Imperforate Hymen in Rett Syndrome Patient in Chandrubeksa Hospital, RTAF.

Abstract

      A 14 years old Thai girl referred to Obstetrics & Gynecology department of secondary level hospital with urinary retention problem from hematocolpos due to imperforate hymen. She is a Rett

Syndrome patient with communication and multiple joints stiffness problems, and includes both hip joints that have limit range of movement. She suffered from urinary retention since ten days ago and went to primary care clinic and referred to primary care hospital. She received treatment by intermittent and retain urinary catheterization for few times but urinary retention did not resolve then she went to sonography examination and found hematocolpos. Her physical examination found imperforate hymen bulging with hematocolpos. A sonography scan revealed a fluid-filled vagina and uterus measuring 11.68*6.5*5.68 cm. Initial treatment for patient is retain urinary catheterization and drain out urine 100 mL and patient suffering was relief. She went to the operating room for surgical treatment, about 250 mL of consistent with menstrual blood were evacuated at time of hymenotomy. Hymenotomy was performed with a cruciate incision. No complications were encountered. Imperforate hymen incidence is 1 in 2,000 people, there have been >40 reported cases of imperforate hymen that causing hematocolpos and subsequent urinary retention. Rett syndrome is one of the most common causes of complex disability in girls and frequency is approximately 1 in 15,000-22,000 children. It is characterized by early neurological regression that severely affects motor, cognitive and communication skills, by autonomic dysfunction and often a seizure disorder. Family support and her maternal care are helpful for physical examination, diagnosis and intraoperative management for this patient. Lesson from this case suggest that girls patient with developmental disorders should have sufficient gynecological examination before reach age of puberty to prevent problems from genital tract congenital malformations.