Outcome of children with pulmonary lymphangiectasis

Pulmonary lymphangiectasis (PL) is a very rare developmental defect of the lungs, which has previously been reported to have a very poor prognosis. However, recent reports have suggested improved outcomes, possibly as a result of advances in neonatal and pediatric intensive care medicine. We performed a retrospective study on the outcome of children with PL between 1990 and 2008 referred to our tertiary center. Seven patients with histologically proven PL were identified over the 18‐year period. Six patients presented in the neonatal period and one patient at 7 months of age, all of them requiring intensive care treatment. Three neonatal patients required extracorporeal membrane oxygenation (ECMO). Six of the seven patients did not survive including all those who received ECMO. Two of the six non‐survivors died of other causes than their underlying disease. The only survivor had an antenatal diagnosis of hydrops and required in utero chest drain insertion. Postnatally he was managed with maximal medical treatment for bilateral pleural effusions and persistent pulmonary hypertension. A 7‐month follow‐up showed this infant to be doing well. In conclusion overall the prognosis of congenital PL remains poor. The one survivor demonstrates that this condition is survivable with aggressive intervention and as current evidence suggests gradual improvement of symptoms may occur over time, and that maximal medical treatment remains warranted. Pediatr Pulmonol. 2009; 44:351–357. © 2009 Wiley‐Liss, Inc.     

Congenital pulmonary lymphangiectasia: a case report of thoracic duct agenesis

We present a 17-year-old Caucasian male with congenital pulmonary lymphangiectasia and an absent thoracic duct. This patient is unique as he did not present with the disorder until age 9.5 years. Since his initial presentation he has had recurrent chylothoraces and has been treated symptomatically. We discuss the possible implications of his disorder as well as some of the limited treatment that is available.     

Pulmonary eosinophilic vasculitis in a neonate with congenital chylothorax

We report a case of congenital bilateral chylothorax, complicated by pulmonary eosinophilic vasculitis and transient eosinophilia. Excluding all known forms of eosinophilic pulmonary disease either histologically or clinically, possible mechanisms of its etiology are discussed. Treatment with corticosteroids was successful, and after a prolonged clinical course the patient recovered by age 2 years.     

Lung function in infancy and childhood following neonatal intensive care

Pulmonary function studies were performed in 11 neonatal intensive care survivors both during infancy and later in childhood. Lung function was compared with the respiratory support given in the neonatal period. The mean +/- SE thoracic gas volume was 96 +/- 4% predicted in infancy and rose to 122 +/- 8% predicted during childhood (P less than 0.005). The specific airway conductance (SGaw) in infancy was 57 +/- 7% predicted and rose to 90 +/- 8% predicted in childhood (P less than 0.0025). Abnormalities in SGaw were found only in ventilated infants, and there was a negative logarithmic correlation between the treatment score in the neonatal period and the SGaw in both infancy and childhood. The data indicate a long-term improvement in airway conductance of moderately affected infants with the development of mild hyperinflation in childhood possibly resulting from residual small airway abnormalities despite a symptomless clinical course. The residual abnormalities in prematurely born infants were in proportion to the intensity of treatment required in the neonatal period.     

Angiojet catheter-based thrombectomy in a neonate with postoperative pulmonary embolism.

A 2-day-old male with pulmonary atresia and an intact ventricular septum underwent central aortopulmonary shunt placement and right ventricular outflow tract reconstruction. Postoperatively, the patient had difficulty weaning from mechanical ventilation and was found to have a large thrombus occluding the entire right lower pulmonary artery. The patient underwent successful percutaneous catheter-based thrombectomy in the early postoperative period with the Possis AngioJet catheter. The patient had immediate improvement in gas exchange and was able to be weaned from the ventilator shortly thereafter. This represents the first published use of mechanical thrombectomy in a postoperative neonate.     

Novel surgical technique for insertion of pleuroperitoneal shunts for bilateral chylous effusions in Ex preterm infant at term corrected age

Neonatal chylothorax is a relatively uncommon condition. Here we describe the case of an extremely preterm infant with bilateral chylous effusions which were resistant to conventional medical management and were ultimately managed with the insertion of pleuroperitoneal shunts using a novel surgical technique. Subsequently the infant recovered and the shunts could be removed. We report this case to highlight that such shunts can be inserted even in tiny preterm infants with minimally invasive techniques, and be extremely successful in managing chylothorax, pending spontaneous recovery. Pediatr. Pulmonol. 2010; 45:840–843. © 2010 Wiley‐Liss, Inc.     

Primary pulmonary vein stenosis in a premature infant without bronchopulmonary dysplasia: A case report

Primary pulmonary vein stenosis (PVS) presenting in childhood is uncommon and is related to premature with bronchopulmonary dysplasia (BPD). Here we present a premature infant with primary PVS and without BPD. In our case, a 19-month-old girl was diagnosed with PVS, atrial septal defect and patent arterial duct by echocardiography and selective pulmonary artery angiography. Interestingly in the first 2 month after birth, there was no clue of PVS by bed echocardiography in the patient. It is important to focus on the pulmonary vein blood velocity in premature infants and very low birth weight infants even without BPD.     

