuses, persistent opening of the fetal mouth was interpreted as fetal hypotonia. One fetus developed progressive postnatal hypertrophic cardiomyopathy and in one case, pulmonic stenosis became apparent at age 6 months. This small series suggests that Noonan syndrome has an evolving phenotype during in utero and postnatal life. Amelioration of early nuchal region findings and late onset of the more "typical" ultrasonographic changes may limit early prenatal detectability.

Adult↗
PubMed2005

Symptomatic pleural effusion without intrathoracic migration of ventriculoperitoneal shunt catheter.

BACKGROUND: Symptomatic pleural effusion following ventriculoperitoneal (VP) shunt insertion is very rare. The patient was an 8-year-old girl who had VP shunt for hydrocephalus as a result of aqueductal stenosis. Six weeks after surgery, she presented with headache, vomiting and drowsiness. She developed respiratory distress with pain in the right lower chest region and right hypochondrium on admission. METHODS: Chest X-ray confirmed right hydrothorax and showed the tip of the peritoneal catheter in the right suprahepatic subphrenic space. Her symptoms abated after the catheter was repositioned from the subphrenic region to the general peritoneal cavity. Repeat chest X-ray confirmed the resolution of the hydrothorax. DISCUSSION: Shunt review without thoracocentesis or thoracostomy is an effective treatment of symptomatic hydrothorax following VP shunt, especially when there is no intrathoracic shunt migration.

Child↗
PubMed1995

Extra-lobar pulmonary sequestration with prenatal diagnosis. A report of 5 cases and review of the literature.

UNLABELLED: THE PURPOSE of this work was to study the pre- and postnatal features of extra-lobar pulmonary sequestration (ELPS) and consider their diagnosis and treatment. METHODS: Five ELPS diagnosed prenatally (1986-1992) were reviewed retrospectively. RESULTS: In 2 cases, prenatal diagnosis was based on the presence of a left suprarenal mass for which tumor markers proved negative postnatally. These 2 infants underwent surgery at 3 weeks of age for supposed neuroblastoma or teratoma. In the other 3 cases, diagnosis was based on the presence of a solid mass at the left base of the thorax. The systemic vessel was visualized in 2 of these cases; mediastinal displacement was noted in one case and hydrothorax (which recurred after puncture) in the other. The latter infant was born at 34 weeks of amenorrhea, and hydrothorax disappeared postnatally after excision of the ELPS. The other two infants were asymptomatic at birth and underwent surgery respectively on the 8th day and during the 6th month of life. CONCLUSION: ELPS can take the form of a mass in the abdomen or at the base of the thorax. For subdiaphragmatic ELPS, surgical excision (possibly preceded by percutaneous puncture) is required if the diagnosis is uncertain. Supradiaphragmatic ELPS can be complicated prenatally by hydrothorax or even hydrops, requiring drainage in utero. If the infant is asymptomatic postnatally, systematic surgical excision should be considered. Extra-lobar pulmonary sequestrations (ELPS) are masses of nonfunctional lung tissue vascularized by an abnormal systemic artery and covered with a pleural layer isolating them from the rest of the parenchyma. The diagnosis of these malformations is based increasingly on obstetrical ultrasonography. The purpose of this study was to specify the prenatal features of these malformations and define the diagnostic approach and therapeutic strategy.

Bronchopulmonary Sequestration↗
PubMed2005

A long surviving case of Pseudomeigs' syndrome caused by Krukenberg tumor of the stomach.

Meigs syndrome is defined as the triad of benign ovarian tumor with ascites and pleural effusion that resolve after resection of the tumor. Pseudomeigs' syndrome is a serious disease characterized by malignant ovarian tumor, but ascites and hydrothorax usually reveal no malignant cells. Here, we report a 47-year-old pre-menapausal female patient with cardia cancer. Nearly 14 months after D3 dissection, she developed Krukenberg tumors on both ovaries causing a Pseudomeigs' syndrome with benign ascites and right hydrothorax, which resolved dramatically after resection of the tumors and rectouterine pouch peritonectomy. She survived nearly 3 years after metastasectomy with a total survival of 46 months. The patient died because of massive liver metastases. The present case suggests that Pseudomeigs' syndrome should be considered in patients with Krukenberg tumors, ascites and hydrothorax and that resection of the tumors may bring long-term palliation.

