Department of Neonatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Pediatr Crit Care Med. 2010 May;11(3):e36-7. doi: 10.1097/PCC.0b013e3181b80a75.
First report of an infant with coexistent omphalocele and alveolar capillary dysplasia.
Descriptive case report.
Neonatal intensive care unit of a tertiary care children's hospital.
We describe a term infant with omphalocele and respiratory insufficiency attributable to pulmonary hypertension. The patient was placed on extracorporeal membrane oxygenation, but the pulmonary hypertension persisted. After 10 days on extracorporeal membrane oxygenation, a lung biopsy was performed. It showed alveolar capillary dysplasia. Because of the lethal prognosis, extracorporeal membrane oxygenation was withdrawn and the patient expired.
This is the first description of an association between omphalocele and alveolar capillary dysplasia. In newborns with omphalocele who have severe respiratory insufficiency and pulmonary hypertension, alveolar capillary dysplasia should be considered.
首例腹裂合并肺泡毛细血管发育不良婴儿报告。
描述性病例报告。
三级儿童医院新生儿重症监护病房。
我们描述了一名足月婴儿，患有腹裂和呼吸功能不全，归因于肺动脉高压。患者接受了体外膜氧合治疗，但肺动脉高压持续存在。在体外膜氧合治疗 10 天后，进行了肺活检。结果显示肺泡毛细血管发育不良。由于预后不良，停止了体外膜氧合治疗，患者死亡。
这是腹裂与肺泡毛细血管发育不良之间关联的首次描述。在患有严重呼吸功能不全和肺动脉高压的腹裂新生儿中，应考虑肺泡毛细血管发育不良。
