Congenital absence of bladder and urethra
ICD-10 Q64.5 is a billable code used to indicate a diagnosis of congenital absence of bladder and urethra.
Congenital absence of the bladder and urethra is a rare but significant congenital malformation characterized by the complete or partial absence of these structures, leading to severe urinary dysfunction. This condition often presents in conjunction with other urinary tract anomalies, such as renal agenesis or bladder exstrophy. The absence of the bladder can result in the inability to store urine, leading to continuous leakage and necessitating surgical intervention for management. Patients may also experience associated complications, including recurrent urinary tract infections and renal impairment. Diagnosis is typically made through imaging studies such as ultrasound or MRI, which can reveal the absence of the bladder and any associated renal anomalies. Management often involves a multidisciplinary approach, including pediatric urologists, nephrologists, and geneticists, to address the complex needs of affected individuals. Early intervention is crucial to improve outcomes and quality of life for these patients.
Detailed clinical notes on urinary function, growth parameters, and developmental milestones are essential.
Management of infants with congenital absence of bladder during NICU stay, surgical planning for bladder reconstruction.
Consideration of the psychosocial impact on families and the need for long-term follow-up.
Genetic testing results, family history of congenital anomalies, and any syndromic associations must be documented.
Genetic counseling for families with a history of congenital urinary tract anomalies.
Awareness of chromosomal abnormalities that may co-occur, such as trisomy 13 or 18.
Used in cases of bladder reconstruction or removal due to congenital absence.
Detailed operative notes and pre-operative assessments.
Pediatric urologists must document the rationale for surgical intervention.
Common associated conditions include renal agenesis, bladder exstrophy, and other urinary tract malformations. Genetic syndromes may also be present, necessitating a thorough evaluation.