儿童症状性Rathke囊肿17例临床分析

Diagnosis and treatment of symptomatic Rathke cleft cysts in 17 children： clinical features and surgical results

目的探讨儿童症状性Ratheke囊肿（RCCs）的诊治经验和手术效果。方法我院2001—2010年收治RCCs患者182例，其中年龄〈18岁17例，回顾性分析17例患儿的临床资料。16例行经蝶窦入路垂体囊肿切除或切开引流术，1例经额下入路开颅。结果17例患儿中发育迟缓5例（29．4％），头痛4例（23．5％），视力下降或视野缺损3例（17．6％），多饮、多尿2例（11．8％），偶然发现3例（1例合并儿童良性癫痫，1例男孩乳腺发育，1例外伤）。10例手术中可见菲薄的囊壁，囊壁全切除5例，囊壁部分切除3例，仅行引流术者2例；余7例未见明确的囊壁，仅行清除囊内容物并敞开鞍底引流。5例术中出现脑脊液漏，4例经修补和鞍底重建术后平卧3～7d后治愈，1例术后出现脑膜炎症状，经腰蛛网膜下腔引流、平卧、抗炎治疗后治愈；其余12例经蝶手术无脑脊液漏的患儿鞍底硬膜敞开引流，未填塞。5例发育迟缓患儿，平均骨龄低于实际年龄，术后生长激素治疗有效。头痛和视力障碍的7例术后症状改善，2例尿崩患儿术后继续对症治疗2个月后逐渐用最小剂量维持，3例偶然发现的患儿术后治愈。平均随访时间3．2年，均未见囊肿复发。结论经蝶窦入路囊肿切除或切开引流是治疗儿童症状性RCCs的首选方法。以发育迟缓起病的患儿术后可用生长激素治疗。

Objective To summarize the cinical features and surgical results of diagnosis and treatments of symptomatic Ratheke cleft cysts（RCCs） in children. Methods The clinical data of 17 cases RCCs of children （younger than 18 years old） selected from 182 cases RCCs admitted into Peking Union Medical College Hospital from 2001 to 2010 were retrospectively analyized. Sixteen patients undertook the transsphenoidal pituitary cyst excision or incision and drainage, 1 case by the frontal approach eraniotomy. Results Five eases of the 17 children with growth retardation （29.4%）, headache in 4 cases （23.5%）, decreased visual acuity or visual field defect in 3 cases （ 17.6% ）, 2 cases of polydipsia, polyuria （ 11. 8% ）, 3 cases of accidental discovery （1 child with benign epilepsy, 1 breast development, 1 exception injury）. The thin cyst wall in 10 surgeries were visible ： whole resection of 5 cases, part of wall resection in 3 cases, the only line of drainage in 2 cases; Remaining 7 cases with cyst wall invisble were given drainage only. Five cases with cerebrospinal rhinorrhea during operations were treated by repairing and recconstructing the sellar floor, and 4 cases got good recoveries only by lying spine. One case, suffering from meningitis, was cured by virtue of lumbar subarachnoid drainage, supine, anti-inflammatory treatment. For other 12 cases, the sellar floor was opened and drained directly with no filhng. Five cases of children with growth retardation, whose average bone age are lower than the actual age got effective growth hormone therapy after surgery. The symptoms of headache and visual disturbance in 7 patient were relieved after treatment. Two cases with diabetes insipidus were treated at least for two months, maintained with minimal dose of minirin. Three cases accidentally found were cured. The average follow-up time lasted 3. Two years, and no cyst recurrence found in all children. Conclusion Transsphenoidal cyst excision or incision and drainage surgery is the preferred therapeutic method for children with symptomatic RCCs; The patients suffered from hypoevolutism can be treated with growth hormone postoperatively.