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Case Reports
. 2020;29(4):183-187.
doi: 10.1297/cpe.29.183. Epub 2020 Oct 3.

Sphenoethmoidal meningoencephalocele with variable hypopituitarism: A case report and review of literature

Sakura Morishima  1 , Miwako Maeda  1 , Tomoyo Itonaga  1 , Nanae Sato-Kawano  1 , Koh-Ichiro Yoshiura  2 , Kenji Ihara  1
Affiliations
Case Reports

Sphenoethmoidal meningoencephalocele with variable hypopituitarism: A case report and review of literature

Sakura Morishima et al. Clin Pediatr Endocrinol. 2020.

Abstract

Sphenoethmoidal meningoencephalocele is a rare congenital meningocele with unclear clinical course. Its clinical symptoms are diverse, and this disease is widely observed across all ages. The prognosis of this disease depends on the severity of the central nervous system complications. We reported a case of sphenoethmoidal meningoencephalocele incidentally discovered in a 2-yr-old patient, with the subsequent appearance of diabetes insipidus at school age. An endocrinological evaluation performed when the patient was nine years old using the TRH/CRH/LH-RH load test showed a low response of gonadotropins and slightly hyper-response and normal response of ACTH and TSH, respectively. GH provocative tests indicated severe GH deficiency. Desmopressin and GH treatment efficiently improved his growth rate and quality of life. His pituitary function had presumably been normal from the neonatal period to infancy, but the dysfunction gradually progressed over the next few years along with his physical growth. The symptoms were suspected to be the product of the natural course of his hypothalamus or pituitary gland degeneration, or were otherwise due to gradual damage by chronic mechanical compression or extension. These findings underscore the importance of conducting careful systemic management in the long term, specifically with respect to the endocrinological evaluation of sphenoethmoidal meningoencephalocele.

Keywords: diabetes insipidus; growth hormone deficiency; hypothalamus; pituitary; sphenoethmoidal meningoencephalocele.

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Conflict of interest statement

The authors declare no conflicts of interest in association with the present study.

Figures

Fig. 1.
Fig. 1.
T2-weighted magnetic resonance imaging at 2 yr old. A sphenoidal meningoencephalocele was incidentally detected.
Fig. 2.
Fig. 2.
Magnetic resonance imaging at 9 yr old. T1-weighted imaging did not clearly reveal the pituitary gland with a thin pituitary stalk (arrow). No major anomalies of the cerebrum, cerebellum, or brain stem were observed, except for a defect in the corpus callosum.
Fig. 3.
Fig. 3.
Growth chart of the patient. The arrows indicated the starting points of desmopressin and GH treatment.
See this image and copyright information in PMC

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