Echeverría Lecuona J, Benito A, Arena Ansotegui J, Collado Espiga V, Rey Otero A, Paisán Grisolía L
Sección de Neonatología, Hospital Aránzazu, San Sebastián.
An Esp Pediatr. 1998 Aug;49(2):161-4.
The aim of this study was to study the prenatal diagnosis, clinical manifestations and karyotype results in congenital chylothorax.
A retrospective survey of a 20 year period was carried out. The prenatal diagnosis, maneuvers following delivery, malformations and clinical outcome were studied.
We found 10 cases of congenital chylothorax. Four were diagnosed by prenatal ultrasound with pleural effusion and no other signs of hydrops and six were diagnosed postnatally. Of the later, five had respiratory distress and one was diagnosed by radiological procedures. In all 10 cases, postnatal thoracocentesis was performed for diagnosis and therapy, five infants required mechanical ventilation and four parenteral nutrition. One patient died 72 hours after birth and three within 24 hours of birth. The clinical outcome of the rest was successful and were placed on a medium-chain triglyceride formula that was given orally. Of the eight karyotypes studied, six trisomy 21 were found.
The prenatal diagnosis of pleural effusion permits prompt recognition of congenital chylothorax and allow for appropriate treatment upon birth. Medical therapy including pleural drainage and total parenteral nutrition and medium-chain triglyceride formula for oral feedings is successful in the majority of cases. Down's syndrome should be considered in fetuses or newborns with pleural effusion.
本研究旨在探讨先天性乳糜胸的产前诊断、临床表现及核型结果。
进行了一项为期20年的回顾性调查。研究了产前诊断、分娩后的处理、畸形情况及临床结局。
我们发现10例先天性乳糜胸。4例通过产前超声诊断为胸腔积液且无其他水肿迹象,6例为产后诊断。其中,5例有呼吸窘迫,1例通过放射学检查诊断。所有10例均进行了产后胸腔穿刺术以进行诊断和治疗,5例婴儿需要机械通气,4例需要肠外营养。1例患者出生后72小时死亡,3例在出生后24小时内死亡。其余患者临床结局良好,采用口服中链甘油三酯配方奶。在研究的8例核型中,发现6例为21三体。
胸腔积液的产前诊断有助于及时识别先天性乳糜胸,并在出生时进行适当治疗。包括胸腔引流、全肠外营养及口服中链甘油三酯配方奶在内的内科治疗在大多数病例中是成功的。胸腔积液的胎儿或新生儿应考虑唐氏综合征。