泌尿 Unilateral renal cystic disease DDX

Except for its unilateral localization, the gross and histological findings of URCD 单侧多囊肾病 are indistinguishable from those of autosomal dominant polycystic kidney disease 常染色体显性多囊肾病 (ADPKD)However, unlike ADPKD, URCD patients show neither a genetic background nor progressive deterioration in renal function.

Besides ADPKD, multilocular cystic renal neoplasms, cystic dysplasia and multiple simple cysts are other categories of cystic diseases.

Multilocular cystic renal neoplasms 多房囊性肾瘤 usually form discrete, encapsulated masses that are well demarcated from the adjacent renal parenchyma. Also, a cystic neoplasm grows slowly and expands to displace normal renal parenchyma and does not contain the islands of enhancing parenchyma on CT, which are found in URCD.

A multicystic dysplastic kidney 多囊性肾发育不良 is usually diffusely cystic, severely dysplastic and non-functioning due to ureteropelvic occlusion. It is usually easily distinguished from URCD with sonography, CT and radionuclide studies. The collecting system draining the dysplastic segment is atretic or obstructed and, therefore, is not usually opacified on contrast-enhanced CT, whereas the collecting system in URCD shows only the displacement.

Multiple simple cysts may be difficult to distinguish from URCD when confined to one kidney. However, they are not as numerous as in URCD.

http://ndt.oxfordjournals.org/cgi/content/full/14/8/1999

五官 Glomus tympanicum 鼓室球瘤

Glomus tympanicum is a benign hypervascular tumor that arises from glomus bodies (neural crest tissue).

Glomus bodies in the middle ear are situated along Jacobson's nerve (a branch of CN IX，鼓室神经) which forms the tympanic plexus. Branches of the tympanic plexus (and potential locations for glomus tympanicum tumors) occur on the cochlear promontory, near the round window, eustachian tube egress, tensor tympani tendon and along the inferior tympanic canaliculus. The classic location is the cochlear promontory.

On CT, the tumors are typically small, do not erode the bone (as opposed to cholesteatomas). On MR they demonstrate marked enhancement. Larger tumors may have a 'salt and pepper' appearance caused by flow voids in the mass.

Glomus tumors occur (in order of frequency) at the jugular foramen, branches of the vagus nerve (Arnold's nerve), carotid bulb or hypotympanum.

Overall glomus tumors are multiple in 15% of patients.

http://education.auntminnie.com/QMachine.ASP?UID=25I0TGNP&PageId=5&Sess=4437525

骨骼 多发性基底细胞痣综合征

痣样基底细胞瘤综合征（nevoid basal cell carcinoma syndrome）又称颌骨囊肿-基底细胞痣-肋骨分叉综合征或Gorlin综合征。1951年Binkley和Johnson首先叙述了此综合征，1960年Gorlin和Goltz进行了详细讨论。此综合征比较复杂，包含多种异常表现，主要有：①多发性牙源性角化囊肿；②多发性皮肤痣样基底细胞癌；③骨骼异常，特别是肋骨（常为分叉）和脊椎骨异常；④额部和颞顶部隆起，眶距过宽和轻度下颌前凸，形成特征性面部表现；⑤钙、磷代谢异常，表现有脑膜钙化和服用甲状旁腺素之后缺乏磷酸盐尿的排出。痣样基底细胞癌患者较年轻，常有家族史，具有常染色体显性遗传特征(fig.16=肋骨分叉；fig.17=颌骨囊肿伴子囊；fig.18=唇基底细胞癌)。

多发性基底细胞痣综合征　为家族性，属于常染色体显性遗传病。其特征是：多发性基底细胞痣或基底细胞癌、颌骨多发性囊肿、肋骨畸形、颅内钙化。临床表现为：①颌骨牙源性角化囊肿（下颌骨多于上颌骨，单发或多发，常为双颌同时累及）；②痣样基底细胞癌（主要发生于面部、颈部、躯干上部、眶周、眼睑、鼻、颧突等部位；上唇为面部最常发部位，一般为单侧。多数病损处于静止状态）；③肋骨畸形（包括分叉肋、融合肋、肋骨发育不全或部分缺失）；④颅内钙化（最常见为大脑镰钙化，其次为小脑幕钙化）。

与常染色体显性遗传有关，外显率高达95％。本综合征特点有：多发性痣样基底细胞上皮瘤、多发性颌骨囊肿、分杈肋及其他骨骼异常、中枢神经系统改变。本病患者可出现颅内钙化，头颅CT扫描可见大脑镰、小脑幕、硬脑膜及基底节区出现钙化灶。


Radiologic Overview of the Diagnosis:
Basal cell nevus syndrome:
Key radiographic findings in basal cell nevus syndrome include odontogenic keratocysts (as described above), rib anomalies, vertebral anomalies, dural calcifications, and short metacarpals. These patients are at increased risk for medulloblastoma and cardiac and ovarian fibromas.

