- 期刊
Cleft Lip and Palate Associated with Malformation of the Central Nervous System: A Prospective Neurosonographic Study
兔唇及顎裂合併中樞神經系統異常－神經超音波學之前瞻性研究
摘要
兔唇及顎裂爲小兒科常見的先天性異常。大約每七百五十位活産兒就有一位。依Sulak等人的統計，有多於二百五十種的症候群合併於兔唇或顎裂。因此醫生對於有這樣缺陷的病兒，必須注意有無其他合併症。在中樞神經系統方面，可能的異常包括單一全面腦症、灰質異位症及大腦胼胝體生成不良等。 爲探討兔或顎裂病兒可能合併的中樞神經異常；以及從外觀上，何種型態的缺陷較可能合併神經系統病變，本研究以前瞻性觀察研究，對一百九十位無明顯合併其他臉形異常的病兒進行腦超音波檢查。臨床上的分類，分爲(1)兔唇：左側、右側、正中、雙側。(2)顎裂：左側、右側、正中、雙側。(3)兔唇兼顎裂：左側、右側、正中、雙側。及(4)不一致性的兔唇及顎裂。比較何者的神經系統異常發生率較高。結果發現，具正中兔唇兼顎裂缺陷者較容易併中樞神經異常(44%)。一例屬雙側兔唇兼顎裂者合併有胼胝體生成浪。一例屬雙側兔唇但正中顎裂者合併透明隔生成不良。 本研究觀察發現具正中兔唇兼顎裂缺陷者有較高的比較合併中樞神經異常。因此臨床醫師遇到此類型缺陷的病人，應該進一步檢查其中樞神經系統。利用神經超音波檢查可幫忙臨床醫師對嬰幼兒患此病症者做迅速的篩檢。
並列摘要
A prospective neurosonographic study was undertaken on 190 patients with non-syndrome cleft lip and/or palate. Among them, six cases(3.2%) demonstrated malformations of the central nervous system. Included were four cases of midline cleft lip and palate showing holoprosencephaly; pne case of bilateral cleft lip and midine cleft palate showing dysplasia of the septum pallicidum and one case of bilateral cleft lip and palate showing dysgenesis of corpus callosum. The patients with midline clefts were not obligatory associated with malformation of the brain, but their incidence was higher (four of nine case or 44.4%). The neurosonogram provides a a portable, low cost, non-invasive and efficient technique in the diagnosis of gross anomaly of the neonatal brain, and can thus serve as a screening test in those patients with clefts.