Thoracic Computed Tomography Scan and Bronchoscopy Appearance of Mounier-Kuhn Syndrome: A Case Report.

Mounier-Kuhn syndrome (MKS) is a rare congenital disease with an autosomal recessive inheritance pattern, characterized by an enlargement of the trachea and bronchi. MKS is secondary to a thinning of the muscular mucosa and atrophy of the longitudinal muscle and elastic fibers of the tracheobronchial tree. As a consequence, tracheal diverticulosis and dilatations in the posterior membranous wall appear, along with bronchiectasis that tend to be cystic in appearance. Overall, there is an impairment of mucocilliary clearance, with an ineffective cough, which predisposes the patient to recurrent lower respiratory tract infections. Clinical manifestations vary from asymptomatic to respiratory failure and death, most patients being diagnosed between the third and fourth decades of life. It is an often undiagnosed disease, with a diagnostic algorithm that includes the use of radiological techniques, alone or in combination with bronchoscopy. Specific diagnostic criteria have been developed, based on patients' tracheal and main bronchi diameter on chest X-ray and thoracic computed tomography scan. We present the case of a 45-year-old African American man who presented with a history of multiples episodes of pneumonia that required management in the intensive care unit, on whom MKS was diagnosed.

Tracheobronchopathia Osteochondroplastica-Clinical, Radiological, and Endoscopic Correlation: Case Series and Literature Review.

Tracheobronchopathia osteochondroplastica (TO) is a rare idiopathic and benign disease that is often underdiagnosed. TO is characterized by multiple submucosal cartilaginous and osseous tracheobronchial nodules that spare the posterior wall. It usually affects the elderly, developing when the person is around 60 years old without gender preference and has a reported incidence of 0.11%. TO can be symptomatic and should be considered in patients with chronic cough, dyspnea, and recurrent pulmonary infections. Diagnosis is usually incidental by computed tomography or bronchoscopy, the latter being the gold standard diagnostic test for TO. Many thoracic imagers are not well acquainted with TO; thus, these patients are often underdiagnosed or misdiagnosed. We came across 5 patients in our institution who were incidentally diagnosed with TO, inspiring us to review the available literature on this disease. A total of 33 patients diagnosed with TO between 2009 and 2019 were identified by our retrospective review. Clinical and imaging data were collected on these patients. We also included the clinical, radiological, and endoscopic data of our 5 cases. TO should be considered in patients with chronic cough, dyspnea, and recurrent pulmonary infections. Our experience is that both computed tomography and bronchoscopy can be used to make a reliable diagnosis. It is crucial for physicians, especially radiologists and pulmonologists, to be aware of the existence of TO in order to ensure proper diagnosis.

Coriocarcinoma metastásico posparto: presentación de un caso / Metastatic Postpartum Choriocarcinoma: Case report

Se informa el caso de una paciente de 18 años de edad, quien consultó por disnea súbita, asociado con dolor torácico y hemoptisis después de una cesárea. Se documentaron niveles elevados de BHCG (ß-Human Chorionic Gonadotrophin, por sus siglas del inglés) y se evidenciaron imágenes nodulares pulmonares, con áreas de sangrado sugestivas de lesiones metastásicas, asociado con hemotórax izquierdo en la escanografía de tórax. Así mismo, se observaron lesiones metastásicas similares en hígado, riñón izquierdo y sacro. Teniendo en cuenta la edad reproductiva, niveles de BHCG elevados y hallazgos clínicos, se consideró un coriocarcinoma manifiesto clínicamente en el puerperio. Se inició quimioterapia con mejoría clínica y descenso de niveles de BHCG.

We report a case of an 18 year old female patient, with sudden onset dyspnea, chest pain and hemoptysis after cesarean delivery. High BHCG blood levels (Beta-Human Chorionic Gonadotrophin) were documented, and multiple pulmonary nodal images were evidenced, with bleeding areas which suggest metastatic lesions, associated with the left hemithorax in the chest scan. Similar images were also observed in the liver, left kidney and sacrum. Because of childbearing age, high BHCG levels, and clinical features, it was considered a metastatic choriocarcinoma which was clinically manifested in the puerperium. Chemotherapy began, with clinical improvement and decreased BHCG levels.

Agenesia pulmonar / Pulmonary agenesis

La agenesia pulmonar es una alteración poco frecuente, con predominio en el sexo femenino y sin preferencia por la lateralidad. Reportamos el caso de un recién nacido masculino con diagnóstico prenatal de hernia diafragmática. Al nacer, se descartó esta patología y se hizo una impresión diagnóstica de malformación adenomatoide quística (malformación congénita de la vía aérea pulmonar) vs. agenesia pulmonar. El paciente falleció a los seis días de vida y la necropsia confirmó una agenesia pulmonar.

Pulmonary agenesis is an infrequent pathology which occurs predominantly among females with no lateral preference. We report on the case of a newborn male diagnosed with prenatal diaphragm hernia though at birth seemed more likely either to be a congenital cystic adenomatoid malformation (congenital pulmonary airway malformation) or pulmonary agenesis. The patient died six days after birth and necropsy confirmed pulmonary agenesis.

Carcinoma escamocelular de vesícula con infiltración al lecho hepático no diagnosticado prequirúrgicamente / Vesicle Scamocelular Carcinoma with Hepatic Bed Infiltration not diagnosed before Surgery

Los tumores malignos de la vesícula biliar son en su gran mayoría adenocarcinomas que, en una pequeña proporción, pueden presentar una diferenciación escamosa focal o extensa. Sin embargo, el carcinoma escamocelular primario como único componente se considera una neoplasia rara y surge en el terreno de un cambio metaplásico en el epitelio que reviste la vesícula biliar. El presente reporte de caso trata de una paciente de 69 años a quien se le practicó una colecistectomía por el diagnóstico prequirúrgico de colecistitis; durante el acto operatorio, se identificó una lesión tumoral de la vesícula biliar que infiltraba el quinto (V) segmento hepático, el cual se resecó en cuña. La histología correspondía a un carcinoma escamocelular de la vesícula biliar puro con infiltración al hígado. La paciente se recuperó satisfactoriamente del procedimiento practicado hace 5 meses.

Pseudoquiste pancreático en niño prematuro de 14 meses / Pancreatic Pseudocyst in a 14-month old Premature Baby

Se presenta el caso de un niño de 14 meses de edad a quien se le encontró una masa abdominal en el examen físico del control de crecimiento y desarrollo. El paciente fue el producto del tercer embarazo, complicado por corioamnionitis en la semana 32, por lo cual se practicó cesárea. Presentó neumonía intrauterina, que requirió hospitalización por un mes en la unidad neonatal, respiración mecánica durante una semana y múltiples transfusiones sanguíneas. Negaron antecedentes traumáticos u otros.