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Abstract：
Retroperitoneal fibrosis (RPF), also referred to as Ormond\'s disease, is a rare fibroinflammatory condition characterized by abnormal fibrous tissue deposition in the retroperitoneal space, which traditionally presents with ureteral obstruction. Nonetheless, our case report showcases an exceptional instance involving a 70-year-old female patient who presented with symptoms suggestive of colonic obstruction, an unusual presentation that is not commonly associated with RPF. Although RPF has established associations with autoimmune conditions such as immunoglobulin G4-related disease and systemic lupus erythematosus, its connection to colonic obstruction remains undocumented in the medical literature. Our patient is a 70-year-old female who presented with constipation, anemia, and fecal occult blood. Her past medical history included a hysterectomy due to fibroids, right breast lumpectomy, type 2 diabetes mellitus, subclinical hyperthyroidism, hypertension, and obesity. Upon physical examination, the patient\'s abdomen appeared protuberant but was non-tender to palpation. Bowel sounds were normal, and there was no distension. Notably, there was no tenderness in the right or left costovertebral angles, nor was there any guarding. Workup with colonoscopy could not be completed due to the inability to pass a colonoscope beyond the rectosigmoid junction. Further workup with barium enema confirmed an apple core lesion seen in the rectosigmoid concerning for a neoplastic or inflammatory process. Finally, a computed tomography scan of the abdomen and pelvis showed a 7.1 cm right pelvic mass attached to the bladder and cecum, moderate right hydroureteronephrosis, and a 5.2 cm left adnexal mass with soft tissue changes narrowing the sigmoid colon. The next step was to take the patient for an exploratory laparotomy. During exploratory laparotomy, extensive adhesions and desmoplastic reactions were observed in the pelvic region, involving the sigmoid colon, bladder, cecum, and appendix. Two firm masses were identified in the retroperitoneum, one located in the left lower quadrant (LLQ) adherent to the posterior wall of the sigmoid colon and one in the right lower quadrant (RLQ) adherent to the posterior wall of the cecum. Three specimens were sent to pathology for further examination: a portion of the sigmoid colon, a resection from the RLQ mass, and a resection from the LLQ mass. Pathology reported dense fibrotic masses with abscess-like formation, reactive in nature and of unclear etiology, and negative for malignancy. They were negative for fibromatosis (β-catenin negative), and IgG4+/IgG+ was approximately 5%. Interestingly, the LLQ mass also contained remnants of the fallopian tube and ovary and benign cystic changes. This case report presents a unique and atypical presentation of RPF, deviating from the conventional presentation of ureteral obstruction. The patient\'s initial symptoms suggested colonic obstruction, a clinical scenario rarely linked to RPF. This case underscores the significance of considering diverse clinical presentations when diagnosing RPF, thereby expanding our comprehension of the condition\'s clinical spectrum and ultimately refining patient care and management.
摘要：
腹膜后纤维化(RPF),也被称为奥蒙德病，是一种罕见的纤维炎症性疾病，其特征是腹膜后间隙中异常的纤维组织沉积，传统上表现为输尿管梗阻。尽管如此，我们的病例报告显示了一例例外情况，涉及一名70岁的女性患者，其症状提示为结肠梗阻，一种不常见的演示文稿，通常与RPF无关。尽管RPF已与免疫球蛋白G4相关疾病和系统性红斑狼疮等自身免疫性疾病建立了联系，它与结肠梗阻的联系在医学文献中仍未被证实。我们的病人是一位70岁的女性，她出现了便秘，贫血,和粪便潜血.她过去的病史包括子宫肌瘤切除术，右乳房肿块切除术,2型糖尿病,亚临床甲状腺功能亢进,高血压,和肥胖。在体检时,患者的腹部出现隆起，但触诊不嫩。肠音正常,也没有扩张.值得注意的是,右或左脊柱角没有压痛，也没有守卫。由于结肠镜无法通过直肠乙状结肠交界处以外的结肠镜检查无法完成。钡灌肠的进一步检查证实，在直肠乙状结肠中看到的苹果核心病变涉及肿瘤或炎症过程。最后,腹部和骨盆的计算机断层扫描显示，膀胱和盲肠有7.1厘米的右骨盆肿块，中度右输尿管肾积水,左附件肿块5.2cm伴软组织改变，乙状结肠变窄。下一步是带患者进行剖腹探查。在剖腹探查术中,在骨盆区域观察到广泛的粘连和促纤维化反应，涉及乙状结肠，膀胱,盲肠,和附录。在腹膜后发现了两个坚固的肿块，一个位于左下象限（LLQ），粘附于乙状结肠后壁，一个位于右下象限（RLQ），粘附于盲肠后壁。将三个标本送去病理学进一步检查：乙状结肠的一部分，从RLQ肿块切除,和从LLQ肿块切除。病理报告致密纤维化肿块伴脓肿样形成，反应性质和病因不明，恶性肿瘤阴性。纤维瘤病阴性（β-连环蛋白阴性），IgG4+/IgG+约为5%。有趣的是,LLQ肿块还包含输卵管和卵巢的残留物以及良性囊性改变。本病例报告提供了RPF的独特和非典型表现，与输尿管梗阻的常规表现不同。病人最初的症状提示结肠梗阻,很少与RPF相关的临床情景。该病例强调了在诊断RPF时考虑不同临床表现的重要性，从而扩大我们对病情的临床范围的理解，并最终完善患者的护理和管理。
