RESUMO
INTRODUCTION: Systemic scleroderma is an autoimmune disease that can affect the respiratory system and the gastrointestinal tract. When diffuse lung disease and pulmonary hypertension develop, a lung transplant is usually considered as treatment. This option, however, is not feasible in the presence of concomitant gastroesophageal reflux disease. In this case, medical therapy is initially warranted. If this fails, surgical approach may be considered in order for the patient to be a lung transplant candidate. CASE PRESENTATION: A 56-year-old female, with previous history of intestinal pneumonitis, mild pulmonary hypertension and gastroesophageal reflux secondary to systemic scleroderma, is considered for lung transplant. Initially, due to persistent gastroesophageal reflux, a transplant was not a viable. This was corrected with an open gastrectomy with roux-en-Y anastomosis. Follow-up one week later revealed normal anatomy, adequate esophageal-jejunal anastomosis, and adequate contrast medium transit via esophagogram. Additionally, there was no evidence of contrast medium reflux indicating a resolved gastroesophageal reflux disease. This led to the patient becoming a candidate for lung transplant. DISCUSSION: We suggest an open gastrectomy with roux-en-Y anastomosis as an alternative to the Nissen fundoplication for patients with connective tissue disease that develop terminal pulmonary consequences and require a lung transplant.
RESUMO
Los paragangliomas son tumores derivados de las células cromafines de la cresta neural y por ello tienen la capacidad de secretar catecolaminas, hormonas y péptidos; al ser tumores extraadrenales del sistema nervioso central se los puede encontrar en la base del cráneo, el cuello, el tórax y el abdomen; se clasifican en funcionales y no funcionales. En este artículo se describe un paciente de sexo masculino que cursó inicialmente con dolor inespecífico en el hipocondrio derecho; con base en los estudios imaginológicos iniciales se sospechó la presencia de un neuroblastoma, pero no fue posible diferenciarlo de un paraganglioma, un ganglioneuroblastoma o un neurofibroblastoma. Se lo intervino quirúrgicamente y el estudio patológico del espécimen reveló áreas hemorrágicas extensas, compatibles con un paraganglioma extraadrenal no funcional. Estos tumores son infrecuentes, de localización diversa y de tratamiento quirúrgico difícil.
Paragangliomas are tumors derived from chromaffin cells from the neural crest. They are able to secrete catecholamines, hormones and peptides. They can be found in the skull base, neck, thorax and abdomen, and may be functional or not-functional. We report the case of a male patient with non-specific pain in the right hypochondrium. Based on the initial imaginological studies a neuroblastoma was suspected, but it not possible to differentiate it from a paraganglioma, a ganglioneuroblastoma or a neurofibroblastoma. The pathological study of the surgical specimen revealed extensive hemorrhagic areas, consistent with a non-functional extra-adrenal paraganglioma. This is an infrequent neoplasia with difficult surgical treatment.