Pulmonary Hyalinising Granuloma: A report of two cases.

Pulmonary hyalinising granuloma (PHG) is a rare fibrosclerosing inflammatory lung condition of unknown aetiology. It is characterised by solitary or multiple pulmonary nodules that are usually found incidentally while imaging the chest for other reasons. We report two cases of histologically proven PHG diagnosed at the Royal Hospital, Muscat, Oman. The first case was a 71-year-old male patient who presented in 2010 with a dry cough, weight loss and bilateral pulmonary nodules. The second case was a 58-year-old male patient who presented in 2012 and was found to have incidental bilateral pulmonary nodules on chest X-ray. Both patients were started on prednisolone and on follow-up the PHG nodules remained stable. Although there is no definitive treatment, PHG generally has an excellent prognosis.

Prenatal diagnosis of Kniest dysplasia with three-dimensional helical computed tomography.

OBJECTIVE: Fetal three-dimensional helical computed tomography (3D-CT) has attracted attention in the diagnosis of fetal skeletal dysplasias because of limited diagnostic capabilities of standard ultrasonography to delineate the skeleton. Here we report the first instance of diagnosing Kniest dysplasia with 3D-CT. METHODS: Fetal 3D-CT was performed for a fetus at 28 weeks' gestation after ultrasonography at 24 weeks had shown moderate shortening of the limbs, mild narrow thorax, and polyhydramnios. The imaging parameters were set so as to reduce estimated fetal irradiation dose to 12.39 mGy of the CT dose index volume and 442 of the dose length product. RESULTS: Fetal 3D-CT revealed dumbbell-shaped femora and platyspondyly with coronal cleft of the lumbar vertebral body. This warranted a diagnosis of Kniest dysplasia and corresponded well with postnatal radiographic findings. In retrospect, however, spinal deformation was somewhat underestimated due to image smoothing associated with image processing in 3D-CT. Genetic testing for COL2A1 confirmed Kniest dysplasia; i.e., a de novo mutation of A-C transversion at the splice acceptor site of the 3' end of intron 16. CONCLUSIONS: The combined use of 3D-CT with ultrasonography is a power tool for the prenatal diagnosis of congenital skeletal dysplasias.

ABCA3 Deficiency: an unusual cause of respiratory distress in the newborn.

Respiratory Distress Syndrome (RDS) is due to deficiency of surfactant and commonly occurs in preterm babies. We report the first confirmed case in Northern Ireland of ABCA3 transporter deficiency which is a rare but important cause of RDS in term babies.A 38 week gestation female infant developed respiratory distress at four hours of age. Chest radiography was consistent with RDS. The baby required repeated doses of surfactant, each resulting in transient periods of decreased ventilatory requirement and improvement in blood gases, but unfortunately she did not survive.DNA sequencing demonstrated two different mutations in the ABCA3 gene, one inherited from each parent. The baby was therefore a compound heterozygote, and both mutations were thought to be functionally significant.ABCA3 transporter deficiency is a genetic disorder that is increasingly recognized as a cause of RDS in term babies in whom congenital deficiency of surfactant B and abnormalities of surfactant protein C have been excluded. It should be considered in mature babies who develop severe RDS.

Hyaline membrane and neonatal radiology--Ian Donald's first venture into imaging research.

While he was working at the Royal Postgraduate Medical School, Ian Donald (later Regius Professor of Midwifery, University of Glasgow and a pioneer of diagnostic ultrasound) collaborated with Albert Claireaux and Robert Steiner on histological and radiological studies of hyaline membrane disease. In 1953, Donald and Steiner published thefirst radiological study of a series of cases. The success of this research stimulated Donald's interest in imaging technologies.

Diagnostic imaging of hyaline membrane disease.

The case of a premature infant with hyaline membrane disease (respiratory distress syndrome) is discussed. On chest X-ray reduced volume and transparency of both lungs with ground-glass appearance and presence of bilateral air bronchogram was visualized. These findings are analyzed with respect to possible diagnostic alternatives.

Potter sequence complicated by congenital cystic lesion of the bladder.

We report a case complicated by oligohydramnios, pulmonary hypoplasia, bilateral renal dysplasia, and cystic lesion of the bladder. He was clinically compatible with Potter sequence. Congenital cystic bladder is the rarest form of the bladder. We can find no report of Potter sequence complicated by cystic lesion of the bladder. This lesion was similar to multilocular bladder. The diagnosis was confirmed it by autopsy, magnetic resonance imaging, and urography after his death.

Pulmonary oxygen consumption: a hypothesis to explain the increase in oxygen consumption of low birth weight infants with lung disease.

