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腹腔镜诊断小儿交通性鞘膜积液合并持续性脾性腺融合

Continuous Splenogonadal Fusion Diagnosed by Laparoscopy in a Child With a Communicating Hydrocele.

作者信息

Berrondo Claudia

机构信息

Surgery/Pediatric Urology, University of Nebraska Medical Center, Omaha, USA.

Pediatric Urology, Children's Nebraska, Omaha, USA.

出版信息

Cureus. 2024 Aug 26;16(8):e67836. doi: 10.7759/cureus.67836. eCollection 2024 Aug.

Abstract

Splenogonadal fusion is a rare congenital anomaly primarily affecting males, characterized by an abnormal fusion of the spleen and the gonad. There are two primary forms: continuous, in which the normal spleen is directly connected to the gonad via a cord of fibrous or splenic tissue, or a combination of both, and discontinuous, in which ectopic splenic tissue fuses to the gonad without connection to the normal spleen. Continuous splenogonadal fusion is often associated with other congenital defects, such as cryptorchidism, limb anomalies, and micrognathia. Due to its rarity and nonspecific symptoms, splenogonadal fusion is typically diagnosed incidentally during surgery for undescended testis or inguinal hernia. We present a case of a five-year-old boy with a communicating hydrocele who underwent surgical repair. Intraoperative findings during inguinal exploration revealed a fibrous connection to the upper pole of the left testicle, extending into the internal inguinal ring. Diagnostic laparoscopy confirmed continuous splenogonadal fusion, demonstrating splenic tissue and a fibrous cord with islands of splenic tissue extending from the spleen to the internal inguinal ring. A portion of the fibrous cord with splenic nodules was excised and examined pathologically, confirming the diagnosis of splenogonadal fusion. The diagnosis of continuous splenogonadal fusion primarily relies on intraoperative findings during surgery for other conditions. Laparoscopy can be instrumental in diagnosing this rare condition.

摘要

脾性腺融合是一种罕见的先天性异常,主要影响男性,其特征是脾脏与性腺异常融合。有两种主要形式:连续性,即正常脾脏通过纤维组织或脾脏组织条索直接与性腺相连,或两者结合;以及非连续性,即异位脾脏组织与性腺融合而不与正常脾脏相连。连续性脾性腺融合常与其他先天性缺陷相关,如隐睾、肢体异常和小颌畸形。由于其罕见性和非特异性症状,脾性腺融合通常在隐睾或腹股沟疝手术中偶然诊断出来。我们报告一例5岁患有交通性鞘膜积液的男孩接受手术修复的病例。腹股沟探查术中发现与左侧睾丸上极有纤维连接,延伸至腹股沟内环。诊断性腹腔镜检查证实为连续性脾性腺融合,显示脾脏组织以及一条纤维条索,其中有脾脏组织岛从脾脏延伸至腹股沟内环。切除部分带有脾脏结节的纤维条索并进行病理检查,证实为脾性腺融合。连续性脾性腺融合的诊断主要依赖于针对其他病症手术时的术中发现。腹腔镜检查有助于诊断这种罕见病症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ec8d/11424387/6308c733d67d/cureus-0016-00000067836-i01.jpg

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