Diencephalic syndrome due to astrocytoma in three infants with failure to thrive

Densupsoontorn, Narumon; Jirapinyo, Pipop; Likasitwattanakul, Surachai; Sanmaneechai, Oranee; Sanpakit, Kleebsabai; Surachatkumtonekul, Thammanoon; Chiewvit, Pipat

doi:10.1111/j.1442-200x.2010.03320.x

Cited by 2 publications

(2 citation statements)

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“…The diagnosis of DS is frequently delayed; considering all published reports indicating this finding, the average age at diagnosis was 45.9 months (range from 4 to 193.2 months), with a mean delay from symptoms of 15.2 months (range from 0.5 to 87 months). In our cases, the correct diagnosis was achieved 6 months after initial signs of FTT in the girl #2, and 8 months in cases #1 and #3, with a mean diagnostic delay of 7.3 months, accordingly with Densupsoontorn et al who found a median interval time of 7 months [ 12 ]. Other previous studies showed a slight longer interval time to reach final diagnosis, as in the cohorts of Kim et al [ 16 ] and Addy et al [ 3 ], reporting a median time of 11 and 13 months, respectively.…”

Section: Discussionsupporting

confidence: 66%

“…The neurological features due to IH are frequently found, being vomiting the most common, followed by headache, instability gait, and, more rarely, papilledema. Ocular manifestations as nystagmus, strabismus, and visual loss, are other common neurological signs related to DS [ 3 – 12 , 34 ]. Since both neurological and ophthalmological signs may be late, delay in diagnosis is frequently reported [ 7 , 10 , 14 – 16 , 35 ].…”

Section: Discussionmentioning

confidence: 99%

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Diencephalic syndrome in childhood, a challenging cause of failure to thrive: miniseries and literature review

et al. 2022

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The aim of our study was to better define the clinical pattern of diencephalic syndrome, a rare but potentially lethal cause of failure to thrive in infancy. Poor weight gain or weight loss, the characteristic presenting feature, often firstly attributed to gastrointestinal or endocrinological or genetic diseases, is secondary to a malfunctioning hypothalamus, caused by a diencephalic tumor. Due to its unexpected clinical onset, diagnostic delay and misdiagnosis are common. We described a case series of 3 children with diencephalic syndrome admitted at our Hospital, over a 5-year period. Furthermore, a narrative review on all pediatric cases published in the last seventy years was performed. Clinical pattern, timing to diagnosis, neuroimaging, management, and outcome were analyzed. Our three cases are singularly described in all clinical and diagnostic findings. Overall, 100 children were selected; all these cases as well as our children presented with failure to thrive: 96% had body mass index or weight-length/height ratio lower than 5th percentile. Vomiting and hyperactivity are reported in 35 and 26% of cases, respectively. The neurological features, mainly nystagmus reported in 43%, may occur late in the disease course. In conclusion, the diagnostic delay is the hallmark of diencephalic syndrome, confirming the lack of knowledge by clinicians. The poor weight gain/loss despite adequate length growth and food intake, especially in children with hyperactivity and good psychomotor development, should alert pediatricians towards this condition, before neurological signs/symptoms occurrence.

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Section: Discussionsupporting

confidence: 66%