uog journal club: bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic...

UOG Journal Club: October 2014

Bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic shunting vs intrafetal vascular laser ablation

M.R. Mallmann, A. Geipel, M. Bludau, K. Matil, I. Gottschalk, M. Hoopmann, A.Müller, H. Bachour, A. Heydweiller, U. Gembruch, C. Berg

Volume 44, Issue 4, Date: October 2014, pages 441 - 446

Journal Club slides prepared by Dr Leona Poon(UOG Editor for Trainees)

• Bronchopulmonary sequestration (BPS) and congenital pulmonary airway malformation (CPAM) are rare lung malformations consisting of a mass of bronchopulmonary tissue that is separate from the tracheobronchial tree

Achiron R et al. Ultrasound Obstet Gynecol 2004;24:107-14.Pryce DM et al. J Pathol Bacteriol 1946;58:457-67.Sade RM et al. Ann Thorac Surg 1974;18:644-58.Cavoretto P et al. Ultrasound Obstet Gynecol 2008;32:769-83

Figure 1. Grayscale (a) and color Doppler (b) images ofBPS at 26 weeks’ gestation, showing a feeding vessel (arrows)

arising from the descending aorta.

• The key sonographic feature for distinguishing BPS from CPAM is demonstration of separate systemic artery, typically originating from the descending aorta

• A considerable number of echogenic lung lesions show histological features of both BPS and CPAM.

• BPS usually regresses in intrauterine period and only few cases are associated rapid growth and/or pleural effusion and warrant intrauterine treatment.

Achiron R et al. Ultrasound Obstet Gynecol 2004;24:107-14.Figure 2. BPS at 28 weeks’ gestation, associated with pleural

effusion, polyhydramnios and mediastinal shift

•Intrauterine treatments include pleuroamniotic shunting, alcohol injection, radiofrequency ablation and interstitial laser coagulation


Mallmann et al., UOG 2014

To assess the incidence of complications among a relatively large cohort of fetuses with BPS and the

success of two different intrauterine treatment modalities

Objective

Patients and Methods

• Retrospective review of all cases with a prenatal diagnosis of BPS detected in a 10-year period (2002-2011) in 2 tertiary referral centers (Universities of Bonn and Cologne, Germany)

• Laterality, size, presence of mediastinal shift, relation to the diaphragm, origin of feeding vessel, associated malformations, presence of pleural effusion/hydrops, intrauterine evolution and neonatal outcome were recorded in all cases.

• Intervention was performed by 3 dedicated specialists in fetal medicine (C.B., A.G. and U.G.). The path of access and operative technique were chosen at the discretion of the fetal medicine specialist performing the intervention.




• Up to May 2010 severe pleural effusions were treated with pleuroamniotic shunting.

• After May 2010 ultrasound-guided laser coagulation of the feeding artery using



Figure 3. Ultrasound-guided laser coagulation of the feeding artery using an Nd:YAG 700-μm laser fiber moved

through an 18-G needle (arrows)

an Nd:YAG laser through an 18-G needle was performed.

A 700-μm laser fiber was moved forward until the tip of the laser fiber 2-3 mm adjacent to the feeding vessel. The feeding vessel was coagulated using an output of 50 Watts for 5-10s. If color Doppler demonstrated residual flow, the tip of the laser fiber was repositioned and coagulation was repeated until complete cessation of blood flow.


• Due to the extended study period and the retrospective study design, the mean duration of procedures and postoperative complications such as separation of membranes, were not evaluated.

• In cases of stable disease or regression of the lesion, delivery and postnatal management were carried out at the discretion of the referring institution.

• Statistical analysis was performed using the Mann-Whitney U-test. All values are given as median (interquartile range) unless indicated otherwise.



Results• 41 fetuses with BPS were included in the study. Four showed additional

abnormalities (one each with congenital diaphragmatic hernia, tetralogy of Fallot, hydrocephalus and supraventricular tachycardia)

• In 29 cases treated conservatively. Complete regression, partial regression and no change were diagnosed in 8 (27.6), 11 (37.9%), and 10 (34.5%) cases, respectively

• Intrauterine intervention was performed in all 12 (29.3%) fetuses with severe pleural effusion and mediastinal shift, all with left-sided extralobar BPS

• 7 fetuses were treated with pleuroamniotic shunting at 29.3 (25.3-29.5) weeks



Results• 5 fetuses were treated with laser ablation of feeding vessel at 30.4 (24.3-31.5)

weeks:• 2 cases required a 2nd intervention within 72 hours because of recurrent

flow in the feeding vessel. • Complete and partial regression were diagnosed in 4 (80.0%) and 1

(20.0%) case(s), respectively.

