Decompensated Cirrhosis with Hepatopulmonary Syndrome in a Patient with Interrupted Treatment for Hypopituitarism.

A 32-year-old male with panhypopituitarism secondary to craniopharyngioma resection developed decompensated cirrhosis and hepatopulmonary syndrome after self-interrupting hormone replacement therapy for 12 years.

• The patient underwent resection of a craniopharyngioma at 8 years of age, resulting in panhypopituitarism.
• Hormone replacement therapy was self-interrupted at 20 years of age, leaving approximately 12 years of untreated panhypopituitarism before presentation at age 32.
• Computed tomography revealed severe fatty liver and cirrhosis.
• Further assessment confirmed a diagnosis of hepatopulmonary syndrome.

Endocrinological evaluation confirmed panhypopituitarism as the underlying etiology in this patient with cirrhosis.

• The diagnosis of panhypopituitarism was established through endocrinological evaluation at the time of presentation with cirrhosis.
• The panhypopituitarism was attributed to the prior craniopharyngioma resection performed during childhood.
• Multiple hormone deficiencies consistent with panhypopituitarism were identified.

Despite initiation of home oxygen therapy and hormone replacement therapy, the patient progressed to liver failure and required listing for liver transplantation.

• Home oxygen therapy was initiated for hepatopulmonary syndrome.
• Hormone replacement therapy was restarted following the endocrinological diagnosis.
• Poorly controlled hypothalamic obesity contributed to ongoing liver failure despite treatment.
• The patient is currently awaiting liver transplantation, indicating failure of medical management to halt disease progression.

Long-term untreated panhypopituitarism was associated with development of non-alcoholic fatty liver disease progressing to cirrhosis with hepatopulmonary syndrome.

• The mechanism proposed involves severe fatty liver accumulation secondary to hormonal deficiencies, particularly growth hormone deficiency and hypothyroidism, promoting hepatic steatosis and fibrosis.
• Hypothalamic obesity resulting from craniopharyngioma and its treatment was identified as a compounding factor contributing to liver disease progression.
• The case illustrates that cirrhosis associated with long-term panhypopituitarism may have a poor prognosis even with hormone replacement therapy.

Liver cirrhosis associated with long-term panhypopituitarism may carry a poor prognosis even when hormone replacement therapy is eventually initiated.

• The authors state: 'Liver cirrhosis associated with long-term panhypopituitarism may have a poor prognosis even with hormone replacement therapy.'
• Disease progression to the point of requiring liver transplantation occurred despite reinstatement of hormone replacement and supportive care.
• Poorly controlled hypothalamic obesity was identified as a key factor limiting the effectiveness of treatment.