Rejin KEBUDİ, Deniz TUĞCU, İnci AYAN, Ömer GÖRGÜN

Optik-kiazmatik-hipotalamik gliom ve diensefalik sendrom: Vaka sunumu

Optik traktus tümörleri optik sinir, kiazma ve hipotalamusdan köken alan ve beynin komşu bölgelerine yayılabilen tümörlerdir. Histolojik olarak bu tümörler, düşük dereceli astrositomlardır ve optik- kiazmatik- hipotalamik gliom olarak da adlandırılırlar. Hipotalamik yerleşimde genellikle sütçocukluğu ve erken çocukluk döneminde ilerleyici zayıflama, büyüme ve gelişme geriliği ile karakterize diensefalik sendrom görülebilir. Diensefalik sendrom, uygun kalori almasına rağmen, büyüme ve gelişme geriliği olan çocukların ayırıcı tanısında düşünülmelidir. Bu sendromun varlığını bilerek, dikkatli anamnez, nörolojik ve fundoskopik muayeneyi de içeren ayrıntılı muayene ve tetkik şarttır. Diensefalik sendromun kemoterapi rejimleri ile tedavisi, belirgin kilo alımına neden olur ve alternatif tedavilere gereksinimi azaltır.

Optic-chiasmatic-hypotolamic glioma and diencephalic syndrome: Case report

Optic pathway tumors generally are those tumors that arise from the optic nerve, chiasm and hypotalamus and may extend from these sites to adjacent brain structures. Histologically, these are low grade astrocytomas and also named optic-chiasmatic-hypothalamic glioma. Diencephalic syndrome, which is mostly seen in infants and young children, and is characterized with emaciation and failure to thrive, may be seen in hypotalamic region. This syndrome should be considered in the differential diagnosis in any children with emaciation despite adequate caloric intake. Careful history taking and physical examination including neurological examination, fundoscopic examination and appropriate imaging investigations,with awareness of this syndrome are crucial. Chemotherapy for the treatment of diencephalic syndrome in young children results in significant weight gain and decreases the need for alternative therapies

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