What CPT (Current Procedural Terminology) code is reported for closure of exstrophy of the bladder performed with epispadias repair?

CPT Coding for Closure of Bladder Exstrophy with Epispadias Repair

The correct CPT code for closure of exstrophy of the bladder performed with epispadias repair is 54390.

Understanding Bladder Exstrophy with Epispadias Repair

Bladder exstrophy-epispadias complex (EEC) represents a spectrum of genitourinary malformations that can involve multiple systems including:

• Urinary system
• Musculoskeletal system
• Pelvis and pelvic floor
• Abdominal wall
• External genitalia
• Sometimes the spine and anus

The condition is characterized by a visible defect of the lower abdominal wall with an evaginated bladder plate in classical bladder exstrophy (CEB) or an open urethral plate in males/cleft in females (epispadias) 1.

CPT Code Selection Rationale

The CPT code 54390 specifically describes "Plastic operation on penis for epispadias distal to external sphincter, with incontinence" which encompasses the comprehensive procedure required for epispadias repair when performed with bladder exstrophy closure.

This is distinct from:

• 51940 (Closure, exstrophy of bladder) - This code only addresses the bladder exstrophy component without the epispadias repair
• 51860 (Cystorrhaphy, suture of bladder wound, injury or rupture) - This code is for simple bladder repair, not appropriate for the complex exstrophy-epispadias repair
• 51880 (Closure of cystostomy) - This code is for closure of a surgically created opening in the bladder, not related to congenital exstrophy repair

Clinical Considerations in Exstrophy-Epispadias Repair

The surgical management of bladder exstrophy with epispadias is complex and typically involves:

1. Secure abdominal wall closure
2. Bladder reconstruction with creation of outlet resistance
3. Cosmetic and functional genital reconstruction

In modern staged repair of exstrophy (MSRE), the procedures are often performed in stages:

• Primary closure (typically at 2 months of age)
• Epispadias repair (typically at 18 months)
• Bladder neck reconstruction (typically at 4-5 years) 2

Coding Pitfalls to Avoid

• Don't code only for the bladder component (51940) when both bladder exstrophy and epispadias repair are performed
• Avoid using general bladder repair codes (51860) for this specific congenital condition
• Remember that 54390 is the comprehensive code that includes the work of epispadias repair with incontinence when performed with bladder exstrophy closure

The surgical approach to bladder exstrophy-epispadias complex requires specialized expertise, with the goal of achieving urinary continence, preserving renal function, and providing adequate cosmetic and functional genital reconstruction 1.