A Case of Septo-Optic Dysplasia Resulting in Central Diabetes Insipidus and Nonobstructive Bilateral Hydronephrosis.
10.3904/kjm.2014.87.2.209
- Author:
Jong Hyun KIM
1
;
Kwang Bok LEE
;
Jeonghun LEE
;
Soo Min NAM
;
Kang Woo LEE
;
Eu Gene HWANG
;
Geon GIL
Author Information
1. Department of Internal Medicine, Daejeon Sun Medical Center, Daejeon, Korea. sum1092@naver.com
- Publication Type:Case Report
- Keywords:
Septo-optic dysplasia;
Central diabetes insipidus;
Hydronephrosis
- MeSH:
Adult;
Brain;
Corpus Callosum;
Cystostomy;
Deamino Arginine Vasopressin;
Diabetes Insipidus;
Diabetes Insipidus, Neurogenic*;
Humans;
Hydronephrosis*;
Hypopituitarism;
Magnetic Resonance Imaging;
Male;
Mesencephalon;
Optic Nerve;
Osmolar Concentration;
Polydipsia;
Polyuria;
Septo-Optic Dysplasia*;
Septum Pellucidum;
Urinary Bladder, Neurogenic;
Urinary Tract Infections;
Urology;
Vision Disorders;
Water Deprivation
- From:Korean Journal of Medicine
2014;87(2):209-214
- CountryRepublic of Korea
- Language:Korean
-
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.
