Danzer E, Finkel R, Gerdes M, Schwartz E M S, Rintoul N N, Adzick N S, Johnson Mark P
The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.
Neuropediatrics. 2010 Jun;41(3):140-3. doi: 10.1055/s-0030-1263164. Epub 2010 Sep 21.
We explored the relationship between seizure activity (SA) and/or chronic epilepsy (CE) and short-term neurodevelopmental outcomes following fetal myelomeningocele (fMMC) surgery. Retrospective databases and a parental questionnaire focusing on common complications of hindbrain herniation associated with MMC were used to determine the incidence of seizures following fMMC surgery. The Bayley Scales of Infant Development II was used to evaluate the neurocognitive outcomes. The available 3-year outcome data were used for analysis. 54 children underwent fMMC closure at our institution between 1998 and 2003. 48 (89%) families participated. The shunt rate was 50% (n=24). Seizures developed in 8/48 (17%) children, 2 (8%) non-shunted and 6 (25%) shunted (P=0.07). Of those six, 3 developed CE. Neurodevelopmental scores in the average range were found in both non-shunted and 3 shunted fMMC children. The remaining 3 shunted toddlers had CE and significant neurodevelopmental delays. Of those, 2 had severe intracranial hemorrhage and one developed frequent apneic spells in combination with epilepsy. The incidence of seizures in fMMC children was similar to previously reported data of postnatally repaired MMC patients. SA alone without CE was not associated with a worse neurocognitive outcome. The occurrence of severe acquired intracranial injury and CE, however, appeared to be correlated with adverse neurocognitive outcome following fMMC surgery.
我们探讨了胎儿脊髓脊膜膨出(fMMC)手术后癫痫发作活动(SA)和/或慢性癫痫(CE)与短期神经发育结局之间的关系。使用回顾性数据库和一份关注与MMC相关的后脑疝常见并发症的家长问卷来确定fMMC手术后癫痫发作的发生率。使用贝利婴儿发展量表第二版来评估神经认知结局。可用的3年结局数据用于分析。1998年至2003年期间,我们机构有54名儿童接受了fMMC修复手术。48个(89%)家庭参与。分流率为50%(n = 24)。48名儿童中有8名(17%)出现癫痫发作,2名(8%)未分流儿童和6名(25%)分流儿童(P = 0.07)。在这6名儿童中,3名发展为CE。未分流和3名分流的fMMC儿童的神经发育评分均在平均范围内。其余3名分流幼儿患有CE且有明显的神经发育延迟。其中,2名有严重颅内出血,1名除癫痫外还频繁出现呼吸暂停发作。fMMC儿童癫痫发作的发生率与先前报道的出生后修复的MMC患者的数据相似。仅SA而无CE与较差的神经认知结局无关。然而,严重获得性颅内损伤和CE的发生似乎与fMMC手术后不良的神经认知结局相关。