Korean J Pediatr.  2015 Jan;58(1):28-32. 10.3345/kjp.2015.58.1.28.

Diencephalic syndrome: a frequently neglected cause of failure to thrive in infants

Affiliations
  • 1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. jkseo@snu.ac.kr
  • 2Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.
  • 3Department of Pediatrics, Seoul National University Boramae Medical center, Seoul, Korea.

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.

Keyword

Failure to thrive; Pituitary diencephalic syndrome; Brain neoplasms; Optic nerve glioma; Astrocytoma

MeSH Terms

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
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