OBJECTIVE: We determined pulmonary oxygen consumption (VO2lung) in low-birthweight infants with acute lung disease to help explain the greater whole-body oxygen consumption (VO2wb) in these infants with than in those without lung disease. METHODS AND MATERIALS: Eleven infants (birth weight 1,076+/-364 g; gestational age 28+/-3 weeks) undergoing mechanical ventilation for respiratory distress syndrome were studied in their first week of life. We measured VO2wb by indirect calorimetry and simultaneously determined systemic oxygen uptake (VO2Fick) as the product of cardiac output (echocardiography) and the arterial-mixed venous oxygen content difference (cooximetry) assuming that VO2wb-VO2Fick accounts for VO2lung. Right atrial blood samples were used to determine mixed venous oxygenation, and infants were excluded if samples returned saturations greater than 89%. RESULTS: VO2lung was 1.92+/-1.74 ml x kg(-1) x min(-1), representing 25% of their VO2wb (7.58+/-1.48 ml x kg(-1) x min(-1)). VO2lung was not correlated with clinical measures of acute disease severity. However, infants with the most severe changes on follow-up radiography (Edwards score 5 as assessed by radiologist blinded for VO2 data) all had a VO2lung level greater than 2.0 ml x kg(-1) x min(-1). CONCLUSION: VO2lung can account for the elevated metabolic rate in low-birthweight infants with lung injury. We speculate that this reflects in part inflammatory pulmonary processes and may herald chronic lung disease.

Hyaline membrane disease in a term neonate.

Hyaline membrane disease is primarily a disorder of preterm infants. Its occurrence in term infants is very uncommon and therefore may escape attention. We describe a term infant who developed severe respiratory distress soon after birth. Diagnosis of hyaline membrane disease was revealed at autopsy.

Radiological analysis of hyaline membrane disease after exogenous surfactant treatment.

BACKGROUND: Exogenous surfactant treatment of hyaline membrane disease is known to modify the pattern of radiological changes on the chest radiograph. OBJECTIVES: To analyse and attempt to explain the radiological changes observed after exogenous surfactant treatment. Materials and methods. Thirty-nine premature infants with typical hyaline membrane disease. RESULTS: Transient asymmetrical clearing with better aeration of the right lung in the absence of malposition of the tip of the endotracheal tube was observed in nine cases (23 %). This asymmetry was patchy in one case. It was due to a complication of mechanical ventilation in three cases [pneumothorax (n = 2) and pneumomediastinum (n = 1)]. In the other six cases, asymmetrical clearing could be related to the anatomical position of the right main bronchus, which facilitates distribution of surfactant to the right lung. However, the course of these premature infants was similar to that of infants with symmetrical chest radiological findings after treatment. CONCLUSIONS: Asymmetrical clearing of chest radiographs, sometimes patchy, after surfactant treatment requires exclusion of pneumothorax or infection but has no influence on clinical outcome.

Lung disease in premature neonates: impact of new treatments and technologies.

Advances in perinatal medicine and neonatology have dramatically changed clinical outcomes for premature neonates and have ushered in a new era of radiological complexity. "Portable" chest radiographs continue to be the mainstay in diagnostic imaging of fragile newborns, but radiologists may be confronted with new and unexpected radiological expressions of once-familiar disease processes. Familiarity with the radiological impact of emerging treatments in premature neonates is essential for accurate film interpretation.

The hypothalamic-pituitary-adrenal axis in preterm infants weighing < or = 1250 g: association with perinatal data and chronic lung disease.

The hypothalamic-pituitary-adrenal axis (HPA) was examined in 34 ventilated preterm infants weighing < or = 1250 g during the first week of life to evaluate the association between adrenal suppression and subsequent chronic lung disease. The second aim of the study was to detect perinatal and clinical differences between the infants with and without persistent suppression of the HPA after completion of dexamethasone treatment for chronic lung disease. To evaluate the HPA, the corticotropin-releasing hormone stimulation test was performed, and the cortisol and adrenocorticotropic hormone (ACTH) levels were measured by radioimmunoassay. No association could be found between the synthesis of cortisol and ACTH at the end of the first week of life and the development of chronic lung disease. After treatment with dexamethasone, baseline cortisol levels < 138 nmol l(-1) were found in 12 infants (46.2%), 8 of whom (30.8%) had cortisol values below 83 nmol l(-1). The perinatal data of these patients did not differ from infants without HPA suppression. However, the infants with cortisol levels < 83 nmol l(-1) after dexamethasone showed a significantly shorter need for mechanical ventilation and supplemental oxygen (p < 0.01) and a lower incidence of chronic lung disease (p < 0.05).

