Abualrub Ahmad M, Malhes Waleed M, Shehadeh Mohammad H, Omari Fadi H, Tuqan Anas R, Ishtawi Shaima, Hindi Tareq
Department of Medicine, Al-Quds University, Jerusalem, PSE.
Department of Pediatrics, Palestine Medical Complex, Ramallah, PSE.
Cureus. 2024 Apr 13;16(4):e58180. doi: 10.7759/cureus.58180. eCollection 2024 Apr.
Renal lymphangiomatosis is a rare congenital condition characterized by the abnormal development of lymphatic channels in the kidney, resulting in cystic dilatations. While more commonly observed in children, it can occur in adults but is extremely rare. Clinical manifestations range from asymptomatic cases to symptoms such as abdominal pain, hypertension, and renal dysfunction. In this case report, we present a rare case of bilateral renal lymphangiomatosis in an eight-year-old male with high blood pressure. Renal ultrasound revealed bilateral kidney enlargement and perinephric hypoechoic collections with septations consistent with lymphangiomatosis. The diagnosis was confirmed through CT imaging which shows bilateral non-enhancing perinephric collections. As a part of the patient's management plan, bilateral perinephric cystic lesions were successfully aspirated without complications. In conclusion, accurate diagnosis is crucial for appropriate management decisions, and treatment primarily focuses on conservative measures to manage associated hypertension, reduce lymphatic fluid accumulation, and alleviate pain, reserving invasive interventions for severe cases or complications.
肾淋巴管瘤病是一种罕见的先天性疾病,其特征是肾脏淋巴管发育异常,导致囊性扩张。虽然该病在儿童中更常见,但也可发生于成人,不过极为罕见。临床表现从无症状到腹痛、高血压和肾功能不全等症状不等。在本病例报告中,我们呈现了一例8岁男性双侧肾淋巴管瘤病伴高血压的罕见病例。肾脏超声显示双侧肾脏增大,肾周低回声液性区伴分隔,符合淋巴管瘤病表现。通过CT成像确诊,CT显示双侧肾周无强化液性区。作为患者管理计划的一部分,成功抽吸双侧肾周囊性病变,无并发症发生。总之,准确诊断对于做出恰当的管理决策至关重要,治疗主要侧重于采取保守措施来控制相关高血压、减少淋巴液积聚并缓解疼痛,对于重症病例或并发症则采用侵入性干预措施。