Pediatric Pulmonary Hypoplasia

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Pediatric Pulmonary Hypoplasia

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Pulmonary hypoplasia (PH) or aplasia is a rare condition that is characterized by incomplete development of lung tissue, which can be unilateral or bilateral. It results in a reduction in number of lung cells, airways, and alveoli that results in impaired gas exchange. See image below.

Pulmonary hypoplasia (PH) may be primary or secondary. Primary PH is extremely rare and routinely lethal. severity of lesion in secondary PH depends on timing of insult in relation to stage of lung development. This typically occurs prior to or after pseudoglandular stage at 6-16 weeks of gestation. In pulmonary hypoplasia, lung consists of incompletely developed lung parenchyma connected to underdeveloped bronchi. Besides disturbances of bronchopulmonary vasculature, re is a high incidence, (approximately 50-85%) of associated congenital anomalies such as cardiac, gastrointestinal, genitourinary, and skeletal malformations. diagnosis can result in a spectrum of respiratory complications ranging from transient respiratory distress, chronic respiratory failure, bronchopulmonary dysplasia to neonatal death in very severe cases. Strict diagnostic criteria are not established for pulmonary hypoplasia; various parameters such as lung weight, lung weight to body weight ratio, total lung volume, mean radial alveolar count and lung DNA assessment have been used to classify pulmonary hypoplasia. [1, 2]

For lung development to proceed normally, physical space in fetal thorax must be adequate, and amniotic fluid must be brought into lung by fetal breathing movements, leading to distension of developing lung. Several studies have demonstrated that gestation age at rupture of membranes (15-28 weeks gestation), latency period (duration between rupture of membranes and birth) and amniotic fluid index (AFI of less than 1 cm or 5 cm) can influence development of pulmonary hypoplasia. [3]

Maintenance of fetal lung volume plays a major role in normal lung development. Normal transpulmonary pressure of about 2.5mm Hg allows fetal lung to actively secrete fluid into lumen. [4] effect of stretch of lung parenchyma induces and promotes lung development. Studies in sheep have demonstrated that tracheal ligation and refore increased lung distension, accelerates lung growth whereas chronic tracheal fluid drainage has opposite effect. [5] Cohen and colleagues have found that in-utero overexpression of cystic fibrosis transmembrane conductance regulator (CFTR) increased liquid secretion into lung, accelerating lung growth in a rat model. [6]

Oligohydramnios is considered to be an independent risk factor for development of pulmonary hypoplasia. This is likely due to reduced distending forces on lung. Studies have demonstrated that severe oligohydramnios decreased lung cell size, alters cell shape and may also negatively affect Type I cell differentiation which ultimately induces pulmonary hypoplasia.

It has been postulated that Rho-ROCK pathway can affect growth of lung epilium. Embryonic mouse models have demonstrated that ROCK protein inhibitor decreases number of terminal lung buds. re are currently several groups studying role of Rho/ROCK pathway which has potential rapeutic implications in reversal of lung hypoplasia. [7, 8]

Several growth factors such as fibroblast growth factor (FGF), epidermal growth factor (EGF), vascular endolial growth factor (VEGF) and platelet derived growth factor (PDGF), promote cell proliferation and differentiation. Transforming growth factor family proteins like TGFß1 can oppose se effects.

Embryologically, lungs arise from foregut. Thyroid transcription factor 1 (TTF-1) is thought to be earliest embryologic marker associated with cells committed to pulmonary development. FGF signaling is thought to be essential in formation of TTF-1 expressing cells and this is thought to occur even before pseudoglandular stage of lung development. Sonic hedgehog (SHH) signaling is furr responsible for branching morphogenesis and mesenchymal proliferation. Disruption of any of se pathway may result in primary pulmonary hypoplasia. [9, 10, 11, 12]   

