Individualized care for patients with intersex (differences of sex development): Diagnosis and treatment of aphallia.

This review discusses issues and concerns in the management of aphallia, updating status of a post-pubertal individual who required further surgery after having initial surgery for aphallia as an infant. Through this case, which discusses an 18-year-old young adult who had penile agenesis, who desired further phalloplasty involving glanuloplasty and implantation of an erectile device, we highlight the importance of periodic evaluation and close follow up. Surgery during infancy or early childhood to create a penis is important for gender development in a boy, especially if there were functional testes during fetal life, even if this surgery would only be the first stage. There is a strong probability of subsequent surgery after initial phalloplasty before puberty, even with the use of currently refined techniques. Here we discuss the changing techniques that document the ongoing, continued refinement of these procedures, highlighting that further outcome data are needed to identify ways to further optimize current techniques.

Safety and outcomes analysis: transcatheter implantation of autologous angiogenic cell precursors for the treatment of cardiomyopathy.

BACKGROUND: Stem cell transplantation is an emerging therapy for severe cardiomyopathy, proffering stem cell recruitment, anti-apoptosis, and proangiogenic capabilities. Angiogenic cell precursors (ACP-01) are autologous, lineage-specific, cells derived from a multipotent progenitor cell population, with strong potential to effectively engraft, form blood vessels, and support tissue survival and regeneration. METHODS: This IRB approved outcome analysis reports upon 74 consecutive patients who failed medical management for severe cardiomyopathy, and were selected to undergo transcatheter intramyocardial or intracoronary implantation of ACP-01. Serious adverse events (SAEs) were reported. Cell analysis was conducted for each treatment. The left ventricular ejection fraction (LVEF) was measured by multi-gated acquisition scan (MUGA) or echocardiogram at 4 months ± 1.9 months and 12 months ± 5.5 months. Patients reported quality of life statements at 6 months (± 5.6 months). RESULTS: Fifty-four of 74 patients met requirements for inclusion (48 males and five females; age 68.1 ± 11.3 years). The mean treatment cell number of 57 × 106 ACP-01 included 7.7 × 106 CD34 + and 21 × 106 CD31 + cells with 97.6% viability. SAEs included one death (previously unrecognized silent MI), ventricular tachycardia (n = 2) requiring cardioversion, and respiratory infection (n = 2). LVEF in the ischemic subgroup (n = 41) improved by 4.7% ± 9.7 from pre-procedure to the first follow-up (4 months ± 1.9 months) (p < 0.004) and by 7.2% ± 10.9 at final follow-up (n = 25) at average 12 months (p < 0.004). The non-ischemic dilated cardiomyopathy subgroup (n = 8) improved by 7.5% ± 6.0 at the first follow-up (p < 0.017) and by 12.2% ± 6.4 at final follow-up (p < 0.003, n = 6). Overall improvement in LVEF from pre-procedure to post-procedure was significant (Fisher's exact test p < 0.004). LVEF improvement was most marked in the patients with the most severe cardiomyopathy (LVEF < 20%) improving from a mean 14.6% ± 3.4% pre-procedurally to 28.4% ± 8% at final follow-up. Quality of life statements reflected improvement in 33/50 (66%), no change in 14/50 (28%), and worse in 3/50 (6%). CONCLUSION: Transcatheter implantation of ACP-01 for cardiomyopathy is safe and improves LVEF in the setting of ischemic and non-ischemic cardiomyopathy. The results warrant further investigation in a prospective, blinded, and controlled clinical study. TRIAL REGISTRATION: IRB from Genetic Alliance #APC01-001, approval date July 25, 2022. Cardiomyopathy is common and associated with high mortality. Stem cell transplantation is an emerging therapy. Angiogenic cell precursors (ACP-01) are lineage-specific endothelial progenitors, with strong potential for migration, engraftment, angiogenesis, and support of tissue survival and regeneration. A retrospective outcomes analysis of 53 patients with ischemic and non-ischemic dilated cardiomyopathy undergoing transcatheter implantation of ACP-01 demonstrated improvements in the left ventricular ejection fraction of 7.2% ± 10.9 (p < 0.004) and 12.2% ± 6.4, respectively, at 12 months (± 5) follow-up. Quality of life statements reflected improvement in 33/50 (66%) patients.

