1/15. Direct intrauterine fetal therapy in a case of bronchopulmonary sequestration associated with non-immune hydrops fetalis.bronchopulmonary sequestration associated with non-immune hydrops fetalis is generally recognized as a uniformly fatal fetal condition without fetal surgical intervention. We describe here the first case of such a condition treated successfully with direct intrauterine fetal therapy using digoxin and frusemide.- - - - - - - - - - ranking = 1keywords = hydrops (Clic here for more details about this article) |
2/15. A new approach to prenatal treatment of extralobar pulmonary sequestration.Fetal hydrothorax and hydrops is a frequent complication of extralobar pulmonary sequestration which is associated with a high perinatal mortality and severe respiratory insufficiency in the newborn. In a 27-week-old fetus with this condition, injection of 1 ml of pure alcohol and pleuro-amniotic shunting achieved resolution of hydrops. The pregnancy progressed to term and a healthy neonate was delivered who did not require postnatal surgery.- - - - - - - - - - ranking = 0.4keywords = hydrops (Clic here for more details about this article) |
3/15. Physiopathology hypotheses and treatment of pulmonary sequestration.Illustrated by this case report, partial obstruction of the venous collector of extrapulmonary sequestration explains pleural effusion and fetal hydrops. Therapy requires fetal thoracentesis and neonatal arterial occlusion of the pedicle artery allowing uneventful follow-up.- - - - - - - - - - ranking = 0.2keywords = hydrops (Clic here for more details about this article) |
4/15. Fetal thoracoamniotic shunting as the only treatment for pulmonary sequestration with hydrops: favorable long-term outcome without postnatal surgery.Although it is established that bronchopulmonary sequestration complicated by fetal hydrops carries a high risk of perinatal mortality, prenatal management remains controversial. Therapeutic options include immediate delivery, medical therapy based on an inotropic regimen, alcohol ablation of the vascular pedicle, open fetal surgery, or thoracoamniotic shunting of fetal pleural effusions. We report a case of pulmonary sequestration with hydrothorax and fetal hydrops which was successfully treated by a single fetal thoracoamniotic catheter drainage. Following emergency catheter placement, hydrothorax and hydrops decreased dramatically. The sequestration gradually disappeared postnatally, and long-term outcome remained normal without any postnatal therapy. Based on this observation, the natural history of pulmonary sequestration and prenatal management options are reviewed.- - - - - - - - - - ranking = 1.4keywords = hydrops (Clic here for more details about this article) |
5/15. Pulmonary sequestration presenting as fetal hydrops.Pulmonary sequestration is a rare developmental anomaly of the lung characterized by nonfunctional pulmonary tissue without communication with the tracheobronchial tree and receiving an aberrant systemic arterial blood supply. Few cases of non-immune hydrops fetalis associated with this entity have been reported. A 2 day old male baby born by cesarean section at 31 weeks gestational age due to fetal hydrops is presented. autopsy revealed a hydropic baby with extralobar pulmonary sequestration and bilateral pulmonary hypoplasia. The clinicopathologic presentation of this unusual pulmonary developmental anomaly is discussed.- - - - - - - - - - ranking = 1.2keywords = hydrops (Clic here for more details about this article) |
6/15. Venous arterialization in extralobar pulmonary sequestration associated with fetal hydrops.BACKGROUND/PURPOSE: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by massive pleural effusion (PE) leading to fetal hydrops. The underlying mechanisms as well as the origin of the fluid are not well understood. This study was performed to find a histological hallmark of an EPS with massive PE. We hypothesized that venous obstruction has a role to play in the mechanisms of fluid production by EPS. methods: We recently experienced 3 cases of fetal EPS complicated by fetal hydrothorax requiring thoracentesis and eventually thoracoamniotic shunt placement. Total protein content and cell count were measured in the aspirates, which were compared with chylothorax cases (n = 5) requiring fetal shunt placement. After birth, all 3 infants required mass resection for the control of PE. The venous wall thickness was measured on pathology slides stained with Elastica van Gieson staining. Thickness of the media and adventitia was measured in approximately 40 veins per case. They