A Newborn Presents With a Scrotal Mass

Abstract

A newborn presents with a scrotal mass.The baby was sent home after 2 days in the well baby nursery with instruction for surgical follow-up for the scrotal mass, which was believed to be a left inguinal hernia. At 6-month follow-up, the infant was normally developed for his age. On genital examination, the right testis was not palpable in the scrotum, the left testis was in the scrotum, and an elastic mass was identified at the upper pole of the left testis. No other physical abnormalities were detected. Results of all laboratory investigations were normal.Surgery revealed a red-brown encapsulated mass fused to the left testis. No connection could be seen between the spleen and the mass. There was no testicular torsion (Fig 1-4). Biopsy was performed after informed consent was obtained from his parents, and pathology identified the mass as splenic tissue.Approximately 150 cases of SGF are reported in the literature. The first description was by Pommer in 1889 in an infant who had limb abnormalities. SGF results from abnormal connection of splenic tissue with gonadal-mesonephric structures. The ectopic spleen generally is found within the tunica albuginea, separated from the testicular tissue by a fibrous capsule.SGF is far more common in males, in whom the appearance usually is a scrotal mass. The higher prevalence among males probably is due to the anatomic location of the testes, allowing ready and frequent palpation. Putschar and Manion classified SGF into two types: continuous (direct connection between the spleen and gonad) and discontinuous (no anatomic connection between ectopic splenic tissue and the principal spleen). Limb defects, micrognathia, or other congenital malformations such as cryptorchidism, ventricular septal defects, anal atresia, microgastria, spina bifida, or continuous type craniosynostosis often accompany SGF.SGF is assumed to occur between 5 and 8 weeks of gestation, before the beginning of gonadal descent. Its cause remains unclear, but two theories predominate. Sneath has suggested that slight inflammation of the peritoneal surfaces over the spleen and gonadal ridge can produce partial fusion of the two organs. von Hochstetter postulated that a retroperitoneal pathway for splenic anlage cells may allow contact with the gonadal anlage.Ultrasonography is the current imaging modality of choice for assessment of scrotal abnormalities, but findings are not conclusive. If SGF is suspected clinically before surgery, the diagnosis may be confirmed by 99m Tc-sulfur colloid imaging, which shows uptake in both the spleen and the accessory splenic tissue within the scrotum. Symptoms attributable to the presence of SGF include testicular pain after gastroenteritis, bowel obstruction, and acute scrotal pain resembling testicular torsion. Because of the rarity of SGF, it usually is not diagnosed preoperatively.This uncommon congenital abnormality is difficult to diagnose both clinically and with imaging. If a patient has congenital limb defects in conjunction with cryptorchidism or high ectopia, the possibility of SGF should be considered. Awareness of the entity is crucial to avoid misdiagnosis of a tumor.Ultrasonography, laparoscopic exploration, and scintigraphic scanning generally are necessary to demonstrate the distribution of splenic tissue, thereby allowing proper diagnosis and treatment. If intraoperative biopsy reveals splenic tissue, orchiectomy is not indicated. Surgery is necessary for the usual or unusual indications, such as hernia, cryptorchidism, and torsion. The ectopic spleen usually can be dissected away and the testis salvaged. Laparoscopic surgery should be considered for most uncomplicated cases.JoDee M. Anderson, MD, Division of Neonatal Medicine, Oregon Health & Science University, Portland, OR