盆腔脂肪增多症1例报告及文献复习_1

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1、从本学科出发,应着重选对国民经济具有一定实用价值和理论意义的课题。课题具有先进性,便于研究生提出新见解,特别是博士生必须有创新性的成果盆腔脂肪增多症1例报告及文献复习 作者:赵振蒙,李 凌,马鸿钧,冯 波,董泽泉,申吉泓 【摘要】 目的 提高盆腔脂肪增多症的认识。 方法 盆腔脂肪增多症1例,男,32岁。影像学检查:B超及IVU示双肾中度积水,双侧输尿管全程扩张。后尿道延长,膀胱呈“竖直灯泡”状改变。结肠气钡双重造影:乙状结肠及直肠上段向后受压变细。CT示:盆腔内均匀低密度脂肪堆积。双肾盂、肾盏及输尿管扩张明显,前列腺位置抬高。尿动力学检查:最大尿流率/s,排尿量114ml,残余尿300ml。膀

2、胱测压;膀胱顺应性正常,非抑制性收缩波较多,逼尿肌功能正常。尿道压正常。采用硬膜外麻醉下剔除膀胱及输尿管周围脂肪组织,松解输尿管下段及双侧输尿管置管方法 治疗 。结合 文献 复习讨论盆腔脂肪增多症的特点。 结果 硬膜外麻醉下先拟行双侧输尿管置管,进镜后见精阜与膀胱颈间距10cm,内有多个息肉样物,未找到输尿管开口。退镜后改平卧位,探查见盆腔内膀胱及乙状结肠周围充满大量脂肪组织,与影像学表现一致。剔除膀胱及输尿管周围多余脂肪组织。同时行双侧输尿管松解。打开膀胱,找到双侧输尿管开口,分别插入8F单“J”管。输尿管管口周围有多个息肉,约1cm1cm大小,分别电灼之。术后病理报告:盆腔增生纤维,脂肪组

3、织中原壁血管增生。术后1周下床活动,4周拔除双侧单“J”管。分别于术后一周和3个月复查B超和IVU,双肾盂积水和双侧输尿管扩张逐渐减轻。 结论 B超、X线、CT为本病的主要诊断依据。开放手术剔除膀胱及输尿管周围脂肪,同时行双侧输尿管松解及置管术是治疗本病的有效方法。 【关键词】 盆腔脂肪增多症;手术 【Abstract】 Objective To improve the awareness of pelvic lipomatosis in clinical practice. Methods A2-year-old male patient with pelvic lipomatosis wa

4、s admitted. Imaging studies: The re were mid-degree of hydronephrosis both side on B ultrasonography and IVU, and a typical“vertical bulb”shape of bladder and extended posterior were also seen on IVU. The sigmoid colon and upper rectum were pressed backward and narrowed on colic photography. CT scan

5、 showed that low density lipid piled in the pelvic cavity. The bilateral renal pelvix and ureter were markedly dilated, the prostate gland was lifted up. Urodynamic examination: The max flow /s, urine volume 114ml, residual bladder was with increased non-inhibited contract wave, normal resilience an

6、d dribbling muscle. The urethral pressure was normal. The lesions in the pelvic cavity and around the ureters were removed and the lower segments of the ureters were loosened under extradural-anesthesia, after which double J tube were placed into the ureters. The clinical characteristics of lipomato

7、sis were reviewed by combination with the literature. Results Under extradural-anesthesia, the cystoscope was placed into the bladder, the seminal colliculus and bladder neck were seen with thEir distance larger than 10 cm. There were multiple polypoid tissue in the bladder. The bilateral catheteriz

8、ation of ureters can not be carried out because of no finding of the ureter orifice. After taking out of the cystoscope, the patient was taken to decubitus position. Intraoperatively, apparently increased lipid tissue was found in the pelvic cavity and there was large amount of lipid tissue filling

9、the space around bladder, rectum and sigmoid colon, which was consistent with the imaging study findings. Superfluous lipid tissue around the bladder and ureter was removed, synchronously the lower ureters were loosened. Single J tubes (8F) were placed into bilateral ureters. The polyp around the ur

10、eter orifice which were about 1cm1 cm were electronically cauterized. Pathologic study: hyperplastic fibrous tissue in pelvic cavity, Hyperplastic capillary vessel in lipid tissue. The patient was discharged 1 week after surgery, and the single J tubes were pulled out after more weeks. Hydronephrosi