Cardia↗
PubMed1996

Congenital chylothorax in a patient with 21 trisomy syndrome.

A female infant with 21 trisomy syndrome associated with congenital chylothorax was reported. She was born at a gestational age of 34 weeks by Cesarean section because of fetal hydrothorax and hydrops fetus, confirmed by ultrasonography at 32 weeks. Emergent resuscitation and immediate thoracentesis were performed soon after birth. After beginning breast feeding, the serous pleural fluid became opalescent and a diagnosis of congenital chylothorax was made. Feeding was changed to medium-chain triglyceride (MCT) feeding and the production of pleural effusion disappeared after thoracentesis was performed several times. Accumulating evidence suggested that MCT feeding and intermittent thoracentesis under echo guide were effective. Some reports on patients, including this one, suggest that there may be more patients with 21 trisomy associated with congenital hydrothorax. Therefore, congenital hydrothorax might be listed as a complication of 21 trisomy.

Chylothorax↗
PubMed2006

Ballantyne syndrome: a case report.

Ballantyne syndrome (also called mirror syndrome or triple edema) describes the unusual association of fetal and placental hydrops with maternal preeclampsia. This is a case report illustrating a 37-year-old patient who was referred to our clinics at 28 weeks of gestation (wg) because of fetal hydrothorax. On examination, the woman did not show signs of preeclampsia. The fetal ultrasound examination revealed bulky hydrothorax, generalized subcutaneous edema, placental edema, and polyhydramnios. It was not possible to find the cause of the fetal hydrops. At 29 weeks and 4 days of gestation, the fetal hydrothorax was removed by two pleuro-amniotic shunts, but at the moment of our intervention anasarca was already present. In the following 3 days, despite observing bed rest, the mother developed edema of hands and face, while blood pressure remained normal. At 30 wg the patient underwent cesarean section because fetal movements ceased and the fetal heart rate monitoring showed loss of variability and decelerations. Before dying, the neonate lived for 20 days in a state of deep hypotension.

Adult↗
PubMed1993

Short-dwell peritoneal dialysis: increased use and impact on clinical outcome.

We reviewed 216 patients on peritoneal dialysis over a 3-year period to assess the effects on patient outcome of short-dwell dialysis (SDD), defined as dwell time below 4 hours with a daily dry (empty peritoneum) interval. Forty-nine patients (23%) required SDD for improved management of ultrafiltration failure (82%), effective blood pressure control (8%), abdominal wall hernia (4%), hydrothorax (4%), and patient convenience (2%). Ultrafiltration failure was recognized as the inability to achieve resolution of clinical overhydration, confirmed by the peritoneal equilibration test (PET), demonstrating high membrane glucose transport (absorption) and observed retention of dialysate volume. Daytime ambulatory peritoneal dialysis (DAPD) was used by 69% of patients and nightly peritoneal dialysis (NPD) with cyclers by 31%. Only one patient (hydrothorax) transferred to hemodialysis. Observations include sustained hydration and blood pressure control in all patients with maintenance of biochemical dialysis adequacy, less reliance on very hypertonic solutions, an increase in dry weight in 25% of patients, decreased use of antihypertensive agents, effective management of hernia and hydrothorax in 3 of 4 patients, and satisfactory patient tolerance of DAPD and NPD regimens, and daily dry intervals. Factors promoting SDD include improved understanding of PET studies, use of disconnect systems, and improvement in cycler design. We anticipate increasing use of SDD as recognition of its usefulness and application techniques expands.