Key Points:
Multiple odontogenic keratocysts should raise concern for the possibility of basal cell nevus syndrome (Gorlin-Goltz syndrome).
This syndrome is a genetic condition characterized by multiple nevoid basal cell carcinomas.
Basal cell nevus syndrome has several other associations including: odontogenic keratocysts, dural calcifications, rib and vertebral anomalies, short metacarpals, and midface hypoplasia.
These patients are at increased risk for medulloblastoma and cardiac and ovarian fibromas.

http://education.auntminnie.com/QMachine.ASP?UID=25G14GM7&PageId=5&Sess=6977960

泌尿 肾的先天性畸形

肾的先天性畸形，肾脏可以发生多种多样的畸形，畸形的肾脏对其他器官可以产生压迫，肾脏本身则可以发生感染、出血、结石，也可导致肾实质性或肾血管性高血压，严重的肾实质减少，则发生肾衰竭。肾不发育可发生在一侧或两侧。额外肾是单独存在的第三个肾脏，为泌尿系最罕见的畸形，发生在左侧为多。
肾结构异常：(一)肾发育不全指肾脏小于正常体积的50％以上，但肾单位及导管的分化和发育是正常的，只是肾单位的数目减少，故肾小盏及肾小叶的数目也减少。1、单纯性肾发育不全：属非遗传性畸形，多数是散发的，个别有家族性，无性别差异。2、节断性肾发育不全：多见于女性，无家族性。从肾被膜外可见肾实质菲薄的部位，该处的肾小管萎缩，充满胶样管型，仅有极少数肾小球，可见残留的髓质。3、少而大的肾单位肾发育不全：多见于男性，常是双侧性，无家族性。
(二)囊性肾发育异常：即多房性肾囊性变，常为单侧性，无家族性，无性别差异。其病因是胎儿早期输尿管梗阻的最严重后果。
(三)多囊肾系遗传性疾病，两侧肾脏弥漫性病变，体积增大，满布大小不等的囊肿，无明显的肾实质发育不全，但由于囊肿压迫肾组织亦可使肾功能受损。1．婴儿型多囊肾属常染色体隐性遗传，其病因不明。发病不限于婴儿，亦可发生在儿童和成人。伴随的肝脏病变为先天性肝纤维化。2．成人型多囊肾属常染色体显性遗传，是肾脏囊性疾病中最常见的一种。双肾弥漫性进行性囊性病变，高血压，出现血尿，肾钙质沉着，进行性肾功能衰竭，急性腰痛。除非进行透析或肾移植，一般在60多岁时死亡。
(四)单纯性肾囊肿：孤立性或多发性，常见于50岁以上成年男性及左肾，故可能是后天性的，囊肿常是孤立的，单侧病变，但可合并对侧肾病变。囊肿自肾实质产生，而不与肾盏或肾盂相通，其大小不一，直径由2～3cm至10cm不等。周围的肾实质被压迫而呈一薄壁，囊壁与肾实质紧密贴合不易剥离。囊内为浆液，含少量的乳酸脱氢酶、氯化物、蛋白与脂肪、胆固醇结晶及少量尿液，如有出血则含血性液。临床常无症状，可因腹部肿物在超声检查或排泄性尿路造影时被偶然发现，显示肾盂肾盏受压、拉长及变形。一般不需治疗，继续观察。如有严重压迫症状，可行囊肿去顶术或肾部分切除术。如有感染、结石、肿瘤与肾盂相通等并发症，或肾有高度损害，则考虑肾切除术。
(五)髓质海绵肾：又称髓质集合管扩张症或称Cacchi-Ricci病。系指一侧或双侧肾内单个或多个锥体内集合管的囊性扩张。认为是先天性异常，其发病机理尚未阐明。有三种病因假设，可能与感染、梗阻、遗传有关。
(六)肾多房性囊肿其病理特点是大而具有完整被膜的肿物，将正常肾组织推移并压迫萎缩。切面可见由很多囊肿构成，囊肿直径达数毫米至数厘米，内含草黄色或血性液体。囊壁被覆规则的扁平及立方上皮细胞。
(七）髓质囊性病：包括常染色体显性遗传的髓质囊性病和常染色体隐性遗传的familialjuvenilenephrophthisis。其特征是肾髓质有多发囊肿，呈慢性进行性肾功能不全，至青少年期出现尿毒症；但也有呈良好状态达成年的病例。肾脏较正常为小，髓质有许多囊肿，其直径自1毫米至1厘米，囊肿被覆扁平上皮。其余肾组织可见肾小球数目减少，肾小管萎缩及肾间质细胞弥漫性纤维化，慢性炎细胞浸润。均在儿童期出现症状，表现为多尿、烦渴及发育迟滞。
肾位置、形态、旋转及血管异常：(一)异位肾胎儿期肾胚芽位于盆腔内，随着发育肾逐渐上升到正常位置，若上升发生障碍或过度上升，或误升向对侧，即形成异位肾。可位于盆腔、髂部、腹部、胸部和对侧或交叉。1．盆腔肾：其发生率为500～1200人中有1例。肾较小，呈扁平、球形、并有一定程度的向前旋转；输尿管也较短，有轻度曲折，肾功能正常，但易于并发尿路梗阻、结石、感染而引起症状，主要为疼痛、血尿、排尿困难、膀胱刺激症、腹部肿块和胃肠症状。无症状的异位肾不需任何治疗，如有并发症则进行相应的处理。2．胸内肾：很少见，左侧及男性略多，可发现于任何年龄。除有肾血管异常外，还并发有先天性膈疝，缺损小者只能通过肾蒂而无其他脏器进入胸腔内。一般无症状，在体检或胸部X线检查时发现膈上有肿物。肾盂造影可以确诊，毋需治疗。3．交叉异位肾：一侧肾脏由原侧跨越中线移位至对侧，而其输尿管仍位于原侧。可分为融合型和非融合型，前者较多见，异位肾一般低于正常侧，肾盂位于肾的前面，输尿管仍从原有径路进入膀胱，开口于正常部位。常合并其他泌尿系畸形。多数无症状，或有下腹痛、肿块及压迫症状。
(二)融合肾：由于原始肾组织块的分裂停顿或发育异常、两侧肾脏相互融合，完全性或部分性，有各种类型如蹄铁形肾、乙状肾、盘形肾、块肾等。由于融合发生于胚胎早期，因而均伴有所处的位置异常和肾旋转不良。其血供可来自骶中动脉和髂总动脉。蹄铁形肾在融合肾中最常见，大约每400人中有1例，多见于男性。
(三)肾旋转异常：在正常发育过程中，肾轴应发生90°旋转。可发生4种旋转异常：腹侧旋转（未旋转），腹中向旋转（旋转不全），侧向旋转（反向旋转）和背侧旋转（旋转过度）。最多见的是旋转不全，即肾盂朝向前方；如旋转过度，则肾盂朝向后侧。尿路造影可明确诊断，如无并发症存在毋需治疗。
(四)肾血管异常：原始的肾脏血管来自骶中动脉，髂动脉或低位腹主动脉。在正常发育下，原有的血管逐渐萎缩而代以肾动脉。如原始供应血管持续存在，则可成为肾血管异常原因之一。或成为肾或输尿管的副血管。多见于输尿管的前方或肾盂输尿管连接处附近。常可影响或阻碍尿液排泄。而供应肾上极的异常血管则不致造成尿路梗阻。在临床上，肾血管异常除可产生输尿管梗阻外，无其他重要意义。

