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.

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.

Massive hiatus hernia associated with scoliosis in a female cadaver

Kyphoscoliosis is often associated with enlarged oesophageal hiatus. It is suggested that this spinal deformity leads to prolonged stretching of the diaphragm causing herniation of abdominal organs, particularly the stomach into the thorax. Such a hiatal hernia can compress thoracic viscera, produce symptoms like shortness of breath and intermittent pain after ingestion of food. This cadaveric report discusses a case of a giant, sliding-type of hiatal hernia accompanied by scoliosis with rightsided deviation of the thoracic aorta and smallsized left lung. All patients presenting gastrointestinal symptoms with coexistence of vertebral column anomalies should be evaluated for the occurrence of intra-thoracic stomach

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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.

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.

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.

Hemitórax opaco / Opaque hemithorax

Se presenta un paciente adulto de 58 años de edad, quien acudió a consulta por síntomas inespecíficos, relacionados con infección respiratoria, remitido desde el área de salud con una radiografía de tórax representativa de un hemitórax opaco que fue interpretada como neumonía lobar. Se realizaron diversas técnicas de imagen que incluyeron tomografía axial computarizada, angiografía pulmonar y angiotomografía que permitieron efectuar el diagnóstico diferencial de hemitórax opaco y definitivo de hipoplasia pulmonar, así como descartar anomalías asociadas. Los estudios imagenológicos permiten realizar el diagnóstico diferencial de hemitórax opaco y definitivo de hipoplasia pulmonar, así como las anomalías vasculares anexas, lo que posibilita tomar conducta y seguimiento médico adecuados.

A male 58-year-old patient presents with non-specific symptoms related to respiratory infection. The patient had been referred from his health area with a chest radiograph showing an opaque hemithorax which was interpreted as lobar pneumonia. Various imaging techniques were performed, including computerized axial tomography, pulmonary angiography and angiotomography, allowing to make the differential diagnosis of opaque hemithorax and the definitive diagnosis of pulmonary hypoplasia, and rule out associated anomalies. Imaging studies make it possible to make a differential diagnosis of opaque hemithorax and a definitive diagnosis of pulmonary hypoplasia, as well as related vascular anomalies, allowing the application of adequate clinical management and follow-up.

Hemitórax opaco / Opaque hemithorax

Se presenta un paciente adulto de 58 años de edad, quien acudió a consulta por síntomas inespecíficos, relacionados con infección respiratoria, remitido desde el área de salud con una radiografía de tórax representativa de un hemitórax opaco que fue interpretada como neumonía lobar. Se realizaron diversas técnicas de imagen que incluyeron tomografía axial computarizada, angiografía pulmonar y angiotomografía que permitieron efectuar el diagnóstico diferencial de hemitórax opaco y definitivo de hipoplasia pulmonar, así como descartar anomalías asociadas. Los estudios imagenológicos permiten realizar el diagnóstico diferencial de hemitórax opaco y definitivo de hipoplasia pulmonar, así como las anomalías vasculares anexas, lo que posibilita tomar conducta y seguimiento médico adecuados(AU)

A male 58-year-old patient presents with non-specific symptoms related to respiratory infection. The patient had been referred from his health area with a chest radiograph showing an opaque hemithorax which was interpreted as lobar pneumonia. Various imaging techniques were performed, including computerized axial tomography, pulmonary angiography and angiotomography, allowing to make the differential diagnosis of opaque hemithorax and the definitive diagnosis of pulmonary hypoplasia, and rule out associated anomalies. Imaging studies make it possible to make a differential diagnosis of opaque hemithorax and a definitive diagnosis of pulmonary hypoplasia, as well as related vascular anomalies, allowing the application of adequate clinical management and follow-up(AU)

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.

The surgical management and outcome of congenital mediastinal malformations.

We reviewed our institutional experience with congenital mediastinal masses and compared the postnatal management and outcome of patients with or without prenatal diagnosis. Between January 1997 and August 2011, 24 patients underwent surgical procedures for congenital mediastinal mass. For eight patients, the mass was detected by prenatal ultrasonography at 27 weeks of gestation (range 22-35). Postnatal management consisted in open surgery for seven patients at a mean age of 9 months (range 1 day-20 months) and sclerotherapy for one lymphangioma at 5 months of life. Sixteen patients had postnatal diagnosis at 137 months (±194) of median age. Eight bronchogenic cysts, seven bronchopulmonary foregut malformations, five teratomas, three lymphangiomas and one haemangioma were operated on. The median age at resection was 28 months (1 day-15 years). There were four emergency procedures and no surgical mortality. The median follow-up was 45 months (3-144). The duration of mechanical ventilation and hospital stay was, respectively, 4.6 h and 7.5 days for antenatal patients and 24.3 h and 14.3 days for postnatal diagnosed patients. Prenatal diagnosis allows early management of congenital mediastinal malformations. Early resection can be performed prior to the occurrence of symptoms ∼1 year of life and is associated with an excellent outcome and less morbidity.

[Pulmonary sequestration and enteric cyst, 2 expressions of the same abnormality]. / Séquestration pulmonaire et kyste entérique, 2 expressions d'une même anomalie. Illustration par un cas.

We report the case of an 18-month-old boy operated on for a right lower lobe bronchopulmonary sequestration. At the immediate postoperative check-up, a septic right thoracic effusion appeared, connected to a cystic mediastinal retrocrural formation on CT. After a drainage attempt and medical therapy, the abdominal lesion was resected. Histological examination showed that the cyst cavity was lined with pseudostratified non-ciliated epithelium, without cartilage, consistent with an enteric cyst. Regardless of the embryological theory, a literature review confirmed that in presence of one of these two lesions, one should systematically look for the other.