Thoracic Abnormalities
Asim Kurjak in CRC Handbook of Ultrasound in Obstetrics and Gynecology, 2019
Congenital cystic adenomatoid malformation of the lung is the most frequent congenital lung abnormality. The disease is caused by an excessive growth of terminal bronchioles and is usually unilateral. According to the morphological appearance of the lesions, the disease is classified in three types.2 Type I is characterized by the presence of large pulmonary cysts. In type II, the cysts are much smaller, being less than 2 cm in size, while in type III, the cystic dilatation of the bronchioles is too small to be visualized by ultrasound. Helpful signs to detect type III disease are mediastinal shift and increased echogenicity of the lungs.3,4
Teratoma, rhabdomyosarcoma and other tumours
Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven in Succeeding in Paediatric Surgery Examinations, 2017
From the statements above, choose the most appropriate diagnosis and treatment for the following cancer scenarios. The same statement can be used more than once to answer any of the questions. A 6-year-old girl presented with vomiting and lethargy. MRI with gadolinium shows a large posterior fossa tumour based in the cerebellum. The patient has hydrocephalus. What is the most likely diagnosis?A 4-year-old boy presents with headaches and vomiting. MRI with gadolinium shows a heterogeneous lesion in the fourth ventricle. What is the most likely diagnosis?A 3-year-old with headache, vomiting and gait ataxia shows on MRI a fourth ventricle–occupying lesion extruding out of the foramen of Luschka into the cerebellopontine angle and encasing the posterior fossa cranial nerves. What is the treatment of choice?A 6-year-old boy with wheezing and cough had been treated as an asthmatic with recurrent episodes of pneumonia. He presented acutely with haemoptysis. CT scan demonstrated a mediastinal and lung mass and distal bronchiectasis. What is the most likely diagnosis?A 4-year-old who had been followed for right lower lobe congenital cystic adenomatoid malformation presents with purulent cough, haemoptysis and fever. CT scan demonstrated a mass in the right lower lobe with mediastinal lymphadenopathy. What is the treatment of choice?
The thorax
Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague in Paediatric Surgical Diagnosis, 2018
Previously called congenital cystic adenomatoid malformation, this is the most common congenital lung abnormality. It is characterised by cystic change at the level of the terminal bronchioles; macrocystic, microcystic (solid) and mixed varieties are recognised. The right or left lower lobe is most often affected. Some lesions are ‘hybrid’, having features of both cystic disease and pulmonary sequestration. Affected patients may present with respiratory distress, infection in the cyst, recurrent chest infections or as an incidental finding.
Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report
Published in Acta Chirurgica Belgica, 2021
A. E. Frick, H. Decaluwé, B. Weynand, M. Proesmans, D. Van Raemdonck
The development of a congenital parenchymatous lung disorder, known as congenital pulmonary airway malformation (CPAM), has previously been described as congenital cystic adenomatoid malformation (CCAM) with an estimated incidence at 1:25,000–1:35,000 births. The classification by Stocker et al. in 1977 originally described 3 different subtypes and was later expanded and renamed into 5 subtypes on the basis of clinical and pathological features [1–3]. Most congenital CPAMs are uncommon and primarily identified in infants and in adults. Depending on the type of CPAM, clinical presentation and prognosis are different. Typical symptoms are recurrent pulmonary infection, productive cough, and hemoptysis. Some patients remain asymptomatic [4]. A number of reports have been published describing the association of adenocarcinoma with type 1 CPAM. The occurrence of rhabdomyosarcoma or pleuropulmonary blastoma arising in a CPAM has also been rarely documented [5,6].
Effect of maternal betamethasone on hydrops fetalis caused by extralobar pulmonary sequestration: a case report
Published in Journal of Obstetrics and Gynaecology, 2019
Cécile Guenot, Karine Dubrit, Karine Lepigeon, Eric Giannoni, David Baud, Yvan Vial
The patient presented with spontaneous labour at 35 + 5 WG and delivered a boy weighing 2570 g, 50 cm, with an Apgar score 8/10/10 at 1, 5 and 10 minutes, pH 7.33/7.41. The chest radiography and ultrasound of the newborn on day 1 confirmed a left pulmonary mass. He rapidly developed a pleural effusion treated with a pleural catheter. Dexamethasone was initiated for one week, with a transient regression of effusion, but it reappeared one week after the end of the treatment. A thoracotomy and segmentar pulmonary resection was realised at one month of life. The post-operative follow-up was uneventful and the patient was discharged home at two months of life. The anatomo-pathological examination confirmed the prenatal diagnosis of BPS. There was no histologic or vascular argument for a congenital cystic adenomatoid malformation (CCAM).
Pleuropulmonary blastoma: Difficulty in diagnosis and treatment of a case in Vietnam
Published in Pediatric Hematology and Oncology, 2021
Bui Ngoc Lan, Le Thi Kim Ngoc, Hoang Ngoc Thach, Phan Canh Duy
Pleuropulmonary blastoma (PPB) is a very rare malignant disease in childhood cancer. The tumor is derived from the pleura or lungs. This primary tumor occurs mainly in children under 6 years old.1 Diagnosis of this tumor is difficult both by imaging and by pathology because of its similarity to other diseases. PPB is classified into three groups. Type I (cystic form) is similar to congenital cystic adenomatoid malformation (CCAM).2 This type is commonly seen in infants and has a more favorable prognosis than type II and III. Type I can be treated successfully with surgical resection, however it can relapse and require neoadjuvant chemotherapy. In contrast, type II (mixed form) or type III (solid form) are aggressive diseases with poor prognosis. Type II and III PPB have metastatic potential to the brain, the bone, and in some cases, to the liver. Both these types need multidisciplinary therapy including surgical resection, intensive chemotherapy and radiation if it is available.3 Effective treatment plans are aggressive and require hospitals with the capability to perform all the above. The international PPB protocol was developed in developed countries and application in developing countries is challenged by lack access to required resources.
Related Knowledge Centers
- Birth Defect
- Cyst
- Stenosis
- Ultrasound
- Cyanosis
- Pulmonary Sequestration
- Pneumothorax
- Prenatal Development
- Echogenicity
- Heart Failure