脾性腺融合合并 Moebius 综合征和 Poland 综合征：首例报告。

Splenogonadal fusion associated with Moebius and Poland syndromes: first case reported.

机构信息

Pediatric Surgery Department. Dr. Roberto Gilbert Elizalde Pediatric Hospital. Guayaquil (Ecuador).

Pediatric Urology Department. Dr. Roberto Gilbert Elizalde Pediatric Hospital. Guayaquil (Ecuador).

出版信息

Cir Pediatr. 2021 Oct 1;34(4):219-222.

PMID:34606704

Abstract

INTRODUCTION

Splenogonadal fusion is a rare congenital anomaly of unknown etiology caused by an abnormal fusion of the splenic tissue and the gonadal tissue.

CASE REPORT

2-year-old patient with paralysis of the 6th, 7th, and 9th cranial nerves, tent-shaped mouth, cleft palate, right pectoralis major hypoplasia, disruptive defect of the right upper limb, and a mass located at the left inguinal region. At inguinal hernia repair surgery, a processus vaginalis with non-reducible content was observed. When opening the hernia sac, a descending segment of splenic tissue merging with the upper pole of the left testis was found. The patient was diagnosed with splenogonadal fusion. The splenic tissue merging with the testis upper pole was resected, and the remaining splenic tissue was reduced towards the abdominal cavity.

DISCUSSION

Splenogonadal fusion is difficult to diagnose. Being familiar with it allows unnecessary orchiectomies to be prevented.

摘要

简介

脾性腺融合是一种罕见的先天性畸形，病因不明，是由于脾组织和性腺组织异常融合所致。

病例报告

一名 2 岁患者，表现为第 6、7、9 颅神经麻痹、舟状口、腭裂、右侧胸大肌发育不良、右上臂中断性缺损以及位于左侧腹股沟区的肿块。在腹股沟疝修补术中，观察到一个不可复性内容物的鞘状突。当打开疝囊时，发现一段与左侧睾丸上极融合的降结肠脾组织。患者被诊断为脾性腺融合。切除融合在睾丸上极的脾组织，将剩余的脾组织还纳入腹腔。