泌尿 肾周淋巴管扩张 Renal lymphangiectasia

Retroperitoneal lymphangiectasia associated with mediastinalextension: Radiologic findings

European Journal of Radiology Extra 63 (2007) 61–63 已下载

泌尿 肾脏外伤分级

Grade 1
Hematuria with normal imaging studies
Contusions
Nonexpanding subcapsular hematomas
Grade 2
Nonexpanding perinephric hematomas confined to the retroperitoneum
Superficial cortical lacerations less than 1 cm in depth without collecting system injury
Grade 3
Renal lacerations greater than 1 cm in depth that do not involve the collecting system
Grade 4
Renal lacerations extending through the kidney into the collecting system
Injuries involving the main renal artery or vein with contained hemorrhage
Segmental infarctions without associated lacerations
Expanding subcapsular hematomas compressing the kidney
Grade 5
Shattered or devascularized kidney
Ureteropelvic avulsions
Complete renal laceration or thrombus of the main renal artery or vein

http://radiographics.rsnajnls.org/cgi/content/full/21/3/557

Renal injuries can be classified into fourlarge categories based on imaging findings.
Category I renal injuriesinclude minor cortical contusion, subcapsular hematoma, minor lac-eration with limited perinephric hematoma, and small cortical infarct.
Category II lesions include major renal lacerations extending to themedulla with or without involvement of the collecting system and seg-mental renal infarct.
Category III lesions are catastrophic renal injuriesand include multiple renal lacerations and vascular injury involving therenal pedicle.
Category IV injuries are ureteropelvic junction injuries.

泌尿 特发性腹膜后纤维化

特发性腹膜后纤维化：特征是密度均匀，与主动脉相连紧密；主动脉本身及其周围器官无明显受压移位；主动脉与椎体间距不变化；输尿管狭窄者向中心牵拉移位。
DDX：淋巴瘤 腹膜后淋巴瘤范围广，显示为大结节性肿块，密度不均，可伴有肠系膜淋巴结肿大，主动脉与椎体间距增大。
DDX：转移性淋巴结及转移性肿瘤 缺乏纤维组织，表现不为连续的主动脉旁肿块或淋巴结肿大。增强后肿块不均匀强化呈结节状。

另：
低密度淋巴结：结核，睾丸癌
高密度淋巴结：Kaposi肉瘤
广泛钙化淋巴结：Castleman‘s病