New-born infants with severe hyaline membrane disease: radiological evaluation during high frequency oscillatory versus conventional ventilation.

OBJECTIVE: The aim of our study was to determine the impact of treatment with exogenous surfactant (ES) and high frequency oscillatory ventilation (HFOV) on the radiological appearance and clinical course of hyaline membrane disease (HMD) in new-born infants. MATERIALS AND METHODS: New-born infants (18) (median weight, 1010 g) with severe HMD (stages 3.5 and 4) who were treated with ES and HFOV were matched by birth weight and severity of disease with 18 new-born infants treated with ES and conventional mechanical ventilation (CV). Chest radiograms taken on days 1, 2/3, 4/5, 7, 14 and 28 were analyzed to check for the severity of generalized parenchymal opacities (GPO), local opacifications, pulmonary interstitial emphysema (PIE), gross air leak, general and localized overinflation, bronchopulmonary dysplasia (BPD) and clinical variables such as survival rates, duration of mechanical ventilation, mean airway pressure and inspired oxygen concentration. RESULTS: At 4 weeks of age, new-born infants treated by HFOV had less severe GPO (median degree 1.5 vs. 3), less PIE (1 vs. 7 patients) and fewer signs of BPD (median BPD degree 1.5 vs. 2.6). The incidence of pneumothorax and of local opacifications were similar in both groups. New-born infants on HFOV had a lower mortality rate (5 vs. 13), needed fewer days of mechanical ventilation (median 15 vs. 23 days) and lower inspiratory oxygen concentrations (median FiO2 0.38 vs. 0.64). CONCLUSION: In new-born infants with HMD, treatment with ES and HFOV resulted in a favourable radiological and clinical outcome as compared to treatment with ES and CV.

Reduction of patient exposure in pediatric radiology.

RATIONALE AND OBJECTIVES: The authors determined whether presently used exposure levels in pediatric imaging can be reduced without loss of information or a decrease in diagnostic accuracy. MATERIALS AND METHODS: Multiple (stacked) image detectors and filters were used to obtain identical compute radiographic images at different exposure levels of neonates with either no active lung disease or hyaline membrane disease. Physical characteristics of the images were measured. A contrast-detail study and a receiver operating characteristic study were conducted to measure observer performance. RESULTS: Physical measurements and results of the contrast-detail study revealed that the dose-reduction images were essentially limited by x-ray quantum noise. Results of the receiver operating characteristic study indicated that diagnostic accuracy did not decrease significantly up to about 75% exposure reduction levels, although image quality rating data decreased with each exposure reduction. CONCLUSION: Decreasing exposure levels to about 75% of current levels may be acceptable in some clinical situations where dose is a concern, such as in pediatric imaging.

[The neonatal respiratory insufficiency syndrome: the role of the chest radiogram]. / Sindrome da insufficienza respiratoria neonatale: ruolo del radiogramma del torace.

INTRODUCTION: Chest radiography is considered the most reliable diagnostic tool to study the respiratory distress syndrome in newborns and one of the few diagnostic investigations which can be used considering the clinical situation of these patients and the need of a prompt diagnosis. MATERIALS AND METHODS: We examined 124 newborns (56 males and 68 females) submitted to chest radiography at least once. Two radiologists analyzed the images investigating: a) chest expansion, b) reticulonodular opacities, c) aerial bronchograms, d) parenchymal consolidation, e) atelectasis, f) pneumothorax. The clinical diagnosis was made by neonatologists on the basis of clinical and instrumental data and of the clinical evolution of the patients. RESULTS: Twenty of 23 clinically negative patients had negative chest radiographs and 3 were false positives. Transient tachypnea was diagnosed in 14 newborns where chest radiography showed reticulonodular opacities. Ten of 62 patients with hyaline membrane disease had a negative chest radiograph, 26 had fine granular opacities, 16 marked hypoexpansion with reticulonodular opacities and aerial bronchogram, and 10 diffuse parenchymal consolidations with aerial bronchogram also in the peripheral lung. Eleven patients had a clinical and radiological diagnosis of segmental or lobar atelectasis; 5 newborns had an aspiration syndrome and radiography showed parenchymal consolidations in 4 and was negative in one. Five patients had pneumothorax, 3 diaphragmatic hernia and 1 infective pneumonia; radiology confirmed the diagnosis in all cases. We had three false positives and 11 false negatives at conventional radiography--i.e., 10 had MMI and one the meconium aspiration syndrome. Diagnostic sensitivity was 89.1%, specificity 86.9% and diagnostic accuracy 88.7%. CONCLUSIONS: Our results are partially limited by the lack of a reference gold standard. Nevertheless, if we consider the clinical condition and the respiratory distress syndrome evolution as a valid reference, the diagnostic accuracy of radiography is good.

