Neonatal pulmonary hypoplasia after prolonged leakage of amniotic fluid.

The clinical, radiological, and pathological features of the ''oligohydramnios tetrad'' (Potter''s facies, limb malpositions, pulmonary hypoplasia, and growth retardation) resulting from chronic amniotic fluid leakage are described in 2 infants who died of respiratory failure within 12 hours of delivery. A third infant with a partial form of the syndrome had prolonged respiratory symptoms and survived the neonatal period: the course of the disease and the radiological findings were compatible with a hypothetical diagnosis of partial pulmonary hypoplasia. Pulmonary hypoplasia should be recognized as a cause of chronic respiratory symptoms in viable infants.     

Neonatal pulmonary hypoplasia after prolonged leakage of amniotic fluid

The clinical, radiological, and pathological features of the 'oligohydramnios tetrad' (Potter's facies, limb malpositions, pulmonary hypoplasia, and growth retardation) resulting from chronic amniotic fluid leakage are described in 2 infants who died of respiratory failure within 12 hours of delivery. A third infant with a partial form of the syndrome had prolonged respiratory symptoms and survived the neonatal period: the course of the disease and the radiological findings were compatible with a hypothetical diagnosis of partial pulmonary hypoplasia. Pulmonary hypoplasia should be recognized as a cause of chronic respiratory symptoms in viable infants.     

Neonatal respiratory distress due to mumps.

We report an infant of 35 weeks'' gestation who developed severe respiratory distress and pneumonitis due to perinatal mumps virus infection.     

Congenital dacryocystoceles controlled by nCPAP via nasal mask in a neonate

Congenital dacryocystocele is a relatively rare type of nasolacrimal duct obstruction that may induce respiratory distress during the early neonatal period. We encountered a case of bilateral congenital dacryocystoceles with intranasal cysts in a premature infant delivered at 34 weeks of gestation. The patient developed symptoms of respiratory failure immediately after birth, but no ophthalmologic symptoms. Treatment with nasal continuous positive airway pressure via a nasal mask, instead of a nasal prong, effectively relieved the symptoms. Early diagnosis and appropriate treatment are critical for infants with nasal obstruction.     

Sleep apnoea profile in preterm infants recovering from respiratory distress syndrome.

Polygraphic recordings were made on 10 preterm infants recovering from respiratory distress syndrome and 12 healthy preterm control infants at 40, 52, and 64 weeks'' conceptual age to study the influence of respiratory distress syndrome on the development of the sleep apnoea profile. Two significant differences were found: infants with respiratory distress syndrome not only had a lower incidence of non-obstructive apnoea and periodic breathing at 40 weeks but also a persistently higher incidence of obstructive and mixed apnoea at 52 and 64 weeks'' conceptual age; the latter finding being related to non-rapid eye movement sleep only. While the lower incidence of both types of apnoea at 40 weeks suggests an advanced maturation of respiratory drive, the persistence of obstructive and mixed apnoea related to non-rapid eye movement sleep may reflect the impact of respiratory distress syndrome on airway structures.     

Combined Esophageal and Pyloric Atresia without Tracheoesophageal Fistula in Down's Syndrome

This is the first report of a newborn infant with Down's syndrome associated with combined congenital pyloric and esophageal atresias without a tracheoesophageal fistula (Gross A type). A mother was noted to have marked abdominal distension and hydramios was suspected at 28 weeks gestation. Ultrasonographic findings of her abdomen demonstrated that the fetus was suspected of having congenital duodenal and esophageal atresia, from the large doubleecho-free spaces occupying the greater part of the abdomen. Chromosome analysis of the amniotic fluid showed a karyotype of 21 trisomy. The newborn baby had clinical characteristics of Down's syndrome and severe abdominal distension after birth. At 19 days after birth, autopsy revealed pyloric and esophageal atresias without trache oesophageal fistula. We conclude that ultrasonographic examination is useful for the prenatal diagnosis of a patient with gastrointestinal obstruction combined with esophageal atresias, without trache oesophageal fistula.     

