Congenital Chylothorax - Successful management with chemical pleurodesis

Congenital Chylothorax is a rare entity which is characterized by abnormal accumulation of chyle in pleural cavity. Chylothorax presenting as non-immune hydrops is even rarer. We report a case of congenital bilateral chylothorax presenting as non immune hydrops and managed successfully with chemical pleurodesis. A term male baby presented at birth with bilateral pleural effusions and subcutaneous edema. It was initially managed with ventilation and intercostals drainage (ICD). After the initiation of feeds, re-accumulation of pleural fluid led to the diagnosis of congenital chylothorax. Management with ICD and octreotide was unsuccessful but responded to chemical pleurodesis with 4% povidine iodine done on 3 separate occasions.     

Recurrent pleural effusions in a neonate

Difficulty breathing is a common presenting complaint for infants in the emergency department (ED). Periodic breathing, respiratory or systemic infection, and congenital heart disease are common diagnoses in this age group. We report the case of a male neonate presenting to the ED on multiple occasions with respiratory distress and recurrent pleural effusions of unique origin. Unlike adult pleural effusions, pediatric effusions are most commonly exudative and of infectious origin. In neonates, acquired chylothorax secondary to surgery or trauma is the most common cause of symptomatic pleural effusions. Congenital chylothorax is a rare entity that has not been presented in the emergency medicine literature. This case illustrates the extremely rare ED presentation of congenital chylothorax and outlines an approach to pediatric pleural effusions.     

Octreotide in the treatment of congenital chylothorax

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 paediatric 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. Treatment was associated with prompt respiratory improvement prior to cessation of pleural drainage. Further studies are required to ascertain its true value in congenital chylothorax.     

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 μg/kg per hour and increased daily by 1 ug/kg per hour to 10 μg/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.     

A genetic reason for male excess in infant respiratory mortality?

Male infants have a 50% higher risk of death from respiratory diseases and a number of congenital heart diseases that can lead to cerebral hypoxia. The most important of these diseases are infant respiratory distress syndrome and sudden infant death syndrome.

Conclusion: The mechanism behind the excess peri-mortality rate in male infants is not known. A genetic factor leading to reduced tolerance to hypoxia is possible.     

Rapid control of norovirus gastroenteritis outbreak in an acute paediatric ward

Aim: To provide a practical action plan for effective infection control of norovirus outbreak in acute paediatric wards.

Methods: We report the infection control measures that were implemented to terminate and to prevent nosocomial spread of norovirus gastroenteritis in an open-designed paediatric ward.

Results: Nine children, one visitor, and one medical student were affected in a norovirus gastroenteritis outbreak in an acute paediatric ward. Vomiting was the main presenting symptom. The outbreak was rapidly terminated three days after implementation of stringent infection control measures and there was no second wave of attack. These measures included strict contact precautions, prompt isolation and cohorting of symptomatic patients, vigorous environmental cleansing with concentrated disinfectant (hypochlorite solution 1000 ppm), meticulous handling of waste products, and efficient contact tracing of exposed patients, family members, and medical students.

Conclusion: Prompt implementation of stringent infection control measures and contact tracing can rapidly terminate the norovirus outbreak and prevent a second wave of infection. Children with unexplained vomiting and those with contact history of gastroenteritis should be properly triaged, isolated, and investigated for possible infective causes, including norovirus-induced gastroenteritis.     

Familial non-immune hydrops fetalis and congenital pulmonary lymphangiectasia

We report on three siblings with non-immune hydrops fetalis. Congenital pulmonary lymphangiectasia was diagnosed in two of them. One of these, a girl still alive and suffering from frequent airway infections, has bilateral pleural effusions and distal congenital lymphoedema. Conclusion To our knowledge, this is the first report of non-immune hydrops fetalis and congenital pulmonary lymphangiectasia occurring in siblings. Received: 4 February 1997 and in revised form: 23 September 1997 / Accepted: 23 September 1997     

pH and nitric oxide synthase activity and expression in bovine aortic endothelial cells

Aim: Nitric oxide (NO) plays an important role in the transition from intrauterine to extrauterine life. If this transition fails, a condition called persistent pulmonary hypertension of the neonate (PPHN) may develop. The current treatment modalities for this disease include induction of alkalosis by hyperventilation or alkali infusion, inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation. There is evidence from animal studies that the elevated pH, not the low pCO₂ is responsible for the resultant pulmonary vasodilatation. In this study, we examined the effect of pH on the activity and expression of endothelial nitric oxide synthase (eNOS) in cultured bovine aortic endothelial cells (BAEC) as a possible explanation for the pH dependent drop in pulmonary vascular resistance.

