Clinicopathological features of resected pediatric congenital lung and mediastinal lesions: A single institution experience.

OBJECTIVES: To evaluate resected congenital lung and mediastinal lesions among children and their characteristics in a single tertiary hospital. METHODS: A retrospective chart review analysis of all patients under 14 years of age who underwent congenital lung and mediastinal lesion resection in a single tertiary center from June 1997 to June 2018 was performed. RESULTS: In total, 108 cases of resected lung and mediastinal lesions were performed from June 1997 to June 2018. Congenital lung and mediastinal lesions were found in 52 (48%) cases. Overall, 23 cases were males (44%) and 29 (56%) were females. The most common histopathology was congenital lobar emphysema. CONCLUSION: This study provides a 21-year review of the clinical and histopathological features of resected congenital lung and mediastinal lesions in a single center. Congenital lung and mediastinal lesions represented 48% of all resected lesions.

Foregut Duplication Cysts in Children.

BACKGROUND AND OBJECTIVES: Duplications of the alimentary tract are rare anomalies. We report our experience with foregut duplication cysts including their clinical presentation, diagnostic modalities, and surgical management. METHODS: We report a 20-year retrospective review of all foregut duplication cysts managed at our institution. RESULTS: Twelve patients with 13 foregut duplication cysts were identified. The ages of the children at the time of surgery ranged from infancy to adolescence, with a mean age of 7.2 years. Half of the patients presented with abdominal pain and vomiting, and the remaining either had respiratory distress or were asymptomatic. All resections were performed electively. Two of the 11 patients had other congenital anomalies, including a congenital pulmonary airway malformation and coarctation of the aorta. One patient had prenatal diagnosis by ultrasonography. Nine patients underwent complete successful excision with no complications. Three patients whose symptoms resolved during hospitalization remained under observation because of parental preference. CONCLUSIONS: Foregut malformation in children may present with a variety of symptoms or can be found incidentally. The decision and timing of surgery is based on the clinical presentation. Surgical intervention in asymptomatic patients should be based on a thorough discussion with the parents.

A congenital foregut malformation with a laryngeal bronchus.

We present the case of a 2-month-old boy who required intubation at birth for stridor, at which point a supraglottic cyst was noted. With recurrence of the cyst, a subsequent computed tomographic scan and magnetic resonance imaging demonstrated a mass encroaching on the larynx, descending into the mediastinum, and encasing the major vessels. A median sternotomy and transcervical incision enabled excision of a well-defined mass with a laryngeal attachment. This attachment was consistent with a bronchus on histopathologic investigation. To our knowledge, this is the first reported case of ectopic lung tissue arising from the larynx and descending into the mediastinum.

The tale of spring water cysts: a historical outline of surgery for congenital pericardial diverticula and cysts.

Congenital pericardial diverticula and cysts are extremely uncommon lesions within the anterior mediastinum. Both lesions derive from the pericardial celom and represent different stages of a common embryogenesis. Initial reports date from the 19th century. Surgical pioneers were Otto Pickhardt, who removed a pericardial cyst at Lenox Hill Hospital in New York in 1931, and Richard Sweet, who accomplished the first resection of a pericardial diverticulum at Massachusetts General Hospital in Boston in 1943. These lesions were also called spring water cysts because they usually contain watery, crystal-clear fluid. This history outlines the milestones of evolving surgical management, from the first report in 1837 up to the present time.

Congenital intrathoracic kidney in a patient undergoing coronary artery bypass surgery.

Intrathoracic kidney is a rare congenital anomaly. It appears as a posterior mediastinal mass on chest X-ray. Most cases are asymptomatic and are discovered accidentally. We present a 48-year-old male patient with intrathoracic kidney discovered during routine investigation for coronary artery bypass surgery.

Age-related changes in CT attenuation of the thymus in children.

BACKGROUND: The CT appearance of the normal and abnormal thymus and its age-related changes have been described. However, there is no information regarding the change in thymus CT attenuation values in children. OBJECTIVE: The aim of the present study was to evaluate the normal CT attenuation of the thymus in infants and children and discover when the decline begins. MATERIALS AND METHODS: CT attenuation values of the thymus were retrospectively evaluated in 152 children between ages of 1 day and up to 14 years. For each patient the mean value of the CT thymus attenuation was calculated and compared to CT attenuation of the chest wall and cardiac muscles. We also examined the correlation between thymic attenuation, gender, and disease. The statistical analysis used was multivariate linear regression. RESULTS: CT attenuation of the thymus declines with age, beginning only after the 1st year of life. The thymus is more hyperdense than the chest wall and cardiac muscles in infants less than 1 year. The thymus is denser in males than females. In malignant conditions, in infants less than 1 year, thymus CT attenuation is decreased. In older children thymus attenuation was similar to that for the chest wall and cardiac muscles. CONCLUSION: The decline in thymic CT attenuation with age is consistent with fatty infiltration of the gland. The measurements given in this report can serve as a basis for comparison to determine whether the thymus of a young child is normal or pathological.

Congenital mediastinal bronchogenic cyst with malignant transformation: an autopsy report.

A rare autopsy case of mediastinal bronchogenic cyst with malignant transformation is presented. The cyst had been located in the anterior mediastinum for at least 28 years in a 52 year old male. Chest X-ray findings showing rapid enlargement of the cyst and biopsy of the spine for lumbago made a clinical diagnosis as suspicious mediastinal cystic teratoma with malignant transformation metastasizing to the spine. Postmortem examination revealed that the cyst was located in the anterior mediastinum extending to the left pulmonary hilum and had no connection with the tracheobronchial tree. The cyst wall consisted of bronchus-like tissue including ciliated epithelium, hyaline cartilage, smooth muscle and mucoserous glands. There were no teratomatous components in the wall. Malignant tumor predominantly consisting of round cells occurred in the thickened cyst wall and grew into the cyst cavity with direct invasion of the lung and metastases to the liver, adrenal glands, bone marrow of the lumbar spine and lymph nodes. An immunohistochemical study showed that the tumor cells frequently expressed cytokeratin, epithelial membrane antigen and carcino-embryonic antigen, occasionally CA19-9, vimentin and neuron-specific enolase. From these findings, the tumor was diagnosed as undifferentiated carcinoma arising in the mediastinal bronchogenic cyst.

Mediastinal arteriovenous fistulae in the adult: case report and review of the literature.

An adult with an asymptomatic mediastinal arterio-venous fistula is presented. The diagnosis was established using angiography and oximetry after noninvasive imaging failed to identify the source of a continuous murmur. The literature is reviewed.

Case report: congenital mediastinal arteriovenous fistula in an adult--diagnosis with digital subtraction angiography.

A case of asymptomatic congenital mediastinal arteriovenous fistula detected in an adult patient is presented. The diagnosis was suspected clinically and confirmed with intravenous digital subtraction angiography. The clinical and radiological features are discussed and the literature reviewed.

Congenital left superior vena cava with absent right superior vena cava: a cause of progressive mediastinal widening.

We wish to report a patient with the rare anomaly of congenital left superior vena cava and absent right superior vena cava. This became apparent on chest radiographs by the development of a left mediastinal "mass" over a one-year period. The role of contrast-enhanced computed tomographic scanning in the detection of venous anomalies is stressed.