FGF7 and FGF10 promote epilial proliferation and formation of bronchial tree. Overexpression of FGF10 can also stimulate formation of cysts in rat lung. [13] EGF promotes lung branching and Type II alveolar cell proliferation. PDGF plays a crucial role in alveolarization. VEGF promotes angiogenesis and differentiation of embryonic mesenchymal cells into endolial cells. Bone morphogenetic protein was thought to oppose lung growth; however recent data suggests that in presence of mesenchymal cells, BMP4 is a potent inducer of tracheal branching. [14, 15, 16, 17] Aberrant expression of se growth factor proteins in amniotic fluid during pregnancy have been implicated in abnormal lung development. Interestingly, higher concentrations of VEGF are seen in amniotic fluid in second and third trimester and may be a molecular marker for hypoxia which requires furr investigation. [15]

pathogenesis of PH associated with congenital diaphragmatic hernia (CDH) remains unclear. Several mechanisms have been suggested. nitrofen model of CDH is widely accepted. Nitrofen is a human carcinogen and retinoid acid signaling pathway is essential for normal development of diaphragm. Perturbation of this pathway with compounds such as nitrofen, can induce CDH and PH. Esumi and colleagues demonstrated that that administration of insulin-like growth factor 2 (IGF2) to nitrofen-induced hypoplastic lungs lead to alveolar maturation. [18, 13, 19, 20, 21, 22] Furrmore, recent data suggests that prenatal treatment with retinoic acid results in increased levels of placental IGF2 and promotes both placental and fetal lung growth in nitrofen induced CDH. [23]

Interestingly, erythropoietin (EPO) is a direct target of retinoic acid. A recent study has demonstrated decreased levels of EPO mRNA in liver and kidney of rats which may explain modifications in pulmonary vasculature in CDH. [22]

A recent study has also suggested a possible role of interleukin 6 (IL-6) in inducing catch-up growth particularly in nitrofen pre-treated explant fetal rat lungs. [24]

In cases of congenital diaphragmatic hernia (CDH) associated with pulmonary hypoplasia, hypertrophy of contralateral lung has been demonstrated, with associated pulmonary artery hypertension. hypoxemia in pulmonary hypoplasia stems from hypo and right-to-left extrapulmonary shunting.

United States

true incidence of pulmonary hypoplasia is unknown. reported incidence is between 9 to 11 per 100,00 live birth which is an underestimation, as infants with lesser degrees of hypoplasia likely survive in neonatal period. [1] Incidence also varies by etiology. Most cases are secondary to congenital anomalies (such as congenital diaphragmatic hernia and cystic adenomatous malformations) or complications related to pregnancy that inhibit lung development. se include, but are not limited to, renal and urinary tract anomalies, amniotic fluid aberrations, diaphragmatic hernia, hydrops fetalis, skeletal and neuromuscular disease and conditions like s, chylothorax and intrathoracic masses that cause compression of fetal thorax. [2]  

incidence of neonatal pulmonary hypoplasia in mid trimester (18-26 weeks gestation) preterm rupture of membranes ranges from 9-28%, with variability attributed to differing diagnostic criteria for pulmonary hypoplasia.


International incidence of pulmonary hypoplasia is not known. In Canada, estimated incidence of CAM is 1 case per 25,000-35,000 pregnancies. According to CDH study group incidence of CDH is 1 in every 2000-4000 births and accounts for 8% of all congenital anomalies. In , occurrence of CDH ranges from 1.7 to 5.7 cases per 10,000 live births, depending on study population and remains largely unchanged. [8, 25] However, re is no direct correlation between se predisposing lesions to incidence of pulmonary hypoplasia.

In different studies, mortality rates associated with PH are reported to be as high as 71-95% in perinatal period. [1, 2]  

following conditions increase risk of mortality [25] :

Earlier gestational age at rupture of membranes, particularly at less than 25 weeks of gestation

Earlier delivery (decreased latency period)

Right-sided lesion

Presence of genetic anomalies

To avoid mortality from severe lung hypoplasia in association with CDH or CAM, fetal surgical intervention has been attempted. Most studies report a mortality rate of 25-30% in neonates with CDH and CAM at high volume centers; mortality can be as high as 45% at peripheral care centers. However, in or cystic lung lesions, most are clinically asymptomatic and may not need aggressive management. [26]

Risk factors for a poor outcome include presence of hydrops fetalis, with a mortality rate as high as 80-90%. Or indicators include type of CAM and its size. All of se factors reflect degree of pulmonary compromise with lesions that result in varying degrees of pulmonary hypoplasia.

re is a recent retrospective study from Barcelona that studied 60 cases of pulmonary hypoplasia between 1995 to 2014, that found a mortality rate of 47% in first 60 days of life and upto 75% in first day of life. [27]  

No racial predilection has been noted.

No sex predilection has been noted.

