ICD10 code of Polycystic Liver Disease and ICD9 code

Polycystic Liver Disease (PLD) is primarily coded as ICD-10 Q44.6 (Cystic disease of liver) and ICD-9 751.69 (Other specified anomalies of gallbladder, bile ducts and liver). These codes are used by healthcare providers to classify Polycystic Liver Disease for billing and clinical record-keeping purposes.

What is Polycystic Liver Disease?

Polycystic Liver Disease is a rare genetic condition characterized by the growth of multiple fluid-filled cysts within the liver. While the liver often remains functional, the progressive enlargement of these cysts can cause significant abdominal discomfort, bloating, and pain. Currently, 7 members of the DiseaseMaps.org community are living with Polycystic Liver Disease, sharing their experiences to help others navigate the complexities of this condition.

How is Polycystic Liver Disease categorized?

Clinically, Polycystic Liver Disease is often categorized into two distinct forms based on its genetic association:

• Isolated Polycystic Liver Disease (PCLD): A rare, typically autosomal dominant condition where cysts are confined to the liver.


• Polycystic Liver Disease associated with ADPKD: A more common manifestation where Polycystic Liver Disease occurs alongside Autosomal Dominant Polycystic Kidney Disease.

Is Polycystic Liver Disease hereditary?

Yes, Polycystic Liver Disease is generally hereditary. In cases of isolated Polycystic Liver Disease, mutations in genes such as PRKCSH, SEC63, or ALG8 are frequently identified. Genetic counseling is highly recommended for those diagnosed with Polycystic Liver Disease, as understanding the inheritance pattern can provide clarity for family members who may also be at risk.

How do clinicians manage Polycystic Liver Disease?

Management of Polycystic Liver Disease focuses on symptom control and monitoring. While most cases are asymptomatic, severe cases may require interventions such as aspiration sclerotherapy, laparoscopic cyst fenestration, or in rare instances, liver transplantation. Because Polycystic Liver Disease affects every patient differently, a multidisciplinary approach involving hepatologists and geneticists is essential.

Next steps

• Consult with a hepatologist specializing in cystic liver disorders to monitor your liver volume.


• Connect with the 7 members of the DiseaseMaps.org community to share lived experiences.


• Request a referral to a genetic counselor to discuss the hereditary implications of your specific diagnosis.

Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Polycystic liver disease.


• Orphanet: Isolated polycystic liver disease (ORPHA:73244).


• OMIM (Online Mendelian Inheritance in Man): Polycystic liver disease 1 (PSCL1).


• National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK).