Neilson I R, Russo P, Laberge J M, Filiatrault D, Nguyen L T, Collin P P, Guttman F M
Department of Pediatric Surgery, Montreal Children's Hospital, McGill University, Quebec, Canada.
J Pediatr Surg. 1991 Aug;26(8):975-80; discussion 980-1. doi: 10.1016/0022-3468(91)90846-l.
A retrospective 10-year review of congenital adenomatoid malformation (CAM) included 10 cases diagnosed in utero by ultrasound and 13 cases that presented postnatally. Two prenatally diagnosed cases were aborted because of associated lethal anomalies. All remaining patients underwent resection. Up to one third of all cases, whether diagnosed prenatally or postnatally, were asymptomatic. Resection is recommended to avoid respiratory distress, infection, or associated malignancy. There were 5 nonsurvivors, including 2 therapeutic abortions and 3 who died postoperatively. All had either polyhydramnios or ascites. All patients who died postoperatively had a respiratory arrest at birth and underwent immediate lobectomy. All died on the first day of life after a brief period and were found to have associated pulmonary hypoplasia. One had undergone a prenatal transthoracic cyst aspiration at 34 weeks gestation in an attempt to allow lung growth and prevent premature labor. Prenatal ultrasound findings suggestive of poor prognosis included polyhydramnios, ascites, mediastinal shift, and noncystic type III CAM. However, there is a spectrum of severity of CAM. The lesion can either regress and be asymptomatic at birth, or it can progress to cause either fetal death from hydrops or neonatal death caused by associated pulmonary hypoplasia. These findings should be considered in prenatal counseling for CAM.
一项对先天性腺瘤样畸形（CAM）的10年回顾性研究纳入了10例经超声在子宫内诊断的病例和13例出生后出现症状的病例。2例产前诊断的病例因合并致命畸形而流产。其余所有患者均接受了手术切除。所有病例中，无论产前还是产后诊断，多达三分之一无症状。建议进行手术切除以避免呼吸窘迫、感染或相关恶性肿瘤。有5例死亡病例，包括2例治疗性流产和3例术后死亡。所有患者均有羊水过多或腹水。所有术后死亡的患者出生时均呼吸骤停并立即接受了肺叶切除术。所有患者均在出生后第一天短期内死亡，且均伴有肺发育不全。其中1例在妊娠34周时接受了产前经胸囊肿抽吸术，试图促进肺生长并预防早产。产前超声提示预后不良的表现包括羊水过多、腹水、纵隔移位和非囊性III型CAM。然而，CAM的严重程度存在差异。病变可能会消退，出生时无症状，也可能进展导致因水肿引起的胎儿死亡或因相关肺发育不全导致的新生儿死亡。在对CAM进行产前咨询时应考虑这些发现。
