ABSTRACT
Congenital cystic adenomatoid malformation of the lung is a rare clinical lung disease.It is the deformity formed by abnormal embryo development of lung tissue.Its pathological morphology is very different from other types of pulmonary cyst.The main pathogenesis,clinical manifestation,diagnosis,differential diagnosis and treatment are described.
ABSTRACT
PURPOSE: To identify neonatal characteristics associated with congenital cystic adenomatoid malformation (CCAM) who required early operations and to introduce preoperative interventions to delay definitive operations until stabilized. METHODS: A retrospective review of dataset was performed from January 2000 to December 2011 for neonates admitted to NICU at Asan Medical Center with prenatally diagnosed CCAM. Variable prenatal and postnatal factors were compared for those who required early operations to those asymptomatic neonates who required elective operations at later age. RESULTS: A total of 60 patients were enrolled and patients were divided into 2 groups according to time of operation. Median time of surgery for group 1 (n=12, 20%) and group 2 (n=48, 80%) was 5.5 days and 504 days, respectively. Maternal characteristics including age, parity, preterm labor, oligohydramnios were similar in between two groups. Factors associated with early operation included prenatal history of polyhydramnios (OR 23, P=0.001), who had undergone fetal interventions (OR 47, P=0.001), low 1 and 5 minute Apgar scores and increasing fetal fluid-filled cystic sizes (OR 26, P=0.013). Of those 3 neonates who required preoperative interventions to decrease air-filled cysts to relieve initial respiratory symptoms were successful and all underwent for definitive operations during NICU hospitalizations. All survived. CONCLUSION: Most neonates with CCAM undergo elective operations during infancy. However, about 20% neonates with polyhydramnios, fetal intervention, increasing fetal cystic mass and born with low Apgar scores required early operations for whom prenatal and postnatal interventions prior to definitive surgery can improve survival rate.
Subject(s)
Female , Humans , Infant, Newborn , Pregnancy , Cystic Adenomatoid Malformation of Lung, Congenital , Hospitalization , Lung , Obstetric Labor, Premature , Oligohydramnios , Parity , Polyhydramnios , Retrospective Studies , Risk Factors , Survival RateABSTRACT
Objective To explore the clinical value and typing characteristic of prenatal ultrasonography for fetal cystic adenomatoid malformation of the lung(CAML).Methods Ultrasonographic features and typing of fetal CAML in 41 cases detected by prenatal ultrasonography were analyzed retrospectively.All cases were followed up until to the induction of labor or birth.Results (1)Site of tumor: in the 41 cases,there were 22 cases on the left side,15 on the right side and 4 on bilateral.(2)Typing diagnosis of prenatal ultrasonography: three cases were CAML type Ⅰ,14 were type Ⅱ,and 24 cases were type Ⅲ.(3)Pathology diagnosis: thirty two cases were induced abortion.CAML was confirmed in 29 cases by autopsy and the classifications were consistent with the prenatal diagnosis of ultrasonography.Three pulmonary sequestration cases were prenatally misdiagnosed as CAML type Ⅲ by ultrasonography.(4)The other nine cases were followed up to birth.Three masses decreased gradually and then disappeared.Six newborns were confirmed as CAML by CT.Their typings were consistent with the postnatal diagnosis.(5)The diagnostic accuracy rate of prenatal ultrasonography for CAML was 92.7%(38/41).Its misdiagnostic rate was 7.3%(3/41).Conclusions Prenatal ultrasonography has a high accuracy rate for the diagnosis and classification of CAML and is the first choice to detect CAML early in pregnancy.It has an important clinical value.Pulmonary sequestration should be distinguished from CAML type Ⅲ because they tend to be confused.
ABSTRACT
La malformación adenomatoidea quística pulmonar (MAQP)es una anomalía de la vía aérea pulmonar poco frecuente cuyodiagnóstico suele realizarse en el período prenatal medianteecografía. Durante el embarazo, debe realizarse seguimiento ecográfico para valorar el desarrollo pulmonar. Presentamos el caso clínico de una paciente de 4 años con diagnóstico prenatal de MAQP, no confirmado mediante radiografía de tóraxrealizada al nacimiento, lo cual retrasó el diagnóstico definitivo; fue intervenida con 4 años de edad tras haber presentado varias neumonías a repetición. Una radiografía de tórax normal realizada al nacimiento no descarta la presencia de estamalformación, por lo que es necesario realizar una tomografía computarizada a las 4 semanas del nacimiento para confirmar o descartar la MAQP. Una vez diagnosticada, el tratamiento quirúrgico debe ser precoz para evitar complicaciones.
Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications.
Subject(s)
Humans , Female , Child, Preschool , Diagnosis, Differential , Prenatal Diagnosis , Cystic Adenomatoid Malformation of Lung, Congenital/surgery , Cystic Adenomatoid Malformation of Lung, Congenital/diagnosis , Cystic Adenomatoid Malformation of Lung, Congenital , PneumoniaABSTRACT
Objective To explore the diagnostic value of ultrasonography of fetal congenital cystic adenomatoid malformation of the lung (CCAM), and to predict the prognosis according to ultrasonographic findings. Methods The chest of 19 fetus with CCAM was multi-sectionally scanned with two-dimensional ultrasonography. The position, appearance and size of mass were observed, and complications were continuously followed. Results CCAM was pathologically confirmed in 13 fetus after induced abortion. One neonatal died, while CCAM in other 5 fetus disappeared before 36 weeks. The mass of typeⅠCCAM became smaller and smaller, and eventually disappeared. The echo-free spaces in typeⅡbecame smaller and fewer, and the strong echo weakened to the same level as normal lung. For type Ⅲ, the echo of solid mass weakened to the same level as normal lung, or transformed to typeⅡ gradually, and finally recovered to normal echo of lung as the gestational age increased. During follow up, there were 10 fetus (10/19, 52.63%) that lung adenoma cyst increased with the gestational age increased, and the heart, mediastinal shifted, pleural effusion, and (or) fetal edema were observed. Conclusion Ultrasound examination is a reliable method for the diagnosis of CCAM, and enable to predict the prognosis of the affected fetuses. If heart and mediustinum displacement, pleural effusion, hydrops fetal or other abnormalities exist, it's necessary to terminate the pregnancy.