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Pediatr Surg Int (2010) 26:795800 DOI 10.1007/s00383-010-2640-3
ORIGINAL ARTICLE
Our 10-year experience of variable Mllerian anomalies and its management
Shivaji B. Mane Pankaj Shastri Nitin P. Dhende Abu Obaidah
Himanshu Acharya Suyodhan Reddy Jameer Arlikar Nitin Goel
Accepted: 16 June 2010 / Published online: 30 June 2010 Springer-Verlag 2010
AbstractPurpose Though the Mllerian duct anomalies are rare in incidence, their variable age, and mode of presentation makes their management difcult.
Methods In the last 10 years authors managed 14 patients with different types of Mllerian anomalies. There were eight patients with complete Mllerian agenesis: three of partial Mllerian agenesis, one patient had uterine didelphys with vertical and transverse vaginal septum on left side, one patient had uterine didelphys with cervical agenesis on the right side, one patient had MRKH with anorectal malformation.
Results Patients with complete Mllerian agenesis underwent bowel vaginoplasty, and patients with partial Mllerian agenesis with hydrocolpos underwent pull-through vaginoplasty. Patient of uterine didelphys one with septum underwent septum excision and in another with cervical agenesis, cervix opened and drain kept. In MRKH with vestibular stula, stulous opening was kept as vaginal opening and rectum brought down as a neoanus.
Conclusion Mllerian duct anomalies can present in any age group and with variable symptoms. Sigmoid colon vaginoplasty is an excellent procedure for complete or partial Mllerian agenesis. Correct evaluation of these patients and proper management is a challenge.
Keywords Mllerian duct anomalies
MayerRokitanskyKsterHauser (MRKH) syndrome
Hematometra Hematosalphinx Hydrocolpos
Uterine didelphy
Introduction
Mllerian agenesis is the second commonest cause of primary amenorrhea, after gonadal dysgenesis. The Mllerian ducts are the primordial anlage of the female reproductive tract. They differentiate to form the fallopian tubes, uterus, the uterine cervix, and the superior aspect of the vagina. A wide variety of malformations can occur when this system is disrupted. They range from uterine and vaginal agenesis to duplication of the uterus and vagina to minor uterine cavity abnormalities. Mllerian malformations are frequently associated with abnormalities of the renal and axial skeletal systems. Incidence varies widely and depends on the study. The reported incidence varies between 0.1 and 3.5%. In women with fertility problems, the incidence of Mllerian duct anomalies (MDAs) is slightly higher at 36%. Despite the infrequency of such anomalies, the surgeon must be...