Use of DNA estimation for growth assessment in normal and hypoplastic fetal lungs.

Total DNA was estimated in the lungs of 80 fetuses and newborn infants varying in gestation from 14 weeks to term. In fetuses of appropriate weight for gestational age total lung DNA increased at a constant rate from about 35 mg at 17 weeks'' gestation to 480 mg at term. The lungs of immature fetuses were heavier and contained more DNA relative to body weight than did those of mature infants. Small-for-dates infants had lower lung DNA levels for gestation than infants with weights appropriate for gestational age, but there was no difference when lung DNA was corrected for body weight. Lung hypoplasia defined in terms of lung/body weight ratio was associated with low lung DNA content for gestation, even when corrected for body weight. The total lung DNA at 34-40 weeks'' gestation in infants with lung hypoplasia associated with fetal anuria or urinary outflow obstruction was equivalent to that seen in normal fetuses at 20-22 weeks'' gestation. We conclude that the early second trimester is a critical period for human fetal lung growth.     

The Marshall-Smith syndrome: a review of the laryngeal complications

The Marshall-Smith syndrome is characterised by a triad of facial dysmorphism, failure to thrive and accelerated osseous maturation. We report a further case of this rare syndrome with the unusual but previously reported complication of laryngeal hypoplasia and review the associated laryngeal anomalies that have been reported to date. Conclusion Severe airway obstruction due to congenital anomalies must be excluded in any dysmorphic child presenting with respiratory distress at birth. Rapid airway assessment will enable early and appropriate intervention and may be important when deciding on the long-term plan for the infant. Received: 28 March 1996 and in revised form: 13 November 1996 / Accepted: 13 November 1996     

Fetal cytomegalovirus infection manifesting as transient pancytopenia

We encountered a patient with a fetal cytomegalovirus infection manifesting as pancytopenia and thoracic hypoplasia. The fetal anemia was treated by transfusion via the umbilical cord, and did not progress after 22 weeks' gestation. The neutropenia resolved spontaneously, and only thrombocytopenia was persistent at birth. The severe thoracic hypoplasia led to pulmonary hypertension and required intensive postnatal respiratory management. Our experience suggests that pancytopenia is a possible manifestation in fetuses infected with cytomegalovirus. This may be transient, resolving spontaneously during fetal life; however, caution should be taken with blood counts, particularly platelet counts, after delivery. In addition, clinicians should carefully follow the thoracic volume in cytomegalovirus‐infected fetuses and consider the possibility of postnatal severe respiratory insufficiency.     

Acute airway obstruction in a seven-month-old infant with epiglottitis

A seven-month-old male infant with epiglottitis developed acute airway obstruction in the operating room during halothane induction. This case suggests that epiglottitis should be suspected in any child, regardless of age, who presents with stridor, respiratory distress, dysphagia, or "tripoding," and it reiterates the need for prompt airway management.     

High frequency oscillation for preterm infants with severe respiratory failure.

High frequency oscillation (HFO) as rescue treatment for preterm infants with severe respiratory failure has been assessed and prognostic factors identified. Thirty six infants with a median gestational age of 27 weeks were studied. Immediately before transfer to HFO, the infants were receiving an inspired oxygen concentration of > or = 85% and/or a mean airway pressure of > or = 12 cm H2O and had a median alveolar-arterial oxygen gradient (A-aDO2) of 73.28 kPa (range 49.34-89.91). Seventeen infants subsequently died. Comparison of those 17 with the remaining 19 infants demonstrated that respiratory distress syndrome and persistent fetal circulation were associated with a significantly better outcome than pulmonary airleak. The A-aDO2 after two and six hours on HFO was significantly higher in those infants who survived compared with those who died. We conclude that a diagnosis of pulmonary airleak and failure to show early improvement in respiratory status indicate a poor prognosis when HFO is used as rescue treatment.     

