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

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.