Primary congenital pulmonary hypoplasia of a neonate

Abstract

Pulmonary hypoplasia is a rare but usually lethal disease. We report a full-term male neonate who presented with respiratory failure immediately after birth. Chest X-ray revealed a small lung volume despite advanced ventilator support. Respiratory failure persisted and this baby died at 40.5 hours of age. The autopsy showed a lung-to-birth weight ratio of 0.69% and a radial alveoli count of 2.97. All this information confirmed the diagnosis of primary congenital pulmonary hypoplasia.

Congenital Acinar Dysplasia (CAD): Case Report of an Extremely Rare Cause of Pulmonary Hypoplasia

“…6 The incidence of congenital pulmonary hypoplasia is 1 in 1000 live birth; this includes both primary and secondary pulmonary hypoplasia. 4 But the incidence of pulmonary hypoplasia due to CAD is unknown and seems to be under recognized, because of diagnostic difficulty. From 1986 to 2004 only seven cases have been reported with a definitive diagnosis of CAD.…”

A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome

“…Congenital pulmonary hypoplasia (CPH) is seen in 1 : 1000 of the newborns and is either primary or secondary. The primary causes of CPH are genetic defects, scimitar syndrome, Down syndrome, and pterygium syndrome, whereas the secondary causes are congenital diaphragmatic hernia, neuromuscular disorders, deficiency of amniotic fluid in pregnancy (oligohydramnios), and disorders accompanied by poor pulmonary blood flow [ 6 , 7 ]. Congenital pulmonary hypoplasia can present itself as respiratory distress, reduced breath sounds, normal or small-bell shaped thorax, or clinical findings of PPH [ 6 ].…”

“…The primary causes of CPH are genetic defects, scimitar syndrome, Down syndrome, and pterygium syndrome, whereas the secondary causes are congenital diaphragmatic hernia, neuromuscular disorders, deficiency of amniotic fluid in pregnancy (oligohydramnios), and disorders accompanied by poor pulmonary blood flow [ 6 , 7 ]. Congenital pulmonary hypoplasia can present itself as respiratory distress, reduced breath sounds, normal or small-bell shaped thorax, or clinical findings of PPH [ 6 ]. The prevalence of persistent pulmonary hypertension of the newborn (PPHN), one of the most frequent causes of respiratory insufficiency in the newborn, is 0.4–0.6 : 1000 [ 8 , 9 ].…”

“…As we also found the same finding in our case, in particular the cases with pulmonary hypoplasia showed insufficient response to therapy [ 9 ]. The rare causes of PPHN are syndromes such as scimitar syndrome, Costello syndrome, Down syndrome, and pterygium syndrome [ 6 , 7 , 13 ]. A limited number of studies have reported that PPHN cases secondary to congenital diaphragmatic hernia give insufficient response to therapy, particularly to therapy with iNO [ 10 ].…”

Сімейна Кістозна Гіпоплазія Легень: Клінічне Спостереження

“…Порожнинні утвори верифікуються в різних відділах бронхіального дерева (частіше на сегментарному і субсегментарному рівнях). Кістозна гіпоплазія сприяє редукції об'єму легені, різкому порушенню дренажної функції бронхів, розвитку хронічного гнійного бронхіту, пневмотораксу, легеневої кровотечі [8]. Діагностують кістозну гіпоплазію тими ж методами, що і просту із обов'язковим включенням комп'ютерної томографії.…”