Adolescent↗
PubMed1999

Prenatal sonographic chest and lung measurements for predicting severe pulmonary hypoplasia.

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.

Bone Diseases, Developmental↗
PubMed2004

Transjugular intrahepatic portosystemic shunts: experience in the oncology setting.

BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) placement has emerged as an effective and minimally invasive method of treating portal hypertension and its associated complications. To the authors' knowledge there is limited documentation of its use for percutaneous shunting in patients with hepatic and extrahepatic malignancies. The current study reports the authors' experience with TIPS in the oncology setting. METHODS: Thirty-eight patients with cancer underwent TIPS procedures. Nineteen patients had a history of hepatic malignancy. All medical records and imaging studies were reviewed retrospectively. The indication for TIPS, the presence of malignancy, procedural details, complications, survival, and treatment success were assessed. RESULTS: Primary technical success was accomplished in 37 of 38 patients (97%) without technical procedure-related complications. Hepatic encephalopathy occurred in 15 of 34 patients (44%), with 3 patients requiring shunt reduction. Premature shunt occlusion (< 30 days) occurred in 3 patients (8%). Recurrent hemorrhage occurred in 1 of 19 patients (5%), and ascites and hepatic hydrothorax resolved or improved subjectively in 9 of 12 patients (75%). Shunts traversed malignancy in 9 patients, and varying degrees of portal compromise were encountered in 12 patients (32%). The overall 30-day and 90-day survival rates were 84% and 60%, respectively. There was a statistically significant difference in 90-day survival rates for patients who had ascites and hepatic hydrothorax indications (27%) compared with patients who had variceal and portal gastropathy indications (84%; P = 0.0075). In addition, the 90-day survival rate was significantly lower in patients who had primary hepatic malignancies (36%) compared with the remainder of the study population (74%; P = 0.0077), and it was significantly lower in patients who had model for end-stage liver disease (MELD) scores > or = 12 (P = 0.0020). CONCLUSIONS: TIPS was performed safely for patients with cancer without increasing rates of procedure-related complications. However, some patients subgroups, such at those with malignancy and ascites, primary hepatic malignancy, or MELD scores > or = 12, had the lowest 90-day survival rates.

Adult↗
PubMed2001

Reliability of prenatal sonographic lung biometry in the diagnosis of pulmonary hypoplasia.

OBJECTIVE: The purpose of the present study was to assess the value of biometric lung measurements for the diagnosis of severe fetal pulmonary hypoplasia by investigating whether a significant correlation between two-dimensional lung biometry measurements and autopsy findings could be established. METHODS: This was a prospective study carried out between 1995 and 1997. Nomograms for normal fetuses of the anterior-posterior and transverse inner thoracic diameters, which describe the growth and shape of the lung, were used as a basis for diagnosis of pulmonary hypoplasia in fetuses at high risk of developing the condition (the fetuses had bilateral renal agenesis or multicystic kidneys; chronic PROM <25 gestational weeks or hydrothorax). Pregnancy was terminated by abortion or intrauterine death in 29/43 high-risk fetuses and autopsies were performed. Only the 29 fetuses for which there were autopsy findings were included in the study. RESULTS: The best plane for diagnosing pulmonary hypoplasia was the four-chamber view. The diagnostic accuracy for this view as expressed by the sensitivity was 57% for the anterior-posterior diameter and 44% for the transverse diameter; as expressed by the specificity it was 42% for the anterior-posterior diameter and 50% for the transverse diameter. The results for the four-chamber view for the various high-risk conditions were as follows: for fetuses with chronic PROM we obtained sensitivities of 75% and 50% (anterior-posterior and transverse dimensions, respectively) and specificities of 80% and 60% (anterior-posterior and transverse dimensions, respectively). The sensitivities of lung biometry in fetuses with hydrothorax were 1% and 80% for the two diameters, but there was a low specificity. In fetuses with bilateral renal agenesis or multicystic kidneys we obtained sensitivities of 36% and 30% (anterior-posterior and transverse dimensions, respectively) and a specificity of 50% (anterior-posterior dimension). CONCLUSIONS: The present results show that two-dimensional lung biometry is not a suitable method for antenatal detection of pulmonary hypoplasia. However, in individual cases with high risk for pulmonary hypoplasia, lung biometry might prove to be an additional diagnostic parameter.

