Korean J Med.  2014 Aug;87(2):209-214. 10.3904/kjm.2014.87.2.209.

A Case of Septo-Optic Dysplasia Resulting in Central Diabetes Insipidus and Nonobstructive Bilateral Hydronephrosis

• Kim JH
• Lee KB
• Lee J
• Nam SM
• Lee KW
• Hwang EG
• Gil G

• KMID: 1710033
• DOI: http://doi.org/10.3904/kjm.2014.87.2.209

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.

Keyword

Septo-optic dysplasia; Central diabetes insipidus; Hydronephrosis

MeSH Terms

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
Deamino Arginine Vasopressin