A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics.
Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47).
COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.
方法:对加拿大/美国的16个机构进行了非随机便利样本的MMC患者(出生>=1972年)的回顾性研究。
结果:在293例死亡(89%分流性脑积水)中,12%发生在婴儿期,35%的童年成年期为53%(记录的COD:74%)。对于261名分流人员,主要的COD是神经(21%)和肺(17%),主要的MOD是感染(34%,包括分流器感染:4%)和非感染性分流器故障(14%)。对于32个未分流的人来说,主要的COD是肺部(34%)和心血管(13%),主要的MOD是感染(38%)和非感染性肺部(16%)。COD和MOD随分流状态和年龄而变化(p<=0.04),不是步行或出生年份(p>=0.16)。泌尿外科相关死亡(尿脓毒症,肾功能衰竭,血尿,膀胱穿孔/癌:10%)在女性中更有可能(p=0.01),独立于年龄,分流,或动态状态(p>=0.40)。COD/MOD与膀胱扩张无关(p=>0.11)。睡眠时无法解释的死亡(4%)与年龄无关,分流状态,和癫痫(p>=0.47)。
结论:COD随分流状态而变化。领导的国防部具有传染性。泌尿外科相关的死亡(10%)与分流状态无关;26%的COD未知。需要终身的多学科护理和准确的死亡率记录。