1 - Serviço de Cirurgia Pediátrica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
2 - Serviço de Cirurgia Geral, IPO, Lisboa
- Poster em Sala no XVI SPCMIN Congress, Évora
The mediastinum is the most common location of chest masses in the pediatric population. Mediastinal masses may be nonvascular or vascular masses and represent congenital anomalies, infections, benign and malignant neoplasms, and pseudomasses. Imaging plays a crucial role in making the diagnosis and developing a treatment plan. Mediastinal masses are placed in one of three mediastinal compartments (anterior, middle, posterior) on the basis of the lateral chest radiograph. Further characterization can be made with ultrasound, CT, and MRI. Combining the characteristic imaging appearances with clinical information (age, physical examination findings, and laboratory analysis) often can provide a probable diagnosis. We present a case of a 17 year old female with a past medical history of neurofibromatosis type 1 (familiar form) with multiple plexiform neurofibromas dispersed through the skin. She presented to the emergency department with complaints of severe posterior thoracic pain. The initial investigation consisted of blood work and EKG with no alterations, and chest radiography with an enlarged mediastinum. A CT-scan as ordered and a large cystic mass was detected in the anterior/medium mediastinum. For a better characterization of the lesion, a thoracic MRI was performed, and detect a cystic mediastinal lesion in direct contact with the entirety of the ascending aorta and aortic arch, prolonging itself to the descending aorta and through the aorto-pulmonary mediastinal window. The image was consistent with a resectable lesion, probably a neurofibroma and possibly a thoracic paraganglioneuroma. At the time of diagnosis, MEK inhibitors were initiated for a possible mass reduction and to achieve a diminished hemorrhagic risk. After the initial treatment with no apparent volume reduction, the patient was submitted to a complete thoracoscopic excision of the mass, with no pos-operative complications, being discharged after 3 days of the resection. Anatomo Pathological findings were consistent with neurofibroma. In conclusion, imaging of mediastinal masses can establish a probability of success of minimally invasive treatment of large mediastinal masses in children.
Palavras Chave: neurofibromatose, quisto mediastinico, toracoscopia