Ventrally Fused Conjoined Twins (Omphaloischiopagus): A Roadmap to Successful Separation.

Conjoined twining is one of the most fascinating and challenging situations which a pediatric surgeon may face in his career. Only few surgeons may have the opportunity to share in separation of such cases. In this report, we aim to share our experience with the successful separation of ventrally fused male conjoined twins (omphaloischiopagus). The case was thoroughly studied via preoperative cross-sectional imaging modalities (magnetic resonance imaging [MRI] and computed tomography [CT] angiography), complemented by data obtained from reviewing similar cases in the literature. A clear delineation of the complex anatomy was achieved preoperatively which proved to be well consistent with the operative findings. A detailed description of the operative procedure to divide/redistribute the shared abdominal/pelvic organs between both twins is provided. To the best of our knowledge, this is the first report to describe the detailed and unique internal anatomy of a common central phallus associating ischiopagus conjoined twins. The penis was centrally located in the perineum in between both twins with an open urethral plate. This common phallus had a peculiar configuration with four crura anchoring ischial bones of both twins together.

How far can one-stage laparoscopic Fowler-Stephens orchiopexy be implemented in intra-abdominal testes with short spermatic vessels?

OBJECTIVE: The aim of the study was to compare the outcome of the prospective cohort who had one-stage laparoscopic Fowler-Stephens orchiopexy (LFSO) with an earlier, similar cohort who had a two-stage LFSO. Both cohorts included only intra-abdominal testes (IATs) with short spermatic vessels. STUDY DESIGN: The present study initially included all patients between the age of six months and preschool age, presenting with an IAT to the authors' tertiary clinic from January 2012 to December 2014. Only intra-abdominal testes with short testicular vessels, correlating to type 3A, 3B and 4A as per the updated Ain Shams classification, were included in this study. One-stage LFSO was performed on all patients. Patients with bilateral intra-abdominal testes had the procedure performed on the contralateral side 6-8 weeks later. Follow-up was performed at 6, 12 and 36 months postoperatively by means of Doppler ultrasound in the first two visits. A similar subgroup of the historical cohort from years 2002 to 2010 had a two-stage LFSO, with the second stage being performed 12-16 weeks later. In bilateral cases, a three-interval surgery based on two-stage LFSO was performed, with a follow-up at 6 months postoperatively. RESULTS: The recent cohort included 16 IATs (10 unilateral and 3 bilateral). The median age of the patients was 1.41 years. At 6-month follow-up, 4 testes were found atrophic (25%), whereas the remaining 12 testes (75%) were viable. In those viable, only half of them had a low scrotal position. No difference was found at 12- or 36-month follow-up. Doppler ultrasound confirmed adequate intratesticular blood flow at 6 and 12 months postoperatively. In the similar subgroup of the historical cohort, 3 of 25 (12%) testes were found atrophied at six months postoperatively, with only one testis (4%) having a high scrotal position. In a comparison of both cohorts, the two-stage LFSO was found to be associated with a halving of the testicular atrophy rate (p value = 0.401) and a higher incidence of low scrotal position (p value = 0.004). A comparison of both cohorts is shown in the summary table. CONCLUSION: Although one-stage LFSO may seem tempting, it still holds a higher rate of testicular atrophy, which is not justified. LEVEL OF EVIDENCE: Level III (Case-control study).

Y-Type Urethral Duplication: A True Variant of the Anomaly or a Misnomer?

Objectives The objective of this study was to define anatomical and radiological features of the so-called Y-type urethral duplication. Methods The study included four male patients and one female patient with congenital connection between the urogenital tract and the external anal orifice. Investigations included renal sonography, urethrograms, and magnetic resonance imaging pelvis in the last patient. The urethrograms of male patients were carefully reviewed, in addition to available urethrograms of similar cases that could be obtained through searching the literature. Results Unlike cases of urethral duplication, the male patients had always a complete prepuce and a functioning anterior urethra in 25%. The accessory uroanal channel had almost always a constant origin from the posterior urethra. Some tension seems to be exerted by the urethroanal tract pulling on and causing a kink in the posterior urethra. Management was simple in patients without anterior urethral hypoplasia (one male and the female patient). Both were treated by simple excision of the communicating ano-urogenital tract through a perineal approach with an excellent outcome. Histopathological examination of excised tracts revealed stratified squamous cell in the former and transitional cell lining in the latter. In patients with hypoplastic anterior urethra, staged urethral reconstruction was performed in two, and progressive dilatation of hypoplastic anterior urethra was tried in the last patient. Conclusion Several observations would support diagnosing the congenital connection between the urinary tract and the external anal orifice in the male as a congenital fistula rather than an accessory urethra. Confirming and accepting this information may have its impact on changing the current surgical approach.