Autopsy↗
PubMed2003

Congenital chylothorax: a case report.

A case of unilateral congenital hydrothorax diagnosed at 21 weeks and treated with a pleuro-amniotic shunt at 23 weeks' gestation is presented. The rapid production of pleural fluid led to the development of polyhydramnios which was treated with a selective Cox inhibitor. Due to worsening of the hydrothorax, a third thoracocentesis was performed at 30 weeks' gestation prior to a Cesarean section. The neonate responded well to mechanical ventilation, a thoracic drainer that was inserted between days 5 and 14, fluid and protein replacement and gradual transition from parenteral to intestinal nutrition. Early diagnosis of chylothorax should be treated by thoraco-amniotic drainage to prevent fetal pulmonary hypoplasia and congestive cardiac failure. Polyhydramnios is a complication of this therapy and may require treatment with a Cox inhibitor.

Adult↗
PubMed2004

Early and long-term clinical and radiological follow-up results of expanded-polytetrafluoroethylene-covered stent-grafts for transjugular intrahepatic portosystemic shunt procedures.

The purpose of this study was to assess the therapeutic efficacy and immediate and long-term safety of expanded-tetrafluoroethylene covered stent-grafts for transjugular intrahepatic portosystemic shunts in patients with portal hypertension-related complications. A cohort of 56 patients suffering from severe portal hypertension-related complications underwent implantation of an expanded-polytetrafluoroethylene-covered stent-graft. All patients suffered from severe liver cirrhosis graded Child-Pugh A (n=8; 16%), B (n=13; 21%) or C (n=35; 63%). In 44 patients, the stent-graft was placed during the initial TIPS procedure (de novo TIPS); in the other 12 patients, the stent-graft was placed to repermeabilize the previously placed bare stent (TIPS revision). Follow-up was performed with clinical assessment, duplex ultrasound and, if abnormal or inconclusive, with invasive venography and pressure measurements. Per- en immediate post-procedural complications occurred in four patients (4/56, 7%). None of them was lethal. During follow-up, stent occlusion appeared in one patient and stenosis in two; no recurrence of bleeding was noted in all patients treated for variceal bleeding (n=28), and 24 of the 28 patients (86%) suffering from refractory ascites and/or hepatic hydrothorax were free of regular paracenteses and/or drainage of pleural effusion after shunt creation. The 30-day and global mortality for the total study population (n=56) was, respectively, 7% (n=4) and 28.5% (n=16). In the patient subgroup with variceal bleeding (n=28), 30-day mortality was 3.5% (n=1) and global mortality 14.2% (n=4). In the ascites and/or hydrothorax subgroup (n=28), 8.1% (n=3) mortality at 30 days was found and global mortality was 32.4% (n=12). In 10 patients of the 56 studied patients (18%), isolated hepatic encephalopathy occurred, which was lethal in 4 (Child C) patients (7%). Three of these four patients died within the 1st month after TIPS placement. A very high primary patency rate of TIPS can be obtained long-term after implantation of an e-PTFE-covered stent-graft, leading to a definitive resolution of portal hypertension-related complications. The incidence of TIPS-induced hepatic encephalopathy is acceptable.

Adolescent↗
PubMed2001

Thoracocentesis helps diagnose diaphragmatic defects in peritoneal dialysis patients.

