The Clinical Analysis Of 193 Cases With MRKH Syndrome

Objective:To explore the clinical spectrum and treatment of the MRKH syndrome.Methods:Clinical,operation and follow-up documents of 193 patients with MRKH syndrome were reviewed.Results:1 Clinical spectrumAmong our cohort of 193 patients,we found 19 patients with skeletal system malformations,11 patients with urinary system malformations,8patients with cardiac dysplasia,8 patients with fallopian tubes dysplasia,6patients with ovarian dysplasia,5 patients with inguinal hernia,4 patients with anal atresia or rectal "vagina" fistula,2 patients with eyes dysplasia.2 Treatment and evaluationThe most common type of vaginoplasty is the sigmoid vaginoplasty,follwed by the peritoneum vaginoplasty.The laparoscopic sigmoid vaginoplasty group had advantages in the blood loss and rate of postoperative complication than the transabdominal sigmoid vaginoplasty group,and the difference had statistical significance(P<0.05);The laparoscopic sigmoid vaginoplasty group had advantages in the operation time ? blood loss ? postoperative duration of hospitalization ? postoperative urinary tube retention time ? vaginal mold replacement time and rate of postoperative complication than the laparoscopic peritoneum vaginoplasty group,and the difference had statistical significance(P<0.05).3 Follow-up results3.1 Psychological function98.86 % of patients with MRKH syndrome were confused,unacceptable to the diagnosis,After psychological guidance and treatment,84.09% of patients could accept the diagnosis.3.2 Sexual life qualityThe survey of these patients' FSFI questionnaire revealed that the sigmoid vaginoplasty had advantages in the sexual arousal and vaginal lubrication than the peritoneum vaginoplasty,and the difference had statistical significance(P<0.05);No statistical difference was found in the total score?sexual desir?sexual orgasm?sexual satisfaction and the sexual pain between the two groups(P>0.05).3.3 Quality of lifeThe survey of these patients' SF-36 life quality questionnaire revealed that the transabdomina sigmoid vaginoplasty group had advantages in the PF?BP?SF than the laparoscopic sigmoid group,and the difference had statistical significance(P<0.05);No statistical difference was found in every dimension of the SF-36 life quality questionnaire between the sigmoid vaginoplasty and the peritoneum vaginoplasty(P>0.05).Conclusion:1.Skeletal malformations were most common in clinical spectrum of patients with MRKH syndrome,followed by renal dysplasia.2.The laparoscopic sigmoid colpoplasty was an ideal treatment for patients with MRKH syndrome.3.Psychological guidance and treatment could improve the mental health of patients with MRKH syndrome.