Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants.
- Author:
Ahlee KIM
1
;
Jin Soo MOON
;
Hye Ran YANG
;
Ju Young CHANG
;
Jae Sung KO
;
Jeong Kee SEO
Author Information
- Publication Type:Original Article
- Keywords: Failure to thrive; Pituitary diencephalic syndrome; Brain neoplasms; Optic nerve glioma; Astrocytoma
- MeSH: Astrocytoma; Brain Neoplasms; Central Nervous System; Central Nervous System Neoplasms; Diagnosis; Diarrhea; Drug Therapy; Early Diagnosis; Failure to Thrive*; Follow-Up Studies; Head; Humans; Hydrocephalus; Hypothalamic Diseases; Infant*; Optic Nerve Glioma; Palliative Care; Retrospective Studies; Seoul; Strabismus; Vomiting; Weight Gain
- From:Korean Journal of Pediatrics 2015;58(1):28-32
- CountryRepublic of Korea
- Language:English
- Abstract: PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18+/-10.5 months, and diagnosis after symptom onset was made at the mean age of 11+/-9.7 months. The mean z score was -3.15+/-1.14 for weight, -0.12+/-1.05 for height, 1.01+/-1.58 for head circumference, and -1.76+/-1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.
