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连续脾性腺融合一例报告

Echidna Splenule: A Case Study of Continuous Splenogonadal Fusion.

机构信息

Loma Linda University, School of Medicine, Loma Linda, CA.

Loma Linda University Children's Hospital, Department of Urology, Loma Linda, CA.

出版信息

Urology. 2024 Jan;183:209-211. doi: 10.1016/j.urology.2023.09.009. Epub 2023 Sep 28.

Abstract

Splenogonadal fusion (SGF) is a rare congenital anomaly of an aberrant accessory spleen-gonad connection. We present a rare case of continuous splenogonadal fusion in a full-term male with a left undescended testis, multiple congenital limb anomalies, and syndromic facies. Diagnostic laparoscopy revealed the "Echidna Splenule," a snake-like intraperitoneal splenule coursing from the spleen along the left paracolic region and engulfing an atrophic intra-abdominal testis preventing spontaneous descent and distally herniating into the left open internal inguinal ring. The atrophic testis and Echidna Splenule were resected. Splenogonadal fusion should be considered in children with left undescended testis and concomitant limb and facial anomalies.

摘要

脾性腺融合(SGF)是一种罕见的先天性异常,表现为异常副脾-性腺连接。我们报告了一例罕见的足月男性病例,其具有左侧未降睾丸、多种先天性肢体畸形和综合征面容,存在连续的脾性腺融合。诊断性腹腔镜检查显示“Echidna Splenule”,即一种蛇形的腹腔内脾副脾,从脾脏沿着左侧结肠旁区走行,包裹一个萎缩的腹腔内睾丸,阻止其自发下降,并向远端疝入左侧开放的内环。切除了萎缩的睾丸和 Echidna Splenule。对于具有左侧未降睾丸和伴发的肢体和面部异常的儿童,应考虑脾性腺融合。

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