Prune belly syndrome
ICD-10 Q79.4 is a billable code used to indicate a diagnosis of prune belly syndrome.
Prune belly syndrome, also known as Eagle-Barrett syndrome, is a rare congenital disorder characterized by a triad of symptoms: abdominal muscle deficiency, urinary tract anomalies, and cryptorchidism (undescended testicles). The condition is primarily seen in males and is thought to result from abnormal development of the mesoderm during embryogenesis. The lack of abdominal wall musculature leads to a distinctive appearance of the abdomen, which appears wrinkled or 'pruned.' Urinary tract malformations can include hydronephrosis, ureteral dilation, and bladder dysfunction, which may lead to renal impairment if not managed appropriately. Cryptorchidism is common and requires surgical intervention to prevent complications such as infertility or testicular cancer. Prune belly syndrome can also be associated with other congenital malformations, including musculoskeletal deformities such as clubfoot, hip dysplasia, and limb reduction defects. Early diagnosis and multidisciplinary management are crucial for optimizing outcomes in affected individuals.
Detailed records of physical examinations, growth parameters, and developmental milestones are essential. Documentation should include any surgical interventions and follow-up care.
Pediatricians may encounter cases during routine examinations, referrals for urinary tract issues, or when assessing developmental delays.
Pediatric coders must be aware of the potential for co-existing conditions and ensure that all relevant diagnoses are captured.
Genetic testing results, family history, and any genetic counseling notes should be documented to support the diagnosis.
Geneticists may see patients for evaluation of familial patterns, risk assessment for future pregnancies, or management of associated congenital anomalies.
Genetic coders should ensure that chromosomal abnormalities are accurately documented and linked to the primary diagnosis.
Performed for cryptorchidism associated with prune belly syndrome.
Operative report detailing the procedure and indications.
Pediatric urologists should ensure that the diagnosis is clearly linked to the procedure.
Common complications include urinary tract infections, renal impairment, and issues related to cryptorchidism. Early intervention and regular monitoring are essential to manage these risks.
