Role of chest ultrasound in neonatal lung disease: a review of current evidences.

Among the common causes of neonatal admission to NICU, respiratory distress is one of the important causes. The neonatal respiratory distress is end result of various pulmonary and non-pulmonary causes. Differentiation of pulmonary causes of respiratory distress is important for the neonatologist as treatment differs with different etiologies. Conventionally, chest X-ray and sometimes CT scan have been used to identify the etiology of respiratory distress but these modalities have several limitations which make their use in NICU doubtful. In recent decades, there has been use of lung ultrasound (LUS) to identify and differentiate the etiologies of respiratory distress. The current available evidence show that LUS has good sensitivity and specificity to identify all the common causes of neonatal distress like respiratory distress syndrome, transient tachypnea of newborn, pneumothorax, and pneumonia. This review will cover the various uses of LUS in neonatal care with current available evidence.

Gorlin-Goltz syndrome: first reported case of bullae in the lungs complicated with tension pneumothorax.

A 13-year-old girl was referred by her general practitioner with acute worsening exertional dyspnoea and sudden onset of left-sided chest pain. There was no associated trauma, palpitations or syncope. Clinical examination revealed that the left lung was hyper-resonant on percussion with reduced air entry on auscultation. Chest X-ray showed a left tension pneumothorax. She was treated conservatively with chest drain. Follow-up X-ray revealed multiple bullae within her left lung. Unfortunately, she redeveloped a pneumothorax and was sent to a tertiary centre. She was under the care of the paediatric cardiothoracic surgeons who organised a CT thorax and performed a lobectomy to remove the bullae. She was discharged from the tertiary centre and currently being followed up under the care of the paediatrician in the district general hospital. She have not developed any further pneumothoraxes.

An abrupt increment in the respiratory rate is a sign of neonatal pneumothorax.

OBJECTIVE: The objective of the present study was to investigate the early clinical signs of neonatal pneumothorax. METHODS: A case-control study at a level III referral center from 2006 to 2012 was conducted. For each neonate with pneumothorax (Group I), one control subject (Group II) was identified by selecting an infant born within the same week with a gestational age of ± 7 days and with a birth weight within 10% of the index case. The primary outcome was to investigate the clinical findings preceding the onset of pneumothorax. Secondary outcome was to assess the risk factors. RESULTS: Of the 1375 infants admitted, 39 had a pneumothorax. Patients with pneumothorax showed significantly increased respiratory rates preceding the definitive diagnosis when compared with control patients (median: 16 (0-58) versus 4 (0-10); p < 0.001). An increase in the respiratory rate was found to be a significant clinical parameter preceding the diagnosis of pneumothorax (odds ratio (OR): 1.47, 95% confidence interval (CI): 1.12-2.14, p < 0.001; R(2) = 0.80). Sensitivity and specificity of this sign were found to be 77 and 90%, respectively. CONCLUSION: An abrupt increase in the respiratory rate would be used as a reliable parameter to help determine an impending pneumothorax in neonates.

Birt-Hogg-Dubé syndrome in a patient presenting with familial spontaneous pneumothorax.

Birt-Hogg-Dubé (BHD) syndrome is a recently discovered autosomal-dominant disease caused by a mutation in the folliculin gene. We report a patient with familial spontaneous pneumothorax who was found to have BHD syndrome. Patients with a personal and family history of pneumothoraces and computed tomographic (CT) findings of multiple pulmonary cysts should alert the thoracic surgeon to this syndrome; additional evaluation and testing may be warranted.

Lung ultrasonography for the diagnosis of neonatal lung disease.

Ultrasound has recently become an important method for diagnostic examination and monitoring of lung disease. Many lung diseases, such as respiratory distress syndrome, transient tachypnea of the newborn, pneumonia, atelectasis and pneumothorax were diagnosed by chest X-ray or CT scan in the past, but can now easily be diagnosed with lung ultrasound. Lung ultrasound has many advantages over X-ray and CT scan including accuracy, reliability, low-cost and simplicity, as well as the fact that ultrasound incurs no risk of radiation damage. It is therefore feasible and convenient to perform at the bedside in a neonatal ward. This review focuses on features of bedside lung ultrasound and diagnosis features of common lung diseases in newborn infants, culminating in suggestions for improving the application of ultrasound in the neonatal field.

Recurrent severe oligohydramnios and fetal pulmonary hypoplasia associated with ErbB4 mutation.

BACKGROUND: Pulmonary hypoplasia resulting from oligohydramnios or anhydramnios can cause severe respiratory compromise in newborn patients. We report a case of recurrent oligohydramnios in a mother with an ErbB4 mutation and speculate that the effects on the placenta through decreased vascularization contributed to oligohdyramnios and subsequent pulmonary hypoplasia in the newborn. CASE: The pregnant mother in this case had two subsequent term pregnancies complicated by severe oligohydramnios. Both pregnancies resulted in live born female neonates with pulmonary hypoplasia, pneumothoraces, and pulmonary hypertension. The mother and second newborn, who died, were found to have the ErbB4 mutation. Examination of the placenta with that pregnancy showed decreased vascularity. CONCLUSION: ErbB4 may have important effects on placental development and hydramnios that also may affect neonatal pulmonary hypoplasia.

