prenatal interventions for fetal lung lesions

PRENATAL DIAGNOSISPrenat Diagn 2011; 31: 628–636.Published online 25 May 2011 in Wiley Online Library(wileyonlinelibrary.com) DOI: 10.1002/pd.2778

REVIEW

Prenatal interventions for fetal lung lesions

Ruben S. Witlox1*, Enrico Lopriore1 and Dick Oepkes2

1Department of Paediatrics, Division of Neonatology, Leiden University Medical Centre, Leiden, The Netherlands2Department of Obstetrics, Leiden University Medical Centre, Leiden, The Netherlands

The widespread availability of high resolution ultrasound equipment and almost universal routine anatomyscanning in all pregnant women in the developed world has lead to increased detection of abnormalities in thefetal thorax. Already in the 1980s, large pleural effusions and significant macrocystic lesions in the fetus wereeasily detected on ultrasound. However, smaller lung tumours were often missed. Nowadays, fetal medicinecentres receive many referrals for evaluation of fetal lung lesions, of which the most common are congenitalcystic adenomatoid malformation and bronchopulmonary sequestration. Almost invariably, both the parentsand the referring physicians experience anxiety after detection of large lung masses in the fetus. However,the vast majority of the currently detected fetal lung lesions have an excellent prognosis without the need forprenatal intervention. In the small group of fetuses in which the prognosis is poor, almost exclusively thosewith concomitant fetal hydrops and cardiac failure, several options for fetal therapy exist, often with a morethan 50% survival rate. Indications, techniques, complications and outcomes of these interventions will bedescribed in this review. Copyright 2011 John Wiley & Sons, Ltd.

KEY WORDS: fetal lung lesions; fetal therapy; CCAM; pulmonary sequestration; hydrops fetalis; thoraco-amnioticshunt

INTRODUCTION

The increased use of obstetric ultrasound and advancesin ultrasound technology have allowed an increase in theprenatal identification of fetal lung lesions.

Most prenatally detected lung lesions are congeni-tal cystic adenomatoid malformations (CCAMs), bron-chopulmonary sequestrations (BPS) or so called ‘hybrid’lesions, containing features of both (Cass et al., 1997).

Fetal lung lesions are rare and occur in 1 in 10 000 to 1in 35 000 pregnancies (Laberge et al., 2001; Duncombeet al., 2002).

Most lesions have a favourable outcome withoutprenatal intervention, despite often impressive appear-ance at mid-gestation. Many lesions regress duringpregnancy, some disappear completely. Conservativemanagement with watchful waiting is commonly mostappropriate. In some cases however, secondary phys-iologic derangements occur because of mass effector haemodynamic changes. This can lead to progres-sive cardiac failure, hydrops and intrauterine demise.Prenatal intervention may be warranted to improveoutcome.

This review focuses on possible prenatal interventionsfor CCAM and BPS, indications, techniques and results.For the interesting debate on whether or not postnatalresection of asymptomatic lung tumours in the neonateor infant should be performed, we refer to a recentreview by Bush (2009). Diagnosis and treatment of

*Correspondence to: Ruben S. Witlox, Department of Paediatrics,Division of Neonatology, J6-S Leiden University Medical Centre,PO Box 9600, 2300 RC, Leiden, The Netherlands.E-mail: [email protected]

isolated fetal pleural effusion (PE) also falls beyond thescope of this review.

PRENATAL ASSESSMENT AND SURVEILLANCE

Most fetal lung lesions are nowadays detected onroutine ultrasound screening at 18–20 weeks’ gestation.The most common appearances are a solid-appearingechogenic tumour or a tumour containing anechogenicmacrocysts surrounded by echogenic soft tissue. PE mayor may not be present. Occasionally, such a tumour islocated below the diaphragm. Fetal therapy has neverbeen described in subdiaphragmatic lesions. Thereforethey will not be addressed further in this review.

