Congenital Cystic Adenomatoid Malformation Of Lung Research Articles

Application and effects of an early childhood education machine on analgesia and sedation in children after cardiothoracic surgery

ObjectiveTo study the effect of an early childhood education machine on sedation and analgesia in children after cardiothoracic surgery.MethodsA prospective randomized controlled study was conducted in a provincial hospital in China. Fifty-two patients (aged from 1 to 5 years) underwent cardiothoracic surgery (including: ventricular septal defect, patent ductus arteriosus, atrial septal defect, pulmonary stenosis, pulmonary sequestration and congenital cystic adenomatoid lung malformation) were divided into the study group (n = 26) and the control group (n = 26). The patients in the study group underwent intervention with an early childhood education machine (uniform type) in addition to routine standard treatment and nursing, while the patients in the control group only received routine standard treatment and nursing. Richmond agitation sedation score (RASS) and face, legs, activity, cry, consolability (FLACC) score of all of the patients were evaluated, and the negative emotions (self-rating anxiety scale (SAS) score and self-rating depression scale (SDS) score) of the parents of the two groups were compared.ResultsThere was no significant difference in the general clinical data between the two groups. The RASS and FLACC scores in the study group were significantly lower than those in the control group, and the SAS and SDS scores of the parents in the study group were significantly lower than those in the control group.ConclusionThe application of an early childhood education machine for children after cardiothoracic surgery can effectively reduce postoperative agitation, improve sedation and analgesia of the patients, and ease the pessimistic mood of the patients’ parents.

The First of Congenital Cystic Adenomatoid Lung Malformation in Iraq: A Radiologic and Pathologic Study

Background: Congenital cystic adenomatoid lung malformation is a very rare condition characterized by replacement of normal pulmonary tissue with cysts of variable size and distribution. The condition is usually unilateral. Very few cases of bilateral and congenital cystic adenomatoid malformation with good outcome after resections of the lesions have been reported, and most cases were stillborn or died early during life. Patients and methods: A forty-day male infant presented with progressive respiratory distress since the first week of life observed at the Children Teaching Hospital of Baghdad Medical City was studied. The literature was reviewed with aim of describing the early documentation of the condition in the literature. Results: Clinically, the infant had significant respiratory distress interfering with feeding and evidence of shift of the mediastinum to the right. Chest X ray showed increased translucency of left lung field and mediastinal shit to the right. CT-scan showed large air-filled cyst in the left lung and small air-filled cyst in the right lung. The child was treated successfully by left upper lobecomy. The gross examination of two gray pieces of lung tissue showed on cut section microcysts that were apparent grossly. Microscopical examination showed intercommunicating cysts lined by cuboidal epithelium with gland like appearance confirming the diagnosis of congenital cystic adenomatoid malformation of the lung. Conclusion: A very rare case of bilateral congenital cystic adenomatoid malformation of the lung is reported and the early documentation of the condition in the literature is described.

A case of congenital cystic adenomatoid malformation of lung with poor outcome

This article describes a case of ultrasound diagnosis of congenital cystic adenomatoid lung malformation (CCALM) of the right lung type I at 31+2 weeks of gestation associated with mediastinum displacement, nonimmune hydrops and massive polyhydramnios. The volume of amniotic liquid was approximately 4000 ml. The newborn’s weight was 2800 g, heart failure and lung disorders were revealed. The diagnosis of CCALM was confirmed by pathologoanatomic examination.

Congenital Cystic Adenomatoid Malformation with Associated Mucinous Bronchioloalveolar Carcinoma in a Neonate

Congenital cystic adenomatoid malformation (CCAM) of lung is a rare hamartomatous disorder characterized by abnormal branching morphogenesis of the lung. We report an unusual case of a 2-day-old male newborn with a pulmonary cystic lesion and lobectomy revealed a CCAM of the lung that has overlapping features of type 1 and type 2, complicating with multifocal mucinous bronchioloalveolar carcinoma (BAC). The case indicates that malignant transformation can occur in very early stage of the infancy in the patients with CCAM of lung.

A rare case report of mucoepidermoid carcinoma arising in congenital cystic adenomatoid malformation

We report a case of 19 years old male presenting with cough and hemoptysis of 10 days duration, chest x-ray showed left hilar enlargement. Computed Tomograpy Scan-Chest showed left hilar adenopathy with segmental collapse in left upper lobe. Fibreoptic bronchoscopy revealed growth partially occluding the left main bronchus. Endobronchial biopsy showed features suggestive of squamous cell carcinoma. Left pneumonectomy with lymph node dissection done. Histopathological examination showed features of Congenital Cystic Adenomatoid Malformation (CCAM) with focus of low grade mucoepidermoid carcinoma. The literature data confirm that CCAM can rarely undergo malignant transformation and because of the risk of malignant change, early removal of CCAM of lung is advisable.

Antenatal and postnatal management of congenital cystic adenomatoid lung malformation diagnosed by ultrasound and Magnetic Resonance Imaging (MRI)

Purpose: Antenatal diagnosis of congenital cystic adenomatoid lung malformation (CCAM) is vital for disease surveillance and postnatal care. Ultrasonography (US) has been the imaging gold standard for antenatal CCAM assessment. However, one of the limitations of US is the “vanishing phenomenon” caused by isoechogenicity of CCAM tissue and adjacent normal lung parenchyma. Methods: Antenatal serial US were concurrently used with magnetic resonance imaging (MRI) to monitor macro- and microcystic lesions. Results: In both pregnant women, antenatal US and MRI confirmed the presence, in the fetus, of cystic lesions and predicted disease regression/progression as well as the need for postnatal surgical intervention. Several advantages were detected by using both—serial US and MRI (over serial US alone)—including improved signal intensity, exact volume size measurements, precise CCAM location in particular for patients with adverse ultrasound conditions. Both neonates underwent surgical resection and had an uneventful post-operative course. Conclusions: Antenatal use of MRI as well as serial US improved information regarding tissue resolution and delineation of CCAM. The information from two imaging modalities was complementary. Our literature review confirmed the emerging role of prenatal MRI for postnatal monitoring and management of CCAM.

