WordNet

something abnormal or anomalous (同)miscreation
of or relating to or resembling a cyst
of or relating to a normal cyst (as the gallbladder or urinary bladder)
present at birth but not necessarily hereditary; acquired during fetal development (同)inborn, innate
a closed sac that develops abnormally in some body structure

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(特に生物体の)奇形;{C}奇形部分
胞嚢(ほうのう)性の
(病気・身体的欠陥など)生まれつきの,先天的な
胞嚢(ほうのう)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/05/22 09:33:23」(JST)

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Congenital cystic adenomatoid malformation (CCAM) is a congenital disorder of the lung similar to bronchopulmonary sequestration. The disorder is often referred to by another name, congenital pulmonary airway malformation (CPAM). In CCAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue. The underlying cause for CCAM is unknown. It occurs in approximately 1 in every 30,000 pregnancies. ^[1]

In most cases the outcome of a fetus with CCAM is very good. However in a rare few cases the cystic mass grows so large as to limit the growth of the surrounding lung and cause pressure against the heart. In these situations, the CCAM can be life-threatening for the fetus.

Diagnosis [edit]

CCAM on chest radiograph in a newborn. Large cystic changes in the left lung, leading to a mediastinal shift to the right due to their mass effect.

CCAMs are often identified during routine prenatal ultrasonography. Identifying characteristics on the sonogram include: an echogenic (bright) mass appearing in the chest of the fetus, displacement of the heart from its normal position, a flat or everted (pushed downward) diaphragm, or the absence of visible lung tissue.

CCAMS are classified into three different types based largely on their gross appearance. Type I has a large (>2 cm) multiloculated cysts. Type II has smaller uniform cysts. Type III is not grossly cystic, referred to as the "adenomatoid" type. Microscopically, the lesions are not true cysts, but communicate with the surrounding parenchyma. Some lesions have an abnormal connection to a blood vessel from an aorta and are referred to as "hybrid lesions."

Treatment [edit]

In most cases, a fetus with CCAM is closely monitored during pregnancy and the CCAM is removed via surgery after birth.^[2] Most babies with a CCAM are born without complication and are monitored during the first few months. Many patients have surgery, typically before their first birthday, because of the risk of recurrent lung infections associated with CCAMs. Some pediatric surgeons can safely remove these lesions using very tiny incisions with the aid of a telescope (thoracoscopy). However, some CCAM patients live a full life without any complication or incident. It is hypothesized that there are thousands of people living with an undetected CCAM and it is only in recent years that the diagnosis of a CCAM due to a fetal ultrasound, that many patients are aware that they live with this condition. Rarely, long standing CCAMs have been reported to become cancerous.

Very large cystic masses might pose a danger during birth because of the airway compression. In this situation, a special surgical type of delivery called the EXIT procedure may be used.

In rare extreme cases, where fetus's heart is in danger, fetal surgery can be performed to remove the CCAM. If non-immune hydrops fetalis develop, there is a near universal mortality of the fetus without intervention. Fetal surgery can improve the survival percentage up to 50-60%. Recently, several studies found that a single course of prenatal steroids (betamethasone) may increase survival in hydropic fetuses with microcystic CCAMs to 75-100%.^[3]^[4] These studies indicate that large microcystic lesions may be treated prenatally without surgical intervention. Large macrocyst lesions may require in utero placement of a Harrison thoracoamniotic shunt.

Pop Culture [edit]

In the 17th episode of season 3 of House entitled "Fetal Position", the primary patient is a pregnant woman whose fetus is ultimately diagnosed with CCAM. In the climax of the episode, the team performs open fetal surgery to remove the cysts.

References [edit]

^ http://synapse.koreamed.org/Synapse/Data/PDFData/0003TRD/trd-72-501.pdf
^ Ehrenberg-Buchner, S; Stapf, AM; Berman, DR; Drongowski, RA; Mychaliska, GB; Treadwell, MC; Kunisaki, SM (2012 Nov 15). "Fetal lung lesions: can we start to breathe easier?". American journal of obstetrics and gynecology. PMID 23159697.
^ Curran PF, Jelin EB, Rand L, Hirose S, Feldstein VA, Goldstein RB, Lee H (2010). "Prenatal steroids for microcystic congenital cystic adenomatoid malformations". J Pediatr Surg. 45 (1): 145–50. doi:10.1016/j.jpedsurg.2009.10.025. PMID 20105595.
^ Peranteau WH, Wilson RD, Liechty KW, Johnson MP, Bebbington MW, Hedrick HL, Flake AW, Adzick NS (2007). "Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival". Fetal Diagn Ther 22 (5): 365–371. doi:10.1159/000103298.

External links [edit]

Congenital Cystic Adenomatoid Malformation | UCSF Fetal Treatment Center

Congenital Cystic Adenomatoid Malformation | Congenital Pulmonary Airway Malformation Radiology and Pathology

Congenital Cystic Adenomatoid Malformation (CCAM) and Bronchopulmonary Sequestration | Center for Fetal Diagnosis and Treatment | The Children's Hospital of Philadelphia

UpToDate Contents

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1. 先天性肺気道（嚢胞性腺腫様）形成異常 congenital pulmonary airway cystic adenomatoid malformation
2. 先天性肺気道奇形の出生前診断およびマネージメント prenatal diagnosis and management of congenital pulmonary airway malformation
3. 肺発達異常の放射線学的所見 radiographic appearance of developmental anomalies of the lung
4. 気管支肺分画症 bronchopulmonary sequestration
5. 先天性肺葉性肺気腫 congenital lobar emphysema

English Journal

FGF10 Signaling differences between type I pleuropulmonary blastoma and congenital cystic adenomatoid malformation.

