Published online Jun 26, 2022. doi: 10.12998/wjcc.v10.i18.6211
Peer-review started: November 13, 2021
First decision: March 7, 2022
Revised: March 25, 2022
Accepted: April 22, 2022
Article in press: April 22, 2022
Published online: June 26, 2022
Craniopharyngioma is a benign tumor that usually develops in children; however, it is located in the center and close to sensitive structures, such as the pituitary gland and hypothalamus. As the hypothalamus plays a crucial role in the homeostasis of anterior pituitary hormone synthesis, damage to the hypothalamus leads to multiple pituitary hormone deficiencies and non-alcoholic fatty liver disease, including hepatopulmonary syndrome (HPS). HPS has limited treatment and poor prognosis.
A girl aged 13 years and 6 mo underwent surgery for craniopharyngioma 6 years prior. Right craniotomy was performed with total resection via the corpus callosum approach, and the tumor at the base was approximately 3.5 cm × 3.5 cm × 4.0 cm. At 1 year postoperatively, she exhibited abdominal distension and weakness, and the laboratory tests revealed fatty liver disease. Thereafter, she had not visited the outpatient clinic for 2 years. Two years ago, she developed decreased activity endurance, severe cyanosis, chest tightness, wheezing, and intermittent and recurrent low fever after mild physical labor. Hepatobiliary ultrasonography, liver biopsy, and contrast echocardiography of the right heart showed cirrhosis and multiple pituitary hormone deficiencies, indicating HPS. After 1 year of treatment with recombinant human growth hormone, the liver function and oxygenation improved; she did not undergo liver transplantation.
Craniopharyngioma surgery can easily cause hypopituitarism, which can lead to nonalcoholic steatohepatitis and HPS in children. Early growth hormone therapy is important to improve the prognosis of these diseases.
Core Tip: Hepatopulmonary syndrome (HPS) is a serious complication of chronic liver disease with a poor prognosis. Currently, liver transplantation is the only available treatment; however, severe hypoxemia before transplantation is a high risk factor for postoperative death. We present a case of postoperative craniopharyngioma in a child with nonalcoholic steatohepatitis and HPS secondary to hypopituitarism. The patient’s liver function and hypoxemia were improved by growth hormone replacement therapy, and liver transplantation was expected to be avoided.