• Following intrauterine shunt placement complete regression of the lesion was significant less frequent (0/7 with shunt placement vs 4/5 with intrafetal laser treatment) and GA at birth was significantly lower, compared to treatment with intrafetal laser.

• Complete regression of the lesion was also significantly more frequent in the laser group compared to cases without intervention.



Results

Characteristics

No intrauterine intervention

(n=29)

Type of intervention

Pleuroamniotic shunt (n=7)

Intrafetal laser(n=5)

GA at diagnosis (w) 23.3 (20.4-27.0)* 29.0 (25.2-29.3)* 24.1 (34.4-31.3)

Type of BPS Intralobar 4 (13.8) 0 (0) 0 (0)

Extralobar 25 (86.2) 7 (100) 5 (100)

Side of BPS Unilateral 23 (79.3) 7 (100) 5 (100)

Bilateral 1 (3.4) 0 (0) 0 (0)

Pleural effusion 0 (0) 7 (100) 5 (100)

Hydrops fetalis 0 (0) 4 (57.1) 1 (20)

Mediastinal shift 13 (44.8) 7 (100) 5 (100)

Polyhydramnios 1 (3.4) 4 (57.1) 5 (100)

Fetal loss 0 (0) 1 (14.3) 0 (0)

Complete regression of BPS 8 (27.6)♯ 0 (0)§ 4 (80)♯§

GA at birth (w) 38.3 (34.0-39.6)* 37.2 (30.3-37.4)*§ 39.1 (38.0-40.0)§

Table 1. Details of 41 fetuses with BPS diagnosed over a period of 10 years. P<0.05: *no intervention vs shunt; ♯no intervention vs laser; §shunt vs laser.



Results



Fetuses with BPS (n=44)

No pleural effusion (n=29) Pleural effusion (n=12)

No intervention (n=29)

Lethal condition (n=3)

Pleuroamniotic shunting (n=7)

IUFD (n=1)

Laser coagulation (n=5)

Live birth (n=29)

Live birth (n=6)

Live birth (n=5)

Sequestrectomy (n=16)






Figure 4. Flow chart showing management and pregnancy outcome of 41 pregnancies complicated by BPS

• This study demonstrates that 65.5% of non-hydropic fetuses there was partial or complete regressions of the lesion during the course of pregnancy. The small subset of fetuses with hydrops is associated with high intrauterine and neonatal mortality.

• A substantial number of echogenic lung lesions are hybrid lesions with concomitance of BPS and CPAM and in some cases differentiation between these entities might not be possible at prenatal ultrasound examination.

• A subgroup of BPS can be distinguished with high reliability: extralobar sequestration with an atypical systemic feeding vessel and associated pleural effusion, features are not associated with microcystic CPAM. This distinction is of utmost importance when prenatal intervention is considered.

Discussion



• In the absence of severe pleural effusion and mediastinal shift, BPS has a high likelihood of spontaneous regression and therefore has a favorable prognosis, which justifies expectant management

• In cases of BPS with hydrops, which are associated with massive pleural effusion, the target of intrauterine therapy is either the abnormal systemic feeding vessel or the pleural effusion

• Established literature for pleuroamniotic shunting, which often results in resolution of hydrops, recognises the need for repeat shunt insertions, due to shunt displacement and recurrent amnioreductions

• Ultrasound-guided intrafetal laser ablation of the abnormal systemic blood supply of BPS might be more effective than shunting as it targets the echogenic lung lesion rather than its symptoms

Discussion



• Retrospective design, differences in morbidity and the fact that postnatal imaging studies and treatment were made at the discretion of the referring institutions

• There was a higher proportion of hydrops in the shunt group than the laser group; this difference in morbidity between the treatment groups adds a further bias to the results and consequently the conclusion about the preferable form of treatment must be considered carefully

Limitations



• In the absence of pleural effusion, the likelihood of spontaneous BPS regression is high and the prognosis is favorable

• In cases with massive pleural effusion, ‘vascular’ laser ablation of the feeding vessel appears to be more effective than pleuroamniotic shunting, with fewer complications

• Laser treatment might also reduce the need for postnatal surgery

• These results should be confirmed by future studies with larger samples and a prospective design

Conclusions



Discussion points

• Should ‘vascular’ laser ablation of the feeding vessel be the first line management of BPS with massive pleural effusion, with or without hydrops?

• Over half of BPS cases without pleural effusion required postnatal surgery, whilst 20% of BPS cases treated with intrauterine laser therapy required postnatal surgery. Is there a role of laser ablation of the feeding vessel in uncomplicated BPS cases?

• If yes, is it for all cases or for cases that remain unchanged during the course of pregnancy?

• Can we predict cases that are unlikely to resolve?



uog journal club: bronchopulmonary sequestration with massive pleural effusion: pleuroamniotic...

Health & Medicine