Current outcome of antenally diagnosed cystic lung disease

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Abstract

Background/purpose

The natural history of parenchymal lung lesions such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS) has been altered by the advent of antenatal ultrasonography. Initial reports were characterized by a high (about 30%) incidence of adverse features (eg, hydrops) and a poor outcome and did not accord with our recent experience. The authors have reviewed the outcome of fetuses that had been diagnosed in a large tertiary referral fetal medicine unit with the aim of delineating current experience. The term cystic lung disease was used throughout to avoid unjustifiable histologic precision.

Methods

The scans of all fetuses that had been diagnosed with cystic lung disease between January 1995 and July 2001 were reviewed. The outcome of each pregnancy was established, and, where possible, all infants underwent appropriate investigations, including thoracic computed tomography (CT) scans.

Results

Sixty-seven fetuses had a cystic lung abnormality diagnosed from January 1995 to July 2001. The median (range) age at diagnosis was 21 (19 to 28) weeks. The lesion was right sided in 29 (43%), left in 36 (54%), and bilateral in 2 (3%); it was characterized as dominantly macrocystic in 27 (40%), microcystic in 35 (52%), and mixed in 5 (8%). Mediastinal shift was present in 30 fetuses (45%). Severe signs of fetal distress (eg, hydrops) were present in 5 fetuses (7%). Antenatal intervention was performed in 4 fetuses (thoraco-amniotic shunts [n = 3] and percutaneous intrauterine laser therapy [n = 1]). Sixty-four (96%) of the fetuses were born alive. There was 1 termination of pregnancy and 2 intrauterine deaths (all severe microcystic lesions). Forty-two infants (63%) underwent thoracotomy and appropriate excisional surgery at a median of 7.5 months (range, 1 day to 34 months). Two infants (which included the fetus having intrauterine laser therapy) died early in the postnatal period. Both were large microcystic lesions and had antenatal features of severe fetal distress. Twelve infants were investigated in the postnatal period but did not undergo surgery. Ten infants were not appropriately investigated or were lost to follow-up. Histologic examination showed definitive diagnostic features of CCAM (n = 25) or PS (n = 6). Other lesions with hybrid features of both were also seen (n = 11). There was a degree of correlation between antenatal ultrasound features (size of cyst [P = .03], in-utero behavior [P = .06], mediastinal shift [P = 0.05]) and the need for surgery but not with the final histologic diagnosis. Surgical excision was required in 45% of lesions showing late-gestation “resolution.”

Conclusions

Antenatally diagnosed “cystic lung disease” has an excellent prognosis in the absence of signs of severe fetal distress. The need for surgery should be based on appropriate postnatal investigations (eg, CT scans), rather than on antenatal behavior.

Section snippets

Materials and methods

Current obstetric practice in the United Kingdom suggests that every pregnancy undergoes fetal ultrasonography at 18 to 20 weeks’ gestation principally to detect congenital fetal anomalies. The Harris Birthright Centre for Fetal Medicine within Kings College Hospital is a large tertiary referral unit established in 1985 that specializes in the diagnosis and management of a wide range of congenital malformations. Computerized records of all fetuses scanned from January 1995 to July 2001 with a

Clinical series

Sixty-seven fetuses had a cystic lung abnormality diagnosed from January 1995 to July 2001 (Fig 1). The median (range) age at diagnosis was 21 (19 to 28) weeks. The lesion was right sided in 29 (43%), left in 36 (54%), and bilateral in 2 (3%). These were characterized as dominantly macrocystic in 27 (40%), microcystic in 35 (52%), and mixed in 5 (8%). Mediastinal shift was present in 30 fetuses (45%).

Five fetuses (7%) had severe signs of fetal distress such as hydrops (n = 4), pleural effusion

Clinical outcome

Sixty-four (96%) of the fetuses were born alive. There was 1 termination of pregnancy and 2 intrauterine deaths. The fetus that was terminated had bilateral microcystic lung disease and associated renal agenesis. The other 2 deaths (one microcystic and 1 mixed appearance) occurred at 22 and 28 weeks’ gestation. All 3 fetuses were hydropic at presentation.

There were 2 neonatal deaths. One infant with a large microcystic lesion died during attempted surgical excision at 6 hours of age. This fetus

Discussion

This series shows that there is a range of possible diagnoses and outcomes for pulmonary cystic change detected in the antenatal period. The 2 most common histologic diagnoses remain CCAM and bronchopulmonary sequestration, although it is increasingly apparent that the distinction can be unclear, and in our series up to 25% were “hybrid” lesions.

The most common lesion that can be defined antenatally, CCAM, was first described in the English-language literature by Ch’in and Tang in 1947.15

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