POC-LUS guiding pleural puncture drainage to treat neonatal pulmonary atelectasis caused by congenital massive effusion.

Point of care lung ultrasound (POC-LUS) has played important roles in diagnosing neonatal lung diseases and assisting in their treatment. A newborn infant with severe respiratory distress diagnosed as pulmonary atelectasis caused by congenital massive pleural effusion, whose consolidated lung recruitment after pleural puncture drainage under POC-LUS guidance. Lung ultrasound can be performed easily and timely at bed-side with free of radiation exposure, thus it should be used extensively in the neonatal department.

Early prenatal ultrasound diagnosis of congenital thoracic malformations.

Objectives: To evaluate and describe the spectrum and rate of congenital thoracic malformations (CTMs) diagnosed by early prenatal sonography (gestational age (GA) less than 16 weeks). Methods: A retrospective, cross-sectional analysis of prenatal ultrasound screening tests in a community-based clinic. Results: In 2001-2017, 31 261 prenatal ultrasound tests detected 31 CTMs at a gestational age of 15.2 (range, 11.6-16.0) weeks. The most common malformation was congenital pleural effusion (CPE) (15 fetuses, 0.48/1000), followed by congenital diaphragmatic hernia (CDH) (10 fetuses, 0.32/1000). Pulmonary hypoplasia (PH), congenital pulmonary airway malformation and broncho-pulmonary sequestration appeared in much smaller proportions (three, two and one fetuses, respectively). Most CTMs were associated with additional fetal lesions (15 fetuses, 48%). All early CDH (10 fetuses) and PH (three fetuses) and 6/15 with CPE had termination of pregnancy or missed abortions. Conclusions: Prenatal ultrasound before 16 GA was able to detect CTMs in 0.99/1000 of screening ultrasound (US) performed. Most CTMs tended to appear with multiple lesions and were associated with unfavorable outcomes. Earlier prenatal diagnosis may enable early termination of pregnancy in fetuses with lethal malformations.

Congenital pleural effusions: 15 years single-centre experience from North-East England.

AIM: The aim of this study is to add to the scant literature on congenital pleural effusions to aid counselling and clinical management decisions. METHODS: Retrospective case series of 15 years of congenital pleural effusions resulting in live birth in a single tertiary foetal medicine/neonatal centre in North East England. RESULTS: Data were available for 21 infants. Mortality rates were 43% overall. All spontaneous resolution occurred within 9 d, and active management was used where effusions persisted beyond this. CONCLUSION: Prematurity was associated with a poor prognosis. Resolution without active management occurred before day 10 and active strategies should be considered by this time.

Complete resolution of hydrops by placement of double basket catheter in a case of macrocystic type multilocular pulmonary sequestration.

OBJECTIVE: We presented a fetus affected by macrocystic lung lesions with progressive hydropic changes during the second trimester, but experienced remarkable resolution of hydrops in the third trimester after a series of in utero interventions. CASE REPORT: A 19-year-old women, G1P0, presented with fetal multilocular thoracic mass and hydropic change at 23+4 weeks of gestation. After non-directive genetic counseling, she opted for intrauterine cyst aspiration followed by intra-cystic OK-432 injection at 24 weeks of pregnancy, as well as sequential thoracoamniotic shunts at 26 weeks and 27+3 weeks of pregnancy when we observed hydrops developed progressively. Finally, the hydrops resolved in the third trimester and a healthy baby was born at 33+3 weeks of pregnancy, in which further surgical intervention was performed at five-month old. CONCLUSION: Thoracoamniotic shunting is a preferred option for all hydropic fetuses resulted from large macrocystic lung lesions to enhance perinatal survival rate.

Use of lung ultrasound in detection of complications of respiratory distress syndrome.