Differential diagnosis of fetal lung tumours includesCCAM, BPS, congenital high airway obstruction, bron-chogenic cysts, congenital lobar emphysema, congenitaldiaphragmatic hernia and mediastinal tumours (Gold-stein, 2006; Bush et al., 2008). Once the lesion isdetected, the location, volume, size and appearance (i.e.macrocystic or microcystic) should be evaluated. In thepast, ultrasound appearance was often described similarto the pathology classification by Stocker, with Stockertype I lesions showing only large cysts (Figure 1), typeIII showing only small cysts (<0.5 mm, Figure 2) andtype II a mix of both (Stocker et al., 1977). Morerecently, the group from Children’s Hospital of Philadel-phia proposed a more practical classification using onlymicrocystic (solid appearance on fetal ultrasound) andmacrocystic types (Adzick et al., 2003). Colour Doppler(2D and 3D) should be used to look for systemic arterialblood supply to the lesion. CCAMs derive their blood

Copyright 2011 John Wiley & Sons, Ltd. Received: 27 February 2011Revised: 20 April 2011

Accepted: 20 April 2011Published online: 25 May 2011

PRENATAL INTERVENTIONS FOR FETAL LUNG LESIONS 629

supply from pulmonary vessels. BPS can be diagnosedwhen a feeding systemic artery originating directly fromthe descending aorta can be identified (Sepulveda, 2009),although this occasionally proves difficult before birth.

BPS occurs in two, anatomically distinct, subtypes(Stocker, 1986). Intralobar sequestration (ILS) is locatedwithin the lung and covered by the visceral pleuraof the lung. Extralobar sequestration (ELS) is locatedoutside the normal lung and covered by its own visceralpleura. ELS can also be located below the diaphragm.Distinction between ILS and intrathoracic ELS is verydifficult prenatally.

Fetal magnetic resonance imaging (MRI) providesmore detailed imaging of the lesion and might thereforeaid in a more definite diagnosis (Hubbard et al., 1999;Levine et al., 2003; Kunisaki et al., 2007; Liu et al.,2010). Specific indications for additional MRI have notbeen described in the literature.

The amount of amniotic fluid (amniotic fluid indexor deepest vertical pocket) should be measured. Masseffect of the lesion can lead to esophageal compressioncausing impaired fetal swallowing (Adzick et al., 1985;Thorpe-Beeston and Nicolaides, 1994). This can leadto polyhydramnios. In case of polyhydramnios, cervicallength should be measured and taken into considerationwhen assessing the necessity of intervention.

Figure 1—Transverse view of the fetal chest showing a macrocysticCCAM

Figure 2—Transverse view of the fetal chest showing a microcysticCCAM

An essential part of the evaluation is a full and detailedanatomical survey of all fetal organs and structures,including echocardiography. Combined occurrence with,e.g. diaphragmatic hernia is not uncommon. The occur-rence of other anomalies in association with CCAM hasbeen reported in 10–20% of cases (Thorpe-Beeston andNicolaides, 1994; Stocker and Dehner, 2002). In BPSassociated anomalies have been reported to occur in upto 10% of cases of ILS and up to 50% of cases ofELS (Wilson et al., 2006) Cardiac evaluation may behampered by displacement of the heart. Aneuploidy hasbeen reported occasionally in fetuses with lung lesions(Laberge et al., 2001; Calvert et al., 2006), but it isnot regularly associated with isolated fetal lung lesion(Pumberger et al., 2003; Wilson et al., 2006). We dosuggest offering karyotyping, or in the near future pos-sibly array Comparative Genomic Hybridization (arrayCGH), to all women carrying a fetus with anomalies onultrasound. In fetuses with multiple congenital anoma-lies or chromosomal aberrations, the prognosis is oftenconsiderably worse. Fetal interventions are generally notoffered in this group. In the remainder of this article, wewill focus on the management of the fetus with isolatedlung lesions.

NATURAL HISTORY

The natural history of fetal lung lesions is variable.Spontaneous regression is not uncommon. CCAM/BPSgrowth generally peaks at 26–28 weeks’ gestation. Inthe weeks thereafter spontaneous regression is regularlydescribed (MacGillivray et al., 1993; Miller et al., 1996;Adzick et al., 1998). As a consequence the lesions canbe hard to find on ultrasound in the third trimester.Postnatal computed tomography or MRI can identifyto what extent the lesion is still present (Winters andEffmann, 2001; Cavoretto et al., 2008). The precisemechanism leading to spontaneous regression is notclear and may be because of outgrowing of the vascularsupply of the CCAM or to spontaneous resolution ofthe underlying bronchial obstruction (Miller et al., 1996;Adzick, 2009).

Reported rates of spontaneous regression of CCAMvary from 15 to 65% (Miller et al., 1996; Dommer-gues et al., 1997; Laberge et al., 2001; Pumberger et al.,2003; Ierullo et al., 2005; Cavoretto et al., 2008; Adz-ick, 2009) The largest published series describes sono-graphic evidence of regression in 76 of 154 CCAMs(49%)(Cavoretto et al., 2008) Spontaneous regression ofBPS is also regularly described, in up to 68% of cases(Adzick, 2009).