were corrected by external diameter and expressed as percentage of medial thickness and percentage of adventitial thickness. An EPS not associated with PE but with congenital diaphragmatic hernia served as a control. RESULTS: Total protein and the cell count of the EPS related PE were 0.6 /- 0.3 mg/dL and 28 /- 14/microL (mean /- SD), which were significantly lower than those of PE in chylothorax (2.2 /- 0.2 mg/dL and 1900 /- 1100/microL). Percentages of adventitial thickness of EPS with PE were 9.8% /- 3.8%, 10.4% /- 3.6%, and 8.3% /- 3.7%, which were significantly increased compared with the control of 3.1% /- 1.3% (P < .01). Percentages of medial thickness of EPS with PE were 7.0% /- 1.9%, 7.3% /- 1.4%, and 6.6% /- 2.3%, which were significantly increased compared with the control of 2.3% /- 0.7% (P < .01). CONCLUSIONS: We conclude that PE associated with EPS is the transudate rather than the lymph. The thickened media and the adventitia found in EPS with PE support the hypothesis that partial obstruction of the venous system leads to an increased transudate production, which ultimately leads to fetal hydrops.- - - - - - - - - - ranking = 1.2keywords = hydrops (Clic here for more details about this article) |
7/15. Pulmonary sequestration: diagnosis with color Doppler sonography and a new theory of associated hydrothorax.The diagnosis of pulmonary sequestration has traditionally relied on angiographic demonstration of a systemic artery to the sequestered lung tissue. Rarely, extralobar sequestration can be associated with tension hydrothorax, which in the past has invariably led to fetal hydrops and death. The authors report the cases of three infants who had initially undergone color and spectral Doppler analysis; in two of them, extralobar sequestrations were associated with congenital hydrothorax. All three patients underwent surgical resection and histopathologic evaluation of their sequestrations. On the basis of the findings, the authors believe that torsion of the sequestration occludes the efferent venous and lymphatic channels, initiating the accumulation of pleural fluid and subsequent hydrops through systemic venous obstruction. color Doppler made possible the identification of minute feeding vessels, obviating further diagnostic studies.- - - - - - - - - - ranking = 0.4keywords = hydrops (Clic here for more details about this article) |
8/15. Antenatal diagnosis and palliative treatment of nonimmune hydrops fetalis secondary to pulmonary extralobar sequestration.The causes of nonimmune hydrops fetalis are multiple. Despite the fact that many of the affected fetuses are otherwise free of fatal abnormalities, many die in the neonatal period from pulmonary hypoplasia. The overall perinatal mortality is as high as 80%. Extralobar pulmonary sequestration is an abnormal and nonaerated mass of extra pulmonary-located lung tissue supplied by a systemic artery, and is a rare cause of nonimmune hydrops. In the only two published reports, the hydropic fetuses died at birth. Both the first antenatal diagnosis of this disorder and the first attempt at palliative in utero therapy are reported herein. Though the fetus ultimately delivered prematurely and succumbed, treatment was associated with both subjective improvements in fetal well-being and objective reversal of the hydrops.- - - - - - - - - - ranking = 1.4keywords = hydrops (Clic here for more details about this article) |
9/15. Disappearing fetal lung lesions.Cystic adenomatoid malformations and sequestrations of the lung are uncommon but potentially devastating problems of the fetus and neonate. We have followed over 50 cases of fetal lung masses from the time of prenatal diagnosis. Serial prenatal ultrasonography demonstrated that 9 large pulmonary lesions dramatically decreased in size or disappeared completely. We conclude that the natural history of prenatally diagnosed fetal lung masses is highly variable. A huge mass associated with fetal hydrops has a dismal outcome. If hydrops is not present, then the initial impression concerning prognosis may not accurately predict outcome, because there may be marked improvement during fetal life.- - - - - - - - - - ranking = 0.4keywords = hydrops (Clic here for more details about this article) |
10/15. Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration.Extralobar pulmonary sequestration was found in a newborn premature infant that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.- - - - - - - - - - ranking = 1.2keywords = hydrops (Clic here for more details about this article) |
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