Median Raphe Cyst: A Clinically Challenging Diagnosis

Abstract

Median raphe cyst is an uncommon developmental anomaly that can develop anywhere along the midline of the external genitals. Only a few hundred cases have been published in the English literature and the lack of awareness of this entity can lead to confusion and misdiagnosis. We report here a case of median raphe cyst located in the midline of the anterior scrotum of a 35-year-old man. Clinically, the patient presented with a scrotal mass increasing substantially in size over two days associated with tenderness, skin erythema, and scrotal pain. Radiologic interpretation of a sonogram and computed tomography scan suggested a thrombosed vessel. The patient was diagnosed with septic thrombophlebitis associated with overlying cellulitis. Despite conservative therapy with antibiotics, the patient developed pyrexia, tachycardia, and leukocytosis prompting surgical excision of the lesion. Histopathologic examination revealed an infected median raphe cyst. The cyst wall was lined by a stratified epithelium that included numerous Alcian blue positive goblet cells. The epithelial cells showed reactive changes with infiltration by numerous neutrophils. Our objective is to bring attention to and thereby facilitate the diagnosis of this unusual entity.

Ruptured Median Raphe Cyst Mimicking a Vascular Penile Mass on Ultrasound

“…Our case is a novel presentation of a median raphe cyst masquerading as a large vascular penile shaft mass. Typical imaging features of MRCs in the literature are those of an avascular cystic lesion on ultrasound [3][4][5][6]. Our case demonstrates several atypical imaging features on ultrasound including an internal echogenicity suggestive of a solid mass.…”

“…Ultrasound and MRI allow for anatomical assessment in order to understand the extent of the lesion and to rule out communication with neighboring structures or vascularity. Typical imaging features described in the literature are that of an avascular cyst on ultrasound [3][4][5][6]. Similarly, a cystic structure with high T2 signal can be seen on MRI imaging [4,7].…”

Extra-testicular Intra-scrotal Median Raphe Epidermoid Cyst Mimicking Polyorchidism in a Child

“…Unlike our patient, rarely the cyst measures a size more than 2 cm (5). (Table 2) and the guardians and patients seek advice for aesthetic purposes or uncommonly to manage complications such as infection, trauma, interference with sexual life or urinary complications (3,5). Differential diagnosis of such swelling could include polyorchidism as the family assumed at the beginning in our case.…”

“…Although physical and radiological assessment suggests the cyst type, histopathological confirmation is needed. Four types have been identified: urethral, epidermoid, glandular, and mixed (5,19). According to the lining, pseudostratified columnar epithelium, stratified squamous epithelium, urothelium, glandular epithelium and coexisting mixed epithelium, respectively.…”

“…These cysts are commonly small, silent and present beyond the age of 10 years. They grow slowly and rarely are complicated by infection as Staphylococcus aureus and Neisseria gonorrhoeae leading to purulent discharge (5). The median raphe cysts presenting in the scrotum might be socially accepted as polyorchidism not warranting medical opinion despite the fact that a third or supernumerary testicle is exceptionally rare (6).…”