Mediastinal and hilar soft tissue mass-like lesions in congenital unilateral pulmonary vein atresia: A retrospective review of seven pediatric patients.

BACKGROUND: Congenital unilateral pulmonary vein atresia (CUPVA) is known to lead to the formation of an abnormal confluent mediastinal and hilar soft tissue mass, thoracic hypoplasia, and interlobular septal thickening on the affected side. The purpose of the present study is to investigate the frequency and severity of mediastinal soft tissue mass-like lesions and examine other abnormal findings associated with CUPVA. METHODS: We retrospectively reviewed seven children with CUPVA who underwent contrast-enhanced CT scans and measured the soft tissue mass volume in the bilateral mediastinum (affected and normal side). The location of abnormal soft tissue was divided into three anatomical sections (paratracheal, peribronchial, and the dorsal aspect of the left atrium). The relationships among soft tissue volume and anatomical section were statistically evaluated. Also, the presence of thoracic hypoplasia, small ipsilateral pulmonary arteries, interlobular septal thickening, and ground-glass opacities were investigated. RESULTS: In all cases, CT scans confirmed the presence of confluent soft tissue mass-like lesions in the affected mediastinum. The soft tissue volume on the affected side was 5.5-fold greater than the volume on the normal side (average: 18.0 cm3 and 4.25 cm3 respectively, P < 0.01). Thoracic hypoplasia and interlobular septal thickening were found in all patients. Small pulmonary arteries and ground-glass opacities were present in six of the seven patients. CONCLUSION: Abnormal mediastinal and hilar soft tissue is commonly found in patients with CUPVA. So, if we encounter the mediastinal soft tissue mass in patients with CUPVA, no further test will be indicated.

Refractory hypercalcemia due to an ectopic mediastinal parathyroid gland in a hemodialysis patient: a case report.

BACKGROUND: Hypercalcemia crisis is a complex disorder rarely induced by tertiary hyperparathyroidism, which clinically presents as nonsuppressible parathyroid hyperplasia with persistent increased PTH levels and hypercalcemia. It is one of the major risk factors of morbidity and mortality in end-stage renal disease. Parathyroidectomy should be in consideration in dialysis patients with severe hyperparathyroidism who are refractory to medical therapy. The implications and consequences of it, however, are not fully understood. CASE PRESENTATION: We present a case of a 70 year-old man disturbed by gastrointestinal manifestations due to hypercalcaemic crisis. The patient had longstanding hypercalcaemia and hyperparathyrodism refractory to calcimimetics, calcitonin, hormone and haemodialysis. A ectopic parathyroid gland in anterior mediastinum was found and elucidated by Tc-99 m scan. Futhermore, a video-assisted thoracoscopic parathyroidectomy was performed. Histologically, the tumour consisted of densely arranged chief cells immunohistochemically positive for PTH antigens. Consequently, calcium and parathormone were declining stably without any complications. CONCLUSIONS: On account of refractory hypercalcemia and hyperparathyroidism, radionuclide scanning is useful in the diagnosis of ectopic parathyroid gland. it is of great significance for multidisciplinary therapy combing anesthesia, surgical, endocrinology and nephrology staff.

Postpartum Diagnosis of Cardiac Paraganglioma: A Case Report.

BACKGROUND: Extra-adrenal pheochromocytomas, or paragangliomas, originate from neural crest chromaffin cells and can be found anywhere along the sympathetic chain from head to toe. CASE REPORT: A 34-year-old female presented 4 days postpartum with episodes of palpitations, hypertension, and shortness of breath. Two episodes in the emergency department confirmed hypertension and supraventricular tachycardia (SVT). A mediastinal mass was noted during workup for pulmonary embolus and was subsequently diagnosed as a cardiac paraganglioma. Our patient underwent surgical resection and was doing well 3 months postoperatively. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case represents a rare presentation of mediastinal paraganglioma with episodic SVT and hypertension postpartum, diagnosed during workup for pulmonary embolus. Although exceedingly rare, emergency physicians should consider paragangliomas in the differential of pregnant or postpartum women who present with episodic hypertension, palpitations, headache, and sweating.

A deep azygoesophageal recess may increase the risk of secondary spontaneous pneumothorax.

PURPOSE: The azygoesophageal recess (AER) is known as a possible cause of bulla formation in patients with spontaneous pneumothorax. However, there has been little focus on the depth of the AER. We evaluated the relationship between the depth of the AER and pneumothorax development. METHODS: We conducted a retrospective study of 80 spontaneous pneumothorax patients who underwent surgery at our institution. We evaluated the depth of the AER on preoperative computed tomography scans. RESULTS: Ruptured bullae at the AER were found in 12 patients (52.2%) with secondary spontaneous pneumothorax (SSP) and 8 patients (14.0%) with primary spontaneous pneumothorax (PSP) (p < 0.001). In patients with ruptured bullae at the AER, 10 SSP patients (83.3%) had a deep AER while only 2 PSP patients (25%) had a deep AER (p = 0.015). CONCLUSIONS: A deep AER was more frequently associated with SSP than with PSP. A deep AER may contributes to bulla formation and rupture in SSP patients.