Nitric oxide inhalation therapy in very low-birthweight infants with hypoplastic lung due to oligohydramnios

BACKGROUND: Although nitric oxide inhalation (iNO) therapy improves arterial oxygenation and reduces the rate of extracorporeal membrane oxygenation in term neonates, the efficacy of this therapy in premature infants is controversial. The objective of the present study was to determine whether iNO therapy improves the survival of very low-birthweight infants with pulmonary hypoplasia due to prolonged rupture of membrane. METHODS: A retrospective comparative study of very low-birthweight infants with pulmonary hypoplasia due to oligohydramnios who had or had not been treated with iNO therapy, was performed (iNO-treated group, eight infants; control group, 10 infants). A neonate was considered to have pulmonary hypoplasia due to oligohydramnios if the following conditions were satisfied: (i) artificial surfactant treatment did not improve the respiratory distress; (ii) prolonged rupture of membrane (PROM) continued for more than 5 days with oligohydramnios; and (iii) sufficient arterial oxygenation did not occur even after giving 100% oxygen, and more than 8 cm H(2)O of mean airway pressure was needed to maintain arterial oxygenation. RESULTS: Nitric oxide inhalation improved arterial oxygenation rapidly and consistently in all eight infants with pulmonary hypoplasia. All eight iNO-treated infants survived longer than 28 days, while five of the 10 control infants died within 24 h of birth (P < 0.05). Before starting iNO, seven of the eight treated infants had shown persistent pulmonary hypertension, which was confirmed by echocardiography. No iNO-treated infant had IVH greater than grade 1, while one control infant had grade 2 IVH. All six long-term survivors in the iNO-treated group are developing normally, while only two of the control infants are developing normally as of February 2002. CONCLUSIONS: The majority of the infants with pulmonary hypoplasia due to oligohydramnios had persistent pulmonary hypertension. iNO improved the arterial oxygenation and significantly improved the survival rate. A controlled study to determine whether iNO therapy improves the survival rate of preterm infants with pulmonary hypoplasia due to oligohydramnios is necessary.     

Dry lung syndrome: complete airway collapse mimicking pulmonary hypoplasia?

We observed a premature baby born after severe oligohydramnios who could not be ventilated efficiently even with very high pressures immediately after birth, but who, after cessation of resuscitation attempts, recovered spontaneous sufficient breathing during the following hour. After this experience we searched our case records for other newborns with dry lung syndrome using the following definition: (1) premature birth after prolonged leakage of amniotic fluid, (2) very high ventilatory requirement after birth, (3) dramatic improvement during the first 24 to 36 h and (4) respiratory distress syndrome and infection excluded. Among 93 prematures with rupture of membranes for 4 days or more we found 3, including the index case, matching this definition. Conclusion Dry lung syndrome appears to be a distinct clinical entity that is possibly underrecognised but recognisable and that merits further study. Its pathogenesis may imply complete collapse of small airways to a degree that capillary forces impede distension by ordinary ventilatory pressures. Received: 18 December 1997 and in revised form: 9 March 1998 / Accepted: 13 March 1998     

Intrauterine Chlamydia Trachomatis Infection in a Premature Infant

Intrauterine Chlamydia trachomatis infection was strongly suspected in a premature infant born in the 32nd week of gestation. The membranes were artificially ruptured at the time of delivery. This infant showed a high titer of specific IgM antibody to Chlamydia trachomatis at one hour after birth. He showed mild respiratory distress and was treated with oral erythromycin for three weeks. He was discharged home at the age of 46 days.     

Inflammation and surfactant

An intact and well-functioning pulmonary surfactant system is critical for normal respiration and protection from lung infection. Surfactant is comprised of phospholipids that reduce surface tension and greatly reduce the work of breathing. The other major component consists of surfactant-associated proteins, which optimise the biophysical function of phospholipids and/or play an important role in host defence by acting as collectins. Alteration of surfactant composition and function occurs with various inflammatory disorders that affect the airways or the lung parenchyma including asthma, infant respiratory distress syndrome/bronchopulmonary dysplasia, cystic fibrosis, acute respiratory distress syndrome and interstitial lung disease. Although surfactant replacement therapy is indicated for infant respiratory distress syndrome, there is no well-proven role for exogenous surfactant in the treatment of inflammatory lung disorders at the present time.     

