Aphallia: a review to standardize management

Area of Science:

• Pediatric Surgery
• Urology
• Medical Genetics

Background:

• Congenital aphallia is an extremely rare congenital anomaly.
• Limited literature exists regarding its management, leading to controversial treatment strategies.

Purpose of the Study:

• To review the most recent English literature on congenital aphallia.
• To focus on a staged management approach for affected individuals.

Main Methods:

• A comprehensive PubMed search was conducted for English literature published in the last 10 years using the term 'aphallia'.
• Articles were screened, and relevant papers were included for analysis, with additional papers identified from reference lists.

Main Results:

• Management strategies were categorized into three distinct phases: short-term, intermediate-term, and long-term.
• Short-term management involves resuscitation and urinary diversion.
• Intermediate-term care includes fistula division and neophallus creation, while long-term care focuses on neophalloplasty, urethroplasty, and prosthetic implantation.

Conclusions:

• A staged, multidisciplinary approach is crucial for managing congenital aphallia.
• Optimal management involves initial resuscitation and urinary diversion, followed by reconstructive procedures and long-term urinary tract protection.
• Shared decision-making with patients and families is integral to the treatment process.