Male form of persistent Mullerian duct syndrome type I (hernia uteri inguinalis) presenting as an obstructed inguinal hernia: a case report

<p>Abstract</p> <p>Introduction</p> <p>Persistent Mullerian duct syndrome is a rare form of male pseudo-hermaphroditism characterized by the presence of Mullerian duct structures in an otherwise phenotypically, as well as genotypically, normal man; only a few cases have been reported in the worldwide literature. We report the case of a 30-year-old man with unilateral cryptorchidism on the right side and a left-sided obstructed inguinal hernia containing a uterus and fallopian tube (that is, hernia uteri inguinalis; type I male form of persistent Mullerian duct syndrome) coincidentally detected during an operation for an obstructed left inguinal hernia.</p> <p>Case presentation</p> <p>A 30-year-old South Indian man was admitted to our facility with a left-sided obstructed inguinal hernia of one day's duration. He had a 12-year history of inguinal swelling and an absence of the right testis since birth. Our patient had well developed masculine features. Local physical examination revealed a left-sided obstructed inguinal hernia with an absence of the right testis in the scrotum. Exploration of the inguinal canal revealed an indirect inguinal hernia containing omentum, the left corner of the uterus and a left fallopian tube. Extension of the incision revealed a well formed uterus, cervix and upper part of the vagina attached to the prostate by a thick fibrosed band. Total excision of the uterus, bilateral fallopian tubes and right testis was performed. A biopsy was taken from the left testis. The operation was completed by left inguinal herniorraphy. Histopathological examination of the hernial contents was consistent with that of a uterus and fallopian tubes without ovaries. Both testes were atrophied, with complete arrest of spermatogenesis. Post-operative karyotype analyses were negative for 46,XY and Barr bodies on buccal smear. A semen examination revealed azoospermia with a low serum testosterone level.</p> <p>Conclusions</p> <p>In cases of unilateral or bilateral cryptorchidism associated with inguinal hernia, as in our patient's case, the possibility of persistent Mullerian duct syndrome should be kept in mind in order to prevent further complications such as infertility and malignant change. Hernia uteri inguinalis is the type I male form of persistent Mullerian duct syndrome, characterized by one descended testis and herniation of the ipsilateral corner of the uterus and fallopian tube into the inguinal canal.</p

Experience of establishing and coordinating a nationwide network for bidirectional intussusception surveillance in India: lessons for multisite research studies

Objectives To document and share the process of establishing the nationally representative multisite surveillance network for intussusception in India, coordination, data management and lessons learnt from the implementation.Design This study combined both retrospective and prospective surveillance approaches.Setting 19 tertiary care institutions were selected in India considering the geographic representation and public and private mixParticipants All children under-2 years of age with intussusceptionPrimary and secondary outcome measures The experience of site selection, regulatory approvals, data collection, quality assurance and network coordination were documented.Results The site selection process involved systematic and objective four steps including shortlisting of potential institutions, information seeking and telephonic interaction, site visits and site selection using objective criteria. Out of over 400 hospitals screened across India, 40 potential institutions were shortlisted and information was sought by questionnaire and interaction with investigators. Out of these, 25 institutes were visited and 19 sites were finally selected to participate in the study. The multistep selection process allowed filtering and identification of sites with adequate capacity and motivated investigators. The retrospective surveillance documented 1588 cases (range: 14–652 cases/site) and prospective surveillance recruited 621 cases (range: 5–191 cases/site). The multilayer quality assurance measures monitored and ensured protocol adherence, complete record retrieval and data completeness. The key challenges experienced included time taken for obtaining regulatory and ethical approvals, which delayed completion of the study. Ten sites continued with another multisite vaccine safety surveillance study.Conclusion The experience and results of this systematic and objective site selection method in India are promising. The systematic multistep site selection and data quality assurance methods presented here are feasible and practical. The lessons from the establishment and coordination of this surveillance network can be useful in planning, selecting the sites and conducting multisite and surveillance studies in India and developing countries

Association of meteorological parameters with intussusception in children aged under 2 years: results from a multisite bidirectional surveillance over 7 years in India

Objectives The study aimed to document the association between intussusception in Indian children and meteorological parameters and examine regional variations.Design A bidirectional (retrospective and prospective) surveillance between July 2010 and September 2017.Setting At 20 hospitals in India, retrospective case record review during July 2010 and March 2016 and prospective surveillance during April 2016 and September 2017 were performed.Participants 2161 children aged 2–24 months with first intussusception episode were included.Interventions The monthly mean meteorological parameters (temperature, sunshine, rainfall, humidity and wind speed) for the study sites were collected.Methods The association between monthly intussusception cases and meteorological parameters was examined at pooled, regional and site levels using Pearson (r) and Spearman’s rank-order (ρ) correlation, factorial analysis of variance, and Poisson regression or negative binomial regression analyses.Results The intussusception cases were highest in summer and lowest in autumn seasons. Pearson correlation analysis showed that temperature (r=0.056; p&lt;0.05), wind speed (r=0.134; p&lt;0.01) and humidity (r=0.075; p&lt;0.01) were associated with monthly intussusception cases. Spearman’s rank-order correlation analysis found that temperature (ρ=0.049; p&lt;0.05), wind speed (ρ=0.096; p&lt;0.01) and sunshine (ρ=0.051; p&lt;0.05) were associated with monthly intussusception cases. Poisson regression analysis resulted that monthly intussusception case was associated with rising temperature (North region, p&lt;0.01 and East region, p&lt;0.05), sunshine (North region, p&lt;0.01), humidity (East region, p&lt;0.01) and wind speed (East region, p&lt;0.01). Factorial analysis of variance revealed a significant seasonal difference in intussusception cases for pooled level (p&lt;0.05), 2–6 months age group (p&lt;0.05) and North region (p&lt;0.01). Significant differences in intussusception cases between summer and autumn seasons were observed for pooled (p&lt;0.01), children aged 2–6 months (p&lt;0.05) and 7–12 months (p&lt;0.05).Conclusions Significant correlations between intussusception cases and temperature, humidity, and wind speed were observed at pooled and regional level in India. A peak in summer months was noted, which may be used for prediction, early detection and referral for appropriate management of intussusception