Median raphe cyst of the perineum is a rare congenital anomaly and was first reported in 1895. We report a 9-year-old male with median raphe cysts in the perineum which had become swollen and then spontaneously ruptured several times since he was 3 years old. There were two separate lesions; one was a cystic lesion and the other was a canal-like lesion. They were completely excised and the inner layer of the cysts was composed of transitional epithelium.
Median raphe cyst is a rare congenital anomaly and was first reported in 1895 by Mermet [
The patient was a nine-year-old boy who visited the outpatient clinic of our institute for the treatment of lesions around the anus that repeatedly became swollen. The parents thought that it was a kind of hemorrhoid and reported that the patient experienced acute swelling of the perineal skin lesion when he was three years old and soon it ruptured and seemed to heal spontaneously. Howeversince then, he experienced swelling and rupture of the perineal lesion several times. The mother of the patient presented a photograph of the skin lesion when it was swollen (
Congenital median raphe cyst is a rare anomaly that was first reported in 1895 by Mermet [
The etiology of median raphe cysts was considered to be one of the following: 1) the epithelial rest becomes
buried under the skin when the urethral folds form the urethra, or 2) the result of abnormal development of paraurethral ducts. The inner layer of the cysts was composed of various epithelia such as stratified squamous epithelium, pseudostratified epithelium and squamous columnar epithelium, which are epithelia of endodermal origin and the components of the male urethra. Indeed, in our case the epithelium of the inner layer of the cysts was composed of transitional epithelium, which also suggested urethral origin. The differential diagnoses of cysts in the perineal area are urethral diverticulum, dermoid cyst, epidermoid cyst [
This lesion often shows no symptoms and in such cases observation may be a reasonable choice but once symptoms appear, it must be excised to relieve the symptoms.
We must keep in mind that this lesion is rare but may be encountered in the outpatient clinic and should be treated properly.