Cystic adenomatoid malformation of the lung: neonatal management of 21 cases

Waszak, Paul; Claris, Olivier; Lapillonne, Alexandre; Picaud, Jean‐Charles; Basson, E; Chappuis, J. P.; Salle, B

doi:10.1007/s003830050592

Cited by 63 publications

(65 citation statements)

References 14 publications

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“…(15,21) The combination of CCAM and certain other malformations, such as pulmonary sequestration, has been demonstrated in various studies, raising the hypothesis that type II CCAM and extralobar pulmonary sequestration have the same embryonic origin. (13,15,18,20,27,30,34,35) However, it is much less common for CCAM to be accompanied by malformations such as facial defects, heart defects, neural tube defects, renal dysplasia/agenesis, and omphalocele. (15,27,34) The lesions vary widely in size and can affect an entire lobe or part of it, as well as an entire lung.…”

Section: Ccammentioning

confidence: 99%

“…(13,15,18,20,27,30,34,35) However, it is much less common for CCAM to be accompanied by malformations such as facial defects, heart defects, neural tube defects, renal dysplasia/agenesis, and omphalocele. (15,27,34) The lesions vary widely in size and can affect an entire lobe or part of it, as well as an entire lung. The lesion can affect both sides of patients with equal frequency and can affect any lung lobe, although it occurs more frequently in the lower lobes and rarely affects more than one lobe (85-95% in only one lobe; Figure 3), being slightly more common in males than in females.…”

Section: Ccammentioning

confidence: 99%

“…Of all congenital lung malformations, 25-30% are CCAMs, (15) and approximately 30% of all patients with CCAM are at risk of respiratory failure at birth. (27) It has been shown that CCAMs are hamartomatous lesions, (27) with focal dysplasia and anomalous development, (4,28) and are characterized by a multicystic mass of lung tissue with proliferation of bronchial structures and lung tissue showing aberrant, differentiated architecture, with various degrees of cyst formation. (5,26,(29)(30)(31)(32) The possible mechanisms by which the lesion develops and the exact period of time over which it develops have yet to be determined; however, there is evidence that CCAMs occur between gestational weeks 5 and 22 and result in a wide variety of pathological and radiological presentations.…”

Section: Ccammentioning

confidence: 99%

“…(4,20,33) Histologically, cartilage is absent, reflecting bronchial malformation; however, patients with CCAM present with small bronchial communications, which are very likely responsible for the infections, and hyperinflation. (27,33) Cystic formations are covered with columnar or cuboidal epithelium.…”

Section: Ccammentioning

confidence: 99%

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Malformações pulmonares congênitas

2011

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Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.Keywords: Cystic adenomatoid malformation of lung, congenital; Bronchopulmonary sequestration; Pulmonary surgical procedures; Diagnosis. ResumoAs malformações congênitas do pulmão são raras e variam muito na sua forma de apresentação clínica e gravidade, dependendo principalmente do grau de envolvimento pulmonar e de sua localização na cavidade torácica. Elas podem se manifestar em qualquer idade e podem ser fonte de importante morbidade e mortalidade em lactentes e crianças. Os indivíduos com malformações congênitas do pulmão podem apresentar sintomas respiratórios ao nascimento, enquanto outros podem permanecer assintomáticos por longos períodos. Atualmente, com o uso rotineiro da ultrassonografia pré-natal, vem ocorrendo um aumento no diagnóstico mais precoce dessas malformações. A manifestação clínica dessas malformações varia desde uma disfunção respiratória pós-natal imediata a um achado acidental na radiografia de tórax. O diagnóstico precoce e o tratamento imediato oferecem a possibilidade de um desenvolvimento pulmonar absolutamente normal. Quando assintomáticos, a conduta para o tratamento dos pacientes com malformações pulmonares ainda é controversa, uma vez que o prognóstico dessas afecções é imprevisível. O manejo dessas lesões depende do tipo de malformação e de sintomas. Devido ao risco de complicação, a maioria dos autores sugere a ressecção da lesão no momento em que essa é identificada. A lobectomia é o procedimento de escolha, fornecendo excelentes resultados a longo prazo. Este artigo descreve as principais malformações pulmonares congênitas, seu diagnóstico e controvérsias quanto o tratamento. (5) is covered by ...

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Section: Ccammentioning

confidence: 99%

Section: Ccammentioning

confidence: 99%

Section: Ccammentioning

confidence: 99%

Section: Ccammentioning

confidence: 99%

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Malformações pulmonares congênitas

2011

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“…Some recommend surgery in the early neonatal period 14 or after 4 weeks of age. 15 Others advocate waiting for at least 3 months, 2,16 operating within the first year of life 10 or delaying surgery until age 24 months.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

268

215

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Summary. Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax. Asymptomatic cysts present a therapeutic dilemma: surgical intervention and ''conservative'' observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable from benign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90% of children. If not excised, cystic PPB evolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of ''malignancy in a congenital lung cyst'' are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

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Prenatal diagnosis of partial trisomy 1q and monosomy X in a fetus with a congenital lung lesion and hydrops fetalis

Pressey

Wilson

Kasperski

et al. 2007

American J of Med Genetics Pt A

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We report on the prenatal diagnosis of partial trisomy 1q and monosomy X in a fetus with a congenital lung lesion and hydrops. The finding of hydrops in a fetus with a small lung lesion, congenital cystic adenomatoid malformation (CCAM) volume to head circumference ratio (CVR) 0.78, prompted cytogenetic analysis of amniotic fluid, revealing an unbalanced translocation between chromosomes X and 1 [46,X,der(X)t(X;1)(p11.2;q25 or q31)]. The incidence of chromosomal abnormalities with CCAM lesions is estimated at 1.6%. This is the first reported case of prenatally diagnosed partial trisomy 1q and monosomy X presenting as a fetal lung lesion and hydrops.

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Cystic adenomatoid malformation of the lung: neonatal management of 21 cases

Cited by 63 publications

References 14 publications

Malformações pulmonares congênitas

Malformações pulmonares congênitas

Pulmonary cysts in early childhood and the risk of malignancy

Prenatal diagnosis of partial trisomy 1q and monosomy X in a fetus with a congenital lung lesion and hydrops fetalis

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