Cystic Adenomatoid Malformation

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Fanqing Meng - One of the best experts on this subject based on the ideXlab platform.

  • congenital Cystic Adenomatoid Malformation of lung in adults 2 rare cases report and review of the literature
    Diagnostic Pathology, 2012
    Co-Authors: Anning Feng, Hourong Cai, Qi Sun, Yifen Zhang, Lulu Chen, Fanqing Meng
    Abstract:

    Congenital Cystic Adenomatoid Malformation (CCAM), also named congenital pulmonary airway Malformation (CPAM), is a congenital abnormality of lung which is uncommon in adults. Here we present 2 adult cases of CCAM with unusual clinical and pathologic findings. One case was complicated with aspergillosis which was seldom reported. The other case was suffered bilateral lesions and the patient's mother had been previously radiographically discovered bilateral Cystic lesions that CCAM could not be ruled out. A review of currently published related literatures has also been provided.

  • congenital Cystic Adenomatoid Malformation of lung in adults 2 rare cases report and review of the literature
    Diagnostic Pathology, 2012
    Co-Authors: Anning Feng, Hourong Cai, Qi Sun, Yifen Zhang, Lulu Chen, Fanqing Meng
    Abstract:

    Congenital Cystic Adenomatoid Malformation (CCAM), also named congenital pulmonary airway Malformation (CPAM), is a congenital abnormality of lung which is uncommon in adults. Here we present 2 adult cases of CCAM with unusual clinical and pathologic findings. One case was complicated with aspergillosis which was seldom reported. The other case was suffered bilateral lesions and the patient's mother had been previously radiographically discovered bilateral Cystic lesions that CCAM could not be ruled out. A review of currently published related literatures has also been provided. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/6406766736634578 .

Christian Domingo - One of the best experts on this subject based on the ideXlab platform.

M R Harrison - One of the best experts on this subject based on the ideXlab platform.

  • resolution of hydrops fetalis in congenital Cystic Adenomatoid Malformation after prenatal steroid therapy
    Journal of Pediatric Surgery, 2003
    Co-Authors: Kuojen Tsao, Samuel Hawgood, Lan T Vu, Shinjiro Hirose, Roman M Sydorak, Craig T Albanese, Diana L Farmer, M R Harrison
    Abstract:

    BACKGROUND/PURPOSE: Development of hydrops fetalis in fetuses with congenital Cystic Adenomatoid Malformations (CCAM) is a significant risk factor for fetal or neonatal demise. In rare cases, resolution of CCAM has occurred, presumably owing to lesion maturation or involution. Steroid therapy, utilized for lung maturity, has been postulated to accelerate this process. The natural history of hydropic fetuses with CCAM after receiving steroid therapy is presented. METHODS: The authors prospectively followed up with 3 patients who had antenatally diagnosed CCAM and nonimmune hydrops fetalis with predicted mortality. All patients declined or were not candidates for fetal intervention and were treated with standard prenatal betamethasone to increase lung maturity. RESULTS: Three fetuses had CCAM and nonimmune hydrops fetalis diagnosed prenatally. After a course of prenatal steroids during the second trimester, all 3 patients had resolution of their hydrops and were delivered at term without respiratory distress. CONCLUSIONS: Nonimmune hydrops fetalis in fetuses with congenital Cystic Adenomatoid Malformation is a harbinger for fetal demise. The resolution of hydrops in these patients after receiving steroid therapy is an interesting and compelling observation. Because the mechanism of this process is speculative, further studies are needed to elucidate the relationship between antenatal steroids and maturation of congenital Cystic Adenomatoid Malformation.

  • resolution of hydrops fetalis in congenital Cystic Adenomatoid Malformation after prenatal steroid therapy
    Journal of Pediatric Surgery, 2003
    Co-Authors: Kuojen Tsao, Samuel Hawgood, M R Harrison, Shinjiro Hirose, Roman M Sydorak, Craig T Albanese, Diana L Farmer, Hanmin Lee
    Abstract:

    Abstract Background/Purpose: Development of hydrops fetalis in fetuses with congenital Cystic Adenomatoid Malformations (CCAM) is a significant risk factor for fetal or neonatal demise. In rare cases, resolution of CCAM has occurred, presumably owing to lesion maturation or involution. Steroid therapy, utilized for lung maturity, has been postulated to accelerate this process. The natural history of hydropic fetuses with CCAM after receiving steroid therapy is presented. Methods: The authors prospectively followed up with 3 patients who had antenatally diagnosed CCAM and nonimmune hydrops fetalis with predicted mortality. All patients declined or were not candidates for fetal intervention and were treated with standard prenatal betamethasone to increase lung maturity. Results: Three fetuses had CCAM and nonimmune hydrops fetalis diagnosed prenatally. After a course of prenatal steroids during the second trimester, all 3 patients had resolution of their hydrops and were delivered at term without respiratory distress. Conclusions: Nonimmune hydrops fetalis in fetuses with congenital Cystic Adenomatoid Malformation is a harbinger for fetal demise. The resolution of hydrops in these patients after receiving steroid therapy is an interesting and compelling observation. Because the mechanism of this process is speculative, further studies are needed to elucidate the relationship between antenatal steroids and maturation of congenital Cystic Adenomatoid Malformation. J Pediatr Surg 38:508-510. Copyright 2003, Elsevier Science (USA). All rights reserved.