The use of recombinant tissue-type plasminogen activator in a newborn with an intracardiac thrombus developed during extracorporeal membrane oxygenation.

Extracorporeal membrane oxygenation (ECMO) support is often used to support infants and children with hemodynamic or respiratory failure. One of the major obstacles of safely treating a child with ECMO is balancing the risk of hemorrhage with the potential for thrombus development. Managing thrombosis in the setting of ECMO is challenging and has no defined algorithm. The use of recombinant tissue-type plasminogen activator (tPA) for thrombolysis has been previously described in cases where thrombi have developed despite adequate anticoagulation. In such situations, the risk of hemorrhage must be carefully balanced with the benefit of dissolving the clot and reestablishing flow. We present a case of an infant who required ECMO because of severe primary pulmonary hypertension and subsequently developed a right atrial thrombus adjacent to the ECMO cannula. The patient was treated with tPA with immediate improvement but had fatal intracranial hemorrhage almost 3 days after the tPA was administered. In this report, we review the current literature on tPA use during ECMO support and suggest a rational approach.

Congenital lobar emphysema: differential diagnosis and therapeutic approach.

BACKGROUND: Congenital lobar emphysema (CLE) is a rare anomaly of lung development that usually presents in the neonatal period with respirator distress and pulmonary lobar hyperinflation. It is commonly confused with pneumothorax. The aim of the present paper was to review the authors' experience in order to emphasize the importance of differential diagnosis with pneumothorax. METHODS: Children with CLE treatment at Department of Thoracic Surgery, Dicle University School of Medicine, Turkey, between January 1993 and June 2004, were reviewed. RESULTS: Ten children consisting of six boys and four girls (age range, 6 h-12 months) had CLE. Major presenting symptoms were tachypnea(n = 100%) and respiratory distress in (n = 80%). On chest radiograph, emphysema was seen in all patients, and shift-herniation to the opposite lung, atelectasis were observed. Computed tomography was performed in all patients, which indicated emphysema in the affected lobes in all cases. Pulmonary perfusion scan was performed in two patients, showing loss of perfusion in the affected lobe. The most common affected lobe was the left upper lobe (50%). In the present series, three patients were mistakenly diagnosed as pneumothorax and intercostal drains were inserted in the emergency department. Eight patients underwent lobectomy, and postoperative course was uneventful. Two patients were followed conservatively. Emphysema was detected in all pathological specimens. One patient was lost to follow up. Mean follow-up duration of all patients was 26.8 +/- 29.24 months (range, 1-89 months). CONCLUSIONS: CLE is established on combined clinical, radiological and scintigraphic imaging. Surgical excision of the affected lobe is the appropriate treatment. Particularly, differential diagnosis should be made between CLE and pneumothorax.

Risk factors of pneumothorax during the first 24 hours of life.

BACKGROUND: Pneumothorax is one of the air leak syndrome and is more common in the newborn period than in any other childhood periods. It can be divided into spontaneous pneumothorax and secondary pneumothorax from underlying lung pathology or assisted ventilation. Pneumothorax results in longer hospital stays and even deaths in some cases. To date, there are few studies that focus on identifying risk factors of pneumothorax. We conducted this study to ascertain risk factors for pneumothorax, in order to create a guideline to prevent this condition. MATERIAL AND METHOD: This is a retrospective case-control study. CASEs were infants with the diagnosis of pneumothorax (P25.1 Pneumothorax originating in the perinatal period) between January 2001 and December 2004. Controls were those whose birth times followed in the immediate chronology to the cases. CASE: control ratio was 1:2. Univariate analysis was used to compare the two groups. Odds ratio and 95% confidence interval were used to identify possible risk factors. Statistical significance was considered as p < 0.05. RESULTS: There are 44 cases and 88 controls. Risk factors are shown as Odds ratio and 95% confidence interval. Infant factors associated with higher risk of pneumothorax are male (2.6; 1.2, 5.6), low birth weight (19.3; 2.3, 160.2), vacuum extraction (20.9; 1.1, 403.4), meconium-stained amniotic fluid (4.5; 1.8, 11.0), low 1-minute Apgar score (78.3; 4.5, 1357.8), and the administration of bag and mask positive-pressure ventilation (29.0; 3.6, 233.5). Maternal factor associated with higher risk of pneumothorax is poor antenatal care (3.5; 1.04, 11.9). CONCLUSION: All pregnant women should be encouraged to have good antenatal care. Mother who has complication(s) during pregnancy and delivery should receive special care to prevent perinatal depression. For mothers with meconium-stained amniotic fluid, close fetal monitoring and tracheal suction for meconium after delivery should be appropriately considered to prevent meconium aspiration. Finally, neonatal resuscitation, when needed, should be done very carefully by following the American Heart Association and the American Academy of Pediatrics guidelines, especially for bag and mask positive-pressure ventilation.

