Cook James, Chitty Lyn S, De Coppi Paolo, Ashworth Michael, Wallis Colin
Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Department of Fetal Medicine, University College Hospitals NHS Foundation Trust, London, UK.
Arch Dis Child. 2017 Sep;102(9):798-803. doi: 10.1136/archdischild-2016-311233. Epub 2017 Jun 5.
A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases.
We describe the long-term clinical course of 119 children diagnosed with these lesions treated at Great Ormond Street Hospital (GOSH). Cases were identified via the GOSH patient database. Study entry required the identification of a cystic lung lesion on prenatal ultrasound and confirmation of CPAM/PS on postnatal CT imaging. Patients followed up for at least 5 years were included.
51 (43%) patients were managed surgically; 8 (6.7%) as an emergency during the neonatal period, 6 (5.1%) electively due to concerning features on CT scan, 20 (17%) following medical advice, 1 (0.8%) following a severe respiratory infection and in 5 (4.2%) the indication was unclear. The indication in 11 (9.2%) was recurrent respiratory infection and median age at surgery was 1.6 years (range 0.4 to 4.6 years). No cases of malignancy were identified on histological examination of resected lesions. 68 (57%) patients were managed conservatively for a median period of 9.9 years (range 5.2 years to 18 years). Seven (10%) were discharged, one was followed-up elsewhere (1.5%) and eight (11%) were lost to follow-up. In four patients (5.9%), the lesion resolved spontaneously. 52 (76%) continue to be followed-up and remain asymptomatic.
This is one of the largest case series documenting the natural history of CPAMs and PS following a prenatal diagnosis and demonstrates that conservative management is a reasonable option in selected cases.
关于先天性肺气道畸形（CPAM）和肺隔离症（PS）自然病史的证据不足，导致无症状病例的管理策略存在差异。
我们描述了在大奥蒙德街医院（GOSH）接受治疗的119例诊断为这些病变的儿童的长期临床过程。通过GOSH患者数据库识别病例。研究纳入要求产前超声检查发现肺囊性病变，并在出生后CT成像上确诊为CPAM/PS。纳入随访至少5年的患者。
51例（43%）患者接受了手术治疗；8例（6.7%）在新生儿期作为急诊手术，6例（5.1%）因CT扫描显示有相关特征而择期手术，20例（17%）遵循医学建议手术，1例（0.8%）在严重呼吸道感染后手术，5例（4.2%）手术指征不明确。11例（9.2%）的手术指征是反复呼吸道感染，手术中位年龄为1.6岁（范围0.4至4.6岁）。切除病变的组织学检查未发现恶性病例。68例（57%）患者接受保守治疗，中位时间为9.9年（范围5.2年至18年）。7例（10%）已出院，1例（1.5%）在其他地方随访，8例（11%）失访。4例患者（5.9%）病变自发消退。52例（76%）继续随访且无症状。
这是记录产前诊断后CPAM和PS自然病史的最大病例系列之一，表明在某些病例中保守治疗是一种合理的选择。