[Diagnostics and treatment of congenital malformations of the lung and mediastinum in newborn children and infants].

The article presents the experience of treatment of newborn children and infants with congenital malformations of the lung and mediastinum, which required a surgery. Children (138 cases) were treated during recent 18 years. There was a prevalence of full-term infants (73%). Fetal malformations were diagnosed in prenatal period in majority of cases. Computed tomography was the main method of diagnostics after delivery. Children (110 cases) were operated out of 138. Children with extrapulmonary sequestration didn't undergo surgery in case of absence of clinical manifestations. The authors made a conclusion that malformations of the lung and mediastinum should be included in number of differentiated diseases in case of respiratory distress syndrome in newborn children. The indications to early surgery should be the danger of contamination and malignant transformation, presence of intrathoracic tension syndrome in neonatal period.

A semi-quantitative approach to variation of the azygos vein course.

The azygos vein (AV) is typically described (illustrated) as ascending vertically on the right of thoracic vertebrae. Most thoracic vein studies have focused on tributary patterns, but some have noted more leftward AV courses. This study statistically documents variation in AV course independent of tributary patterns. A more statistical approach to the probable position of AV at different vertical levels may aid clinicians in locating and assessing it in clinical contexts. The AV course was exposed in 84 cadavers by removing overlying viscera between the aortic hiatus and tracheal bifurcation. Subjectively, non-pathological specimens were digitally photographed in anterior view. For each photo, a scaled grid was used to mark the horizontal position of the AV center at each of five vertical levels. The summated numerical distributions showed the following: ∼5% of the AVs ascended on the right side (classical) position, ∼30% did not cross the midline, ∼70% included part or all of their course left of the midline, and ∼14% reached the extreme left side. Based on this data, the modal AV course (1) begins at, or to the right of, the midline, (2) deviates leftward, (3) crosses the midline below mid-level, (4) reaches a leftward maximum at about 3/5 of its course, (5) then deviates rightward (often only reaching the midline at the uppermost level). In several noticeable cases, the leftward maximum was associated with large connections to left-side veins, suggesting a possible tension mechanism exerting traction on the AV over time.

Mediastinal foregut duplication cyst of enteric type containing a persistent thymus, imitating a pericardial cyst.

UNLABELLED: Mediastinal foregut duplication cysts are rare congenital masses caused by developmental disorders of the anterior part of the embryonic primitive gut. In adults they can be discovered as an incidental finding on chest radiograph. They can mimic other intrathoracic pathologies as it was initially the case in our patient. A 51-year-old woman was incidentally found to have homogenous mass at the right cardiophrenic angle on the chest radiograph. Based on that finding and echocardiography a pericardial cyst was suspected. Computed tomography demonstrated a simple anterior mediastinal cyst. Because of the growth with a mild progression of the compression of the right atrium, the cyst was resected. Histology and immunohistochemistry revealed the mass to be a mediastinal foregut duplicatory cyst of enteric type containing persistent thymus. To our knowledge, this is the first report in which a mediastinal foregut cyst contained both enteric mucosa and ectopic persistent thymus (Fig. 9, Ref. 13). KEYWORDS: foregut cyst, mediastinal cyst, thymus, enteric cyst, pericardial cyst.

Cardiothoracic imaging findings of Proteus syndrome.

In this work, we sought to delineate the prevalence of cardiothoracic imaging findings of Proteus syndrome in a large cohort at our institution. Of 53 individuals with a confirmed diagnosis of Proteus syndrome at our institution from 10/2001 to 10/2019, 38 individuals (men, n = 23; average age = 24 years) underwent cardiothoracic imaging (routine chest CT, CT pulmonary angiography and/or cardiac MRI). All studies were retrospectively and independently reviewed by two fellowship-trained cardiothoracic readers. Disagreements were resolved by consensus. Differences between variables were analyzed via parametric and nonparametric tests based on the normality of the distribution. The cardiothoracic findings of Proteus syndrome were diverse, but several were much more common and included: scoliosis from bony overgrowth (94%), pulmonary venous dilation (62%), band-like areas of lung scarring (56%), and hyperlucent lung parenchyma (50%). In addition, of 20 individuals who underwent cardiac MRI, 9/20 (45%) had intramyocardial fat, mostly involving the endocardial surface of the left ventricular septal wall. There was no statistically significant difference among the functional cardiac parameters between individuals with and without intramyocardial fat. Only one individual with intramyocardial fat had mildly decreased function (LVEF = 53%), while all others had normal ejection fraction.