Repeated chest radiography is required for the diagnosis and follow-up of neonates with respiratory distress syndrome (RDS) and carries the risk of radiation hazards. Lung ultrasound (LUS) is a non-invasive bedside diagnostic tool that has proven to be effective in the diagnosis of RDS. Our aim was to assess the role of LUS with respect to the standard chest X-ray (CXR) in the detection of complications of RDS in neonates. Ninety premature newborns of both genders with RDS (mean gestational age = 29.91 ± 1.33 wk) and 40 premature babies as a control group were involved in this study. All patients underwent initial clinical assessment as well as CXR and LUS. Those who presented with respiratory distress and/or exhibited deterioration of oxygenation parameters were followed by CXR and, within 4 h, by LUS. Alveolo-interstitial syndrome and pleural line abnormalities were detected in all cases (100%) in the initial assessment, patchy consolidation was detected in 34 cases and white lung was detected in 80 cases. Alveolo-interstitial syndrome was detected in 19 controls. In follow-up of the patients, LUS was superior to CXR in detection of consolidation and sub-pleural atelectasis, but not in detection of pneumothorax. We concluded that bedside LUS is a good non-hazardous alternative tool in the early detection and follow-up of RDS in the neonatal intensive care unit; it could be of value in reducing exposure to unnecessary radiation.

Congenital lung lesions: Prenatal diagnosis and intervention.

Congenital lung lesions are common sonographic findings in pregnancy, usually detected at the routine 20 weeks scan. The most common is cystic adenomatous malformation of the lung (CCAM). This usually causes few prenatal problems; however, fetal hydrops occurs in about 5%. Prenatal intervention for these is possible in many to allow survival to birth. Bronchoplumonary sequestration (BPS), with an aberrant "feeder" vessel arising from the aorta may co-exist but is detectable as a separate entity by visualization of this vessel. Symptomatic or curative prenatal intervention is again possible in the few severe cases where hydrops or pleural effusions develop. Pleural effusions may be due to a primary leak usually of chylous fluid: prenatal thoracoamniotic shunting may prevent pulmonary hyoplasia or cure the consequent fetal hydrops. More often, however, effusions are a consequence of an underlying abnormality, including many structural or chromosomal abnormalities that may also cause co-existing fetal hydrops. Congenital high airway obstruction (CHAOS) is commonly fatal but cases potentially amenable to prenatal intervention or to immediate perinatal management may be identified using ultrasound or MRI.

Evaluation of lymphatic dysplasia in patients with congenital pleural effusion and ascites using indocyanine green lymphography.

OBJECTIVES: To investigate the use of indocyanine green (ICG) lymphography in the diagnosis and assessment of the severity of lymphatic dysfunction in infants and neonates with congenital lymphatic pleural effusion and ascites. STUDY DESIGN: We performed ICG lymphography on 10 neonates and infants with congenital lymphatic pleural effusion and ascites. After the subcutaneous injection of ICG, circumferential fluorescent images of lymphatic drainage channels in the extremities and trunk were identified using an infrared camera system. The lymphographic findings were classifiable into 2 patterns-those showing a linear lymphatic pattern, suggesting normal lymphatic flow, and those showing lymphatic channels with retrograde lymphatic flow (dermal backflow pattern), suggesting an abnormal lymphatic flow. We analyzed the severity of the ICG lymphography findings and the clinical outcomes. RESULTS: Based on the ICG lymphography, the severity of lymphatic dysplasia were classified into 4 categories: mild dysplasia, moderate dysplasia, severe dysplasia, and lymphatic hypoplasia. All cases diagnosed with mild (n = 3) or moderate dysplasia (n = 2) survived, and 2 of the 4 cases diagnosed with severe dysplasia died. The duration of endotracheal intubation ranged from 1 to 17 days (median, 7) in the patients with mild or moderate dysplasia and from 25 to 110 days (median, 77) in those with severe dysplasia. CONCLUSIONS: The ICG lymphographic findings were consistent with the clinical conditions. This imaging technique may be important to the future clinical management of lymphatic dysplasia in neonates and infants.

Isolated congenital pleural effusion in two neonates.

Congenital isolated pleural effusion, a non-specific accumulation of fluid in the pleural space, is an uncommon anomaly which can be associated with structural malformations, inflammatory or iatrogenic problems, genetic syndromes or fetal hydrops. Here, we present two neonates with isolated congenital pleural effusion, one of which was associated with Down syndrome and the other with empyema and bloodstream infection caused by Burkholderia gladioli septicemia. We wanted to discuss the diagnosis and management of this rare clinical entity.

Fetal pleural effusions with spontaneous resolution: a case report.

A 42-year-old pregnant woman was referred with massive fetal bilateral pleural effusions. Observation with serial ultrasound was made. The documented spontaneous resolution of fetal pleural effusion was recorded. Neonatal examination revealed a completely healthy infant with normal respiration. Fetal pleural effusion can cause fetal lung compression, abnormal neonatal respiration and finally, neonatal mortality. Regular ultrasounds are one of the supportive options due to spontaneous resolution that can occur in 9 to 22% of the cases.

