CAJ | 학술논문

目的总结儿童Kallmann综合征(KS)的影像表现,提高对该病的认识.方法搜集经临床证实且有影像资料的KS患儿13例,患儿均经颅脑MR、左腕骨X线检查,其中8例行腹盆腔B超检查,分析总结其影像表现.结果 13例患儿中,9例双侧嗅球、嗅束缺如；4例一侧嗅球小、嗅束细小或显示不清,另一侧嗅球嗅束缺如.8例双侧嗅沟不同程度浅小,其中2例一侧嗅沟消失；3例一侧嗅沟浅、一侧正常；2例双侧嗅沟正常.3例伴垂体前叶小.6例骨龄落后.B超检查8例中,双侧睾丸均小.结论儿童KS均有嗅脑发育不良的MRI表现,部分伴垂体前叶发育不良；近半数伴骨龄落后；双侧睾丸均小.
목적 총결인동Kallmann종합정(KS)적영상표현,제고대해병적인식.방법 수집경림상증실차유영상자료적KS환인13례,환인균경로뇌MR、좌완골X선검사,기중8례행복분강B초검사,분석총결기영상표현.결과 13례환인중,9례쌍측후구、후속결여；4례일측후구소、후속세소혹현시불청,령일측후구후속결여.8례쌍측후구불동정도천소,기중2례일측후구소실；3례일측후구천、일측정상；2례쌍측후구정상.3례반수체전협소.6례골령락후.B초검사8례중,쌍측고환균소.결론 인동KS균유후뇌발육불량적MRI표현,부분반수체전협발육불량；근반수반골령락후；쌍측고환균소.
Objective To summarize imaging features of Kallmann syndrome (KS) in children and to improve diagnostic level of the disease.Methods The imaging manifestations of 13 patients with clinically proved KS were retrospectively studied.MRI of rhinencephalon and left wrist X-ray examinations were performed in all the 13 children.Ultrasound of abdomen and pelvis was implemented in the 8 of 13 patients.Results All the patients had abnormalities of olfactory system.Bilateral olfactory bulbs and tracts were absent in 9 patients.Unilateral olfactory bulbs and tracts were absent in 4 patients,in which contralateral olfactory bulbs and tracts were hypoplastic.These patients presented 2 aplastic,17 hypoplastic and 7 normal olfactory sulci.Anterior pituitary was hypoplastic in 3 patients.Bone age showed retardation in 6 of 13 patients.Bilateral testicles were small in all the 8 cases by ultrasound.Conclusions Aplastic or hypoplastic olfactory bulbs and tracts and sulci are seen in children with KS.Anterior pituitary dysplasia is present in part of patients and bone age backward happens in approximate half of the children.