Methods: BAEC were exposed to a pH gradient of 7.1-7.6 for 4 h (short-term) and 16 h (long-term). Standard Western blotting technique was used to detect expression of eNOS. Activity was measured by an indirect assay using bovine aortic smooth muscle cells (BASM) as reporter cells and measuring cGMP levels as a marker of NO production. The cells were exposed to the pH gradient for a total of 4 h and measurement were made at 30, 60 and 90 min, and 2, 3 and 4 hours.

Results: eNOS activity and expression remained unchanged during the four and sixteen hours of exposure.

Conclusion: In this in vitro experiment, we could not demonstrate an alkalosis-induced increase in eNOS activity and expression. The clinically observed pH dependent vasodilatation does not appear to be directly mediated through the induction of eNOS.     

Mid-term follow-up of neonatal pleural effusion

Objectives

To investigate the clinical course and mid-term prognosis of neonates admitted with pleural effusion.

Methods

Case records of 38 neonates admitted with pleural effusion were retrieved and analyzed.

Results

16 (42%) patients had congenital and 22 (58%) patients had acquired causes of pleural effusion. The most common causes of congenital pleural effusion and acquired pleural effusion were chylothorax (18%) and congestive heart failure (13%), respectively. Poorer outcome was observed with fetal hydrops, preterm birth (<34 weeks) and associated defects.

Conclusions

Most of the neonates with pleural effusion have good outcome in the mid-term follow-up.     

Randomized controlled trial of intrapleural streptokinase in empyema thoracis in children

Aim: To compare intrapleural streptokinase and placebo in paediatric empyema. Methods: Children with empyema greater than stage 5 received intrapleural streptokinase (n = 19) or normal saline (n = 21) along with intercostal drainage. Clinical and serial sonographic outcomes were compared. Results: Although there was no difference in clinical and sonographic outcome, none of the children with stage 7 empyema (multi-loculated empyema) who received streptokinase developed pleural thickening 30 d later.

Conclusion: There is no short-term clinical benefit of intrapleural streptokinase in paediatric empyema; this therapy may be reserved for those with stage 7 empyema to prevent pleural thickening in the long term.     

Congenital chylothorax: lymphopenia and high risk of neonatal infections

AIM: To describe the clinical course of patients with congenital chylothorax focusing on infectious complications. Congenital chylothorax is a common manifestation of non-immune hydrops fetalis (NIHF). The drainage of chyle leads to loss of cellular and plasmatic factors that influence the patient's immune response and increase the risk of infections. METHODS: In a retrospective analysis of 24 preterm infants with NIHF treated between 1998 and 2002, congenital chylothorax was diagnosed in 7 patients. RESULTS: All 7 patients were treated conservatively with pleural drainage over a median period of 22 d (range 10-36 d). Lymphopenia was found in all patients (median of minimal lymphocyte counts 285/microl, range 80-770). The nadir was on day 5 (2-6 d). Lymphopenia lasted for 12 d median (range 4-39 d) and was significantly correlated with the duration of lymph drainage (p = 0.001). Cell-surface analysis of peripheral blood lymphocytes was performed in two patients. Both patients had a decreased number of total T cells. Four out of seven (57%) patients developed nosocomial infections. This incidence of nosocomial infections in patients with congenital chylothorax is about three times higher than that in other neonatal patients. None of the children suffered from fungal or viral infection. Although there was a very high incidence of infections, no correlation between lymphopenia and the occurrence of infections could be shown. CONCLUSION: Drainage of congenital chylothorax results in the loss of lymphocytes and bears a high risk of infections.     