Lezmi G, Verkarre V, Khen-Dunlop N, Vibhushan S, Hadchouel A, Rambaud C, Copin MC, Rittie JL, Benachi A, Fournet JC, Delacourt C.AbstractBACKGROUND: Type I pleuropulmonary blastoma (PPB) and congenital cystic adenomatoid malformation of the lung (CCAM) are cystic lung diseases of childhood. Their clinical and radiological presentations are often similar, and pathologic discrimination remains difficult in many cases. As a consequence, type I PPB and CCAM are frequently confused, leading to delayed adequate management for type I PPB. Recent studies have suggested a role for fibroblast growth factor (FGF) 10 signal pathway in CCAM pathogenesis. The objective of our study was to determine whether FGF10 signaling differs between CCAM and type I PPB.
Orphanet journal of rare diseases.Orphanet J Rare Dis.2013 Sep 3;8(1):130. [Epub ahead of print]
BACKGROUND: Type I pleuropulmonary blastoma (PPB) and congenital cystic adenomatoid malformation of the lung (CCAM) are cystic lung diseases of childhood. Their clinical and radiological presentations are often similar, and pathologic discrimination remains difficult in many cases. As a consequence,
PMID 24004862

[Congenital cystic adenomatoid malformations of the lung: Diagnosis, treatment, pathophysiological hypothesis].

Lezmi G, Hadchouel A, Khen-Dunlop N, Vibhushan S, Benachi A, Delacourt C.SourceService de pneumologie pédiatrique, centre de référence pour les maladies respiratoires rares de l'enfant, hôpital Necker-Enfants-Malades, AP-HP, 149, rue de Sèvres, 75743 Paris cedex 15, France; Inserm, U955, IMRB, équipe 04, Créteil, France. Electronic address: lezmi.g@hotmail.com.
Revue de pneumologie clinique.Rev Pneumol Clin.2013 Aug;69(4):190-7. doi: 10.1016/j.pneumo.2013.06.001. Epub 2013 Jul 11.
Congenital cystic adenomatoid malformations (CCAM) of the lung are the most frequent congenital lung malformations. Their diagnosis is based on histological features. CCAM consist of bronchopulmonary cystic lesions which are classified according to the presence and cysts size. Type I CCAM are compos
PMID 23850268

Pre and perinatal aspects of congenital cystic adenomatoid malformation of the lung.

Lima JS, Camargos PA, Aguiar RA, Campos AS, Aguiar MJ.SourceMedical School .
The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians.J Matern Fetal Neonatal Med.2013 Jul 10. [Epub ahead of print]
Abstract Objective: To identify the incidence of congenital cystic adenomatoid malformation of the lung (CCAM) at birth; to evaluate prenatal and perinatal history, association with clinical and sociodemographic variables and concordance between CT scan results and anatomopathology studies. Method:
PMID 23697759

Japanese Journal

先天性横隔膜ヘルニアに肺分画症およびCCAMを合併した1症例

本田正樹,金場俊二,山本裕俊
日本小児外科学会雑誌 48(7), 1024-1028, 2012-12-20
症例は日齢0,女児.出生後仮死が進行したため当院紹介され,胸腹部単純レントゲン写真より先天性左横隔膜ヘルニア(CDH)と診断した.新生児遷延性肺高血圧症改善後の日齢6に経腹的CDH根治術を施行した.その際左胸腔前壁に肺葉外肺分画症を認め同時に摘出術を行った.術後weaningを進めることができず,胸部単純レントゲン写真で左下肺野に増大する嚢胞陰影を認めた.術後17日目のCT検査にて左肺下葉先天性嚢 …
NAID 110009562246

研究・症例アスペルギローマを合併したcongenital cystic adenomatoid malformationの1成人例

三井いずみ,加賀美武,吉田正史 [他]
日本胸部臨床 71(12), 1247-1253, 2012-12-00
NAID 40019513021

先天性嚢胞性腺腫様奇形(CCAM)の2切除例

川口庸,花岡淳,橋本雅之,北村将司,寺本晃治,朝倉庄志
日本呼吸器外科学会雑誌 = The journal of the Japanese Association for Chest Surgery 26(7), 739-745, 2012-11-15
NAID 10031126002

Related Pictures

★リンクテーブル★

リンク元	「先天性嚢胞性腺様奇形」「CCAM」「先天性嚢胞性腺腫様奇形」
拡張検索	「congenital cystic adenomatoid malformation of lung」
関連記事	「malformation」「congenita」「congenital」「cystic」

「先天性嚢胞性腺様奇形」

Congenital cystic adenomatoid malformation
	Classification and external resources
ICD-10	Q34.8 (EUROCAT Q33.80)
ICD-9	748.4
DiseasesDB	32408
eMedicine	ped/534 radio/186
MeSH	D015615