Antenatal and Postnatal Management of Congenital Cystic Adenomatoid Malformation

Kotecha, Sailesh; Barbato, Angelo; Bush, Andrew; Claus, Filip; Davenport, Mark; Delacourt, Christophe; Deprest, Jan; Eber, Elena; Frenckner, Björn; Greenough, Anne; Nicholson, Andrew G.; Antón‐Pacheco, Juan L.; Midulla, Fabio

doi:10.1016/j.prrv.2012.01.002

Cited by 97 publications

(105 citation statements)

References 113 publications

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“…CPAM growth accelerates at 20 weeks and is maximal around 28 weeks, but decreases thereafter [7]. Earlier diagnosis of CPAMs is possible with 3D ultrasound, which is also helpful in the diagnosis of other fetal malformations and dysmorphology, while color Doppler may help in diagnosing hybrid lesions of BPS.…”

Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

“…Earlier diagnosis of CPAMs is possible with 3D ultrasound, which is also helpful in the diagnosis of other fetal malformations and dysmorphology, while color Doppler may help in diagnosing hybrid lesions of BPS. MRI, though not commonly used, may diagnose small lesions and cystic CPAMs [7]. Large CPAM lesions require close follow up, as they may be associated with polyhydramnios and hydrops fetalis [1].…”

Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

“…The most rapid increase in the CVR ratio seems to occur between 20 to 25 weeks. CPAMs with a CVR ratio of < 0.16 correlates with a 94% survival rate and a < 3% risk of developing hydrops fetalis [7]. Fetal intervention is available for certain selected cases with normal karyotype and the absence of associated anomalies.…”

Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

“…Close surveillance is important as some CPAM lesions may increase in size and some may even regress partially. Prenatal betamethasone treatment for CPAMs with hydrops fetalis has improved survival rates [7,4]. Pregnancies complicated by CPAMS should be monitored in tertiary care centers, but once the lesions are detected on ultrasound, the mothers can be managed in regional centers [3].…”

Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

“…These infants are not only at risk for frequent lung infections, pneumothorax, and lung abscesses, but also have a slightly increased risk for malignancy. Though there are some controversies regarding the management of these asymptomatic children, most authors recommend surgical resection during the first year to prevent infection and malignant transformation, as well as to allow compensatory growth [1,9,3,7]. Air travel should be avoided prior to surgical resection due to the risks of pneumothorax [10].…”

Section: Postnatal Managementmentioning

confidence: 99%

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Term Infant with Echogenic Fetal Lung Mass on Prenatal Sonogram

Navaei¹,

Álvarez²,

Rajegowda³

et al. 2015

J Neonatal Biol

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Case HistoryA full term male infant was delivered via normal spontaneous vaginal delivery (NSVD) to a 35 year-old G6P6006 mother with good prenatal care. Maternal antenatal laboratory results were normal. A maternal prenatal sonogram at 20 weeks gestation showed a solid echogenic mass in the left hemithorax ( Figure 1). A second ultrasound confirmed the initial findings, and showed a solid echogenic mass of 2.7 × 3.9 cm in size ( Figure 2). The mother received a Penicillin prophylaxis for positive Group B streptococcus colonization. The infant's Apgar score was 9/9 at 1/5 minutes respectively.The infant weighed 3070 g at birth and physical examination was appropriate for its age, with no abnormality detected. The infant remained clinically stable on room air without developing respiratory distress. The infant's chest rotengogram (CxR) was normal ( Figure 3). The computerized tomogram (CT) of the infant's chest showed a well demarcated, consolidated mass in the left lower lobe which had a solid and a cystic component, suggesting a diagnosis of a congenital pulmonary adenomatoid malformation (CPAM) or a bronchopulmonary sequestration (BPS) of the lung (Figure 4). A Doppler ultrasound of the left lung showed a solid mass, with no feed vessel thereby suggesting BPS as a likely diagnosis ( Figure 5). The baby remained asymptomatic and was discharged home to the parents with scheduled follow up appointments with a pulmonologist and a general pediatrician. The parents were also educated about the infant's clinical condition.The infant did well post-natally with normal growth and development. The infant underwent a thoracoscopic lobectomy of the left lower lobe (LLL) of the lung at 6 months of age. The final diagnosis was intra-lobar sequestration. The infant has been doing well since surgery and no complications were reported. DiscussionCongenital fetal parenchymal lung malformations are uncommon but well documented lung abnormalities. The incidence of such abnormalities is 1 in 10,000 to 35,000 live births, with congenital pulmonary adenomatoid malformation (CPAM) and bronchopulmonary sequestration (BPS) being the most common. These are also the most common lung abnormalities that are diagnosed on prenatal ultrasound [1]. With the improvement in imaging techniques over the past decade, the diagnosis and management of lung abnormalities has completely changed [2].The exact pathophysiology of congenital lung abnormalities is unclear, but CPAMs are characterized by localized abnormal growth of lower respiratory structures during the pseudo-glandular period of fetal development (7 to 17 weeks) [3]. They are characterized by a cystic or solid intrapulmonary mass, usually unilobar with a slight predilection for the lower lobes that is usually diagnosed on routine antenatal ultrasound performed around 18 to 20 weeks of gestation [1]. They receive their blood supply from the pulmonary circulation. By contrast, BPS could manifest as an intra-lobar or extra-lobar malformation which may derive its blood supply from the ascendin...

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Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

Section: Prenatal Diagnosis and Managementmentioning

confidence: 99%

Section: Postnatal Managementmentioning

confidence: 99%

See 3 more Smart Citations

Term Infant with Echogenic Fetal Lung Mass on Prenatal Sonogram

Navaei¹,

Álvarez²,

Rajegowda³

et al. 2015

J Neonatal Biol

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A 9‐year audit of fetal chest masses in an Australian maternal—fetal medicine cohort

Gopikrishna

Henry

Kaur

et al. 2019

Australas J Ultrason Med

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Introduction: To assess fetal vs. neonatal diagnoses, pregnancy outcomes and need for surgery in babies prenatally diagnosed with congenital pulmonary airway malformation (CPAM) or bronchopulmonary sequestration (BPS). Methods: Retrospective single-centre cohort study of fetuses with a prenatal diagnosis of CPAM or BPS between 2006 and 2014. Data collected included serial antenatal ultrasound information and neonatal/infant diagnoses and outcomes. Results: Initial ultrasound diagnosis (n = 63) was CPAM in 51 and BPS in 12: nineteen (30%) fetuses had mediastinal shift and 2 (3%) had hydrops. All neonates with known birth outcome (n = 56) were liveborn. Final diagnosis in 52 infants (83%) with neonatal imaging and/or histopathology confirmed CPAM in 17/44 (39%) and BPS in 6/9 (67%). Of 34 prenatally suspected but unconfirmed CPAM lesions: 10 had no lesion on neonatal imaging, one no neonatal imaging performed, five confirmed BPS, 11 other lung/thoracic lesions, seven were lost to follow-up. There was one infant death. 27/63 infants (43%) had post-natal surgery. More neonates requiring neonatal respiratory support/resuscitation had surgery compared to those who did not (67% vs. 29%, P = 0.008). Patients with suspected CPAM or BPS on both initial and final ultrasound were more likely to have post-natal surgical management than when a lesion was no longer visible on final antenatal ultrasound (68% vs. 23%, P = 0.001). Conclusions: Over 50% of antenatally suspected CPAM/BPS either regressed or had an alternate post-natal diagnosis. Perinatal outcome was good, with the majority of neonates/infants conservatively managed. Persistently visible antenatal lesion and need for neonatal respiratory support predicted ultimate surgical management.

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