Hepatopulmonary syndrome: a rare manifestation of cirrhosis in patient with diencephalic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma
N. A. Mazerkina,
A. N. Savateev,
S. K. Gorelyshev,
S. A. Mariashev,
S. A. Beregovskaya,
A. N. Konovalov https://doi.org/10.14341/probl12723
Abstract
We describe a 15-year girl, who developed panhypopituitarism and diencephalic obesity after surgical excision of craniopharyngioma, followed by nonalcoholic fatty liver disease and cirrhosis 5 years after surgery. Cirrhosis in this case manifested by hypoxia due to hepatopulmonary syndrome, and despite cure of craniopharyngioma by surgery and radiosurgery treatment and adequate hormonal substitution therapy patient died 9 years after surgery. Growth hormone substitutional therapy in patients with hypopituitarism, and steatohepatitis may decrease liver triglyceride accumulation and prevent end-stage liver disease.
About the Authors
N. A. Mazerkina
Burdenko Neurosurgery Institute
Russian Federation
Competing Interests:
Russian Federation
Nadia A. Mazerkina - MD, PhD, Professor.
16, 4 Tverskaya-Yamskaya, 125047, Moscow.
eLibrary SPIN: 1012-2923
Competing Interests:
No conflict of interest.
A. N. Savateev
Business Center for Neurosurgery
Russian Federation
Competing Interests:
Russian Federation
Alexander N. Savateev – MD.
Moscow.
eLibrary SPIN: 4505-7703
Competing Interests:
No conflict of interest.
S. K. Gorelyshev
Burdenko Neurosurgery Institute
Russian Federation
Competing Interests:
Russian Federation
Sergey K. Gorelyshev - MD, PhD, Professor.
16, 4 Tverskaya-Yamskaya, 125047, Moscow.
eLibrary SPIN: 6686-3132
Competing Interests:
No conflict of interest.
S. A. Mariashev
Burdenko Neurosurgery Institute
Russian Federation
Competing Interests:
Russian Federation
Sergey A. Mariashev - MD, PhD.
16, 4 Tverskaya-Yamskaya, 125047, Moscow.
eLibrary SPIN: 3980-1278
Competing Interests:
No conflict of interest.
S. A. Beregovskaya
Сenter for therapy and preventive medicine
Russian Federation
Competing Interests:
Russian Federation
Svetlana A. Beregovskaya.
Moscow.
eLibrary SPIN: 1119-4245
Competing Interests:
No conflict of interest.
A. N. Konovalov
Burdenko Neurosurgery Institute
Russian Federation
Competing Interests:
Russian Federation
Alexandre N. Konovalov - MD, PhD, Professor, Acad. RAS.
16, 4 Tverskaya-Yamskaya, 125047, Moscow.
eLibrary SPIN: 1462-1783
Competing Interests:
No conflict of interest.
References
1. De Vile CJ, Grant DB, Kendall BE, et al. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg. 1996;85(1):73-81. doi: https://doi.org/10.3171/jns.1996.85.1.0073
2. Müller HL. Craniopharyngioma. Endocr Rev. 2014;35(3):513-543. doi: https://doi.org/10.1210/er.2013-1115
3. Müller HL, Gebhardt U, Teske C, et al. Post-operative hypothalamic lesions and obesity in childhood craniopharyngioma: results of the multinational prospective trial KRANIOPHARYNGEOM 2000 after 3-year follow-up. Eur J Endocrinol. 2011;165(1):17-24. doi: https://doi.org/10.1530/EJE-11-0158
4. Elowe-Gruau E, Beltrand J, Brauner R, et al. Childhood Craniopharyngioma: Hypothalamus-Sparing Surgery Decreases the Risk of Obesity. J Clin Endocrinol Metab. 2013;98(6):2376-2382. doi: https://doi.org/10.1210/jc.2012-3928
5. Clark AJ, Cage TA, Aranda D, et al. A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Child’s Nerv Syst. 2013;29(2):231-238. doi: https://doi.org/10.1007/s00381-012-1926-2
6. Lena G, Paredes AP, Scavarda D, Giusiano B. Craniopharyngioma in children: Marseille experience. Child’s Nerv Syst. 2005;21(8-9):778-784. doi: https://doi.org/10.1007/s00381-005-1207-4
7. Lee Y-Y, Wong T-T, Fang Y-T, et al. Comparison of hypothalamopituitary axis dysfunction of intrasellar and third ventricular craniopharyngiomas in children. Brain Dev. 2008;30(3):189-194. doi: https://doi.org/10.1016/j.braindev.2007.07.011
8. Özyurt J, Thiel CM, Lorenzen A, et al. Neuropsychological Outcome in Patients with Childhood Craniopharyngioma and Hypothalamic Involvement. J Pediatr. 2014;164(4):876-881.e4. doi: https://doi.org/10.1016/j.jpeds.2013.12.010
9. Zhang YQ, Ma ZY, Wu ZB, et al. Radical Resection of 202 Pediatric Craniopharyngiomas with Special Reference to the Surgical Approaches and Hypothalamic Protection. Pediatr Neurosurg. 2008;44(6):435-443. doi: https://doi.org/10.1159/000172965
10. Roth CL. EASL Clinical Practice Guidelines: Liver transplantation. J Hepatol. 2016;64(2):433-485. doi: https://doi.org/10.1016/j.jhep.2015.10.006