Congenital Cystic Adenomatoid Malformation: Is There a Need for Pregnancy Termination?

Aim. Congenital cystic adenomatoid lung malformation is a rare unilateral dysplasia of the lung. Three pathologic types are described in the literature: type I with cysts >2 cm, type II with cysts <1 cm, and type III with microcysts. The aim of this paper is to present a case of a fetus with congenital cystic adenomatoid lung malformation and discuss the necessity for pregnancy termination according to its prognosis and future mortality. Case. A 36-year-old pregnant woman (para: 1, gravida: 1) presented in our department for anatomy ultrasound screening at 20 + 1 weeks of gestation. The ultrasound detected a cystic adenomatoid right lung malformation measuring 1.45 × 1.67 cm which caused mediastinal shift of the heart and the lung to the left side. Other findings were cysts of the choroid plexus and echogenic intracardiac foci. The parents after genetic counseling decided pregnancy termination. The pregnant received cabergoline for ablactation. Conclusion. Congenital cystic adenomatoid lung malformation has different prognosis according to the type (69% in type I, 0% in types II and III). Fetal hydrops, cardiac and skeletal anomalies, Potter's syndrome, and gastrointestinal atresia are common cofindings. Genetic counseling is necessary, and pregnancy termination is proposed to the cases with poor prognosis.

P30.06: Prenatal and postnatal management of congenital cystic adenomatoid lung malformation diagnosed by ultrasound and MR imaging: two case reports

P30.06: Prenatal and postnatal management of congenital cystic adenomatoid lung malformation diagnosed by ultrasound and MR imaging: two case reports

Complex extralobar sequestration in a 24-year-old woman

Extralobar sequestration is a congenital acquired disease more frequently observed after birth or during infancy. In half of the cases, it is associated with another malformation. In the following case we report the observation of a 24-year-old female with right extralobar sequestration associated with a diaphragmatic hernia and containing gastric mucosa and a congenital cystic adenomatoid lung malformation. Such malformative lung tissue is known to potentially degenerate and justifies surgery even when patients are asymptomatic.

The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis.

The aim of this study was to evaluate the role of MR imaging of the fetus to improve sonographic prenatal diagnosis of congenital anomalies. In 40 fetuses (not consecutive cases) with an abnormality diagnosed with ultrasound, additional MR imaging was performed. The basic sequence was a T2-weighted single-shot half Fourier (HASTE) technique. Head, neck, spinal, thoracic, urogenital, and abdominal fetal pathologies were found. This retrospective, observational study compared MR imaging findings with ultrasonographic findings regarding detection, topography, and etiology of the pathology. The MR findings were evaluated as superior, equal to, or inferior compared with US, in consent with the referring gynecologists. The role of these findings in relation to pregnancy management was studied and compared with postnatal follow-up in 30 of 40 babies. Fetal MRI technique was successful in 36 of 39 examinations and provided additional information in 21 of 40 fetuses (one twin pregnancy with two members to evaluate). More precise anatomy and location of fetal pathology (20 of 40 cases) and additional etiologic information (8 of 40 cases) were substantial advantages in cerebrospinal abnormalities [ventriculomegaly, encephalocele, vein of Galen malformation, callosal malformations, meningo(myelo)cele], in retroperitoneal abnormalities (lymphangioma, renal agenesis, multicystic renal dysplasia), and in neck/thoracic pathology [cervical cystic teratoma, congenital hernia diaphragmatica, congenital cystic adenomatoid lung malformation (CCAM)]. This improved parental counseling and pregnancy management in 15 pregnancies. In 3 cases, prenatal MRI findings did not correlate with prenatal ultrasonographic findings or neonatal diagnosis. The MRI provided a more detailed description and insight into fetal anatomy, pathology, and etiology in the vast majority of these selected cases. This improved prenatal parental counseling and postnatal therapeutic planning.

Outcome of the Prenatally Diagnosed Congenital Cystic Adenomatoid Lung Malformation: A Canadian Experience

Congenital cystic adenomatoid malformation of the lung (CCAM) is diagnosed by prenatal ultrasonography with an increasing frequency but controversy persists as to its prognosis and prenatal management. Method: A multi-institutional study of cases of CCAM diagnosed antenatally identified by ultrasonographers and by a review of hospital charts. Results: We obtained 48 cases from five centers. We estimate the incidence of CCAM at 1:25,000 to 1:35,000 pregnancies. The incidence of voluntary abortions was 15% (7/48), of spontaneous abortions 2% (1/41) and of postnatal death 10% (4/40). One of the postnatal deaths was from trisomy 18. Of the 7 aborted fetuses, 2 had multiple malformations and 1 had severe hydrops and oligohydramnios; the other 4 had a large mass with mediastinal displacement but without hydrops. When pregnancy was allowed to continue, 56% of the lesions regressed spontaneously, even though one third of these had initial progression. In 17 cases (42%) the mediastinal shift corrected itself, sometimes by simple growth of the fetus but most often by a decrease in the size of the lung mass. In 1 fetus, repeated needle decompressions followed by double-pigtail catheter drainage of large cysts allowed regression of hydrops. Despite this, neonatal death occurred from pulmonary hypoplasia. Conclusion: CCAM can lead to fetal or neonatal demise from hydrops, lung hypoplasia, prematurity or severe associated malformations, but has a good prognosis in the majority of cases.