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Transcription Factors

Growth Factors

Thyroid transcription factor-1 (TTF-1) [12]

Vascular endolial growth factor receptors (VEGFR1 and VEGFR2) [15]

GATA-4 [20]

Insulinlike growth factors (IGF-1 and IGF-2) and ir receptors (IGF-1R and IGF-2R) [18]

FOG-2 [23]

Epidermal growth factors and its receptor family (eg, ErbB receptors) [21]

Hepatocyte nuclear factor (HNF3ß10)

Mitogen-activated protein kinases


Connective tissue growth factor [31]

Small Fetal Thoracic Volume

Prolonged Oligohydramnios

Decreased Fetal Breathing Movements

Congenital Heart Diseases With Poor Pulmonary Blood Flow


Fetal renal agenesis

Central nervous system (CNS) lesions

Tetralogy of Fallot

Cystic adenomatoid malformation (CAM)

Urinary tract obstruction

Lesions of spinal cord, brain stem, and phrenic nerve

Hypoplastic right heart

Pulmonary sequestration

Bilateral renal dysplasia

Neuromuscular diseases (eg, myotonic dystrophy, spinal muscular atrophy)

Pulmonary artery hypoplasia

Pleural effusions with fetal hydrops, hydrothorax

Bilateral cystic kidneys

Arthrogryposis multiplex congenital secondary to fetal akinesia

Scimitar syndrome causing a unilateral right-sided pulmonary hypoplasia

Thoracic neuroblastomas

Prolonged rupture of membranes (PROM)

Maternal depressant drugs

Trisomies 18 and 21

Malformations of thorax (eg, asphyxiating thoracic dystrophy)

Premature PROM



Diaphragmatic anomalies (eg, abdominal wall defects, eventration of diaphragm)

Potter syndrome



Musculoskeletal disorders (eg, achondroplasia, thanatophoric dysplasia, osteogenesis imperfecta)




Abdominal masses causing compression




Terry W Chin, , PhD Associate Clinical Professor, Department of Pediatrics, University of California, Irvine, School of Medicine; Associate Director, Cystic Fibrosis Center, Attending Staff Physician, Department of Pediatric Pulmonology, Allergy, and Immunology, Memorial Miller Children’s Hospital

Terry W Chin, , PhD is a member of following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Federation for Clinical Research, American Thoracic Society, California Society of Allergy, Asthma and Immunology, California Thoracic Society, Clinical Immunology Society, Los Angeles Pediatric Society, Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Nandini Kataria,  Fellow in Pediatric Pulmonology, Miller Children’s and Women’s Hospital Long Beach, University of California, Irvine, School of Medicine

Disclosure: Nothing to disclose.

Mary L Windle, Phar Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Girish D Sharma, , FCCP, FAAP Professor of Pediatrics, Rush Medical College; Director, Section of Pediatric Pulmonology and Rush Cystic Fibrosis Center, Rush Children’s Hospital, Rush University Medical Center

Girish D Sharma, , FCCP, FAAP is a member of following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, Royal College of Physicians of Ireland

Disclosure: Nothing to disclose.

Susanna A McColley,  Professor of Pediatrics, Northwestern University, Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children’s Memorial Medical Center of Chicago

Susanna A McColley, is a member of following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, American Thoracic Society

Disclosure: Received honoraria from Genentech for speaking and teaching; Received honoraria from Genentech for consulting; Partner received consulting fee from Boston Scientific for consulting; Received honoraria from Gilead for speaking and teaching; Received consulting fee from Caremark for consulting; Received honoraria from Vertex Pharmaceuticals for speaking and teaching.

Khanh Van Lai,  Fellow in Pediatric Pulmonology, Miller Children’s Hospital, University of California, Irvine, School of Medicine

Khanh Van Lai, is a member of following medical societies: American Thoracic Society

Disclosure: Nothing to disclose.

Bich-Trang H,  Fellow in Pediatric Pulmonology, Miller Children’s Hospital, University of California, Irvine, School of Medicine

Disclosure: Nothing to disclose.

current authors would like to acknowledge contributions of following to this article:

Heidi Connolly, Associate Professor of Pediatrics and Psychiatry, University of Rochester School of Medicine and Dentistry; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center;

Girija Natarajan, , Assistant Professor, Division of Neonatology, Children’s Hospital of Michigan, Wayne State University School of Medicine; and

Ibrahim Abdulhamid, , Associate Professor of Pediatrics, Wayne State University School of Medicine; Director of Pediatric Pulmonary Medicine, Clinical Director of Pediatric Sleep Laboratory, Children’s Hospital of Michigan

Disclosure: Nothing to disclose.

Pediatric Pulmonary Hypoplasia



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