Aphallia - congenital absence of the penis: a systematic review.

BACKGROUND: Aphallia is a rare congenital anomaly often associated with other urogenital anomalies. The management of aphallia cases for both the immediate and long-term treatment of patients with aphallia pose a major dilemma. Patients are at risk for psychosocial and psychosexual challenges throughout life. METHODS: A systematic review was conducted on aphallia cases. We searched online databases until March 2023 for relevant articles and performed according to the PRISMA-P guidelines. RESULTS: Of the 43 articles screened, there were 33 articles included. A total of 41 patients were analyzed qualitatively. Asia is the region with the most aphallia cases with 53% (n:22), while the United States is the country with the most most reported aphallia cases 31% (n:13). Most cases were identified as male sex (n: 40), and most cases were neonate with 68% (n:28) cases. Physical examination generally found 85% (N = 35) with normal scrotal development and palpable testes. The most affected system with anomalies is the genitourinary system with fistulas in 80% (n:29) cases. Initial management in 39% (n:16) of patients involved vesicostomy. Further management of 31% (n:13) included phalloplasty or penile reconstruction, and 12% (n:5) chose female sex. 17% (n:7) of patients refused medical treatment or were lost to follow-up, and 12% (n = 5) patients deceased. CONCLUSION: Aphallia is a rare condition and is often associated with other inherited genitourinary disorders. In most cases, physical examinations are normal except for the absence of a phallus, and laboratory testing shows normal results. The initial management typically involves the vesicostomy procedure. Subsequent management focuses on gender determination. Currently, male sex is preferred over female. Due to the significant variability, the rarity of cases, and the lack of long-term effect reporting in many studies on aphallia, further research is needed to minimize bias.

Risk factors for urethrocutaneous fistula following hypospadias repair surgery in Indonesia.

INTRODUCTION: Hypospadias is one of the most common congenital malformations with a worldwide increasing trend over the years. Despite advancements in hypospadias repair, complications still occur. One of the most common complications of hypospadias repair surgery is Urethrocutaneous fistula. Studies attempting to analyze the association between the complication and risk factors are always beneficial, especially for studies performed in different areas of the world. We hypothesize that several evaluated risk factors among Indonesian hypospadias patients could be associated with the occurrence of urethrocutaneous fistula after the repair procedure. OBJECTIVE: To determine the risk factors associated with urethrocutaneous fistula after hypospadias repair surgery by collecting and analyzing data obtained from multiple centers in Indonesia. MATERIALS AND METHODS: A nationwide, retrospective study with 12 hospitals in Indonesia of children with a diagnosis of hypospadias was conducted. The collected data, taken from patients admitted in 2018, from each center's medical records consisted of patient identity, repair technique used, neourethra length, percutaneous cystostomy, and splint size as independent variables speculated to be possible risk factors correlated to the presence of urethrocutaneous fistulae. Binomial logistic regression analysis was performed using SPSS 21.0 to determine the relationship between urethrocutaneous fistulae as a post-repair complication and possible risk factors. RESULTS: We collected 591 hypospadias cases from 12 centers in 9 cities in Indonesia. Most patients came when they were already at the age of more than four years old (60.4%). The chordee-only and failed urethroplasty groups are excluded from the analysis as they are not classified as true hypospadias. Most repairs were performed by using the Tubular Incised Plate (TIP) with Thiersch-Duplay technique (44.16%). Most of the reconstructed neourethra are 2-3 cm in length (32.13%). The 8 Fr urethral splint (46.41%) was mostly used during the operation. Most surgeons decided not to perform cystostomy throughout the procedure (61.03%) based on personal preferences. Urethrocutaneous fistula was found in 80 patients (15.27%) out of the total patients who underwent the surgery. The binomial logistic regression analysis shows that age (OR = 1.398, p = 0.015), the decision to not perform cystostomy (OR = 2.963, p = 0.014), and splint size (OR = 1.243, p = 0.023) are significantly associated (p < 0.05) with the development of urethrocutaneous fistula. CONCLUSION: Age and splint size are significant risk factors for urethrocutaneous fistula after hypospadias repair in Indonesia, whereas performing percutaneous cystostomy during the repair decreases the risk for urethrocutaneous fistula occurrence.

Common Practice of Hypospadias Management by Pediatric Urologists in Indonesia: A Multi-center Descriptive Study from Referral Hospitals.

BACKGROUND: Hypospadias is the second most common congenital anomalies among human congenital disabilities. There are over 300 surgery techniques being introduced to treat hypospadias. The successful of hypospadias repair is assessed by several outcomes as well as complications following surgery. AIM: This study aims to show the multicenter hypospadias data in Indonesia descriptively. METHODS: All the data were compiled based on questionnaires, which were distributed to Indonesian pediatric urologists. The questionnaire includes several questions containing demographic aspect, preferred techniques being used, and complications being found regarding hypospadias repair. RESULTS: Eighteen Indonesian pediatric urologists from 12 centres involved in this study. The data were collected from June - September 2018 based on the surgeon's experience throughout 2017. From 591 cases based on the returned questionnaire, penile-type hypospadias was the most common type of hypospadias being treated (35.7%) followed by penoscrotal (28.9%) and scrotal-type (12.9%). Moderate severity of chordee was mostly seen among all cases (40.6%). Tubularised incised plate (TIP), + Thiersch Duplay, was the most common technique being used to treat hypospadias (44.3%), followed by onlay island preputial flap (14.9%) and two-stage technique (14%). The incidence of urethrocutaneous fistulae in this study was 13.9%. CONCLUSION: This study showed how Indonesian pediatric urologists dealt with hypospadias cases. TIP + Thiersch Duplay procedure being the preferred technique used by most participants and the rate of urethrocutaneous fistulae as one of the complications was comparable with previous studies.