Current outcome of antenally diagnosed cystic lung disease
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
References (61)
- et al.
Prenatal ultrasonography and early surgery for congenital cystic disease of the lung
J Pediatr Surg
(1992) - et al.
Congenital cystic adenomatoid malformation in the fetusNatural history and predictors of outcome
J Pediatr Surg
(1996) - et al.
Chronic drainage of fetal pulmonary cyst
Lancet
(1987) - et al.
Antenatally diagnosed surgical anomaliesThe psychological effect of parental antenatal counselling
J Pediatr Surg
(1998) - et al.
Congenital cystic adenomatoid malformation of the lung. Classification and morphological spectrum
Hum Pathol
(1977) - et al.
Diagnosis and management of congenital cystic disease of the lung in children
J Pediatr Surg
(1986) - et al.
Limited pulmonary resections for congenital cystic adenomatoid malformation of the lung
J Pediatr Surg
(1992) - et al.
The antenatal sonographic detection of lung masses
Am J Obstet Gynecol
(1984) - et al.
Intralobar pulmonary sequestrationA clinical and pathological spectrum
J Pediatr Surg
(1993) - et al.
Intrabdominal pulmonary sequestrationDiagnostic difficulties
Pathology
(1997)