11. Roth CL. Hypothalamic Obesity in Patients with Craniopharyngioma: Profound Changes of Several Weight Regulatory Circuits. Front Endocrinol (Lausanne). 2011;2:43. doi: https://doi.org/10.3389/fendo.2011.00049
12. Ondruch A, Maryniak A, Kropiwnicki T, et al. Cognitive and social functioning in children and adolescents after the removal of craniopharyngioma. Child’s Nerv Syst. 2011;27(3):391-397. doi: https://doi.org/10.1007/s00381-010-1301-0
13. Puget S, Garnett M, Wray A, et al. Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg Pediatr. 2007;106(1):3-12. doi: https://doi.org/10.3171/ped.2007.106.1.3
14. Wijnen M, van den Heuvel-Eibrink MM, Janssen JAMJL, et al. Very long-term sequelae of craniopharyngioma. Eur J Endocrinol. 2017;176(6):755-767. doi: https://doi.org/10.1530/EJE-17-0044
15. Erfurth EM, Holmer H, Fjalldal SB. Mortality and morbidity in adult craniopharyngioma. Pituitary. 2013;16(1):46-55. doi: https://doi.org/10.1007/s11102-012-0428-2
16. Müller HL. Increased Daytime Sleepiness in Patients with Childhood Craniopharyngioma and Hypothalamic Tumor Involvement: Review of the Literature and Perspectives. Int J Endocrinol. 2010;2010:1-7. doi: https://doi.org/10.1155/2010/519607
17. Tomlinson J, Holden N, Hills R, et al. Association between premature mortality and hypopituitarism. Lancet. 2001;357(9254):425-431. doi: https://doi.org/10.1016/S0140-6736(00)04006-X
18. Milić S, Lulić D, Štimac D. Non-alcoholic fatty liver disease and obesity: Biochemical, metabolic and clinical presentations. World J Gastroenterol. 2014;20(28):9330-9337. doi: https://doi.org/10.3748/wjg.v20.i28.9330
19. Hoffmann A, Bootsveld K, Gebhardt U, et al. Nonalcoholic fatty liver disease and fatigue in long-term survivors of childhood-onset craniopharyngioma. Eur J Endocrinol. 2015;173(3):389-397. doi: https://doi.org/10.1530/EJE-15-0422
20. Jung SY, Lee YJ, Lee HJ, et al. Nonalcoholic fatty liver disease in long-term survivors of childhood-onset craniopharyngioma. Ann Pediatr Endocrinol Metab. 2017;22(3):189-196. doi: https://doi.org/10.6065/apem.2017.22.3.189
21. Cremona G, Higenbottam TW, Mayoral V, et al. Elevated exhaled nitric oxide in patients with hepatopulmonary syndrome. Eur Respir J. 1995;8(11):1883-1885. doi: https://doi.org/10.1183/09031936.95.08111883
22. Fujio A, Kawagishi N, Echizenya T, et al. Long-Term Survival with Growth Hormone Replacement after Liver Transplantation of Pediatric Nonalcoholic Steatohepatitis Complicating Acquired Hypopituitarism. Tohoku J Exp Med. 2015;235(1):61-67. doi: https://doi.org/10.1620/tjem.235.61
23. Jung D, Seo GH, Kim Y-M, et al. Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report. Ann Pediatr Endocrinol Metab. 2018;23(1):51-55. doi: https://doi.org/10.6065/apem.2018.23.1.51
24. Bredella MA, Schorr M, Dichtel LE, et al. Body Composition and Ectopic Lipid Changes With Biochemical Control of Acromegaly. J Clin Endocrinol Metab. 2017;102(11):4218-4225. doi: https://doi.org/10.1210/jc.2017-01210
Supplementary files
|
|
1. Figure 1. MRI of the patient's brain in dynamics. A - before surgery. ; B - 6 months after subtotal removal of CF of the craniopharyngioma (a small remainder of the tumor in the chiasmatic-sellar region is determined). ); B - tumor recurrence (increase in residual) 2 years after removal before radiosurgical treatment. ; D - reduction of the remainder of the tumor one year after radiosurgical treatment. | |
| Subject | ||
| Type | Исследовательские инструменты | |
View
(773KB)
|
Indexing metadata ▾ | |
|
|
2. Figure 2. Dynamics of the patient's height and weight before and after surgery. | |
| Subject | ||
| Type | Исследовательские инструменты | |
View
(512KB)
|
Indexing metadata ▾ | |
|
|
3. Figure 3. The patient's appearance. A - cyanosis of the nasolabial triangle; B - changes in the nail phalanges of the type of drumsticks and acrocyanosis. | |
| Subject | ||
| Type | Исследовательские инструменты | |
View
(306KB)
|
Indexing metadata ▾ | |
Review
For citations:
Mazerkina N.A., Savateev A.N., Gorelyshev S.K., Mariashev S.A., Beregovskaya S.A., Konovalov A.N. Hepatopulmonary syndrome: a rare manifestation of cirrhosis in patient with diencephalic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma. Problems of Endocrinology. 2021;67(5):58-66. (In Russ.) https://doi.org/10.14341/probl12723
Views:
1600
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND 4.0).
Email this article 
.jpg){kind=logo}
