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.
CONCLUSIONS: 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.

UNASSIGNED: Aphallia (absent penis) is an extremely rare congenital anomaly happening in 1 in 30 million births worldwide. It can occur alone or in combination with other congenital anomalies.
UNASSIGNED: A two-day-old neonate presented to Arba Minch General Hospital with an absent penis. The delivery was performed at home by a 34-year-old primigravida mother with no antenatal follow-up. As the mother described, the baby cried immediately after birth. The baby was sucking initially but failed to suck hours after delivery. The neonate died of neonatal distress syndrome after two hours of resuscitation.
UNASSIGNED: Aphallia is a complex, extremely rare congenital anomaly that is commonly associated with other congenital anomalies. Sometimes, it may be associated with a lethal anomaly and requires sophisticated workup to diagnose and treat grave anomalies early.

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.

Aphallia is a very uncommon congenital condition with an incidence of about one in 30 million births. It is characterized by the absence of a penis which is due to the failure of the genital tubercle to form or develop fully. In this case report, we present a 3-month-old male infant with aphallia from a remote part of Eritrea who was subsequently lost to follow-up and discuss the challenges that are faced when dealing with such a complex congenital anomaly in a developing country. This report highlights the importance of having accessible healthcare services for early detection, appropriate management, and counseling of the affected individual and their families. Finally, healthcare providers should work together in the future to improve the quality of life of affected individuals and their families as the management of aphallia requires multidisciplinary collaboration and comprehensive care to reduce the potential long-term psychological and social consequences of the condition.

Aphallia is a rare congenital disorder pertaining to genotypic males. Early surgical creation of a neophallus is recommended to reinforce the child\'s male gender-identity, favoring proper psychosexual development. Modern microsurgical techniques used to create a neophallus in adults are not recommended in children due to the invasiveness and complexity of the procedures, along with high complication rates. Scrotal flap phalloplasty is a simple and reproducible technique to create a temporary neophallus in prepubertal boys with aphallia.
We present a multi-institutional experience, ten years after the initial description of the scrotal flap phalloplasty (SFP) technique, in which a flap from the well-developed scrotum is used to build a temporary neophallus, without obvious scars in patients with aphallia.
The records of surgical neophalloplasty for aphallia patients from 4 centers between 2011 and 2021 were reviewed. All patients had at least one year follow-up to assess for short and long-term complications. Age at initial operation, associated anomalies, and other related surgical procedures were analyzed.
The post-operative aesthetic result in all patients was satisfactory and has been maintained in the long-term follow-up, with all patients presenting a cylindrical structure resembling an uncircumcised penis, without evidence of significant contraction or loss of length. (Summary Figure) DISCUSSION: Non-microsurgical neophalloplasty techniques in patients with penile agenesis are temporary procedures that help to establish the body image and preserve the psychosexual development of the patient with aphallia. These techniques do not involve tissue transplant from a distant region, and are simpler to perform, with less scarring at the donor sites. Due to significant donor scars and considerable morbidity and complexity associated with the definitive phalloplasty techniques, we created a simple, reproducible and straightforward procedure to serve as a temporary neophallus in young boys with aphallia. As affected patients usually have a well-formed scrotum with normal and orthotopic testicles, it is the ideal donor site for a temporary neo-phallus in childhood. Furthermore, other donor sites are preserved for a definitive phalloplasty. There are limitations to this study, as quality of life could not be assessed and psychological or gender-identity investigations have not been carried out. None of these children have reached puberty, and hence decision and outcomes of definitive neophallus reconstruction has not been considered to date.
Scrotal flap phalloplasty is a minimally invasive, simple and reproducible technique used to create a temporary neophallus in boys with aphallia, while waiting for definitive reconstructive surgery after puberty.

Aphallia or penile agenesis is a rare case of the genitourinary system which has an association with upper urinary tract disorder. Failure of fetal genital tubercle formation in the embryonic period is the cause of this disorder. The incidence of aphallia according to previous studies is about 1 in 10-30 million births. We report a case of a child with the absence of the penis with associated kidney hypoplasia and vesicorectal fistula.

Fashioning a functioning neo urethra in a boy with aphallia is one of the unsolved problems in this condition.
We present our technique and outcome of operative exposure and neo urethral construction in four aphallic boys.
Retrospective study of the records of four aphallic boys operated in the period 2015-2019 was undertaken. The demographics, presentation, operative procedure, current follow up and voiding outcome was noted.
The neo phallus was constructed by the De Castro technique in four aphallic boys aged 1-8 years. Pre operative assessment revealed bladder outflow obstruction in two and urinary incontinence in one boy. The operative exposure afforded after reflection of the De Castro flap was utilized in doing a limited pubic symphysiectomy. This exposed the bladder neck, urethra and the urethral termination into the ano rectum. The healthy native urethra was isolated by disconnecting it from its ano rectal termination and a neo urethra constructed from a segment of proximal sigmoid colon by its reconfiguration into a Monti type tube. The neo urethra was then anastomosed to the native urethra and laid within the bed of the De Castro flap so as to reach the neo phallic termination. The De Castro flap was then tabularized over the neo urethra to form the new phallus. Appendicular or ileal Monti Mitrofanoff was also added to the reconstruction. Three boys are voiding well. One boy had a partial bladder outflow obstruction resulting from operative correction of incontinence and is dry on intermittent catheterization. Follow up was for 1-4 years and upper tracts are stable or improved.
We describe the transpubic approach to the construction of a reliable neo urethra in the form of a Monti tube from the sigmoid colon in four boys with aphallia. We believe this to be a useful addition to the De Castro procedure for neo phallic construction.

UNASSIGNED: Penile agenesis occurs in 1 in 30 million births. The cause of this anomaly is the failure of development of genital tubercle. Case Report: A 1-day-old neonate was born with complete penile agenesis. Imaging investigations showed an ectopic urethral insertion in the anterior wall of the rectum, and a grade 3 left vesicoureteral reflux. Conclusion: Penile agenesis is a complex genital abnormality. It can be associated with urinary malformations that should be systematically investigated.

Aphallia or penile agenesis is very rare congenital anomaly of unknown cause occurring 1 in 30 million live births. Very little has been written in literature about aphallia. There is absent phallus and urethra may open abnormally in perineum or into rectum posing various surgical, social and psychological implications as the child grows. We are presenting 03 cases of aphallia with associated congenital anomalies such as unilateral renal agenesis, bilateral undescended testes, anorectal malformation and rectovasical fistula.

Congenital aphallia is a rare anomaly with little supporting literature and controversial management. The aim of this review is to assess the most recent literature with a focus on staged management of these cases. We performed a PubMed search of all English literature in the past 10 years using the term aphallia. Twenty-three articles were identified of which six were excluded. A further three papers meeting our criteria were found in the references to papers initially identified. We found that management can be staged in three phases: short, intermediate and long-term. We conclude that optimal short-term management centers on resuscitation and urinary diversion as necessary, intermediate-term management entails urethrorectal fistula division, urethrostomy and neophallus creation and long-term management results in successful neophalloplasty, urethroplasty, prosthetic implant and continued protection of the upper urinary tracts with a Mitrofanoff. All this within a multidisciplinary team ensuring shared decision-making with the patient and their family.