11、s and dilation of ureters were gradually relieved according to B ultrasonography and IVU done after 1 week and months. Conclusion B ultrasonography, X-ray and CT scan are the most valuable examination in diagnosis of lipomatosis. Removing the lipid in the pelvis and around the bladder and loosening

12、the ureters, then ureters catheterization by opening operation are effective treatment of pelvic lipomatosis. 【Key words】 pelvic lipomatosis; surgery 盆腔脂肪增多症为一种病因未明的良性罕见病。1959年Engels首次描述此病,Fogg和Smyth于1968年正式命名此症,定义为直肠与膀胱周围盆腔空间内正常脂肪组织的过度增生1。XX年8月我们收治1例,采用盆腔及输尿管周围脂肪剔除及双侧输尿管置管术,疗效满意。结合文献复习讨论。现报告如下。 1 临

13、床资料 病例资料 患者,男,32岁。因“反复腰背痛伴尿频,尿急5年,大便变细2年”于XX年8月18日入院。2年前因“肾结石”服中药后排出结石一枚。查体:血压120/80mmHg,身高175cm,体重75kg。下腹饱满,腹软,未触及包块。生化检查肾功能正常。影像学检查:B超可见双肾中度积水,双侧输尿管全程扩张,最大直径。KUB加IVU:双肾中度积水,双输尿管全程扩张。CT:盆腔内均匀低密度脂肪堆积。双肾盂、肾盏及输尿管扩张明显,前列腺位置抬高。膀胱镜不能置入膀胱。膀胱尿道造影:尿道注入造影剂,见后尿道延长。膀胱显影,呈“竖直灯泡”状改变,密度均匀,边缘光滑,未见充盈缺损。排尿观察:膀胱收缩良好,

14、膀胱颈部呈梭形改变,上抬受压。结肠气钡双重造影:乙状结肠及直肠上段向后受压变细,管壁光滑,黏膜连续,未见破坏,中断。 尿动力学检查:最大尿流率/s,排尿量114ml,残余尿300ml。膀胱测压;膀胱顺应性正常,非抑制性收缩波较多,逼尿肌功能正常。尿道压正常。 1. 治疗方法及结果 硬膜外麻醉下先拟行双侧输尿管置管,进镜后见精阜与膀胱颈间距大于10cm,内有多个息肉样物,膀胱黏膜光滑,未找到输尿管开口。退镜后改平卧位,下腹正中切口,探查见膀胱明显抬高,膀胱周围脂肪较多,切除膀胱前及双侧后壁脂肪,于右侧找到扩张的右输尿管,向下分离并剔除输尿管周围多余的脂肪直至近膀胱入口处。同法剔除左侧输尿管及周围

15、的多余脂肪。剔除过程中见脂肪组织较硬,血运丰富,纤维较多,粘连。打开膀胱,未见双侧输尿管开口,但见尿液经膀胱颈口内流出,切开颈口找到双侧输尿管开口,分别插入8F单“J”管。管口周围有多个息肉,均1cm大小,分别电灼之。术后病理报告:盆腔增生纤维,脂肪组织中原壁血管增生。患者无不适症状, 目前 仍在随访中。 讨论 盆腔脂肪增多症是一种大量脂肪组织增生堆积于盆腔,挤压、包绕压迫及牵引局部脏器,使器官变形狭窄或移位,从而产生以泌尿系及下消化道症状为主的良性病变。 .1 流行病学及病因 本病临床罕见,发病年龄多数在2060岁之间2。病因不明,有学者认为与慢性泌尿系统感染所致的盆腔炎症、激素代谢紊乱、先

16、天性静脉血管异常有关2。有学者认为本病是肥胖的局部表现3。一组51例患者资料显示65%的患者有不同程度的肥胖,29%根本没有肥胖,6%为瘦弱2。也有学者认为本病与肥胖无关2。Battista4等动物实验证明带有截短的HMGI-C基因的转基因大鼠表现为一种以腹部或盆腔脂肪增多症占优势的巨大表型,认为可能与HMGI-C基因有关;在人类包括脂肪瘤的各种良性间质肿瘤发现了12号染色体上的HMGI-C基因发生易位4,5。Tong等6报道越南籍兄弟2人均患有盆腔脂肪增多症。国内报道1例合并先天性隐睾、多发性肠源囊肿3。Kume等7报道软骨发育不全并发盆腔脂肪增多症,而软骨发育不全属先天性遗传性疾病。因此,

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