Bilateral cystic adenomatoid lung malformation type III – a rare differential diagnosis of pulmonary hypertension in neonates
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M. C. Banerjea
Abstract
Congenital cystic adenomatoid lung malformation (CCAM) is a rare disorder characterized by replacement of normal pulmonary tissue with cysts of variable size and distribution. Only sporadic cases of CCAM type III have been described that were stillborn or died in the early neonatal period. To our best knowledge we report the first infant with bilateral CCAM type III that was not ventilator-dependent for the first 10 weeks of life and survived until the age of 5 months. Postnatally the newborn presented with signs of only mild respiratory distress and chest radiograph did not show evidence of cystic lesions. In the following weeks the major clinical symptom was persistent pulmonary hypertension with severe tachypnea, O2 requirement and CO2 retention. Pulmonary hypertension did not respond to HFOV ventilation, NO ventilation or prostacyclin inhalations.
Conclusions: (1) Persistent pulmonary hypertension can be a major clinical feature in neonates with bilateral CCAM type III. (2) A normal chest X-ray does not exclude the possibility of bilateral type III CCAM. (3) Computerized tomography appears to be superior to conventional chest X-ray imaging in detection of small cystic lesions in infants with CCAM type III. (4) In cases of prenatally suspected CCAM type III, parents should be informed that short-term survival is possible.
Copyright © 2002 by Walter de Gruyter GmbH & Co. KG
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Artikel in diesem Heft
- Editorial
- The odds of delivering one, two or three extremely low birth weight (<1000 g) triplet infants: a study of 3288 sets
- Evaluation of a device for objective determination of cervical consistency: A pilot study of devices validity on uterine specimens obtained by total abdominal hysterectomy for benign uterine disease
- Clinical risk factors for deep venous thrombosis in pregnancy and the puerperium
- Waterbirth: water temperature and bathing time mother knows best!
- Anal sphincter injury during vaginal delivery – an argument for cesarean section on request?
- Doppler sonography of uterine arteries at 20–23 weeks: risk assessment of adverse pregnancy outcome by quantification of impedance and notch
- Cadmium concentration in maternal and cord blood and infant birth weight: a study on healthy non-smoking women
- The effects of oral carbohydrate administration on fetal acid base balance
- Labor induction and meconium: in vitro effects of oxytocin, dinoprostone and misoprostol on rat ileum relative to myometrium
- Catch-up growth in fetal malnourished term infants
- Role of surfactant inhibitors in amniotic fluid in respiratory distress syndrome
- Congenital tuberculosis proven by percutaneous liver biopsy: report of a case
- A case of peripartum cardiomyopathy with a transient increase of plasma interleukin-6 concentration occurred following mirror syndrome
- Bilateral cystic adenomatoid lung malformation type III – a rare differential diagnosis of pulmonary hypertension in neonates
- An unexpected case of primary pulmonary hypertension of the neonate (PPHN). Potential role of topical administration of enoxolone
- Urethral catheterization in neonates – how far is too far?
- Congress Calendar