Partial trisomy 10p12.33 and partial monosomy 13q32.1: case report and a literature review.

We report on a preterm neonate with a deletion of the distal long arm of chromosome 13q32.1 and partial trisomy of the short arm of chromosome 10p12.33. The patient has intrauterine growth retardation, microphthalmia, macrocephaly, holoprosencephaly, patent ductus arteriosus, aortic isthmus hypoplasia, right renal agenesis, imperforate anus, ambiguous genitalia, pleural effusion and vertebral anomaly. Analysis using an oligonucleotide microarray (U-array Cyto6000 array platform (Human Genome build: hg 18) indicated that there was a partial trisomy of chromosome 10(19.5 Mb gain) involving 298 oligonucleotides from 10pter to 10p12.33, and a partial monosomy of chromosome 13(18.3 Mb deleted) involving 313 oligonucleotides from 13q32.1 to 13qter. This is the first report of a patient with partial trisomy 10p12.33 and partial monosomy 13q32.1.

[Are in-utero interventions justified? - perspective of neonatologists. - Part II. Spina bifida, obstructive uropathy, pleural effusion]. / Czy operacje wewnatrzmaciczne sa uzasadnione? - perspektywa neonatologa - czesc II. Przepuklina oponowo-rdzeniowa, uropatia zaporowa, wysiek oplucnowy.

INTRODUCTION: Spina bifida, obstructive uropathy and congenital pleural effusion (PE) belong to the group of congenital defects in which attempts of in-utero treatment were undertaken. OBJECTIVE: Main objective of our study was to search for scientific evidence that would justify offering, and performing, in utero interventions in fetuses with spina bifida, obstructive uropathy and PE. METHODS: Using Pubmed as the main source, all publications relevant to the subject of in-utero interventions in fetuses with spina bifida, obstructive uropathy and PE were sought and carefully reviewed. An extra effort was made to identify all randomized controlled trials and meta-analyses. RESULTS: Up to date, none of the aforementioned in-utero interventions was evaluated in the randomized controlled trial. Two ongoing studies, one for patients with spina bifida, and one for patients with obstructive uropathy are still actively recruiting the subjects. As suggested by the results of meta-analysis, vesico-amniotic shunt might be recommended for selected group of fetuses with obstructive uropathy. For fetuses with unilateral or bilateral PE, in-utero drainage seems to improve the outcome only in cases complicated by hydrops fetalis. However only case series are available. CONCLUSIONS: Because of insufficient scientific evidence, offering in utero intervention to women with pregnancy complicated by spina bifida, obstructive uropathy and fetal PE on the routine basis is not justified. Until more data, preferably from randomized controlled trials are available, these procedures should only be performed in specialized centers as a part of carefully designed clinical trial.

Congenital monoblastic leukemia presenting as jaundice, pleural effusion, and ascites: case report and literature review.

Congenital leukemias are a rare group of hematologic neoplasms with a wide range of clinical signs and symptoms. Here we reported a neonate presenting with jaundice, pleural effusion and ascites. The total protein and serum albumin were markedly low at 48 and 12 g/L. Computerized tomography showed the density of liver was asymmetry with several hypoechoic regions. Initial blood routine examination revealed only thrombocytopenia while blood white cells increased to 30.0×10(9)/L with 17% blast cells several days later. Bone marrow biopsy showed the proportion of blasts and promonocytes increased and she was diagnosed as acute monoblastic leukemia.

Fetal thoracic and bladder shunts.

Congenital obstructive lesions involving the bladder and the lung can lead to serious complications for the newborn. The in-utero placement of a diverting shunt in the fetal bladder or thoracic cavity can decrease the morbidity and mortality associated with these obstructive conditions. This review focuses on the indications for prenatal evaluation, technique, and outcomes for those fetuses with a lower urinary tract obstruction, congenital pleural effusion or macrocystic congenital cystic adenomatoid malformation after placement of a vesicoamniotic or thoracoamniotic shunt.

Aberrant venous flow measurement may predict the clinical behavior of a fetal extralobar pulmonary sequestration.

