Characteristics of congenital cystic adenomatoid malformations associated with nonimmune hydrops and outcome

Vu, Lan; Tsao, KuoJen; Lee, Hanmin; Nobuhara, Kerilyn K.; Farmer, Diana L.; Harrison, Michael R.; Goldstein, Ruth B.

doi:10.1016/j.jpedsurg.2007.03.039

Cited by 61 publications

(46 citation statements)

References 18 publications

(40 reference statements)

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“…Stocker's classification was based upon 38 cases and reflects the pattern of the time since many of the lesions were characterised from autopsied infants with a predominance of type II and type III lesions [1]. Later studies showed that the prognosis of prenatally diagnosed lesions depends on the presence or absence of hydrops [21]. Other prognostic factors [2,15,22,23] include: (i) the size of the lesion and its secondary effects (mediastinal shift, the extent of pulmonary hypoplasia, polyhydramnios, cardiovascular compromise); (ii) the degree of development of the unaffected lung, and (iii) the presence or absence of other congenital anomalies, such as extralobar sequestration, diaphragmatic hernia, pulmonary hypoplasia, cardiovascular malformation, hydrocephalus, skeletal malformation, jejunal atresia, bilateral renal agenesis/dysgenesis and Pierre Robin syndrome [11,24].…”

Section: Challenging Diagnosis and Classificationmentioning

confidence: 99%

“…Anasarca and placentomegaly appear in advanced cases [54,55]. Three features were found to be highly specific for foetal hydrops in foetuses with large masses: a mass-thorax ratio of at least 56%, cystic predominance and diaphragm eversion [21]. Gigantic foetal lung lesions have also other pathophysiologic consequences, including oesophageal compression by the thoracic mass, which interferes with amniotic fluid swallowing and results in polyhydramnios [15,56].…”

Section: Diagnosismentioning

confidence: 99%

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Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation

2016

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Congenital pulmonary airway malformation (CPAM) is one of the most common lung lesions detected prenatally. Despite the research efforts made in the past few years, controversy and lack of clarity in the literature still exist regarding nomenclature, classification, pathogenesis and the management of CPAM. Therefore, it is of greatest importance to delineate the natural history of CPAMs and to create a consensus to guide the management and follow-up of these lesions. This review will focus on classification systems, highlighting the most recent advancements in pathogenesis, and current practice in the prenatal diagnosis of CPAM. Strategies of prenatal management and postnatal management will be reviewed. Long-term follow-up, including lung cancer risk, is discussed and an outcome perspective is presented.

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Section: Challenging Diagnosis and Classificationmentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation

2016

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“…1 Later studies showed that the prognosis of prenatally diagnosed lesions depends on the presence or absence of hydrops. 7 Other prognostic factors include: the size of the lesions and its secondary effects namely mediastinal shift, the extent of pulmonary hypoplasia, polyhydramnios, cardiovascular compromise, the degree of development of the unaffected lung, and the presence or absence of other congenital anomalies such as extra lobar sequestration, diaphragmatic hernia, pulmonary hypoplasia, cardiovascular malformation, hydrocephalus, skeletal malformation, jejunal atresia, bilateral renal agenesis/dysgenesis and Pierre Robin syndrome. The natural history of prenatal cystic lung lesions varies from complete regression in utero to life threatening hydrops foetalis.…”

Section: Classification Of Ccamsmentioning

confidence: 99%

Congenital cystic adenomatoid malformation (CCAM): antenatal and postnatal management

Kanavi

Thangaraj

Thomas

2017

Int J Reprod Contracept Obstet Gynecol

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Background: Congenital cystic adenomatoid malformation (CCAM) is a rare abnormality of lung development. The estimated incidence ranges from 1: 10,000 to 1.35,000 live births. It is diagnosed by prenatal screening at 18-20 weeks of gestation. Surgical excision of symptomatic lesions is relatively straight forward, but management of asymptomatic lesions is controversial. Methods: Among women who delivered at St. Johns medical college and hospital, Bangalore between Jan 2011 to Dec 2016, those with the diagnosis of CCAM during anomaly scan were included in the study. Antenatal and Postnatal period and their outcomes were evaluated. Follow up was extend up to the childhood in the affected foetus. Results: There were 5 cases of CCAM in 13057 deliveries during 5-year study. Incidence was 1:2611. Mean gestational age at diagnosis was 21.6±2.5weeks. All foetus had CVR (CCAM volume) ratio more than 1.6 and there was no compromise on lung volume. Mean lung volume was 62.8±8.6 cc. and mean Apgar score at 1minute was 6.8±2.7 and at 5 minutes was 8.0±2.2. Among 5 foetuses, 2 foetuses had regression of cyst by birth and 3 underwent surgery for resection after birth. Conclusions: CCAM remains a challenge for obstetricians, neonatologists and paediatric surgeons. The combination of prenatal MRI and serial ultrasound studies optimize foetal surveillance and postnatal care. In asymptomatic CCAM, babies should be followed up to adolescence and adulthood, as they can manifest with malignant changes.

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“…Only if the mass is progressively enlarging with no sign of regression in the last 10 wk of the pregnancy, or if there is fetal hydrops, should any intervention be contemplated. If intervention is undertaken [11][12][13][14][15][16], the options are given in Table 5; the evidence base for any of these is limited, and all should be considered as a last resort.…”

Section: Management Of Antenatally Presenting Congenital Thoracic Malmentioning

confidence: 99%

Rare Lung Diseases: Congenital Malformations

Bush

2015

Indian J Pediatr

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Increasingly, congenital thoracic malformations (CTMs) are diagnosed on antenatal ultrasound, but we lack the evidence to suggest rational management, not least because descriptive terms are used inconsistently. This review describes a simplified clinical classification of CTMs, and contrasts it with pathological descriptions. The age related presentations of CTM are described, together with the differential diagnoses of cystic masses presenting both antenatally and postnatally. Antenatally diagnosed CTMs rarely require intervention before birth; and urgent treatment is only required postnatally if the baby is symptomatic and does not respond to medical management. The asymptomatic baby with an antenatal diagnosis of a CTM presents a management conundrum. Definitive imaging is with high-resolution computed tomography (HRCT), but the optimal timing of imaging is unclear. Whether surgery should be offered to asymptomatic infants is also unclear; in the medium term, 5 % of asymptomatic babies will require surgery for complications of the disease. The most vexed question is malignant change; the risk in the medium term is probably less than 5 %, but we have no way of delineating a high-risk group. Indeed, malignancy has been described even after complete resection of a CTM. The author's personal management is to advocate surgery in the second year of life for all except for the most trivial CTMs, but many would differ and advocate conservative management. More data are needed if we are to rationalise our approach to these infants.

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Characteristics of congenital cystic adenomatoid malformations associated with nonimmune hydrops and outcome

Cited by 61 publications

References 18 publications

Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation

Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation

Congenital cystic adenomatoid malformation (CCAM): antenatal and postnatal management

Rare Lung Diseases: Congenital Malformations

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