Nusrat Khan  ( The Children’s Hospital, Pakistan Institute of Medical Sciences, 0-8/3, Islamabad. )
Shamim A. Qazi  ( The Children’s Hospital, Pakistan Institute of Medical Sciences, 0-8/3, Islamabad. )
Naeem Khan  ( The Children’s Hospital, Pakistan Institute of Medical Sciences, 0-8/3, Islamabad. )

A rare case associated with congenital hematometrocolpos in a circumcised 13 years old Somallan girl is presented. Emphasis on proper histoiy is highlighted. Mutilation caused by bad cultural practice of female circumcision which is although not a problem in Pakistan, is brought into notice for those who may be unaware of perineal appearance of a circumcised female and stitched overvagina.

A 13 years old Somalian girl was admitted with complaints of periodic abdominal pain and lower abdominal swelling of two months duration. Pain was predominantly in the lower abdominal and pelvic area and swelling had gradually increased in size. On general examination, she was a healthy normal looking girl with a lower abdominal globular, non-tender and smooth mass of 11x8 cm in diameter arising from pelvis with an appearance of a distended bladder. But on emptying the bladder, mass persisted. The perineal examination revealed flat vulva with absent labia majora and stitched over skin obscuring the vaginal opening. Clitoris was also missing having been amputated at the time of circumcision. Parents had not given the history of previous circumcision. Rest of the general physical examination was normal. A provisional diagnosis of vaginal stenosis due to circumcision injury was entertained. But on examination under anaesthesia, once the stitched over the skin in front of vagina was removed, there was an intact imperforate hymen which had resulted inhematocolpos. Acruciate incision on the hymenyielded old altered menstrual blood resolving the supra pubic mass of hematocolpos.

Congenital anomalies like hydrometrocolpos, or hematocolpos, are well recognized lesions which occur due to distal obstruction from conditions like imperforate hymen or vaginal atresia^1-4. Obstruction may also be caused due to stenosis produced by mutilating practice of female circumcision incertainMiddle Eastern and African countries⁵. The presentation of hydrometrocolpos is manifest atbirth orin early infancy^3-5, whereas in hematocolpos signs are delayed till the age of menarche⁴. The usual presentation is lower abdominal mass arising from pelvis and maybe confused with distended bladder. Perineal examination is essential to arrive at diagnosis and avoid embarassment.

Perineal examination is often neglected especially in conservative societies where cultural taboos and inhibitions make it much more difficult Therefore, imperforate hymen may often be discovered at puberty, when it presents as primary amenorrhoea, cryptomenorrhea and dyspareunia³. The abnormalities like vaginal agenesis, atresia and hydrometmcolpos present since birth and are discovered at neonatal age, or in early infancy due to presence of an abdominal mass, urinary symptoms of incontinence or dribbling of urine, causedby mechanical pressure of distended uterus^6,7. In countries where females are circumcised as a cultural practice the injuries and infections may produce obliteration of vaginal orifice which will produce the same effect⁸ as in imperforate hymen or in a case of vaginal atresia. Surgeiy however, may be more extensive to ieconstnict distally stenosed vagina, as opposed to imperforate hymen where minimal procedure is inovolved as was in our case. Where vaginal reconstruction is performed, regular dilatations with Hagar’sdilators shouldbedoneto maintainpatency. Insunimaiy, female circumcisionis one of the worst kind of female mutilation practised in some countries even in this era and should be abolished. Secondly, countries where it is not practised doctors should be made aware to recognize this serious pathology.

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