Elias D L, Kawamoto H K, Wilson L F
UCLA Division of Plastic Surgery.
Plast Reconstr Surg. 1992 Dec;90(6):951-8. doi: 10.1097/00006534-199212000-00002.
Holoprosencephaly encompasses a series of midline defects of the brain and face. Most cases are associated with severe malformations of the brain which are incompatible with life. At the other end of the spectrum, however, are patients with midline facial defects and normal or near-normal brain development. Although some are mentally retarded, others have the potential for achieving near-normal mentality and a full life expectancy. The latter patients do not fit clearly into the previously defined classification system. Proposed is a new classification focusing on those patients with normal or lobar brain morphology but with a wide range of facial anomalies. The classification aids in planning treatment. Coupled with CT scan findings of the brain and a period of observation, patients unlikely to thrive can be distinguished from those who will benefit from surgical intervention. Repair of the false median cleft lip and palate may suffice in patients with moderate mental retardation. Patients exhibiting normal or near-normal mentality with hypotelorbitism and nasomaxillary hypoplasia can be treated with a simultaneous midface advancement, facial bipartition expansion, and nasal reconstruction.
前脑无裂畸形包括一系列脑和面部的中线缺陷。大多数病例与严重的脑部畸形有关，这些畸形与生命不相容。然而，在谱系的另一端，是患有中线面部缺陷且脑发育正常或接近正常的患者。虽然有些患者智力迟钝，但其他患者有潜力达到接近正常的智力水平和正常的预期寿命。后一类患者并不完全符合先前定义的分类系统。提出了一种新的分类方法，重点关注那些脑形态正常或呈叶状但有广泛面部异常的患者。这种分类有助于制定治疗计划。结合脑部CT扫描结果和一段时间的观察，可以区分出不太可能茁壮成长的患者和那些将从手术干预中受益的患者。对于中度智力迟钝的患者，修复假性正中唇腭裂可能就足够了。表现出正常或接近正常智力水平、伴有眼距过窄和鼻上颌发育不全的患者，可以通过同时进行中面部推进、面部二分法扩张和鼻重建来治疗。