Positive pressure ventilation at neonatal resuscitation: review of equipment and international survey of practice

Background: The equipment used to provide positive pressure ventilation to newborns needing resuscitation at delivery varies between institutions. Devices were reviewed and their use surveyed in a sample of neonatal centres worldwide. Aim: To determine which equipment is used to resuscitate newborns at delivery in a sample of teaching hospitals around the world. Methods: A questionnaire was sent via e-mail to a neonatologist at each of 46 NICUs in 23 countries on five continents, asking which resuscitation equipment they used. If it was not returned, follow-up was by e-mail. Results: Data were obtained from 40 (87%) centres representing 19 countries. Round face masks are used at 34 (85%) centres, anatomically shaped masks are used exclusively at six (15%) and a mixture of types are used at 11 (28%). Straight endotracheal tubes are used exclusively at 36 (90%) centres; shouldered tubes are used infrequently at three of the four centres that have them. The self-inflating bag is the most commonly used manual ventilation device (used at 33 (83%) centres), the Laerdal Infant Resuscitator™ the most popular model. Flow-inflating bags are used at 10 (25%) centres. The Neopuff Infant Resuscitator™ is used at 12 (30%) centres. Varying oxygen concentrations are provided during neonatal resuscitation at half of the centres, while 100% oxygen is routinely used at the other half.

Conclusions: This survey shows considerable variation in practice, reflecting this lack of evidence and consequent uncertainty among clinicians. Comparison of the two most popular manual ventilation devices, the Laerdal Infant Resuscitator and the Neopuff Infant Resuscitator, is urgently required.     

Respiratory syncytial virus infections in congenital heart defects--hospitalizations and costs

Aims: To register hospitalizations for respiratory syncytial virus (RSV) infections and estimate costs of prophylaxis with humanized monoclonal antibodies (palivizumab) against RSV, compared to hospital care, in cases with congenital heart defects (CHDs). Methods: Population based study with prospective registration of CHDs. Costs for hospital treatment of RSV-infections in CHD-patients calculated by means of the Norwegian Diagnosis Related Groups system. Results: In 43 470 infants live born in the population through the 18-year period 1987-2004 a structural CHD was diagnosed in 527 (1.2%). A total of 898 (2.1%) hospitalizations for RSV-infections occurred in the study population 1987-2005. The hospital admittance rate was significantly higher for CHD-cases (4.8%) than for children without CHD (2%) (P=0.002). Severe CHDs (need for surgery or catheter intervention) had a higher admittance rate (9.2%) compared to the group of remaining CHDs (3.3%) (P=0.01). Number needed to treat with palivizumab to avoid one hospitalization for RSV-infection in cases of severe CHDs was calculated to 24, at costs of US$ 195 000. The expenses for palivizuamab prophylaxis in severe CHDs were 31 times that of hospital treatment.

Conclusion: Prophylaxis with palivizumab in severe CHDs is not cost-effective.     

Does Helicobacter pylori infection relate to iron deficiency anaemia in prepubescent children under 12 years of age?

Aim: To investigate the association between Helicobacter pylori (H. pylori) infection and iron deficiency anaemia (IDA). Methods: Haemoglobin levels, iron parameters and serum IgG antibodies to H. pylori were measured in 693 children aged 9 to 12 y. Results: No significant differences in the seroprevalence of H. pylori infection and antibody titres to H. pylori were found between the IDA group and the non-anaemic controls.

Conclusion: H. pylori infection does not seem to contribute to iron deficiency in prepubescent children.     

Use of supplementary equipment for resuscitation of newborn infants at tertiary perinatal centres in Australia and New Zealand

Aim: Neonatal resuscitation is a common and important intervention. International consensus statements advise how newborns should be resuscitated and suggest equipment to be used. Use of equipment not specifically recommended in these guidelines has been advocated. We wished to determine how widely this supplementary equipment is used in a geographically defined region. Methods: Each of the 25 tertiary perinatal centres with on-site deliveries in Australia and New Zealand was surveyed. The questionnaire asked about the use of the following items during delivery room resuscitation: pulse oximetry, exhaled carbon dioxide detection, polyethylene wrapping, oxygen blenders, laryngeal mask and oropharyngeal airways. Results: Data were obtained from all centres. Pulse oximetry is used at 12 (48%) centres. Exhaled CO ₂ detection is used to confirm endotracheal tube placement at three (12%) of the centres. Polyethylene wrapping is used to prevent heat loss in very-low-birthweight infants at delivery at 11 (44%) centres. Oxygen blenders are used to modify the amount of oxygen delivered at nine (36%) centres. Laryngeal mask airways are infrequently used at two (8%) centres. Oropharyngeal airways are infrequently used at five (20%) centres.

