Korean J Med > Volume 87(2); 2014 > Article
The Korean Journal of Medicine 2014;87(2):209-214.
Published online August 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.87.2.209   
A Case of Septo-Optic Dysplasia Resulting in Central Diabetes Insipidus and Nonobstructive Bilateral Hydronephrosis
Jong Hyun Kim, Kwang Bok Lee, Jeonghun Lee, Soo Min Nam, Kang-Woo Lee, Eu Gene Hwang, Geon Gil
1Departments of Internal Medicine, Daejeon Sun Medical Center, Daejeon, Korea
2Departments of Urology, Daejeon Sun Medical Center, Daejeon, Korea
양측성 비폐쇄성 수신증 및 중추성 요붕증을 유발한 중격-시신경 형성장애 1예
김종현, 이광복, 이정훈, 남수민, 이강우, 황유진, 길건
1대전선병원 내과
2대전선병원 비뇨기과
Correspondence: 
Soo Min Nam, Tel: +82-42-220-8577, Fax: +82-42-252-5505, Email: sum1092@naver.com
Received: 27 November 2013   • Revised: 11 February 2014   • Accepted: 24 February 2015
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
A 27-year-old male with nonobstructive hydronephrosis was referred from the urology department for polyuria evaluation and management. The patient was hospitalized for urinary tract infection and cystostomy was performed due to neurogenic bladder of unknown origin. The patient was of short stature and had visual impairment. From the interview, we discovered he had been suffering from polyuria and polydipsia for more than 20 years. Urine output was 13 L/day and urine osmolarity was 85 mOsm/kg. The results of a water deprivation test were consistent with central diabetes insipidus. Septo-optic dysplasia (SOD) was observed on brain magnetic resonance imaging (MRI). SOD is a very rare condition characterized by agenesis of the septum pellucidum or corpus callosum, which may cause optic nerve aplasia or hypoplasia, midbrain abnormalities and/or hypopituitarism. After desmopressin treatment, polyuria and hydronephrosis were improved. We report a case of a 27-year-old male diagnosed with SOD including diabetes insipidus, resulting in nonobstructive hydronephrosis.
Key Words: Septo-optic dysplasia, Central diabetes insipidus, Hydronephrosis
주제어: 중격-시신경 형성장애; 중추성 요붕증; 수신증


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