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脾性腺融合:病例报告及文献复习。

Splenogonadal fusion: a case report and review of the literature.

机构信息

Department of Urology, The Children's Hospital, Zhejiang University, School of Medicine, National Clinical Research Center for Child Health, 57 Zhugan Xiang, Hangzhou, 310053, China.

出版信息

BMC Urol. 2021 Feb 3;21(1):16. doi: 10.1186/s12894-021-00781-z.

DOI:10.1186/s12894-021-00781-z
PMID:33536002
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7860507/
Abstract

BACKGROUND

Splenogondal fusion (SGF) is a rare congenital anomaly characterized by abnormal association between the splenic tissue and the gonads or mesonephric remnants. SGF that requires separate two-stage laparoscopic staged Fowler-Stephen orchiopexy on both the left and right sides is extremely rare. SGF could be misdiagnosed as testicular malignancy and leads to unnecessary orchiectomy.

CASE PRESENTATION

This is a case of an 8-month old male infant presented with bilateral cryptorchidism, B-mode ultrasound visualized the left and right testes in the lower abdominal cavity and the upper margin of the left testicle as a hypoechoic mass extending to the spleen, indicating an undescended right testis and possible SGF on the left side. Single-site laparoscopic examination confirmed the diagnosis of SGF on the left side and an undescended right testis. As both testes were high and the right spermatic vessel was poorly developed and short, a routine single stage orchiopexy would be difficult and risky, therefore, separate two-stage laparoscopic staged Fowler-Stephen orchiopexies for both sides were implemented. Stage 1 of the staged Fowler-Stephen orchiopexy for the right side was performed first without treating the left side, Stage 2 for the right side, separation of the left testis from the spleen as well as Stage 1 for the left side were performed 7 months later, and Stage 2 for the left side was performed 7 months after that. Follow-up ultrasound 1 year after the surgery revealed no obvious abnormalities in the shapes of the testes or their blood supply. This treatment strategy prevented unnecessary orchiectomy.

CONCLUSIONS

We reported a rare case of SGF that needed separate two-stage laparoscopic staged Fowler-Stephen orchiopexies for both sides, and a review of the recent literature. SGF is a rare congenital anomaly often diagnosed incidentally during exploration/surgery for scrotal swelling/mass, cryptorchidism or inguinal hernia in young patients. Surgeons, especially pediatric surgeons should be aware of this rare condition to avoid unnecessary, life-altering radical orchiectomy. When routine single stage orchiopexy is not feasible or risky for either side, separate two-stage laparoscopic staged Fowler-Stephen orchiopexies could be performed on both the left and right sides to avoid unnecessary orchiectomy.

摘要

背景

脾性腺融合(SGF)是一种罕见的先天性异常,其特征是脾组织与性腺或中肾残余物之间异常关联。需要分别对双侧进行腹腔镜分期 Fowler-Stephen 睾丸固定术的 SGF 极为罕见。SGF 可能被误诊为睾丸恶性肿瘤并导致不必要的睾丸切除术。

病例介绍

这是一例 8 月龄男婴,双侧隐睾,B 型超声显示左侧和右侧睾丸位于下腹部和左侧睾丸上缘的低回声肿块延伸至脾脏,提示右侧未降睾丸和左侧可能存在 SGF。单部位腹腔镜检查证实左侧 SGF 和右侧未降睾丸的诊断。由于双侧睾丸较高,右侧精索血管发育不良且短,常规的单阶段睾丸固定术会很困难且有风险,因此对双侧分别进行分期腹腔镜 Fowler-Stephen 睾丸固定术。先进行右侧分期 Fowler-Stephen 睾丸固定术的第一阶段,而不处理左侧,然后 7 个月后进行右侧的第二阶段、左侧睾丸与脾脏的分离以及左侧的第一阶段,再 7 个月后进行左侧的第二阶段。手术后 1 年的超声随访显示睾丸的形状和血供无明显异常。这种治疗策略避免了不必要的睾丸切除术。

结论

我们报告了一例罕见的双侧 SGF 需要分别进行分期腹腔镜 Fowler-Stephen 睾丸固定术的病例,并对近期文献进行了回顾。SGF 是一种罕见的先天性异常,在年轻患者因阴囊肿胀/肿块、隐睾或腹股沟疝进行探查/手术时经常偶然发现。外科医生,特别是小儿外科医生应该意识到这种罕见情况,以避免不必要的改变生活的根治性睾丸切除术。当双侧的常规单阶段睾丸固定术不可行或有风险时,可以对双侧分别进行分期腹腔镜 Fowler-Stephen 睾丸固定术,以避免不必要的睾丸切除术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa77/7860507/538c6859bcd1/12894_2021_781_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa77/7860507/b32d9e897f59/12894_2021_781_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa77/7860507/c9e6108c0e40/12894_2021_781_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa77/7860507/538c6859bcd1/12894_2021_781_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa77/7860507/b32d9e897f59/12894_2021_781_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa77/7860507/c9e6108c0e40/12894_2021_781_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aa77/7860507/538c6859bcd1/12894_2021_781_Fig3_HTML.jpg

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