OBJECTIVE: Fetal extralobar pulmonary sequestration (EPS) is sometimes complicated by a massive pleural effusion, leading to tension hydrothorax and fetal hydrops. The goal of this study was to examine sonographic signs of venous obstruction in fetal EPS with or without pleural effusion. METHODS: Records of fetal ultrasound from 6 patients with EPS were reviewed with special attention to aberrant arterial and venous flow. The results were correlated with their clinical outcomes. RESULTS: Four of the 6 cases (cases 1-4) were complicated by massive pleural effusion and required fetal thoracentesis; thoracoamniotic shunt placement was required in 3 of these 4 patients (cases 1-3). The other 2 patients (cases 5 and 6) were not associated with pleural effusion despite the comparable size of the mass and did not require any treatment, either prenatally or postnatally. In cases 1-3, aberrant venous flow was difficult to detect and, even when detected, the arterial-to-venous flow velocity ratio was >6. This is in contrast to the uncomplicated cases 5 and 6 in whom aberrant venous flow was easily detected with an arterial-to-venous flow velocity ratio of 2-3. Arterial-to-venous flow velocity ratios of 3-6 were observed in case 4. This case was complicated by pleural effusion but not by fetal hydrops. CONCLUSIONS: These data support the hypothesis that venous obstruction is related to the production of pleural effusion in fetal EPS. Ample flow in the aberrant vein may indicate benign clinical behavior, while difficulty in detecting aberrant venous flow may be correlated with the development of massive pleural effusion.

Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.

OBJECTIVE: Case report and literature review for congenital pulmonary lymphangiectasis (CPL) CASE REPORT: Male fetus with bilateral pleural effusion, thoracoamniotic shunt, preterm delivery, and prolonged neonatal course with neonatal death at 3 months. Autopsy-identified CPL. DISCUSSION: Review of pathology, clinical course, and genetics of CPL. CONCLUSION: This postnatal diagnosis of CPL/Hennekam syndrome must be considered with prenatal counseling regarding a fetus with bilateral pleural effusions. This pathological entity is autosomal recessive and has a significant risk of lethality.

Hemodynamic evaluation of a prenatal thoracoamniotic shunt for fetal pleural effusion.

AIMS: To evaluate the efficacy of a thoracoamniotic shunt for the treatment of pleural effusion (PE) in the view of hemodynamics. METHODS: The preload index (PLI) in the inferior vena cava (IVC), the maximal flow velocity of the descending aorta (VAomax), skin edema on the thorax and the ratio of lung to the thorax transverse area (L/T) as measured by ultrasound were evaluated before and after thoracoamniotic shunt placement for 5 fetuses with PE. RESULTS: The PLI and skin edema on the thorax decreased significantly after shunt placement compared to before shunt placement (PLI before: 0.488 +/- 0.036, after: 0.348 +/- 0.043, P < 0.05; edema before: 15.3 +/- 2.06 mm, after: 9.00 +/- 0.63 mm, P < 0.05). Furthermore, the L/T increased significantly after shunt placement compared to before (before: 0.220 +/- 0.013, after: 0.260 +/- 0.011, P < 0.01). No significant difference in VAomax was seen between before and after shunt placement (before: 101.5 +/ -6.39 cm/s, after: 10.7.6 +/ -5.41 cm/s, P = 0.16). CONCLUSIONS: The shunt for PE improved PLI especially in the fetal hemodynamics significantly.

Congenital chylothorax treated with octreotide.

Congenital chylothorax is the accumulation of lymphatic fluid within the pleural space. Cases unresponsive to conservative management usually require surgery. Octreotide has been used successfully to treat post-traumatic chylothoraces in the pediatric and adult population. Its exact mode of action is uncertain but it is believed to reduce lymphatic drainage by a direct action on splanchnic lymph flow. We report a case of congenital chylothorax where surgery was avoided with the compassionate trial of the somatostatin analogue, octreotide. A 33 week gestation female infant, born with the presence of large bilateral pleural effusion, was unresponsive to conservative management. Octreotide was commenced on day 15, with 10 days of an octreotide infusion, initially 0.5 microg/kg per hour and increased daily by 1 microg/kg per hour to 10 microg/kg per hour. Treatment was associated with prompt respiratory improvement prior to cessation of pleural drainage over the 10 day. She remains well at 6 months of age. Further studies are required to ascertain its true value in congenital chylothorax.