On the other hand, secondary physiological derange-ments can occur, mostly because of the mass effect ofthe lesion in the fetal thorax. Oesophageal compres-sion can interfere with fetal swallowing causing poly-hydramnios (Adzick et al., 1985; Thorpe-Beeston andNicolaides, 1994). The mass effect of the lesion cancause mediastinal shift and may, although surprisinglyrare, restrict lung growth causing pulmonary hypopla-sia (Sauvat et al., 2003; Davenport et al., 2004). The

Copyright 2011 John Wiley & Sons, Ltd. Prenat Diagn 2011; 31: 628–636.DOI: 10.1002/pd

630 R. S. WITLOX et al.

mass effect can also cause obstruction of the vena cava,impairment of venous return and cardiac compressionultimately leading to fetal hydrops.

Serial ultrasonographic assessment is important tofollow the, often unpredictable, growth pattern of fetallung lesions and to identify the early occurrence of fetalhydrops.

To aid in the prediction of occurrence of fetal hydrops,a prognostic tool using sonographic measurement ofthe CCAM volume was developed. The CCAM vol-ume ratio (CVR) is obtained by dividing the CCAMvolume (length × width × height × 0.52) by head cir-cumference. A CVR greater than 1.6 is predictive ofincreased (75%) risk of the development of fetal hydrops(Crombleholme et al., 2002).

INDICATIONS FOR PRENATAL INTERVENTION

An important conclusion from the existing literatureis that the prognosis for a fetus with a lung lesionis generally favourable. A clear distinction needs tobe made between lung lesions with and without fetalhydrops.

Cavoretto et al. (2008) reviewed the literature on theoutcome of fetal lung lesions and found a survival ofmore than 95% in cases of CCAM without hydropsand cases of BPS without PE. When fetal hydrops doesdevelop, however, mortality rates increase dramatically.Cavoretto et al. (2008) reported death before or afterbirth in 95% of cases with CCAM and hydrops managedexpectantly.

Knox et al. (2006) systematically reviewed the liter-ature trying to determine the effect of in-utero drainageon perinatal survival in fetuses with congenital cysticlung lesions No randomized studies were found. Theavailable studies showed that treatment had a negativeassociation with outcome overall [odds ratio (OR) forsurvival 0.56, 95% confidence interval (CI) 0.32–0.97].However in cases accompanied by fetal hydrops, a sig-nificantly higher chance of survival in treated cases wasreported (OR 19.28, 95% CI 3.67–101.27).

Several case reports also suggest that in BPS withPE, outcome is very poor without prenatal treatment(Reece et al., 1987; Dolkart et al., 1992; Brus et al.,1993; Yildiz et al., 2005; Yildirim et al., 2008).

Fetuses that develop hydrops are therefore candidatesfor prenatal intervention.

Several centres advocate that hydropic fetuses at orafter 32 weeks’ gestation can best be delivered, withor without an ex-utero intrapartum treatment procedure,with reasonable chances of survival. This could implythat fetal interventions, with their inherent risks of rup-tured membranes, preterm birth and other complicationsmay be restricted to hydropic fetuses below 32 weeks’gestation. However, a severely hydropic neonate witha large lung tumour born at 33 or 34 weeks can bevery hard to resuscitate, and urgent lobectomy in sucha baby is a high-risk procedure. In addition, althoughextracorporeal membrane oxygenation certainly can bea life-saving technique, there are many drawbacks and

complications. Fetal therapy, with the outlook for thefetus to remain in utero on placental support whilerecovering from hydrops in our view is preferable overpreterm birth of a very sick child. Therefore, we andothers believe that fetal interventions in potentially treat-able fetal hydrops, due to lung lesions or in fact anyother treatable condition, should be seriously consid-ered up to 37 weeks’ gestation. The group from the fetalmedicine unit in Toronto have always promoted shuntingfor hydrothorax up to 37 weeks’ gestation (Yinon et al.,2008). Successful intervention and postponing of deliv-ery should lead to reduction of hydrops and increasingmaturation of the lungs and other organs, making post-natal surgery much less risky. In addition, the possiblecomplications of minimally invasive interventions suchas rupture of membranes lose much of their importanceafter 32 weeks’ gestation.

