Kaplan L C
Harvard Medical School, Boston, Massachusetts.
Otolaryngol Clin North Am. 1989 Jun;22(3):661-72.
This article has attempted to describe an approach to the assessment and management of the child with choanal atresia whose airway abnormality is one component of a multiple anomaly condition (CHARGE association). The risks to the child with CHARGE association exceed those of any one of his or her anomalies, and the clinician must assess the child deliberately, realizing that morbidity may be increased if any of the child's other systemic anomalies are given a secondary role in long-term management. The CHARGE association baby is an atypical choanal atresia patient, as he or she is an atypical cardiac or hearing-impaired patient; for each child there may be a logical approach to prioritizing such issues as nutrition, airway management, and cardiovascular support. Unlike known "syndromes" in which natural history may be definable, the child with CHARGE association depends considerably on the effects of various interventions to determine outcome. Some basic principles useful in guiding these interventions have been presented to assist the clinician in this process.
本文试图描述一种评估和管理患有后鼻孔闭锁且气道异常是多重畸形综合征(CHARGE综合征)一部分的儿童的方法。患有CHARGE综合征的儿童所面临的风险超过了其任何一种畸形单独带来的风险,临床医生必须审慎地评估患儿,要意识到如果在长期管理中对患儿的任何其他全身畸形给予次要地位,其发病率可能会增加。患有CHARGE综合征的婴儿是一种非典型的后鼻孔闭锁患者,正如他或她是非典型的心脏或听力受损患者一样;对于每个孩子,在确定营养、气道管理和心血管支持等问题的优先级时,可能会有一种合理的方法。与已知其自然病史可明确的“综合征”不同,患有CHARGE综合征的儿童在很大程度上依赖于各种干预措施的效果来决定预后。本文已提出一些有助于指导这些干预措施的基本原则,以协助临床医生开展这一过程。