Complex split cord malformation with mediastinal extension of a teratoma and simultaneous ventral and dorsal bony spur splitting the cord.

INTRODUCTION: Association of split cord malformation with teratoma is an extremely rare condition. It is very unusual to find split cord malformation with teratomas extending into the posterior mediastinum and still rarer to find bony spur arising dorsally and ventrally at the same level. CASE REPORT: The authors describe the case of a 2-year-old child who presented with dorsal kyphosis and a dermal sinus. The neurological examination was unremarkable. MRI and noncontrast CT of the spine revealed Pang's type I split cord malformation at the D9-12 level. Bony spur was seen arising both dorsally and ventrally at the D9-12 level. Intraoperatively, intradural teratoma was found coming extradurally both posteriorly between the 2 spurs and anteriorly into the posterior mediastinum above the bony spurs through a defect in the vertebral body of 2 cm diameter. Near total excision was performed. DISCUSSION: To the best of the authors' knowledge, this is the second case report of an intradural teratoma extending into the mediastinum, occurring with split cord malformations and other spinal anomalies. A similar case was reported from our institute in 2006. This is the first report of its kind of simultaneous dorsal and ventral bony spur at the same level.

Measurement of the great vessels in the mediastinum could help distinguish true from false-positive coarctation of the aorta in the third trimester.

OBJECTIVE: We investigated the utility of analyzing prenatal mediastinal measures of the great arteries in distinguishing true coarctation of the aorta (CoA) from false-positive CoA. METHODS: All fetuses in this study had suspicion of CoA based on the presence of right-left heart disproportion. We defined 3 study groups: group 1, true fetal CoA; group 2, false-positive fetal CoA with a narrow aortic arch; and group 3, false-positive fetal CoA without a narrow aortic arch. RESULTS: In group 1, the mean mediastinal pulmonary artery (PA) to ascending aorta (Ao) diameter ratio +/- SD was 2.03 +/- 0.48, and in group 2, the ratio was 1.60 +/- 0.23. The difference was statistically significant (P = .0018, t test). In group 3, the mean PA:Ao ratio was 1.35 +/- 0.14. The difference between groups 1 and 3 was statistically significant (P = .0002, t test). In our study group, for a PA:Ao ratio of 1.60, sensitivity was 83.0%; specificity, 85.0%; positive predictive value, 62.5%; and negative predictive value, 94.0%. CONCLUSIONS: In the third trimester, the main PA:Ao ratio as measured in the fetal mediastinum can be a helpful tool in distinguishing true CoA requiring neonatal cardiac surgery from false-positive CoA and simple disproportion requiring medical attention but no surgery in the first month of postnatal life.

Split notochord syndrome with congenital unilateral Horner's sign.

A 2-year-old boy exhibited congenital right Horner's sign and right finger, wrist, and elbow flexion arthrogryposis. He had dyspnea and feeding difficulty 12 hours after birth. Radiologic examination revealed a thoracoabdominal intestinal tube and mediastinal cystic lesion at the right side, with vertebral anomaly at the cervical level. Histopathologically, the intestinal tube was diagnosed as bowel duplication. Because the mediastinal lesion could not be resected surgically, no histopathological diagnosis was made. Embryologically, the combination of transdiaphragmatic duplication, mediastinal cystic lesion, anterior spina bifida, and hemivertebra suggested notochord malformation. The diagnosis was split notochord syndrome, an extremely rare embryological malformation syndrome. Congenital unilateral Horner syndrome often has unknown etiology. In this case, cervical vertebral anomalies and mediastinal cystic lesion implied a compressed nerve root, resulting in Horner syndrome and right finger, wrist, and elbow flexion joint contracture. Split notochord syndrome should be included in differential diagnosis of congenital unilateral Horner syndrome.

The usefulness of a trans-illuminated introducer during the Nuss procedure.