Neo-phalloplasty in children and adolescents for acquired penile loss.

INTRODUCTION: From 2000 to 2019, the De Castro's neo-phalloplasty was used in 47 patients with congenital and acquired penile loss. PURPOSE: Herein, the technical aspects of penile reconstruction and the outcomes in 17 children and adolescents treated for total or sub-total acquired penile loss are reported. MATERIAL AND METHODS: The median age at the time of injury was 3 months (range: 2 days-15 years). Twelve patients were born with normal penis but suffered injuries (11) or underwent surgical resection of the penis (1). The remaining 5, affected by bladder exstrophy (2) or cloacal exstrophy (3), had penile loss due to surgical complications. RESULTS: The median age at the time of surgery was 5 years (range: 2-20 years). The median length of the procedure was 5 h (range 4-8 h). Skin expander were inserted in preparation of phalloplasty in 9 patients. Corpora-cavernosa remnants were found and incorporated in the neophallus in 12 patients. In 7 patients, urethral remnants were also incorporated into the repair, placing the meatus at the tip of the neophallus. In the remaining patients the urethra was left in the acquired position after penile loss creating a perineal (2), scrotal (3), peno-scrotal (3), or posterior penile (1) urethrostomy. The first patient of this series was the only patient to receive simultaneously phalloplasty and total urethroplasty, with failure of urethral reconstruction. The median follow-up was 2 years (range 1-11 years). The overall complication rate was about 47%. All of the complications were late and required surgical revision. The results were assessed regarding overall functionality (voiding, erection/erogenous areas, masturbation/sexual intercourse) and aesthetic outcome using subjective and objective parameters. Psychological evaluation for both patient and parents was performed in 4 cases. DISCUSSION: There are no specific guidelines for treating significant penile injuries in the pediatric population. We do not think secondary severe penile impairments should wait until adult age for repair. In this specific subgroup of patients technical aspects differ from phalloplasty done for congenital aphallia, due to the possible presence of corpora-cavernosa/urethral remnants and the feasibility to perform a "functional phalloplasty" with very encouraging results. CONCLUSION: The De Castro's neo-phalloplasty remains one of the few techniques available for total penile reconstruction in pediatric age group. Data demonstrate that this technique for acquired aphallia is feasible and reproducible, however, it is a challenging procedure with a high complication rate and likelihood of revisions.

A salvage procedure for redo penile prosthesis implantation in phalloplasty (De castro technique) for congenital aphallia.

INTRODUCTION: We present a different salvage procedure for penile prosthesis implantation in a De Castro neophallus. METHODS: A patient with congenital aphallia had been operated at the age of 11. Ten years later he had two non successful attempts of prosthesis implantation. Surgery consisted of adapting the implants into the aortic graft and folding it in the middle over the cylinders to reinforce its strength. RESULTS: Patient had a successful postoperative course and has now 6 months of follow-up. DISCUSSION: In a systematic review of penile prosthetic outcomes and complications in gender-affirming surgery, most of the prostheses were inflatable (83.6%) and single-cylinder (61.0%). At a mean follow-up of 3.0 years: 36.2% of prosthesis complications, 60.0% of patients had their original implant present and 83.9% reported achieving penetration. We decided to use vascular grafts as a scaffold because of former non successful attempts. CONCLUSION: The use of vascular prosthesis as a device to implant the penile prosthesis seems to be a safe and effective alternative in an attempt to offer erectile function to the neophallus in case of congenital aphallia.

Neophalloplasty in boys with aphallia: A systematic review.