PRENATAL INTERVENTIONS

Prenatal interventions for fetal lung lesions aim toalleviate the mass effect by decompression or resectionof the lesion. A number of surgical and non-surgicaloptions have been reported.

In macrocystic lesions decompression can beattempted by single needle thoracocentesis or permanentdrainage via ultrasound guided thoraco-amniotic shuntplacement (Knox et al., 2006).

In microcystic lesions, cysts are too small fordrainage. In these cases open fetal surgery has been per-formed. When a systemic feeding vessel is found, per-cutaneous laser coagulation or injection of a sclerosingagent can be successful. Recently, maternal betametha-sone treatment, often used to promote lung maturity, wassuggested to have beneficial effects on large CCAM’s.We will evaluate the literature on these treatment modal-ities in more detail.

Prenatal steroid therapy

Fetuses with microcystic CCAM are not amenable forthoracocentesis and cyst aspiration or thoraco-amnioticshunting. Open fetal surgery with lobectomy seemedto be the only available option. Resolution of a largeCCAM after steroid therapy given for lung maturationwas first described by Higby et al. (1998). Tsao et al.(2003) reported on three fetuses with large, solid fetallung lesions showing unexpected resolution of hydropsshortly after injection of the mother with betamethasoneto promote lung maturity for expected preterm birth.They postulated that steroids could have a beneficialeffect on large CCAMs. Since then, several othershave indeed observed the same effect, after giving thestandard dose of two times 12 mg betamethasone, 24 hapart.

Peranteau et al. (2007) reported on a series of 11patients, with microcystic CCAM and fetal hydropsand/or CVR >1.4 treated with maternal betamethasonetherapy. Resolution of hydrops was seen in four of five



patients with fetal hydrops. The non-responding fetuswas treated by fetal surgery. All patients survived.

Later series showed a more variable response onmaternal betamethasone treatment. Morris et al. (2009)treated 15 high risk fetuses (macro- and microcysticCCAM with fetal hydrops and/or CVR >1.8). Theyfound resolution of hydrops in only 54% of cases and asurvival rate of 53%.

Curran et al. (2010) treated 13 fetuses with predom-inantly microcystic CCAM and hydrops and/or CVR>1.6. They found resolution of hydrops in 78% of whatthey described as high risk cases, with a survival rateof 85%. Their group, from University of California, SanFrancisco, who also published the first three cases, plansto find more evidence for the quite promising effectsof steroids by a randomized controlled trial (clinical-trials.gov NCT00670956). In the mean time, currentevidence suggests that in large CCAMs with hydrops,a course of steroids appears to be a reasonable first linetherapy, also because of the virtual absence of maternalside-effects. A slight concern remains because of a casereport from Hong Kong, of a fetus with a large CCAMand hydrops, that resolved after steroids followed bysudden and unexplained intrauterine demise of the fetusat 34 weeks’ gestation (Leung et al., 2005). Whethersteroids should also be used in CCAMs without hydropsis more questionable, as the prognosis without interven-tion is generally good and spontaneous regression oftenoccurs.

Thoracocentesis

Thoracocentesis with aspiration of fluid can be usedto reduce the size of dominant cysts in macrocysticCCAM or to remove PEs occurring from BPS or hybridlesions. This mode of treatment can therefore be used toallow for lung expansion and/or resolution of hydrops(Nugent et al., 1989). After single thoracocentesis thefluid usually reaccumulates in the macrocyst or pleuralcavity over a period of days to weeks. Serial aspirationshave been described resulting in resolution of hydropsand survival of the fetus (Brown et al., 1995). Singlethoracocentesis can be used to evaluate the amount ofshrinkage of CCAM or resolution of PE, followed bythoracoamniotic shunting if hydrops reappears.

In total 13 hydropic fetuses with macrocystic CCAMonly treated by single or multiple thoracocenteses aredescribed in the literature(Neilson et al., 1991; Brownet al., 1995; Sugiyama et al., 1999; Bunduki et al.,2000; Crombleholme et al., 2002; Gornall et al., 2003;Pumberger et al., 2003; Tran et al., 2008; Chao et al.,2010)

A minimum of one and an maximum of six serialaspirations per case were reported. Median gestationalage of treatment was 27 weeks as mentioned. All fetuseswere live born. Neonatal survival at discharge was 9/13(69%).