Systemic air embolism in an extremely low birthweight infant treated with high-frequency oscillatory ventilation

An extremely low birthweight infant in whom systemic air embolism occurred during high-frequency oscillatory ventilation is reported. He was delivered at 25 weeks and 6 days gestation weighing 600 g. He was diagnosed as having respiratory distress syndrome and had intermittent mandatory ventilation. At 2 days of age, there was pulmonary hemorrhage and high-frequency oscillatory ventilation was begun. The maximum mean airway pressure during high-frequency oscillatory ventilation was 22 cmH₂O. At 9 days of age, X-ray film revealed air in the intracardiac cavity and major systemic vessels. Systemic air embolism can occur in an extremely low birthweight infant having high-frequency oscillatory ventilation with a mean airway pressure of 22 cmH₂O or higher.     

Absent pulmonary valve syndrome diagnosed by fetal echocardiography

Prenatal echocardiographic diagnosis of heart defects is important because it permits counseling of the parents with regard to prognosis and treatment options and prepares the medical team for the treatment postnatally. A male infant with absent pulmonary valve syndrome diagnosed prenatally at 22 weeks' gestation is reported. This congenital anomaly is characterized by absent or rudimentary pulmonary valve cusps, conoventricular septal defect, and massive dilatation of the pulmonary arteries. Soon after delivery the infant developed cyanosis and respiratory distress. The infant was placed in prone position for the relief of bronchial compression and nasal continuous positive airway pressure was (CPAP) started. Although the clinical status of the infant improved after supportive treatment, he deteriorated acutely and died at the age of five days. Fetal diagnosis remains an integral part of successful management of children with heart disease. Despite the potential benefits of prenatal diagnosis, it is hard to show significant improvement in mortality, especially in severely affected cases.     

Moebius syndrome with pharyngo-laryngeal paralysis in a premature infant

A case of Moebius syndrome in a premature baby is reported. After a phase of neonatal severe respiratory distress syndrome, the baby presented with a persistent facial paralysis, already present at birth and inability to close the eyes (also present in her father), without ophthalmoplegia. An unusual pharyngeal and laryngeal paralysis was also present: it led to tracheal intubation then tracheostomy and gastrostomy. CT scan at 15 months of age showed hypoplasia of brain stem. The difficulties of managing bulbar paralysis in such a premature baby are emphasized.     

Factors adversely affecting lung growth

Antenatal lung growth is adversely affected in conditions where there is intra-or extrathoracic compression, impaired fetal breathing movements and/or reduction in amniotic fluid volume. Postnatally, factors in infancy most commonly associated with abnormal lung function are passive smoking and premature delivery, its associated complications and management. Many conditions affecting lung growth are readily diagnosed by antenatal ultrasonography. Postnatally, pulmonary hypoplasia should be suspected if an infant requires high airway pressures during resuscitation and subsequent ventilatory support, in the absence of severe parenchymal disease on the chest radiograph. Antenatal therapy has been directed at relieving lung compression, but the efficacy of such interventions has not been proven in randomized trials. Certain disorders associated with impaired lung growth have a high perinatal mortality. Infants who are less severely affected suffer respiratory symptoms with lung function abnormalities or may only be distinguished by chronic tachypnoea during infancy.     

FOETAL TRANSFUSION AND NEONATAL DIALYSIS

Knowledge of the place and the hazards of intra-uterine transfusion in the severely erythroblastotic foetus, and of the management after delivery, is steadily growing. This procedure was undertaken in an infant who was subsequently delivered at 34 weeks' gestation by emergency Caesarean section following the development of foetal distress. Respiratory distress developed soon after birth and he remained anuric during the 12 days that he survived. An exchange transfusion to ‘wash out’ maternal antibody, and a peritoneal dialysis to combat severe hyperkalaemia were undertaken at the age of 3 hours and 4 days respectively. At post-mortem, the kidneys were found to be nonviable. The early post-natal events were monitored by observations of the electrolytes and the acid-base balance; the fluids used in dialysis and the dialysates were also analysed. These observations are reported and their implications are discussed.