Homozygous loss of function mutations in genes encoding anti-Müllerian hormone (<i>AMH</i>) or its receptor (<i>AMHRII</i>) lead to persistent Müllerian duct syndrome (PMDS). PMDS is characterized by the presence of a uterus, fallopian tubes, cervix, and upper vagina in fully virilised 46,XY males. Both surgical management and long-term follow-up of these patients are challenging. Four cases with PMDS presented with cryptorchidism and inguinal hernia, and laparoscopic inguinal exploration revealed Müllerian remnants. Three of the patients had homozygous mutations in the <i>AMH</i> gene, one with a novel c.1673G>A (p.Gly558Asp) mutation, and one patient had an <i>AMHRII</i> mutation. All patients underwent a single-stage laparotomy in which the fundus of the uterus was split along the midline to release testes and to avoid damaging the vas deferens or the deferential artery. Biopsy of Müllerian remnants did not reveal any malignancy. The cases presented here expand the clinical and molecular presentation of PMDS. Cryptorchidism and inguinal hernia in the presence of Müllerian structures in an appropriately virilised 46,XY individual should suggest PMDS. Long-term reproductive and endocrinological surveillance is necessary.
Background:
The major influencing factors for complications during hypospadias repair are the original location of the meatus, previous urethral surgery, surgical technique, and surgeon’s experience. While the effect of the technique of regional analgesia on complication rates has been considered in some studies, this issue remains controversial.hile the effect of the technique of regional analgesia on complication rates has been considered in some studies, this issue remains controversial.
Aims:
To determine the effect of caudal block and penile block on complication rates of hypospadias repair in patients with subcoronal hypospadias.
Study Design:
Retrospective cross-sectional study.
Methods:
Data of children who underwent hypospadias repair between 2011 and 2019 in our clinic (n = 770) were reviewed retrospectively. Only patients with subcoronal hypospadias and who underwent tubularized incised plate urethroplasty, performed by the same two experienced surgeons (n = 279), were included in the study. The exclusion criteria were incomplete data and follow-up time shorter than 12 months. Data of 279 patients were analyzed. Patients were divided into two groups according to the analgesia type: caudal block (n = 95) and penile block (n = 184), and complication rates were compared between these groups.
Results:
The median age was 36 months in the caudal block group and 30 months in the penile block group (
P
= 0.390). The median follow-up times were 54 and 42.7 months in the caudal and penile block groups, respectively. Total complication rates did not differ significantly between the groups and were determined as 10.5% and 12.5% in the caudal and penile groups, respectively. Urethrocutaneous fistula rates were observed as 2.1% and 4.3% (p=0.50). None of the patients had penile chordee and no penile plication was performed in the whole group.
Conclusion:
This study shows that there is no increase in post-repair complications in patients with subcoronal hypospadias who underwent caudal block.
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