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尿崩症的发病率和下丘脑错构瘤切除后发作

作者:刘爽 编译 来源: 日期:2015-03-29
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关键字:  尿崩症 |  |  | 发病率 |  |  | 下丘脑错构瘤 |  

Pediatric Critical Care Medicine:

May 2014 - Volume 15 - Issue 4_suppl - p 64

doi: 10.1097/01.pcc.0000448997.35614.cc

INCIDENCE AND ONSET OF DIABETES INSIPIDUS POST RESECTION OF HYPOTHALAMIC HAMARTOMA: A CASE SERIES REVIEW

Samson, M.

ABSTRACT

Background and aims: A hypothalamic hamartoma (HH) is a rare (1 in 200,000) benign brain tumor of the hypothalamus. These tumors can cause debilitating gelastic and dacrystic seizures that can become progressive and are usually not responsive to medical management. HH patients can also have cognitive impairment, emotional and behavioral difficulties, precocious puberty and Pallister-Hall syndrome. Since 2003, the Barrow Neurologic Institute (BNI) has developed a program for transcallosal surgical resection of hypothalamic hamartomas. In 2011, the BNI joined with Phoenix Children’s Hospital to continue to offer a variety of surgical approaches for the treatment of HH in children.

Aims: Post operative management of these patients includes care in the PICU with close monitoring for seizures and the development of diabetes insipidus (DI). We are collecting data on the post operative course to develop a clinical pathway to optimize and stardardize the care of these patients.

Methods: After approval from IRB, we did a chart review of 9 patients, ranging in age from 9 months to 13 years, who underwent HH resection from the years 2011–2013.

Results: 3 of the 9 patients developed DI which equals 33%. All 3 developed DI within 48 hours of surgery.

Conclusions: In this small case series review, 33% of patients developed DI within 48 hours of surgery. This would indicate that post operative HH patients should stay in the PICU a minimum of 48 hours and families can be told this in the pre-operative phase. Fluid balance and sodium checks should be ordered accordingly.

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