Intestinal intussusception and occlusion caused by small bowel polyps in the Peutz-Jeghers syndrome. Management by combined intraoperative enteroscopy and resection through minimal enterostomy: case report

A síndrome de Peutz-Jeghers é uma doença de caráter hereditário que freqüentemente requer intervenções endoscópicas e cirúrgicas repetidas, levando a complicações secundárias como, por exemplo, a síndrome do intestino curto. RELATO DE CASO: Relatamos neste artigo o caso de paciente de 15 anos, masculino, com história familiar para a doença, que foi submetido ao procedimento cirúrgico para tratar oclusão devido a intussucepção de intestino delgado. DISCUSSÃO: Associou-se método fibroscópico intra-operatório com a finalidade de detecção e ressecção de numerosos pólipos distribuídos em todo o intestino delgado. Realizaram-se enterotomias para a retirada dos pólipos maiores, restringindo-se, portanto, a ressecção intestinal a segmentos menores. A evolução clínica foi boa. CONCLUSÃO: Ressalta-se a importância do manejo de forma conservadora dos pacientes portadores desta síndrome, principalmente naqueles cujas manifestações clínicas de importância cirúrgica aparecem precocemente.The Peutz-Jeghers syndrome is a hereditary disease that requires frequent endoscopic and surgical intervention, leading to secondary complications such as short bowel syndrome. CASE REPORT: This paper reports on a 15-year-old male patient with a family history of the disease, who underwent surgery for treatment of an intestinal occlusion due to a small intestine intussusception. DISCUSSION: An intra-operative fiberscopic procedure was included for the detection and treatment of numerous polyps distributed along the small intestine. Enterotomy was performed to treat only the larger polyps, therefore limiting the intestinal resection to smaller segments. The postoperative follow-up was uneventful. CONCLUSION: We point out the importance of conservative treatment for patients with this syndrome, especially those who will undergo repeated surgical interventions because of clinical manifestation while they are still young

Multicentric pheochromocytoma and involvement of the inferior vena cava

CONTEXT: Extension of pheochromocytomas to the inferior vena cava is rare. Multicentric tumors are rare as well, being present in up to 10% of cases. Surgery is the treatment of choice because of the long-term survival free of disease. DESIGN: Case report. CASE REPORT: We report on a case of right adrenal pheochromocytoma with extension to the supra-diaphragmatic vena cava, which underwent surgical excision through thoracophrenic laparotomy without the need for cardiopulmonary bypass. In a 6-year follow-up, another pheochromocytoma was found in the infra-renal Zuckerkandl's organ. Complete surgical excision of the tumor was performed by a median laparotomy and complete retroperitoneal dissection. In both cases, the total removal of the pheochromocytoma has been guaranteed by having margins free of tumor and a normal post-operative level of catecholamines. The pathological study revealed a malignant pheochromocytoma with margins free of neoplasia in both specimens

Multicentric pheochromocytoma and involvement of the inferior vena cava

CONTEXT: Extension of pheochromocytomas to the inferior vena cava is rare. Multicentric tumors are rare as well, being present in up to 10% of cases. Surgery is the treatment of choice because of the long-term survival free of disease. DESIGN: Case report. CASE REPORT: We report on a case of right adrenal pheochromocytoma with extension to the supra-diaphragmatic vena cava, which underwent surgical excision through thoracophrenic laparotomy without the need for cardiopulmonary bypass. In a 6-year follow-up, another pheochromocytoma was found in the infra-renal Zuckerkandl's organ. Complete surgical excision of the tumor was performed by a median laparotomy and complete retroperitoneal dissection. In both cases, the total removal of the pheochromocytoma has been guaranteed by having margins free of tumor and a normal post-operative level of catecholamines. The pathological study revealed a malignant pheochromocytoma with margins free of neoplasia in both specimens