Diencephalic Syndrome: A Rare Entity with Its Anaesthetic Management and Post-operative Complications of Diabetes Insipidus and Thrombocytopenia

S. Kadian, Sarin John, S. Chakraborty, S. Agrawal
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Abstract

Russell first described the diencephalic syndrome in 1951. It is a rare syndrome and usually presents in children as a cause of failure to thrive despite normal, or even increased appetite, with preservation of linear growth. The treatment options vary from endoscopic biopsy followed by chemotherapy to definitive surgical resection of the tumour. The authors here describe a case of an 8-year-old 10 kg emaciated child who presented with headache, vomiting, rage attacks, decreased weight, and diminution in vision. The child had bilateral optic atrophy; however, hormonal profiles were within normal limits. MRI of the brain gave an impression of craniopharyngioma.
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间脑综合征:尿崩症及血小板减少症的麻醉处理及术后并发症
罗素在1951年首次描述了间脑综合征。这是一种罕见的综合征,通常出现在儿童身上,尽管食欲正常,甚至食欲增加,但却无法茁壮成长,并保持线性生长。治疗选择从内镜活检后化疗到最终手术切除肿瘤不等。作者在这里描述了一个8岁10公斤的瘦弱儿童,他表现出头痛、呕吐、愤怒、体重下降和视力下降。患儿双侧视神经萎缩;然而,激素谱在正常范围内。脑部MRI显示颅咽管瘤。
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