Background There has been an increase in the number of patients undergoing the Nuss procedure for cosmetic purposes, thus increasing the need for safer surgery. However, there are reports of massive hemorrhage and organ damage during the Nuss procedure which involves dissection of the anterior mediastinum. We have developed the trans-illuminated introducer that allows safe surgery while maintaining a small surgical incision of less than 1 cm. Methods This study was a retrospective review of 306 patients aged 3-40 years who underwent the Nuss procedure using the trans-illuminated introducer at our hospital between April 2006 and December 2014. Results There were 29 (9.5%) early postoperative complications. The most common early complication was pneumothorax (15 cases, 4.9%). Five (1.6%) patients developed hemothorax in the early postoperative period, which occurred independently of the dissection process of the anterior mediastinum. None of these patients required reoperation or blood transfusion. There were no complications caused by the introducer during dissection of the anterior mediastinum. Conclusions Using the trans-illuminated introducer, we were able to dissect the anterior mediastinum without a major complication, such as massive hemorrhage from the mediastinum, while maintaining a small surgical incision for cosmetic purposes. Therefore, we consider that the trans-illuminated introducer is useful for improving the outcome of the Nuss procedure.

Giant retrocardiac foregut duplication cyst presenting with left atrial compression and palpitations.

A 35-year-old man presented with a 3-month history of palpitations and shortness of breath. An ECG showed premature atrial contractions and episodes of supraventricular tachycardia. A subsequent echocardiogram showed a retrocardiac cystic mass that was compressing the left atrium. A CT scan confirmed these findings by showing a large left-sided posterior mediastinal cyst compressing the left atrium and pulmonary veins. The cyst was successfully excised from the retrocardiac position via left thoracotomy after which there was complete resolution of the palpitations. Histopathology showed it to be a mediastinal cyst, most likely a foregut duplication of the enterogenic variant. This is an extremely unusual case of foregut duplication cyst presenting with compression of the left atrium and pulmonary veins leading to atrial arrhythmias.

Associated malformations and chromosomal anomalies in 42 cases of prenatally diagnosed diaphragmatic hernia.

We present a retrospective study of the frequency and type of associated malformations and chromosomal anomalies in 42 consecutive cases of congenital diaphragmatic hernia (CDH) diagnosed in utero during the period from 1985 to 1999. In 26% (11/42) of the cases, associated malformations were detected. Chromosomal anomalies were present in 9.5% (4/42). In this group of 15 cases (15/42 = 36%) with associated malformations or chromosomal anomalies, all cases, except one, had prenatal sonographic evidence of additional problems. The survival rate of fetuses with CDH and associated malformations or chromosomal anomalies was poor (1/15). Therefore, the overall survival rate of in utero-diagnosed CDH was only 31% (13/42), while isolated left CDH had a survival rate of 52% (12/23). The in utero diagnosis of CDH implies a detailed echographic examination to exclude additional anomalies. The risk for a syndromal or chromosomal malformation becomes small when no additional anomalies are seen on ultrasound.

Mediastinal window: a cause of simultaneous bilateral spontaneous pneumothorax.

A case of bilateral spontaneous pneumothorax with critical collapse of both lungs in a previously healthy 19-year-old woman is described. A congenital defect of mediastinal septum was suspected on the basis of roentgenographic findings and confirmed by right-sided thoracotomy. This mediastinal window between the pleural spaces allowed air leaking from a ruptured right lung apical bulla to collapse not only the right lung but also the left. Apical TA stapler resection and right-sided parietal pleurectomy was performed to prevent recurrence.

A unique congenital mediastinal malformation.

We report a case of a 35-year-old patient presenting with a unique asymptomatic malformation associating extralobar pulmonary sequestration communicating with a bronchogenic cyst of the esophageal wall via the aortopulmonary window, dextroisomerism, and complete agenesia of the left pericardium. Despite computed tomography (CT) scan and magnetic resonance imaging (MRI), the diagnosis could not be established before left thoracotomy. The sequestrated lobe and bronchogenic cyst were then successfully resected.

Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology.

Congenital anomalies of the chest are an important cause of morbidity in infants, children, and even adults. The evaluation of affected patients frequently requires multiple imaging modalities to diagnose the anomaly and plan surgical correction. The authors analyze and illustrate practical aspects of certain common and uncommon congenital anomalies affecting the tracheobronchial tree, lung, and mediastinum, with emphasis on radiologic manifestations. Other thoracic anomalies such as rib anomalies and vascular rings are discussed when they are associated with anomalies of the tracheobronchial tree. The usefulness of the various imaging modalities in the diagnosis and treatment of these conditions is also evaluated. Specific topics addressed include tracheal conditions such as tracheal stenosis, tracheomalacia, tracheal bronchus, tracheal atresia, and bronchogenic cyst; anomalies of the lung such as lung underdevelopment (agenesis and hypoplasia), scimitar syndrome, congenital cystic adenomatoid malformation, congenital lobar emphysema, and pulmonary sequestration; esophageal anomalies such as esophageal atresia, tracheoesophageal fistula, and esophageal duplications; and vascular rings. The embryologic and pathologic basis of the radiologic findings are discussed in appropriate cases. Differential diagnoses, as well as pitfalls and diagnostic difficulties, are included.