Sonographic prediction of chronic lung disease in the premature undergoing mechanical ventilation.

The aims of the study are to investigate the possible role of ultrasound (US) of the chest in predicting the development of chronic lung disease (CLD) in patients with hyaline membrane disease (HMD) and to determine the optimal age for the sonographic examination. One hundred and five consecutive prematures undergoing mechanical ventilation were prospectively studied by US of the chest. The US examinations were performed at birth and at least once a week until discharge from the neonatal unit. The sonographic patterns observed behind the diaphragm and their evolutions were recorded and correlated with the clinical and radiological data at day 28, which corresponds to the currently accepted limit for determining the presence of CLD. CLD is currently defined as oxygen dependency on day 28 with radiographic abnormalities. A diffuse retrodiaphragmatic hyperechogenicity was observed in all the patients with HMD. The hyperechogenicity resolved completely in patients with an uncomplicated clinical evolution. In contrast, in patients with CLD the hyperechogenicity resolved only partially, resulting in less diffuse and less extensive hyperechogenicity. Day 18 was the earliest day where the persistence of the abnormal retrodiaphragmatic hyperechogenicity was observed in 100% of the patients presenting CLD at day 28. At that time, 95.2% of the patients without abnormal hyperechogenicity showed uncomplicated evolution and no CLD. US can be a useful diagnostic tool to determine the occurrence of CLD and to predict as early as day 18 the prematures at risk for the disease.

The lungs in immature infants: how important is surfactant therapy in preventing chronic lung problems?

Seventy-five premature infants weighing between 600 and 3200 g were studied over a period of 1 year. All of the infants received surfactant therapy for hyaline membrane disease immediately after birth and, thereafter, up to four doses every 6 h. The roentgenographic findings in all patients were documented at birth and at 2 days, 7-10 days, and 21-28 days of life. Larger babies responded to surfactant therapy better than did smaller infants. The smaller infants, even after initial clearing, were prone to develop pulmonary edema and the bubbly lungs of bronchopulmonary dysplasia. These data suggest that small infants, while initially responding to surfactant therapy with clearing of their lungs, are still at considerable risk of developing chronic lung disease in the form of pulmonary edema and bronchopulmonary dysplasia. An explanation is offered for why this occurs; at the same time it is suggested that, in view of our findings and those in the literature, the problems of pulmonary edema and bubbly lungs be more clearly separated.

Radiological incidence and course of bronchopulmonary dysplasia in 100 consecutive low birth weight neonates.

Bronchopulmonary dysplasia (BPD) is an important cause of chronic respiratory distress in low birth infants. The radiological incidence and course of BPD were assessed in 100 consecutive low birth weight infants. Chest radiographs were examined on admission, at the ages of 3 days, 7 days, 2 weeks and 4 weeks and at later follow-up until the examinations were normal. Twelve of the children died. The severity and typical radiological abnormalities of BPD were assessed. Among the children alive, there were 26 with BPD (29.5%). The BPD incidence was highest between the age of 2 weeks and 3 months (18-21%) declining to 3.4% at the age of 12 months. Radiological evidence of BPD was already seen at the age of 2 weeks in 16 of the children. Most cases (73%) had their maximum BPD score at the age of 1 to 3 months. The normalisation of the chest radiography occurred predominantly in the age between 3 and 6 months. The most frequent underlying condition in BPD was hyaline membrane disease in 81%.

Chest radiographs of neonates with respiratory failure caused by congenital syphilis.

Congenital syphilis still occurs in newborn babies and the prevalence has increased in recent years, especially in developing countries. This has led to an increase in the number of babies with congenital syphilis requiring intensive care for respiratory failure. The early recognition of this disease could lead to the institution of timely and appropriate treatment. In this study the radiological picture of syphilitic pneumonitis is described in 20 neonates admitted to our neonatal intensive care unit requiring ventilation for respiratory failure. The radiological picture of the babies with syphilis was compared to 20 babies with other causes of respiratory distress. The radiological picture in 17 babies demonstrated a coarse nodular pattern in addition to band-like opacities radiating from the hilar regions. The nodular opacities became confluent on follow-up radiographs. In 13 cases, the proximal humeri showed changes typical of congenital syphilis. Two of the three babies with syphilis who did not have the typical chest radiological picture had bony involvement visible on the chest radiograph. Both the sensitivity and specificity of radiographic diagnosis were 75% with a positive and negative predictive value of 75%. The diagnosis of congenital syphilitic pneumonitis can therefore be suspected on chest radiographs and should be included in the differential diagnosis of any baby who presents with an interstitial pattern on chest radiography.