Surgical management of persistent Müllerian duct syndrome in children
10.3760/cma.j.cn101070-20240705-00420
- VernacularTitle:儿童苗勒管永存综合征的手术治疗
- Author:
Qingming MENG
1
;
Tianhua LUO
1
;
Xuhui ZHANG
1
;
Caihong WANG
1
;
Baifeng CHEN
1
;
Wei WANG
1
;
Dong WANG
1
;
Chenxin MENG
1
Author Information
1. 山西省儿童医院泌尿外科，太原 030001
- Publication Type:Journal Article
- Keywords:
Child;
Persistent Müllerian duct syndrome;
Surgical management
- From:
Chinese Journal of Applied Clinical Pediatrics
2025;40(3):214-217
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore feasible surgical options for management of persistent Müllerian duct syndrome (PMDS) in children.Methods:A retrospective case summary was made.The clinical data of 8 patients who were diagnosed with PMDS and treated at Shanxi Children′s Hospital from September 2011 to November 2020 were retrospectively reviewed.Of the 8 PMDS patients, 6 were sporadic cases, 2 were twins (monochorionic), with an age range from 1 year to 12 years.All cases had normal male external genital organs and a chromosomal karyotype of 46, XY.During the operation, the surgical plan was selected according to clinical classification, vas structure and the development of the initial uterus.After the operation, the Müllerian structure in the abdominal cavity and the testicular development of the children were followed up through color ultrasound at the outpatient department.Results:Four patients had oblique inguinal hernia, 3 had bilateral cryptorchidism, and 1 had transverse testicular ectopia.Müllerian inhibitory hormone (MIH) levels were significantly decreased in 5 cases and normal in 1 case.The other 2 cases were not tested for MIH.All bilateral gonadal biopsies were testicular tissue, but undifferentiated gonadal tissue was detected in 3 cases.Four cases underwent radical surgery after gonadal biopsy.Among these 4 children, 3 received one-stage radical operation, and 1 received repair of oblique hernia before the radical surgery.Three patients had a well-defined vas structure and underwent hysterectomy and bilateral orchidopexy; three patients had a vague vas structure and received orchidopexy with the uterus preserved; one case underwent hysterectomy, orchidopexy on one side and orchiectomy on the other side; one case had bilateral orchidectomy in Fowler-Stephens stage.All the 8 children were followed up for 2 to 12 years.Ultrasound showed that the descending testis was well developed, with no cryptorchidism retraction or hernia recurrence.No tumor was observed in the preserved uterus in the abdominal cavity.Conclusions:The plan for PMDS management in children should be determined based on its clinical classification.Gonadal biopsy is essential, and whether to remove the Müllerian structure depends on the vas structure and the development of the uterus.