OBJECTIVE: Aphallia is a rare penile congenital abnormality. The aim of this systematic review was to assess all available literature on neophalloplasty in infancy with an interest in technical alternatives and clinical outcome. MATERIALS AND METHODS: We performed a Pubmed search using the terms: neophalloplasty, neophallus, and phalloplasty, and selected articles that presented technical alternatives for penile construction in cases of penile dysgenesis, especially aphallia in children and/or adolescents. RESULTS: A total of 319 articles were identified in the initial search. Among the different techniques presented in 19 papers collected, we categorized reconstructive procedures into two groups: microsurgical and non-microsurgical techniques. Among the microsurgical techniques, procedures such as the osteocutaneous fibular flap (n = 18), the myocutaneous latissimus dorsi flap (n = 24), and the radial forearm free flap (n = 293) were reported. Among the non-microsurgical techniques, we found a wider range of technical options (6 in total). Most were based on groin, abdominal, and scrotal flaps. Long-term reports on the functionability of the neophallus and psychological aspects of the patients are lacking. CONCLUSION: In assessing recent literature, it seems rational that neophalloplasty should be regarded as the preferred strategy for boys born with aphallia, in opposition to female gender assignment.

Soroprevalência de Maedi-visna em rebanhos ovinos do estado do Maranhão, Brasil

Apesar de ter sido relatada em vários Estados, não há informação sobre o Vírus da Maedi Visna (MVV) no Maranhão, e com o crescimento de sua ovinocultura, aumenta o fluxo de animais de outras regiões. Com isso objetivou-se determinar a soroprevalência do MVV em rebanhos ovinos das três principais mesorregiões produtoras do Estado do Maranhão, através da pesquisadas de 1.495 amostras sanguíneas de ovinos, com idade superior a seis meses, pertencentes a 83 rebanhos de 23 municípios das mesorregiões Cento, Leste e Norte. O diagnóstico sorológico da infecção pelo vírus MVV foi realizado por meio do teste de imunodifusão em gel de ágar (micro-IDGA). Constatou-se uma prevalência geral de 0,7% (IC95%:0,4-1,3%) de ovinos soropositivos e prevalências nas mesorregiões Centro, Leste e Norte de 0,5% (IC95%:0,1-1,4%), 0,7% (IC95%:0,2-1,8%) e 1% (IC95%:0,3-2,4%) respectivamente. Em relação à variável sexo, não foi observado diferença significativa (P>0,05) entre machos (0,5%, IC95%:0-2,7%) e fêmeas (0,8%, IC95%:0,4-1,4%), assim como quanto a genética comparando ovinos de raças puras (1,5%, IC95%: 0,4-8,1%), mestiços (1%, IC95%:0,4-2,0%) e SRD (0,3%, IC95%:0,04-1,1%). A análise quanto a idade não demonstrou diferença significante (P>0,05). Conclui-se que a infecção pelo MVV está presente em ovinos das mesorregiões estudadas, sendo este o primeiro registro desta enfermidade no Estado do M

Phalloplasty and urethroplasty in a boy with penile agenesis.

OBJECTIVE: New concepts in ambiguous genitalia have questioned the strategy of female gender reassignment in cases of penile agenesis. Hence, we present a video of the construction of a phallus according to the De Castro technique as an alternative approach for this devastating condition. METHODS: A 12-year-old boy with congenital penile agenesis and posterior urethra emerging in the distal rectum was selected. An ASTRA approach was used and a phallus was constructed with a skin flap from the abdominal wall, tubularized to produce a phallic appearance. A buccal mucosa graft was tubularized to create the new urethra. RESULTS: Immediate outcome was excellent. Partial dehiscence of the dorsal urethral sutures occurred and the patient started voiding through a scrotal urethrostomy at 9 months postoperatively. Phalloplasty provided an adequate male appearance with a good cosmetic aspect. CONCLUSION: The technique is a feasible alternative. A procedure in 2 stages by first creating the neourethra at a later date might be an interesting option to try to avoid the urethral complications observed. Long-term follow-up is however necessary to confirm the initial results.

Surgical treatment of high urogenital sinuses using the anterior sagittal transrectal approach: a useful strategy to optimize exposure and outcomes.