  • management of the fetus with a Cystic Adenomatoid Malformation
    World Journal of Surgery, 1993
    Co-Authors: Scott N Adzick, M R Harrison
    Abstract:

    Prenatal detection and serial sonographic study of fetuses with congenital Cystic Adenomatoid Malformation (CCAM) now makes it possible to define the natural history of this lesion, determine the pathophysiologic features that affect clinical outcome, and formulate management based on prognosis. In a series of 22 prenatally diagnosed cases, the overall prognosis depended on the effects of the lung mass and the consequent physiologic derangement: A large mass may cause mediastinal shift, hypoplasia of normal lung tissue, polyhydramnios, and cardiovascular compromise leading to fetal hydrops. Two types of CCAM were distinguished by gross anatomy, ultrasonographic findings, and prognosis. MicroCystic lesions (cysts 5 mm) are not usually associated with hydrops and have a more favorable prognosis. Four initially large lesions partially disappeared on serial prenatal sonography, suggesting that improvement can occasionally occur during fetal life. The finding that fetuses with hydrops are at high risk for fetal or neonatal demise led to fetal surgical resection of the massively enlarged pulmonary lobe (fetal lobectomy) in two cases. Fetuses with CCAM but without hydrops have a good chance for survival with maternal transport, planned delivery, and immediate neonatal resuscitation and surgery.

Anning Feng - One of the best experts on this subject based on the ideXlab platform.

  • congenital Cystic Adenomatoid Malformation of lung in adults 2 rare cases report and review of the literature
    Diagnostic Pathology, 2012
    Co-Authors: Anning Feng, Hourong Cai, Qi Sun, Yifen Zhang, Lulu Chen, Fanqing Meng
    Abstract:

    Congenital Cystic Adenomatoid Malformation (CCAM), also named congenital pulmonary airway Malformation (CPAM), is a congenital abnormality of lung which is uncommon in adults. Here we present 2 adult cases of CCAM with unusual clinical and pathologic findings. One case was complicated with aspergillosis which was seldom reported. The other case was suffered bilateral lesions and the patient's mother had been previously radiographically discovered bilateral Cystic lesions that CCAM could not be ruled out. A review of currently published related literatures has also been provided.

  • congenital Cystic Adenomatoid Malformation of lung in adults 2 rare cases report and review of the literature
    Diagnostic Pathology, 2012
    Co-Authors: Anning Feng, Hourong Cai, Qi Sun, Yifen Zhang, Lulu Chen, Fanqing Meng
    Abstract:

    Congenital Cystic Adenomatoid Malformation (CCAM), also named congenital pulmonary airway Malformation (CPAM), is a congenital abnormality of lung which is uncommon in adults. Here we present 2 adult cases of CCAM with unusual clinical and pathologic findings. One case was complicated with aspergillosis which was seldom reported. The other case was suffered bilateral lesions and the patient's mother had been previously radiographically discovered bilateral Cystic lesions that CCAM could not be ruled out. A review of currently published related literatures has also been provided. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/6406766736634578 .

V Spina - One of the best experts on this subject based on the ideXlab platform.

  • Cystic Adenomatoid Malformation of the lung clinical evolution and management
    European Journal of Pediatrics, 1999
    Co-Authors: Pietro Bagolan, A Nahom, Claudio Giorlandino, A Trucchi, E Bilancioni, A Inserra, G Gambuzza, V Spina
    Abstract:

    Cystic Adenomatoid Malformation of the lung (CAML) is a rare pulmonary maldevelopment resulting from an abnormal growth of the terminal bronchial structures. This study proposes a possible management of prenatally diagnosed CAML. A group of nine cases of CAML diagnosed prenatally between January 1990 and December 1995 was studied retrospectively. The evolution of lesions was followed in utero by serial ultra- sound monitoring. Chest X-ray was performed at birth in all neonates and CT only in the symptomatic ones. CAML was macroCystic in three cases and microCystic in six. No polyhydramnios, hydrops or associated Malformations were seen. In four cases, CAML was confirmed at birth and required surgery. The lesion decreased in size prenatally in five fetuses, of whom only one underwent surgery. In two further cases a prenatal diagnosis of CAML was changed to diaphragmatic hernia. Conclusion Given the possible clinical disappearance or resolution of Cystic Adenomatoid Malformation of the lung, we believe that surgery is justified at birth only in symptomatic and radiologically positive neonates. It could be safely delayed in those asymptomatic patients with either positive or negative chest X-ray. The former need computed tomography at birth, whereas, in the latter, it should be performed at 6 months of age for a more definitive assessment of the patient.

  • Cystic Adenomatoid Malformation of the lung clinical evolution and management
    European Journal of Pediatrics, 1999
    Co-Authors: Pietro Bagolan, A Nahom, Claudio Giorlandino, A Trucchi, E Bilancioni, A Inserra, G Gambuzza, V Spina
    Abstract:

    Cystic Adenomatoid Malformation of the lung (CAML) is a rare pulmonary maldevelopment resulting from an abnormal growth of the terminal bronchial structures. This study proposes a possible management of prenatally diagnosed CAML. A group of nine cases of CAML diagnosed prenatally between January 1990 and December 1995 was studied retrospectively. The evolution of lesions was followed in utero by serial ultrasound monitoring. Chest X-ray was performed at birth in all neonates and CT only in the symptomatic ones. CAML was macroCystic in three cases and microCystic in six. No polyhydramnios, hydrops or associated Malformations were seen. In four cases, CAML was confirmed at birth and required surgery. The lesion decreased in size prenatally in five fetuses, of whom only one underwent surgery. In two further cases a prenatal diagnosis of CAML was changed to diaphragmatic hernia.