Hydrothorax is an infrequent complication of peritoneal dialysis. Hydrothorax is often secondary to diaphragmatic defects. Available diagnostic tools are usually insensitive and false-negative results are common. We describe a modification to the standard isotopic peritoneogram that proved effective when other tests yielded false-negative results. We conclude that thoracocentesis prior to the standard isotopic peritoneogram facilitates the diagnosis of diaphragmatic defects.

Child, Preschool↗
PubMed1983

Chylothorax in a twin pregnancy of 34 weeks--sonographically diagnosed.

In a twin pregnancy of 34 gestational weeks sonographic follow-up revealed a rapidly spreading hydrothorax in one twin and slight ascites in the other. Analysis of aspirated fluid by intrauterine needle puncture suggested a chylothorax. Pathogenesis of chylothorax is discussed. Differential diagnosis of hydrothorax is presented. Intrauterine puncture is the only method of achieving prenatal diagnosis and therapy.

Adult↗
PubMed1991

Starling forces and lymphatic drainage in pleural liquid and protein exchanges.

Pleural liquid volume and protein concentration (C) were determined in rabbits 60 min after a 2 ml hydrothorax with various albumin concentrations in Ringer, homologous serum or plasma. The absorption rate of the hydrothorax decreased with the increase in colloid osmotic pressure of the pleural liquid (pi), being 0.56 +/- 0.03, 0.32 +/- 0.02 and 0.17 +/- 0.05 ml/h with Ringer, 1.1 and 3 g% albumin, respectively, and nil with 5% albumin, serum or plasma. C increased with Ringer and 1.1% albumin, did not change with 3% albumin, and decreased with 5% albumin, serum or plasma. The protein content in the pleural liquid increased with Ringer, did not change with 1.1% albumin, and decreased with the other hydrothoraces. These findings indicate that with hydrothoraces of this size: (1) the Starling forces plus the solute-coupled liquid absorption [Agostoni and Zocchi (1990) Respir. Physiol. 81: 19-28] provide most of the pleural liquid absorption when pi is less than or equal to physiological; (2) the lymphatic drainage increases with pi, providing most of the liquid outflow when pi is similar to that of plasma. This increase in lymphatic drainage, however, does not compensate for the effects of the changes in Starling forces produced by the increased pi.

Absorption↗
PubMed1993

Active Na+ transport and coupled liquid outflow from hydrothoraces of various size.

The net rate of liquid flow and Na+ flux across the pleura was determined in anesthetised rabbit during hydrothoraces 0.5 to 5 ml in size, without and with amiloride. In the hydrothoraces with amiloride the net liquid flow and Na+ flux reversed when the volume injected approached zero. This indicates that the active Na+ transport and the consequent liquid absorption occur also under physiological conditions. The difference between the data obtained without and with amiloride provides the net solute-coupled liquid outflow and active Na+ efflux. These parameters increased linearly with the hydrothorax size up to 2 ml (0.39 ml/h and 54 muEq/h, respectively), and then levelled off. The linear relationship allowed their extrapolation to physiological conditions: 0.15 ml/h (0.07 ml.h-1.kg-1) and 21 muEq/h (0.1 muEq.h-1.cm-2). The increase in these parameters with the hydrothorax size seems due to the protein dilution caused by the Ringer injection, because it did not occur if Ringer was added with albumin to keep the protein concentration in the pleural liquid similar to that under physiological conditions.

4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfo↗
PubMed2003

Supradiaphragmatic accessory liver: a rare cause of respiratory distress in a neonate.

The authors describe a case of supradiaphragmatic accessory liver seen on prenatal ultrasound examinations, mimicking fetal hydrothorax and causing life-threatening respiratory failure after birth. The accessory liver with a transdiaphragmatic pedicle to the caudate lobe of the normal liver was found at throracotomy after failure to expand the lung by chest drainage. The "hydrothorax" turned out to be multilocular mesothelial cyst collection surrounding the ectopic liver. This is the first report of the development of a thoracic accessory liver followed by obstetric ultrasound examinations.

Choristoma↗