Transcatheter treatment of muscular ventricular septal defect and pulmonary valvar stenosis in an infant.

An 11-month-old, 8 kg girl with muscular ventricular septal defect and pulmonary valvar stenosis had successful transcatheter treatment of both anomalies. The stenotic pulmonary valve was dilated first by balloon catheter, then the VSD was approached and closed using a 6 mm Amplatzer VSD occluder device. Due to technological improvements, transcatheter techniques are more and more the procedure of choice in a larger number of congenital heart diseases in younger and younger patients.     

Chest wall mesenchymal hamartoma in an infant: Evaluation with electrical impedance tomography

Mesenchymal hamartoma of the chest wall is a rare benign nonneoplastic lesion of infancy arising from chondro‐osseous tissue. Although its natural history suggests spontaneous regression, we describe a fatal case in a neonate with significant respiratory compromise. We explored the use of electrical impedance tomography to evaluate the dynamic impact of such space occupying lesions on a ventilated infant.     

Percutaneous selective pulmonary artery bands (Joeys) in a pulmonary overcirculation model.

BACKGROUND: Control of distal pulmonary artery (PA) pressure and flow is a critical step in palliating infants with complex congential heart disease. Surgical procedures to protect or isolate the pulmonary circulation carry significant risk and can be unpredictable. Interventional control of pulmonary pressure/flow with an intravascular device (band) could reduce risk and improve regulation of pulmonary flow. METHODS: Internal pulmonary bands were created from woven nitinol to create an internal orifice estimated to reduce distal pulmonary arterial pressure by 50%. Two designs were tested, a single eccentric lumen and two symmetrical lumens. The devices were approximately 7-mm long and the external diameter varied to suit the pulmonary arterial lumen size. A total of 15 devices were implanted in seven lambs with a stented ductus arteriosus to create high pulmonary pressure/flow. Four lambs were followed with devices and stent in place for a mean of 37 +/- 13 days. RESULTS: Fourteen of 15 devices were successfully implanted. One was acutely removed since the size was too large. Both device designs created greater than a 50% reduction in distal pulmonary pressure. Main PA pressure was 70/38, mean 50 +/- 13 mm Hg, and distal PA pressure 25/16, mean 20 +/- 7 mm Hg (P < 0.05). Angiography demonstrated protection of the internal banded pulmonary segments from overcirculation. Late follow-up showed that the device lumen(s) remained patent. Histology showed the vessel media to be intact but there was intimal proliferation where the device approximated the wall. There were no thrombotic emboli detected. CONCLUSION: Percutaneous internal banding of the pulmonary arteries was feasible and successful in lambs with high pulmonary flow/pressure analogous to infants with complex congenital heart disease.     

Primary pulmonary hypertension in adults

Primary pulmonary hypertension is an enigmatic disease found predominantly in young women, but it also affects a significant number of middle-aged and elderly males and females. Its onset, characterized by progressively worsening dyspnea, fatigue, and chest pain, is insidious. Three distinct histopathologic subtypes have been identified, and the natural history of the disease process has been well-defined. Pharmacologic treatment options have, in general, been disappointing, and it appears that heart-lung transplantation will be applied only to a small minority of young patients with primary pulmonary hypertension in the near future. We review the histopathology, evaluation, treatment, and prognosis of primary pulmonary hypertension.     

Congenital pulmonary varix: Two case reports

Rationale:Patients with congenital pulmonary varix are asymptomatic and require no treatment, but the radiological characteristics of a pulmonary varix are similar to those of a pulmonary arteriovenous malformation, which requires treatment. Pulmonary angiography is useful for obtaining information about the dynamics of pulmonary blood flow to differentiate a pulmonary varix from a pulmonary arteriovenous malformation for the purpose of treatment planning. Two cases of congenital pulmonary varices that were differentiated from pulmonary arteriovenous malformations based on pulmonary angiography findings are presented.Patient concerns and diagnosis:The first patient was an asymptomatic 39-year-old man. Non-contrast-enhanced computed tomography performed as part of the treatment course for pneumonia showed pulmonary arteriovenous malformation in the right lung. Pulmonary angiography was performed and showed that it was a pulmonary varix. The second patient was an asymptomatic 23-year-old woman. As part of her regular health check-up, she underwent plain chest X-ray examination, which showed an abnormal shadow. Non-contrast-enhanced computed tomography was performed, and pulmonary arteriovenous malformation was suspected. However, contrast-enhanced computed tomography findings suggested that the patient had a congenital pulmonary varix rather than a pulmonary arteriovenous malformation. Pulmonary angiography was subsequently performed for diagnosis, and a pulmonary varix was confirmed.Interventions and outcomes:No treatment was administered to either patient. The first patient was followed up for four years, and the second patient for two years. Both patients had no symptoms or complications during the follow-up period.Lessons:Two cases of congenital pulmonary varices were reported. Information about the dynamics of pulmonary blood flow obtained by performing pulmonary angiography was effective in distinguishing between pulmonary arteriovenous malformation and congenital pulmonary varix.     