PURPOSE: Surgical management of the high urogenital sinus remains challenging. The anterior sagittal transrectal approach provides optimal exposure, facilitates vaginal dissection and separation from the urethra, and allows reconstruction of the bladder neck musculature. In this study we report our initial experience with this technique. MATERIALS AND METHODS: We performed a retrospective review of a 6-year multi-institutional experience treating patients with a urogenital sinus anomaly using the anterior sagittal transrectal approach without preoperative colostomy or prolonged postoperative fasting. Variables analyzed included patient age, associated malformations, the need for additional procedures and surgical outcomes. RESULTS: A total of 23 children with a mean age of 2.3 years (range 3 months to 17 years) who underwent surgery between 2003 and 2010 were included in the study. Mean followup was 3.4 years (range 14 months to 7 years). All children had a high urogenital sinus with (16) or without (7) congenital adrenal hyperplasia. There were 3 isolated cases treated with additional procedures. Only 1 anterior sagittal transrectal approach related complication was encountered when a perineal infection developed in a child and required temporary diverting colostomy without compromising the repair. There were no postoperative urethrovaginal fistulas. All toilet trained patients were continent for feces and most were voiding normally per urethra (21), except for 2 with associated urological malformations. There were 15 patients who underwent followup examination under anesthesia, and demonstrated separate urethral and vaginal openings. CONCLUSIONS: The anterior sagittal transrectal approach provides excellent exposure for the management of a high urogenital sinus, facilitating the separation of urogenital structures. Good outcomes in terms of urinary/fecal continence as well as the absence of urethrovaginal fistulas were achieved in the majority of cases, supporting its consideration for the surgical management of this congenital abnormality.

Retrograde ureteral access after cross-trigonal ureteral reimplantation: A straightforward technique.

PURPOSE: Retrograde ureteral access after cross-trigonal ureteral reimplantation can be challenging. We present our experience with percutaneous retrograde ureteral catheterization, status post cross-trigonal ureteral reimplantation. MATERIALS AND METHODS: We evaluated all patients who underwent attempted percutaneous retrograde ureteral catheterization after cross-trigonal ureteral reimplantation. All clinical data, radiographic images and operative reports were reviewed. Ureteral access was obtained by percutaneously entering the bladder with an intravenous needle/catheter under cystoscopic guidance. The needle was then removed leaving the catheter in place. The ureteral orifice was then accessed through the intravenous catheter by a ureteral access wire and/or ureteral catheter under cystoscopic guidance. RESULTS: From 1978 to 2008, 13 patients (11 boys and 2 girls) with a history of cross-trigonal ureteral reimplantation underwent percutaneous retrograde ureteral catheterization. The procedure was unilateral in 12 patients and bilateral in one. Indications for the procedure included: retrograde pyelography and double-J stent insertion for ureteropelvic junction or ureterovesical junction obstruction (10); removal of migrated stent (2); and treatment of a ureteral stone (1). The procedure was performed successfully in all patients and without complications. CONCLUSIONS: Percutaneous retrograde ureteral catheterization is a safe, straightforward, and effective modality for obtaining retrograde ureteral access in children, status post cross-trigonal ureteral reimplantation.

Histological evaluation of an alternative method of neophalloplasty based on two lower abdominal skin flaps and simultaneous buccal mucosa graft in the ventral surface of the neophallus (two-stage urethroplasty): experimental study in rabbits.

OBJECTIVE: To evaluate, in an experimental study in rabbits, a new model of neophalloplasty based on two lower abdominal skin flaps and ventral buccal mucosa graft for planned two-stage urethroplasty procedure. MATERIAL AND METHODS: Sixteen rabbits were operated and divided into four equal groups which were sacrificed at 2, 4, 8 and 12 weeks. The inflammatory pattern, presence of sub-epithelial fibrosis and epithelial changes in the grafted area were evaluated histologically. RESULTS: There were no deaths and no dehiscence of the wound was seen. One animal in the 2-week group developed an ulcer in the grafted area. We found minimal contracture of the neophallus, but this was not statistically significant between groups. Buccal mucosa graft showed good uptake in all groups, with vascular support from subcutaneous tissue of the flaps. The grafted area developed epithelial metaplasia, showing a decrease in cell layers with time, with disappearance of the sub-epithelial papillae and appearance of stratum granulosum and keratinization of the epithelial graft surface. A decrease in sub-epithelial fibrosis with replacement of immature by mature (eosinophilic) collagen was found. In the later groups was also observed an important decrease in inflammatory response, and the chorion of the grafted area presented a dilated capillary network, indicating that the process of neoangiogenesis was effective. CONCLUSION: Buccal mucosa displayed histological integration in the abdominal flaps with epithelial metaplasia in all groups. The surgical aspect of the neophallus was cosmetically acceptable, with minimal contracture.

