Pleuroamniotic Shunt Research Articles

Pulmonary sequestrations: Prenatal ultrasound diagnosis, treatment, and outcome

Background/Purpose : With the development of antenatal diagnosis of pulmonary sequestrations, the authors decided to define more accurate perinatal operative indications. Methods/Results : Antenatal ultrasound scanning (US) enabled the diagnosis of congenital pulmonary malformation in 10 cases between the twentieth and the thirty-third week of amenorrhea (WA; average, 26 WA). An absolute or relative regression of the thoracic mass size was observed in five patients. The systemic arterial blood supply was identified in four patients by Doppler US. Two fetuses required treatment. One of them suffered from a voluminous sequestration, larger than one hemithorax, with polyhydramnios. Three successive paracentesis of ascites and amniotic fluid allowed the pregnancy to continue until term. The second fetus had a sudden left hydrothorax at 30 WA and was treated by a pleuroamniotic shunt. Five spontaneous partial involutions of the mass during the antenatal period were observed. The 10 patients underwent surgery after birth. There was no mortality. Morbidity occurred in one case of antenatal treatment. Twenty-eight other cases of antenatal diagnosis of pulmonary sequestration have been described in the medical literature. Spontaneous involution of the mass has been reported in eight fetuses and its complete disappearance in two cases. Thirteen fetuses had polyhydramnios. Five of these progressed spontaneously without treatment; only two survived. Two other fetuses were drained or punctured, and one survived. Premature deliveries were undertaken for the six other fetus; there was one perinatal death. Conclusions: Sequestrations with polyhydramnios may be treated in an early prenatal period. Mortality and morbidity rates are still high. At birth, large-sized sequestrations (more than half a hemithorax) must be operated on, even in cases of no respiratory distress. Medium-sized sequestrations must be operated on to remove the mass. Small and asymptomatic sequestrations must be operated on in case of intralobar forms (often cystic), or with a big blood supply. The artery may be responsible for severe complications (hemoptysis, aneurysm).

Pre- and postnatal treatment of a pulmonary sequestration presenting as a unilateral hydrothorax

We report a case of a neonatal sequestration diagnosed antenatally as a progressive left hydrothorax when ultrasound (US) was done at 30 weeks' gestation for polyhydramnios. Postnatal US and computed tomography failed to demonstrate any lesion; magnetic resonance imaging showed a spherical mass in the left chest adjacent to the left lower lobe. At the age of 4 weeks a thoracotomy was performed and the mass was excised. Pulmonary sequestrations may present with a pleural effusion and polyhydramnios as a part of non-immune hydrops fetalis. In our case hydrops was prevented by insertion of a pleuro-amniotic shunt.

Management of non-immune hydrops: 8 years' experience.

During a period of 8 years (1985-92), 100 fetuses were diagnosed to have non-immune hydrops on the basis of ultrasonographic findings and absence of rhesus isoimmunization. Both the mother and the fetus were thoroughly evaluated by a set protocol that included a detailed fetal abnormality scan with echocardiography and fetal blood sampling. A cause for non-immune hydrops could be identified in 81% of the fetuses. Cardiovascular abnormalities (23%) and alpha(1)-thalassemia (22%) were almost equally common etiological factors in the South-East Asian population under investigation. A chromosomal abnormality was detected in 10% of the fetuses with non-immune hydrops. Twenty-six fetuses were found to be suitable for in utero therapy. In utero therapy included one or more of the following: (1) fetal intravascular blood transfusion; (2) direct fetal drug therapy; and (3) fetal pleuroamniotic shunting. Eighteen of the 26 babies (69.2%) were alive and well at 1 month after delivery. It is concluded that in well-selected cases appropriate in utero fetal therapy can lead to significant improvement in fetal salvage.

Resolution of unilateral fetal hydrothorax with associated non‐immune hydrops after intrauterine thoracentesis

Congenital hydrothorax has been successfully managed in utero by two different treatment modalities: thoracentesis and pleuroamniotic shunting. Unfortunately, there is a paucity of data as to which method is better for the management of this problem. This case report supports the use of thoracentesis as the initial procedure for primary fetal hydrothorax. We report a pregnancy complicated by primary fetal hydrothorax with non-immune hydrops that completely resolved after two thoracenteses in the early third trimester. Complete resolution was maintained throughout pregnancy without the need for further antenatal or neonatal intervention, i.e. further thoracentesis, pleuroamniotic shunt placement, intubation, or chest and/or abdominal tube placement. The child was delivered at 42 weeks and is doing well without problems at 1 year of age. We believe that intrauterine thoracentesis should be the initial procedure of choice for the treatment of primary fetal hydrothorax with mediastinal shift, and pleuroamniotic shunting should be reserved for cases that require repetitive thoracenteses.

Respiratory function in infancy following pleuro-amniotic shunting.

Respiratory status was assessed at a median of 12 (range 3-60) months in 17 infants who had undergone pleuro-amniotic shunting to drain fetal pleural effusions. The shunts had been inserted at a median of 29 weeks (range 21-35) gestation; 12 fetuses were hydropic at the time of shunting. In all 17 cases effective chronic drainage of the pleural effusions was achieved. Respiratory status at follow-up was assessed by documentation of respiratory symptoms and measurement of functional residual capacity (FRC) by a helium gas dilution technique. Six infants suffered from recurrent respiratory symptoms, this incidence (37%) did not differ significantly from that found in a control group. The mean FRC of the study population was 28 ml/kg (range 19-34 ml/kg), only 2 infants' FRCs were below the reference range. These results suggest that pleuro-amniotic shunting may, be effective drainage of pleural effusion and hence prevention of chronic antenatal intrathoracic compression, avoid impairment of antenatal lung growth.

Pleural Effusion in Aneuploidy

Three consecutive cases of isolated pleural effusion in aneuploid fetuses are reported. The presence of pleural effusion in trisomy 13 had not been described in the literature previously. Early development of pleural effusion is usually associated with fetal hydrops and carries a poor prognosis. Late development of pleural effusion can be treated in utero. A karyotype should be performed in all cases of pleural effusion prior to in utero pleuroamniotic shunting.

624 In-utero treatment of fetal hydrothorax with pleuro-amniotic shunting at 23 weeks gestation

624 In-utero treatment of fetal hydrothorax with pleuro-amniotic shunting at 23 weeks gestation

Noninvasive management of isolated bilateral fetal hydrothorax

Fetal outcome was studied in eight cases of isolated bilateral fetal hydrothorax. All patients were referred because of polyhydramnios. Spontaneous resolution of pleural effusion was observed twice. No remarkable change in the degree of hydrothorax was demonstrated in the remaining six cases. All eight cases resulted in the birth of a live infant without other abnormalities. Postnatal intubation, which was carried out in all six fetuses with hydrothorax, was always followed by spontaneous respiration. Subsequent thoracocentesis resulted in the collection of 50 to 500 ml of serous fluid. Sustained intubation (7 days) was necessary in only two infants because of developing respiratory distress as a result of prematurity or recurrent pleural fluid accumulation. All eight infants were alive and well at the age of 1 month. Although in the present study noninvasive management of isolated fetal hydrothorax seems to have been justified, a larger multicenter study is needed to compare survival with and without pleuroamniotic shunting.

Pleuro-amniotic shunting for fetal chylothorax

The immediate neonatal course of two babies with gross hydrops fetalis, associated with chylothorax, is compared. One fetus managed by antenatal insertion of a pleuro-amniotic shunt had no evidence of pleural effusion at birth and required less resuscitation.