Chronic pulmonary thromboembolism in a patient with a fontan circulation: percutaneous management.

Chronic pulmonary embolism is a common complication in patients with Fontan circulations. When anticoagulation is ineffective and surgery is contraindicated, percutaneous techniques may be considered. The authors report the first case of successful catheter intervention in a 30-year-old woman with a Fontan circulation who presented with NYHA class IV symptoms and chronic and complete obstruction of her left pulmonary artery.     

Congenital pulmonary capillary hemangiomatosis: Report of two cases and review of the literature

Pulmonary capillary hemangiomatosis (PCH) is a rare disease characterized by pulmonary hypertension and excessive neovascularization within the pulmonary interstitium, vasculature, and airways. We describe two unusual cases of congenital PCH. Both cases had concurrent anomalies, including renal and urinary bladder agenesis and hypertropic cardiomyopathy. In one case, capillary proliferation caused significant impingement of the proximal bronchial airways. A review of the current literature is described.     

Association of three different congenital malformations in a same pulmonary lobe in a 5‐year‐old girl

We report the case of a 5‐year‐old girl with persistent chest X‐ray abnormalities following an episode of pneumonia who has a complex congenital pulmonary malformation comprising of a congenital pulmonary airway malformation, an intralobar sequestration and two bronchogenic cysts, all present within the same lobe. The observation suggests a common embryological origin of these malformations. Pediatr. Pulmonol. 2010; 45:832–835. © 2010 Wiley‐Liss, Inc.     

The outcome of pulmonary artery stents following surgical manipulation

Background: Pulmonary artery (PA) stents are utilized to treat branch pulmonary stenosis (BPS). Often patients with PA stents undergo subsequent cardiac surgery for other indications, and the stents can be manipulated during the procedure. Objective: The purpose of this study was to evaluate the outcome of branch PA stenoses following surgical manipulation of previously implanted PA stents and to determine factors associated with future reintervention. Methods: Catheterization data, operative reports, and clinical summaries were reviewed on patients with PA stents placed between September 1989 and December 2006 undergoing subsequent cardiac surgery. Surgical manipulation was recorded as removed, trimmed, or longitudinally transected, and patched. Those that were not manipulated were defined as untouched. Results: 459 patients had branch PA stents placed. About 54 patients, with 70 stents in branch PA's. subsequently had further cardiac surgery. The median age of stent placement was 7.5 (0.5–32.4) years with a median age of surgery of 12.7 (5.1–39.6) years. Surgical manipulation was performed in 23 (33%) PA's and 47 (66%) stents were untouched. Stent removal occurred in 11 (16%), with transecting longitudinally and patching in 5 (7%), and trimming in 7 (10%). Comparing the surgical manipulation and the untouched groups, there was no difference in median age of stent placement [7.2 (0.5–30.2) versus 7.6 (1.8–32.4) years, p = 0.40], wt [21.0 (5.3–86.5) versus 24.7 (9.0–96.0) kg, p = 0.42], or residual catheterization gradient across the stent [3 (0–59) versus 4 (0–50) mmHg, p = 0.81]. Catheter reintervention (stent n = 6 or balloon dilation n = 14) on the previously stented PAs was similar between the surgically manipulated (median 7.5 years) and untouched groups (median 11.5 years) (p = 0.31). In multivariate analysis, the factors associated with future catheter reintervention were having the stent transected longitudinally and patched (p = 0.003) and a lower weight (p = 0.006) at the time of stent placement. Conclusions: Surgical stent manipulation is often performed in patients who have PA stents. Surgical manipulation does not alter the need for future reintervention and catheter re‐intervention may be more likely when the stents are transected longitudinally and patched. © 2010 Wiley‐Liss, Inc.     

Spontaneous pulmonary interstitial emphysema in a term unventilated infant

Pulmonary interstitial emphysema (PIE) is a form of air block most frequently seen in ventilated preterm infants with severe lung disease; it is rarely reported in spontaneously breathing term infants. We report on an infant previously diagnosed with laryngomalacia and congestive heart failure and with evidence of antenatal stroke before the onset of pulmonary disease. He presented at 6 weeks of age with spontaneous pneumothorax. Focal cystic changes were seen on imaging studies of the lungs. There was no prior history of mechanical ventilation. Prior chest X-rays did not show cystic changes. He subsequently underwent resection of the affected lung areas. Pathologic examination revealed persistent PIE with cystic expansion, pleural blebs, and reactive pleuritis, as well as subpleural air-space enlargement. The patient did well postoperatively and was discharged home without further problems. This case demonstrates that PIE can occur in an infant without any history of mechanical ventilation, suggesting the need for a high index of suspicion for PIE, even in nonventilated and spontaneously breathing term neonates. PIE should be included in the differential diagnosis of cystic lung lesions in all young infants.