Vitor Sofia, Oliveira Ferreira Alexandre, Lopes João, Velosa José
Gastrenterologia, Centro Hospitalar Lisboa Norte, Portugal.
Gastrenterologia, Centro Hospitalar do Algarve - Portimão, Portugal.
Rev Esp Enferm Dig. 2016 Aug;108(8):500-1.
We present the case of an 18-year-old male patient that was referred to our gastrenterology department with history of intermittent painless hematochezia since childhood. During such instances, he was diagnosed with bowel intussusception, eosinophilic gastroenteritis and inflammatory bowel disease at 4, 6 and 8 years old, respectively. He underwent treatment with 5-aminosalicylic acid for two years, without improvement of symptoms. He was then lost to follow-up until our observation. His physical examination was unremarkable except for digital rectal examination which found a nodular compressible mass by the palpating finger. Blood tests revealed a mild iron deficiency anemia. The colonoscopy showed an extended reddish and bluish multinodular submucosal mass in the rectum, suggesting diffuse cavernous hemangioma of the rectum (DHCR). The Magnetic Resonance Imaging, showed diffuse thickening of the entire rectum extending into the distal sigmoid with the mesorectum revealing multiple serpiginous structures, corresponding to abnormal blood vessels. After discussion, we considered to perform a sphincter-sparing procedure, namely pull through transection and coloanal anastomosis. However, intervention was ruled out by the patient because of his fear of anal incontinence and permanent colostomy. We adopted a conservative strategy with clinical surveillance and iron supplementation. At the present, the patient remains with intermittent rectal bleeding, referring poor quality of life due to his ongoing symptoms. This is a rare case of DHCR. Despite of being a benign disease, the management of DHCR requires a sphincter mucosectomy and pull-through coloanal sleeve anastomosis which has become the first-line procedure. The surgical outcomes are non-expectable in 32% with permanent sphincter lesion or with incomplete DHCR removal. As in this case, the surgeons or patients refusal to perform the intervention is common which represents a challenge to the clinical follow-up.
我们报告一例18岁男性患者，该患者自童年起就有间歇性无痛便血病史，转诊至我院胃肠病科。在这些情况下，他分别在4岁、6岁和8岁时被诊断为肠套叠、嗜酸性胃肠炎和炎症性肠病。他接受了两年的5-氨基水杨酸治疗，但症状没有改善。此后他失访，直到我们对其进行观察。他的体格检查无异常，除了直肠指检发现可触及的结节状肿块。血液检查显示轻度缺铁性贫血。结肠镜检查显示直肠有一个延伸的红蓝色多结节黏膜下肿块，提示直肠弥漫性海绵状血管瘤（DHCR）。磁共振成像显示整个直肠弥漫性增厚，延伸至乙状结肠远端，直肠系膜可见多个蜿蜒的结构，对应异常血管。经过讨论，我们考虑进行保留括约肌的手术，即拖出式横断和结肠肛管吻合术。然而，由于患者担心肛门失禁和永久性结肠造口术，排除了干预措施。我们采取了临床监测和补充铁剂的保守策略。目前，患者仍有间歇性直肠出血，由于持续的症状，生活质量较差。这是一例罕见的DHCR病例。尽管是良性疾病，但DHCR的治疗需要进行括约肌黏膜切除术和拖出式结肠肛管套叠吻合术，这已成为一线手术。32%的患者会出现永久性括约肌损伤或DHCR切除不完全，手术效果不理想。在这种情况下，外科医生或患者拒绝进行干预很常见，这对临床随访构成了挑战。