Phalloplasty and urethroplasty in children with penile agenesis: preliminary report.

PURPOSE: Female gender has been assigned to 46,XY newborns affected by aphallia, possibly resulting in subsequent gender dysphoria. Prenatal and postnatal effects of the androgens on the brain and sexual orientation cannot be modified later. Therefore, patients affected by aphallia should be raised as males. Because definitive forearm flap phalloplasty is generally not recommended before puberty, we performed a preliminary penile reconstruction during childhood in 4 patients. MATERIALS AND METHODS: Four patients with aphallia who had no sex reassignment at birth were treated at age 9, 17 and 36 months, and 12 years in a single operation. The urethral channel was dissected through an anterior-sagittal-transanorectal approach, and then phalloplasty and urethroplasty were carried out using an abdominal skin flap and a bladder/buccal mucosa free graft. RESULTS: Immediate postoperative outcome was excellent in all the patients. One patient had excellent functional and cosmetic results at 5 years, while 2 had a partial dorsal urethral dehiscence resulting in an epispadiac urethra at 2 years, and 1 had necrosis of the distal urethra and was voiding through a scrotal urethrostomy at 9 months postoperatively. Phalloplasty survived and provided an adequate male appearance in all patients. CONCLUSIONS: Opposite gender should not be assigned in patients affected by penile agenesis, who are better raised according to their karyotype and hormonal production. Definitive phalloplasty in adults may achieve good results. Nevertheless, this procedure is generally performed in postpubertal boys and it is not easily available everywhere. Therefore, we believe that social and psychological concerns justified this type of phalloplasty as a palliative preliminary procedure in 3 of our patients. In those countries where definitive forearm phalloplasty is not available our method may also be justified in older children (as in 1 of our patients) as an attempt at a definitive procedure.

Treatment of vaginal atresia at a missionary hospital in Bangladesh: results and followup of 20 cases.

PURPOSE: We report a 9-year experience with successful treatment of patients with vaginal atresia at a missionary hospital with decreased facilities in Bangladesh. MATERIALS AND METHODS: From 1995 to 2002, 20 patients 10 to 29 years old (average age 18.4) with Mayer-Rokitansky-Kuster-Hauser syndrome underwent total vaginal replacement. Ten of the 20 females were married and the anomaly was discovered after marriage. In the remaining 10 cases the diagnosis was suspected by the parents because of absent menstruation. In all patients the neovagina was created using a 12 to 14 cm segment of distal sigmoid colon. RESULTS: Short-term morbidity was minimal. At the long-term followup, which was available for 16 patients, the neovagina had a good-appearing introitus. No stenosis, stones or colitis was reported. Six patients already had an active sexual life, which was reported to be satisfactory. Five couples had already adopted 1 or more children. CONCLUSIONS: Good perioperative preparation and assistance, assurance of cyclical followup and a trained surgical team permitted successful treatment of a complex genital malformation at a missionary hospital with modest services. Sigmoid vaginoplasty in a developing country seems to be the best choice because of simple management and followup. Young women unable to procreate because of vaginal atresia seem to have an unexpected normal family and social acceptance in Bangladesh after complete vaginal replacement.

Estudio sobre la situación de los recursos humanos en servicios cardiológicos en la República Dominicana / Study on the status of human resources in cardiology services in the Dominican Republic

El documento consta de cuatro capítulos, en éstos se aborda la distribución de los cardiólogos en la República Dominicana, su formación, en donde se contempla la situación de las residencias, el perfil educativo y ocupacional y recomendaciones de los talleres para la formación de estos profesionales. También presenta la situación de la atención cardiológica, aquí se desglosa información sobre la prestación de servicios hospitalarios y consulta externa, la disponibilidad de camas, los recursos humanos y la producción y rendimiento del servicio de cardiología. Finalmente apunta algunas conclusiones y recomendaciones al respecto