Thoraco-amniotic shunting

Drainage of fetal fluid-filled spaces using a draintowards the amniotic cavity was first described in the

1980s for fetal bladder drainage. Seeds and Bowes(1986) described this procedure in the treatment of fetalhydrothorax. Many fetal therapy centres worldwide stilluse this technique, insertion through a 2–3 mm diame-ter needle of a double pigtail catheter under ultrasoundguidance, for these two indications. In a recent reviewarticle, techniques and complications were described indetail (Yinon et al., 2008). Figure 3 shows a neonatewith a thoraco-amniotic shunt still in place. Insertion ofsuch a catheter in a large cyst of a CCAM has beensuccessful too, first reported by Nicolaides et al. (1987).

Thereafter several case reports and small seriesdescribe the results of this technique in hydropic andnon-hydropic fetuses with CCAM or BPS.

Thoraco-amniotic shunting with the aim of decom-pressing a large cyst in macrocystic CCAM has beendescribed in 68 cases in the literature. This includes 44hydropic fetuses. In addition 24 non-hydropic fetuseswith a large cyst causing major mediastinal shift weredescribed. Outcome is summarized in Table 1.

Of the hydropic fetuses 89% (39/44) were livebornand nine infants died in the neonatal period. Overallperinatal survival in this group was thus 68% (30/44).

Of the non-hydropic fetuses (n = 24) all were live-born. Three infants died in the neonatal period due topulmonary hypoplasia. Overall survival was therefore87.5%.

Thoraco-amniotic shunting can also be used to drainPE in BPS with or without prior thoracocentesis. Adzicket al. (1998) describe two hydropic fetuses with BPStreated by thoraco-amniotic shunt placement. In bothhydrops resolved after treatment and both survivedafter birth, all requiring ventilatory support and surgicalexcision of the lesion. Lopoo et al. (1999) describetwo hydropic fetuses with BPS treated by thoraco-amniotic shunt placement. In both hydrops resolved andboth did well after birth. Hayashi et al. (2006) describethree hydropic fetuses with BPS treated by thoraco-amniotic shunt placement. All received thoracocentesisfirst, but the hydrothorax reaccumulated necessitatingfurther treatment. In all three cases hydrops resolved andall survived after birth, requiring ventilatory support andsurgical excision of the lesion.

Figure 3—Neonate with thoraco-amniotic shunt still in place



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Tho

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Bee

ston

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Nic

olai

des

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Lab

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Gor

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Asa

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uano

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Isna

rdet

al.,

2007

;V

uet

al.,

2007

;R

uano

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.,20

08).



In addition seven single cases of hydropic fetuseswith BPS treated by thoracoamniotic shunt placementhave been described (Weiner et al., 1986; Slotnick et al.,1990; Hernanz-Schulman et al., 1991; Favre et al., 1994;Salomon et al., 2003; Picone et al., 2004; Odaka et al.,2006) In one case PE reaccumulated probably because ofshunt occlusion (Weiner et al., 1986) In all other caseshydrops resolved and fetuses survived after birth.

Laser and sclerosing agents in thetreatment of CCAM and BPS

Attempts at percutaneous ablation of a microcysticCCAM in a hydropic fetus using Nd : YAG laser havebeen described four times in the literature (Fortunatoet al., 1997; Bruner et al., 2000; Davenport et al., 2004;Ong et al., 2006) In all cases a 600 µm laser fibrewas passed through the lumen of an 18G-needle afterwhich the tumour itself was photocoagulated using anNd : YAG laser. In one case (Ong et al., 2006) hydropsresolved and the fetus survived needing postnatal respi-ratory support and surgical resection the lesion. In onecase (Bruner et al., 2000) the fetus died prenatally. Inone case (Davenport et al., 2004) the fetus died 4 daysafter birth. In the last case resolution of hydrops wasdescribed but no further outcome was reported (Fortu-nato et al., 1997)

The group of Quintero reported on three cases ofCCAM complicated by fetal hydrops and treated withpercutaneous insertion of a sclerosing agent directly intothe CCAM (Bermudez et al., 2008) In all cases hydropsresolved and all fetuses were born alive. One neonatedied after 10 days because of nosocomial sepsis.