Conclusion: There is considerable variation in the equipment and techniques used to resuscitate newly born infants. Use of equipment not specifically recommended in international consensus statements is widespread. These are potentially effective tools to improve resuscitation. The evidence supporting their use is, however, limited. Urgent evaluation of their efficacy and safety is required before even more widespread use occurs.     

Wholehearted life support of babies born at 23 weeks of gestation

Justification of life support by all means to babies born at 23 wk of gestation.

Conclusion: Depends upon the needs and wants of the family.     

Health before and after adoption from Eastern Europe

Design: A population-based study of pre-adoption, arrival and post-adoption health. Aim: To report prenatal and postnatal background factors, morbidity, growth and development in adoptees from Eastern Europe. Subjects and Methods: All 99 children born in Eastern Europe between 1990 and 1995 and adopted to western Sweden during 1993-1997 were invited to participate in the study. Altogether, 76 (77%) participated. Medical records from the birth countries, from the examination at arrival and from medical reports made during a mean post-adoption period of 5 years were evaluated. Results: Low birth weight (≤2500 g) occurred in 48%. Congenital malformations were found in 22%. The biological mothers of 33% of the children had been considered alcoholics, and 16% of the children's mothers had been diagnosed with a psychiatric disability. A high incidence of infectious diseases, neurodevelopment disorders and growth retardation had been noted during the pre-adoption period. Upon arrival in Sweden 75% were diagnosed with a medical condition, most often an infection. After a 5-year post-adoption period, small head circumference was associated with alcohol exposure during pregnancy and 46% had at least one neurodevelopment or behavioural disorder.

Conclusion: Adverse prenatal and perinatal factors, congenital malformations and post-adoption neurodevelopment disorders were common. Adoptees and adopters have complex needs for health support and information.     

Book Reviews

Volume 11: Respiratory and Alimentary Tract Diseases Edited by H. S. Rosenberg and J. Bernstein Perspectives in Pediatric Pathology Basel: Karger, 1987, 218 pages, $128.00

Sickle-Cell Anemia and Thalassemia. A Primer for Health Care Professionals. R. G. Huntsman St. John's, Newfoundland, Canada: Canadian Sickle Cell Society, 1987, 223 pages, $10.00

Kawasaki Disease Edited by Stanford T. Shulman New York: Alan R. Liss, 1987, 591 pages, $98.00

Genetic Counseling for Clinicians Edited by Kenneth L. Garver and Sandra G. Marchese Chicago: Year Book Publishers, 1986, 356 pages, $46.50

The Battered Child Edited by Ray E. Heifer and Ruth S. Kempe Chicago: The University of Chicago Press, 1987, 470 pages, $35 (paperback, $16)     

Is screening ultrasound of the urinary tract indicated in infants with hypertrophic pyloric stenosis?

Aim: Few retrospective studies have evaluated infants with hypertrophic pyloric stenosis (HPS) for associated urological anomalies. They have led to contradictory conclusions. The aim of this study was to evaluate the incidence of urinary tract anomalies in infants with HPS and to establish the clinical significance of this association. Methods: One hundred and twenty-two infants (100 boys) who underwent pyloromyotomy between 1992 and 2002 were prospectively evaluated. Screening ultrasound (Us) of the urinary tract was performed in 107 infants, while 15 did not attend their ultrasound appointment. Results: Renal ultrasound was abnormal in 4 (4%) of 107 screened patients with HPS. Three patients were found to have mild hydronephrosis and, in one patient, a small, normal kidney was detected. Two patients with hydronephrosis had Us follow-up and the third patient underwent Tc-99 mercaptoacetyl triglycine (MAG 3) scan. In all three patients, the hydronephrosis resolved completely on follow-up scan.

Conclusion: The incidence of abnormal renal ultrasound in children with HPS is similar to the reported incidence of 3-6% determined with routine ultrasound screening of healthy newborns. The abnormalities detected were not clinically relevant and did not require surgical intervention. We do not recommend screening of the urinary tract in infants with HPS.