Symptomatic spontaneous pneumothorax in term newborn infants.

The association between congenital major urinary tract anomalies (CMUTA) and spontaneous pneumothorax in term newborn infants (SPTNI) is controversial. We conducted a case-control study to test the hypothesis that SPTNI is associated with CMUTA. We compared 80 term infants with spontaneous pneumothorax to 80 healthy control infants. We recorded risk factors, clinical course, therapy, and outcome. Only 1 infant of 60 infants (1.7%) had CMUTA with SPTNI, as revealed by renal ultrasound studies. This is comparable to the 1.4% rate reported for CMUTA in healthy newborn infants by Steinhart et al. ([1988] Pediatrics 82:609-614). SPTNI were significantly more likely in males with higher birth weights and with vacuum delivery. Sixty-seven (84%) infants with SPTNI had follow-up for a mean and median of 46.4 and 39 months, respectively (range, 1-126 months), without manifesting any renal or pulmonary complications.

Clinics in diagnostic imaging (86). Ruptured bronchogenic cyst.

An eight-year-old boy presented with a sudden onset of chest pain. He had been diagnosed to have a left spontaneous pneumothorax. Chest radiographs and computed tomography of the chest showed a thin-walled cyst in the left lower lobe. Thoracotomy and a segmentectomy of the apical segment of the lower lobe was performed, confirming the diagnosis of a ruptured bronchogenic cyst. Imaging findings of various pulmonary cystic lesions in children are discussed.

Primary congenital pulmonary hypoplasia--genetic component to aetiology.

Primary congenital pulmonary hypoplasia, defined as congenital pulmonary hypoplasia occurring in the absence of other congenital anomalies, is an exceedingly rare condition of unknown aetiology. We report on two cases that presented as severe progressive respiratory failure immediately after birth in siblings of a consanguinous marriage, and we postulate that a genetic aetiology may be responsible for the arrest of lung maturation in utero. The possibility of a genetic component to the aetiology has not been previously documented in the literature.

[Pneumothorax in neonates and infants]. / Der Pneumothorax bei Neugeborenen und Säuglingen.

Pneumothorax in newborns and infants can have different etiologies: alveolar disruption following mechanic ventilation or reanimation, surgery for congenital diaphragmatic hernia or esophagus atresia, staphylococcal pneumonia, or thoracic traumas. We studied 105 cases of pneumothorax (96 newborns) treated in our hospital during the last 15 years. Pleural puncture with drainage and antimicrobial therapy were the treatments of choice. Due to early diagnosis and treatment of the pneumothorax and concomitant anomalies mortality was reduced to 17.4%.

[Pneumothorax and subvesical urinary tract obstruction in newborn infants--a pathogenically related symptom complex]. / Pneumothorax und subvesikale Harnwegsobstruktion beim Neugeborenen--ein ursächlich im Zusammenhang stehender Symptomenkomplex.

Within a period of 3 years 4 mature male neonates were observed with an unusual complex of symptoms: subvesical obstruction (valve) with spontaneous pneumothorax/pneumomediastinum. All infants showed immediately postnatal respiratory insufficiency. There are pathogenetical relations between renal and urinary tract malformations and pulmonary changes, which are recognized in Potter's sequence with pulmonary hypoplasia. The authors postulate a deranged dynamic of pulmonary fluid by oligohydramnios, causing increased vulnerable lungs also in such cases without typical x-ray signs of pulmonary hypoplasia. A spontaneous pneumothorax/pneumomediastinum in newborn infants should be the sign to perform nephro-urologic (sonographic) examination.

Congenital cystic adenomatoid malformation of the lung: an unusual presentation.

A case of congenital cystic adenomatoid malformation of the lung is described in a twelve-year-old boy presenting with signs and symptoms of a tension pneumothorax.

[Unusual cutaneous emphysema in a newborn infant following bilateral pneumothorax]. / Ungewöhnliches Hautemphysem bei einem Neugeborenen nach doppelseitigem Pneumothorax.

We report a patient with subcutaneous emphysema after a bilateral pneumothorax of gigantic extent: Subcutaneous emphysema extended over the chest, neck, axilla and both arms. The scalp was severed from the galea by an enormous air-cushion. Theories about the pathogenetic mechanisms are discussed. Therapy consisted of treatment of the underlying condition e. g. thoracocentesis with pleural drainage and artificial ventilation. The subcutaneous emphysema resolved without further therapeutic measures; only the subgaleal air was removed by aspiration.