Lung hypoplasia

Alfonso LF, Arnaiz A, Alvarez FJ, Qi BQ, Dies-Pardo JA, Vallisi-Soler A, Tovar JA (1996) Lung hypoplasia and surfactant system immaturity induced in the fetal rat by prenatal exposure to nitrofen. Biol Neonate 69 94-100... 

Collins MH, Moessinger AC, Kleinerman J, Bassi J, Rosso P, Collins AM, James LS, Blanc WA (1985) Fetal lung hypoplasia associated with maternal smoking A morphometric analysis. Paediatr Res, 19 408-412. 

ACE inhibitors Oligohydramnios, potential lung hypoplasia, postnatal renal failure... 

Three cases of lung hypoplasia after prenatal exposure to valproate have been reported in two female siblings and an unrelated girl (115). The two siblings had no other malformations, whereas the third neonate had a number of major birth defects. All three died a few hours after birth. The possibility that lung hypoplasia was caused by valproate remains speculative. 

Janas MS, Arroe M, Hansen SH, Graem N. Lung hypoplasia—a possible teratogenic effect of valproate. Case report. APMIS 1998 106(2) 300-4. 

PPHN is associated with diverse cardiopulmonary disorders, including asphyxia, sepsis, meconium aspiration syndrome, congenital diaphragmatic hernia, respiratory distress syndrome, lung hypoplasia, and others, or can... 

Pasutto, R, Sticht, H., Hammersen, G. et al. 2007. Mutations in STRA6 cause a broad spectrum of malformations including anophthalmia, congenital heart defects, diaphragmatic hernia, alveolar capillary dysplasia, lung hypoplasia, and mental retardation. Am J Hum Genet 80 550-560. 

Polycystic kidney diseases can also be detected in utero. In the typical presentation of the recessive type, the kidneys are very large (4-4 - -F8 SD) with increased echogenicity and lack of cortico-medul-lary differentiation more unusual presentations include the presence of medullary cysts or reversed cortico-medullary differentiation (Fig. 13.15) oligohydramnios and lung hypoplasia indicate a poor prognosis. In the dominant type, the renal cortex appears hyperechoic, increasing the cortico-medul-lay differentiation. Cysts may be detected in utero. Other cystic diseases are usually related to syndromes (i.e., Meckel-Gruber syndrome and Bardet-Biedl syndrome) (Avni et al. 2006). 

After birth, severe bilateral renal disease can be associated with pneumothorax, lung hypoplasia, and life-threatening respiratory distress (Fig. 13.17) (Perlman et al. 1973). This respiratory failure is an emergency situation, and mechanical respiratory assistance is mandatory even before any treatment can be considered for the urinary tract anomaly. Conversely, whenever a neonate presents seemingly spontaneous pneumothorax, an US examination should be performed in order to verify the status of the urinary tract. 

Fig. 13.17. Right pneumothorax and bilateral lung hypoplasia in a neonate with recessive type polycystic kidney disease...

Mature cartilage or bone lungs kidneys liver pancreas adrenal gland pituitary gland Fairly low Hypoplasia secondary damage to the fine vasculature and connective tissue elements... 

Children with complex congenital heart disease and hypoplasia of either ventricle frequently receive a bidirectional superior cavopulmonary shunt as a palliative procedure in anticipation of further surgical operations to reinstate all systemic venous return directly to the pulmonary artery. Thus, pulmonary blood flow occurs passively through the lungs in the absence of a subpulmonary ventricle. The pulmonary vascular resistance may influence both pulmonary blood flow and adequate systemic ventricular preload. Following such a superior cavopulmonary shunt intense cyanosis may occur... 

Complete agenesis of the pancreas is usually incompatible with life. Therefore, pancreatic aplasia or hypoplasia is uncommon in humans. Patients with pancreatic hypoplasia will have a normal development of the pancreas in size and shape but later in life will have a replacement of the glandular elements with fatty tissue. Some of these patients will present with an abnormal exocrine pancreatic insufficiency and normal endocrine function. Children with syndromes such as Schwachman-Diamond, Beckwith-Wiedemann, polysplenia bilobed lungs, and congenital heart disease have a higher relative risk of hypoplasia of the pancreas (Gazelle et al. 1998). 

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