Interruption of flow in the systemic feeding vesselof a BPS has been described as a treatment option inhydropic fetuses with BPS. Successful ultrasound guidedlaser coagulation of the feeding artery of BPS usingNd : YAG laser through a 18G-needle was described byour group in 2007 and 2009 (Oepkes et al., 2007; Witloxet al., 2009). Figures 4–7 show images of the secondcase. In both cases hydrops resolved after treatmentand the fetuses survived uneventfully. In the last case,only one puncture with the 18G-needle was used first toinsert the laser fibre for coagulation of the vessel, thenfor drainage of the unilateral hydrothorax and lastly fordrainage of the polyhydramnios.

One technically successful case was performed byNicolaides, unfortunately ending in neonatal demise(Davenport et al., 2004). Ryan et al. (2003) used thesame laser technique combined with placement of athoraco-amniotic shunt in a hydropic fetus with BPS,with success. This combined procedure makes it difficultto assess which of the two interventions contributes mostto the success. Mass size reduced in both cases.

Ruano et al. (2007) described a case in which coagu-lation of the vessel was incomplete after laser therapy.The mass’s volume increased without reappearance ofhydrops. The child needed ventilatory support in theneonatal period and thoracotomy and lobectomy wereperformed.

Figure 4—Ultrasound image of a large lung lesion with PE

Figure 5—Colour Doppler showing systemic artery, thus diagnosis ofpulmonary sequestration

Figure 6—Colour Doppler after laser therapy showing absence offlow to the lung lesion

Rammos et al. (2010) described resolution of hydropsin two hydropic fetuses with BPS treated with laser. Inboth cases the feeding vessel remained open after lasertreatment. One fetus needed thoracoamniotic shuntingfor residual hydrothorax. The other child needed a thorax



Figure 7—Ultrasound image 10 weeks after laser showing tinyremaining lesion

shunt after birth. Both lesions were resected after birth,in one child respiratory distress was noted after birth.

Interruption of blood flow in the feeding vessel ofBPS has also been described using injections of purealcohol in one case (Nicolini et al., 2000), polidocanolin three cases (Bermudez et al., 2007) and N-butyl-2-cyanoacrylate in one case (Sepulveda et al., 2010). Inall cases the agent was injected directly into the feed-ing vessel of the BPS. Nicolini combined this treatmentwith placement of a thoraco-amniotic shunt. Hydropsresolved in all cases after treatment. The pregnan-cies continued uneventfully and the children were bornasymptomatic. One child, treated with polidocanol scle-rotherapy died in the neonatal period from operativecomplications after resection of the remaining lesion.

Open fetal surgery

Open surgical resection of fetal lung lesions has beenperformed in a small number of fetal treatment centers,mainly in the USA. The largest series to date has beenpublished by the group from Children’s Hospital ofPhiladelphia (Adzick, 2010).

Adzick describes outcome after fetal lobectomy formassive multicystic or predominantly solid CCAMs in24 cases between 21 and 31 weeks of gestation. Thirteen(54%) healthy survivors were reported with uneventfulfollow-up at 1–16 years of age. Resolution of hydropswas seen within 1–2 weeks in these cases.

Of the 11 non-survivors 7 died intraoperatively due tocardiovascular collapse during surgery, 2 became brady-cardic and died within the first day after surgery and 3died of maternal problems (mirror syndrome, postoper-ative chorioamnionitis and preterm contractions).

Recently Cass et al. (2011) reported on three otherhydropic fetuses with large fetal lung lesions (2 CCAMs,1 BPS) that underwent fetal surgery. In two fetuseshydrops resolved and children were liveborn. One child

had no problems after birth and is growing and develop-ing well. One infant suffered from significant tracheo-bronchomalacia and respiratory insufficiency requiringtracheostomy and ventilation. The third fetus died intra-operatively, probably because the disease process haddeveloped too far at the time of intervention.

SUMMARY

We conclude that in the majority of pregnancies wherethe fetus is diagnosed with an isolated lung lesion,the parents can be reassured that the outcome is likelyfavourable. In the absence of hydrops, even large lesionscan be treated expectantly, obviously with frequent(weekly) monitoring and selection of an appropriatesite for delivery. In CCAMs with hydrops, a courseof steroids may be beneficial when gestational ageis under 32 weeks. Although promising, and certainlyattractive given its noninvasive nature, more evidenceis needed to establish its role. Minimally invasive fetalinterventions such as thoracoamniotic shunting of largecysts, or occlusion of the feeding artery in pulmonarysequestrations often lead to good outcome. In uteroresection of large life-threatening solid or microcysticfetal lung tumours, in case of failure of the steroidtreatment, is probably the currently best application oropen fetal surgery in experienced centres.

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